1

65

4
2



2

4
1

fasciculation
Motor neuron disease (MND) Amyotrophic Lateral Sclerosis (ALS)

ALS

T 36.8C
PR 108 regular
RR 28
BP 140/70
alert, rapid laboured breathing with accessory muscles
Heart sounds : normal
Lungs : clear
Speech : soft, slurred
Tongue : could not be protruded, wasting and fasciculation
were present
Upper Limbs : moderate wasting and weakness of deltoids,
biceps, triceps and fore arm muscles of both sides
marked wasting and weakness of small muscles of
both hands.
Fasciculation ++ in deltoids
Lower Limbs : moderate wasting and weakness of quadriceps,

gastrocnemius, anterior tibial and peroneal muscles

of both sides
slight increased in tone of both legs
R
L
Reflexes : SJ +++
+++
BJ +++
+++
TJ +++
+++
Vo +++
++
AJ
P

Sensation in all modalities : normal

()
(striated muscle)
(ventilatory failure)

MND ALS fasciculation
lower motor neuron motor
Fasciculation
nuclei anterior horn cells
motor nerve root peripheral (motor) nerve
benign fasciculation Deep
reflexes tone 2 upper
motor neuron Babinski response Plantar response flexor
extensor

upper motor neuron 1 5 Babinski sign
sensation modality MND
diaphragm external
intercostal muscles
internal intercostal muscles
maximum
voluntary ventilation (MVV) inspiratory expiratory pressure (IP/EP)
residual volume (RV)

sniff nasal
pressure
MND
MND ALS

 Charcot
MND 19
Aran progressive
muscular atrophy lower motor neuron .. 1850

Aran Charcot Aran

fibrillary contractions ( fasciculation )
Aran
MND
5
10 Chancellor Warlow
MND

60
MND
diagnostic test

20-30
40 .. 1983 ( 3)
2
20 (
2) MND
10
67 2-3 50

MND

motor neuron
oxidative stress (glutamate) excitatory
neurotransmitter
motor neuron
Riluzole
MND The Cochrane review Miller
100 12
26 39 (Weighted event rates)
15

Gabapentin 400-600 3

800-1000
cyclooxygenase-2 (COX-2)
SC 236 celecoxib


SC 236 motor neuron
COX-2 prostaglandins
astrocyte glutamate
free
radical glutamate motor neuron
ALS COX-2 celecoxib
( 16)

non-invasive
positive pressure ventilation NIPPV /
bulbar
dysfunction

( 7 9)

1. AL-CHALABI A, Nigel Leigh P. Recent advances in amyotrophic lateral

sclerosis. Curr Opin Neurol 2000; 13: 397-405.
2. Vejjajiva A, Foster JB and Miller H. Motor neuron disease. A clinical
study. J Neurol Sci 1967; 4: 299-314.
3. Vejjajiva A. Motor neuron disease. A short analysis of 40 patients
from Thailand. In Amyotrophic Lateral Sclerosis in Asia and Oceania.
Eds. Chen KM and Yase Y. Publisher: Shyan-Fu Chou, National
Taiwan University. Taipei. 1983; pp. 125-30.
4. : 2542
5. Williams AC. Motor Neuron Disease. Chapman and Hall Medical.
London. 1994
6. Wokke JHJ. Diseases that masquerade as motor neuron disease.
Commentary. Lancet 1996; 347: 1347-8.
7. Rowland LP. Whats in a name? Amyotrophic lateral sclerosis,
motor neuron disease, and allelic heterogeneity. Editorial.
Ann Neurol 1998; 43: 691-693.
8. Chancellor AM, Warlow CP. Adult onset motor neuron disease :
worldwide mortality, incidence and distribution since 1950.
J Neurol Neurosurg Psychiatry 1992; 55: 1106-15.

9. Hadjikoutis S, Eccles R, Wiles CM. Coughing and choking in motor

neuron disease. J Neurol Neurosurg Psychiatry 2000; 68: 601-4.
10. Tomik B, Nicotra A, Ellis CM, Murphy C, Rabe-Hasketh S, Parton M,
Shaw CE, Leigh PN. Phenotypic differences between African and
white patients with motor neuron disease : a case-control study.
J Neurol Neurosurg Psychiatry 2000; 69: 251-53.
11. Fitting J-W, Paillex R, Hirt L, Aebischer P, Schluep M. Sniff Nasal
Pressure : A sensitive respiratory test to assess progression of
Amyotrophic Lateral Sclerosis. Ann Neurol 1999; 46: 887-93.
12. Parton MJ, Lyall R, Nigel Leigh P. Motor neuron disease and its
management. J R Coll Physicians Lond 1999; 33: 212-8.
13. Wokke J. Riluzole. Drug Profile. Lancet 1996; 348: 795-799.
14. Lacomblez L, Bensimon G, Nigel Leigh P, Guillet P, Meininger V,
for the Amyotrophic Lateral Sclerosis / Riluzole Study Group II.
Lancet 1996; 347: 1425-31.
15. Miller RG, Mitchell JD, Moore DH. Riluzole for amyotrophic lateral
sclerosis (ALS) / motor neuron disease (MND). Cochrane Review,
latest version 26 October 1999. In : The Cochrance Library.
Oxford : Update Software. ACP Journal Cub. 2000; 133: 54.
16. Simmons Z, Bremer BA, Robbins RA, Walsh SM, Fischer S.
Quality of life in ALS depends on factors other than strength and
Physical function. Neurology 2000; 55: 388-92.
17. Shaw PJ. Calcium, Glutamate, and Amyotrophic Lateral Sclerosis :
More evidence but no certainties. Editorial. Ann Neurol 1999;
46: 803-4.
18. Drachman DB, Rothstein JD. Inhibition of cyclooxygenase-2 protects
motor neurons in an organotypic model of amyotrophic lateral
sclerosis. Ann Neurol 2000; 48: 792-95.

37

1

1



/ 1-2

generalized pruritis


1.
(jaundice) primary
biliary cirrhosis
2. polycythaemia rubra vera
3.

myxoedema
pruritis vulva candida
4. Malignancy lymphoma T-cell leukaemia
5. advanced uraemia
dialysis
6.
7. chloroquine
8.

2 primary biliary cirrhosis

6-7


T 37.2C
PR 72 regular
BP 120/80
not pale, mildly icteric sclerae
Abdomen : soft, Liver and Spleen not palpable
Skin : xanthelasma present and also prurigo nodularis and simplex
()
xanthelasma primary
biliary cirrhosis prurigo nodularis and simplex

Liver function alkaline phosphatase serum
cholesterol

Serum T.B. 2.4
DB 1.1
Cholesterol 347 mg/100 ml
Alk. Phos 1290 (normal 20-90)
SGOT 187 (normal 14-33)
PT 203 normal (6-36) GGT 894 (normal 5-38)
T.P 83.2 g/L Alb 44.3 g/L
Ultrasound : small cyst at upper pole of kidney
tiny stone at middle pole of L kidney
no other abnormality
Hct 35 Hb 10.7 WBC 5800 P 60 L32 E 3
Platelets 294,000 RBC 4.35 x 106
Micro: few Hypo: few Target cells few
PTT 30.7sec (25-38)
PT 10.7sec (11-15) TT 8.4sec (7-11)
Cr 0.8
BUN 25
Needle Liver biopsy : intense infiltration with chronic inflammatory cells
in the triads together with loss of biliary
ductules.
The interface between liver
parenchyma and trids are sharply demarcated.
The findings are highly compatible with primary biliary cirrhosis.
ERCP : normal pancreatic duct and common bile duct.
and L intrahepatic ducts were irregular with some area
of diminished circumferences. The findings are compatible
with Primary sclerosing cholangitis.

Primary biliary cirrhosis

 Pruritis
(itching)

epidermis dermis
histamine, bradykinin bile acids intrahepatic extrahepatic
cholestasis bile acids
Primary biliary
cirrhosis (PBC)

37C 40 - 41C
PBC
cholestasis
Pruritis
opioids
Opioid

receptors ( )
opioid agonists morphine opioid antagonist
cholestasis opioidergic tone
cholestasis opioid receptors
up-regulation opioid antagonist nalmefene
cholestasis withdrawal reactions
codeine pruritis
PBC
endogenous opioids
/
opioids
neuromodulation opioidergic receptors
opioid antagonist withdrawal
morphine codeine non-antagonistic opioids receptors
endogenous opioids competitive inhibitors
withdrawal Buprenorphin partial -agonist
cholestasis

Primary biliary cirrhosis Hanots cirrhosis

major histocompatibility complex (MHC) II
HLA-DR8 auto-immune autoantibodies
mitochondria nuclei

Oxford Textbook of
Medicine 2 !

1. Bassendine MF. Primary biliary cirrhosis. In Oxford Textbook of

Medicine. Third Edition. Vol. 2 Eds. Weatherall DJ, Ledingham JGG
and Warrell DA. Oxford Medical Publications. 1996; pp. 2074-77.

2. Neuberger J. Primary Biliary Cirrhosis. Lancet 1997; 350: 875-79.

3. Jones EA, Weissenborn K. Neurology and the liver. J Neurol
Neurosurg Psychiatry 1997; 63: 279-293.
4. Juby LD, Wong VS, Losowsky MS. Buprenorphin and hepatic
pruritis. Br J Clin Pract 1994; 48: 331.
5. Jones EA, Bergasa N. Why do cholestatic patients itch? Gut 1996;
38: 644-45.
6. Greaves MW, Wall PD. Pathophysiology of itching. Lancet 1996;
348: 938-40.
7. Zylicz Z, Krajnik M. Codeine for pruritis in primary biliary cirrhosis.
Lancet 1999; 353: 813.
8. N Engl J Med. Case Records of the Massachusetts General Hospital.
Weekly Clinicopathological Exercises. Eds. Scully RE, et al. 2000;
342: 1893-1900.
9. Fleischer Jr AB. The Clinical Management of Itching. Parthenon
Publishers. Carnforth. 2000; 186 pp.
10. Bussaratid V, Wilairatana P, Krudsood S, Silachamroon U, Walsh DS,
Looareesuwan S. Frequency of pruritis in Plasmodium
vivax malaria patients treated with chloroquine in Thailand. In press.

3

31

5
7


1

1
2-3

(brain stem)
vestibular nuclei
cranial nerve nuclei 6 7 10
Acute brain stem lesion

arterio-venous malformation
demyelination vascular

PR 80 regular BP 140/80 mm. Hg

Heart sounds were normal. No murmur. No neck bruit. No neck
stiffness. Abnormal neurological signs included dysarthria, partial ptosis Unequal
pupils. ( 3 mm L 4 mm reactive to light) vertical nystagmus and horizontal
rotatory nystagmus to
LR palsy
LMN facial palsy
Palate moved to L on phonation
Limbs : Tone and Power were normal. Sensation to pin prick diminished on
L side
Babinski response L

 ()

Cranial nerves 6 7 vertical nystagmus

spastic dysarthria Cranial nerve 10
long tract signs sensory motor

pons medulla
vertebro-basilar
posterior inferior cerebellar artery (PICA) Horners
syndrome mid lower cranial nerve sensory tract
motor tract
lateral medulla vertebral
artery incomplete basilar artery occlusion

arterio-venous malformation (AVM) Aneurysm
1 subarachnoid haemorrhage WBC
eosinophil MRI
MRI posterior

paramedian ponto-medullary and lower pontine infarction vertebral artery

dissection MRA distal cervical proximal
part basilar artery shaggy appearance

Ponto-medullary infarction
Right vertebral artery dissection

Vertebral artery dissection (VAD)

MRI MRA Dissection
(tunica intima)
(tunica media)

arteriosclerosis

connective tissue (
5)


VAD
( 7) VAD
trauma
chiropractor

1. Hart RG. Vertebral artery dissection. Neurology 1988; 38: 987-89.

2. Hart RG, Easton JD. Dissection and trauma of cervico-cerebral arteries.
In Stroke. Pathophysiology, Diagnosis and Management. Vol 2. Eds.
Barnett HJM, Stein BM, Mohr JP, Yatsu FM. Churchill Livingstone.
New York. 1986; pp. 775-88.
3. Furie KL, Ogilvy CS, Smrcka M. et al. Cerebrovascular disease.
In Atlas of Clinical Neurology. Ed. Rosenberg RN.
Butterworth-Heinemann. Boston. 1998; pp. 5.45-5.46.
4. Bloem BR, Lammers GJ, Van Buchem MA. MRI and vertebral artery
dissection. J Neurol Neurosurg Psychiatry 1999; 67: 691-92.
5. Burneo JG, Shatz R, Papamitsakis NIH, Mitsias PD. NeuroImages.
Amusement park stroke. Neurology 2000; 55: 564.
6. d Anglejean - Chatillon J, Ribeiro V, Mas JL, et al. Migraine : a risk
factor for dissection of cervical arteries. Headache 1989; 29: 560-61.
7. Brandt T, Hausser I, Orberk E, et al. Ultrastructural connective tissue
abnormalities in patients with spontaneous cervico cerebral artery
dissections. Ann Neurol 1998; 44: 281-85.
8. Tzourio C, Amrani ME, Robert L, Alprovitch A. Serum elastase activity
is elevated in migraine. Ann Neurol 2000; 47: 648-51.

9. Crum B, Mokri B, Fulgham J. Spinal manifestations of vertebral artery

dissection. Neurology 2000; 55: 304-06.

4

51

2

3
5
(pneumonia)
rhonchi crepitations Penicillin
Gentamicin 2 Doxycycline 2

Chloramphenicol

1) lobar pneumonia pneumonitis Strept.
pneumoniae rhonchi
wheezes crepitations
classic lobar consolidation bronchial breathing
pneumococcal pneumonia

2) immunocompromised host
Community-acquired pneumonia B.
pseudomallei

Penicillin Gentamicin 2 Doxycycline


T 38.5C
RR 36
PR 100 regular BP 120/80 mm/Hg
Chest: both sides expanded equally on inspiration
rhonchi all over both lungfields and crepitations over L
lung
Extremities: erythematous nodule 0.5 cm. in diameter at L arm,
two nodules of the same size at thigh

broncho-pneumonia

nodular erythema
Melioidosis
subcutaneous cutaneous abscesses

PA Chest Xray : haziness in the superior segment of L lower lobe
Hb 11.5
Hct 35 WBC 9200 N 84 L14 M2 Platelets
219,000
FBS 180 anti - HIV -ve
Urine : WBC 1-5 per HPF, otherwise no abnormality
Blood culture : no growth
P. pseudomallei / Ab/ PHA : negative / 1:80
one week later positive / 1:160
()
Melioidosis
1 1:80 1:160

4-5
()
Burkholderia pseudomallei
virulence ( Ara B. pseudomallei)

Melioidosis
Thalassemia


imipenem

cefoperazone/
sulbactam cotrimoxazole ceftazidime
co-amoxiclav (20 ), fluoroquinolones (15 ),
doxycycline (12-18 ) ciprofloxacin + azithromycin (12 )
Cotrimoxazole + doxycycline
20

 Melioidosis

1. Chaowagul W et al. Melioidosis : a major cause of community-acquired

septicemia in Northeastern Thailand. J Inf Diseases. 1989; 159: 890-9.
2. Leelarasamee A, Bovornkitti S. Melioidosis : review and update.
Review of Infectious Diseases 1989; 11: 413-25.
3. Dance DAB. Melioidosis. In Oxford Textbook of Medicine. Third Edition.
Vol 1. Eds. Weatherall DJ, Ledingham JGG and Warrell DA. Oxford
Medical Publications. Oxford. 1996. pp. 590-5.
4. . Melioidosis
.. 2538-2542 (Update on Melioidosis 1995-1999)
Melioidosis 2
RDC 3/04/2542. 2542

26

1
3

6

non - icteric sclerae

dysarthric, mask like face
coarse tremor of outstretched hands and arms at shoulders
rigidity of all limbs DTRs ++ ++
no Babinski response
midline - abdominal incisio scar
spider naevi over chest

2
1.
Parkinsonism
basal ganglia
2. 20
porta-caval shunt
spider naevi lenticular
degeneration Wilsons Disease hepatocerebral degeneration chronic portal-systemic encephalopathy
tremor Wilsons
Disease
chronic portal-systemic encephalopathy
coma

flapping tremor pyremidal signs tremor Wilson disease

wing-beating tremor Babinski response

Kayser-Fleischer ring (KF)


KF ring 2

Wilsons disease

serum caeruloplasmin 3.0 /ug/dl ( )

Serum copper 19 /ug/dl (
)
copper 24 82 /ug/dl ( 2-80)
Serum iron 103 mg/dl total iron binding capacity 289 mg/dl
MRI ill-defined areas of non-enhanced iso- / hypersignal T1 T2 dorsal brain stem substantia nigra mild, diffuse
cerebral atrophy
Wilson disease 40
40 movement disorder (dystonia, parkinsonism) 20

Wilson disease autosomal recessive mutations in a widely

expressed gene for a P type ATPase. This nuclear-encoded protein is present in the
trans-Golgi network and is also transported to mitochondria although its precise
location and function within that organelle are unknown.
()

KF ring
Wilson
slit-lamp
Wilson disease autosomal recessive
(mutation) gene chromosome 13 encode P type
ATPase transport copper encode nucleus trans-Golgi network
mitochondria organelle

Wilson 1 3 4
copper copper
free form
basal ganglia (cirrhosis)
basal ganglia

 Hepato-lenticular degeneration Wilsons disease

Wilson 19

.. 1883 Westphal 2

pseudo-sclerosis disseminated multiple sclerosis
2 nystagmus .. 1906 Sir
William Gowers Queens Square
London tetanoid chorea
chorea tetanus liver
cirrhosis ..
1912 Kinnier Wilson Gowers Queens Square
natural history

hepato-lenticular degeneration
Wilsons disease
Queens Square London
Sir William
Gowers Dr. Kinnier Wilson
copper
chelating agent B.A.L. British Anti-Lewisite copper
John Cumings
Penicillamine Wilsons disease

Dr. John Walshe

Walshe

Wilsons disease Dr. John Walshe Sir

Francis Walshe
20
Queens Square

.. 2508 20

Thalassaemia Hb E
hypersplenism
Kayser Fleischer (KF) Ring

cirrhosis slitlamp KF ring 30

2

25

Wilson KF ring
40 Parkinson
Wilsons disease
.. 1902 Kayser KF ring


Multiple Sclerosis .. 1909 Fleischer
KF ring Kayser

Oculogyric crisis

Parkinson (Encephalitis lethargica)
neuroleptics

Oxford
Wilson
Kinnier Wilson .. 1913
Wobbly handwriting The Lancet !

vacuous smile
Wilson
30

20 3 (11)

(14-16)
(17)

* 1) Gowers WR. Rev Neurol Psychiat 1906; 9: 249.

** 2) Wilson SAK. Brain 1912; 34: 295.
** 3) Cumings JN. Brain 1948; 71: 410.
4) Kelly R. Clinical Features of Disorders of Copper Metabolism.
In Biochemical Aspects of Neurological Disorders. Eds. Cumings JN
and Kremer M. Blackwell Scientific Publications. Oxford. 1959;
pp. 108-116.
5) Cumings JN. Biochemical Aspects of Copper Metabolism. In Biochemical
Aspects of Neurological Disorders. Eds. Cumings JN and Kremer M.
Blackwell Scientific Publications. Oxford. 1959; pp. 98-107.
6) Hyman NM, Phuapradit P. Reading difficulty as a presenting symptom in
Wilson disease J Neurol Neurosurg Psychiatry 1979; 42: 478-80.

7) Lee MS, Kim YD and Lyoo CH. Oculogyric crisis as an initial

manifestation of Wilsons disease. Neurology 1999; 52: 1714-15.
8) Phuapradit P. Kayser Fleischer ring. Rama Med J 1995; 18: 125.
9) Teeprasertsuk W, Kurathong S, Phuapradit P. Wilsons disease : a clinical
study. Rama Med J 1997; 20: 84-90.
10) Walshe JM, Yealland M. Wilsons disease : the problem of delayed
diagnosis. J Neurol Neurosurg Psychiatry 1992; 55: 692-96.
11) Heckmann JG, Lang CJG, Neundrfer B, Kchle M. Kayser-Fleischer
corneal ring. NeuroImages. Neurology 2000; 55: 280.
12) Goyal V, Tripathi M. Sunflower cataract in Wilsons disease.
J Neurol Neurosurg Psychiatry 2000; 69: 133.
13) del Rosario MAF, Davis MM, Chong SKF. Wobbly handwriting.
Case Report. Lancet 1998; 351: 336.
14) Pyeritz RE. Genetic Heterogeneity in Wilson Disease : Lessons from
Rare Alleles. Ann Intern Med 1997; 127: 70-71.
15) Maier Dobersberger T, Ferenci P, Polli C, Bala P, Dienes HP,
Kaserer K, et al. Detection of the His 1069 Gln Mutation in Wilson
Disease by Rapid Polymerase Chain Reaction. Ann Intern Med
1997; 127: 21-26.
16) Lutsenko S, Cooper MJ. Localization of the Wilson disease protein
product to mitochondria. Proc Natl Acad Sci USA 1998; 95: 6004-09.
17) Gu M, Cooper JM, Butler P, Walker AP, Mistry PK, Dooley JS,
Schapira AHV. Oxidative-phosphorylation defects in liver of patients
with Wilsons disease. Lancet 2000; 356: 469-74.
18) Stuerenburg HJ, Eggers C. Early detection of non-compliance in
Wilsons disease by consecutive copper determination in cerebrospinal
Fluid. J Neurol Neurosurg Psychiatry 2000; 69: 701.
* Wilson J. Genetically Determined Neurological Diseases in Children.
In Biochemical Aspects of Neurological Disorders. Third Series. Eds.
Cumings JN

and Kremer M. Blackwell Scientific Publications. Oxford. 1968; pp 250267.

**

4) 5)

52

2 3
25

35 1 B
Wertheims Hysterectomy
2-3

3
10 Norfloxacin Motilium
2

T 37.2C
PR 100 regular
RR 36
marked pitting oedema of both legs up to knees
periorbital oedema - slightly pale
Heart sounds normal
Lungs clear
Abdomen : no mass felt (Liver not palpable)

BP 190/110

2
(small intestine)
10

/

generalized oedema
nephrotic syndrome

serum albumin
albumin (protein-losing gastroenteropathy)

nephrotic syndrome
(1) proteinuria ? (2) serum albumin ?
(3) serum
cholesterol ?


Urine : protein 3+
RBC 40-50/HPF
WBC 10-20/HPF
Serum albumin/globulin 2.6/2.1
BUN 12
Cr 1.0
Serum Cholesterol 317
complement CH50 12%
st
ANCA ve Anticardiolipin antibodies ve
ESR 86 mm/1 hour
Cryoglobulin +
()
Nephrotic syndrome Nephritic
features RBC red cell cast
glomerulitis
membranoproliferative glomerulonephritis (MPGN)

SLE
serum
complement ESR cryoglobulin
MPGN
virus C (HCV)
antibody virus
blood transfusion kidney
biopsy Norfloxacin

Blood : anti HBS + ve anti HBC + anti HCV +

kidney biopsy
Liver biopsy chronic persistent
hepatitis

Nephrotic Syndrome from MPGN

Hepatitis C virus infection

1. Orth SR, Ritz E. The Nephrotic Syndrome. N Engl J Med 1998;

338: 1202-10.
2. Couser WG. Glomerulonephritis. Lancet 1999; 353: 1509-15
3. Johnson RJ, Willson R, Yamabe H, et al. Renal manifestations of hepatitis
C virus infection. Kidney Int 1994; 46: 1255-62.

4. Misiani R, Bellavita P, Fenili D, et al. Hepatitis C virus infection in

patients
with essential mixed cryoglobulinemia. Ann Intern Med 1992;
17: 573-84.
5. Yamabe H, Johnson RJ, Gretch DR, et al. Hepatitis C virus infection and
membranoproliferative glomerulonephritis in Japan. J Am Soc Nephrol
1995; 6: 220-27.
6. NIH Conference : Pathogenesis, Natural History, Treatment and
Prevention of Hepatitis C. Ann Intern Med 2000; 132: 296-305.

24

4

5

xray

impaired sensation to pin prick

Masseter muscle
Numb chin syndrome

mental nerve mandibular nerve

metastatic tumour mandible

trauma MRI
biopsy


MRI : diffuse infiltration of bone marrow of the mandible,
bilateral pterygoid plates, greater sphenoid wings and right petrous apex
with prominent
L anterior submandibular lymph nodes and abnormal enhancing buccal and lingual
mandibular mucosa, compatible with marrow replacement process such as
lymphoma, leukemia, histiocytosis X, metastasis or less likely osteomyelitis.
Biopsy : osteomyelitis

Numb chin syndrome

? neoplasm ? osteomyelitis

Sir Charles Bell

.. 1820 Bells palsy
3

.. 1954 Syndrome of the numb chin

Calverley and Mohnac .. 1963

(Numb cheek syndrome)

lesion infra-orbital nerve Numb cheek limp lower lid syndrome

infra-orbital nerve
facial nerve 2 malignant
infiltration Numb chin syndrome

1. Furukawa T. Charles Bells description of numb chin syndrome.

Neurology 1988; 38: 331.
2. Furukawa T. Numb chin syndrome in the elderly. J Neuro Neurosurg
Psychiatry 1990; 53: 173.
3. Bruce KW, McDonald JR. Metastatic adenocarcinoma of the mandible
from the sigmoid colon : report of a case. Oral Surg 1954; 7: 772-77.

4. Castigliano SG and Rominger CJ. Metastatic malignancy of the jaw.

Am J Surg 1954; 87: 496-507.
5. Sterling JA, Tassman GC, Goldsmith R. Solitary metastatic lesion to the
gingivae masquerading as inflammation. Oral Surg 1954; 7: 403-05.
6. Calverley JR, Mohnac AM. Syndrome of the numb chin. Arch Intern
Med 1963; 112: 819-21.
7. Furukawa T. Buried contributions of the last century. In A Short
History of Neurology. The British Contribution 1660-1910 Ed. Rose FC.
Butterworth Heinemann, Oxford. 1999; pp. 132-144.
8. Pryse - Phillips W. Companion to Clinical Neurology. Little, Brown and
Company. New York. 1995; pp. 624-25.

36

2
3

eosinophilia ? !

T 37C
PR 84 regular
Healthy looking, no jaundice

RR 20

B.P. 110/70

Abdomen
soft, no ascites
Liver : palpable, its edge was 2 finger breadth below right
costal margin. A slightly tender mass of one
inch
in diameter was felt in the liver.
no other abnormalities.
()


3-4
(Liver fluke)
Opisthorchis viverrini
Clonorchis
sinensis O. viverrini
Fasciola hepatica
strongyloid

trichiniasis, toxocariasis,
echinococcus granulosa cysticercosis
eosinophilia
ultrasound

Hb 11.0
Hct 34.6
WBC 9760/cu mm. N 56% E 25% B 1%
L 10% M 8%
Platelets 353,000
PTT 37 seconds (normal 33-44)
PT 13.5 seconds (12-14.5)
INR 0.94
Stool examination : soft, brown, faeces, no occult blood,
no ova, cyst or parasite
Ultrasound abdomen : a space occupying lesion of 5.4 x 7.8 cm
in the liver probably an abscess
Liver aspiration
brown, turbid fluid.
Microscopy showed some red blood cells, numerous WBCs and numerous CharcotLeyden crystals
Culture : no growth
E. histolytica negative
()
Fasciola indirect immunofluorescence
counterimmunoelectrophoresis ( 3)

wedge resection of the liver

cholecystectomy

 multiple abscesses with severe eosinophilia

Cestode fluke 1.4 x 0.65 x 0.1 cm chronic cholecystitis
gallstone 1 Praziquantel

Fasciola hepatica

1. Bunnag D, Cross JH, Bunnag T. Liver fluke infections. In Hunters

Tropical Medicine and Emerging Infectious Diseases. Eighth Edition.
Ed. Strickland GT. WB Saunders Company. Philadelphia. 2000.
pp. 844-47.
2.
1 :
2539 232-46
3. La Pook JD, Magun AM, Nickerson KG, Meltzer J1. Case Report.
Sheep, watercress, and the Internet. Lancet. 2000; 356: 218.

42 .

2 1

2

2

T 38C
PR irregular 108/min
BP 130/80 mm.Hg
Slightly drowsy, but could still answer questions, no dysarthria
ptosis L , Pupils L < R,
reacted normally
- Eye movements were full, but there was nystagmus on horizontal
gaze bilaterally and on vertical gaze
- Hearing impaired in both ears
- flaccid weakness of all limbs, R > L
- bilateral Babinski response

vascular (arterial) occlusion haemorrhage
lesion brain stem vertical
nystagmus Horners syndrome quadriparesis hearing impairment

Embolic infarction brain stem
?

PR : totally irregular i.e. atrial fibrillation

PMI 5th ICS 1 inch lateral to mid-clavicular line
M1 , grade III mid-diastolic rumbling murmur at apex, opening snap
heard
no evidence of emboli in eyes and elsewhere
()

 mitral stenosis atrial fibrillation (AF)

embolus vertebro-basilar system pontomedullary infarction embolus infective embolus
bacterial endocarditis (BE) evidence embolic
phenomenon BE 90
AF

red blood cells

Echo cardiogram vegetation
Haemoculture

Embolic brain stem (pontine) infarction

Atrial Fibrillation
Rheumatic mitral stenosis

2 central lesion brain stem

internal
auditory artery basilar artery

VIII nerve nuclei 2 brain stem
quadrigeminal branch superior cerebellar artery pons 2
glioma multiple sclerosis
100% cochlear nerve central
process axon brain stem ( medulla oblongata pons)
dorsal cochlear nucleus ventral cochlear nucleus
pons fibres dorsal
ventral nuclei
basilar artery

Cortical Deafness
nerve fibres
synapse cells dorsal ventral cochlear nuclei pons
fibres motor sensory tract
auditory cortex Heschels transverse gyrus temporal lobe auditory
cortex nerve fibres 2 Heschels gyrus

 infarct gyrus 2
Cortical deafness infarction
Heschels gyrus (Khurana 1981)
pathway cranial nerve 8
brain stem visual pathway motor
sensory tract

1. Khurana RK, ODonnell PP, Suter CM, Inayutallah M. Bilateral deafness

of vascular origin. Stroke 1981; 15: 521.
2. Bramwell E. A case of cortical deafness. Brain 1927; 50: 579-580.
3. Haymaker W. Bings Local diagnosis in neurological diseases. 15th Edition
C.V. Mosby Company. 1969 pp 203-205.
4. Earnest MP, Monroe PA, Yarnell PR. Cortical deafness : demonstration
of the pathologic anatomy on CT scan. Neurology 1977; 22: 1172-75.
5. Jerger J, Weikers NJ, Sharbrough FW, Jerger S. Bilateral lesions of the
temporal lobe. Acta Oto-Laryngol Supp. 1969; 258: 1-51.
6. Leicester J. Central deafness and subcortical motor aphasia. Brain Lang
1980; 10: 224.

10

32 .

2




3 Carbamazepine

T 37C
PR 72 regular
BP 130/80 mm.Hg
Ptosis L
Pupils L > R
L 3 mm.
2 mm.
L reacted to light sluggishly
Eye movements : L eye LR full, SR, IR, MR < 80% of normal
eye : full
Other cranial nerves intact
no other abnormal signs

painful 3rd nerve palsy

3
3rd nerve palsy
pupil cranial nerve
3 posterior communicating aneurysm tentorial
herniation 3rd nerve
palsy vasa nervorum

cranial neuritis ( polyneuritis cranialis cranial nerve
1 ) Tolosa-Hunt Syndrome


1) ESR Tolosa-Hunt
Giant cell arteritis
2) VDRL TPHA rule out syphilis
3) Fasting Blood Sugar

 ESR 2 3
Tolosa-Hunt Syndrome cortico-steroid
-2 ocular palsy 4-5
MRI CT scan
Sinusitis nasopharyngeal cancer

VDRL TPHA

VDRL TPHA non-reactive
Urine
Fasting Blood Sugar 101 mg% WBC 5400/cu.mm. N 74% L 23% M 3%
Eosinophil
ESR 57 mm. 1
clear fluid RBC 50
protein 66 mg% glucose 59 mg%

Painful third cranial nerve palsy, cranial neuritis (Tolosa-Hunt

syndrome)

Prednisolone 60 mg 2

3

3rd cranial palsy

Tolosa-Hunt vasa nervorum nerve infarct

/ arteriosclerosis
posterior communicating aneurysm carcinoma naso-pharynx
cranial nerve 6 3
Tolosa-Hunt Syndrome

1. Hunt WE, Meagher JN, Le Fever HE, Zeman W. Painful ophthalmoplegia

- its relation to indolent inflammation of the cavernous sinus. Neurology
(Minneap.) 1961; 11: 56-62.

2. Tolosa E. Periarteritic lesions of the carotid siphon with the clinical

features of a carotid infraclinoidal aneurysm. J Neurol Neurosurg Psychiat
1954; 17: 300-302.
3. Symonds CP. Recurrent multiple cranial nerve palsies. J Neurol
Neurosurg Psychiat 1958; 21: 95-100.
4.Ratanavale GS. Cranial polyneuritis A distinct clinical entity. Proc Aust
Ass Neurol 1968; 5: 527-529.
5. Steele JC, Vasuvat A. Recurrent multiple cranial nerve palsies : a
distinctive syndrome of cranial polyneuropathy. J Neurol Neurosurg Psychiat
1970; 33: 828-832.
6. Juncos JL. Beal MF Idiopathic cranial polyneuropathy. A fifteen-year
experience. Brain 1987; 110:197-211.
7. Chakravarty K, Scott DGI. Polyneuritis cranialis and C-ANCA : is it
limited Wegeners granulomatosis. J R Soc Med 1993; 173-174.
8. Rucker CW. Paralysis of the third, fourth and six cranial nerves.
Am J Ophth 1958; 46: 787-794.

11

42

8
6
deep vein thrombosis (DVT)

PR 72 regular BP 190/100 mm.Hg

Heart sounds were normal. No murmur
Flaccid paralysis of arm and leg without deep reflexes over half
of face, body, arm and leg
Babinski response sensory loss to pin prick raised multiple
symmetrical erythematous papules over both legs

3
1) acute hemiplegia
vascular L cerebral hemispheric
infarct
carotid middle cerebral artery occlusion
haemorrhage basal ganglia

atherosclerosis
2) skin lesions 2
2 erythema nodosum
Tuberculosis, sarcoidosis, betahaemolytic streptococcal infection rheumatic fever, chronic meningococcal
septicaemia, coccidiodomycosis, histoplasmosis
ulcerative colitis Behets disease
Behet Recurrent
oral aphthous ulcers genital ulcers uveitis

3) deep vein thrombosis Behet

 MRI CT

CT ventricle
fronto-temporo-parietal infarct

Behets disease

Behet
10 20

meningitis
meningoencephalitis brain-stem lesion
dementia
Behet

(80-370 ) 13.5-20

1 100,000
Silk Route
Silk Route Disease
Behet
HLA-B51 allele
HLA allele Behet
HLA-B51

HLA-B51 (5101, 5103 5105 subtypes) HLA-B51 7 14
(Relative risky HLA-allele Behet 15.7)
Behet Hippocrates
5 3
Tsung O. Cheng Washington DC
The New England Journal of Medicine 24 ..
2000 ( 588)
There were other forms of fever.Many developed
aphthae, ulcerations. Many ulcerations about the genital parts.Watery
ophthalmies of a chronic character, with pains; fugus excretions of the eyelids
externally, internally, which destroyed the sight of many personsThere
were fungous growths on ulcers, and on those localised on the genital organs. Many
anthraxes through the summer.other great affections; many large herpetes.

 Behet
1. Sakane T, Takeno M, Suzuki N and Inaba G : Behets Disease.
N Engl J Med 1999; 341: 1284-91.
2. Pickering MC and Haskard DO. Behets syndrome. JR Coll Physicians
Lond 2000; 34: 169-77.
3. Buchanan JAG, Morris A, Fortune F. Case Report. A mechanic who
coughed up blood for 15 months. Lancet 1997; 350: 28.
4. Nantiruj K, Verasertniyom O, Totemchokechyakarn K, Janwityanujit S
and Vatanasuk M. Behets disease in Ramathibodi Hospital.
Rama Med J 1998; 21: 54-59.
5. Akman-Demir G, Baykan-Kurt B, Serdaroglu P et al. Seven year
follow up of neurological involvement in Behet syndrome
Arch Neurol 1996; 53: 691-4.
6. Akman-Demir G, Serdaroglu P, Tasci B. The Neuro-Behcet Study Group.
Clinical patterns of neurological involvement in Behcets disease:
evaluation of 200 patients. Brian 1999; 122: 2171-81.
7. Kidd D, Steuer A, Denman AM et al. Neurological complications in
Behets syndrome. Brain 1999; 122: 2183-94.
8. Mead S., Kidd D, Good C and Plant G. Behets syndrome may
present with partial seizures. J Neurol Neurosurg Psychiatry
2000; 68: 392-93.
9. Letters to the Editor. N Engl J Med 2000; 342: 587-89.

12

43
2
10 4
19
2 15

carpopedal
spasm
tetany neuromuscular excitability ionized calcium

parathyroid
48

(carpopedal spasm)
2 metacarpophalangeal joints
main d accoucheur
risus
sardonicus

Wt 51 kgs. Ht 150 cms.

Thyroidectomy scar at the neck
Carpalpedal spasm
+
Chvosteks sign
+
Trousseaus
+
PR 72 regular
Heart sounds normal
no murmur
Eyes : no cataract

BP 120/80 mm. Hg.

Nervous system : no abnormality

()
hypoparathyroidism
Chvosteks sign
facial nerve excitability
Trousseaus sign sphygmomanometer cuff
systolic 10
2-3 main d accoucheur ( 6)
Tetany
D
Malabsorption syndrome respiratory alkalosis hyperventilation
metabolic Alkalosis alkali
cystic fibrosis F508
CFTR
mutation 33 tetany
chloride potassium
( 8)

Total serum Ca++ 6.6 mg% Po4 5.6 Mg 1.2
K+ 3.2
albumin 41.8 g/l
Na+ 148
Cl- 106
HCO3 26.0
ECG QTc = 0.434
10% Calcium gluconate 30 ml. 5%
D/W 500 ml. IV drip 40 ml.
Calcium carbonate Vitamin D2
2 serum Ca++ 8.7, Po4 4.8

serum
hypopara-thyroidism ionized Ca++
electrolytes
serum intact parathyroid hormone 20-90 ug/ml

 tetany
parathyroid
trauma
parathyroid hormone
intracranial
hypertension papilloedema
vertigo
Ca++ otolith posterior semi-circular canal Benign
Paroxysmal Posterior Canal Vertigo hypoparathyroidism
candidiasis
Xray calcification basal ganglia
30
Calcium metabolism
1, 2 3 4 5
Oxford
Oxford
30
Calcium Metabolism Hypoparathyroidism
12 clinical science 30
4

1. Marx SJ. Hyperparathyroid and hypoparathyroid disorders. N Engl J

Med 2000; 343: 1863-75.
2. Bushinsky DA, Monk RD. Calcium. Lancet 1998; 306-11.
3. Kanis JA. Disorders of calcium metabolism. In Oxford Textbook of
Medicine. Third Edition. Eds. Weatherall DJ, Ledingham JGG
and Warrell DA. Vol. 2. Oxford University Press. Oxford.
1996 pp. 1622-39.
4. Smart GA. Disorders of Metabolism : Calcium Metabolism. In Prices
Textbook of the Practice of Medicine. Tenth Edition. Ed. Sir Ronald
Bodley Scott. Oxford University Press. Oxford. 1966 pp. 362-4.
5. Bayliss RIS. Diseases of the Endocrine Glands : Hypoparathyroidism.
In Prices Textbook of the Practice of Medicine. Tenth Edition. Ed. Sir
Ronald Bodley Scott. Oxford University Press. Oxford. 1996 pp. 428-9.

6. Meininger ME, Kendler JS. Images in clinical medicine. Trousseaus

sign. N Engl J Med 2000; 343: 1855.
7. Robinson PK. The clinical aspects of disorders of calcium and
phosphorus metabolism. In : Biochemical Aspects of Neurological Disorders.
Eds. Cumings JN and Kremer M. Blackwell Scientific Publications.
Oxford. 1959. pp. 86-97.
8. Johnson DW, Parnham A, Herzig K, Wittmann J. Case Report.
Sunshine, sweating and main d accoucheur. Lancet. 1999; 353: 1492.

13

25

2
1 - 2


2

3-4

1

1) foot drop L. lateral popliteal
(peroneal) nerve lesion lesion L5 S1 root anterior tibial peroneal
muscles posterior tibial muscles
(invertor) lesion nerve root sensory
impairment to pin prick
2)

small intestinal colic
3) urticaria
4) bronchial asthmatic attacks
collagen vascular disease
polyarteritis nodosa hypereosinophilic syndrome

T 37.5C
RR 20
PR 84
Thin, pale woman
Liver palpable 1 cm. below costal margin

BP 110/70

Spleen - not palpable

N.S.
Legs

Power

Dorsi-flexors ankle

4/5

0/5
ext. hallucis longus

4/5

1/5
plantar flexors ankle

3/5

2/5
Deep reflexes
absent Ankle jerk, others present
hypalgesia in the distribution of

L 4-5

dermatomes
Arms
slight wasting and weakness of thenar muscles
and hypalgesia over median nerve distribution
Hb 11

Hct 32

WBC 51700 N 49 E 40 L 2 M 7 B

2
Platelets 1,200,000
sarcocystis spp. cysts
Bone marrow biopsy : hypercellular marrow with myeloid
hyperplasia
and eosinophilia. Megakaryocytes 3
+

gastro-duodenitis

gastroscopy

Hypereosinophilic syndrome (HES)

() Sarcocystis two-host coccidian

Sarcosporidiosis definitive host species
sporocyst

 (Sarcocystis suihominis) (S. bovihominis)

(bradyzoites) cyst. gametes fertilize

oocyst sarcocyst sporocyst

sporocysts Isopora hominis

one-host coccidia
Sarcocystis species intermediate host


6 ()
Hypereosinophilic syndrome
1 eosinophil 1500 ..
mononeuropathy multiplex radiculopathy
myelinated nerve fibres
white matter encephalopathy

post-mortem
endothelial cells endocardium vasa
nervorum product eosinophil Tissue
damage thrombosis
mononeuropathy
multiplex

endocardial fibrosis
interleukin-5 eosinophilopoietic cytokine
HES interleukin (IL)
IL 2 IL15 T cell proliferation natural killer cell

albendazole high dosage prednisolone
6
foot drop power 0/5 23/5 WBC 14700 N 80 E 2% L 14 M 4 Platelets 353,000

Mononeuropathy multiplex
Hypereosinophilia
? HES
?? associated with sarcocystis

infestation

1. Frankel JK. Sarcosporidiosis. In Hunters Tropical Medicine and Emerging

Infectious Diseases. Eight Edition. Ed. Strickland GT. WB Saunders Co,
Philadelphia. 2000; pp. 707-709.
2. Bunyaratvej S, Bunyawongwiroj P, Nitiyanant P. Human intestinal

sarcosporidiosis report of 6 cases. Am J Trop Med Hyg 1982;

31: 36-41.
3. Bunyaratvej S, Unpunyo P. Combined Sarccocystis and gram-positive
bacterial infection. A possible cause of segmental enterocolitis
in Thailand. J Med Assoc Thai 1992; 75 (Suppl 1): 38-44.
4. Bunyaratvej S, Visalsawadi P, Likitarunrat S. Sarcocystis infection and
actinomycosis in tumorous eosinophilic enterocolitis. J Med Assoc Thai
1992; 75 (Suppl 1): 71-75.
5. .
. 1 : 2539
66-72.
6. Gutierez Y. Intestinal Apicomplexa. Sarcocystis hominis and sarcocystis
suihominis. In : Diagnostic Pathology of Parasitic Infections with
Clinical Correlations. Second Edition. Oxford University Press,
Oxford 2000, pp. 186-195.
7. Rothenberg ME. Eosinophilia. N Engl J Med 1998; 338: 1592-1600.
8. Chusid MJ, Dale DC, West B C, Wolff SM. The Hypereosinophilic
syndrome. Analysis of fourteen cases with review of the literature
Medicine (Baltimore) 1975; 54: 1-25.
9. Fauci AS, Harley JB, Roberts WC, Ferrans VJ, Gralnick HR, Bjornson BH.
The idiopathic hypereosinophilic syndrome. Clinical, pathophysiologic,
and therapeutic considerations. Ann Intern Med 1982; 97: 78-92.
10. . .

.. 2530 1-30.

11. Moore PM, Harley JB, Fauci AS. Neurologic dysfunction in the
idiopathic
hypereosinophilic syndrome. Ann Intern Med 1985; 102: 109-114.
12. Jootar S, Vejjajiva A. Neurological Manifestations of
Hypereosinophilia :
An analysis of 6 patients, Abstract of the Proceeding of the 1st
Annual
Conference of the Royal College of Physicians of Thailand. 1985; p. 49.
13. Wichman A, Buchthal F, Pezeshkpour GH, Fauci AS. Peripheral
neuropathy in hypereosinophilic syndrome. Neurology 1985;
35: 1140-45.
14.

Hypereosinophilic Syndrome.
2536; 16: 260-68.
15. Parillo JE, Fauci AS, Wolff SM. Therapy of the hypereosinophilic
syndrome. Ann Intern Med 1978; 89: 167-172.
16. Means-Markwell M, Burgers T, DeKeratry D, ONeil K, Mascola J,
Fleisher T and Lucey D. Eosinophilia with aberrant T cells and
elevated serum levels of Interleukin - 2 and Interleukin - 15.
N Engl J Med 2000; 342: 1568-71.

14

36

4
4

2
3

2 3
6 2

Raynands syndrome
Raynands phenomenon

Raynands disease
2
cervical rib thoracic outlet syndrome

systemic
disease connective tissue disease SLE, progressive systemic sclerosis
cryoglobulinaemia

T 38C
PR 108 regular
RR 20
BP 120/70
ulcer ulcer
2 3

hyperpigmentation

()



cryoglobulin serum
clotted blood 2 ml. serum serum
pipette serum serum
cryoglobulin Sia test

4C serum

ESR connective tissue disease

50 100 protein
anti-DNA auto-antibodies
LE cells

Urine : protein 3+ RBC 15-20 WBC 10-15 / HPF
24 hour protein = 1.8
ESR 96 mm
Hb 11.1 Hct 35 WBC 13600 P 70 L 14 M8 E 1 B2
Atypical
BUN 15 lymphocytes 4% Platelets 74,400 Cr 0.7 Uric Acid
3.3
TB/DB 0.3/0.3
SGOT 106 (14-33)
SGPT 163 (636)
GGT 485 (5-38)
Alk Phos 271 (20-90)
TP/Alb 96.5/47.4
PTT 39 (25-38) PT 12.3 (10-15) TT 10.5 (7-11)
INR 0.9
Cryoglobulin + ve
ANA + ve (fine speckle) > 1:256
Anti DNA - ve
anti SM + ve
anti n-RNP + 1:128 (CIE)
Anti-centromere - ve
+
1:650,000 (PHA)
Anti SCP - ve
CH 50/C3 100% / 961
vasculitis acute
inflammation ulcerative gangrene with tissue necrosis focal panniculitis
SLE anti SM
antibody
antibody n-RNP Mixed
Connective Tissue Disease (MCTD)

SLE ? MCTD

1. Black CM, Scott DCI. Connective tissue disorders and vasculitis. In :

Oxford Textbook of Medicine. Third Edition. Vol 3. Eds. Weatherall DJ,
Ledingham JGG and Warrell DA. Oxford University Press. Oxford
1996; pp. 3008-10.
2. Black CM. Systemic sclerosis : Overlap Syndrome. In : as 1, pp. 303435.
3. Black CM. Raynands phenomenon, scleroderma, overlap syndromes, and
other fibrosing syndromes. Current Opinion in Rheumatology 1989;
1(4): 473-523.
4. Black CM. Raynands phenomenon, scleroderma, overlap syndromes, and
other fibrosing syndromes. Current Opinion in Rheumatology 1990;
2(6): 917-67.
5. Black CM. Raynands phenomenon, scleroderma, overlap syndromes, and
other fibrosing syndromes. Current Opinion in Rheumatology 1990;
3(6): 941-95.
6. Janwitayanuchit S, Vanichapuntu M, Verasetniyom O, Totemchokchyakarn
K,Vatanasuk M. Antinucleolar antibodies and their disease association.
Asian Pac J All Immunol 1994; 12: 43-49.
. Serologic Tests in Rheumatic Diseases
Rheumatology for the Non-rheumatologist.
.
2543 61-71.
7.

8. Scolding N. Neurological complications of rheumatological and connective

tissue disorders. In : Immunological and Inflammatory Disorders of the
Central Nervous System. Ed. Scolding N. Butterworth Heinemann.
Oxford 1999; pp. 147-80.

15

acute leukaemia
18

1

acute lymphoblastic
leukaemia (ALL) Hct 31.5% WBC 32,200 (Lymphoblast
80-90%)
lymphoblast 90%
Vincristine + Doxombicin + L-asparaginase
Prednisolone

5-6

osmotic diuresis hyperglycaemia glycosuria
hypercalcaemia diabetes insipidus

T. 36C
PR 120/min BP 115/75 mm
Moderately dehydrated, slightly pale, no jaundice
Abdomen : soft, slightly distended
mild generalized tenderness, more so
at epigastrium
Liver:

2 FB right costal margin. Spleen: not

palpable
Bowel sounds were decreased
No other abnormalities
()


+ electrolytes)

ketoacidosis
(DKA) (Blood Sugar, serum ketone

BS 730 mg% serum ketone +

Na+ 125, K+ 6.16, Cl- 90, Co2 11.4
Blood pH 7.282, p Co2 18.3 pO2 182 HCO3 8.4,
Sat O2 99.2%
Hct 32.5 WBC 1700 (lymphoblast 48%, N 28%)
Platelet 48,000

L-asparaginase DKA
DKA
acute pancreatitis serum amylase
ultrasound CT
IV NSS 4680 ml
14 I.V. drip 10
6 250 mg% K+
rebound tenderness. Bowel
sound
serum amylase 6044 units/ml lipase 650 units/ml
Ultrasound abdomen pancreas CT

Acute pancreatitis and diabetic ketoacidosis from L-asparaginase

1 febrile neutropenia
cefipime 4 haemoculture Flavobacterium

L-Asparaginase corticosteroid 20
10

1. Gillette PC, Hill LL, Starling KA and Fernbach DJ : Transient

diabetes
mellitus secondary to L-asparaginase therapy in acute leukaemia.
J Pediatr 1972; 81:109.

2. Charan VD, Desai N, Sing AP, Chondhry VP : Diabetes mellitus

and pancreatitis as a complication of L-asparaginase therapy.
Indian Pediatr 1993; 30: 809-10.
3. Rao SP, Castells : Hyperglucagonemia in L-asparaginase induced
diabetes mellitus. Am J Pediatr Hematol Oncol 1986 Spring;
8(1): 83-5.
4. Lavine RL, DiCinto DM : L-asparaginase diabetes mellitus in
rabbits: differing effects of two different schedules of
L-asparaginase administration. Horm Metab Res. 1984;
16 (Suppl 1): 92-6.

16

40

2

1 3-4

18

3
2

T 37C not icteric but slightly pale, clean tongue, greyish white hair,
greyish pubic hair
PR 84
BP 130/70 mm.Hg
Precordial maximal impulse in 5th intercostal space slightly lateral
to mid clavicular line
normal heart sounds no murmur
midline abdominal incision scar
no mass felt
Nervous System
Fundi : normal
VAR 20/70 VAL 20/70
Cranial nerves intact
Limbs : upper 3
Lower : symmetrical weakness of flexors and extensors of feet
(grade 3/5 and 4/5)
Coordination 3
KJ +
+
AJ no Babinski response
- marked impairment of joint position sense at big toes
- impaired vibration sense in both feet and legs up to iliac crests
- impaired light touch and pin prick up to thighs


locomotor ataxia
ataxia posterior column spinal cord
joint position sense
vibration peripheral neuropathy

ankle jerk
pubic hair
12 Cobalamin

12

hypersegmented neutrophil
barium swallow

Hb 11
Hct 31.5
MCV 100 MCHC 35
Macrocytic RBC 2 +
Hypochromia 2 +
WBC 9830 N 47 L 43 M 9 E 1 hypersegmented
neutrophil
Platelets 429,000
Long GI study total gastrectomy
barium particle clumped ileum
()
12 Hb
hypersegmented neutrophil
visual acuity 20/70 2
refractive error correct
caecocentral scotoma

12

(dementia)

Mental Function
Visual Evoked
Potentials 2 EMG Nerve Conduction Velocity

 sensorimotor polyneuropathy
axonopathy
Bone marrow mild megaloblastoid changes serum vitamin B12
321 pg/ml ( 307-969)

12

12 Pernicious anaemia
Subacute combined degeneration of the spinal cord
autoimmune disorder
antibody gastric parietal cells

12
vegans

(total
gastrectomy)
12
Magnetic resonance
imaging (MRI)
(Shaky-Leg Syndrome) cerebellum
12 4-5
12

Sensorimotor polyneuropathy and posterior column degeneration

Megaloblastic anaemia
Vitamin B12 deficiency from total gastrectomy

1. Hemmer B, Glocker FX, Schumacher M, Deuschl G, Lcking CH.

Subacute combined degeneration : clinical, electrophysiological, and
magnetic resonance imaging findings. J Neurol Neurosurg Psychiat 1998;
65: 822-27.
2. Benito-Len J, Porta-Etessam J. Shaky-leg syndrome and Vitamin B12
Deficiency. N Engl J Med 2000; 342: 981.
3. Milea D, Cassoux N, LeHoang P. Blindness in a strict vegan. N Engl J
Med

2000; 342: 897-98.

4. Li K, McKay G. Images in Clinical Medicine. Ischemic retinopathy
caused by severe megaloblastic anemia. N Engl J Med 2000; 342: 860.
5. Perkin GD, Murray-Lyon I. Neurology and the gastrointestinal system.
J Neurol Neurosurg Psychiat 1998; 65: 291-300.
6. Savage DG, Lindenbaum J. Neurological complications of acquired
cobalamin deficiency : clinical aspects. Baillieres Clin Haematol
1995; 8: 657-78.
7. Provan D, Weatherall D. Red cells II : acquired anaemias and
polycythaemia. Lancet 2000; 355: 1260-68.
8. Case Records of the Massachusetts General Hospital. N Engl J Med
2000; 343: 1951-58.

17

35

6



2
1

T 38C
PR 80
BP 120/80
Stupor, neck stiffness and Kernigs sign were present
no other abnormal neurological signs
scaly erythematous rashes over upper lip, at nares and below lower lip,
hyperpigmented patches at peri-oral region and nares

Kernigs sign meningitis

1 pneumococci
meningococci
herpes simplex
herpes


HIV

- HIV negative
- Hb 13.4 Hct 42 WBC 15500 P. 74 L 20 M 6
- CSF 380 .

CSF cells 110 mononuclear cells 97% neutrophil 3%

protein 203 mg% sugar 31 mg% (blood sugar 150 mg%) CSF

()
CSF subacute bacterial infection
Streptococcus suis Strept. group R
Cryptococcus
CSF pressure
skin lesion
Strept. suis meningitis


?

Streptococcus
Group R Penicillin G sodium 4 4

MRI calcified cyst 2 cysts
Cysticercosis

Streptococcus suis meningtitis

Cysticercosis
Streptococcus suis meningitis
30 100
Richard Kay
cochlear sepsis cochlear aqueduct
cochlear cranial nerve 8 Richard Kay

 ()
()

1. Chau PY, Huang CY, Kay R. Streptococcus suis meningitis : an important

underdiagnosed disease in Hong Kong. Med J Aust 1983; 1: 414-17.
2. Kay R, Cheng AF, Tse CY. Streptococcus suis in Hong Kong. Q J Med
1995; 88: 39-47.
3. Kay R. The site of the lesion causing hearing loss in bacterial meningitis :
a study of experimental streptococcal meningitis in guinea-pigs.
Neuropathology and Applied Neurobiology 1991; 17: 485-93.
4. Phuapradit P, Boongird P, Boonyakarnkul S, Niramarnsakul S et al.
Meningitis caused by Streptococcus suis. Internal Medicine (Thailand)
1987; 3: 120-22.
5. Pootong P, Boongird P, Phuapradit P. Streptococcus suis meningitis
at Ramathibodi Hospital. Rama Med J 1993; 16: 203-7.
6. Leelarasamee A, Nilakul C, Tien-Grim S, Srifuengfung S, Susaengrat W.
Streptococcus suis Toxic-shock syndrome and meningitis.
J Med Assoc Thai 1997; 80: 63-68.
7. Watkins EJ, Brooksby P, Schweiger MS, Enright SM. Septicaemia in
in a pig-farm worker. Case Report. Lancet 2001; 357: 38.

18

25

20

2-3
20

2


2
Tone spasticity rigidity
pyramidal tract basal ganglia
myotonia
rigidity
basal ganglia
myotonia
spasticity
myelopathy
sensory spastic legs
flexor
spasm

spastic gait


PR 80
RR 20
BP 110/70 (supine) 60
(erect)
alert, cooperative
dark skin with hyperpigmentation over knuckles and skin creases,
in oral cavity and tongue
N.S.
Cranial nerves : normal
Limbs : increased tone in both legs - slightly increased in both
arms
Power
deltoids
biceps
triceps
brachioradialis
quadriceps
hamstring
tibialis anterior 4/5

3/5
4/5
4/5
4/5
3/5
3/5
4/5

3/5
4/5
4/5
4/5
3/5
3/5

DTRs
BJ
TJ
SJ
KJ
AJ
P

++
++
++
+++
+++

++
++
++
+++
+++

sensation : normal
spastic gait
Other systems : no abnormality
()

1) spinal cord lesion
bilateral
corticospinal tract signs cranial nerves sensation modality
myelopathy

2) skin hyperpigmentation orthostatic hypotension

hypoadrenocorticalism
Addisons disease
2
adrenomyelo- neuropathy
serum Na+ K+ Cl cortisol Addison

3 7
6 ( ) 1

30

12

Serum Na+ 104 K+ 5.9 Cl- 66 HCo3 10
serum cortisol 4.1 g/dl (normal
)
Hb 14 Hct 40 WBC 10700 P 71 L 27 M 4 E
4 ESR 15
FBS 120 Cr 0.8 BUN 18
VDRL - ve
Histoplasma antibody titre - ve
CPK 1180
MRI spine : small sized or atrophic thoracic spinal cord
()

 electrolytes cortisol
hypoadrenalism
Thomas Addison Guys
primary hypoadrenalism classic monograph ..
1855 Addison Bateman
skin hyperpigmentation Addisons disease primary adrenalism

autoimmunity
Polyglandular deficiency type I type II Schmidt syndrome
haemochromatosis, amyloidosis, metastatic tumour
lymphoma histoplasmosis
coccidioidomycosis HIV hypoadrenalism
gene chromosome X peroxisomal
enzyme (lignoceroyl CoA ligase) oxidation very-long-chain fatty acid

adrenoleukodystrophy 20-30

steroid zona fasciculata zona
glomerulosa lamellar inclusions
macrophage Schwann cells
Addisons disease 2 Kendall
cortisol NaCl
15 Cortisol
25-37.5
hydrocortisone 30
(NaCl)
Adrenoleukodystrophy (ALD)
Adrenomyoloneuropathy
1984 5 Lorenzo Odone
(Augusto Michaela Odone) Lorenzo
2 Augusto Michaela
Lorenzos oil triglycerides monounsaturated oleic acid erucic acid
4 1 very-longchain fatty acids (VLCFAs)
Lorenzo 22
Augusto Michaela The Myelin Project
Augusto
Lorenzos Oil
controlled clinical trials


VLCFAs biochemical marker

 Wilson Refsum


biopsy


neoplasm

Medicine and the movies
( 9)

1. Mendis D. Thomas Addison and his contribution to teaching at Guys.

Guys Hosp. Gazette 1999; Feb-March issue, 67-72.
2. Edwards CRW. Addisons disease. In : Oxford Textbook of Medicine.
Third Edition. Vol. 2. Eds: Weatherall DJ, Ledingham JGG and
Warrell DA. Oxford University Press. 1996. pp 1652-56.
3. Oelkers W. Adrenal insufficiency. N Engl J Med 1996; 335: 1206-12.
4. Beishuizen A, Vermes I, Hylkema BS, Haanen C. Relative eosinophilia
and functional adrenal insufficiency in critically ill patients. Lancet
1999; 353: 1675-76.
5. Moser HW. Adrenoleukodystrophy : phenotype, genetics, pathogenesis
and therapy. Brain 1997; 120: 1485-1508.
6. Edwin D, Speedie LJ, Kohler W, Naidu S, Kruse B, Moser HW.
Cognitive and brain magnetic resonance imaging findings in
adrenomyeloneuropathy.
Ann Neurol 1996; 40: 675-78.
7. Kaplan PW, Kruse B, Tusa RJ, Shankroff J, Rignani JE, Moser HW.
Visual system abnormalities in adrenomyeloneuropathy. Ann Neurol
1995; 37: 550-52.
8. van Geel BM, Assies J, Haverkort EB, Koelman JH, Verbeeten B Jr,
Wanders RJ, et al. Progression of abnormalities in adrenomyeloneuropathy
and neurologically asymptomatic X-linked adrenoleukodystrophy despite
treatment with Lorenzos oil J Neurol Neurosurg Psychiatry 1999;
67: 290-99.

9. Jones AH. Medicine and the movies : Lorenzos oil at centurys end.
Ann Intern Med 2000; 133: 567-71.
10. Verrips A, Willemsen MAAP, Rubio-Gozalbo E, De Jong J, Smeitink JAM.
Simvastatin and plasma very-long-chain fatty acids in X-linked
Adrenoleukodystrophy. Ann Neurol 2000; 47: 552-53.

19

64

2
7
Glibencamide 1
2

3


4-5
Acute lobar
pneumonia pneumonitis Strept. Pneumoniae

Acute pyelonephritis



10 John Ryle
20 inbranasal gastric tube
10 John
Ryle

1.
2.

3. ? 2-3
4.
5.
6.
7. ? colic
8.

9.
colic
10. ?

midline origin
duodenum diverticula
duodenum Liver, gall bladder pancreas
colicky pain
(hollow viscus) peristaltic wave
(duodenum)
foregut colic
intestinal colic
peristaltic wave
mid gut duodenum jejunum ileum
appendix superior mesenteric
artery refer hypogestrium
anatomy embryology

T. 38.5C PR 100 BP 140/80

No jaundice
Abdomen : Liver 2 FB below costal margin not tender
()

3

(Opisthorchis viverrini)
ultrasound CT
haemo-culture


O. viverrini
CT abdomen diaphragm
malignancy needle biopsy
cholangiocarcinoma, peripheral type

Cholangiocarcinoma
Opisthorchis viverrini infestation

Opisthorchis viverrini
.. 1908 O. felineus
.. 1915 Leiper
.. 1927 O. felineus
17 Sadun
.. 1953-1955 O. viverrini
30
3



(
2539)

Clonorchis sinensis
Primary sclerosing cholangitis X-linked immunodeficiency

AIDS-related sclerosing cholangitis cholangio-carcinoma

1. De Groen PC, Gores GJ, La Russo NF, Gunderson LL, Nagornay DM.
Biliary tract cancers. N Engl J Med 1999; 341: 1368-78.
2. Hayward AR, Levy L, Facchetti F, Notarangelo L, Ochs HD, Etzioni A.
Cholangiopathy and tumors of the pancreas, liver and biliary tree
in boys with X-linked immunodeficiency with hyper-IgM (XHIM).
J Immunol 1997; 158: 977-83.

3. Hocqueloux L, Molina JM, Gervais A. Cholangiocarcinoma and

AIDS-related sclerosing cholangitis. Ann Intern Med 2000;
132: 1006-07.

20

40 .

1
1 2


2
9
1-2 6 5-6 10 5

Periodic paralysis

Familial Periodic Paralysis
60 16
8
paramyotonia

NaCl NaCl solution IV
Poskanzer Kerr ( 4)
5
Thyrotoxic hypokalaemic periodic paralysis

Wt. 56.2 kg.

Limbs :
Power

PR 120/min BP 160/70 mm Hg
fine tremor of both hands
lid lag present
no wasting, no fasciculation

Upper: deltoids
biceps
triceps
wrist ext.
wrist flex
finger ext.
finger flex.
Lower: hip flex.
quadriceps
DF

2/5

2/5

4/5
4/5
5/5
5/5

4/5
4/5
5/5
5/5
5/5

5/5

5/5
2/5
4/5
4/5

5/5
2/5
4/5
4/5

DTRs
SJ
++
++
BJ
++
++
TJ
++
++
KJ
AJ
+
+
Plantar Response

2
deep tendon reflexes knee jerks
(myopathy) Pulse
pressure
lid lag
Thyrotoxic hypokalaemic periodic paralysis
serum K+ ECG Thyroid
Function

serum K+ 2.8
Na+ 144
Cl- 108
Co2 19.3
Serum Free T4 4.9 g/dl ( 1.0-2.8)
T3 403 g/dl ( 90-250)
TSH < 0.005 mU/L (0.49-4.67)
Hb 15
Hct 48
WBC 11400 Platelets 214000
FBS 123
Cr 0.8
ECG sinus tachycardia HR 115/min non-specific ST-T abnormality


Thyrotoxic hypokalaemic
periodic paralysis (TPP)
35 TPP 30
10

6 20 80
20 40 TPP
0.1-0.2
10

TPP
( 1)

TPP




2-3

ECG TPP

atrial ventricular ectopic beats ECG
prolonged PR interval second degree A-V block Wenckebach (
2)

Dextrose / Saline infusion diuretics

IV Propanolol
acetazolamide

methimazole 15 -
propanolol 10 3
6

 antithyroid

()
10
Andersen

( 8)

ECG prolonged QT interval

Periodic Paralyses
channel

ion
(channelopathies)
sodium
channel
autosomal dominant calcium channel gene
CACN 1A3 1q32 dihydropyridine receptor L-type channel
( 14)
TPP gene
HLA markers HLA A2, BW22, AW19 B17
( 15) HLA DR3

Na, K-ATPase activity

Na+ pump activity lymphocytes platelets
activity
Na, K-ATPase activity
TPP gene Na, K-ATPase activity
gene
TPP Familial hypokalaemic paralysis

9
( 16 17)

serology

1) Vejjajiva A. Neurology in Thailand. In Tropical Neurology. Ed. Spillane JD.

Oxford University Press. Oxford 1973 pp. 335-52.

2) Muenthongchin N, Vejjajiva A, Jumbala B. Disturbance of atrioventicular

conduction in patient with thyrotoxic periodic paralysis. J Med Ass Thailand.
1970; 53: 814-19.
3) Vejjajiva A. Geographical variations in neurological disease : Southeast
Asia.
In Oxford Textbook of Medicine. Second Edition. Eds. Weatherall DJ,
Ledingham JGG, Warrell DA. Oxford University Press. Oxford. 1987;
pp. 21.269-21.272.
4) Poskanzer DC, Kerr DNS. A third type of periodic paralysis with
normokalemia
and favorable response to sodium chloride. Am J Med 1961; 31: 3285) Kodali V, Jeffcote B, Clague RB. Thyrotoxic periodic paralysis : a case
report and review of the literature. J Emerg Med 1999; 17: 43-45.
6) Marlier S, Raccah D, Disdier P, et al. Thyrotoxic periodic paralysis :
discussion of the role of Na, N-ATPase apropos of a case.
Rev Med Intern 1995; 16: 209-11.
7) Smit WJF. Hyperkalaemia in a Thai man. Lancet 1999;
1182.
8) Sansone V, Griggs RC, Meola G, et al. Andersens syndrome : A distinct
Periodic paralysis. Ann Neurol 1997; 42: 305-12.
9) Greenberg DA. Calcium channels in neurological disease. Ann Neurol
1997; 42: 275-82.
10) Hanna MG, Wood NW, Kullmann DM. Ion channels and neurological
disease : DNA based diagnosis is now possible, and ion channels may be
important in common paroxysmal disorders. J Neurol Neurosurg Psychiatry
1998; 65: 427-31.
11) Tawil R, Mc Dermot MP, Brown R Jr, et al. Randomized trials of
Dichlorphenamide in the Periodic Paralyses. Ann Neurol 2000;47: 46-53.
12) Tricarico D, Barbieri M, Camerino DC. Acetazolamide opens the muscular
Kca2+ channel : A novel mechanism of action that may explain the
therapeutic effect of the drug in Hypokalemic Periodic Paralysis.

Ann Neurol 2000; 48: 304-12.

13) Cannon SC. Molecular physiology of ion channelopathies in skeletal
muscle : From mutation to myotonia and paralysis. Ann Neurol 2000; 48: 687.
14) Rdel R, Hanna MG, Lehmann-Horn F. Muscle channelopathies :
Malignant hyperthermia, Periodic paralyses, Paramyotonia and
myotonia. In Muscle Diseases. Blue Books of Practical Neurology .
Eds. Schapira AHV, Griggs RC. Butterworth Heinemann. Boston
1999pp. 135-75.
15) Ko GTC, Chow CC, Yeung VTF, Chan HHL, Li JKY and Cockram
CS.Thyrotoxic periodic paralysis in a Chinese population. Q J Med
1996; 89: 463-8.
16) Szeto CC, Chow CC, Ko GTC, Li KY, Yeung VTF, Cockram CS.
A patient with Graves disease, thrombocytopenia and chronic
hepatitis B. Postgrad Med J 1997; 73: 39-48.
17) Kodali VRR, Jeffcote B, Clague RB. Thyrotoxic periodic paralysis :
A case report and review of the literature. J Emergency Med
1999; 17: 43-45.

41

3
3


2-3

2

3 3Fs Fit Faint

Fake
Fit

epileptic attack epileptic seizure
epileptic convulsion
Faint syncope

Fake Fit Faint

narcolepsy Fake
Fit Faint

Fit Faint and Fit

Fit Faint ?

Faint


major Fit
Faint vaso-vagal attack
(micturition syncope)
(cough syncope)
(cardiac syncope) Aortic stenosis (AS) Atrial myxoma
(AM) 2 AS
(Effort Syncope) AM

heart block cardiac arrest ventricular
tachycardia fibrillation
Brugada

10

Lancet ( 1)

3Fs
2
( 10 13)

T 37C
PR 84/min. regular
B.P. 130/80
JVP flat
Heart : no heaving, no thrill
Normal 1st and 2nd sounds, no murmur
Peripheral pulses present
Other Systems were normal

()
murmur
Faint cardiac arrhythmia
ECG
Fit 25
Cerebral cysticercosis MRI CT
EEG
ECG


ECG V1 - V3 variable ST-segment elevation
Cardiac catheterization coronary angiogram
( )
ECG : Ventricular fibrillation flecainide IV

pace maker

coranary artery disease
Brugada ventricular fibrillation
(idiopathic IVF) Brugada 2
.. 1992 ( 2)

( 90)
ECG V1-V3 right-bundle-branch block
left-axis deviation ECG anteroseptal myocardial
infarction ECG flecainide
sodium channel ECG
diagnostic challenge IVF Brugada


Bangungut Pokkuri
26-38

(
)
()
()
()
( 3)
Brugada
gene SCN 5A

-subunit sodium channel

Brugada

Brugada

 (Major depression)
tricyclic antidepressant (nortriptyline 175 )
phenothiazine (trifluoperazine 3 ) benzodiazepine (alprazolam 0.5
) 7

cardiac arrhythmia
16 22
23

1) Grace AA. Brugada syndrome. Lancet 1999; 354: 445-6.

2) Brugada P, Brugada J. Right bundle branch block, persistent ST
segment elevation and sudden cardiac death : A distinct clinical and
electrocardiographic syndrome. J Am Coll Cardiol 1992; 20: 1391-6.
3) Brugada J, Brugada R, Brugada P. Right bundle-branch block and STsegment elevation in leads V1 through V3 : a marker for sudden death in
patients without demonstrable structural heart disease. Circulation 1998;
97: 457-60.
4) Nademanee K, Veerakul G, Nimmannit S, Chaowakul V, Bhuripanyo K,
Likittanasombat K, Tungsanga K, Kuasirikul S, Malasit P, Tansupasawadikul S,
Tatsanavivat P. Arrhythmogenic marker for the sudden unexplained death
Syndrome in Thai men. Circulation 1997; 96: 2595-600.
5) Matsuo K, Kurita T, Inagaki M, et al. The circadian pattern of the
development in patients with Brugada syndrome. Eur Heart J 1999; 20:
465-70.
6) Ailings M, Wilde A. Brugada syndrome : clinical data and suggested
pathophysiological mechanism. Circulation 1999; 99: 666-73.
7) Sanders P, Farouque O, Cehic DA, Young GD. An usual cause of
arrhythmic syncope : the Brugada syndrome. Aust NZ J Med 1999; 29:
737-8.
8) Chen Q, Kirsch GE, Zhang D, et al. Genetic basis and molecular
mechanism for idiopathic ventricular fibrillation. Nature 1998; 392: 293-6.

9) Priori SG, Barhanin J, Hauer RN, et al. Genetic and molecular basis of
cardiac arrhythmias : impact on clinical management. Eur Heart J 1999;
20: 174-95.
10) Linzer M, Yang EH, Estes NA, Wang P, Vosperian V, Kapoor WN.
Diagnosing syncope. Part 1 : Value of history, physical examination, and
electrocardiography. The Clinical Efficacy Assessment Project of the
American College of Physicians. Ann Intern Med 1997; 126: 989-96.
11) Linzer M, Yang EH, Estes NA, Wang P, Vosperian V, Kapoor WN.
Diagnosing syncope. Part 2 : Unexplained syncope. The Clinical Efficacy
Assessment Project of the American College of Physicians. Ann Intern Med
1997; 127: 76-86.
12) Ammirati F, Colivicchi F, Santini M, on behalf of the investigators of the
OESIL study. Diagnosing syncope in clinical practice. Implementation of a
simplified diagnostic algorithm in a multi centre prospective trial the OESIL
2 study (Osservatorio Epidemiologico della Sincope nel Lazio). Eur Heart J
2000; 21: 935-40.
13) Shridharani K, McGinn T. Differential Diagnosis. A simple algorithm
improved physicians, diagnostic performance for patients presenting with
syncope. Commentary. ACP Journal Club November/December 2000 p.
121.
14) Smith PEM. If its not epilepsy. J Neurol Neurosurg Psychiatry 2001;
70 (suppl II): II 1- II 12.
15) Basson CT. A molecular basis for Wolff-Parkinson White syndrome.
Editorials. N Engl J Med 2001; 344: 1861-4.
16) Gollob MH, Green MS, Tang AS-L, et al. Identification of a gene
responsible for familial Wolff-Parkinson-White syndrome. N Engl J Med
2001;
344: 1823-31.
17) Kapoor WN. Syncope. N Engl J Med 2000; 343: 1856-62.
18) Fenton AM, Hammill SC, Rea RF, Low PA, Shen W-K. Vasovagal
syncope. Ann Intern Med 2000; 133: 714-25.

19) Mathias CJ, Deguchi K, Schatz I. Observations on recurrent syncope

and presyncope in 641 patients. Lancet 2001; 357: 348-53.
20) Davison J, Seifer C, Kamper AM. Recurrent syncope and presyncope.
Lancet 2001; 357: 1801-2.
21) Veerakul G, Nademanee K. Sudden unexplained death syndrome in
Thailand. Abstract. Intern Med J Thai 2001; 17 (Suppl): 33.
22) Huikuri HV, Castellanos A, Myerburg RJ. Sudden death due to cardiac
arrhythmias. N Engl J Med 2001; 345: 1473-82.
23) Harbison J, Newton JL, Seifer C, Kenny RA. Stoke Adams attacks
and cardiovascular syncope. Lancet 2002; 359: 158-60.

45

5

2-3 2
10

7 2-3


5

1)
2)

1) (pericardium)
2)
3)

methaemoglobin
4)
5)
6)
6






bronchial
asthma, chronic bronchitis chronic obstructive pulmonary disease COPD

alveoli restriction
(hypoxaemia)
diffuse

night sweat

lymphoma connective tissue Wegeners granulomatosis


!
sertraline selective serotonin reuptake inhibitor
fluvoxamine

sertraline (
8)



drenching night sweat

( 9)

T 36.8C
145/80

PR 90 regular

RR 32

B.P.

Breathless, not pale, no clubbing of fingers

no oedema
JVP not engorged
Heart : apexbeat 5th ICS, MCL no heave, no thrill
sounds normal, no murmur
Chest : normal chest expansion
trachea in midline
bronchial breath sounds over L middle lung field
decreased breath sounds over lung

crepitations over both lungs

Abdomen : Liver and Spleen not palpable

(crepitations crackles)
(coarse, fine or medium)


alveoli rhonchi wheezes
2 trachea 2

alveoli bronchioles
bronchial breath sounds
Consolidation bronchi
trachea
(atelectasis collapse)
diffuse pulmonary alveolar disease
consolidation middle lung field
?

malignancy

connective tissue
pulmonary alveolar

proteinosis

( 5)
Arterial Blood Gas (ABG)


PA Chest xray : bilateral diffuse
fine reticulo-nodular and alveolar infiltration
Hb 13.7
Hct 45.2
MCV 73.1
MCH 22.1
MCHC
30.3
RBC 6.19 x 106
WBC 12800 N 86 L 8 M 4 E 1 B 1
Platelets 793,000
Anti-HIV - ve
Stool : occult blood + ve
S 96
BUN 13
Cr. 1.4
Uric Acid 5.3
Chol 382
AP 255
SGOT 72
PT 57
GGT
108
TP 82.8

alb/glob 36.2/18.4
129

6.11

99

13.4

ABG : pH 7.385

pCO2 23.5
pO2 34.2
HCO3 13.7
O2 Saturation 58
O2 10 /
pH 7.407 pCO2 27.8 pO2 80 HCO3 17.1
O2 Saturation 94.8

2
1) arterial blood gas pH
hypoxaemia
pCO2 CO2 10

O2 Saturation 58 94.8 pH 7.4

alveolar-capillary block restrictive ventilation
obstructive airway disease
2) diffuse fine reticulo-nodular and alveolar
infiltration

sarcoidosis

(occupational lung disease) allergic lung

diseases Pulmonary alveolar proteinosis (PAP)

ultrafast scan

 : high resolution CT (HRCT)

diffuse peribronchio-vascular interstitial thickening with ground glass opacity of both
lung fields
Dexamethasone IV 5 mg 6
hypersensitivity
Pneumonitis
PAP bronchoscope under endotracheal tube broncho
alveolar lavage fluid
whole lung lavage 4 (
2 ) 6 10
bronchial
lavage acid fast bacilli mycobacterium scrofulaceum
Streptomycin, Ofloxacin, INH, Rifampicin, PZA Ethambutol

8 2
Klacid 500 mg
Streptomycin 750 mg Ofloxacin 600 mg 5
Pulmonary Alveolar Proteinosis (PAP)

2 3 4-5 1

41
5-6 CPC
New Engl J Med ( 7)

1)

.. 1958

PAP 2-5 1
2
lipoprotein

alveoli
fibrosis
ventilation restrict shunting alveolar-capillary level
hypoxia

 T-cell function Norcardia, aspergillus, candida,

crypto, cytomegalo, histoplasma, mucormycosis, mycobacteria, pneumocystis

pulmonary oedema

PAP alveolar secretions

PAS

( 6)

1) Rosen SH, Castleman B, Liebow AA. Pulmonary alveolar proteinosis. N

Engl J Med 1958; 258: 1123-42.
2) Hendrick DJ. Pulmonary alveolar proteinosis. In : Oxford Textbook of
Medicine. Third Edition. Eds. Weatherall DJ, Ledingham JGG, Warrell DA.
Oxford University Press. Oxford. 1996; Vol 2, 17.10.12, pp. 2833-5.
3) ..
8 2517; 57: 468-75.
4) . . 2541;
50: 813-5.
5) . .
2514; 206 .
6) Parker LA, Novotny DB. Recurrent alveolar proteinosis following double
lung transplantation. Chest 1997; 111: 1457-8.
7) Case Records of the Massachusetts General Hospital. N Engl J Med
2001; 344: 212-20.
9) Babbott SF, Pearson VE. Sertraline - related night sweats. Ann Intern
Med 1999; 130: 242-3.
9) . . 2544; 10: 28-31.
10) Gross TJ, Hunninghake GW. Idiopathic pulmonary fibrosis. N Engl J
Med 2001; 345: 517-24.

3

47 .

3-4

5 . 2

meningoencephalitis
encephalopathy 2

T 38.2C
PR 60
BP 160/95
fully conscious but mildly confused answered questions but slowly
and occasionally, pale cold extremities, no sweating, malar flush
few petechial haemorrhages over trunk and legs
neck stiffness present
Legs : Hip flexors
3/5
4/5
Quadriceps
5/5
5/5
DTRs 2+ in all limbs
P
Other systems : no abnormality
()
meningoencephalitis

dengue
Torniquet test

Hb 16.8
WBC 4200

PCV 48.1
RBC 5.55 x 106
N 70 L 23
M7
atypical lymphocytes present
Platelets 24000
Haemoculture : no growth

anti-HIV -ve FTA-ABS non-reactive

TB 1.7 DB 1.4
SGOT 3413
PT 640 GGT 561
Alk Phos 315 (27-86)
CPK 182
Urine : protein 2+ sp gr 1.015
WBC 0-2 / HPF
RBC 0
CSF : open pressure 120 mm H2O
clear, 20 mononuclear cells / cu mm
protein 150 mg%
sugar 81 mg%
culture no growth
Serology for leptospira -ve
OX19 1:20
OXK 1:20 OX2 1:40
Dengue titre : HI 1:1280
Capture Elisa Dengue-IgM 130 units 76 ( 2
7 )
(normal < 40)
JE IgM 12 3
acute dengue infection

()
arbovirus
100 2500

()
(dengue haemorrhagic fever, DHF)

378
4

spasticity
2 transverse myelitis spastic paraparesis
( 1) 7


1) Solomon T, Dung NM, Vaugh DW, et al. Neurological manifestations of
dengue infection. Lancet 2000; 355: 1053-59.
2) Rigau-Prez JG, Clark GG, Gubler DJ, Reiter P, Sanders EJ, Vorndam
AV. Dengue and dengue haemorrhagic fever. Lancet 1998; 352: 971-77.
3) N Engl J Med. Case Records of the Massachusettes General Hospital.
Eds. Scully RE, et al. 1989; 321: 957-65.
4) Malheiros SMF, Oliveira ASB, Schmidt B, CamargoLima JG, Gabbai AA.
Dengue. Muscle biopsy findings in 15 patients. Arq Neuropsiquiatr 1993;
51: 159-64.
5) Thisyakorn U, Thisyakorn C. Dengue infection with unusual
manifestations. J Med Assoc Thai 1994; 77: 410-13.
6) Avirutnan P, Malasit P, Seliger B, Bhakdi S, Husmann M. Dengue virus
infection of human endothelial cells leads to chemokine production,
complement activation and apoptosis. The Journal of Immunology 1998;
161: 6338-46.
7)
. 4 .. 25422543. 2001; 10: 688-94.
8) Ooi EE, Hart TJ, Tan HC, Chan SH. Dengue seroepidemiology in
Singapore. Lancet 2001; 357: 685-86.

4

60 . .

10 .

2
2 2

rickettsia
streptococcus suis leptospirosis
eschar

T 39.5C
PR 108
BP 140/90
fully conscious, neck stiffness +
no Kernigs sign
no other abnormality
no eschar

meningitis


CSF : opening pressure 150 mm.
150 cells, all are mononuclear
Protein 157 mg%
Glucose 63 mg% (blood glucose 100)
Indian ink stain : negative
Hb 14.8
WBC 7600
N 88
L7
M5
Platelets 219000
FBS 130 BUN 23 Cr 1.4
serum alb. 45.3
glob 42.7
SGOT 89
PT 138
GGT 208
Urine : protein 1+ WBC 1-2/HPF
Chest xray : normal
()
CSF opening pressure

platelets SGOT PT GGT Rickettsia
enzymes
eschar 50 eschar
serology

serology IFA murine typhus +ve (IgM 1:3200 IgG 1:3200)
IFA scrub typhus ve
Cryptococcus Ag ve
Doxycycline 100 mg 2 2

Murine typhus Rickettsia typhi

(flea)



Xenopsylla cheopis (Ctenocephalides felis)
opossums ( 2 4)
opossum (marsupial)



( 1)
3 137
10 72



doxycycline

George Watt ( 9) HIV-1
HIV-1

( 10) !

1) Silpapojakul K, Ukkachoke C, Krisanapan S, Silpapojakul K. Rickettsial
meningitis and encephalitis. Arch Intern Med 1991; 151: 1753-57.
2) Roberts SA, Ellis-Pegler RB. Murine typhus in the Kaukapakapa area
again. Aust NZ J Med 1997; 27: 446-7.
3) Wilson ME, Brush AD, Meany MC. Murine typhus acquired during short
term urban travel. Am J Med 1989; 87: 233-4.
4) Sorvillo FJ, Gondo B, Emmons R, et al. A suburban focus of endemic
typhus in Los Angeles county : association with seropositive domestic cats
and opossums. Am J Trop Med Hyg 1993; 48: 269-73.
5) Chua CJ, Tan KS, Ramli N, Devi S. Scrub typhus with central nervous
system involvement : a case report with CT and MR imaging features.
Neurol J Southeast Asia 1999; 4: 53-7.
6) Trishnananda M. Travel medicine and tropical infections. Intern Med J
Thai 2001; 17: 361-65.
7) Watt G, Kantipong P, Jongsakul K, Watcharatpichat P, Phulsuksombati,
Strickman D. Doxycycline and rifampicin for mild scrub-typhus infections
in northern Thailand : a randomised trial. Lancet 2000; 356: 1057-61.

8)
2542
2544; 10: 526-32.
9) Watt G, Kantipong P, de Souza M, et al. HIV-1 suppression during
acute scrub-typhus infection. Lancet 2000; 356: 475-9.
10) - 2543
815.

5

18 .

colic

colic

parasympathetic

duodenum ascending colon peristalsis
superior mesenteric nerve plexus
1
porphyria

colic parasympathetic nerve plexus protoporphyrins
haem products



?

lead line

4-5
3
foil

T 37.2C
PR 118
RR 28
B.P. 165/100
pale, slightly icteric
no lymphadenopathy
Gum : no lead line
Heart sounds normal
Abdomen : slight generalized tenderness over epigastrium and
umbilicus
- no mass felt
CNS : no abnormality
()
Lead line


lead sulphide


porphyria

basophilic stippling

basophilic stippling 1-2
2 2

1
2

Hb
10.0
8.6
13-18.5 g/dl
PCV
30.8
25.6
40-55%
MCV
77
77
3
80-95 um

MCH
25.8

25.1
27-32 pg

MCHC
32.6

32.5
32-36

WBC
5200
N
L

11200
4500-10700
95

56
3

40
M

4
RBC
3.99

4.6.6.2 x 10

3.9

aniso-

few

few
poikilo

few

few
microcytic
hypochromic
polychromasia
Burr cells

1+
1+
1+

1+
1+
1+
few

few
Ovalocytes
few
Reticulocytes
19.1%
basophilic stippling
1%
Platelets
247,000
150,000-400,000
G.6PD
normal
Hb typing A 97
A2 3%
Urine : sp gr 1.025 pH 6.5
no protein, glucose or blood
Blood lead level 112.78
zinc protoporphyrin 146
ALA-D
12
Urine :
ALA > 6 mg/L (normal 0-6)
CP3 1712.20 g/L (0-200)

()

few
19%
2%
32,700


chelating agent EDTA

Calcium EDTA

(mg/L)

(L)

(mg)

1250.55

1.97

2375
24

2090.05

3.45

48

2043

4.0

7210.67
8172

9 72 B.P.
120/80 . 48.5 mg/dl

2000 Hippocrates
(lead colic)



10 g dl
enzymes haem
aminolevulinic acid dehydratase ferrochelatase 5aminolevulinic acid (ALA) zinc protoporphyrin (ZPP)
ALA ZPP


1) Aw TC, Vale JA. Poisoning from metals. In : Oxford Textbook of
Medicine. Third Edition. Vol. 1. Eds. Weatherall DJ, Ledingham JGG,
Warrell DA. Oxford University Press. Oxford. 1996; pp. 1109-11.
2) McColl KEL, Dover S, Fizsimons E, Moore MR. Porphyrin metabolism
and the porphyrias. In : Oxford Textbook of Medicine. Third Edition. Vol.
2. Eds. Weatherall DJ, Ledingham JGG, Warrell DA. Oxford University
Press. Oxford. 1996; pp. 1388-99.
3) Visvanathan R. Is it truly dementia ? Case Report. Lancet 2001;
357: 684.
4) Rosen JF, Mushak P. Primary prevention of childhood lead poisoning The only solution. N Eng J Med 2001; 344: 1470-1.
5) Rogan WJ, Dietrich KN, Ware JH, et al. The effect of chelation therapy
with succimer on neuropsychological development in children exposed to
lead. N Engl J Med 2001; 344: 1421-6.
6) Sirivarasai J, Kaocharern S, Wananukul W, Sirapha C, Tongpoo A,
Srisomeran P. Blood lead levels in a high-risk occupational group. J Environ
Med 2001; 3: 71-9.
7) .

2544; 10: 91-6.
8) Stewart WF, Schwartz BS, Simon D, Bolla KI, Todd AC, Links J.
Neurobehavioral function and tibial and chelatable lead levels in 543 fomer
organolead workers. Neurology 1999; 52: 1610-7.
9) Sithisarankul P. Molecular epidemiology and lead poisoning. Intern Med
J Thai 2001; 17: 147-56

6

69

1
3

laparoscopic cholecystectomy
3

1


3
1) Encephalopathy

1.1 General systemic diseases

metabolic hyponatremia, uraemia, liver

failure
hypothyroidism
1.2 thiamine
Wernickes encephalopathy, hypertensive encephalopathy
porphyria
2) 2.1 encephalitis

cerebritis
2.2
disseminated encephalomyelitis SLE
2.3
angiitis thrombotic thrombocytopaenic purpura cerebral venous
occlusion
3) chronic subdural
haematoma, brain abscess brain tumour silent area focal motor
sensory signs frontal lobe

ptosis
1) oculomotor (third cranial nerve) palsy
2) Horners syndrome (sympathetic paralysis)
3) myasthenia gravis
(1) (2)
pupil Horners syndrome
oculomotor nerve palsy pupil
cranial nerves

T 37C
PR 84
BP 150/80
drowsy, no neck stiffness
Fundi :
Eyes : 1 ptosis L 3
movements limited as shown
70%
50%
50%
80%

70%

50%
70%

70%
Pupils

3 mm

2.5 mm
reacted to light normally
JJ +
grasping reflexes
++
DTRs +++ in all
P

++

Other systems : no abnormality

anterior cranial fossa

2 orbital apex 2 cranial
nerve 3, 4, 6 cranial nerve 3 frontal
lobe sign grasp reflex 2 maxillary
ethmoid sinuses aspergillosis mucormycosis

nasopharynx
Wegeners granuloma

MRI CSF

MRI rim-enhancing thin subdural collection with

subjacent brain parenchymal involvement at the antero-inferior aspect of both frontal
lobes
Hb 9.6 Hct 28.7
MCV 81
MCH
MCHC 33.2
N 92 L 5 M 3
RBC 3.59x106 WBC 9800
Platelets 383,000
Urine : protein 1+
glucose : trace
FBS 157, 199
Chest xray : no abnormality
Sinus xrays : severe pansinusitis
CSF : OP/CP 15/14 clear RBC 200 WBC 250

26.8

N 70

L 30
Protein 216 Sugar 83 (blood sugar 287)

craniotomy multiple abscesses frontal lobe 2

biopsy necrotic debris with few residual neutrophils and collection
of branching true hyphae of Aspergillus fumigatus
amphotericin B
nasal sinuses amphotericin B 2656 mg.
itroconazole 600 mg.


Aspergillus Ascomycetes

350 7-8
Aspergillus fumigatus, A. niger A. flavus
Oppe .. 1897
Dr. Guillain GuillainBarr syndrome
.. 1935

3 ( 3)

7 ( 12)
3-4 CPC
1

1) Saravia Gomez J. Aspergillosis of the central nervous system. In :

Handbook of Clinical Neurology. Vol 35. Eds. Vinken PJ and Bruyn GW.
Elsevier / North- Holland Biomedical Press. North-Holland Publishing
Company. Amsterdam 1978; pp. 395-400.
2) Guillain G, Bertrand I, Jereboullet J. Etude anatomoclinique sur un
abscs mycosique du lobe frontal. Rev neurol 1935; 64: 684-9.
3) Visudhiphan P, Bunyaratavej S, Khantanaphar S. Cerebral aspergillosis.
Report of three cases. J Neurosurg 1973; 38: 472-6.
4) Lewis MB, Henderson B. Invasive intracranial aspergillosis secondary to
intranasal corticosteroids. J Neurol Neurosurg Psychiatry 1999; 67: 416-7.
5) de Shazo RD, Chapin K, Swain RE. Fungal sinusitis. N Engl J Med
1997; 337: 254-9.
6) Nichols L. Fungal sinusitis. N Engl J Med 1997; 337: 1633.

7) Hedges TR, Leung LS. Parasellar and orbital apex syndrome caused by
aspergillosis. Neurology 1976; 25: 117-20.
8) Yumoto E, Kitani S, Okamura H, Yanakihara N. Sino-orbital aspergillosis
associated with total ophthalmoplegia. Laryngoscope 1985; 95: 190-2.
9) Denning DW, Stevens DA. Antifungal and surgical treatment of invasive
aspergillosis : review of 2121 published cases. Rev Infect Dis 1990; 12:
1147-201.
10) Beal MF, OCarroll CP, Kleinman BM, Grossman RJ. Aspergillosis of the
nervous system. Neurology 1982; 32: 473-9.
11) Lowe J, Bradley J. Cerebral and orbital Aspergillus infection due to
invasive aspergillosis of the ethmoid sinus. J Clin Pathol 1986; 39: 774-8.
12) Pongbhaesaj P, Dejthevaporn C, Tunlayadechanont S, Witoonpanich R,
Sungkanuparph S, Vibhagool A. Aspergillosis of the central nervous system
: A catastrophic opportunistic infection. Neurol J Southeast Asia 2001; 6:
52.
13) NG TTC, Denning DW. Liposomal amphotericin B (AmBisome) therapy
in invasive fungal infections : evaluation of United Kindom Compassionate
use data. Arch Intern Med 1995; 155: 1093-8.

7

37

2 1


1

2 2

1
3

2
glaucoma

visual field upper nasal field defect

uvea uveitis acute optic
neuritis

systemic disease uveal tract retina

Vogt-Koyanagi-Harada (VKHS)
30


2-3

 : 2

T 37C
PR 80 regular
BP 110/70 mm Hg
Eyes : mildly injected conjunctivae VAR : PL VAL : PL
Lenses :
clear
Eyeground : small attennated retinal vessels
no papilloedema
Hearing : impaired L > R
Webers not lateralized, AC > BC bilaterally
no neck stiffness, Kernigs sign absent
no abnormal neurological signs
Joints and Skin : no abnormality
Ophthalmologists findings : panuveitis c exudative retinal diseases
bilaterally
Audiological examination : mild sensorineural hearing loss bilaterally L
>R

VKHS
CSF
mono-nuclear cells ESR
autoantibodies

Blood

Hb 14.4 Hct 43
WBC 7840 N 63 L 29 M 5 E 3
FBS 114
Chol 274
TG 264
st
ESR 15 mm/1 hour
SGOT 38
PT 57
VDRL & FTA-ABS non-reactive
anti - HIV negative
RF : negative
ANA : positive speckled 1:40
CSF : opening pressure 85 mm H2O
cells : 360 mononuclear ~ 100%
protein 28 mg%, glucose 103 mg% (blood 209)
X-ray lumbo-sacral spine : fusion and sclerotic changes of
superior half of S.I joint

()

CSF

sacro-iliac joint Reiter

Behet

VKHS .. 940
.. 1793 Francesco Goya

(vitiligo) VKHS
Vogt-Koyanagi Harada
anterior uveitis posterior uveitis retinal
detachment .. 1932 Babel VKHS
60
vitiligo

-2
autoimmune
corticosteroids cyclosporin A

3 VAR
20/200 PL VAL 20/60 1 VAR 20/125 VAL 20/50
15

1) Inomata H, Kato M. Vogt-Koyanagi-Harada disease. In : Handbook of

Clinical Neurology. Vol. 56. Viral Disease. Eds. Vinken PJ, Bruyn GW,
Klawans HL.Elsevier Science Publishers, Amsterdam. 1989; pp. 611-26.
2) Kanchanaranya C, Konyama K, Boonswasdi P. Haradas disease. Report
of a case. J Med Assoc Thai 1970; 57: 524-31.
3) Panthumchinda K, Kanchanapongkul J, Locharernkul C. Vogt-KoyanagiHarada Syndrome. J Med Assoc Thai 1989; 72: 144-9.
4) Rubsamen PE, Gass JD. Vogt-Koyanagi-Harada syndrome. Clinical
course, therapy, and long-term visual outcome. Arch Ophthalmol 1991;
109: 682-7.

8
30
30

2
7
2

3
7

5 30
parasagittal motor area


primary subarachnoid haemorrhage ruptured anterior
communicating aneurysm
anterior cerebral artery spasm Arterio-venous
malfomation


toxoplasma HIV


cerebral venous thrombosis

(1) ?
(2) ?
(3) ? ?

10


T 37C
PR 72
BP 130/80
fully conscious, no neck stiffness
Fundi : bilateral papilloedema, no haemorrhage or exudate
Other Cranial nerves : normal
Limbs : weakness of L leg
DF 2/5
Quad 5/5
Hamstring 3/5
HF 3/5
DTRs normal
L extensor plantar response
JPs and PPs normal
Other systems : no abnormality
()
parasagittal motor area
Babinski sign
bilateral papilloedema
cerebral venous occlusion
10 superior
sagittal sinus
haemorrhagic infarct
MRI
MRI CT WBC eosinophilia
anti-HIV ESR protein C, S anti-thrombin

CT brain : generalized brain oedema, presence of empty delta sign

at
superior sagittal sinus
Haematologic studies
Hb, Hct & WBC : normal
Blood Group A
Protein C 123% (64-141)
S 64% (61-127)
anti-thrombin III 146% (80-128)
Factor VIII
155%
serum homocysteine 6.07 mol/L (5-12)
anti-ANA, -DNA, -SM and -RNP : all negative
ESR anti-HIV negative

 ()
empty delta-sign superior sagittal sinus
venous sinus occlusion thrombosis anticoagulant

heparin

Cerebral venous sinus occlusion

2-3

CT scan cerebral angiography
1
CT scan
anticoagulant
10

venous thromboembolism
12 cyproterone 4
levonorgestrel

1) Carroll JD, Leak D, Lee HA. Cerebral thrombophlebitis in pregnancy and

the puerperium. Q J Med, (New series XXV), 1966; 139: 347-68.
2) de Bruijn SFTM, de Hann RJ, Stam J for the Cerebral Venous Sinus
Thrombosis Study Group. Clinical features and prognostic factors of cerebral
venous sinus thrombosis in a prospective series of 59 patients. J Neurol
Neurosurg Psychiatry 2001; 70: 105-108.
3) Vandenbroucke JP, Rosing J, Bloemenkamp KWM, et al. Oral
contraceptives and the risk of venous thrombosis. N Engl J Med 2001;
344: 1527-35.

4) Seligsohn U, Lubetsky A. Genetic susceptibility to venous thrombosis.

N Engl J Med 2001; 344: 1222-31.
5) Quattrone A, Bono F, Oliveri RL, et al. Cerebral venous thrombosis and
isolated intracranial hypertension without papilledema in chronic daily
headache. Neurology 2001; 57: 31-36.
6) van Gijn J. Cerebral venous thrombosis : pathogenesis, presentation
and prognosis. J R Soc Med 2000; 93: 230-33.
7) Wardlow JM, Lammie GA, Whittle IR. A brain haemorrhage ? Lancet
1998; 351: 1028.
8) Rosendaal FR. Venous thrombosis : a multicausal disease. Lancet
1999; 353: 1167-73.
9) Fink JN, McAuley DL. Cerebral venous sinus thrombosis : a diagnostic
challenge. Intern Med J 2001; 31: 384-90. 10) Meyer MA. Cerebral
sinovenous thrombosis. N Engl J Med 2001; 345: 1777-8.
11) Raizer JJ, DeAngelis LM. Cerebral sinus thrombosis diagnosed by MRI
and MR venography in cancer patients. Neurology 2000; 54: 1222-6.
12) Fink JN, McAuley DL. Safety of anti-coagulation for cerebral venous
thrombosis associated with intracerebral hematoma. Neurology 2001; 57:
1138-9.
13) Vandenbroucke JP, Rosing J, Bloemenkamp KWM, et al. Oral
contraceptives and the risk of venous thrombosis. N Engl J Med 2001;
344: 1527-35.
14) Vasilakis-Scaramozza C, Jick H. Risk of venous thromboembolism with
cyproterone or levonorgestrel contraceptives. Lancet 2001; 358: 1427-9.

45 .

1 6
2

1) (motor weakness)
2) increased tone spasticity (upper motor neuron
lesion)
rigidity (Parkinsonism)
3) vestibular system
4) proprioception joint sense
5) cerebellum cerebellar tract

3)
joint sense
5) cerebellar ataxia
Cerebellar ataxia
cerebellar intoxication
phenytoin metronidazole
paroxysmal cerebellar ataxia familial
40
1) hypothyroidism cerebellar degeneration
( 7 8)
2) inorganic mercury
Hatters shakes (mercuric

nitrate)

( 11)

50 methylmercury

3) immune response paraneoplastic

cerebellar degeneration
antibodies anti-Hu
cerebellum gluten
gluten ataxia anti-gliadin antibodies coeliac disease

biopsy
( 9 10)
4) cerebellar degeneration spino-cerebellar
ataxia


ECG
(cerebellum)
niacin multiple
sclerosis

2 ECG sinus
tachycardia ST-T wave propranolol
thyroid function
1 2


PR 90 regular
BP 130/80
Heart : normal S1 + S2
systolic click at apex
no KF ring
slight dysarthria
Fundi : normal
EOMs : full, gazed-evoked slow beat nystagmus
Bilaterally, L > R
Limbs : slight spasticity both legs R > L
motor power 5/5
Coordination : impaired Finger-nose test
L>R
dysdiadokokinesia L +
Heel-shin test impaired bilaterally
DTRs

SJ
BJ
TJ
KJ
AJ
P

+++
+++
+++
++++
+++

+++
+++
+++
++++
patellar clonus L +
+++

Joint sense : normal

Rombergs : absent

()
cerebellar pyramidal signs spino cerebellar
ataxia (SCA) autosomal dominant
Anita Harding ADCA (autosomal dominant cerebellar
ataxia)
3 SCA (spino-cerebellar ataxia)
16
gene chromosome
SCA 1 chromosome 6p CAG Repeat Expansion
2 pure cerebellar ataxia
hereditary spastic paraplegia
ataxia spasticity upper motor neurone signs deep reflex

Babinski sign ophthalmoplegia, optic

atrophy, extrapyramidal signs neuropathy

MRI moderate olivo-ponto-cerebellar atrophy cervical

cord atrophy
DNA SCA Type 1

ataxia (disorder)
Hippocrates 19 Duchenne
locomotor ataxia proprioception joint position sense
tabes dorsalis syphilis Nicolaus Freidreich
hereditary ataxia .. 1863
Freidreichs ataxia
autosomal recessive gene protein frataxin
25 Pierre Marie
Sir Gordon Holmes
autosomal dominant
12 2
14 16

Harry Orr SCA 1 (
17)

1) Bates D. Ataxia. In : Frenchs Index of Differential Diagnosis.

Thirteenth edition. Eds. Bouchier IAD, Ellis H, Fleming PR. Butterworth
Heinemann. Oxford 1996; pp 39-41.
2) Handbook of cerebellar diseases. Ed. Lechtenberg R. Marcel Dekker,
Inc. New York. 1993; 573 pp.
3) Parker HL. Periodic ataxia. Mayo Clinic Proc 1946; 38: 642-5.
4) Ng WK, Tan CT. Paroxysmal cerebellar ataxia : use of phenytoin as a
provocation test. Aust NZ J Med 1994; 24: 583.

5) Woodruff BK, Wijdicks EFM, Marshall WF. Reversible metronidazoleinduced lesions of the cerebellar dentate nuclei. N Engl J Med 2002;
346: 68-9.
6) Trivedi R, Mundanthanam G, Amyes E, Lang B, Vincent A. Antibody
screening in subacute cerebellar ataxia. Lancet 2000; 356: 565-6.
7) Jellinek E, Kelly R. Cerebellar syndrome in myxoedema. Lancet 1960;
ii: 225-7.
8) Barnard RD, Campbell MJ, McDonald MI. Pathologic findings in a case
of hypothyroidism with ataxia. J Neurol Neurosurg Psychiatry 1971; 34:
755-60.
9) Cooke WT, Smith WT. Neurological disorders associated with adult
celiac disease. Brain 1966; 89: 683-722.
10) Hadjivassiliou M, Grunewald RA, Chattopadhyay AK, et al. Clinical
radiological, neurophysiological and neuropathological characteristics of gluten
ataxia. Lancet 1998; 352: 1582-5.
11) Aw TC, Vale JA. Poisoning from metals. Mercury. In : Oxford
Textbook of Medicine. Third edition. Vol 1. Eds. Weatherall DJ, Ledingham
JGG, Warrell DA. Oxford Medical Publications. Oxford University Press.
Oxford. 1996; pp 1111-2.
12) Harding AE. The hereditary ataxias and related disorders. Clinical
Neurology and Neurosurgery Monographs. Vol 6. Churchill Livingstone,
Edinburgh. 1984; pp 1-21.
13) Harding AE. Clinical features and classification of inherited ataxias.
Adv Neurol 1993; 61: 1-14.
14) Durr A, Bruce A. Clinical and genetic aspects of spinocerebellar
degeneration. Curr Opin Neurol 2000; 13: 407-13.
15) Engert JC, Brub P, Mercier J, et al. ARSACS, a spastic ataxia
common in northeastern Quebec, is caused by mutations in a new gene
encoding
an 11.5 kb ORF. Nature Genet 2000; 24: 120-5.

16) Wood NW. The spinocerebellar ataxias : genotype - phenotype

correlation. Bull Neurol Soc Thai 2001; 17(1)(Suppl): 11-7.
17) Orr HT. Hereditary Ataxia. An unfolded protein.
2001; 358: S 35.

Lancet (Suppl)

18) Holtmann M, Opp J, Tokarzewski M, Korn-Merker E. Human epilepsy,

episodic ataxia type 2, and migraine. Lancet 2002; 359: 170-1.

10
.....
31

15
2 2

15 3
1) Meleiodosis rickettsia
2)
3) connective tissue SLE, Rheumatoid arthritis

(chondritis)

T 38C
PR 84
RR 18
BP 100/60
slightly pale, no jaundice
no neck stiffness
Heart sounds normal
Liver : palpable one cm. below costal margin, span 13 cms.
Spleen not palpable
no lymphadenopathy

 ()


leukaemia lymphoma lymphoma
connective tissue

CBC ESR
autoimmune antibodies

Hb 7.9 Hct 24 MCV 80.9 aniso+ polychromasia+ tear drop 1+ Retic
1.8%
WBC 29900 N 87 L 8 M 5
Platelets 869,000
no malaria parasite
Urine : protein 1+
ESR 144 mm. 1st hour
SGOT 39
PT 40
GGT 309
Alk Phos 525
Alb / glob 30/47
anti-HIV -ve
VDRL & FTA-ABS non-reactive
Meleiodosis titre -ve, serology for scrub typhus, murine typhus ve
E. hist titre -ve fetoprotein 0.908 ng/ml (normal 0-7.02)
Coombs test -ve
ECG : normal
Chest xray : normal
Ultrasound abd : enlarged liver without space-taking lesion
Bone marrow : myeloid metaplasia

anaemia
ESR 100 . 2-3
paraproteinaemia MM
connective tissue

 anaemia
globulin rheumatoid arthritis Adult Stills disease
Stills disease SLE, Polyarteritis nodosa

Rheumatoid factor 41.9 iu/M (

15)

Adult Stills disease 90 Rheumatoid factor

Rheumatoid arthritis 100
George Still rheumatoid arthritis
E.G.L. Bywaters

Canadian Red Cross Hospital Taplow ( 1)

Epstein-Barr cytomegalovirus
Yersinia Mycoplasma pneumonia


(
2) 3-4
( 3 6)

( 7 13)
Paul Beeson

( 8) Beeson
Nuffield Professor

( .. 1970)
Beeson

pyrogen

interleukin 1 Beeson
Seattle Beeson

 Richard Rapport

!

1) Bywaters EG. Stills disease in the adult. Ann Rheum Dis 1971; 30:
121-33.
2) . Adult Stills disease.
2544; 12: 184-98.
3) Larson EB. Adult Stills disease, evolution of a clinical syndrome and
diagnosis, treatment and follow-up 17 patients. Medicine 1984; 63: 82-91.
4) Sampalis JS, et al. A controlled study of the long term prognosis of
adult Stills disease. Ann J Med 1995; 98: 384-8.
5) Mok CC, Lau CS, Wong RW. Clinical characteristics, treatment and
outcome of adult Stills disease in southern Chinese. J Rheumatol 1998;
25: 2345-51.
6) Schiller D, Mittermayer H. Hyperferritinemia as a marker of Stills
disease. Clin Infect Dis 1998; 26: 534-5.
7) Davies GR, Finch RG. Fever of unknown origin. Clin Med JRCPL 2001;
1: 177-9.
8) Petersdorf RG, Beeson PB. Fever of unexplained origin : report on 10
cases. Medicine 1961; 40: 1-30.
9) Arnow PM, Flaherty JP. Fever of unknown origin. Lancet 1997; 350:
575-80.
10) Blumenthal I. Fever - concepts old and new. J R Soc Med 1997;
90: 391-4.
11) Saper CB, Breder CD. The neurologic basis of fever. N Engl J Med
1994; 330: 1880-6.
12) Greenwood B. Fever and malaria. Lancet 1996; 348: 280-1.

13) Drenth JPH, Van der Meer JWM. Hereditary periodic fever. N Engl J
Med 2001; 345: 1748-57.
14) Rapport R. Physician : The life of Paul Beeson. Barricade Books, Fort
Lee, New Jersey 2001; 277 pp.

11

51

3 ..


1
10 ..

cranial nerves 8, 5 7
cranial nerve 10

cerebello-pontine angle acoustic neurinoma
meningioma
granuloma
nasopharyngeal cancer

conductive nerve deafness cancer nasopharynx

conductive deafness Rinnes test (tuning fork)

T 37.3C
PR 72 regular
BP 130/80
Nervous system : fully conscious, 1 ptosis eye
Fundi : normal
EOMs : full
Pupils : 1.5 mm.
L 2.5 mm.
v nerve : Corneal reflexes : impaired
hypalgesia over 1st + 2nd division
LMN facial weakness
Hearing : slightly impaired
Uvula : moved to L on phonation
Gag reflex : impaired on
no other abnormality

L normal

General Examination
no cervical lymphadenopathy
Abd : no mass felt. Liver and spleen were not palpable

cranial nerves 10, 9, 8, 7

5 ( 1 2)


1) CT skull base
2) chest xray
3) ESR
4)
ESR cast
mid-line granuloma Wegeners granulomatosis
autoantibodies ANCA anti-DNA

CT : nasopharyngeal mass on the right side extending through skull

base also maxillary, sphenoid and ethmoid sinusitis
Chest xray : interstial and alveolar infiltration bilaterally
Urine : protein 1+
RBC 10-15/HPF
WBC 1-5/HPF
no cast
Hb 13.7
PCV 41.4
WBC 8100
N68 L21 E6 M1
ESR 125 mm/hr
Cr 1.1
SGOT 94
PT 133
TP 74.8
alb 34.3
glob 40.5
Protein electrophoresis alb 28.5 (40.3-62.3)
1 6.4 (2.97-4.0)
2 20.8 (8.1-11.3)
12.2 (10.3-13.4)
32.1 (20.9-26.7)
ANCA + 1:20 VDRL : non reactive
Anti DNA / PHA + 1:4
RF + 288.8 iu/ml
C-ANCA titre 1: 20 (ethanol fixed)
Wegeners granuloma

 .. 38 - 40 C
Bone Marrow aspiration culture nocardia
biopsy nasopharynx granulomatous inflammation
multinucleated giant cell caseation organisms
Wegeners granulomatosis
dexamethasone cyclophosphamide
1
prednisolone cyclophosphamide ESR 36 mm/1st
hour

Wegeners granulomatosis
autoimmune SLE Klinger ..
1931 5 Wegener 3 nasal granuloma
periarteritis nodosa
Wegener ( 1)
primary systemic vasculitis Churg-Strauss
syndrome Henoch-Schnlein purpura polyarteritis nodosa
Takayasu giant cell arteritis
( 10)
Wegeners granulomatosis
40-50
granuloma

3 (1) necrotising granuloma
(2) generalised focal necrotising vasculitis
(3) glomerulitis fibrinoid necrosis glomerular
tufts granuloma 4

case
records CPC N Engl J Med 3 10
( 5, 6 7)
7
pachymeningitis
( 8
9)


11, 12
13
10 systemic
necrotising vasculitis corticosteroids cyclophosphamide
5 48 80
15
systemic necrotising vasculitides
trimethoprim-

sulfamethoxazole co-trimoxazole
DeRemee
Escherlichia coli Wegener
( 12 13)

1) Stern GM. Wegeners granulomatosis. In : Handbook of Clinical

Neurology. Vol 39. Neurological manifestations of systemic diseases. Part
II. Eds. Vinken PJ, Bruyn GW. North-Holland Publishing Co. Amsterdam.
1980; pp 343-5.
2) Swanson JW. Neurological disorders in Wegeners granulomatosis. In :
Handbook of Clinical Neurology. Vol 27. Systemic diseases Part III. Eds.
Aminoff MJ, Goetz CG. Elsevier, Amsterdam. 1998; (71) pp. 173-89.
3) Stern GM, Hoffbrand AV, Urich H. The peripheral nerves and skeletal
muscles in Wegeners granulomatosis : clinicopathological study of four
cases. Brain 1965; 88: 151-64.
4) Godman GC, Churg J. Wegeners granulomatosis. Arch Path 1954;
58: 533.
5) N Engl J Med. Case Records of the Massachusetts General Hospital.
Weekly clinicopathological exercises. Eds. Scully RE, et al. 1989;
320: 1677-86.
6) N Engl J Med. Case Records of the Massachusetts General Hospital.
Weekly clinicopathological exercises. Eds. Scully RE, et al. 1990;
323: 1474-81.
7) N Engl J Med. Case Records of the Massachusetts General Hospital.
Weekly clinicopathological exercises. Eds. Scully RE, et al. 1999;
340: 945-53.
8) Wegeners
Granulomatosis 1 2544; 53: 161-5.
9) Manonuku. J, Atisook K, Wongbunnate S, Parichatikanond P,
Sookpatdhee V. Wegeners granulomatosis : Pulmonary necrotizing
granulomas mimicking Tuberculosis. Siriraj Hosp Gaz 1990; 42: 342-7.

10) Carruthers DM. Rational therapy in systemic necrotising vasculitis. Clin

Med JRCPL 2001; 1: 7-9.
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syndrome resembling Wegeners granulomatosis with low surface expression
of HLA class-I molecules. Lancet 1999; 354: 1598-1603.
12) Stegeman CA, Tervaert JWC, De Jong PE, Kallenberg CGM, For the
Dutch Co-trimoxazole Wegener study group. Trimethoprim-sulfamethoxazole
(co-trimoxazole) for the prevention of relapses of Wegeners granulomatosis.
N Engl J Med 1996; 335: 16-20.
13) DeRemee RA. Empiricism and Wegeners granulomatosis. Editorial. N
Engl J Med 1996; 335: 54-5.
14) Sumethkul V, Changsirikulchai S, Radinahamed P, Chalermsanyakorn P.
Antineutrophil cytoplasmic antibody (ANCA) and rapidly progressive
crescentic glomerulitis in Thai population. Asian Pac J Allergy Immunol
1999; 17: 281-7.

12

3
32 . .

3-4
1

1-6-12

1 2

2

3

sensory motor cranial nerve (CN)
5 lower motor neuron facial palsy (CN 7)
CN 10
acute polyradiculoneuritis Guillain-Barr syndrome
Epstein Bar virus

T 37.1C
PR 72
BP 120/80
alert, cooperative
lower motor neuron facial palsy
poor gag reflex and palatal movement on phonation
Limbs :
Tone normal

deltoids
biceps
triceps
wrist extenson
hand grip
hip flexors
quadriceps
hamstrings

4/5
4

4/5
4/5
4/5

3/5
2/5
4/5
4/5
3/5

4/5
3/5
2/5
4/5
4/5
3/5

absent deep reflexes in all limbs

P
JP normal
decreased sensation to pin prick in both hands and feet

Blood : anti HIV ve

CSF normal presssure, 5 mononuclear cells/co.mm
protein 130 mg%
sugar
50 mg% (blood sugar 94)
serum
anti GM1 0.015
0.034
10 days later anti GM1 0.069
0.054
Urine : porphobilinogen -ve
arsenic < 10 g/L

ALA 2.3 g/L

EMG : decreased amplitude of muscle potentials, conduction velocity

median nerve 59 metres/sec
L 56 metres/sec.
Findings are consistent with axonopathy

endotracheal tube
IVIg 400 u.

 5
plasmapheresis (2 IVIg) 5 plasmapheresis
2 42 endotracheal
tube 59 4 1
4
areflexia 2 vibration sense

()

Guillain-Barr syndrome 50
plasmapheresis
IVIg
intensive care unit
Guillain-Barr
Haymaker Kernohan ( 1)
Landry .. 1859
Guillain, Barr Strohl .. 1916 1956 Miller Fisher
Harvard

ophthalmoplegia, ataxia areflexia Miller Fisher syndrome

2
3
1) Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)

2) Acute motor axonal neuropathy (AMAN)
3) Acute motor sensory axonal neuropathy (AMSAN)
Miller Fisher syndrome, acute
panautonomic neuropathy pure sensory neuropathy
AMSAN EMG compound muscle
action potential amplitudes motor conduction velocities

corticosteroids GBS
40 20
Richard Hughes Guys
Plasma Exchange IVIg
Plasma Exchange

 (
34 35)
Guillain-Barr syndrome

40


37 !

1) Haymaker W, Kernohan JW. The Landry-Guillain-Barr syndrome : a

clinical pathologic report of 50 fatal cases and a review of the literature.
Medicine 1949; 28: 59-141.
2) Asbury AK. Guillain-Barr syndrome : Historical aspects. Ann Neurol
1990; 27 (suppl) : S2-S6.
3) Fisher CM. An unusual variant of idiopathic polyneuritis (syndrome of
ophthalmoplegia, ataxia and areflexia) N Engl J Med 1956; 255: 57-65.
4) Vejjajiva A. Acute inflammatory polyradiculoneuropathies : a clinical
study from Thailand. In Peripheral Neuropathy. Ed. Sobue I. Excerpta
Medica. Amsterdam. 1984; pp 114-22.
5) Young RR, Asbury AK, Corbett JL, Adams RD. Pure pan-dysautonomia
with recovery. Brain 1975; 98: 613-36.
6) Feasby TE, Gilbert JJ, Brown WF, et al. An acute axonal form of
Guillain-Barr polyneuropathy. Brain 1986; 109: 1115-26.
7) McKhann GM, Cornblath DR, Griffin JW, et al. Acute motor axonal
neuropathy : a frequent cause of acute flaccid paralysis in China. Ann
Neurol 1993; 33: 333-42.
8) Gibbels E, Giebisch U. Natural course of acute and chronic monophasic
inflammatory demyelinating polyneuropathies (IDP).
A retrospective
analysis of 266 cases. Acta Neurol Scand 1992; 85: 282-91.
9) Hahn AF. Management of Guillain-Barr syndrome (GBS). Baillieres
Clin Neurol 1996; 5: 627-44.

10) Guillain-Barr Syndrome Steroid Trial Group. Double-blind trial of

intravenous methyl prednisolone in Guillain-Barr syndrome. Lancet 1993;
341: 586-90.
11) Irani DN, Cornblath DR, Chaudhry V, et al. Relapse in Guillain-Barr
syndrome after treatment with human immune globulin. Neurology 1993;
43: 872-5.
12) Castro LH, Ropper AH. Human immune globulin infusion in Guillain-Barr
syndrome : worsening during and after treatment. Neurology 1993; 43:
1034-6.
13) French Cooperative Group on Plasma Exchange in Guillain-Barr
syndrome. Efficiency of plasma exchange in Guillain-Barr syndrome : role
of replacement Fluids. Ann Neurol 1987; 22: 753-61.
14) Bickerstaff ER, Cloake PCP. Mesencephalitis and rhombencephalitis. Br
Med J 1951; ii: 77-81.
15) Winer JB. Bickerstaffs encephalitis and the Miller Fisher syndrome.
J Neurol Neurosurg Psychiatry 2001; 71: 433-5.
16) Mori M, Kuwabara S, Fukutake T, Yuki N, Hattori T. Clinical features
and prognosis of Miller Fisher Syndrome. Neurology 2001; 56: 1104-6.
17) Jacobs BC, Van Doorn PA, Schmitz PI, et al. Campylobacter jejuni
infections and anti-GM1 antibodies in Guillain-Barr syndrome. Ann Neurol
1996; 40: 181-7.
18) Gregson NA, Jones D, Thomas PK, et al. Acute motor neuropathy with
antibodies to GM1 ganglioside. J Neurol 1991; 238: 447-51.
19) Ropper AH, Wijdicks EFM, Truax BT. Guillain-Barr syndrome. F.A.
Davis, Philadelphia, 1991.
20) Hann AF. Guillain-Barr syndrome. Lancet 1998; 352: 635-41.
21) Adogu AA. Guillain-Barr syndrome following severe exertion. JRSM
1991; 84: 116-7.
22) Hoffman O, Reuter U, Schielke E, Weber JR. SIADH as the first
symptom of Guillain-Barr syndrome. Neurology 1999; 53: 1365.

23) Combarros O, Pascual J, de Pablos C, Ortega F, Berciano J. Taste

loss as an initial symptom of Guillain-Barr syndrome. Neurology 1996; 47:
1604-5.
24) Hughes RAC. Sensory form of Guillain-Barr syndrome. Lancet 2001;
357: 1465.
25) Koga M, Yuki N, Hirata K. Antecedent symptoms in Guillain-Barr
syndrome : an important indicator for clinical and serological subgroups.
Acta Neurol Scand 2001; 103: 278-87.
26) Hadden RDM, Karch H, Hartung H-P, et al and the Plasma
Exchange/Sandoglobulin Guillain-Barr Syndrome Trial Group. Preceding
infections, immune factors, and outcome in Guillain-Barr syndrome.
Neurology 2001; 56: 758-65.
27) Fletcher DD, Lawn ND, Wolter TD, Wijdicks EFM. Long-term outcome
in patients with Guillain-Barr syndrome requiring mechanical ventilation.
Neurology 2000; 54: 2311-5.
28) Kuwabara S, Mori M, Ogawara K, Hattori T, Yuki N. Indicators of
rapid clinical recovery in Guillain-Barr syndrome. J Neurol Neurosurg
Psychiatry 2001; 70: 560-2.
29) Ahmed S, Libman R, Wesson K, Ahmed F, Einberg K. Guillain-Barr
syndrome : an unusual presentatin of West Nile virus infection. Neurology
2000; 55: 144-6.
30) Visudtibhan A, Visudhiphan P, Chiemchanya S, Wiengperm M. GuillainBarr syndrome in Thai children : Retrospective analysis of the clinical
features and outcome prior to intravenous immune globulin era. J Med
Assoc Thai 1998; 81: 750-6.
31) Phuapradit P. Guillain-Barr syndrome. Rama Med J 1995; 18: 145-6.
32) Pulkes T, Phuapradit P, Witoonpanich R, Boongird P. Guillain-Barr
syndrome : a clinical study. Rama Med J 1995; 18: 150-8.
33) Goh KJ, Ng WK, Vaithialingham M, Tan CT. A clinical and
electrophysiological study of Guillain-Barr syndrome in Malaysia. Neurol J
Southeast Asia 1999; 4: 67-72.

34) Wollinsky KH, Hlser P-J, Brinkmeier H, et al. CSF filtration is an

effective treatment of Guillain-Barr syndrome. Neurology 2001; 57: 77480.
35) Feasby TE, Hartung H-P. Drain the roots. A new treatment for
Guillain-Barr syndrome? Neurology 2001; 57: 753-4.
36) Hartung H-P, Kieseier BC, Kiefer R. Progress in Guillain-Barr
syndrome. Curr Opin Neurol 2001; 14: 597-604.
37) Kissel JT, Cornblath DR, Mendell JR. Guillain-Barr syndrome. In :
Diagnosis and management of peripheral nerve disorders. Contemporary
Neurology Series 59. Eds. Mendell JR, Kissel JT, Cornblath DR. Oxford
University Press, Oxford. 2001; pp 145-72.

13

25

10
2 3-4
2

acute disseminated encephalomyelitis, autoimmune

thrombotic thrombocytopaenic purpura SLE

T 37.5C conscious and followed command

neck stiffness present
EOMs full, bilateral LMN facial weakness
weakness of both legs (gr 2/5)
absent KJs and AJs
no Babinski sign
()
meningoencephalomyelitis CN 7 2

immune-mediated Guillain-Barr syndrome
10
serology


WBC 15800 N 91 L 9
SGOT 73
PT 152
CXR LLL pneumonitis
CSF : clear, 301 cells L 83 N 15 E 2
protein 70 sugar 91
Culture : no growth
PCR for TB and cryptococci -ve
Herpes IgM -ve
JE IgM +ve
EMG : small amplitude of compound muscle action potentials,
absent F-waves and normal sensory conduction
MRI : high signal T2 - weighted changes at both thalami, internal
capsules,
basal ganglia, midbrain, pons, cerebellum and temporal
cortices
with minimal leptomeningeal enhancement and intradural
tubular
enhancement along the cervical canal, conus medullaris and
cauda equina.
Serology
Dengue
JE
IgM
IgG
IgM
JgG
111
81
51

176
110

1st

22

2nd (10 days later)

11

3rd (

"

126
12

137

101
10

71

197

171

141
Japanese Encephalitus

JE 50,000
15,000 .. 1870
1935 Flavivirus

(flavi - yellow fever virus

)

1, 2
3 3
JE 2
Solomon Nicholas
White ( 4)

JE
( 5)
Gourie-Devi Bangalore (
7)
JE

(
8) SA 14-14-2
Scott Halstead
( 9)

Neurol J Southeast Asia ( 10)

1) Johnson RT. Japanese encephalitis virus. In : Viral infections of the

nervous system. Second edition. Lippincott-Raven. Philadelphia. 1998; pp
122-24.
2) Ravi V, Desai A, Shankar SK, Gourie-Devi M. Japanese Encephalitis.
In : Infectious Diseases of the Nervous System. Eds. Davis LE, Kennedy
PGE. Butterworth Heinemann. Oxford. 2000; pp 231-57.
3) Solomon T, Dung NM, Kneen R, Gainsborough M, Vaughn DW, Khanh
VT. Japanese encephalitis. J Neurol Neurosurg Psychiatry 2000; 68: 40515.
4) Solomon t, Kneen R, Dung NM, et al. Poliomyelitis-like illness due to
Japanese encephalitis virus. Lancet 1998; 351: 1094-7.
5) Edelman R, Schneider RJ, Vejjajiva A, Srikrirk-Krich N, Phornphiboul R,
Voodhikul P. Persistence of virus - specific IgM and clinical recovery after
Japanese Encephalitis. Am J Trop Med 1976; 25: 733-8.

6) Sharma S, Mathur A, Prakash V, Kulshreshtha R, Kumar R, Chaturvedi

UC. Japanese encephalitis virus latency in peripheral blood lymphocytes and
recurrence of infection in children. Clin Exp Immunol 1991; 85: 85-9.
7) Ravi V, Desai AS, Shenoy PK, Satishchandra P, Chandramuki A, GourieDevi M. Persistence of Japanese encephalitis virus in the human nervous
system. J Med Virol 1993; 40: 326-29.
8) Hoke CH, Nisalak A, Sangawhipa N, et al. Protection against Japanese
encephalitis by inactivated vaccines. N Engl J Med 1988; 319: 608-14.
9) Bista MB, Banerjee MK, Shin SH, et al. Efficacy of single dose SA 1414-2 vaccine against Japanese encephalitis : a case control study. Lancet
2001; 358: 791-95.
10) Phoncharoensri D, Witoonpanich R, Tunlayadechanont S, Laothamatas J.
Meningoencephalomyeloradiculopathy associated with Japanese encephalitis
virus : a case report with clinical, electrophysiological and neuroradiological
correlation. Neurol J Southeast Asia 2001; 6: 46.

14

27

6
2 2
2

1

2

2
3-4
1

2

retinitis optic neuritis

2

cauda equina

HIV 4

T 37.4C
PR 96
no neck stiffness
Eyes: VAR : no PL

BP 110/70
VAL 20/30

pale, no jaundice

retinitis c phlebitis
Pupils : fixed 6 cm.
L 3 mm. reacted to light
full movements
other cranial nerves : intact
Upper limbs : normal
Lower limbs : slight wasting of calf muscles

HF
2/5
Q
3/5
Hamstrings
2/5
DF
2/5
PF
KJ
AJ
P

hypalgesia over saddle area

loose anal sphincter tone

L
2/5
3/5
2/5
2/5
2/5

2/5

()
cauda equina
cytomegalovirus (CMV) HIV CMVPRAM (polyradiculomyelopathy) CRM (CMV radiculomyelitis)
polymorphonuclear
cells PCR

Hb 9.6
PCV 30.1
MCV 79
WBC 4400
P 83
L 15
M1
E1
anti-HIV + ve
CXR : no abnormality
CSF clear opening pressure 100 mm H2O
90 cells
PMN 60%
L 40%
protein 687 mg%
sugar
54 mg%
Indian ink stain : - ve


CSF cytospin preparation
immunostain CMV CMV inclusions nuclei cells

CMV

HIV 2
PRAM multifocal neuropathy (MN) 2
HIV
CMV-PRAM HIV CMV

2-4 CMV-MN
2 3-4
EMG axonal neuropathy ( 5)
nerve biopsy CMV inclusions nuclei multifocal endoneurial
necrosis
CMV-MN CMV-PRAM MN
PRAM
CMV-PRAM CMV-MN 3
gancyclovir, foscarnet cidofovir CMV retinitis
foscarnet gancyclovir Prognosis HIV
CMV
4 9 ( 8)

1) Brew BJ. HIV Neurology. Contemporary Neurology Series. Vol 61.

Oxford University Press. New York. 2001; 276 pp.
2) Anderson J. Infections in HIV disease. Clin Med JRCPL 2001; 1:
292-5.
3) Das CP, Sawhney IMS. Neurological complications of HIV infection.
Neurology India 1998; 46: 82-93.
4) Sacktor N, Lyles RH, Skolasky R, et al. HIV-associated neurologic
disease incidence changes: Multicenter AIDS Cohort Study, 1990-1998.
Neurology 2001; 56: 257-60.
5) Dalakas MC, Cupler EJ. Neuropathies in HIV infection. In : Baillieres
Clinical Neurology. International Practice and Research. Peripheral
Neuropathies.
Part II. Ed. Hartung H-P. Bailliere Tindall. London. 1996; 5: 199-218.

6) Bale Jr. JF, Jordan MC. Cytomegalovirus. In : Handbook of Clinical

Neurology. Vol 56. Viral Disease. Eds. Vinken PJ, Bruyn GW, Klawans HL.
Elsevier Science Publishers. Amsterdam. 1989; pp. 263-79.
7) Siritantikorn S, Nantharukchaikul S, Chavalidthamrong P, Sutthent R, Wasi
C, Tongcharoen P. Detection of human cytomegalovirus in urine of infants
by polymerase chain reaction. J Med Assoc Thai 1994; 77: 414-20.
8) Tsellis A, Lavi E. Cytomegalovirus infection of the adult nervous
system. In : Infectious Diseases of the Nervous System. Eds. Davis LE,
Kennedy PGE. Butterworth Heinemann. Oxford 2000; pp. 109-37.

15
!
29 .

2 7
12 2
2

1
2

optic neuritis (ON) demyelinating disease multiple

sclerosis (MS)
systemic lupus erythematosus
(SLE)

T 37.2C
PR 84
BP 130/80
slightly pale, round face
VAL: PL
VAL: nil
bilat pale disc with clear edges
Legs: power 0-1/5 in all muscles

Reflexes:
reflexes:

SJ

BJ
TJ
KJ
AJ
P
hypalgesia up to T10

L
+++

+++
+++
+++
++

+++
+++
+++
++

level

+++

Abd

 ()
T8
reflexes bilateral primary optic
atrophy

Hb 10.4
PCV 33.9
MCV 70
MCHC 30.8
MCH 21.5
RBC 4.86 x 106
WBC 6800
L 67 L 24 M 7 E 2
Platelets 272,000
ESR 26 mm/1st hour
Urine: protein trace
serum alb. 39.4
glob. 33.5
cholesterol 244
CH-50 37%
C3C 531 (normal 790-1400)
CSF clear, opening pressure 180 mm H2O
10 mononuclear cells
protein 111 mg%
sugar
63 mg% (blood sugar 171 mg%)

12

LE cells ANA +ve speckled > 1:256

Prednisolone 3
1
ANA +ve ring pattern 1:4

homogeneous

1:16
speckled > 1:256
anti DNA +ve 1:40
anti SM ve
C3C 285
anti RNP +ve
CH50 18
prednisolone cyclophosphamide 1
prednisolone

 SLE




Lancet
( 1)
10
migraine ESR

SLE
migraine SLE
( 2 3)
ON
MS Devics disease (neuromyelitis
optica) ESR LE cells
prednisolone
MS ( 4)
Michael Kremer Queens Square
Middlesex .. 1955-1975
Deborah Doniach
(Hashimoto) Brain .. 1972 (
5) lupoid sclerosis
acute myelitis SLE CSF mononuclear cells

direct smear india ink stain

Siekert Clark SLE
.. 1955 5 Granger (
6)
.. 1969 ( 7) SLE

1) Vejjajiva A. System lupus erythematosus presenting as acute

disseminated encephalomyelitis. Lancet 1965; 1: 352-3.
2) Brandt KD, Lessell S. Migrainous phenomena in systemic lupus
erythematosus. Arthritis Rheum 1978; 21: 7-16.

3) Vazquez-Cruz J, Traboulssi H, Rodriquez A, Geli C, Roig C, Diaz C.

A prospective study of chronic or recurrent headache in systemic lupus
erythematosus : is there an entity of lupus headache? Neurol J
Southeast Asia 1997; 2: 51-6.
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Multiple Sclerosis in Asia. Ed. Kuroiwa Y. University of Tokyo Press, Tokyo
1976; pp. 11-16.
5) Fulford KWM, Catterall RD, Delhanty JJ, Doniach D, Kremer M. A
collagen disorder of the nervous system presenting as multiple sclerosis.
Brain 1972; 95: 373-86.
6) Johnson RT, Richardson EP. The neurological manifestions of systemic
lupus erythematosus. A clinical-pathological study of 24 cases and review
of the literature. Medicine (Baltimore), 1968; 47: 337-69.
7) .
. 7 2512; 52:
673-86.
8) Deesomchok U, Tumrasvin T. Clinical features of systemic lupus
erythematosus in Thai males and females. J Med Assoc Thai 1992; 75:
133-40.
9) Kasitanon N, Louthrenoo W, Sukitawut W. Neuropsychiatric
manifestations in systemic lupus erythematosus (NPSLE): a 15-year
experience. Intern Med J Thai 2001; 17 (Suppl): 92.
10) Pender MP, Chalk JB. Connective tissue disease mimicking multiple
sclerosis. Aus NZ J Med 1989; 19: 469-72.
11) Goh KJ, Ahmad Sarji S, Omar S, Tan CT. Severe lupus
leukoencephalopathy a case report. Neurol J Southeast Asia 1997; 2:
185-90.
12) Donders RCJM, Kappelle LJ, Derksen RHWM, et al. Transient
monocular blindness and antiphospholipid antibodies in systemic lupus
erythematosus. Neurology 1998; 51: 535-40.

13) Ainiala H, Loukkola J, Peltola J, Korpela M, Hietaharju A. The

prevalence of neuropsychiatric syndromes in systemic lupus erythematosus.
Neurology 2001; 57: 496-500.
14) Wongchinsri J, Tantawichien T, Deesomchok U, Akkasilpa S. Infections
in Thai patients with systemic lupus erythematosus : A review of
hospitalized patients. Intern Med Thai 2001; 17 (Suppl): 90.
15) Ruiz-Irastorza G, Khamashta MA, Castellino G, Hughes GRV. Systemic
lupus erythematosus. Lancet 2001; 357: 1027-32.
16) Carpentier AF, Chassande B, Amoura Z, Benyahia B, Piette J-C,
Delattre J-Y. Systemic lupus erythematosus with anti-Hu antibodies and
polyradiculoneuropathy. Neurology 2001; 57: 558-9.

16

69 .

1
7
15 10
coronary artery bypass cholesterol
statin aspirin gr 1 1


nifedipine

(ventricles)

ventricle chronic subdural haematoma

MRI CT

fully conscious, answered questions correctly but speech was slightly

slurred and tended to fall asleep.
rather immobile facies
Fundi : bilateral early papilloedema
Limbs : Tone Coordination
but unsteady on standing
and walking
hip flexors & foot dorsiflexors 3/5 5/5
DTRs
+
+
equivocal plantar response bilaterally

PR 60 regular
Other systems :

BP 180/100 mm Hg
normal

MRI

MRI 4

MRI subdural haematoma

MRI

2

()
Chronic subdural haematoma (CSDH)
CT MRI

MRI
papilloedema


aspirin
-2 CSDH 3-4
(ventricle)

aspirin
lobar haematoma ( 11)
CSDH
( 5)
CT
CSDH ( 3)
CSDH

( 9)


( 7 8)
CSDH
Valentine
Logue Queens Square
( 1)

1) Logue V. Chronic subdural effusions. In : Modern Trends in

Neurology. Ed: Feiling A. Butterworth and Co., London. 1951; pp 363-90.
2) Markwalder TM. Chronic subdural haematoma : a review. J
Neurosurgery 1981; 54: 637-45.
3) Chou SM, Gutmann L. Deteriorating parkinsonism and subdural
hematomas. NeuroImages. Neurology 2001; 57: 1295.
4) Utsunomiya A, Narita N. Spontaneous occipital subdural haematoma
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10) .
3 2544; 17: 1075-7.

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2298-301.

17

26

3
3 2

Cerebral Malaria

T 36.9C
PR 84
RR 20
Stuporose, moderately jaundiced
no neck stiffness
Pupils
Liver and spleen were not palpable
Limbs : power 3-4/5
DTRs 2+

BP 120/80

Hb 10.5
Hct 31
MCV 83
Platelets 16,000
Plasmodium Falciparum malaria : ring form + ~ 10%
FBS 81 BUN 77
Cr 3.6
+
+
Na 147 K 5.03
Cl- 109
CO2 11.6
TB / DB 21/11 SGOT 115 PT 30 Alk Phos 147
Urine : protein 2+ glucose -ve
RBC 15-20
WBC 2-3


Artesunate quinacrine haemodialysis
2 Platelets 273,000 Hb 9.3
Hct 27
1
TB/DB 2 2/1.6
23
()

(Cerebral malaria)

artemisinin derivatives haemodialysis

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Psychiatry 2000; 69: 433-41.
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14) .
. 2540; 13: 669-80.
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2002; 359: 80.
20)
. 2001;
18 (4): 9-19.
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Oxford. 2000; pp. 1-41.
22) White NJ. Cerebral malaria.
.
(22)
.

18


70 .

7

.. 2518 7 2


11 .. 2518
1-2


(visual agnosia) visual fields
confrontation
tone DTRs Babinski response 2

VDRL FTA-ABS non-reactive
CSF cell 50 mg%
VDRL FTA-ABS CT scan
Creutzfeldt Jakob disease (CJD)
Heidenhain
1-2

myoclonic jerks
triphasic spike slow waves Hertz
CJD brain biopsy
spongiform gliosis neuronal
loss CJD

3


CJD

sporadic CJD 2

!
CJD .. 1920 Creutzfeldt
22 (
CJD ) Jakob 4
2 (myoclonic jerks)

(cortical blindness) Heidenhain type
Kiloh Nevin Queen Square
(EEG) cerebellum
ataxia, nystagmus dysarthria 1
GerstmannStrussler-Scheinker disease

30
scrapie
mink spongiform encephalopathy (BSE
)
Kuru
Fore

spongiform


!
CJD
Carleton Gajdusek
(NIH)
30 Dr. Clarence Gibbs
( 13) Gajdusek
(spongiform encephalopathies)

mononuclear cells slow virus

Stanley Prusiner San Francisco
Prion (proteinaceous infectious particle)
12
! Prusiner

CJD cornea

dura mater graft
growth hormone
pituitary


prion

(hypochlorite)

CJD 1 .. 2526
( 17)

CJD 4 . 20 (
18)

.. 1982 variant CJD (vCJD)

vCJD CJD
vCJD
( 29 30)

Robert
Will

4

The Lancet (Suppl.)

prion ( 31)

Arthur Beyless ( 32)
( 33 34)
quinacrine chlorpromazine (
Largactil malaria quinacrine)
( 36)
CJD 2
!
CJD vCJD


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33) . 2544;
10: 314-16.

34) .

2544; 10: 321-25.

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disease. 2001; 358: 563.

19
!
20

5




!

16

Gilles de la
Tourette


!
Gilles de la Tourettes syndrome (GTS) Tourettes disorder
tic

5
10,000 18
haloperidol 0.5

10 dopamine antagonist
haloperidol pergolide baclofen

Goerges Gilles de la
Tourette 9


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20

70 .
1 2518

1
2

3 4
48 (.. 2496 .. 1953)


1

2 58 (.. 2506)


gout

.. 2500

alert, cooperative and intelligent

no dysphasia, dyspraxia or agnosia
no L-R disorientation
Intellectual Function tests : all normal
Fundi normal
Visual fields : full to confrontation
Limbs : Tone
Power Gord
DTRs ++
no neck bruit
no abnormality on general examination
PR 72 regular
BP 110/70 mm.Hg

Heart and peripheral pulses : normal

Transient Global Amnesia

(TGA)

11 2538

110/70 mm.Hg 95/70

aspirin 1

.. 2518 (20 )
92 95
28 .. 2543
TGA 4-5
TGA .. 2518

20

Transient Global Amnesia (TGA) 50

Bender
TGA Guyotat Courjon
Miller Fisher Raymond Adams Boston
(monograph)
.. 1964 ( 1)
TGA 50


TGA

TGA



(working memory)
( procedural memory)
TGA
TGA
20 Transient Epileptic Amnesia (TEA) ( 13 15) TEA
2-3 1 TGA

 1 TEA
TEA TGA
(verbal memory)

TGA
spreading depression

transient ischaemic attack (TIA)

acetazolamide
( 17 18)

John Hodges
( 6, 7 22) TGA
mesial temporal lobes 2
spreading depression
Sander ( 24)
TGA venous ischaemia Steven Lewis
Lewis superior vena cava
TGA Valsalva manoeuvre
venous ischaemia
mesial temporal lobes diencephalon
Akkawi ( 25)
TGA 30
TGA
jugular valve
incompetence
Akkawi air-contrast ultrasound
venography (ACUV)


Tegeler 3 ( 23) Akkawi
TGA

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 1

1


1 Motor Neuron Disease
(MND) Amyotrophic Lateral Sclerosis (ALS)
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2

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3 Vertebral artery
dissection (VAD)

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Neurology 2001; 57: 1155-60.
4 Melioidosis
( 1)
Burkholderia pseudomallei
Melioidosis
Travel Medicine ( 2 3)

melioidosis
glanders-like disease
melioidosis ( 4)

bronchiectasis 5
( 5)
() (
6) 3-4
( 7)


Toxoplasmosis ( 8)

(typhus) () (malaria)
(ornithosis, psittacosis) (haemorrhagic fever) 9
(10)
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2) . - . .
2544; 10: 342.
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4) .
( ) : 2527.
5) 23 (-) 2
. .. 2534-2535 14465-9.
6) . . . 2544;
10: 565.
7) . . .
2544; 10: 764.
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Toxoplasma gondii in kidney recipients J Med Assoc Thai 2001; 84: 1137-41.
9) - 1 .

.. 2543 814-5.
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in association with staphylococcal endocarditis. Intern Med J 2001; 31: 130-1.
5 Wilsons disease
1
Walshe
1 penicillamine
3
10 ( 5)
Lancet !
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JRCPL
2001; 1: 72-4.
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Neurology
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shaver.
Lancet 2001; 358: 34.
6 Membranoproliferative glomerulonephritis (MPGN)
hepatitis C virus infection (HCV)
HCV 170

10

Hak Hotta

 Hotta
molecular
epidemiology HCV reprints

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 10

- Tolosa-Hunt Syndrome

Kline LB, Hoyt WF. The Tolosa-Hunt syndrome. Nosological Entities ?
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Behets disease with cerebral vasculitis. Internal Medicine Journal 2001;
31: 560-1.
6) Terrin G, Borelli O, Di Nardo G, Pacchiarotti C, Cucchiara S. A child with aphthae
and diarrhoea. Lancet 2002; 359: 316.

12 - Hypoparathyroidism

1) . Subtotal
thyroidectomy

 2544; 10: 70-5.

2) Ma RCW, Cockram CS. Clinical picture. Metastatic calcification. Lancet 2001;
359: 293.
13 . Mononeuropathy multiplex
Hypereosinophilia : HES ? associated with sarcocystis infestation

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14: 122-8.
14

1) Bunker CB, Reavley C, OShaughnessy DJ, Dowd PM. Calcitonin gene-related peptide
in treatment of severe peripheral vascular insufficiency in Raynauds phenomenon.
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Lancet 1993; 342: 1451-4.
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blood flow after a cold hand test in systemic lupus erythematosus in patients with
Raynauds syndrome. Lancet 1999; 354: 2135-6.
4) Mathieu A, Sanna G, Passiu G, Cauli A, Piga M. Raynauds syndrome in systemic
lupus erythematosus. Lancet 2000; 355: 1102-3.

 15 acute leukaemia - Acute

pancreatitis and diabetic ketoacidosis from L-asparaginase
pancreatitis
pancreatitis

sclerosing pancreatitis
1) OReilly DA, Kingsnorth AN. A brief history of pancreatitis. J R Soc Med 2001;
94: 130-2.
2) Hamano H, Kawa S, Horuichi A, et al. High serum Ig 4 concentrations in patients
with sclerosing pancreatitis. N Engl J Med 2001; 344: 732-8.

16 - Vitamin B 12 deficiency

1) Powers JM, Rosenblatt DS, Schmidt RE, et al. Neurological and neuropathologic
heterogeneity in two brothers with cobalamin C deficiency. Ann Neurol 2001;
49: 396-400.
2) Lindenbaum J, Healton EB, Savage DG, et al. Neuropsychiatric disorders caused
by cobalamin deficiency in the absence of anemia or macrocytosis. N Engl J Med
1988; 318: 1720-8.
3) Beck WS. Cobalamin and the nervous system. N Engl J Med 1988; 318: 1752-4.
4) Spence D. Uses of error : Knowledge gaps. Lancet 2001; 358: 1934.

17 - Streptococcus suis meningitis

1) Kay R, Wu A. Infection of the nervous system : an update on recent developments.
HKMJ 2001; 7: 67-72.
2) Fongcom A, Pruksakorn S, Mongkol R, Tharavichitkul P, Yoonim N. Streptococcus
suis infection in northern Thailand. J Med Assoc Thai 2001; 84: 1502-8.

 18 - Adrenomyeloneuropathy

1) Bezman L, Moser A B, Raymond GV, et al. Adrenoleukodystrophy : Incidence,
new mutation rate, and results of extended family screening. Ann Neurol
2001; 49: 512-7.
2) van Geel BM, Bezman L, Loes DJ, Moser HW, Raymond GV. Evolution of phenotypes
in adult male patients with X-linked adrenoleukodystrophy. Ann Neurol
2001; 49: 186-94.
3) Raymond GV. Peroxisomal disorders. Curr Opin Neurol 2001; 14: 783-7.
4) Berger J, Moser HW, Forss-Petter S. Leukodystrophies : recent development in
genetics, molecular biology, pathogenesis and treatment. Curr Opin Neurol
2001; 14: 305-12.
5) Achermann JC, Silverman BL. Dehydroepiandrosterone replacement for patients with
adrenal insufficiency. Lancet 2001; 357: 1381-2.
6) Di Rocco M, Doria-Lamba L, Caruso U. Monozygotic twins with X-linked
adrenoleukodystrophy and different phenotypes. Ann Neurol 2001; 50: 424.

20 - Thyrotoxic hypokalaemic periodic paralysis

1) Davies NP, Eunson LH, Samuel M, Hanna MG. Sodium channel gene mutations in
hypokalemic periodic paralysis : An uncommon cause in the UK. Neurology
2001; 57: 1323-5.
2) Davies NP, Hanna MG. The skeletal muscle channelopathies : basic science, clinical
genetics and treatment. Curr Opin Neurol 2001; 14: 539-51.
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Eds. Rose MR, Griggs RC. Butterworth Heinemann. Oxford. 2001; pp 326-34.
4) Tawil R. The periodic paralyses : hyperkalaemic and hypokalaemic paralysis.

In : Channelopathies of the nervous system. Eds. Rose MR, Griggs RC.

Butterworth Heinemann. Oxford. 2001; pp159-66.
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defects are associated with acetazolamide - exacerbated hypokalemic periodic
paralysis. Ann Neurol 2001; 50: 417-20.
6) Lam KSL, Benson EA, Yeung RTT, Wang C. Erythrocyte sodium-potassium pump
in thyrotoxic periodic paralysis. Aust NZ J Med 1989; 19: 6-10.

1
?
25 .

3
4
1
1-2

sensory cortex transient
ischaemic attacks TIAs
focal sensory
epilepsy

Sir Francis Walshe
Queen Square
50
(epileptic)
irritative phenomenon
motor cortex sensory cortex

motor area sensory cortex

paralytic phenomenon

TIAs
1)
carotid middle
cerebral artery atrial myxoma fibroelastoma

2) emboli aortic arch

(aortitis carotid arteritis)
3) cerebral arteriovenous malformation parietal cortex



1)
2) bruit 2 bruit
2
3)
4)
5) ophthalmoscope fundi

slightly obese but healthy looking woman

T 37C
PR 72 regular but weak radial pulses, R > L
B.P. 200/150 mm. Hg. at L popliteal fossa
Pulses

radial
femoral
popliteal
dorsalis pedis ++

L
+

+
++
+

+++
++
+++

Bruits were audible at both carotid, subclavian, femoral and

abdominal aorta
Nervous system: no abnormality
()
Takayasus arteritis

Aortogram / MRIA
ESR
FANA, LE cells anti-DNA

ESR 40 mm in 1st hour

Blood VDRL, TPHA non-reactive
Rheumatoid Factor, FANA - ve
anti-HIV - ve
Aortogram: moderate stenosis of abdominal aorta, both renal arteries
and both subclavian and carotid arteries
MRIA: findings compatible with Takayasus arteritis


Takayasu
aorta
2

8 2 ( 1)

( 2)
intima, media
adventitia autoimmune HLA A24-B52-DR 2
haplotype aortic arch aorta

descending aorta renovascular hypertension cerebral vascular
accident
mid-aortic syndrome
Takayasu American Journal of Medicine
( 3)
Takayasu


( 2 )
Takayasu vasculitis abducens nerve palsy
( 4) natural history
( 5 11) 12 14
1
Mikito Takayasu Kanazawa
Rokushu
Yamamoto .. 1830 Yamamoto
Takayasu
.. 1905
Takayasu 21 optic fundi

K.
Onishi T. Kagosha

Takayasu-OnishiKagosha Arteritis!
Eric Altschuler
Jeffrery Wheat Morgagni-Takayasu
Arteritis V. Di Giacoma Giovan Battista
Morgagni 40

 .. 1761 (
15) ! ?
Morgagni-Takayasu -
!

1) Numano F, Okawara M, Inomata H, Kobayashi Y. Takayasus arteritis.

Lancet 2000; 356: 1023-5.
2) Suwanwela N, Piyachon C. Takayasu arteritis in Thailand: clinical and
imaging features. Int J Cardiol 1996; 54(suppl): S 117-34.
3) Vinijchaikul K. Primary arteritis of the aorta and its main branches
(Takayasus arteriopathy). A clinico-pathologic autopsy study of eight cases.
Amer J Med 1967; 43: 15-27.
4) Amnueilaph R, Charoenvej P, Vejjajiva A. Pulseless disease presenting
with isolated abducens nerve palsy and recurrent cutaneous angiitis. BMJ
1973; 3: 27-8.
5) Strachan RW. The natural history of Takayasus arteriopathy. Q J Med
1964; 33: 57-69.
6) Ishikawa K. Natural history and classification of occlusive
thromboaortopathy (Takayasus disease). Circulation 1978; 57: 27-35.
7) Lupi-Herrera E, Sanches-Torres G, Marcoshamer J. Takayasu arteritis:
clinical study of 107 cases. Am Heart J 1977; 83: 94-103.
8) Chugh KS, Sakuja V. Takayasu arteritis as a cause of renovascular
hypertension in Asian countries. Am J Nephrol 1992; 12: 1-8.
9) Sun Y, Yip P-K, Jeng J-S. Ultrasonographic study and long-term
follow-up of Takayasus arteritis. Stroke 1996; 27: 2178-82.
10) Habermann CR, Mnzel T. Clinical Picture: Takayasus arteritis.
Lancet 2001; 358: 1050.
11) Yoshida S, Nakata T. Clinical picture: Takayasus arteritis. Lancet
2002; 359: 557.

12) Fraga A, Mintz G, Valle L, Flores-Izquierdo G. Takayasus arteritis:

frequency of systemic manifestations (study of 22 patients) and favorable
response to maintenance steroid therapy with adrenocorticosteroids (12
patients). Arthritis Rheum 1972; 15: 617-24.
13) Bali HK, Jain S, Jain AK. Stent supported angioplasty in Takayasu
arteritis. Int J Cardiol 1998; 66(suppl): S 213-7.
14) Bali HK, Bhargava M, Bhatta YK, Sandhu MS. Single stage bilateral
common carotid artery stenting in a patient of Takaya arteritis. Neurology
India 2001; 49: 87-90.
15) Altschuler EL, Wheat J. Morgagni-Takayasu arteritis. Lancet 2000;
356: 2013.

2

35 .

3





10 1

pericardial effusion pleural effusion

T 37.9C
RR 28
PR 120 regular
BP 100/70
alert, mildly pale and thin, no oedema
JVP 7 cm. above sternal angle
apex beat not palpable
distant heart sounds, no murmur, no pericardial rub
Chest: decreased tactile vocal fremitus L side
Decreased breath sounds over L side
Abdomen: Liver enlarged, palpable 2 FB below costal margin,
span 11 cm.
Spleen not palpable
Enlarged, rubbery, non-tender lymph node, 3 cm. in diameter
in L supraclavicular fossa

 jugular venous pressure

pericardial effusion


30-60

sternal
angle
pericardial effusion
Kussmauls sign

jugular venous pulse (JP)

JP
a x v y a v crests x
y trough c crest a x
z y cycle !
a atrium
(systole) atrial fibrillation a wave
a
( giant a wave) tricuspid pulmonary
stenosis complete heart block right atrium
tricuspid a cannon wave (cannon
)

pericardial effusion
pericardial effusion
paradoxical pulse apex beat
(percussion)
pericardial effusion
intercostal space effusion
250 !

effusion pericardial rub
effusion
pericardial
effusion
Paul Wood
50

Brompton

70
( 1)

!

 pericardial effusin
pericardial
effusion
1) pericardial effusion
2) effusion
ECG Echo cardiogram
effusion fluid HIV

CXR: cardiomegaly (globular shape), no pulmonary congestion

ECG: sinus tachycardia - low voltage and T wave abnormality
Echo: large pericardial effusion
Blood: anti-HIV +ve
Hb 9.8
32.9

PCV 29.8

MCV 82.7

MCH 27.2

MCHC

WBC 5810
N 82
L 11
M7
RBC 3.6x106
Urine: no abnormality
K+ 5.25
FBS 90
BUN 13
Cr 1.1
Na+ 136
Cl- 95
CO2 23.7
SGOT 53
PT 93
CPK/MB 65/18
TP/alb 85/31.9
ASO 143 iu/ml (normal 0-200)
HBsAg
-ve,
anti-HBS -ve
anti HBC/PHA +ve
anti HCV -ve
CD4 17 cells/ul
CD8 223
CD4/CD8 ratio
=

= 4%
(normal 28.5-60.5)
= 54% (11.1-38.3)
0.07 (0.9-3.6)

Pericardial tap: straw coloured fluid, 800 ml withdrawn

Fluid: RBC 3110
WBC 75 cells/cu.mm.
P 80% L 20%
Gram stain -ve
AFBs +
INH, rifampicin, ethambutol, pyrazinamide, Vit B6
co-trimoxazole 1

()

Pericardial effusion (TbPE)

mediastinum
40
3-4 2
TbPE 15
HIV

review
( 8)
calcific constrictive pericarditis 1 2 ( 12)
Paul Wood
.. 1961 ! pulsus paradoxus
Adolf Kussmaul
( 14)

1) Wood P. Physical signs. In: Diseases of the Heart and Circulation.

Second, revised and enlarged edition. Eyre and Spottiswoode. London
1959; pp. 26-72.
2) Drazner MH, Rame JE, Stevenson LW, Dries DL. Prognostic importance
of elevated jugular venous pressure and a third heart sound in patients with
heart failure. N Engl J Med 2001; 345: 574-81.
3) (i) Cayley WE and (ii) Ector H. Letters to the Editor in response to
(2) and the authors reply. N Engl J Med 2001; 345: 1912-3.
4) Harvey AM, Whitehill MR. Tuberculous pericarditis. Medicine 1937;
16: 45.
5) Peel AAF. Tuberculous pericarditis. Brit Heart J 1948; 10: 195.
6) Hageman JH, DEsopo ND, Glen WWL. Tuberculosis of the pericardiumA long-term analysis of forty-four cases. N Engl J Med 1964; 270: 327.
7) Strang JIG. Tuberculous pericarditis in Transkei. Clinical Cardiology
1984; 7: 667-70.
8) Karnkawinpong O, Sritara P. Tuberculous pericarditis. Rama Med J
1995; 18: 136-9.

9) Gibson DG. Pericardial disease. In: Oxford Textbook of Medicine.

Third Edition. Vol 2. Eds: Weatherall DJ, Ledingham JGG, Warell DA.
Oxford University Press, Oxford, 1996; pp. 2474-82.
10) Wragg A, Strang JI. Tuberculous pericarditis and HIV infection. Heart
2000; 84: 127-8.
11) Hakin JG, Temouth I, Mushangi E, Siziya S, Robertson V, Malin A.
Double blind randomised placebo controlled trial of adjunctive prednisolone
in the treatment of effusive tuberculous pericarditis in HIV seropositive
patients. Heart 2000; 84: 183-8.
12) Trautner BW, Darouiche RO. Tuberculous pericarditis: optimal
diagnosis and management. Clin Infect Dis 2001; 33: 954-61.
13) Ling LH, Oh JK, Breen JF, Schaff HV, Danielson GK, Mahoney DW, et
al. Calcific constrictive pericarditis: Is it still with us? Ann Intern Med
2000; 132: 444-50.
14) Bilchick KC, Wise RA. Paradoxical physical findings described by
Kussmaul: pulsus paradoxus and Kussmauls sign. Lancet 2002;
359: 1940-42.

40 .

2

. 3
6

2

5-10
2

6 6

4
visual field defect

2
binocular diplopia 2
monocular diplopia
anterior
posterior chambers binocular diplopia
lateral rectus medial rectus


2
lateral rectus cranial nerve 6 abducens nerve
superior inferior recti superior
/ inferior oblique cranial nerve 3 (oculomotor) cranial
nerve 4 (trochlea)

LR6 (SO4)3 lateral rectus

(LR) cranial nerve 6 superior oblique (SO) cranial nerve
4 cranial nerve 3 !



5
subdural haematoma
visual field defect subdural
haematoma

visual field defect
homonymous hemianopia

6

homonymous hemianopia parietal lobe signs
localizing signs

T 38C
PR 78 regular
BP 120/80
fully conscious, and thin looking person
no neck stiffness
- bilateral papilloedema
- homonymous hemianopia
Eyes movements : paresis of LR
Face
Tongue
Limbs : Power and coordination
slight weakness of hip flexors and dorsi flexors of both feet
SJ
++
++
BJ ++
++
TJ
++
++
KJ
+++
+++
AJ ++
++
P

sensation : doubtful impaired sensation to pin prick over

side
()

2 LR
homonymous hemianopia
2 DTRs Babinski response 2

 2 CT MRI

immunocompromised host
HIV, CBC CT scan

Blood : anti-HIV +ve
Hb 13
PCV 38.7
WBC 6700
N 74 L 17 M 5
E4
Platelets 316,000
Urine : protein trace
CT brain : multiple ring enhancing lesions involving right frontal,
temporal, right occipital lobes and right cerebellar hemisphere with extensive
perilesional oedema. A mass lesion at the left thalamus causes displacement of the
third ventricle to the right. There was increased leptomeningeal enhancement along
tentorium cerebelli and interhemispheric falx cerebriat the occipital region.
The findings are consistent with an infectious process such as
toxoplasmosis or tuberculosis.

HIV
(cerebellum)

contrast media toxoplasmosis

tuberculoma
Dye test toxoplasmosis

dye test
toxoplasmosis sulphadiazine pyrimethamine

co-trimoxazole pseudocystis carinii

()
HIV


104
(
20)
toxoplasmosis

Toxoplasmosis (protozoan) Toxoplasma

gondii toxos arch plasma
form gondii gundi (Ctedactylus gundi)
Monceau 100

(rat)
.. 1965 ..

.. 1980 ( 5)
3
1) definitive host ()

2)

3)
(choroidoretinitis) hydrocephalus
calcification congenital toxoplasmosis
dye

test Sabin Feldman

30 ~ 80 antibody



8000 T. gondii antibody
12 HIV 1200
500 HIV 200
200 ( 6 11)
antibody T. gondii gold standard dye
test
(
11) dye test

antibody T. gondii dye test
( T. gondii antibody ) antibody


( 1)
!
HIV
( 2) HIV HAART
(highly active antiretroviral therapy) 17

3
5000 !

Toxoplasmosis
Leptospirosis
!

1) Gutierrez Y. Tissue Apicomplexa. In : Diagnostic Pathology of

Parasitic Infections with Clinical Correlations. Second Edition. Oxford
University Press. Oxford 2000; pp. 201-34.
2) Mariuz P, Steigbigel RT. Toxoplasma infection in HIV-infected patients.
In : Toxoplasmosis. A comprehensive clinical guide. Eds: Joynson DHM,
Wreghitt TG. Cambridge University Press. Cambridge 2001; pp 147-177.
3)
2545; 27: 1128-35.
4) Tanphaichitra D. Toxoplasmosis. Intern Med 1987; 3: 181-7.
5) Bunyaratvej S, Chaimuangraj S, Pairojkul C, Tanpaichitr D.
Toxoplasmosis in adults : Report of three fatal cases by serological, light
and electron microscopic studies. J Med Assoc Thai 1980; 63: 568-77.
6) Chintana T, Sukthana Y, Bunyakai B, Lekkla A. Toxoplasma gondii
antibody in pregnant women with and without HIV infection. Southeast
Asian J Trop Med Public Health 1998; 29: 383-6.
7) Sukthana Y. Difference of Toxoplasma gondii antibodies between Thai
and Austrian pregnant women. Southeast Asian J Trop Med Public Health
1999; 30: 38-41.

8) Sukthana Y, Chintana T, Lekkla A. Toxoplasma gondii antibody in HIVinfected persons. J Med Assoc Thai 2000; 83: 681-4.
9) Sukthana Y, Chintana T, Supathanapong W, Siripanth C, Lekkla A,
Chiabchalard R. Prevalence of toxoplasmosis in selected populations in
Thailand. J Trop Med Parasitol 2000; 23: 53-58.
10) Sukthana Y, Damrongkitchaiporn S, Chintana T, Lekkla A. Serological
study of Toxoplasma gondii in kidney recipients. J Med Assoc Thai 2001;
84: 1137-41.
11) Sukthana Y, Chintana T, Supatanapong W, Siripan C, Lekkla A,
Chaebchalard R. Predictive value of latex agglutination test in serological
screening for Toxoplasma gondii. Southeast Asian J Trop Med Public Health
2001; 32: 314-8.
12) Wijdicks EFM, Borleffs JCC, Hoepelman AIM, Jansen GH. Fatal
disseminated hemorrhagic toxoplasmic encephalitis as the initial manifestation
of AIDS. Ann Neurol 1991; 29: 683-6.
13) Luft BJ, Haffner R, Korzun AH, et al. Toxoplasmic encephalitis in
patients with the acquired immunodeficiency syndrome. N Engl J Med
1993; 329: 995-1000.
14) Dina TS. Primary central nervous system lymphoma versus
toxoplasmosis in AIDS. Radiology 1991; 179: 823-8.
15) Raffi F, Aboulker JP, Michelet C, Reliquet V, Pelloux H, Huart A, et al.
A prospective study of criteria for the diagnosis of toxoplasmic encephalitis
in 186 AIDS patients. AIDS 1997; 11: 177-84.
16) Mathew MJ, Chandy MJ. Central nervous system toxoplasmosis in
acquired immunodeficiency syndrome : an emerging disease in India.
Neurol India 1999; 47: 182-7.
17) Maschke M, Kastrup O, Esser S, Ross B, Hengge U, Hufnagel A.
Incidence and prevalence of neurological disorders associated with HIV
since the introduction of highly active antiretroviral therapy (HAART).
J Neurol Neurosurg Psychiatry 2000; 69: 370-80.

18) Ammassari A, Cingolani A, Pezzotti P, De Luca A, Murri R, Giancola

ML, et al. AIDS-related focal brain lesions in the era of highly active
antiretroviral therapy. Neurology 2000; 55: 1194-1200.
19) Sacktor N, Lyles RH, Skolasky R, Kleeberger C, Selnes OA, Miller EN,
et al. HIV-associated neurologic disease incidence changes : multicenter
AIDS cohort study 1990-1998. Neurology 2001; 56: 257-60.
20)
.
: . 2545; 54:330-7.

4
:
29 .

5
6

2

2 6

septic arthritis
symmetrical polyarthritis

minor trauma

Gonococcal arthritis
50 Guys

gonorrhoea plantar fasciitis
bacterial endocarditis
2
immuno-reactive arthritis
urethral discharge
prostatic massage

T 38C
PR 84 regular
BP 130/70 mm. Hg.
no oral ulcer, erythematous patches over L forearm and arm
Joints: red, swollen first metacarpal joints of both hands
swollen wrist
swollen and warm knee with evidence of fluid

slightly swollen L ankle and dorsum of L foot

Cardiovascular system: normal
Abdomen: no mass felt
Lymph node 1.5 cm in diameter palpable in the inguinal
region
()

fluid glucose

Hb 13 gm%
WBC 25800
N 78 L 13
M6
st
anti HIV ve
ESR 39 mm/1 hour
Urine: protein trace, RBC 0-1, WBC 2-3/HPF
Rheumatoid factor + ve 22.6 iu/ml (normal 0-15)

E2

fluid 15 ml Gram stain

fluid glucose 14 mg% blood glucose 85 mg%
Haemoculture fluid Neisseria
gonorrhoea - betalactam positive

cloxacillin 1 6 . 1
ceftriazone 1 12 4 penicillin
ampicillin sensitive ceftriazone, norfloxacin amoxycillin / clavulinic
acid
2
8

sterno-clavicular temporo-mandibular joints
tenosynovitis

30 papular petechiae
endotoxin Neisseria pustules necrotic lesions

10-15 pericarditis
endocarditis

 Reiters Reiter

conjunctivitis, stomatitis keratoderma blenorrhagica

157 60
20 gonococci ( 1) 2
(

2)
3
( 4 6)
( 6 7)

1) Deesomchok U, Tumrasvin T. A clinical spectrum of infectious arthritis

at Chulalongkorn Hospital. Intern Med 1989; 5: 85-90.
2) Deesomchok U, Tumrasvin T. A clinical study of gonococcal arthritis.
Intern Med 1986; 2: 65-9.
3) Rosenthal J, Bole GG, Robinson WD. Acute gonococcal infectious
arthritis. Arthritis Rheum 1980; 23: 889-97.
4) Holmes KK, Counts GW, Mahony JDH. Disseminated gonococcal
infection. Ann Intern Med 1971; 74: 979-93.
5) OBrien JP, Goldenberg L, Rice PA. Disseminated gonococcal
infection: A prospective analysis of 49 patients and a review of
pathophysiology and immune mechanisms. Medicine 1983; 62: 395-406.
6) Csonka GW. Gonorrhoea. In: Oxford Textbook of Medicine. Second
Edition. Volume 1. Eds: Weatherall DJ, Ledingham JGG, Warrell DA.
Oxford University Press. Oxford. 1987; pp. 5.409-15.
7) Barlow D, Ison CA. Neisseria gonorrhoea. In: Oxford Textbook of
Medicine. Third Edition. Volume 1. Eds: Weatherall DJ, Ledingham JGG,
Warrell DA. Oxford University Press. Oxford. 1996; pp. 544-50.

23

2

2

2
2 cerebellar ataxia
nystagmus
cerebellum

pathognomonic sign

alert, no dysarthria
Fundi: normal
Pupils: normal
EOMs full Phasic nystagmus on looking to L and sustained
downbeat nystagmus on looking downwards and to L
were present
Face and Tongue: normal
Limbs:
Upper: slight spasticity L
Lower: moderate spasticity on both sides
Coordination: within normal limit
DTRs:

L
SJ
+
+++ c FF
BJ +
+++
Abd.
reflexes
TJ
+
+++
KJ
+++
+++
AJ ++
++

JPS
VS
Stance: rather unsteady
Gait: rather broad based
General examination: normal

PP

()
pyramidal signs 2
papilloedema
downbeat nystagmus (DBN)

cranio-vertebral junction
Chiari malformation type I multiple
sclerosis MRI
MRI
presence of Tonsillar ectopia. The cerebellar tonsil is located 2.7
cms. lower than the foramen magnum and the cervico-medullary junction is also
herniated lower than the foramen magnum. There are cranio-vertebral anomaly with
mild shortening of the clivus canal angle and sclerosis of the tip of dens. The 4th
ventricle and posterior fossa are well formed.
Diagnosis: Chiari type I and a short segment of syringohydromyelia
at the upper cervical cord ( )

posterior fossa decompression with duraplasty with nylon tube placement
suboccipital craniectomy with C1-C2 laminectomy cerebellar ectopia and
patent foramen caecum.
1
cerebellum
spasticity 2 DBN
6 DBN

Chiari malformation cerebellum

tonsil foramen magnum
Hans Chiari
.. 1891 1896 Chiari Julius
Arnold Heidelberg John
Cleland

 Chiari
4
Type 1 cerebellar tonsils
medulla oblongata spinal canal
Type 2 hydrocephalus pons
medulla spinal canal hydromyelia myelomeningocoele
diastematomyelia conus medullaris sacrum
Type 3 malformation tentorium cerebelli
cerebellum 4th ventrical cervical canal hydromyelia
ventricle occipital meningocoele
Type 4 cerebellar hypoplasia cyst Dandy-Walker cyst
(Dandy Earl Walker 2
)
Arnold spina bifida hydrocephalus
.. 1894 Chiari Type 2
Cleland 1 spina bifida hydrocephalus Jean
Cruveilhier 50
spina bifida hydrocephalus
Chiari malformation type 1 2 3..
Chiari malformation MRI

Chamberlains line, Macgregors line, Bulls angle,
Fischgolds line
digastric line ! MRI !
Downbeat nystagmus (DBN)
DBN
multiple sclerosis, cerebellar degeneration familial paraneoplastic,
brainstem infarction, Wernickes encephalopathy lithium
phenytoin DBN Chiari
malformation
DBN
DBN cerebellar flocculus Purkinje cells flocculus
central connection anterior semicircular canal
cerebellar flocculus DBN ( 10)
DBN
Chiari malformation positional down beating
nystagmus (pDBN) DBN 50 nystagmus

Dix-Hallpike manoeuvre pDBN cerebellum

anterior semi-circular canalithiasis ( 16)
Chiari malformations 17
MRI


nystagmus
( 19)
web site (www.jnnp.com) !

1) Pearce JMS. Historical Note. Arnold Chiari, or Cruveilhier Cleland

Chiari malformation. J Neurol Neurosurg Psychiatry 2000; 68: 13.
2) Spillane JD, Pallis C, Jones AM. Developmental abnormalities in the
region of the foramen magnum. Brain 1957; 80: 11-48.
3) Spillane JD. Developmental anomalies in the region of the foramen
magnum. In: Modern Trends in Neurology (Second Series). Ed: William D.
Butterworth & Co, London. 1957; pp. 240-57.
4) Bennett HS. Chiari malformations. In: Handbook of Cerebellar
Diseases. Ed: Lechtenberg R. Marcel Dekker, Inc. New York. 1993; pp.
141-5.
5) Pryse-Phillips W. Companion to Clinical Neurology. Little Brown and
Company, Boston. 1995; p-169.
6) Stovner LJ, Cappelen J, Nilsen G, Sjaastad O. The Chiari type I
malformation in two monozygotic twins and first degree relatives. Ann
Neurol 1992; 31: 220-2.
7) Santamarta D, Kusak ME, De Campos JM, Sierra JM. Increased
cerebrospinal fluid flow through the foramen of Magendie after
decompression for Chiari I malformation. J Neurol Neurosurg Psychiatry
1999; 66: 799.
8) Cogan DG. Down beat nystagmus. Arch Ophthalmol 1968; 80:757-68.
9) Baloh RW, Spooner JW. Down beat nystagmus: a type of central
vestibular nystagmus. Neurology 1981; 31: 304-10.
10) Zee DS, Yamazaki A, Butler PH, Gucer G. Effects of ablation of
flocculus and paraflocculus on eye movements in primate. J Neurophysiol
1981; 46: 878-99.

11) Halmagyi GM, Rudge P, Gresty M, Sanders MD, et al. Downbeating

nystagmus. A review of sixty-two cases. Arch Neurol 1983; 40: 777-84.
12) Bronstein AM, Miller DH, Rudge P, Kendall BE. Down beating
nystagmus: magnetic resonance imaging and neuro-otological findings. J
Neurol Sci 1987; 81: 173-84.
13) Yee RD. Down beat nystagmus: characteristics and localizations of
lesions. Trans Am Ophthalmol Soc 1989; 87: 984-1032.
14) Leigh RJ, Zee DS. Nystagmus caused by central vestibular imbalance.
In: The Neurology of Eye Movements. Eds: Leigh RJ, Zee DS. Edition 2.
Contemporary Neurology Series. FA Davis Company, Philadelphia.
1991; pp. 385-91.
15) Bhmer A, Straumann D. Patho-mechanism of mammalian downbeat
nystagmus due to cerebellar lesion: Simple hypothesis. Neurosci Lett
1998; 250: 127-30.
16) Bertholon P, Bronstein AM, Davies RA, Rudge P, Thilo KV. Positional
down beating nystagmus in 50 patients: cerebellar disorders and
possible anterior semicircular canalithiasis. J Neurol Neurosurg Psychiatry
2002; 72: 366-72.
17) Hadley DM. The Chiari malformations. J Neurol Neurosurg Psychiatry
2002; 72 (Suppl II): ii38-ii40.
18) Marti S, Palla A, Straumann D. Gravity dependence of ocular drift in
patients with cerebellar downbeat nystagmus. Ann Neurol 2002; 52: 712-21.
19) Serra A, Leigh RJ. Diagnostic value of nystagmus : spontaneous and
induced ocular oscillations. J Neurol Neurosurg Psychiatry 2002; 73:615-8.

54

1
2-3 1

1-2 20

T1-T2
T2 intercostobrachial nerve
sympathetic paralysis T1
Horners syndrome
50 20

2
superior pulmonary sulcus Pancoast syndrome
lower cervical upper thoracic spinal cord

1) finger clubbing
2) Horners syndrome
3) spinal cord compression
transverse
segmental sensory loss Babinski sign

T 37C

RR 20

PR 84

BP 160/100

moderate clubbing of fingers

no lymphadenopathy
Chest: decreased B.S. over RUL
TVR
tachea deviated to ?
Eyes: ptosis R
Pupils 3 mm. L 5 mm.
Face: forehead felt dry on the side
Other cranial nerves: normal
Limbs: Upper: slight wasting and weakness of hand muscles (gr
3/5)
Lower: moderate weakness of both hip flexors,
hamstrings and dorsi flexors of
feet

DTRs

SJ
BJ
TJ
KJ
AJ
P

+
++
++
+++
++

++
++
+++
++

Hypalgesia over legs and trunk up to C4-T2 junction and along the
inner border of arm and axilla (T2-C8)
()
cervical cord

Horners Syndrome finger clubbing

Pancoast tumour

CXR: shadow of mass in the right upper quadrant of the lung field
transbronchial biopsy poorly differentiated
carcinoma


Pancoasts syndrome finger clubbing
( !)
.. 1932 Henry Pancoast

superior pulmonary sulcus

Horner
( 1)
Pancoasts syndrome Pancoast-TobiasCiuffini syndrome Hare syndrome
lower nerve trunk brachial plexus (painful lower
brachial plexopathy with an added Horners syndrome) Pancoast
45 2-3 ( 2, 3 4)

( 3) Pancoast
Schwannoma ( 4)
clubbing

clubbing
clubbing
clubbing
( )

clubbing bronchiectasis, lung

abscess, empyema,
finger clubbing diffuse pulmonary fibrosis
clubbing asbestosis fibrosing alveolitis
Clubbing
cyanosis right to left shunt subacute bacterial endocarditis
clubbing congenital cyanotic heart clubbing

patent ductus arteriosus (PDA) aorto-pulmonary window (APW)
reversed shunt pulmonary hypertension
! shunt
subclavian artery
PDA APW
2 (cyanosed)
!
clubbing inflammatory bowel disease
ulcerative colitis
HIV ( 7 8) clubbing

 ( 9 10)
clubbing hypertrophic pulmonary osteoarthropathy (HPOA) 95
clubbing
vagus
vagus (vagotomy) clubbing

clubbing
systemic circulation reduced
ferritin

1) Pancoast HK. Superior pulmonary sulcus tumor: tumor characterized by

pain, Horners syndrome, destruction of bone and atrophy of hand muscles.
JAMA 1932; 99: 1391-6.
2) Arcasoy SM, Jett JR. Superior pulmonary sulcus tumours and
Pancoasts syndrome. N Engl J Med 1997; 337: 1370-6.
3) Phimphilai M, Bumroongkit C, Chaiwun B, Thongprasert S. A case of
small cell lung carcinoma with Pancoasts syndrome. Intern Med 1999; 15:
17-20.
4) Bozkurt AK. Schwannoma as a cause of Pancoasts syndrome. Internal
Medicine Journal 2002; 32: 108-9.
5) Douglas N. Fingers, clubbed. In: Frenchs Index of Differential
Diagnosis. Thirteenth Edition. Eds: Bouchier IAD, Ellis H, Fleming PR.
Butterworth Heinemann. Oxford. 1996 pp. 219-20.
6) Lane DJ. The clinical presentation of chest diseases. In: Oxford
Textbook of Medicine. Third Edition. Volume 2. Eds: Weatherall DJ,
Ledingham JGG, Warrell DA. Oxford University Press, Oxford. 1996 pp.
2642-52.
7) Graham SM, Daley HM, Ngwira B. Finger clubbing and HIV infection in
Malawian children. Lancet 1997; 349: 31.
8) Smyth A, Roberto N, Parker S, Tong CYW, Hart CA. Finger clubbing as
sign of HIV infection in children. Lancet 1997; 349: 575.

9) Reynen K, Daniel WG. Idiopathic clubbing. N Engl J Med 2000;

343: 1235.
10) Baragwanath P. Idiopathic clubbing. N Engl J Med 2001; 344: 611.

7

24

2



3-4
2 2

10

2
1. non-fluent motor
aphasia
2.


Transient ischaemic attack (TIA)
epileptic attack purpura,
ecchymoses menorrhagia
/
coagulation defect
2
thrombotic thrombocytopenic purpura (TTP)

acute leukaemia idiopathic
thrombocytopenic purpura ITP

T 38.2C
PR 120 regular
RR 20
BP 130/90
moderately pale, petechiae in both legs and at buccal mucosa
no neck stiffness
no enlarged lymph nodes
Liver: enlarged, 2 FBs below R costal margin
Spleen: palpable 1 FB below L costal margin

CNS:

dysphasic, unable to name familiar objects,

unable to write her name
Cranial nerves and limbs: normal
DTRs +++ and Babinski sign present bilaterally

()
acute leukaemia
nominal aphasia, agraphia Babinski sign 2
TTP ischaemic
attacks thromboses
MRI

Hb 6.6
Hct 19.6 Platelets 9000
WBC 8100 N 75 L 17 M 5 E 3
aniso 1+ poikilo 1+
few microspherocytes, rare schistocytes
SGOT 44
PT 34
LDH 847
anti DNA -ve, ANA positive, speckled, titre 1:40
CH50 75%
Coombs: direct and indirect -ve
Protein C 80% S 88%, antithrombin III 95%
Haemoculture -ve
anti -HIV, anti HBV, anti HCV
-ve
Urine: normal
erythroid hyperplasia thrombocytopenia with
adequate precursors suggestive of peripheral destruction
MRI: moderately irregular narrowing of bilateral transverse venous
sinuses, mild narrowing of both sigmoid sinuses and
parasagittal
sinus
thrombotic thrombocytopenic
purpura plasmapheresis prednisolone
1
Hct 39.3 prednisolone


TTP 3-4
CPC New England ( 1)

clopidogrel ( 2) simvastatin ( 3)

Moschowitz
.. 1925
TTP 40 85
CPC New England ( 4)
TTP ( 70)

aphasia

arterioles capillaries hyaline thrombi
CT MRI CT

(
5 7)

(
8) (
9) ( 10 - 12) TTP
(mutation) ADAMTS 13 active
protease von Willebrand factor TTP
IgG protease

1) N Engl J Med Case records of the Massachusettes General Hospital. Case

40-1998. Ed. Scully RE. 1998; 339: 2005-12.
2) Bennett CL, Connors JM, Carwile JM, Moake JL, Bell WR, Tarantolo SR, et
al. Thrombotic thrombocytopenic purpura associated with clopidogrel.
N Engl J Med 2000; 342: 1773-7.
3) McCarthy LJ, Porcu P, Fausel CA, Sweeney CJ, Danielson CFM.
Thrombotic thrombocytopenic purpura and simvastatin. Lancet 1998; 352:
1284-5.
4) N Engl J Med Case records of the Massachusetts General Hospital. Case
30-1991. Ed. Scully RE. 1991; 325: 265-73.
5) Kay AC, Solberg LA Jr, Nichols DA, Petitt RM. Prognostic significance of
computed tomography of the brain in thrombotic thrombocytopenic purpura.
Mayo Clinic Proc 1991; 66: 602-7.

6) Tardy B, Page Y, Convers P. Thrombotic thrombocytopenic purpura.

MR findings. AJNR 1993; 14: 489-90.
7) Bakshi R, Shaikh ZA, Bates VE, Kinkel PR. Thrombotic thrombocytopenic
purpura: brain CT and MRI findings in 12 patients. Neurology 1999; 52:
1285-8.
8) Oberlander DA, Biller J, McCarthy LJ. Thrombotic thrombocytopenic
purpura: a neurological perspective. J Stroke Cerebrovasc Dis 1995; 5: 175-9.
9) Hertzberg MS, Koutts J. Oral cyclophosphamide for refractory or relapsing
thrombotic thrombocytopenic purpura (TTP). Aust NZ J Med 1997; 27: 439.
10) Senior K. A molecular mechanism for thrombotic thrombocytopenic
purpura. Lancet 2001; 358: 1162.
11) Moake JL. Thrombotic microangiopathies. Mechanisms of disease.
N Engl J Med 2002; 347: 589-600.
12) Letters to the Editor : following (11) Thrombotic microangiopathies.
(i) Tarr PI, Tsai H-M, Chandler WL. (ii) Nzerue CM and the reply.
N Engl J Med 2002; 347: 2171-2.

8

47 .

2
4-5 atenolol
2
2
2
210/130 ..

Essential hypertension
Pheochromocytoma

Renal artery stenosis

Renal arterial fibromuscular
hyperplasia Takayasu Pheochromocytoma

T 37C

PR 84
BP 130/80 mm.Hg.
alert and healthy looking
Fund : hypertensive retinopathy grade 2.
Heart and Lungs : normal
Abd : no mass felt

()

paroxysmal hypertension

4 Keith, Wagner
Barker .. 1939 ( 1)
(essential hypertension)

1 :
retina

arteriolar light
reflexes
2 : A-V nicking

3 : flame-shaped haemorrhages soft

exudates

( retinal infarct) 1 2
4 : optic disc swelling (

oedema)

3
star-shaped exudate macula

Pheochromocytoma 2
(1) (imaging)

CT, MRI, SPECT ( [123I] MIBG

radioactive iodine 123 meta-iodo-benzyl guanidine) PET !
(sensitivity) (sensitivity)
CT
( 93)
MRI SPECT
metastasis ( 18)
2 (radial artery)
(femoral artery) Takayasu midaortic symdrome aortic co-arctation
bruit
2

2 bruit

PCV 37.3
BUN and blood electrolytes : normal
CT abdomen : solid mass in L adrenal gland, size 4.8 x 3.4 cm.
Myoma uteri about 3 cm. In diameter
Urine : 24-hour VMA 19.2 mg. (normal 0.5-12.0)

Pheochromocytoma
VMA 24
4 mg. 140/90 . 9

()

Pheochromocytoma chromaffin cell tumours

1 200
( 2) 38,596
Pheochromocytoma 22 (1 2031 0.05)
adrenal incidentaloma (
3) Pheochromocytoma 4
Chromaffin cell tumour pheochromocytoma
symapathetic ganglia organ of Zuckerkandl aortic bifurcation
paraganglioma 95
neural crest

10
rule of 10 Pheochromocytoma
10 10 1
10 10 10
10 ( 4)

(medical imaging)
(clinical study) 10 ( 5) !
Pheochromocytoma
Pheochromocytoma
Diencephalic epilepsy
.. 1929 Wilders Penfield
(Montreal)
( 6)
autonomic discharge

( 7) Pheochromocytoma

2
65

CT
CPC NEJM
70 .
pheochromocytoma
myasthenia gravis 4 (
.. 1995) 66 propranolol
myasthenia (

8) pheochromocytoma
CPC
NEJM 9 52
vena cava
atrium pheochromocytoma

( 10) 34
( 11) 26 stroke
cardiomyopathy ( 12) 13
36 von Hippel-Lindau 12
Robert Dluhy
Harvard 14 The Lancet
19

15 52 serum amylase
1109 U/L
( 38-119) 150/80 .
210/140
serum amylase pheochromocytoma acute
pancreatitis serum amylase

bronchial cell
cancer myeloma pheochromocytoma salivary isoenzyme
amylase
pheochromocytoma

25 (mutation) gene
gene
4 VHL, RET, SDHD SDHB ( 16)

pheochromocytoma

17 (
17)
NIH Conference Annal of Internal
Medicine ( 18) 19 20
!

1) Keith NM, Wagner HP, Barker NW. Some different types of essential
hypertension their course and prognosis. Am J Med Sci 1939;197: 332-43.
2) McNeil AR, Burke MP, Blok BH, Hilton JM, Koelmeyer TD.
Pheochromocytoma discovered during coronial autopsies in Sydney,
Melbourne and Auckland. Aust NS J Med 2000; 30: 648-52.

3) Mantero F, Terzolo M. Arnaldi G, Osella G, Masini AM, Ali A, et al. A

survey on adrenal incidentaloma in Italy. Study Group on adrenal tumors
of the Italian Society of Endocrinology. J Clin Endocrinol Metab 2000;
85: 637-644.
4) Bravo EL, Gifford RW Jr. Pheochromocytoma : diagnosis localization
and management. N Engl J Med 1984; 311: 1298-1303.
5) Dluhy RG. Pheochromocytoma Death of an axiom. N Engl J Med
2002; 346: 1486-8.
6) Penfield W. Diencephalic epilepsy. Arch Neurol Psychiatry 1929; 22:
358-74.
7) Charoenlarp K, Buranakitjaroen P, Gulprasutidilog S, Prayoonwiwat N,
Jaroonvesama N. Diencephalic epilepsy resembling pheochromocytoma :
A first reported case in Thailand. J Med Assoc Thai 1995; 78: 332-6.
8) Choi KL, Wat MS, Ip TP, Kung AWC, Lam KSL. Pheochromocytoma
associated with myasthenia gravis precipitated by propranolol treatment.
Aust NZ J Med 1995; 25: 257
9) Rote AR, Flint LD, Ellis Jr. FH. Intracaval recurrence of
pheochromocytoma extending into right atrium. Surgical management using
extracorporeal circulation. N Engl J Med 1977; 296: 1269-72.
10) N Engl J Med Case records of the Massachusetts General Hospital.
Weekly Clinicopathological Exercises. Eds. Scully RE, Mark EJ,
McNeely BU. Case 9-1985. 1985; 312: 568-75.
11) N Engl J Med Case records of the Massachusetts General Hospital.
Case 6-1986. Eds. Scully RE, Mark EJ, McNeely BU. 1986; 314: 431-39.
12) N Engl J Med Case records of the Massachusetts General Hospital.
Case 15-1988. Eds. Scully RE, Mark EJ, McNeely WF, McNeely BU.
1988; 318: 970-81.
13) N Engl J Med Case records of the Massachusetts General Hospital.
Case 16-1991. 1991; 324: 1119-28.
14) N Engl J Med Case records of the Massachusetts General Hospital.
Case 13-2001. Eds. Scully RE, Mark EJ, McNeely WF, et al. 2001;

344: 1314-20.
15) Ma RCW, Chan WB, Chow CC, Cockram CS. A woman with vomiting
and hyperamylasaemia. Lancet 2002; 359: 42.
16) Neumann HPH, Bausch B, McWhinney SR, Bender BU, Gimm O,
Franke G, et al for the Freiburg-Warsaw-Columbus Pheochromocytoma
Study group. Germ-line mutations in non syndromic pheochromocytoma.
N Engl J Med 2002; 346: 1459-66.
17) Pheochromocytoma
17 .. 2545
245-255.
18) Pacak K, Linehan WM, Eisenhofer G, Walther MM, Goldstein DS.
NIH Conference. Recent advances in genetics, diagnosis, localization and
treatment of pheochromocytoma. Ann Intern Med 2001; 134: 315-29.
19) Neumann HPH, Reincke M, Case 13-2001 : Genetic testing in
pheochromocytoma. N Engl J Med 2001; 345: 547-8.
20) N Engl J Med Germ-line mutations in nonsyndromic
pheochromocytoma. Letters to the Editor. 2002; 347: 854-5.

9

50 .

1
2
1


2
10 3
4

lower motor neuron lesion (LMN)
cranial nerve 7 upper motor
neuron lesion
2 facial nerve nucleus pons
facial nerve

2 cranial nerve nucleus 10 nerve

corticospinal tract
cerebellum
cerebellar tract cerebellum

10 3
2
cerebellum lower brain
stem

PR 66
regular
B.P. 100/60
thin, not anaemic
CNS :
Fundi : normal
slight ptosis

EOM : full, but nystagmus on lateral gaze to either side

Facial sensation : normal
L LMN facial palsy
decreased hearing L
gag reflex absent
Tongue : normal
Limbs : wasting of L deltoid and scapular muscles
power grade 3/5
L biceps and hand grip power 4/5
incoordination of all limbs L > R
DTRs +
+
P

no sensory loss
Tandem walk : abnormal
Other systems : no abnormality

()
cerebellum ataxia nystagmus
cranial nerve 7 8 (
) 10 upper motor neuron
deltoid scapula biceps hand grip
cervical nerve root brachial plexus
sympathetic nerve Horners syndrome

finger clubbing
cerebellum
cranial nerve cervical nerve root
metastasis ependymoma primary lymphoma
MRI biopsy

barium swallow
Hb 11.4
PCV 32.3
MCV 80.2
MCHC 35.3
WBC 7230 N 57 L 33 M 3 E 4
Platelets 236,000
TP 68.7
alb 41.7
Alk Phos 40
SGOT 13
PT 35

MRI : periventricular L
paramedian cerebellar vermis solid
th
enhancing mass around 4 ventricle and diffuse tumoural seeding with nodular
deposits
at bilateral frontal horns, lateral and 3rd ventricular floors,
infundibular recess
and glomus of bilateral choroid plexuses associated
with enhancing cisternal
portion of the left 3rd, 7th, 8th, 9th, 10th, 11th and
pre/post chiasmatic optic nerves and tracts. The most likely diagnosis is CNS
lymphoma ( )
Brain biopsy :
lymphocytic type.

malignant lymphoma, mixed small and large

Immunohistochemical studies revealed strong immunoreactivity for CD

20 and LCA but negative for CD 3 indicating B-cell lymphocytic subtype of
lymphoma

SIADH serum urine

electrolytes osmolarity

Na
-

Cl

SERUM

125

4.0

URINE

53

28

89

27

68

CO2
Osm 251
FBS 101
Cr 0.8 BUN 7

362

mOsm/kgH2O

SIADH methotrexate, Ca leucovorin,

dexamethasone viscristine
MRI 6
radiotherapy

Lymphoma (Primary CNS

lymphoma) lymphatic
system T-lymphocytes -
lymphocytes B-cells
lymphoma B-lymphocyte

1 2 -

ventricles
diabetes inspidus (
3)
( 4)
30

HIV 50
CNS lymphoma
low-grade astrocytoma meningioma 10
( 5)
( metastases )
Lymphoma 1 2
methotrexate
( 6 7)

( 8 9)
MRI ( 10)
( 11 14) lymphoma
T-cell 15
6 7 15
16 lymphoma
17 20

1) De Angelis LM. Primary central nervous system lymphoma. J Neurol

Neurosurg Psychiatry 1999; 66: 699-701.
2) Kppers R, Klein U, Hansmann M-L, Rajewsky K. Cellular origin of
human B-cell lymphomas. N Engl J Med 1999; 341: 1520-9.
3) N Engl J Med. Case records of the Massachusetts General Hospital.
Eds. Scully RE, et al. Case 36-1994. 1994; 331: 861-8.

4) N Engl J Med. Case records of the Massachusetts General Hospital.

Eds. Harris NL, et al. Case 10-2002. 2002; 346: 1009-15.
5) Nuckols JD, Liu K, Burchette JL, McLendon RE, Traweek ST. Primary
central nervous system lymphomas : a 30-year experience at a single
institution. Mod Pathol 1999; 12: 1167-73.
6) Freilich RJ, Delattre JY, Monjour A. Chemotherapy without radiation
therapy as initial treatment for primary central nervous system lymphoma
in older patients. Neurology 1996; 46: 435-9.
7) Herrlinger U, Schabet M, Brugger W, et al. German Cancer Society
Neuro-Oncology Working Group NOA-03 multicenter trial of single-agent
high-dose methotrexate for primary central nervous system lymphoma.
Ann Neurol 2002; 51: 247-52.
8) De Angelis LM, Cairncross JG. A better way to find tumor in the CSF?
Neurology 2002; 58: 339-40.
9) Gleissner B, Siehl J, Korfel A, Reinhardt R, Thiel E. CSF evaluation in
primary CNS lymphoma patients by PCR of the CDR III IgH genes.
Neurology 2002; 58: 390-6.
10) Bhring U, Herrlinger U, Krings T, Thiex R, Weller M, Kker W.
MRI features of primary central nervous system lymphomas at presentation.
Neurology 2001; 57: 393-6.
11) Ayuso-Peralta L, Ort-Pareja M, Zurdo-Hernndez M, Jimnez-Jimnez
FJ, Tejeiro-Martnez J, Ricoy JR, et al. Cerebral lymphoma presenting as
a leukoencephalopathy. J Neurol Neurosurg Psychiatry 2001; 71: 243-6.
12) De Angelis LM. Cerebral lymphoma presenting as a non enhancing
lesion on computed tomographic/magnetic resonance scan. Ann Neurol
1993; 33: 308-11.
13) Shams PN, Waldman A, Plant GT. B cell lymphoma of the brain stem
masquerading as myasthenia. J Neurol Neurosurg Psychiatry 2002;72: 271-3.
14) Abrey LE, De Angelis LM, Yahalom J. Long-term survival in primary
CNS lymphoma. J Clin Oncol 1998; 16: 859-63.

15) Gijtenbeek JMM, Rosenblum MK, De Angelis LM. Primary central

nervous system T-cell lymphoma. Neurology 2001; 57: 716-8.
15) Ferreri AJM, Reni M, Pasini F, Calderoni A, Tirelli U, Pivnik A, et al
for the International Extranodal Lymphoma Study Group. A multicenter
study of treatment of primary CNS lymphoma. Neurology 2002; 58:
1513-20.
16) Herrlinger U, Kker W, Platten M, Dichgans J, Weller M. First-line
therapy with temozolomide induces regression of primary CNS lymphoma.
Neurology 2002; 58: 1573-4.
17) Malkin D. Simian virus 40 and non-Hodgkin lymphoma. Lancet 2002;
359: 812-3.
18) Vilchez RA, Madden CR, Kozinetz CA, Halvorson SJ, White ZS,
Jorgensen JL, et al. Association between simian virus 40 and non-Hodgkin
lymphoma. Lancet 2002; 359: 817-23.
19) Shivapurkar N, Harada K, Reddy J, Scheuermann RH, Xu Y, McKenna
RW, et al. Presence of simian virus 40 DNA sequences in human lymphomas.
Lancet 2002; 359: 851-2.
20) Pagano JS. Viruses and lymphomas. N Engl J Med 2002; 347: 78-9.

10

55 .

2
3 2
NSAID
1

2

footdrop
anterior tibial peroneal
(flaccid footdrop) tone (gastrocnemeus soleus)
posterior tibial muscles spastic footdrop
hemiplegia stroke foot-drop 2
flaccid footdrop
flaccid footdrop
lateral
popliteal peroneal nerve nerve root L 5 S
1 tibialis posterior invert
tibialis posterior
flaccid footdrop lateral popliteal nerve

sciatic nerve sciatic nerve

lateral popliteal medial popliteal nerve


footdrop lateral
popliteal nerve
fibula ( !)
3 polyarteritis nodosa (PAN) leprosy

connective tissue PAN footdrop
vasa nervorum nerve infarct

cryoglobulinaemia
HIV hypereosinophilia (
1 3)
PAN mononeuritis multiplex


PAN
( 4)
lateral
popliteal nerve
2 PAN
1) 2)
3)
leprosy amyloidosis
4)
PAN mononeuritis multiplex
cryoglobulinaemia

T 37.5C
PR 90 regular
B.P. 150/90 mmHg
moderately pale
palpable lymph node, 1 x 1 cm in L cervical region
N.S. L foot: extensors 3/5 peroneal muscles 3/5
Postrtibial 5/5 gastrocnemius
foot normal

5/5

L hand: slight wasting of interosseous and

hypothenar muscles power 3/5, adductor pollicis
3/5
hand: normal
Impaired pin prick sensation over outer border of L foot
and palmar aspect of little and ring fingers of hand
()

lateral popliteal ulnar

mononeuropathy multiplex
PAN
ESR
connective tissue EMG nerve
conduction velocity

kidney muscle
biopsy PAN


Hb 8.6
PCV 25 MCV 83
MCH 28.8
Platelets 680,000
ESR 160 mm/1st hr.
Urine: protein 3+ (24 hr. protein = 1260 mg)
RBC > 20, WBC 15-20/HPF
Blood Cr 2.7 T.P./alb 77.7/31.5
Coombs test -ve
anti HIV -ve
Anti-HCV -ve
Serum Protein Electrophoresis

1.0)

albumin
1
2

%
33.7
5.9
16.4

(0.5-1.1)

MCHC 34.7

G/dl

Normal
2.1
(3.2-5.3)
0.4 (0.1-0.4)
1.0
(0.4-

12.0

0.8

31.9

2.0
(0.7-1.7)
Total 6.3 (6.2-8.2)
serum c-ANCA +ve > 1:320
(ethanol fix)

EMG showed signs of denervation in L anterior tibial, peronial

hypothenar and dorsal interosseous muscles. Nerve
Conduction
studies showed moderate slowing of clinically affected nerves as
compared to those on the opposite side. Sensory nerve and
compound muscle nerve action potentials were of low amplitude,
characteristic of a predominantly axonal process.
Chest xray: RUL infiltration compatible with T.B.
Kidney biopsy: crescentic glomerulonephritis

PAN
PAN SLE
30
150 Von Rokitansky .. 1852

periarteritis nodosa Kussmal

Maier .. 1866 polyarteritis

polyarteritis ( 5)
PAN vasculitis
autoimmune

PAN
connective tissue SLE
Rheumatoid arthritis
PAN sulphonamide serum
sickness metamphetamine

PAN PAN
polyangiitis
( 6)
( 7)
antibody cytoplasmic antigen polymorphonuclear
cell systemic vasculitis
indirect immunofluorescence ethanol-fix
neutrophils 2 3 cytoplasmic cANCA
Wegener perinuclear PANCA microscopic polyangiitis
Churg-Strauss glomerulonephritis
4
8
ANCA

vasculitic neuropathy
9 12
pulse methyl prednisolone
cyclophosphamide azathioprine

cyclophosphamide

( 11
12)
systemic vasculitis
13


1) Abramsky O. Neurologic manifestation of cryoglobulinemia. In:
Handbook of Clinical Neurology. Vol 39. Neurological manifestations of
systemic Diseases. Part II. Eds: Vinken PJ, Bruyn GW. Elsevier/NorthHolland Biomedical Press. Amsterdam. 1980; pp. 181-8.
2) Stricker RB, Sanders KA, Owen WF, Kiprov DD, Miller RG. Mononeuritis
multiplex associated with cryoglobulinemia in HIV infection. Neurology
1992; 42: 2103-5.
3) . 13
1
.. 2544 107-16.
4) Scolding N. Cerebral Vasculitis. In: Immunological and Inflammatory
Disorders of the Central Nervous System. Ed. Scolding N. Butterworth
Heinemann. 1999; pp. 210-57.
5) Goetz CG. Polyarteritis nodosa In: Handbook of Clinical Neurology.
Vol 39. Neurological manifestations of systemic diseases. Part II. Eds:
Vinken PJ, Bruyn GW. Elsevier/North-Holland Biomedical Press. Amsterdam.
1980; pp. 295-311.
6) Guillevin L, Lhotte F. Distinguishing polyarteritis nodosa from
microscopic polyangiitis and implications for treatment. Curr Opin Rheumatol
1995; 7: 20-24.
7) Lie JT. Nomenclature and classification of vasculitis: plus ca change,
plus c'est la meme chose (editorial). Arthritis Rheum 1994; 37: 181-6.
8) Sumethkul V, Changsirikulchai S, Radinahamed P, Chalermsanyakorn P.
Antineutrophil cytoplasmic antibody (ANCA) and rapidly progressive
Crescentic glomerulitis in Thai population. Asian Pac J Allergy Immunol
1999; 17: 281-7.

9) Kissel JT, Reithman JL, Omerza J, Rammohan KW, Mendell JR.

Peripheral nerve vasculitis: immune characterization of the vascular lesions.
Ann Neurol 1989; 25: 291-7.
10) Davies L. Vasculitic neuropathy. In: Clinical Neurology. International
Practice and Research. Inflammatory neuropathies. Ed. McLeod JG.
Baillire Tindall, London. 1994; 3: 193-210.
11) Said G. Vasculitic neuropathy. In: Clinical Neurology. International
Practice and Research. Peripheral Neuropathies. Part I. Ed. Hartung H-P.
Baillire Tindall, London. 1995; 4: 489-503.
12) Kissel JT, Collins MP, Mendell JR. Vasculitic neuropathy. In Diagnosis
and Management of Peripheral Nerve Disorders. Contemporary Neurology
Series 59. Eds: Mendell JR, Kissell JT, Cornblath DR. Oxford University
Press, Oxford. 2001; pp. 202-32.
13) Savage COS. The evolving pathogenesis of systemic vasculitis.
Clin Med JRCPL 2002; 2: 458-64.

11

48 .

2
5
4-5
2

!



5
hypoglycaemia
compensatory autonomic response


hypoglycaemia insulinoma

30 hypoglycaemic
coma insulinoma 2-3

coma
1
!
.

.


2 insulinoma
insulinoma 3



45-50 100 .
(hepatoma)
Hypoglycaemia paraneoplastic

syndrome Retroperitoneal fibrosarcoma

hypoglycaemia small cell
( 1)

Wt 90 kgs.
Ht 171 cms.
T 37C
BP 150/100
alert, cooperative
Heart: PMI 5th ICS MCL
normal sounds, no murmur
Abd: no mass felt
no other abnormality

PR 96

()

Hb 15
PCV 45.5
WBC 10900
N 78 L 13 M 5
E1
anti HIV -ve
FBS 50, 38, 37 mg% ( 3 )
Chest and Skull xrays: normal
serum T4 : total 7, free 0.9 T3 98 TSH 3.6
serum cortisol 7.8
prolactin 20.9 ( 2-15)
LH
4.5 ( 3-13)
FSH
15
( 0.9-15)
insulin
41 g/dl ( FBS 38 mg%)
GH
0.3
Oral glucose tolerance test
at 60 min
3.4 160
90 min
4.9
153
Ultrafast CT abdomen: mass, size 2x2.1 cm at pancreatic tail

 distal pancreatectomy FBS 118

mg%

2
insulinoma

ultrafast CT

12 13
sulphonylureas hypoglycaemia
C-peptide insulinoma
37 90

1) Seckl MJ, Mulholland PJ, Bishop AE, Teale JD, Hales CN, Glaser M, et
al. Hypoglycaemia due to an insulin-secreting small-cell carcinoma of the
cervix. N Engl J Med 1999; 341: 733-6.
2) Service FJ. Hypoglycemic disorders. N Engl J Med 1995; 332: 1144-52.
3) Malouf R, Brust JCM. Hypoglycemia: causes, neurological
manifestations and outcome. Ann Neurol 1985; 17: 421-30.
4) Lawrence JM, Smith T, Iles D, Reckless JPD. Difficult insulinoma. J R
Soc Med 2001; 94: 349-50.

5) Bazil CW, Pack A. Insulinoma presenting as seizure disorder.

Neurology 2001; 56: 817-18.
6) Lowy AJ, Chisholm DJ. Insulinoma masked by pregnancy. Intern Med J
2001; 31: 128-29.
7) Crimmins D, McLeod JG, Marks J, Morris JGL. Fits, confusion and
peripheral neuropathy in a young woman. Aust NZ J Med 1990; 20:623-8.
8) Mehndiratta MM, Garg N, Sareen D, Chowdhary A, Garg RK.
Insulinoma presenting with dementia and hemiplegia. Neurology India 1998;
46: 147-9.
9) Service FJ, McMahon MM, OBrien PC, Ballard DJ. Functioning
insulinoma incidence, recurence, and long-term survival of patients: a 60
year study. Mayo Clin Proc 1991; 66: 711-9.
10) Brightbill TC, Templeton EO, Sperling D, Mooney LP. Insulinoma:
detection by intraoperative ultrasonography. J Clin Ultrasound 1992; 20:
615-6.
11) Bliss RD, Carter PB, Lennard TWJ. Insulinoma: a review of current
management. Surg Oncol 1997; 49-59.
12) Marks V, Teale JD. Tumours producing hypoglycaemia. Endocr Relat
Cancer 1998; 5: 111-29.
13) Kwong PYP, Teale JD. Screening for sulphonylureas in the
investigation of hypoglycaemia. J R Soc Med 2002; 95: 381-5.

12

41 .

3 .

1) acute glaucoma 2)
optic neuritis 2
giant cell arteritis 50

VAR 20/200
VAL 20/20
Fundi: Swollen disc L normal
optic neuritis (papillitis)

methyl prednisolone (pulse) 1 3

1 2

VAR: hand movement only VAL: fingercount

EOM: bilateral lateral rectus paresis
80
50

exophthalmos chemosis

()
bilateral optic neuritis
lateral rectus muscles
cranial nerve 6 abducens nerve

 Wegeners granulomatosis
(arteritis) exophthalmos
Graves disease (ophthalmopathy)
granuloma granuloma
lymphocytic infiltration
orbital pseudo-tumour orbital myositis
MRI orbit
ESR ANA profile

MRI orbit : swelling of extra-ocular muscles compressing the 6th

and
optic nerves
Hb 12
Hct 40
WBC 10,200 N 77 L 14 M 4 E 5
st
ESR 36 mm/1 hour
TP 78.2 alb 42.8
Urine: Faeces: no ova, cysts or parasites
Prednisolone

2 VAR
20/100 VAL 20/70 ESR 14

optic neuritis
multiple sclerosis
ESR
orbital myositis optic neuritis

Lyme disease
(URI)
Whipples disease
Crohns disease Rheumatoid arthritis, SLE
paraneoplastic syndrome

1) Casteels I, De Bleecker C, Demaerel P. Orbital myositis following an

upper respiratory tract infection : contribution of high resolution CT and
MRI. J Belge Radiol 1991; 74: 45-47.

2) Seidenberg KB, Leib ML. Orbital myositis with Lyme disease. Am J

Ophthalmol 1990; 15: 13-16.
.
3) Orssaud C, Poisson M, Gardeur D. Orbital myositis, recurrence of
Whipples disease. J Fr Ophthalmol 1992; 15: 205-8.
4) Thurairajan G, Hope-Ross MW, Situnayake RD, Murray PI.
Polyarthropathy, orbital myositis and posterior scleritis : an unusual adverse
reaction to influenza vaccine. Br J Rheumatol 1997; 36: 120-3.
5) Dumo CA, Ehrlich R, Taylor R, Buncic JR, Hughes P, Griffiths AM.
Keeping an eye on Crohns disease : orbital myositis as the presenting
symptom. Can J Gastroenterol 1997; 11: 497-500.
6) Panfilio CB, Hernndez-Cossio O, Hernndez-Fustes OJ. Orbital myositis
and rheumatoid arthritis. Case Report. Arq Neuropsiquiatr 2000; 58: 174-7.
7) Grimson BS, Simons KB. Orbital inflammation, myositis, and systemic
lupus erythematosus. Arch Ophthalmol 1983; 101: 736-8.
8) Harris GJ, Murphy ML, Schmidt EW, Hanson GA, Dotson RM. Orbital
myositis as a paraneoplastic syndrome. Arch Ophthalmol 1994; 112: 3806.
9) Hankey GJ, Silbert PL, Edis RH, Nicoll AM. Orbital myositis : a study
of six cases. Aust NZ J Med 1987; 17: 585-91.
10) Mannor GE, Rose GE, Moseley IF, Wright JE. Outcome of orbital
myositis : clinical features associated with recurrence. Ophthalmology
1997; 104: 409-14.
11) Dua HS, Smith FW, Singh AK, Forrester JV. Diagnosis of orbital
myositis by nuclear magnetic resonance imaging. Br J Ophthalmol 1987;
71: 54-57.
12) Shah SS, Lowder CY, Schmitt MA, Wilke WS, Kosmorsky GS, Meisler
DM. Low-dose methotrexate therapy for ocular inflammatory disease.
Ophthalmology 1992; 99: 1419-23.
13) Mombaerts I, Koornneef L. Current status in the treatment of orbital
myositis. Ophthalmology 1997; 104: 402-8.

13

72 .

1 2
2
2
2
5-6
5

2
1
intermittent claudication of the cauda equina Blau
Valentine Logue .. 1961 ( 1)
cauda equina (limp)
leg claudication

(lumbar disc) (central protrusion) Blau Logue
Dejerine
Dejerine intermittent claudication of the spinal cord ..
1911

cauda equina

lumbar spinal canal lumbar canal stenosis
ankylosing spondylitis
intermittent
claudication of the cauda equina 50
sciatica

2
2 claudication

cauda equina
lumbar canal stenosis

lumbar canal


lumbo sacral plexus pelvic arteries
internal iliac arteries ( 6)
cauda equina

tendon reflex

PR 66 regular
healthy looking

BP 140/80

Legs: Peripheral arterial pulses are present

no wasting of muscles
Muscle power - all 5/5 (including extensor hallucis longus)

L
SLR
> 60
> 60
KJ
+
+
AJ
+
P

no sensory impairment
Lumbar spine movements: slight limitation of extension
()
reflex
S 1 2 cauda equina
claudication 5-10
extensor hallucis longus
L 5 reflex (KJ) L 3 L 4

MRI

MRI spine ( ) : L 3/4, 4/5

disc degeneration c lumbar canal stenosis

 ( )
2 1

SLR KJ AJ (1+
)

1) lumbar spinal canal /

cauda equina
2)

lumbar spinal canal
Blau Logue ( 1 4)
cauda equina

lumbar spinal stenosis lumbar spondylosis


sciatica
(intermittent

ischaemia) intermittent claudicatin of the cauda

equina

polyneuropathy

lumbar support

7
8

Lumbar spinal stenosis


1) Blau JN, Logue V. Intermittent claudication of the cauda equina. An
unusual syndrome resulting from central protrusion of a lumbar intervertebral
disc. Lancet 1961; i: 1081-6.
2) Jennett WB. A study of 25 cases of compression of the cauda equina
by prolapsed intervertebral discs. J Neurol Neurosurg Psychiat 1956;
19: 109-16.
3) Kavanagh GJ. Pseudoclaudication syndrome produced by compression
of the cauda equina. JAMA 1968; 206: 2477-81.
4) Blau JN, Logue V. The natural history of intermittent claudication of the
cauda equina. A long term follow-up study. Brain 1978; 101: 211-22.
5) Shaw PJ, Allcutt DA, Bates D, Crawford PJ. Cauda equina syndrome
associated with multiple lumbar arachnoid cysts in ankylosing spondylitis:
improvement following surgical therapy. J Neurol Neurosurg Psychiat
1990; 53: 1076-9.
6) Wohlgemuth WA, Rottach KG, Stoehr M. Intermittent claudication due
to ischaemia of the lumbosacral plexus. J Neurol Neurosurg Psychiat
1999;
67: 793-5.
7) Onel D, Sari H, Donmez C. Lumbar spinal stenosis; clinical-radiologic
therapeutic evaluation in 145 patients, conservative treatment or surgery?
Spine 1993; 18: 291-8.
8) Patel N. Surgical disorders of the thoracic and lumbar spine : A guide
for neurologists. J Neurol Neurosurg Psychiatry 2002; 73 (Suppl 1): i42i48.

14

37

7
2


3

c fibres

T8 7
lateral spinothalamic tract

c fibres
(myelin) c fibres lateral spinothalamic tract

2

3-4

1) (acute transverse myelitis)

multiple sclerosis

2) spinal cord infarction embolus anterior

spinal artery feeding arteries Adamkiewiazs arteries
paraplegia

3) spinal arteriovenous malformation

primary subarachnoid haemorrhage
spinal cord ischaemia

4) gnathostomiasis
c fibres

spinothalamic tract

reversed Brown-Sequard syndrome hemisection
spinal cord paralytic phenomenon hemi-irritation !
paraplegia
!

10
subarachnoid haemorrhage
WBC eosinophilia
!

healthy looking
neck stiffness present
1 ptosis L eye
Pupils L 2 mm. 3 mm., both reacted to light
Cranial nerves: L v ii and iii : impaired
pin prick sensation
Limbs :
motor power

arm 4/5, L 3/5

leg 1/5, L 0
leg weakness was detected in hip flexors, knee flexors and
foot dorsiflexors

Reflexes:

SJ
BJ
TJ
KJ
AJ
P

+++
+
+++
+
+

L
+
+
+

Sensation: JPS impaired in both big toes

impaired pin prick sensation from C 2 downwards

Anal sphincter tone: loose

()
C 2
Babinski sign
Horners syndrome

WBC CSF MRI

Hct 39 WBC 8700

N 60
E 17
L 15
M8
st
Platelets 317,000
ESR 54 mm/1 hour
CSF: clear, normal pressure
cells 60 E 60% L 40 %
protein 130 mg%
glucose 43 (blood glucose 157)
MRI spinal cord : slightly enlarged cord from cervico-medullary
junction to T 2 level presence of
tortuous
(? parasitic) tract from C 2 6 on the left side.

antibody
Angiostrongylus cantonensis

Rosen .. 1967 ( 5)
( 6 7)

4 ( 8)
radiculomyelitis 50
(radiculomyeloencephalitis)

(encephalitis) subarachnoid haemorrhage

Eric Schmutzhard
fellow 15
primary intracerebral haemorrhage 5
( 9)

 Schmutzhard
Innsbruck neurointensive care

.. 1836 Sir
Richard Owen
( 10)
..
1933 ( 11)

monograph
( 12)

1) Kerr DA, Ayetey H. Immunopathogenesis of acute transverse myelitis

Curr Opin Neurol 2002; 15: 339-47.
2) Parsons M. Vascular diseases of the spinal cord. In: Vascular
Disease of the Central Nervous System. Second Edition. Ed. Ross Russell
RW. Churchill Livingstone. Edinburgh. 1983; pp. 439-52.
3) Aminoff MJ, Logue V. Clinical features of spinal vascular malformations.
Brain 1974; 97: 197-210.
4) Tattersall R, Turner B. Brown-Squard and his syndrome. Lancet
2000; 356: 61-63.
5) Chitanond H, Rosen L. Fatal eosinophilic encephalomyelitis caused by
the nematode Gnathostoma spinigerum. Amer J trop Med Hyg 1967;
16: 638-45.
6) Punyagupta S, Juttijudata P, Bunnag T, Comer DS. Two fatal cases of
eosinophilic myeloencephalitis a newly recognized diseease caused by
Gnathoma spinigerum. Trans roy Soc trop Med Hyg 1968; 62: 801-9.
7) Punyagupta S, Limtrakul P, Vichipanthu C, Kanchanachetanee C, Nye
SW. Radiculomyeloencephalitis associated with eosinophilic pleocytosis Report of nine cases. Amer J trop Med Hyg 1968; 17: 551-60.

8) Boongird P, Phuapradit P, Siridej N, Chirachariyavej T, Chuahiran S,

Vejjajiva A. Neurological manifestations of gnathostomiasis.
J Neurol Sci 1977; 31: 279-91.
9) Schmutzhard E, Boongird P, Vejjajiva A. Eosinophilic meningitis and
radiculomyelitis in Thailand, caused by CNS invasion of Gnathostoma
spinigerum and Angiostrongylus cantonensis. J Neurol Neurosurg Psychiat
1988; 51: 80-87.
10) Owen R. Gnathostoma spinigerum n. sp. Proc zool Soc (Lond) Part
4. 1836; 123-6.
11) Prommas C, Daengsvang S. Preliminary report of a study on the life
cycle of Gnathostoma spinigerum. J Parasitol 1933; 19: 287-92.
12) Daengsvang S. A monograph on the genus Gnathostoma &
Gnathostomiasis in Thailand. SEAMIC Publication No. 21, Tokyo. 1980; 85
pp.

15
50
51

5
5 2
1

50

4-5
1) (
) optic
nerve
(frontal and maxillary sinuses)
sphenoid sinus

2)
optic nerve arterial
aneurysm anterior cerebral artery
aneurysm
3) optic neuritis


4) non-arteritic anterior ischaemic optic atrophy (AION)
arteriosclerosis

5) giant cell arteritis
temporal artery ophthalmic artery

acute glaucoma

 visual field confrontation test

5

visual fields
visual field retinal infarct sector field defect
ESR

temporal artery
biopsy

T 37.4C
PR 78 regular
BP 120/80
Fully conscious, no neck stiffness
Fundi: disc blurred, veins not congested, oedema at macula
L disc normal, hard retinal exudates
VAR 5/200
VAL 20/50
Visual fields :

full
finger
counts
hand
movements
only
Other cranial nerves : normal
Limbs : absent AJs, otherwise no abnormality
Other systems : normal
Temporal arteries : not palpated
()
ankle jerk hard exudate
(neuropathy and retinopathy) sector field defect
macular oedema central retinal artery occlusion blurred disc
giant cell arteritis ESR biopsy


ESRs 62 mm./1st hour, 75 mm./1st hour ( 3 )
VDRL, FTA-ABS & TPHA non-reactive
Hb 14.7
PCV 44.6
WBC 11500
N 62 L 36 M 1 B 1
Urine : protein 1+
glucose 2+
CSF : clear, colourless fluid, normal pressure
2 mononuclear cells / cu.mm
protein 32 mg%, glucose 144 mg%

giant cell arteritis

!
giant cell temporal arteritis
50
3-4
150-200 Gteborg
9-13 28.6

50 Olmsted County
Minnesota
17 ( 1)

.. 2508 50
3-4

ESR ( !) giant cell arteritis
ESR
Polymyalgia rheumatica giant
cell arteritis 60 ( 2)
Bruce .. 1888 ( 3)
rheumatic gout
Bagratuni Oxford
10 7
Ann Rheum Dis 1953 anarthritic rheumatoid

disease Lancet BMJ ( 4 5) ..

1957 Barber polymyalgia rheumatica (
6)
giant cell arteritis
45
37.6C
ESR 45 . prednisolone 2-3
ESR 20
ESR 40 .
( 2)
Giant cell arteritis Ali Ibn Isu
Baghdad

10 Sir Jonathan Hutchinson

( 3 !)
19 Hutchinsons
triad interstitial keratitis notched teeth deafness
congenital syphilis! giant-cell arteritis Gilmour
( 7) Horton
( 8)
giant-cell arteritis

9 13

30 31


1)

vertebral artery

1 embryology
Ross Russell Wilkinson ( 14)
stroke 5
( 15)
2)
16
(
17)
( 18)
( 19)
( 21)
( 22)
3) ESR (

 20) biopsy
color duplex ultrasonography ( 23)
4)

Prednisolone

!
5) Prednisolone 2040 mg. 60-80 mg.
ESR
interleukin-6 ( 24) Prednisolone 2
( 25)
6) methotrexate prednisone ( 26)
corticosteroid folic acid
methotrexate ( 27)
7) (audit)
( 28)
8)
( 29-30)

( 31 32) CPC New England (

33)

1) Mason JC, Walport MJ. Giant cell arteritis : Probably underdiagnosed

and overtreated. BMJ 1992; 305: 68-9.
2) Salvarani C, Macchioni P, Boiardi L. Polymyalgia rheumatica. Lancet
1997; 350: 43-7.
3) Bruce W. Senile rheumatic gout. BMJ 1888; 2: 811-13.
4) Bagratuni L. Anarthritic rheumatoid disease. Lancet 1956; 2: 694-7.
5) Bagratuni L. Prognosis in the anarthritic rheumatoid syndrome. BMJ
1963; 1: 513-18.
6) Barber HS. Myalgic syndrome with constitutional effects : polymyalgia
rheumatica. Ann Rheum Dis 1957; 16: 230-37.

7) Gilmour JR. Giant-cell chronic arteritis. J Pathol Bacteriol 1941; 53:

263-77.
8) Horton BT, Magath TB, Brown GE. An undescribed form of arteritis of
the temporal vessels. Proc Mayo Clin 1932; 7: 700-1.
9) Russell RWR. Giant-cell arteritis. A review of 35 cases. Quart J Med
N.S. 1959; 28: 471-89.
10) Hamilton Jr CR, Shelley WM, Tumulty PA. Giant cell arteritis :
including temporal arteritis and polymyalgia rheumatica. Medicine 1971; 50:
1-27.
11) Graham E, Holland A, Avery A, Russell RWR. Prognosis in giant-cell
arteritis. BMJ 1981; 282: 269-71.
12) Huston KA, Hunder GG, Lie JT, Kennedy RH, Elveback LR. Temporal
arteritis : a 25-year epidemiologic clinical and pathological study.
Ann Intern Med 1978; 88: 162-7.
13) Scolding N. Cerebral Vasculitis. In: Immunological and Inflammatory
Disorders of the Central Nervous System. Ed. Scolding N. Butterworth
Heinemann. Oxford 1999; pp. 210-57.
14) Wilkinson IMS, Russell RWR. Arteries of the head and neck in giant
cell arteritis. A pathological study to show the pattern of arterial
involvement. Arch Neurol 1972; 27: 378-91.
15) Zeidler M, Hughes T, Zeman A. Confused by arteritis. Lancet 2000;
355: 374.
16) N Engl J Med Case Records of the Massachusetts General Hospital.
Weekly Clinicopathological Exercises. Eds. Scully RE, et al. Case 23-1986.
1986; 314: 1564-72.
17) Burton EA, Winer JB, Barber PC. Giant cell arteritis of the cervical
radicul vessels presenting with diaphragmatic weakness J Neurol Neurosurg
Psychiatry 1999; 67: 223-26.
18) Pignet V, Friedli A, Pessina P-A, Chavez P, Saurat J-H. Clinical picture
: Aching alopecia. Lancet 2002; 359: 1199.

19) N Engl J Med Case Records of the Massachusetts General Hospital.

Weekly Clinicopathological Exercises. Eds. Scully RE, et al. Case 35-1986.
1986; 315: 631-9.
20) Salvarani C, Hunder GG. Giant cell arteritis with low erythrocyte
sedimentation rate : frequency of occurrence in a population-based study.
Arthritis Rheum 2001; 45: 140-5.
21) Galetta SL, Balcer LJ, Liu GT. Giant cell arteritis with unusual flowrelated neuro-ophthalmologic manifestations. Neurology 1997; 49: 1463-5.
22) Caselli RJ, Hunder GG. Neurologic complications of giant cell
(temporal) arteritis. Semin Neurology 1994; 14: 349-53.
23) Schmidt WA, Kraft H, Vorpahl K, Vlker L, Gromnika-Ihle EJ. Color
duplex ultrasonography in the diagnosis of temporal arteritis. N Engl J Med
1997; 337: 1336-42.
24) Joseph FG, Scolding NJ. Cerebral vasculitis : a practical approach.
Practical Neurology 2002; 2: 80-93.
25) Hayreh SS. Steroid therapy for visual loss in patients with giant-cell
arteritis. Lancet 2000; 355: 1572-3.
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Fernndez-Gutirrez B. Combined treatment of giant-cell arteritis with
methotrexate and prednisone. A randomized, double-blind,
placebo-controlled trial. Ann Intern Med 2001; 134: 106-14.
27) Morgan SL, Alarcn GS. Methotrexate in giant-cell arteritis. Ann
Intern Med 2001; 135: 1006-7.
28) Dalbeth N, Lynch N, McLean L, McQueen F, Zwi J. Audit of the
management of suspected giant cell arteritis in a large teaching hospital.
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2000; 20: 67-72.

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Saporiti N. (iv) Blockmann DE. N Engl J Med 2002; 347: 2083-5.
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Eds. Harris NL, et al. Case 23-2002. 2002; 347: 272-8.

16

19

4

2 . .
.
3

2
1)

primary
subarachnoid haemorrhage (SAH)
2) sex headache

primary subarachnoid haemorrhage
1) ruptured cerebral aneurysm
20

2) ruptured cerebral arterio-venous malformation (AVM)

3)
4)

5) spinal
AVM cavernous malformation
spinal subarachnoid space

Kernigs sign
Fundi subhyaloid haemorrhage
(bruit) cerebral AVM


T 37C
PR 72 regular
BP 120/80
alert, cooperative
stiff neck +
Kernigs sign +
Limbs : slight spasticity of both legs
DTRs

KJ
AJ
P

+++ +++
++++ ++++ (clonus +)

primary subarachnoid haemorrhage

2 DTRs Babinski sign
spinal AVM
CBC WBC eosinophilia LP
eosinophil MRI

Hb 14.8
Hct 45
WBC 12600/cu.mm. N 78
L 19
M3
LP : blood stained CSF, RBC 11,000/cu.mm. WBC 1,600 P 22
L 78
VDRL : non reactive
CT brain
MRIA : worm-like structure intramedullary at C 5-6 spinal level with
feeding arteries from both vertebral arteries
: ruptured spinal AVM
spinal angiogram embolization

AVM

2 dural intradural (+ intramedullary)
Type 1 Type 2 2
AVM


AVM 1
AVM

 SAH 2 embolization

Michael Aminoff
Muscle & Nerve

Valentine Logue 30
1 5
AVM SAH cavernous angioma
haemangioma
7
Spinal cord
strokes Caplans Stroke 3 ( 10)

1) Aminoff MJ, Logue V. Clinical features of spinal vascular malformations.

Brain 1974; 97: 197-210.
2) Aminoff MJ, Logue V. The prognosis of patients with spinal vascular
malformations. Brain 1974; 97: 211-8.
3) Aminoff MJ, Barnard RO, Logue V. The pathophysiology of spinal
vascular malformations. J Neurol Sci 1974; 23: 255-63.
4) Aminoff MJ. Spinal angiomas. Blackwell, Oxford. 1976.
5) Logue V. Angiomas of the spinal cord : review of the pathogenesis,
clinical features, and results of surgery. J Neurol Neurosurg Psychiatry
1979; 42: 1-11.
6) Di Chiro G, Doppman JL, Dwyer AJ. Tumors and arteriovenous
malformations of the spinal cord : Assessment using MR. Radiology
1985; 156: 689-97.
7) Kim CH, Kim HJ. Neurological Picture. Cervical subarachnoid floating
cavernous malformation presenting with recurrent subarachnoid
haemorrhage. J Neurol Neurosurg Psychiatry 2002; 72: 668.
8) Zevgaridis D, Mendele RJ, Hamburger C, et al. Cavernous
haemangioma of the spinal cord. A review of 117 cases. Acta Neurochir
(Wien) 1999; 141: 237-45.

9) Acciarri N, Padovani R, Pozzati E, et al. Spinal cavernous angioma : a

rare cause of subarachnoid hemorrhage. Surg Neurol 1992; 37: 453-61.
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approach. Third Edition. Butterworth Heinemann, Boston. 2000; pp. 43543.

17

61

3
1
..
..

2
3
1

1)

1 ..
2)

2 bruit 2

PR 84/min. regular B.P. 225/135 170/110 mm.Hg arm

JVP flat
Heart: PMI 6th ICS, outside MCL
normal heart sounds, no gallop
no murmur
arterial pulses: equal in all extremities
no carotid or abdominal bruit
CNS: Cranial nerves no facial palsy EOM: full but nystagmus on

vertical and lateral gazes were present

Limbs: impaired coordination arm (F. Ntest)
leg: slightweakness in all muscle groups
(4/5)
KJ
+++ +++
AJ
++
++
P

sensation to pin prick impaired in leg

()

pons vertical nystagmus

pons pyramidal signs
2 extensor plantar Babinski responses ataxia

infarction pons

reno-vascular hypertension
pheochromocytoma

2 1

dissecting aortic aneurysm
ECG
myocardial infarction

CT brain: acute infarction of L cerebellum and L side of pons

ECG: normal sinus rhythm, L axis deviation
ST - T abnormality suggestive of lateral wall ischaemia
CXR: dilated thoracic aorta with linear calcification
- to consider aortic dissection
Ultrafast CT: showed aneurysm of distal aortic arch and descending
thoracic aorta extending to the origin of superior
mesenteric artery approximately 28.8 cms. in
length.
The maximal diameter of the aneurysm is at the
descending thoracic aorta, measuring 9.5 cms.
There is atelectasis of the lower lobe of the left
lung


aortic aneurysm
dissection thoracic aorta
atheroma
syphilis
ascending aortic arch atheroma ascending
aorta, arch descending aorta thoracic aortic aneurysm

aneurysm superior vena cava
(oesophagus) trachea
renal artery ultrafast scan

renovascular hypertension
infarct pons cerebellum MRI
MRI posterior
fossa MRIA
aneurysm
atheroma
aortic aneurysm


abdominal cortic aneurysms

1) Morris PJ. Aortic aneurysms. In: Oxford Textbook of Medicine. Third

Edition. Vol 2. Eds: Weatherall DJ, Ledingham JGG, Warrell DA. Oxford
University Press, Oxford. 1996; pp. 2364-5.
2) Lederle FA, Johsnon GR, Wilson SE, Chute EP, Littooy FN Bandyk D,
et al, for the Aneurysm Detection and Management (ADAM) Veterans
Affairs Study Group. Prevalence and associations of abdominal aortic
aneurysm detected through screening. Ann Intern Med 1997; 126: 441-9.
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aneurysm. Factors in the etiology. Arch Environ Health 1969; 19: 167-82.
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caused by atherosclerosis? Circulation 1992; 85: 205-11.

6) Doll R, Peto R, Wheatley K, Gary R, Sutherland I. Mortality in relation

to smoking: 40 years observation on male British doctors. BMJ 1994;
309: 901-11.
7) MacSweeney ST, Ellis M, Worrell PC, Greenhalgh RM, Powell JT.
Smoking and growth rate of small abdominal aortic aneurysms. Lancet
1994; 344: 651-2.
8) Jatchavala J, Tuntiwiwut C, Pekanan P, Pharachai M, Aimachariyachai M.
Abdominal aortic aneurysms: CT features in ruptured and non-ruptured
aneurysms. Rama Med J 1998; 21: 104-11.
9) Sriussadaporn S, Pak-art R, Chiamananthapong S, Tangchai W,
Nivatvongs S, Sirichindakul B, et al. Surgery of the abdominal aorta:
experience of a university hospital in Thailand. J Med Assoc Thai 2001;
84: 1655-60.
10) Lederle FA, Wilson SE, Johnson GR, Reinke DB, Littooy FN, Acher CW,
et al for the Aneurysm Detection and Management Veteran Affairs
Cooperative Study Group. Immediate repair compared with surveillance
of small abdominal aortic aneurysms. N Engl J Med 2002; 346: 1437-44.
11) The United Kingdom Small Aneurysm Trial Participants. Long-term
outcomes of immediate repair compared with surveillance of small abdominal
aortic aneurysms. N Engl J Med 2002; 346: 1445-52.
12) Thompson RW. Detection and management of small aortic aneurysms.
N Engl J Med 2002; 346: 1484-6.
13) The Multicentre Aneurysm Screening Study Group. The multicentre
aneurysm screening study (MASS) into the effect of abdominal aortic
aneurysm screening on mortality in men : a randomised controlled trial.
Lancet 2002; 360: 1531-9.

18

33 .

1
3 2

3
1) encephalopathy
metabolic
1

2) encephalitis
autoimmune acute multiple sclerosis
3) temporal lobe

1) herpes simplex
2)
3-4
3) Cryptococcus neoformans

4)
5)


dissemminated encephalitis, acute necrotizing leucoencephalitis

acute

T 37.5C
PR 84
BP 120/80
alert, slight neck stiffness
snout reflex +
sucking & rooting reflex +
Fundi
Limbs: slight spasticity both arms and legs
DTRs 2+, no Babinski response
no other abnormality
()
meningoencephalitis
primitive reflexes

1) MRI CT 2) 3) WBC
serology 4)
cysticercosis

Hb 12.9
PCV 38.2
WBC 9100 N 82 L 13 M 3 E 1 B 1
Platelets 137,000
L.P.: CSF OP/CP 22/14 cm H2O, clear colourless fluid
WBC 510, all were mononuclear cells
Protein 126, glucose 63 (blood glucose 106)
Direct smear: AFB stain, India Ink -ve
no growth on culture
Blood: anti-HIV -ve
VDRL, FTA-ABS -ve
MRI brain: numerous ring enhancing cystic lesions in brain
parenchyma, supra and infra tentorially
Chest, thigh and arm x rays: no calcification
: cerebral cysticercosis

 Dexamethasone IV 5 mg 6 7

Albendazole 400 mg -
MRI
2 cysts

()
Cysticercosis
30
4-5

meningoencephalitis
30 CT scan MRI
antibody
mild jaundice SGOT PT SLE
LE cells
eosinophil 17
3-4
cysticercosis

cerebral angiogram arteritis
hydrocephalus theco-peritoneal shunt
neurocysticercosis

1 Bickerstaff

5 Brutto
6
MRI
Spina-Franca
cysticercus antigen
10
20
Spina-Franca 80

4
neurocysticercosis
8
Spina-Franca
1500 60 9 11

!

 ( 13)
44 3
.. 2541-2544

1) Bickerstaff ER. Cebral cysticercosis.

manifestations. BMJ 1955; i: 1055-58.

Common but unfamiliar

2) Madrazo I, Olhagaray B, Becerra M, Sandoval MA, Soto R. Acute

cysticercosis encephalitis : description of a histologically confirmed case.
Neurosurgery 1983; 13: 593-5.
3) Rangel R, Torres B, Del Brutto O, Sotelo J. Cysticercotic encephalitis :
a severe form in young females. Am J Trop Med Hyg 1987; 36: 387-92.
4) Ratanakorn D. Neurocysticercosis. Travel Medicine International 1997;
15: 24-29.
5) Del Brutto OH. Neurocysticercosis. Curr Opin Neurol 1997; 10: 26872.
6) Del Brutto OH, Rajshekhar V, White AC, Tsang VCW, Nash TE,
Takayanagui OM, et al. Proposed diagnostic criteria for neurocysticercosis.
Neurology 2001; 57: 177-83.
7) (i) Garg RK (ii) Katti MK Letters to the editor in response to (6) and
the authors reply. Neurology 2002; 58: 1315-6.
8) Dumas JL, Visy JM, Belin C, Gaston A, Goldlust D, Dumas M.
Parenchymal neurocysticercosis : follow-up and staging by MRI.
Neuroradiology 1997; 39: 12-8.
9) Spina-Franca A, Livramento JA, Machado LR. Cysticosis of the central
nervous system and cerebrospinal fluid. Immunodiagnosis of 1573 patients
in 63 years (1929-1992). Arq Neuropsiquiatr 1993; 51: 16-20.
10) Bueno EC, Vaz AJ, Machado LDR, Livramento JA. Neurocysticercosis.
Detection of IgG, IgA and IgE antibodies in cerebrospinal fluid, serum
and saliva samples by ELISA with Taenia solium and Taenia crassiceps
antigens. Arq Neuropsiquiatr 2000; 58: 18-24.

11) Machado LR, Livramento JA, Vaz AJ, Bueno EC, Mielli SR, Bastouly V,
et al. IgG intrathecal synthesis and specific antibody index in patients with
neurocysticercosis. Arq Neuropsiquiatr 2002; 60: 395-9.
12) Odashima NS, Takayanagui OM, de Castro Figueiredo JF. Enzyme
linked immunosorbent assay (Elisa) for the detection of IgG, IgM, IgE and
IgA against cysticercus cellulosae in cerebrospinal fluid of patients with
neurocysticercosis. Arq Neuropsiquiatr 2002; 60: 400-5.
13) , , ,
, , .
Neurocysticercosis : Clinical manifestations and assessments.
2545; 54: 394-402.

19

30

5
5
2

2

sella turica
1) sella pituitary gland
2)
sella

cranio-pharyngioma,
meningioma optic glioma cerebral aneurysm
3) sphenoid sinus
2

sella turcica sella

pituitary tumour
Cushing
ACTH 1
pituitary tumour

PR 76 regular
BP 130/90
Cushingnoid appearance with moon face, supraclavicular pads of fat
and buffalo hump
hersuitism +
truncal obesity c purplish striae
hyperpigmented areola
Fundi and visual fields : normal
Limbs :
power
upper
lower proximal 4/5

5/5
4/5

5/5

()
Cushings disease
myopathy
corticosteroid 3
1)
2) pituitary fossa adenoma sella-turcica
basophil adenoma ACTH
chromophobe non-secreting adenoma
3) CBC

Hb 15
PCV 48
FBS 81
Na+ 137 K+ 4.1 Cl 105
Cr 1.0
CO2 23
serum cortisol 3-4 8.00 . 15.3, 32 22 g/dl
16.00 . 13 g/dl
( 5-23 g/dl 8.00 . 3-15 g/dl 16.00
.)
24 hr urinary oxosteroids 2 10.8 14.4 mg
( 10 mg)
dexamethasone suppression test 2 mg 2
cortisol 15.5 g urinary-oxosteroids 8.6 mg/24
hr.
dexamethasone 8 mg 2

 2.6 g 5.3 mg
inferior petrosal sinus sampling
MRI pituitary adenomata 0.9 x 1.1 x 0.9 cm
L
0.6 x 0.5 x 0.8
transphenoial approach

Cushings syndrome
(..
2002) The Lancet schizophrenia
( 1) adrenal tumour

Cushings disease
Cushings
syndrome

2 5 2
cortisol 5
! 6, 7 8

Cushings syndrome (
9 13)
Cushing
2 25 ( 14)
Cushing pituitary-dependent Cushings disease
1
50 5 (pituitary
apoplexy)

Harvey Williams Cushing
(.. 1869-1939) Cushing

William Halstead Johns Hopkins
Sir William Osler Cushing Osler
Osler .. 1925 ( 15)
Cushing
Sir William Macewen (1848-1924) Sir Victor Horsley

(1857-1916) Cushing
(pituitary gland)
Cushing
.. 1988 ( 16) Cushing

3 ( 17, 18 19)
Harvey Cushing
( 20, 21 22)
( 23 24)

1) Zielasek J, Bender G, Schlesinger S, Friedl P, Kenn W, Allolio B, Lauer

M. A woman who gained weight and became schizophrenic. Lancet
2002; 360: 1392.
2) Boscaro M, Barzon L, Fallo F, Sonino N. Cushings syndrome. Lancet
2001; 357: 783-91.
3) Anderson JR, Antoun N, Burnet N, Chatterjee K, Edwards O, Pickard
JD, et al. Neurology of the pituitary gland. J Neurol Neurosurg Psychiatry
1999; 66: 703-21.
4) Newell-Price J, Grossman A. Diagnosis and management of Cushings
syndrome. Lancet 1999; 353: 2087-8.
5) Shimon I, Melmed S. Management of pituitary tumors. Ann Intern
Med 1998; 129: 472-83.
6) Oldfield EH, Doppman JL, Niemann LK. Petrosal sinus sampling with
and without corticotrophic-releasing hormone for the differential diagnosis of
Cushings syndrome. N Engl J Med 1991; 325: 897-905.
7) Miller DL, Doppman JL, Peterman SB, Niemann LK, Oldfield EH, Chang
R. Neurologic complications of petrosal sinus sampling. Radiology 1992;
185: 143-7.
8) :
.. 2545
65-90.

9) Kimmerle R, Rolla AR. Iatrogenic Cushings syndrome due to

dexamethasone nasal drops. Am J Med 1985; 79: 535-7.
10) Prebtani APH, Donat D, Ezzat S. Worrisome striae in pregnancy.
Lancet 2000; 355: 1692.
11) Arioglu E, Doppman J, Gomes M, Kleiner D, Mauro D, Barlow C, et al.
Cushings syndrome caused by corticotrophin secretion by pulmonary
tumorlets. N Engl J Med 1998; 339: 883-6.
12) Cuthbertson DJR, Vinjamuri S, Bowen-Jones. Clinical picture : Occult
ectopic ACTH syndrome. Lancet 2001; 357: 419.
13) Hui JYH, Woo PCY, Lo SS, Chan JCS. Over-the counter medication
and its effects. Lancet 2002; 359: 1120.
14) Chouenkitmongkol S, Bunnag P, Rajatanavin R, Ongphiphadhanakul B,
Puavilai G, Himathongkam T, et al. Treatment of Cushings
disease with bilateral adrenalectomy : a 25-year follow-up experience at
Ramathibodi Hospital. Rama Med J 2002; 25 (Suppl.): 93.
15) Cushing H. The life of Sir William Osler. Clarendon Press, Oxford.
1925.
16) Haas LF. Harvey Williams Cushing (1869-1939). Neurological Stamp.
J Neurol Neurosurg Psychiatry 2002; 73: 596.
17) Cushing H. The Puitary Body and its Disorders. (Clinical states
produced by disorders of the hypophysis cerebri). J B Lippincott Company,
Philadelphia. 1912.
18) Cushing HW. The basophil adenomas of the pituitary body and their
clinical manifestations (pituitary basophilism). Bull Johns Hopkin Hosp
1932; 3: 505-51.
19) Cushing H. The basophil adenomas of the pituitary body and their
clinical manifestations (pituitary basophilism). In : Papers Relating to the
Puitary Body, Hypothalamus and Parasympathetic Nervous System.
Charles C. Thomas. Springfield, Illinois. 1932.
20) Fulton JF.
Springfield. 1946.

Harvey Cushing, a biography.

Charles C. Thomas,

21) Nathanson M. Vignette. Harvey Cushing and the ANA. Ann Neurol
1991; 30: 221.
22) Pryse-Phillips W. Companion to Clinical Neurology. Little, Brown and
Company. Boston. pp. 228-9.
23) Harvey Cushing (1) .
2 37 (1-15 ) .. 2544 43.
24) Harvey Cushing (2) .
2 38 (16-31 ) .. 2544 36.

20

35 .

17

.. 2511 35

(
)
17
Phenobarbital

rock

.. 2512
1
temporal lobe epilepsy (TLE)
spike

35
70
diphenylhydantoin
carbamazepine clonazepam 2-3

.. 2545 33

TLE
mesial sclerosis
musicogenic epilepsy (ME)
ME
Merzheevsky
St. Petersburg .. 1884
Trutovsky 31
Oppenheimer 3 .. 1905
ME MacDonald Critchley
musicogenic epilepsy ( 1) Critchley
Queens Square
20
1900 consultant 28

3 99
ME Critchley 10
Critchley ME
2 Critchley 10
ME 1
ME reflex epilepsy

- (hot water epilepsy)
(reading epilepsy) reflex epilepsy ME
( 3)
Critchley ( 2)
ME .. 1931
Queen Square

(popular music) (
!) Critchley
Denny Brown ( Denny Brown
Putnam Professor of Neurology
) Denny Brown

Tchaikovsky Valse des Fleurs

Berlin State Opera Orchestra
Critchley plantar response

 2 flexor extensor response Babinski

sign
ME
62 Henry Miller
( 5)
(acoustical physicist)
frequency band
290-1120 cycles Henry Miller
45 ( .. 1962)
ME 2

(affective and
associative response)
subcortical level
Heschls convolution auditory associative cortex
Henry Miller
.. 1955-1970

MacDonald Critchley
research fellow .. 1963-1965

Henry
Miller Dr. John Walton Miller
Miller 62
Walton

Walton

Master Green College
Knight Commander of the British Empire (KBE) ( Sir)

Lord
Human Fertilisation and Embryo 12 (
6)
3
The Enigma of Music
Storr ( 7) Variations on the musical brain Warren
( 8) Schneider
Heschls gyrus
! ( 9)

1) Critchley M. Musicogenic epilepsy. Brain 1937; 60; 13-27.

2) Critchley M. Musicogenic epilepsy. (1) The beginnings. In: Music

and the brain. Studies in the neurology of music. Eds. Critchley M, Henson
RA. William Heineman Medical Books Ltd. London. 1977; pp. 344-53.
3) Scott D. Musicogenic epilepsy. (2) The later story : its relation to
auditory hallucinatory phenomena. Ibid 3. pp. 354-64.
4) Critchley M. The Ventricle of Memory. Derek Denny-Brown. Raven
Press, New York. 1990; pp. 55-62.
5) Poskanzer DC, Brown AE, Miller H. Musicogenic epilepsy caused only
by a discrete frequency ban of church bells. Brain 1962; 85: 77-92.
6) Human Fertilisation and Embryology Act 1990. Chapter 37. London :
HMSO. 1990; 39 pp.
7) Storr A. The enigma of music. J R Soc Med 1999; 92: 28-34.
8) Warren JD. Variations on the musical brain. J R Soc Med 1999; 92:
571-5.
9) Schneider P, Scherg M, Dosch HG, Specht HJ, Guttschalk A, Rupp A.
Morphology of Heschls gyrus reflects enhanced activation in the auditory
cortex. Nature Neurosci 2002; 5: 688-94.


Abd. R
alb
Alk Phos
AP
AJ
ANA
ANCA
ASO
BJ
BS
B.S.
C
CNS
CP
CPC
DF
DTR
EOM
ESR
ext.
FB
FBS
FF
Flex.
FSH
GH
glob
HF
HPF
JJ
JPS
JVP
KJ
L
LH
LMN
LR

=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=
=

abdominal reflex
albumin
alkaline phosphatase
antero-posterior
ankle jerk
anti-nuclear antibody
anti-neutrophil cytoplasmic antibody
antistreptolysin O
biceps jerk
blood sugar
breath sounds
cervical cord
central nervous system
close pressure
clinicopathologic conference
dorsiflexors of feet
deep tendon reflexes
external ocular muscle / movement
erythrocyte sedimentation rate
extensor (s)
fingers breadth
fasting blood sugar
finger flexion
flexor (s)
follicle stimulating hormone
growth hormone
globulin
hip flexor
high power field
jaw jerk
joint position sense
jugular venous pressure
knee jerk
left lumbar cord
luteinizing hormone
lower motor neuron
lateral rectus muscle

MCH
MCHC
MCV
mOsm
MR
NS
NSAID =
OP
Osm
P
PCR
PCV
PP
PT
PTT
Quad
R
RF
RUL
SIADH =
SLE
SGOT
SGPT
SJ
T
TP
TJ
TT
TVR
VA

=
mean corpuscular haemoglobin
=
mean corpuscular haemoglobin concentration
=
mean corpuscular volume
=
milliOsmole
=
medial rectus muscle
=
nervous system
non-steroidal inflammatory drug
=
opening pressure
=
osmolarity
=
plantar response
=
polymerase chain reaction
=
packed cell volume
=
pin prick
=
prothrombin time
=
partial thromboplastin time
=
quadriceps
=
right
=
rheumatoid factor
=
right upper lobe
syndrome of inappropriate secretion
of antidiuretic hormone
=
systemic lupus erythematosus
=
serum glutamic oxaloacetic transaminase
aspartate transaminase (AST)
=
serum glutamic pyruvic transaminase
alanine transaminase (ALT)
=
supinator jerk
=
temperature thoracic cord
=
total protein
=
triceps jerk
=
thrombin time
=
tactile vocal resonance
=
visual acuity

VS

vibration sense

 1 2
2
1 2

60

AMYOTROPHIC LATERAL SCLEROSIS MOTOR NEURON DISEASE

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CENTRAL DEAFNESS ( 9 1)
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CYTOMEGALOVIRUS INFECTION ( 14 2)
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MALARIA ( 17 2)
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5) , .

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26 2002 !
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THYROTOXIC PERIODIC PARALYSIS ( 20 1)
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VERTEBRAL ARTERY DISSECTION ( 3 1)

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27 .

3
6
6 .

3 2 3
1 2 2-3

10

.

3
3
24 200 ( 1)

1)
2)

3)

Wt. 33.2 kgs

Ht. 148 cms

T 37C
PR 120 regular
BP 100/60
RR 32
alert, well orientated
mild, pale, dry skin, thin hairs
hyperpigmented maculopapular rashes over trunk. Raw, beefy tongue.
Legs : pitting oedema 2+
Abdomen : scaphoid, no ascites
Liver 3FB below costal margin
spleen not palpable
increased bowel sounds
no lymphadenopathy
Other systems : normal
malabsorption syndrome
10 Niacin B12
12 folic acid

1)
immunocompromised host HIV
giardiasis
Cryptosporidiosis
HIV Strongyloidiasis
Capillaria philippinensis

x ray


Crohns disease ( 2 3) Whipples
disease intestinal lipodystrophy Tropheryma whippelii
gram-positive actinomycete George Whipple
Johns Hopkins .. 1907 ( 4)
50



2) 1 Crohns disease
ulcerative colitis microscopic colitis
2 collagenous lymphocytic colitis
10-20 90
56 ( 5)
3) Coeliac disease
HLA DQ2 DQ8 gluten

 barley rye
( 6)
4) autoimmune connective tissue disease SLE

protein-losing enteropathy
1 ( 7)
5) intestinal lymphoma alpha
chain disease (CD) Mediterranean lymphoma
immunoproliferative small intestinal disease (IPSID)
lymphoma ( 8)
6) chronic calcific
pancreatitis

Hb 8.0 PCV 23.6

MCV 82
MCH 24.6 MCHC 31.3
RBC
6
2.88x10
HbA 83.4
HbE 16.6
WBC 20200 N 93 E 2 M 4 B1
Platelets
Urine : protein trace, WBC 10/HPF
glucose -ve
TP 66.5/alb 23.5
TB 1.5/DB 0.5
Chol 139
TG 209
SGOT 103
PT 50
GGT 470
Alk Phos 497
FBG 171 BUN 29
Cr 5.7
+
+
K 2.9 Cl- 117
Na 124
CO2 19.5 Mg++ 1.4
Serum Fe 23.6 TIBC 34.7 = 67%
Chest x ray
Stool Exam : yellow-greenish, soft
numerous fat globules (Sudan black stain)
occult blood negative
adult form of Capillaria identified, no eggs
Rectal swab culture grew E. coli, enterococci, coagulase negative staph. and
yeasts


Capillaria philippinensis (nematode)
The Capillariids 16 genera 3 Aonchotheca
Capillaria C philippinensis - ..
1962 1400
.. 1967-1970 95

.. 1973
( 9)
( 10 14) 20-50


intermediate host
definitive host
John Cross
10 ( 15 16)

(
17)

mebendazole albendazole 10-20

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1999; pp. 293-5.

2

65 .

4

4


2 (Bipolar
disorder) 4

toxic/metabolic
encephalopathy (encephalitis)


lithium carbonate

Haloperidol 5 2 Benzhexol 2 mg 2
Lithium carbonate 300 mg - Lorazepam
1 mg

T 37C
PR 78 regular
BP 120/80
drowsy, no neck stiffness
Fundi: normal
Pupils: equal, 2 mm. in diameter and reacted to light
Limbs: increased tone in all
ankle clonus + R>L
DTRs symmetrical 3+
P
()
toxic/metabolic encephalopathy
Lithium electrolytes, BUN, Cr, SGOT, PT

Hb 10.7
Hct. 31.6
Platelets 270,000
MCV 86 MCHC 33.9
WBC 15,700
N 89 L 5 M 6

 Lithium 3.92
TOXICO Scan REMED1 benzquinamide metabolite

BUN 44
Cr 2.0
FBS 108
UricAcid 10.9
+
Cl 108
Na 138
K 3.99
2+
Ca 8.7 Inorg. Phos. 2.4 Mg2+ 2.3 mg/dl
SGOT 24 PT 26
TP 82.3/alb 36.5
Arterial Blood gas, Urine, CSF and CT brain: no abnormality
lithium intoxication
IV drip 4 6
lithium 2.92 1.84 1.23 0.75 mmol/L

Lithium carbonate .. 1948

lithium
( 1)
lithium
therapeutic range ( tremor
asterixis) myoclonus ( 2) ataxia
downbeat nystagmus periodic alternating nystagmus ( 3 4)

cerebellar dysfunction ( 5) Li+ therapeutic index (2-3)
NSAIDs, ACE inhibitors
Li+ Li+
1 mEq.
Li+ 2
3
haemodialysis (
6 7) Lithium
ion channels channelopathy

lithium (Li+) (element) Periodic

Li+ atomic
Table IA Na+ K+ Rb (Rhubidium) Cs (Cesium)
weight 7 Na+ 23 K+ 39 Ca2+ Mg2+
IIA ( 8)
Na+ Li+ Li+
Na+ channel
2 Lithium Cluster headache
lithium
amyloid-peptide
( 9) lithium

 Nature : science update. online

( 17)
2 3
( 18)
peripheral leukocytosis
lithium monocytes colony stimulating factor
Lithium
( 10)
autoimmune Lithium antibody
( 11, 12 13)
thyroid function thyroid autoantibodies
lithium
14
lithium lithium
Creutzfeldt-Jakob ( 15 16)
!

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Churchill Livingstone, New York. 2001 pp. 593-615.
15) Smith SJM, Kocen RS. A Creutzfeldt-Jakob like syndrome due to lithium
toxicity. J Neurol Neurosurg Psychiatry 1988; 51: 120-3.
16) Kikyo H, Furukawa T. Creutzfeldt-Jakob-like syndrome induced by lithium,
levomepromazine, and phenobarbitone. J Neurol Neurosurg Psychiatry
1999; 66: 802-3.
17) Pilcher HR. The ups and downs of lithium. Nature Science update.
http://www.nature.com. 22 September 2003.
18) Belmaker RH. Bipolar disorder. N Engl J Med 2004; 351: 476-86.

17 .

10 .

.
1 3

23

Effort syncope


epileptic seizure
left ventricular outflow tract
obstruction hypertrophic
obstructive cardiomyopathy (HOCM) myxoma left atrium
23
HOCM

Body wt. 75 kgs

alert, cooperative
PR 80 regular
BP 110/60
L arm
Radial pulses
+
+
Dorsalis pedis
+
+
th
heaving apex beat, 5 intercostal space outside mid-clavicular line

- no

thrill
harsh grade 3/6 diamond-shaped ejection systolic murmur at L sternal
border and apex radiating to aortic and pulmonary areas and to axilla
()
murmur
thrill

 HOCM 1 2 4 3
systolic murmur murmur
hallmark HOCM harsh crescendo-diminundo (decrescendo) diamondshaped left sternal border apex HOCM
mitral regurgitation murmur apex holo-
pansystolic murmur jugular venous pulse
( 1) HOCM
ventricular septum right ventricular compliance
a wave
HOCM
echocardiogram
left
ventricular systolic function mild mitral regurgitation right ventricular hypertrophy
borderline left atrial enlargement atrioventricular
trileaflet
beta-blocker (propranolol) valsartan-K (Cozaar)
pace-maker

Hypertrophic cardiomyopathy
haemodynamic stress amyloid glycogen
myocyte disarray (
2)
HOCM mutations sarcomeric protein genes
1 500
40 Guys
Russell Brock Lord Brock (
... )
aortic subvalvar stenosis ( 3) Brock Chevers
Guys Hospital Report .. 1842 ( 4)
Lord Brock ( 5) Donald Teare
( 6) (
7 8) Engene Braunwald
( 9)
echocardiography
MRI physiological hypertrophy dilatation
cardiomyopathies MRI HOCM
( 10)
septal
myomectomy alcohol-induced septal ablation
2 11
!


1) . :
3. .. 2546 21-30.
2) Elliott P, McKenna WJ. Hypertrophic cardiomyopathy. Lancet 2004; 363:
1881-91.
3) Brock R, Fleming PR. Aortic subvalvar stenosis. A report of 5 cases diagnosed
during life. Guys Hosp Rep 1956; 105: 391-408.
4) Chevers N. Observations on the diseases of the orifice and valves of the aorta.
Guys Hosp Rep 1842; 7: 387-442.
5) Brock R. Functional obstruction of the left ventricle. Guys Hosp Rep 1957;
106: 221-38.
6) Teare D. Asymmetrical hypertrophy of the heart in young adults. Br Heart J
1958; 20: 1-8.
7) Goodwin JF, Hollman A, Cleland WP, Teare D. Obstructive cardiomyopathy
simulating aortic stenosis. Br Heart J 1960; 22: 403-14.
8) Frank S, Braunwald E. Idiopathic hypertrophic subaortic stenosis: clinical
analysis of 126 patients with emphasis on the natural history. Circulation 1968; 37:
759-88.
9) Wynne J, Braunwald E. The cardiomyopathies and myocarditides: Hypertrophic
cardiomyopathy. In: Heart Disease. A Textbook of Cardiovascular Medicine.
Second Edition. Ed. Braunwald E. WB Saunders Co. Philadelphia 1984;
pp. 1409-21.
10) Constantine G, Shan K, Flamm SD, Sivananthan MU. Role of MRI in clinical
cardiology. Lancet 2004; 363: 2162-71.
11) Nishimura RA, Holmes DR. Hypertrophic obstructive cardiomyopathy. N Engl J
Med 2004; 350: 1320-7.

4

26 .

4
6 . 7

methimazole

ophthalmopathy 2

myasthenia gravis (MG) MG
(ptosis)

Wt. 57 kgs Ht. 168 cms

PR 78
BP 120/80
slight proptosis of eye
1 ptosis L
EOMs : slight L LR and SR paresis
orb oc. 4/5
4/5
sternomastoids and limb muscles : normal power
DTRs : present

()
ptosis proptosis weakness
external ocular muscles orbicularis oculi MG

Tensilon test acetyl choline receptor (AchR)

antibody MRI chest

Tensilon test +ve

serum AChR antibody 0.18 nmol/L (normal < 0.25, equivocal = 0.25-0.40,
reactive = > 0.40)
PA chest xray : normal
MRI chest : small anterior mediastinal soft tissue mass, size 2 x 4 x 6.5
cms.
compatible with thymic hyperplasia
serum CPK 21 (normal 20-192)
Free T4 2.95 ng/dl (normal 0.93-1.7)
T3 166.9 ng/dl (normal 84-201.8)
TSH < 0.005 uIU/ml
3
serum T4 236 (normal 55-160 nMol/L)
T3 1.9 (1.2-3.3)
FTI 244 (50-180 nMol/L)

Edrophonium Tensilon test MG

AchR antibody 87 MRI chest thymic hyperplasia
MG
prevalence 25-150 1 ( 1 3)
(HT) 1.9 1.6
( 4) HT
1 MG MG 3 HT
MG .. 1969 1990
291 51 HT 17.5
6 ( 5) MG & HT
34 2 13 prednisolone
(HDP) 100-120 11 HDP /
+ 10
MG anticholinesterase pyridostigmine Mestinon
34 10 (Ty) HDP
MG HT Ty MG
HDP MG Ty HT
Prednisolone 15
30, 40, 50 60
50 4 60
4
6
18 10-15 HT
18 serum T4 T3 TSH
!


1) Osserman KE, Genkins G. Studies in myasthenia gravis: review of a twenty-year
experience in over 1200 patients. Mt Sinai J Med 1971; 38: 497-537.
2) Grob D. Natural history of myasthenic gravis. In: Myasthenia Gravis and
Myasthenic Disorders. Ed. Engel AG. Oxford University Press. Oxford. 1999; pp.
131-45.
3) Robertson NP, Deans J, Compston DAS. Myasthenia gravis: a population based
epidemiological study in Cambridgeshire, England. J Neurol Neurosurg Psychiatry
1998; 65: 492-6.
4) Tunbridge WM, Evered DC, Hall R, Appleton D, Brewis M, Clark F, et al.
The spectrum of thyroid diseases in a community: the Whickham survey.
Clin Endocrinol (Oxf). 1977; 7: 481-93.
5) Ratanakorn D, Vejjajiva A. Long-term follow-up of myasthenia gravis patients
with hyperthyroidism. Acta Neurol Scand 2002; 106: 93-8.

5

54 .

1 .
4-5 . 2
.

1

connective tissue disease

renal carcinoma

T 38C alert, slightly pale, no jaundice

PR 90
BP 110/70
Heart sounds normal, no murmur
Neck : no enlarged lymph node, biopsy scar over side
Abdomen : liver enlarged, soft, 4FB below costal margin
spleen not palpable
CNS : no neck stiffness
no leg weakness

RR 20

DTRs ++ symmetrical
P
tender paravertebral muscles
()

 lymphoma
.

osteomyelitis spinal epidural abscess staphylococus aureus

epidural abscess



brucellosis
antibody
MRI

Hb 9.8
Hct 31.9
WBC 4590 N 73 L 22 M 4 B 1
6
microc. 1+
RBC 4.6 x 10
aniso 1+
poikilo 1+
hypochromia 1+
Platelets 197,000
ESR 64
HbA 95.9 A2 4.1
LFT : alb 35/glob 48 g/L TB 0.8
DB 0.2
SGOT 154
PT 204
AP 254
anti HIV ve
HBs Ag ve
HB Abs +
Ultrasound : gall bladder, no abnormality
MRI thoracic and lumbar spine : normal
MDCT scan of abdomen : mild hepato splenomegaly without focal mass,
no para-aortic lymphadenopathy
lymph node biopsy . reactive hyperplasia
Bone marrow biopsy : hypercellular, increased erythropoiesis and
myeloid hyperplasia
Liver biopsy : non-specific reactive hepatitis and scattered microgranuloma
AFB stain : negative
Gram stain : gram negative coccobacilli present
Blood culture grew Brucella melitensis sensitive to beta-lactams, quinolone,
aminoglycoside SMX/TMP
brucella agglutinins +ve titre > 1 : 32

B.
melitensis gentamicin IV drip, doxycycline ciproflocaxin

 3
6
10 Hb 13.1 Hct 41.8 ESR 7 LFT
B. melitensis
pasteurized B. abortus
( 1)
brucella
proton pump inbitor GERD gastroesophageal reflux disease

brucellosis 1
( 2)

brucella agglutination (Brucella suis) ( 3 4)
Brucellosis .. 1860 Mediterranean gastric
remittent fever Malta fever
undulant fever undulant

1-2 39.5 - 40C
37C
7
Brucellosis ..
1887 David Bruce
sleeping sickness Trypanosoma brucei
Bang
Malta fever Mary Elizabeth
Steel Bruce Alice Evans
Micrococcus melitensis Bangs bacillus species genus
Dr. Alice Evans
pasteurization ( 5)

(
6 7)

Brucellosis
2 brucella
mycobacterium tuberculosis reticuloendothelial system Mycobacterium
granuloma brucella microgrnauloma


1) Williams E. Brucellosis in veterinary surgeons in Wales. J R Coll Phys Lond
1976; 10: 351-62.
2) Visudhiphan S, Na-Nakorn S. Brucellosis. First case report in Thailand.
J Med Assoc Thai 1970; 53: 289-93.
3) Vejjajiva S, Yenbutr D, Pranich K, Piptikul S. Brucella agglutinins in the Thais.
J Med Assoc Thai 1970; 53: 407-10.
4) Subharngkasen S. Brucellosis in Thailand. Bull Off Int Epizoot 1970; 73: 9-15.
5) Shakir RA. Brucellosis. In: Tropical Neurology. Eds. Shakir RA, Newman PK,
Poser CM. WB Saunders Co. Ltd. London 1996; pp. 167-81.
6) Lulu AR, Araj GF, Khateeb MI, Mustafa MY, Yusuf AR, Fenech FF. Human
brucellosis: a progressive study of 400 cases. Q J Med 1988; 66: 39-54.
7) Madkour MM. Brucellosis. In: Oxford Textbook of Medicine. Third Edition
Vol. 1. Eds. Weatherall DJ, Ledingham JGG, Warrell DA. Oxford Medical Publications.
Oxford University Press. Oxford 1996; pp. 619-23.

6

42

2 3
2 .
3 2
7


10-15 2 2
3

6 . HIV

C fibres
lateral spinothalamic tract
A-fibres
cauda equina L5 S 1, 2, 3
4 2
S 2, 3 4
conus medullaris
reflexes

T 37.4C
Wt. 80 kgs
Ht. 185 cms
PR 78 regular BP 120/70
CNS
Cranial nerves intact
Upper and lower limbs : no wasting or weakness
Tone and Coordination
SJ
++
++
BJ
++
++
TJ
++
++

KJ
++
++
AJ
P

impaired pin prick sensation in the back of both legs and in both feet
and buttock
anal sphineter tone : loose
no other abnormality
()
cauda equina

MRI thoraco-lumbar spine CBC, FTA-Abs, HIV

FTA-Abs : non-reactive
HIV -ve
Chest x ray
MRI spine suspected lesion at conus medullaris and
bony involvement
? metastasis

. 37.5C 7 .
hypalgesia L C 6-7 dermatomes
1.5 .
granulomatous lymphadenitis isoniazid, pyrazinamide, ethambutol
streptomycin 5
6
streptomycin 6 1
5 2
AJ + 2

lumbar radiculomyelitis TB

40
Wadia
Dastur 2 MRI

 2 ( 1 2)
intra-dural spinal tuberculomas ( 3 4)
. TB
granulomas conus medullaris
MRI
( 5 6)

isoniazid pyrazinamide 2 9
1 ( 7) isoniazid
rifampin 2
( 8)
CMV
HIV ( 9) HIV

Enterovirus 70
( 10)



( 11 12)

1) Wadia NH, Dastur DK. Spinal meningitides with radiculo-myelopathy, Part 1:

Clinical and radiological features. J Neurol Sci 1969; 8: 239-60.
2) Dastur DK, Wadia NH. Spinal meningitides with radiculomyelopathy, Part 2:
Pathology and pathogenesis. J Neurol Sci 1969; 8: 261-97.
3) Wadia NH. Radiculomyelopathy associated with spinal meningitides
(arachnoiditis) with special reference to the spinal tuberculous variety. In: Tropical
Neurology.Ed. Spillane JD. Oxford University Press. London. 1973; pp. 63-72.
4) Parsons M, Pallis CA. Intradural spinal tuberculomas. Neurology 1965; 15:
1018-22.
5) Tandon PN, Pathak SN. Tuberculosis of the central nervous system. In:
Tropical Neurology. Ed. Spillance JD. Oxford University Press. London. 1973; pp.
37-62.

6) Tandon PN. Tuberculous meningitis (cranial and spinal). In: Handbook of

Clinical Neurology. Vol. 33. Infections of the Nervous System Part I. Eds.
Vinken PJ, Bruyn GW. North-Holland Publishing Co. Amsterdam. 1978; pp. 195-262.
7) Phuapradit P, Vejjajiva A. Treatment of tuberculous meningitis: Role of shortcourse chemotherapy. Q J Med 1987; 62: 249-58.
8) Espinal MA, Laszlo A, Simonsen L, Boulahbal F, Kim SJ, Reniero A, et al, for the
World Health Organization International Union against Tuberculosis and Lung
Disease
Working Group on Anti-Tuberculosis Drug Resistance Surveillance. Global trends
in resistance to antituberculous drugs. N Engl J Med 2001; 344: 1294-303.
9) . .
2 . ..
2545 141-7.
10) Vejjajiva A. Acute hemorrhagic conjunctivitis with nervous system
complications. In: Handbook of Clinical Neurology. Vol. 56. Viral Disease. Eds.
Vinken PJ,
Bruyn GW, Klawans HL. Elsevier Science. New York. 1989; pp. 349-54.
11) N Engl J Med Case Records of the Massachusetts General Hospital. Case 82004: A 28-year old man with abdominal pain, fever, and a mass in the region of
the pancreas. 2004; 350: 1131-8.
12) Jha AK, Shojania KG, Saint S. Clinical Problem Solving. Forgotten but not
gone. N Engl J Med 2004; 350: 2399-404.

7
25
25 .

2
.

2-3
7
4
3-4

ricketsia leptospira
ricketsia . leptospirosis

leptospirosis 2

T 38.5C
PR 110
RR 20
BP 100/60
alert, jaundiced
Throat : not injected, a few white patches seen over both tonsils
slightly enlarged lymph nodes in posterior triangles of neck
Liver and spleen : not palpable
()

infectious mononucleosis (IM)

cytomegalovirus (CMV) scrub typhus

toxoplasmosis eschar scrub typhus
blood smear atypical lymphocytes
IM


Hb 13
Hct 38
3 Hb 9.6
Hct 27.8
WBC 10500 N 32 L 54 (atypical 14) M 7 E 2
Platelets 95,000
T.B. 18.8
DB 11.7
FBS 77
Cr 0.6
SGOT 165
PT 203
TP 70.4/alb 39.8
Direct Coombs test +ve
Indirect ve
Urine : protein 1+ WBC 3-5/HPF
serum IgM antibodies to EB viral capsid antigen (VCA) +ve

infectious mononucleosis (IM) .. 1889

Pfeiffer glandular fever (
1) Pfeiffer atypical lymphocytes
Burns .. 1904 Sprunt Evans .. 1920
( 1 2)
Sir
Anthony Epstein Burkitt lymphoma .. 1964
4 Henle ( 3) Epstein-Barr virus (EBV)
infectious mononucleosis (Barr Yvone Barr, PhD.
Epstein 4) EBV herpes
lymphoma ( 5, 6, 7
8)
IM
kissing disease
3
membrane anginose type Vincent angina
Borrelia vincenti Fusobacterium (Diphtheria)
febrile typhoid type
25 50 (periorbital
oedema)

haemolytic anaemia EBV 4-8
ampicillin
-haemolytic streptococcus macrolide ( 9 10)
blood smear atypical
lymphocytes Wright stain
cytoplasm
vacuoles cytoplasm
vacuoles (foamy) ( 1)

 IgM IgG antibodies EBV capsid antigen (VCA)

heterophile antibody IgM
Paul-Bunnell test titre 1 : 224 Monospot test
JR Paul WW Bunnell
( 9) heterophile antibody
VCA EBV 5
(
11 12) EBV 1 200

plasmapheresis ( 13)

blood smear
steroid

1) . Infectious mononucleosis. :
.

2513 791-7
2) Sprunt TP, Evans FA. Mononuclear leucocytosis in reaction to acute infections
(infectious mononucleosis). Bull Johns Hopkins Hosp 1920; 31: 410-7.
3) Henle W, Henle G. Epstein-Barr virus and infectious mononucleosis. In: Human
and herpes virus infections: clinical aspects. Eds. Glaser R, Gottlieb-Stematsky T.
Marcel Dekker Inc. New York. 1982; pp. 151-67.
4) Firkin BG, Whitworth JA. Dictionary of Medical Eponyms. The Parthenon
Publishing Group. Carnforth, Lancs, UK. 1987; pp. 147-8.
5) Epstein MA, Achomg BG, Barr YM. Virus particles in cultured lymphoblasts from
Burkitts lymphoma. Lancet 1964; 1: 702-3.
6) Epstein MA, Crawford DH. The Epstein-Barr virus. In: Oxford Textbook of
Medicine. Third Edition. Vol. 1. Eds. Weatherall DJ, Ledingham JGG, Warrell DA.
Oxford University Press. Oxford. 1996; pp. 352-7.

7) Thorley-Lawson DA, Gross A. Persistence of the Epstein-Barr virus and the

origins of associated lymphomas. N Engl J Med 2004; 350: 1328-37.
8) Rossi G, Bonetti F. Letter to editor. EBV and Burkitts lymphoma. N Engl J
Med 2004; 350: 2621.
9) Juel-Jensen BE. Infectious mononucleosis: Epstein-Barr virus disease.
In: Oxford Textbook of Medicine. Second Edition. Vol. 1: Eds. Weatherall DJ,
Ledingham JGG, Warrell DA. Oxford University Press. Oxford. 1987; pp. 5.72-5.75
10) White NJ, Juel-Jensen BE. Infectious mononucleosis hepatitis. Seminars in Liver
Disease 1984; 4: 301-6.
11) Gautier-Smith PC. Neurological complications of glandular fever. Brain 1965;
88: 323-34.
12) Ross JP, Cohen JI. Epstein Barr virus. In: Infections of the Central Nervous
System. Second Edition. Eds. Scheld WM, Whitley RJ, Durack DT. Lippincott-Raven.
Philadelphia. 1997; pp. 117-27.
13) Schmahmann JD. Plasmapheresis improves outcome in postinfectious cerebellitis
induced by Ebstein-Barr virus. Neurology 2004; 62: 1443.

8

26

3
1 . 1
7 3-4

2
3 . 2

2
6 pons 3
6 medial longitudinal fasciculus (MLF) pons
2 pons
optic nerve

brain stem

alert, normal speech

Fundi : normal
VAR 20/40
no ptosis
100%
100%

VAL 20/40

90%

100%

100%

90%

100%

100%

spontaneous nystagmus on lateral gazes

Cranial v n. vertical nystagmus present
Limbs : Tone
Power
Coordination : Finger-nose and Heel-shin tests impaired on
SJ
+++ +++
BJ +++ +++
TJ
+++ +++
KJ
+++ +++
AJ
++
++
P

Other systems : normal

()
pons
multiple sclerosis (MS)
arterio-venous malformation (AVM) pontine infarct

5 pontine infarct MRI

MRI
ill-defined hyposignal T/hypersignal T2 lesion at lower pons, whole medulla
more prominent on the left, cervico-medullary junction and upper cervical cord.
better defined more hypersignal T2 lesion scattering in the central portion of
the lesion involving the floor of 4th ventricle and posterior medulla without enhancement.

demyelinating lesion pons, medulla

acute brain stem MS

 hiccup ( hiccough
1) (diaphragm)
(inspiratory intercostal muscles) glottis
30 milli-seconds
15-30
PaCO2 PaCO2
amplitude ( 2)
medulla nucleus tractus solitarius


Plato

416
pharynx

baclofen 10 mg

1987 Birkhead Friedman

MS ( 3) (
4) MS 1
carbamazepine Funakawa
MS sleep apnoea ( 5) Chang
MRI
plaque medulla .. 1994 (
6)



MS 4 ( 7)
MS medulla
pulmonary oedema ( 8 13)
nucleus tractus solitarius dorsal motor nucleus vagus
medial reticular formation
hydrostatic pressure
MS .. 1970
MS ( 14)

( 15 18)
40
( 19) !

1) Pearce JMS. A note on hiccups. J Neurol Neurosurg Psychiatry 2003; 74:

1070.
2) Newsom-Davis J. An experimental study of hiccup. Brain 1970; 93: 851-72.

3) Birkhead R, Friedman JH. Hiccups and vomiting as initial manifestations of

multiple
sclerosis. J Neurol Neurosurg Psychiatry 1987; 50: 232-3.
4) McFarling DA, Susac JO. Hoquet diabolique: intractable hiccups as a
manifestation
of multiple sclerosis. Neurology 1979; 26: 797-801.
5) Funakawa I, Hara K, Yasuda T, Terao A. Intractable hiccups and sleep apnoea
syndrome
in multiple sclerosis: report of two cases. Acta Neurol Scand 1993; 88: 401-5.
6) Chang Y-Y, Wu H-S, Tsai T-C, Ling J-S. Intractable hiccup due to multiple
sclerosis:
MR imaging of medullary plaque. Canad J Neurol Sci 1994; 21: 271-2.
7) Witoonpanich R, Pirommai B, Tunlayadechanont S. Hiccups and multiple
sclerosis.
J Med Assoc Thai 2004; 84: 1168-71.
8) Simon RP, Gean-Marton AD, Sander JE. Medullary lesion inducing pulmonary
oedema: a magnetic resonance imaging study. Ann Neurol 1991; 30: 727-30.
9) Gentiloni N, Schiavino D, Della CF, Ricci E, Colosimo C. Neurogenic pulmonary
oedema: a presenting symptom in multiple sclerosis. Ital J Neurol Sci 1992;
13: 435-8.
10) Simon RP. Neurogenic pulmonary oedema. Neurol Clin 1993; 11: 309-23.
11) Crawley F, Saddeh I, Barker S, Katifi H. Acute pulmonary oedema: presenting
symptom of multiple sclerosis. Mult Scler 2001; 7: 71-2.
12) Summerfield R, Tubridy N, Sirker A, Banner NR, Peatfield RC. Pulmonary oedema
with multiple sclerosis. J R Soc Med 2002; 95: 401-2.
13) van de Beek MT, Taal W, Veldkamp RF, Vecht CJ. A woman with multiple
sclerosis and pink saliva. Lancet Neurol 2003; 2: 254-5.
14) Vejjajiva A. Demyelinating diseases in Thailand. Clin Neurol (Japan) 1970; 10:
36-40.

15) Vejjajiva A. Some clinical aspects of multiple sclerosis in Thai patients. In:
Multiple Sclerosis East and West. Eds. Kuroiwa Y, Kurland LT. Kyushu University
Press. Fukuoka. 1982; pp. 117-21.
16) Vejjajiva A. Multiple sclerosis in Thailand. Neurol J Southeast Asia 1997;2: 7-10.
17) Kira J. Multiple sclerosis in the Japanese population. Lancet Neurol 2003; 2:
117-27.
18) Ebers G. Multiple sclerosis. In: Horizons in Medicine 15. Updates on major
clinical advances. Ed. Weber J. Royal College of Physicians of London. 2003; pp.
65-72.
19) , .
2547; 29: 282.

9

54 . .

1
7 .
2-3 2

.
. 3

subarachnoid haemorrhage

sphenoid sinus sella turcica

3
.
.
T 38C
PR 78
BP 90/60 slightly drowsy, bilateral ptosis L > R
neck stiffness present
. 3
T 35.5C
PR 66
BP 100/60
RR 20
alert but slow response
no neck stiffness
marked bilateral ptosis
Fundi : pale optic discs
VAR 20/400
VAL 20/400
Visual fields full to confromtation
Eye movts impaired as shown
R

L
0

100%

50%

0
40%
80%

0
0

Pupils 3 mm. both eyes

sluggishly reacted to light
no other abnormality

(1)
optic nerve optic chiasma
(2) 3, 4 2
6 (3) .
3 (35.5C)
pituitary apoplexy
sphenoid sinus
CT scan MRI electrolytes
cortisol thyroid sex hormones
.
K+ 4.1
serum Na+ 120
BUN 11
Cr 1.1
CT : intrasellar mass

Cl- 86

HCO3- 21

.
Hb 13.1
Hct 38.7
RBC 4.96 x 106
MCV 78.1
MCHC 33.8
WBC 3810 N 73 L 23 M4
ESR 23
serum osmolarity 273/urine osm. 271
serum cortisol 0.63 g/dl (9.00 AM)
T4 1.16 g/dl
T3 40 ng/dl
TSH 0.05 g/dl
LH 0.1
FSH 2.0
Prolactin 4.1
()

Na+ adrenal insufficiency

pituitary apoplexy pituitary hormones

 cortisol optic
chiasmal compression
Pituitary apoplexy 5 20
(adenoma) 3
pituitary apoplexy ( 1)
3 ( !)
1)
2) visual loss (
)
3) ACTH
cortisol 2 3

( 2 3)
MRI CT
cortisol IV
fluid electrolytes diabetes insipidus SIADH
optic chiasmal compression

3, 4 6 ( 2, 4
5)
MRI 6
Pituitary apoplexy

1) Randeva HS, Schoebel J, Byrne J, Esiri M, Adams CB, Wass JA. Classical
pituitary apoplexy: clinical features, management and outcome. Clin Endocrinol
(Oxf) 1999; 51: 181-8.
2) Ma RCW, Tsang MW, Ozaki R, Tong PC, Cockram CS. Fever, headache, and
a stiff neck. Lancet 2004; 363: 1868.
3) Brouns R, Crols R, Engelborghs S, De Deyn PP. Pituitary apoplexy presenting as
chemical meningitis. Lancet 2004; 364: 502.
4) Bonicki W, Kasperlik-Zaluska A, Koszewski W, Zgliczynski W, Wislawski J.
Pituitary apoplexy; endocrine, surgical and oncological emergency. Incidence,
clinical course and treatment with reference to 799 cases of pituitary adenomas.
Acta Neurochir 1993; 120: 118-22.

5) Tang-Wai DF, Wijdicks EFM. Pituitary apoplexy presenting as post-operative

stupor. Neurology 2002; 58: 500-1.
6) Glass LC. Images in clinical medicine. Pituitary apoplexy N Engl J Med
2003; 349: 2034.

10

34 .

6 .
6 .
nifedipine
6 . 2

.
5-6

2 Cushings syndrome
primary aldosteronism Conns syndrome
Cushings syndrome
myopathy primary aldosteronism
( 1)
Cushings syndrome
striae primary hyperaldosteronism Conns
syndrome

Wt. 91 kgs.
Ht. 171 cms
General appearance : normal
PR 84 regular
BP 140/110 mmHg arm
Heart sounds normal, no murmur
Peripheral pulses were normal
Chest : slight generalized wheezing bilaterally
Abdomen : no mass felt
no striae
Legs : no oedema
Limb muscles : no wasting
Power
deltoids
4/5
4/5
hip flexors
4/5
4/5
others were normal
DTRs
+
+

 ()
Conns syndrome
serum K+ CT scan

CBC, BUN, Cr normal

K+ 2.83
Cl- 100.4
CO2 30
Serum Na+ 151
+
+
Spot Urine Na 34.2
K 62.7
Cl 32.2
MDCT scan : upper abdomen : mass at lateral limb of L adrenal gland

(left adrenalectomy)
8 x 3 x 1.5
18.5
section adrenal cortical adenoma

Primary aldosteronism Jerome Conn

.. 1955 ( 2) Conns
syndrome 1

10
renin angiotension primary aldosteronism 12 (
3)
adrenal hyperplasia 2
aldosterone
gene aldosterone synthase CYP 11B2
adrenal 11-beta hydroxylase meiosis
aldosterone aldosterone ACTH
dexamethasone
Liddles syndrome mineralocorticoid-type hypertension
( 4)
Conns syndrome

( 5 6)
1 145 Conn ( 7)
2.5
2 metastases
( 8)
Conns syndrome (
9, 10 11) adrenal scan 12

. CT scan 20 ( 13)

aldosterone Sylvia Tait

28 .. 2003 86 2
50
aldosterone Sylvia Tait 2
aldosterone .. 1952
Courtauld Institute of Biochemistry Middlesex
14
!

1) Huang YY, Hsu BR, Tsai JS. Paralytic myopathy: a leading clinical presentation
for primary aldosteronism in Taiwan. J Clin Endocrinol Metab 1997; 82: 2377-8.
2) Conn JW. Presidential address. II. Primary aldosteronism, a new clinical
syndrome. J Lab Clin Med 1955; 45: 3-17.
3) Gordon RD, Zlesak MD, Tunny TT, Stowasser M, Klemm SA. Evidence that
primary aldosteronism may not be uncommon: 12% incidence among
antihypertensive
drug trial volunteers. Clin Exp Pharmacol Physiol 1993; 20: 296-8.
4) Cleland SJ, Connell JMC. Endocrine hypertension. JR Coll Physicians Lond
1998; 32: 104-8.
5) Sawka AM, Young WF, Thompson GB, Grant CS, Farley DR, Leibson C, et al.
Primary aldosteronism: factors associated with normalization of blood pressure
after surgery. Ann Intern Med 2001; 135: 258-61.
6) Martinez D. Adrenalectomy for primary aldosteronism. Ann Intern Med 2003;
138: 157-9.
7) Conn JW, Knopf RF, Nesbit RM. Clinical characteristics of primary
aldosteronism from an analysis of 145 cases. Am J Surg 1964; 107: 159-72.
8) Rossi GP, Vendraminelli R, Cesari M, Pessina AC. A thoracic mass with
hypertension and hypokalaemia. Lancet 2000; 356: 1570.
9) Ganguly A. Primary aldosteronism. N Engl J Med 1998; 339: 1828-34.

10) Stewart PM. Mineralocorticoid hypertension. Lancet 1999; 353: 1341-47.

11) Velakoulis D, Lubman DI, Brett A, Russell D. Conns dementia. Lancet Neurol
2002; 1: 131.
12) Ansari A. Clinical Picture. Primary aldosteronism. Lancet 2002; 360: 617.
13) Tiyapant A, Ua-Apisithwongse S. Primary aldosteronism, adrenal adenoma
demonstrated by CT scan: a case report. Intern Med Thai 1985; 1: 144-7.
14) Lancet Obituary. Sylvia Tait. Biologist who discovered aldosterone. 2003;
361: 1571.

11

32 .

1
6
1 .

2
.
2

1


1) voluntary
involuntary movement
2)

chorea myoclonus athetosis

3)

Eyes : no K-F ring, EOMs full

alert, cooperative
jerky, non-repetitive movements of fingers of both hands and right foot
slightly increased tone in both arms and moderate spasticity in both legs.
F-N test slow but no incoordination
H-S could not be performed
DTRs

+++
P

+++

Tandem walk impaired

 ()

basal ganglia

3
1. tremor
2.

3.
3

chorea myoclonus
chorea
coordination
action myoclonus
Chorea
caudate nucleus
putamen basal ganglia

1
chorea
1
2
rheumatic Sydenhams chorea
beta haemolytic streptococcus
Chorea rheumatic fever
chorea SLE coeliac disease
paraneoplastic syndrome
(chorea gravidarum) ( 1 8)
chorea
Huntington disease (HD) Wilson disease chorea
K-F ring benign
hereditary chorea HD ( 9
10) chorea-acanthocytosis neuro acanthocytosis
autosomal recessive HD
acanthocyte (
11 14)

40

=
=

40

40+

FBS 103 mg%

serum ceruloplasmin 30.2 mg/dl
urinary copper 1.74 g/L (24 hours urine 1.2 L)
MRI : moderate brain atrophy

()
autosomal dominant Huntington
disease (HD) DNA
HD .. 1872 George
Huntington East Hampton, Long Island

George Huntington (GH) 3
Long Island GH
HD
autosomal dominant
HD

 New England East Anglia

( 15 16)


( 17)
HD
chromosome
4p16.3 (IT 15, IT Interesting Transcript) CAG
40-50 36 CAG
polyglutamine huntingtin
(mutation)
Huntingtons chorea Huntington
disease

chorea

IT 15
! ( 18)

motor
neurons
transport system dynein-dynactin complex
( 19)
(apoptosis programmed cell death) necrosis
2 HD caudate nucleus, putamen
cortex caspases
caspase 1, 3, 8, 9
genes caspase 1,
3gene caspase
caspase minocycline creatine
HD ( 20, 21, 22)
gene poly Q
poly Q HD (
23 24) HD
Wilsons disease

!
HD
schizophrenia


valproate olanzapine
olanzapine ( 25)
HD


huntingtin
huntington glyceraldehyde -3- phosphate
dehydrogenase enzyme glucose glycolysis

glucose ( 18) Huntington

( 45)
26 48

1) Lessof M. Sydenhams chorea. Guys Hosp Rep 1958; 107: 185-206.

2) Church AJ, Cardoso F, Dale RC, Lees AJ, Thompson EJ, Giovannoni G.
Anti-basal ganglia antibodies in acute and persistent Sydenhams chorea.
Neurology 2002; 59: 227-31.
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J Neurol Neurosurg Psychiatry 1998; 65: 436-45.
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thyrotoxicosis. Neurology 1971; 21: 55-7.
5) Kitiyakara T, Jackson M, Gorard DA. Refractory coeliac disease, small-bowel
lymphoma and chorea. J R Soc Med 2002; 95: 133-4.
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resulting from paraneoplastic striatal encephalitis. J Neurol Neurosurg Psychiatry
2000; 69: 512-5.
7) Warren JD, Kneebone CS, Firgaira F, Blumbergs PC, Thompson EM, Thompson
PD. The causes of sporadic and senile chorea. Aust NZ J Med 1998; 28: 429-31.
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chorea: another condition associated with anti-basal ganglia antibodies. J Neurol
Neurosurg Psychiatry 2004; 75: 327-8.
9) Harper PS. Benign hereditary chorea: clinical and genetic aspects. Clin Genet
1978; 13: 85-95.
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al. Clinical and genetic heterogeneity in benign hereditary chorea. Neurology 2002;
59: 579-84.

11) Critchley EMR, Clark DB, Wiklen A. Acanthocytosis and neurological disorder
without abetalipoproteinemia. Arch Neurol 1968; 18: 134-40.
12) Levine IM, Estes JW, Ioonez JM. Hereditary neurological disease with
acanthocytosis: a new syndrome. Arch Neurol 1968; 19: 403-09.
13) Hardie RJ, Pullon HW, Harding AE, Owen JS, Pires M, Daniels GL, et al.
Neuroacanthocytosis: a clinical, hematological and pathological study of 19 cases.
Brain 1991; 114: 13-49.
14) Bohlega S, Al-Jishi A, Dobson-Stone C, Rampoldi L, Saha P, Murad H, et al.
Chorea-acanthocytosis: Clinical and genetic findings in three families from
The Arabian Peninsula. Mov Disord 2003; 18: 403-07.
15) Critchley M. Huntingtons chorea: historical and geographical considerations.
In: The Black Hole and Other Essays. Pitman Medical Publishing Company Ltd.
1964; pp. 210-9.
16) Pearce JMS. Huntingtons chorea. In: Fragments of Neurological History.
Ed. Pearce JMS. Imperial College Press, London. 2003; pp. 118-22.
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disease in Malaysia: a clinical and genetic study. Neurol J Southeast Asia 1997;
2: 57-63.
18) Bruyn GW, Bruyn RPM. Huntingtons chorea. In: Neurological Eponyms.
Eds. Koehler PY, Bruyn GW, Pearce JMS. Oxford University Press. New York.
2000; pp. 330-4.
19) Crosby AH. Disruption of cellular transport: a common cause of
neurodegeneration ? Lancet Neurol 2003; 2: 311-6.
20) Friedlander RM. Apoptosis and caspases in neurodegenerative diseases. N Engl
J Med 2003; 348: 1365-75.
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open label study. Neurology 2003; 60: 883-4.
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al. Creatine therapy for Huntingtons disease: clinical and MRS findings in a 1-year
pilot study. Neurology 2003; 61: 141-2.

23) Bates G. Huntingtin aggregation and toxicity in Huntingtons disease. Lancet

2003; 361: 1642-44.
24) Blum D, Hourez R, Galas M-C, Popoli P, Schiffmann SN. Adenosine receptors
and Huntingtons disease: implications for pathogenesis and therapeutics.
Lancet Neurol 2003; 2: 366-74.
25) Grove Jr. VE, Quintanilla J, DeVaney GT. Improvement of Huntingtons disese
with olanzapine and valproate. N Engl J Med 2000; 343: 973-4.
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testing and Huntingtons disease: issues of employment. Lancet Neurol 2004; 3:
249-52.
27) Hayden MR. Predictive testing for Huntingtons disease: a universal model ?
Lancet Neurol 2003; 2: 141-2.
28) Lancet Neurol Newsdesk. Huntingtons disease drives mother to murder.
2002; 1: 203.
29) Wheelock VL, Tempkin T, Marder K, Nance M, Myers RH, Zhao H, et al and
the Huntington Study Group. Predictors of nursing home placement in
Huntington disease. Neurology 2003; 60: 998-1001.
30) Lees AJ. Odd and unusual movement disorders
Psychiatry 2002; 72(Suppl 1): i17-i21.

J Neurol Neurosurg

31) Lennox BR, Lennox GG. Mind and movement: The neuropsychiatry of
movement disorders. J Neurol Neurosurg Psychiatry 2002; 72(Suppl 1): i28-i31.
32) Lefaucheur J-P, Bachoud-Levi A-C, Bourdet C, Grandmougin T, Hantraye P,
Cesaro P, et al. Clinical relevance of electrophysiological tests in the assessment
of patients with Huntingtons disease. Mov Disord 2002; 17: 1294-1301.
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Huntingtons disease. Clinical correlates of disability and progression.
Neurology 2003; 61: 1085-92.
34) Rosenblatt A, Abbott MH, Gourley LM, Troncoso JC, Margolis RL, Brandt J, et
al. Predictors of neuropathological severity in 100 patients with Huntingtons disease.
Ann Neurol 2003; 54: 488-93.

35) Caramins M, Halliday G, McCusker E, Trent RJ. Genetically confirmed clinical

Huntingtons disease with no observable cell loss. J Neurol Neurosurg Psychiatry
2003; 74: 968-70.
36) Lancet Neurol Newsdesk. Mitochondrial problems may underlie Huntingtons
disease 2002; 1: 203.
37) Lancet Neurol Newsdesk. Huntingtin halts axonal transport. 2003; 2: 651.
38) Lucetti C, Del Dotto P, Gambaccini G, DellAgnello G, Bernardini S, Rossi G.
IV amantadine improves chorea in Huntingtons disease. An acute randomized,
controlled study. Neurology 2003; 60: 1995-7.
39) Kassubek J, Juengling FD, Kiochies T, Henkel K, Karitzky J, Kramer B, et al.
Topography of cerebral atrophy in early Huntingtons disease: a voxel based
morphometric MRI study. J Neurol Neurosurg Psychiatry 2004; 75: 213-20.
40) Bushara KO, Nance K, Gomez CM. Antigliadin antibodies in Huntingtons
disease. Neurology 2004; 62: 132-3.
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et al. Huntingtons disease of the endocrine pancreas: insulin deficiency and
diabetes mellitus due to impaired insulin gene expression. Neurobiol Dis 2002; 11:
410-24.
42) Neurology Today Genes and environment affect age of onset of Huntingtons
disease. News from the Venezuela kindreds study. 2004; 4: 21-2.
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V, et al. Functional brain changes in presymptomatic Huntingtons disease. Ann
Neurol 2004; 55: 879-83.
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Onset and rate of striatal atrophy in preclinical Huntingtons disease. Neurology
2004; 63: 66-72.
45) Walker FO, Raymond LA. Targeting energy metabolism in Huntingtons disease.
Lancet 2004; 364: 312-3.
46) Lancet Neurol Newsdesk. Huntingtin transports brain-derived neurotrophic
factor (BDNF). 2004; 3: 514.

47) Seo H, Sontag K-C, Isacson O. Generalized brain and skin proteosome inhibition
in Huntingtons disease. Ann Neurol 2004; 56: 319-28.
48) Simpson SA. The management of Huntingtons disease. Pract Neurol 2004; 4:
204-11.

12

32 .

2 1 .
2 38 2
220/120 . protein 3+
10 1
34 150/110
150/90 1 120/80
4-5 . 2

1
150/100 .

pre-eclampsia 20

occipital lobe 2 cortical blindness posterior leucoencephalopathy
syndrome
malignant hypertension

cyclosporin, tacrolimus, inteferon-alfa erythropoietin AIDS TTP

vertebro-basilar circulation
CT MRI occipital lobes
visual area oedema

PR 96 regular
BP 200/110
Fundi: retinal haemorrhages and soft exudates
AV ratio 1:3
EOM: full
visual fields visual acuity

 Hb 10.6 Hct
31.7 WBC 21,730 N83 L16 M1 platelets 69,000/.. BUN 28 Cr 2.1 SGOT
1,320
PT 872 HELLP Syndrome ()
IV 10% magnesium sulphate Caesarean
section
CCU
sodium
nitroprusside drip 1
Caesarean section

Pre-eclampsia 3 5
20

Pre-eclampsia placentation
endothelium

oxidative stress
8 up-regulation soluble
fms-like tyrosine kinase 1 (sFlt 1) m RNA
sFlt 1 free vascular endothelial growth
factor (VEGF) free placental growth factor (PlGF)
glomeruli
HELLP

haemolysis, elevated liver enzymes and low platelet count (
100,000 150,000 -
10 11) HELLP Syndrome
( 9) sFlt 1 PlGF
pre-eclampsia

(maternal and foetal medicine)

1) Solomon CG, Seely EW. Pre-eclampsia searching for the cause. N Engl J Med
2004; 350: 641-2.
2) Walker JJ. Pre-eclampsia. Lancet 2000; 356: 1260-5.
3) Roberts JM, Cooper DW. Pathogenesis and genetics of pre-eclampsia. Lancet
2001; 357: 53-6.

4) Pipkin FB. Risk factors for pre-eclampsia. N Engl J Med 2001; 344: 925-6.
5) Skjaerven R, Wilcox AJ, Lie RT. The interval between pregnancies and
the risk of pre-eclampsia. N Engl J Med 2002; 346: 33-8.
6) Belfort MA, Anthony J, Saade GR, Allen JC, for the Nimodipine Study Group.
A comparison of magnesium sulfate and nimodipine for the prevention of
eclampsia. N Engl J Med 2003; 348: 304-11.
7) Greene MF. Magnesium sulfate for pre-eclampsia. N Engl J Med 2003; 348:
275-6.
8) Lancet Medicine and Health Policy. Placental protein may have a role in
pre-eclampsia. 2003; 361: 940.
9) Levine RJ, Maynard SE, Qian C, Lim K-H, England LJ, Yu KF, et al.
Circulating angiogenic factors and the risk of pre-eclampsia. N Engl J Med
2004; 350: 672-83.
10) Weinstein L. Prevention of eclampsia Letter to the Editor. N Engl J Med
2003; 348: 2154-5.
11) Weinstein L. Syndrome of hemolysis, elevated liver enzymes and low platelet
count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol
1982; 142: 159-67.
12) Veltkamp R, Kupsch A, Polasek J, Yousry TA, Pfister HW. Late onset
postpartum eclampsia without pre-eclamptic prodromi: clinical and neuroradiological
presentation in two patients. J Neurol Neurosurg Psychiatry 2000; 69: 824-7.
13) Vaughan CJ, Delanty N. Hypertensive emergencies. Lancet 2000; 356: 411-7.
14) Tsatsaris V, Goffin F, Foidart JM. Circulating angiogenic factors and preeclampsia Letter to the Editor. N Engl J Med 2004; 350: 2003.

13
!
27

1

.

optic nerve
optic fundus ophthalmoscope
optic disc primary optic atrophy visual field
field defect (temporal)
optic chiasma optic nerve
temporal field defect upper
lower quadrantic field defect
pituitary tumour craniopharyngioma
meningioma visual field
cranial nerve
1 anosmia olfactory
groove meningioma

Wt. 80 kgs.

Ht. 162 cms.

T 36.8C

PR 72

110/70
VAR 20/40
Fundi : normal
L

VAL : no PL

pale disc

Visual fields : temporal hemianopia

BP

Genitalia : normal
no other abnormality
()
L optic nerve anterior optic
chiasma pituitary tumour
MRI

MRI large well-defined lobulated extra-axial mass occupying the whole

suprasellar cistern, more on the L side

CBC UA
FBG, Na+ K+ Cl- CO2, TP, albumin, Cr, cholesterol, TG, Ca++, SGOT, PT
alkaline phosphatase
Serum prolactin 11,100 ng/ml ( 20)

prolactin MRI
Prolactinoma pituitary craniopharyngioma
prolactin 200 ng/ml
Verapramil risperidone, butyrophenones, metoclopramide
tri-cyclic
antidepressants prolactin 100 ng/ml
dopamine agonist

Bromocriptine (2.5 mg) 2 4 2

CT
2 prolactin 950 ng/ml
3 12.7 ng/ml

prolactin
1 100
pituitary gland 5 pituitary tumour 100
prolactinoma 40 !
prolactin ( 90)
sella turcica

pituitary

 dopamine prolactin dopamine agonist

bromocriptine, cabergoline

1) Schlechte JA. Prolactinoma. N Engl J Med 2003; 349: 2035-41.

2) Bevan JS, Webster J, Burke CW, Scanlon MF. Dopamine agonists and pituitary
tumor shrinkage. Endocr Rev 1992; 13: 220-40.
3) Molitch M. Prolactinomas. In: Melmed S., ed. The pituitary. 2nd ed. Malden,
Mass.: Blackwell, 2002: 455-95.
4) Colao A, Di Sarno A, Cappabianca P, Di Somma C, Pivonello R, Lombardi G.
Withdrawal of long-term carbergoline therapy for tumoral and nontumoral
hyperprolactinemia. N Engl J Med 2003; 349: 2023-33.
5) N Engl J Med 2004; 350: 1054-7. Prolactinomas. Correspondence relating to
4).
6) Colao A, Lombardi G. Growth-hormone and prolactin excess. Lancet 1998;
352: 1455-61.
7) Talkad AV, Kattah JC, Xu MY, Orth EH, Chang JY. Prolactinoma presenting as
painful postganglionic Horner syndrome. Neurology 2004; 62: 1440-1.
8) van Uum SHM, van Alfen N, Wesseling P, van Lindert E, Pieters GFFM,
Nooijen P, et al. Massive reduction of tumour load and normalisation of
hyperprolactinaemia after high dose cabergoline in metastasised prolactinoma
causing thoracic syringomyelia. J Neurol Neurosurg Psychiatry 2004; 75: 1489-91.

14

49 .

1
2 .

3
1

. 5

gout
metastatic tumour humerus

hypernephroma renal-cell carcinoma

T 38.3C
PR 100
RR 24
BP 110/70
mildly pale, no finger clubbing
Chest : occasional rhonchi on side anteriorly
L shoulder : not swollen, limited movement in all direction by pain
Abd : no mass felt
no other abnormality
()
x ray CBC,
PSA, Ca alk phosphatase
++

Urine : sp gr 1.020
pH 6
no protein, glucose or ketone
RBC 0-1 WBC 5-10/HPF
granular casts 0-1
Hb 10.2 PCV 31 MCV 85.8
MCH 28.3
6
WBC 9600 N 60 L 30
RBC 3.6x10

MCHC 33
M 10

Platelets 703,000
BUN 21 Cr 1.6 Uric Acid 6.4
Ca++ 14.7
P 4.1
Alk Phos. 669 (40-105)
TP 72.5/alb 33.4
SGOT 73
PT 133
GGT 584
ESR and PSA not available
CXR : nodule in middle lobe
bony metastases in scapula, L 8th and 9th ribs anteriorly,
12th rib anteriorly and L 7th, 8th and 11th ribs posteriorly
99-technitium MDP bone scan showed increased uptake in
both shoulder joints, right scapula and those ribs and kidney
Ultrasound abdomen showed large lobulated mixed hypo-and hyper-echoic
mass in the upper pole of L kidney
. 2-3
37-38.5C 14 .
renal-cell carcinoma .
10 .
5

(renal-cell carcinoma, RC)

75
RC ( 16) RC

( 24-75)

( 1)
4-5 RC 3 50
35 2

(erythrocytemia polycythaemia) erythropoietin
tumour metastases
( 2) RC
inferior vena cava RC vascular endothelium
vascular endothelial growth factor (VEGF)
tumour suppressor gene VHL VEGF
bevacizumab neutralizing antibody VEGF RC
metastases ( 3) RC
1 3 metastases

( 4)
( 5 6) ( 7)
( 8)
RC .. 1999
267450 ( non-melanoma skin cancer) RC 6000 2
( 2) 41300
( 15) 38190 ( 14)
35410 13 ( 9)
RC hypernephroma Grawitz tumour
P.S. Grawitz Rudolf Virchow
Grawitz
.. 1887 37
(RC) Grawitz .. 1932 82
( 10)

1) Frank IN. Urology. In: Principles of Surgery. 4th Edition. Vol. 2. Eds.
Schwartz SI, Shires GT, Spencer FC, Storer EH. McGraw Hill Book Co. New York
1984; pp. 1705-7.
2) Petzold GC, Valdueza JM, Zimmer C. Cerebral metastasis of renal carcinoma
mimicking haemorrhagic infarction. J Neurol Neurosurg Psychiatry 2004; 75: 477.
3) Yang JC, Hawarth L, Sherry RM, Hwu P, Schwartzentruber DJ, Topalian SL, et
al. A randomized trial of bevacizumab, and anti-vascular endothelial growth factor
antibody for metastatic renal cancer. N Engl J Med 2003; 349: 427-34.
4) Vogelzang NJ, Stadler WM. Kidney cancer. Lancet 1998; 352: 1691-6.
5) Jocham D, Richter A, Hofman L, Iwig K, Fahlenkamp D, Zakrzewski G, et al.
Adjuvant autologous renal tumour cell vaccine and risk of tumour progression
in patients with renal-cell carcinoma after radical nephrectomy. Lancet 2004;
363: 594-9.
6) Kommu S. Correspondence. Renal-cell carcinoma: vaccination and risk of
tumour progression. Lancet 2004; 363: 1557.
7) Jones PH, Christodoulos K, Dobbs N, Thavasu P, Balkwill F, Blann AD, et al.
Combination antiangiogenesis therapy with marimastat, captopril and fragmin
in patients with advanced cancer. Br J Cancer 2004; 91: 30-6.

8) Vincenzi B, Santini D, La Cesa A, Tonini G. Zoledronic acid delays the onset of

skeletal-related events and progression of skeletal disease in patients with advanced
renal cell carcinoma. Cancer 2004; 100: 1987-8.
9) McKinney PA. Brain tumours: incidence, survival, and aetiology. J Neurol
Neurosurg Psychiatry 2004; 75 (Suppl II): ii12-ii17.
10) Firkin BG, Whitworth JA. Dictionary of Medical Eponyms. The Parthenon
Publishing Group. Carnforth, Lancs, UK. 1987; pp. 206-7.

15

34 .
12 2532

3
3 1
2
3


paracetamol flunarizine 10 1
2-3 2-3
2-3

2
2
2547
18

intelligent, healthy looking

PR 72 BP 120/80
Fundi : normal
no abnormality

Cluster headache (CH)

Gerhard van Swieten
.. 1745 ( 1
2) Hansruedi E. Isler
van Swieten CH

International Headache Society .. 1988 ( 3)

A healthy, robust-man of middle age was, each day, at the same hour
troubled by pain above the orbit of the left eye, where the nerve leaves through the bony
frontal opening; after a short time the left eye began to redden and tears to flow; then he
felt as if his eye was protruding from its orbit with so much pain that he became mad.
After a few hours all this evil ceased and nothing in the eye appeared at all changed.

 CH
Thomas Willis (.. 1621-1675)
CH Sir Wilfred Harris
Queen Square CH
.. 1926 periodic migrainous
neuralgia of Wilfred Harris. ( 1, 4 5) Harris
CH
Histaminic Cephalgia Hortons Syndrome Bayard Taylor Horton
Mayo Clinic, Rochester, Minnesota CH .. 1939
Horton Horton
histamine
dihydroergotamine Cafergot
( 6 7)
CH ( 8)
2.5 56 1 (95% CI 31.3-92.4)
( 9) Balla Walton
Newcastle CH
6
3 (1
-48 , median 3 ) 1 3 1 3
CH CH
( 10)
( 11) CH
aura ( 12)
( 13) CH

( 14)
CH


hypothalamus
CH CH positron emission tomography (PET)
hypothalamus ( 15) Leon
( 16) posterior hypothalamic grey matter
CH CH
17, 18 19
CH
triptans sumatriptan zolmitriptan
( 20) 100%
7 15

methysergide
4-6 2-3 3-4

 verapamil 80 3
( 21 23)
Lithium 300-600
corticosteroids valproate 2
corticosteroids
valproate
somatostatin
analog octreotide 100 g (
24) CH
posterior hypothalamus ( 16 19)
CH
CH (primary headache)
1 500
first-degree relatives 5-18
second-degree relatives 3 autosomal dominant
CH
CH ( 25) ion
channel genes clock genes

1) Pearce JMS. Cluster headache. In: Fragments of Neurological History.

Imperial College Press. London. 2003; pp. 159-63.
2) Pearce JMS. Gerhard van Swieten and episodic cluster headache. In:
Fragments of Neurological History. Imperial College Press. London. 2003; pp.177-8.
3) Isler H. Episodic cluster headache from a textbook of 1745: van Swietens
classic description. Cephalgia 1993; 13: 172-4.
4) Harris W. Neuritis and Neuralgia. Oxford University Press. London. 1926; pp.
293-314.
5) Harris W. The Facial Neuralgia. Oxford University Press. London. 1937; pp.
70-6.
6) Pearce JMS. Hortons syndrome. In: Neurological Eponyms. Eds. Koehler PJ,
Bruyn GW, Pearce JMS. Oxford University Press. New York. 2000; pp. 322-9.
7) Ekbom KA. Ergotamine tartrate orally in Hortons histaminic cephalgia
(also called Harriss ciliary neuralgia) Acta Psychiatr Neurol Scand 1947;
46: 105-13.

8) Tonon C, Guttmann S, Volpini M, Naccarato S, Cortelli P, DAlessandro R.

Prevalence and incidence of cluster headache in the Republic of San Marino.
Neurology 2002; 58: 1407-09.
9) Balla JI, Walton JN. Periodic migrainous neuralgia. Br Med J 1964; 1: 219-21.
10) van Vliet JA, Eekers PJE, Haan J, Ferrari MD, for the Dutch RUSSH study group.
Features involved in the diagnostic delay of cluster headache. J Neurol Neurosurg
Psychiatry 2003; 74: 1123-5.
11) Klapper JA, Klapper A, Voss T. The misdiagnosis of cluster headache: a nonclinic, population based, internet survey. Headache 2000; 40: 730-5.
12) Silberstein SD, Nicknam R, Rozen TD, Young WB. Cluster headache with aura.
Neurology 2000; 54: 219-21.
13) Blau JN, Engel HO. A new cluster headache precipitant: increased body heat.
Lancet 1999; 354: 1001-02.
14) Rozen TD, Nicknam RM, Shechter AL, Young WB, Silberstein SD. Cluster
headache in woman: clinical characteristics and comparison with cluster headache
in men. J Neurol Neurosurg Psychiatry 2001; 70: 613-7.
15) May A, Bahra A, Bchel C, Frackowiak RSJ, Goadsby PJ. Hypothalamic
activation in cluster headache attacks. Lancet 1998; 352: 275-8.
16) Leone M, Franzini A, Bussone G. Stereotactic stimulation of posterior
hypothalamic gray matter in a patient with intractable cluster headache. N Engl J
Med 2001; 345: 1428-9.
17) Goadsby PJ. Pathophysiology of cluster headache: a trigeminal autonomic
cephalgia. Lancet Neurol 2002; 1: 251-7.
18) Overeem S, van Vliet JA, Lammers GJ, Zitman FG, Swaab DF, Ferrari MD.
The hypothalamus in episodic brain disorders. Lancet Neurol 2002; 1: 437-44.
19) May A, Leone M. Update on cluster headache. Curr Opin Neurol 2003; 16:
333-40.
20) van Vliet JA, Bahra A, Martin V, Ramadan N, Aurora SK, Mathew NT, et al.
Intranasal sumatriptan in cluster headache. Randomized placebo-controlled
double-blind study. Neurology 2003; 60: 630-3.

21) Leone M, DAmico D, Frediani F, Moschiano F, Grazzil, Attanasio A, et al.

Verapamil in the prophylaxis of episodic cluster headache: A double-blind study
versus placebo. Neurology 2000; 54: 1382-5.
22) Matharu M, Goadsby P. Cluster headache. Update on a common neurological
problem Prac Neurol 2001; 1: 43-9.
23) Bahra A, May A, Goadsby PJ. Cluster headache. A prospective clinical study
with diagnostic implications. Neurology 2002; 58: 354-61.
24) Matharu MS, Levy MJ, Meeran K, Goadsby PJ. Subcutaneous octreotide in
cluster headache: Randomized placebo-controlled double-blind cross-over study.
Ann Neurol 2004; 56: 488-94.
25) Russell MB. Epidemiology and genetics of cluster headache. Lancet Neurol
2004; 3: 279-83.

16

55 .

1 .
6 10
VA 3
Metronidazole 3
VA 20/40
1 .
2-3

2

2
creatinine 5.5

pale, thin
no oedema
T 37C
PR 66 regular RR 20
BP 150/80
Heart
Abd
no lymphadenopathy
NS
Hb 25
WBC 3,280
N 49 L 49 M 2
Platelets 182,000
Rouloux formation +
Urine : sp.gr. 7.0
protein 2+
WBC 2-3/HPF
BUN 40
Cr 11.6
T.P. 106
Alb 25.3
++
Ca 11.2
Skull x ray : multiple punched-out (osteolytic) lesions

Ultrasound abdomen : kidney 9.5 cms. L 9.3 cms.

no post-renal obstruction
Protein electrophoresis : Monogammopathy IgA (56.1)
2M 37.7 (normal 0.7-3.4)
Bone marrow biopsy : diffuse plasmocytic infiltrates, diffuse mature and
immature plasma cells with markedly
decreased normal
haematopoiesis
Kidney biopsy : myeloma deposits in glomeruli with tubular dysfunction

rouloux formation serum

protein electrophoresis
multiple
myeloma (MM) Bence Jones protein

MM William Macintyre
Thomas McBean 45
Macintyre .. 1850
Henry Bene Jones 31
St. Georges Hospital ( 1)
MM 4
65
MM
20 2

70 1 5 10
50

thalidomide, CE5013 bortezomib myeloma cell
bisphosphonate MM
7-8

1) Kyle RA. History of multiple myeloma. In: Multiple Myeloma. Eds. Gahrton G,
Durie BGM. Arnold, London. 1996; pp. 1-11.
2) Sirohi B, Powles R. Multiple myeloma. Lancet 2004; 363: 875-87.
3) Richardson PG, Barlogie B, Berenson J, Singhal S, Jagannath S, Irwin D, et al.
A phase 2 study of bortezomib in relapsed, refractory myeloma. N Engl J Med
2003; 348: 2609-17.

4) Attal M, Harorsseau J-L, Facon T, Guilhot F, Doyen C, Fuzibet J-G, et al.

for the InterGroupe Francophone du Mylome. Single versus double autologous
stem-cell transplantation for multiple myeloma. N Engl J Med 2003; 349: 2495502.
5) Stadtmauer EA. Multiple myeloma 2004 one or two transplants ? N Engl J
Med 2003; 349: 2551-3.
6) Mitchell BS. The proteasome an emerging therapeutic target in cancer. N
Engl J Med 2003; 348: 2597-8.
7) Child JA, Morgan GJ, Davies FE, Owen RG, Bell SE, Hawkins K, et al. for the
Medical Research Council Adult Leukaemia Working Party. High-dose chemotherapy
with hematopoietic stem-cell rescue for multiple myeloma. N Engl J Med 2003;
348: 1875-83.
8) Tian E, Zhan F, Walker R, Rasmussen E, Ma Y, Barlogie B, et al. The role of
the Wnt-Signaling antagonist DKK1 in the development of osteolytic lesions in
multiple myeloma. N Engl J Med 2003; 349: 2483-94.
9) N Engl J Med 2004; 350: 1464-7. Correspondence relating to 8) and 4).
10) Imrie K, Esmail R, Meyer RM and the Members of the Haematology Disease
Site Group of the Cancer Care Ontario Practice Guidelines Initiative. The role of
high-dose chemotherapy and stem-cell transplantation in patients with multiple
myeloma: A practice guideline of the Cancer Care Ontario Practice Guidelines
Initiative. Ann Intern Med 2002; 136: 619-29.
11) N Engl J Med Case Records of the Massachusetts General Hospital. 2002;
346: 603-10.
12) Owens P, Morgan-Hughes G, Kelly S, Ring N, Marshall AJ. Myeloma and a mass
in the heart. J R Soc Med 2003; 96: 288-9.
13) Weintraub R, Pramanik S, Levitt L. Diffuse small-bowel myelomatosis. N Engl J
Med 2004; 350: 842-3.
14) Bradwell AR, Carr-Smith HD, Mead GP, Harvey TC, Drayson MT. Serum test for
assessment of patients with Bence Jones myeloma. Lancet 2003; 361: 489-91.

17

45

epileptic convulsion

venous sinus

metastasis, abscess cysticercosis

Wt. 46.5 kgs. T 37C

PR 96, irregularly irregular, HR 90
130/80 mm.Hg.
drowsy, no neck stiffness
Eyes deviated to
L UMN facial weakness
Tongue : deviated to L
L limb weakness, power grade 3/5
DTRs 1+
L 2+
P

th
Heart : apex beat at 6 intercostal space
just lateral to mid-axillary line
no heaving or thrill
pan-systolic murmur at apex and parasternal border
Lungs : clear

BP

Abdomen : no abnormality
Legs : no oedema
()
atrial fibrillation pulse deficit (PR HR
) mitral regurgitation pansystolic murmur apex
cardiogenic embolic infarct middle cerebral
artery CT scan
anticoagulant Echocardiogram clot
left atrium

CT scan brain haematoma

Echocardiogram mild mitral regurgitation, mild to moderate tricuspid
regurgitation and suspected clot in left atrium
Blood :

Hb 19
Hct 57.6
RBC 6.12 x 106
WBC 10,000
N 84 L 15 M 1
Platelets 206,000
TP 67.7
alb 40
LFTs and electrolytes were normal

()


digoxin
anticoagulant coumadin INR 3.0
Hb 19 PCV 57.6
17.7 52.2 ( 1, 2) 300
500 ml. Hb PCV

300 ml.
enalarpil diphenylhydantoin

digoxin, coumadin, pentoxyfyline,

Hb Hct 5 Hb 14.7
L hemiparesis

PCV 43.8

 atrial fibrillation
(AF) polycythaemia
AF
embolic
stroke
anticoagulant coumadin
( 3, 4, 5
12)

Polycythaemia pack cell

volume 3
1) Primary polycythaemia Polycythaemia rubra vera
gene 50
PCV

2) Secondary polycythaemia erythropoictin

hypoxia, congenital cyanotic heart disease,

inappropriate
erythropoietin increase hypernephroma, polycystic kidney disease
hepatoma, uterine leiomyoma cerebellar haemangioblastoma

3) Apparent polycythaemia
PCV red-cell mass Erythrocytosis plasma volume
red cell mass
50 3
Gaisbocks Syndrome ..
1905 ( 17.7) PCV 52%


T.C. Pearson

cerebral blood flow (CBF) ml. 100
haematocrit 51% red cell mass (RCM)
39 45.8 ml. 100 haematocrit
42.1 CBF 68.6 ( 6) Pearson
idiopathic erythrocytosis 12 30
( 40) primary proliferative polycythaemia 6
5 7
( 7)
40
stroke PCV
300-500 ml. 5-6 4-5
1 44 PCV 55 Hb
18.2
apparent polycythaemia 2


20
65 1-2 haematocrit 51

polycythaemia (
8 9)
stress polycythaemia( 10) Pearson
PCV micro-haematocrit electronic
counters PCV
Pearson red cell mass plasma volume
PCV 51 PCV 48% apparent
polycythaemia idiopathic erythrocytosis ( 3 )
PCV 55 PCV 45 PCV 54.951
( 11)
stroke

1) Weatherall DJ. Disorders of the blood. In: Oxford Textbook of Medicine.

Second Edition. Vol. II. Eds. Weatherall DJ, Ledingham JG and
Warrell DA. Oxford Medical Publications. Oxford University Press.
Oxford. 1987; pp. 19.4.
2) Provan D, Weatherall D. Red cells II: acquired anaemias and polycythaemia.
Lancet 2000; 355: 1260-8.
3) Narayan SM, Cain ME, Smith JM. Atrial fibrillation. Lancet 1997; 350: 94350.
4) Falk RH. Atrial fibrillation. N Engl J Med 2001; 344: 1067-78.
5) Ferro JM. Cardioembolic Stroke: an update. Lancet Neurol 2003; 2: 177-88.
6) Humphrey PR, Du Roulay GH, Marshall J, Pearson TC, Rusell RWR, Symon L, et
al. Cerebral blood-flow and viscosity in relative polycythaemia. Lancet 1979;
2: 873-7.
7) Pearson TC, Wetherley-Mein G. The course and complications of idiopathic
erythrocytosis. Clin Lab Haematol 1979; 1: 189-96.

8) Moore-Gillon JC, Pearson TC. Smoking, drinking and polycythaemia. Br Med J

1986; 292: 1617-8.
9) Moore-Gillon J. Smoking a major cause of polycythaemia. J R Soc Med
1988; 81: 431.
10) Lawrence JH, Berlin NI. Relative polycythemia the Polycythemia of Stress.
Yale J Biol Med 1952; 24: 498-505.
11) Pearson TC. Stress polycythemia. In: Advanced Medicine 23.
Eds. Pounder RE, Chiodini PL. Royal College of Physicians of London.
Baillire Tindall. London. 1987; pp. 263-74.
12) Early MJ, Sporton SC. Atrial fibrillation: current perspectives. Clin Med
2004; 4: 22-6.
13) Donnan GA, Dewey HM, Chambers BR. Warfarin for atrial fibrillation:
the end of an era? Lancet Neurol 2004; 3: 305-08.

18

25

2
3

primary subarachnoid haemorrhage (SAH)
aneurysm arterio-venous malformation
1SAH

T 37.3C
PR 90
BP 170/120 mm.Hg.
drowsy, but could answer questions
ptosis L eye
neck stiffness present
Pupils : unequal L 4 mm.
2 mm.
L external squint c limitation of upward, downward and inward movements
Other cranial nerves were intact
Limbs : no abnormality
Kernigs sign +ve
Heart : no murmur
Peripheral pulses normal
Abdomen
mass in L flank, moved with respiration
bimanually palpated

()
Kernigs sign 1SAH
cranial 3rd nerve

 posterior communicating aneurysm (PCA) aneurysm

3rd nerve basilar artery PCA
25

Polycystic Kidney Disease (PKD) intracranial aneurysm
5-15 ( 1) 500

Lumbar Puncture : blood stained CSF
Ultrasound abdomen : large irregular kidneys, L > R and slight hepatomegaly
MRIA : L posterior communicating aneurysm seen

2
PKD autosomal dominant
recessive
ADPKD
chromosome 16p13.3 chromosome 4q13-q23
ADPKD
2 .. 1888
.. 1899 PKD1 PKD2 encode
2 polycystin-1 -2 cilia epithelial cells
cilia ADPKD
( 2 8)
intracranial aneurysm
4-5 ( 9 14)
1SAH ultrasound
ADPKD MRI !

1) Burn DJ, Bates D. Neurology and the kidney. J Neurol Neurosurg Psychiatry
1998; 65: 810-21.
2) Wilson PD. Polycystic kidney disease. N Engl J Med 2004; 350: 151-64.
3) Ong ACM, Wheatley DN. Polycystic kidney disease the ciliary connection.
Lancet 2003; 361: 774-6.

4) The Lancet Medicine and health policy. Cilia dysfunction link to cystic kidney
disorders strengthened. 2003; 362: 301.
5) Peters DJM, Breuning MH. Autosomal dominant polycystic kidney disease:
modification of disease progression. Lancet 2001; 358: 1439-44.
6) Gabow PA. Autosomal dominant polycystic kidney disease: more than a renal
disease. Am J Kidney Dis 1990; 16: 403-13.
7) Rosetti S, Chauveau D, Kubly V, Slezak JM, Saggar-Malik AK, Pei Y, et al.
Association of mutation position in polycystic kidney disease 1(PKD1) gene
and development of a vascular phenotype. Lancet 2003; 361: 2196-201.
8) Bleyer AJ, Hart TC. Letter to the editor. Polycystic kidney disease. N Engl J
Med 2004; 350: 2622.
9) van den Berg JS, Limburg M, Pals G, Arwert F, Westerveld A, Hennekam RC, et
al. Some patients with intracranial aneurysms have a reduced type III/type I collagen
ratio. A case-control study. Neurology 1997; 49: 1546-51.
10) Friedman JA, Piepgras DG, Pichelmann MA, Hansen KK, Brown Jr. RD, Wiebers
DO. Small cerebral aneurysms presenting with symptoms other than rupture.
Neurology 2001; 57: 1212-6.
11) Juvela S, Porras M, Heiskanen O. Natural history of unruptured intracranial
aneurysms: a long-term follow-up study. J Neurosurg 1993; 79: 174-82.
12) Rinkel GJ, Djibuti M, Algra A, van Gijn J. Prevalence and risk of rupture of
intracranial aneurysms: a systematic review. Stroke 1998; 29: 251-6.
13) Ronkainen A, Hemesniemi J, Puranen M, Niemitukia L, Vanninen R, Ryynanen M,
et al. Familial intracranial aneurysms. Lancet 1997; 349: 380-4.
14) White PM. How to manage the patient with a family history of aneurysmal
subarachnoid haemorrhage. Practical Neurology 2004; 4: 88-103.

19

18

3

1 .
10 2

(dysphagia)
( 1) 2
1) (striated muscle)


myasthenia
gravis motor neuron disease stroke,
multiple sclerosis

2)

2
2.1
Ludwigs angina cellulitis
anaerobes angioneurotic oedema
odynophagia
globus sensation dysphagia
odynophagia

aortic aneurysm, dilated left atrium
mitral stenosis, retrosteral thyroid goitre
dysphagia lusoria subclavian
oesophagus Plummer-Vinson Brown-Kelly-Patterson
web hypopharynx oesophagus
glossitis koilonychia

 ( Plummer Vinson Mayo

Clinic Brown-Kelly Patterson
4 )

(medical eponyms)
insertion inferior oesophago-phrenic ligament Schatski ring
1930 ( 2)

candida

2.2
oesophageal motility
disorders 4-5 diffuse oesophageal
spasm 50

gastro-oesophageal reflux barium

3
corkscrew oesophagus
gastro-oesophageal reflux gastro-esophageal reflux
disease GERD

Barretts metaplasia
(
4 5)
Norman Barrett


Trypasonoma cruzi Chagas
achalasia

Wt. 49 kgs
T 37C
PR 78
pale
Heart : normal
Abdomen : no mass felt
Limbs : normal muscle power

BP 120/80

()

10

 3 15 achalasia
2-3
Allgrove syndrome achalasia
alachrimia, autonomic disturbance adrenal insufficiency ACTH insensitivity
(4As) ( 6 7) achalasia

Hb 8.6
Hct 27.2
WBC 3700 N 48 L 38 E 3 B 1
6
RBC 3.30 x 10
hypochromia 1+
Platelets 478,000
HbA 98.1 A2 1.9
Serum Fe 54.8
TIBC 204.4
Coombes test : direct and indirect -ve
ANA -ve

Ultrafast CT scan chest and abdomen : diffuse oesophageal dilatation without
oesophageal or stomach lesion.
Oesophagoscopy : dilated oesophagus with retained food particles, decreased
contractility, normal looking oesophago-gastric junction at 38 cms,
mildly swollen gastric mucosa.
Oesophageal manometry : achalasia

Achalasia cardia 300 Thomas

Willis ( circle of Willis ) .. 1674
( 8) peristalsis sphincter
(myenteric neurons)
1-2 200,000 ( 9)

HLA-DQA*0101 ( 10)

barium
oesophageal manometry
sensitive ( 8 9)
achalasia
Thomas Willis ( 8)

( 11 12) botulinum toxin ( 13)

 Hellers oesophagomyotomy
( 14 15)

achalasia
( 16)

1) Heading RC. Dysphagia. In: Frenchs Index of Differential Diagnosis.

Thirteenth Edition. Eds. Bouchier IAD, Ellis H, Fleming PR.
Butterworth Heinemann. Oxford. 1996; pp. 159-63.
2) Firkin BG, Whitworth JA. Dictionary of Medical Eponyms. The Parthenon
Publishing Group. Canforth, Lancs., U.K. 1987; 591 pp.
3) Dheer S, Chang R. Corkscrew esophagus. N Engl J Med 2003; 348: 1671.
4) Jankowski JA, Harrison RF, Perry I, Balkwill F, Tselepis C. Barretts metaplasia.
Lancet 2000; 356: 2079-85.
5) Inadomi JM, Sampliner R, Lagergren J, Lieberman D, Fendrick M,Vakil N.
Screening and surveillance for Barrett esophagus in high-risk groups:
a cost-utility analysis. Ann Intern Med 2003; 138: 176-86.
6) Kimber J, McLean BN, Prevett M, Hammans SR. Allgrove or 4 A syndrome:
an autosomal recessive syndrome causing multisystem neurological disease.
J Neurol Neurosurg Psychiatry 2003; 74: 654-7.
7) Arit W, Allolio B. Adrenal insufficiency. Lancet 2003; 361: 1881-93.
8) Richter JE. Oesophageal motility disorders. Lancet 2001; 358: 823-8.
9) Dent J. Diseases of the oesophagus. In: Oxford Textbook of Medicine.
Third Edition. Vol 2. Eds. Weatherall DJ, Ledingham JGG, Warrell DA.
Oxford University Press. Oxford. 1996; pp. 1865-76.
10) De la Concha EG, Fernandez-Arquero M, Mendoza JL, Conejero L, Figueredo MA,
Perez de la Serna J, et al. Contribution of HLA class II genes to susceptibility in
achalasia. Tissue Antigens 1998; 52; 381-4.

11) Harris AM, Dresner SM, Griffin SM. Achalasia: management, outcome and
surveillance in a specialist unit. Br J Surg 2000; 87: 362-73.
12) Alonso-Aguirre P, Aba-Garrote C, Estevez-Prieto E, Gonzalez-Conde B,
Vazquez-Iglesias JL. Treatment of achalasia with the Witzel dilator: a prospective
randomized study of two methods. Endoscopy 2003; 35: 379-82.
13) Zaninotto G, Annese V, Costantini M, Del Genio A, Costantino M, Epifani M, et
al. Randomized controlled trial of botulinum toxin versus laparoscopic Heller myotomy
for esophageal achalasia. Ann Surg 2004; 239: 364-70.
14) Falkenback D, Johansson J, Oberg S, Kjellin A, Wenner J, Zilling T, et al.
Hellers esophagomyotomy with or without a 360 degrees floppy Nissen
fundoplication for achalasia. Long-term results from a prospective randomized study.
Dis Esophagus 2003; 16: 284-90.
15) Beninca G, Garrone C, Rebecchi F, Giaccone C, Morino M. Robot-assisted
laparoscopic surgery. Preliminary results at our Center. Chir Ital 2003; 55: 321-31.
16) Sandler RS, Nyren O, Ekbom A, Eisen GM, Yuen J, Josefsson S. The risk of
esophageal cancer in patients with achalasia. A population-based study. JAMA
1995; 274: 1359-62.

20

75 .

5

.
5 2

2

12

Restless Legs Syndrome (RLS)

alert and cooperative

PR 72 regular BP 150/80
CNS :
Fundi
Tongue
Limbs : no wasting
Tone
Power
Stance
Gait
SJ
+
+
BJ +
+
TJ
+
+
KJ
+
+

Coordination

AJ
P

JPS

no sensory loss

Hb 16.6
Hct 47.3
WBC 5300 N 58 L 29
M8
E4
B1
ESR 4
FBS 85 mg%
Cr 1.0
Cholesterol 221
LDL 158
Uric Acid 8.9
SGOT 46
PT 85
Alk P. 68
Serrlogy for Lyme disease, Hepatitis B & C negative
ANA profiles, anti-MAG, sulfatide, GD1b
GQ1b antibodies negative
Protein electrophoresis and Bone marrow biopsy normal
Conclusion from Nerve conduction studies : mild patchy sensori
polyneuropathy
xray spine : mild degenerative changes of lumbar spine, no lytic or sclerotic
lesion
!

2 Restless Legs
Syndrome (RLS) Ekbom Syndrome
.. 1944 asthenia crurum
paraesthetica ( 1) .. 1960 ( 2)
Ekbom 2
asthenia crurum dolorosa
Moritz Romberg (.. 1795-1873)
A Manual of the Nervous Diseases of Man

John Pearce ( 3)
Anxietas tibiarum, a sense of painful restlessness in the lower extremities, especially in the
legs and feet. The patient did not know what to do with themthough there was relief
by change of position. RLS Moritz
Romberg Romberg sign Parry-Romberg
syndrome (progressive hemifacial atrophy) Thomas Willis
RLS ( 4)
Romberg
!
RLS
2.5 15 ( 5) ADHD
attention deficit hyperactive disorder ( 6)

 RLS 24 15
RLS ( 7) RLS

Periodic Limb Movements (

5)
RLS


RLS
dopamine striato-nigral system
RLS dopaminergic agents

bromocriptine, levodopa decarboxylase inhibitor (Ferrous
sulphate) 300 RLS
ferritin ( 50 )
RLS RLS
polysomnography ( 8) dopamine agonist
ergoline pramipexole ropinirole
RLS
( 9 44) RLS
!

1) Ekbom KA. Asthenia crurum paraesthetica (irritable legs). A new syndrome

consisting of weakness, sensation of cold and nocturnal paraesthesia in the legs
responding to a certain extent to treatment with priscol and doryl; a note on
paraesthesia in general. Acta Med Scand 1944; 118: 197-209.
2) Ekbom KA. Restless legs syndrome. Neurology (Minneap) 1960; 10: 868-73.
3) Pearce JMS. Romberg on Ekboms restless legs syndrome. In: Fragments of
Neurological History. Ed. Pearce JMS. Imperial College Press. London. 2003; pp.
454-5.
4) Willis T. The London practice of physick. Basset and Crooks, London, 1685
(from Pearce, 3).
5) Earley CJ. Restless legs syndrome. N Engl J Med 2003; 348: 2103-9.
6) Sayed MA, Katagal P, Foldvary-Schaefer N, Bae C. Restless legs syndrome in
children. Ann Neurol 2004; 56 (Suppl 8): S41.

7) Nichols DA, Allen RP, Granke JH, Brown JB, Rice ML, Hyde PR, et al. Restless
legs syndrome symptoms in primary case. A prevalence study. Arch Intern Med
2003; 163: 2323-9.
8) Sun ER, Chen CA, Ho G, Earley CJ, Allen RP. Iron and the restless legs
syndrome. Sleep 1998; 21: 371-7.
9) Chaudhuri KR, Appiah-Kubi LS, Trenkwalder C. Restless legs syndrome.
J Neurol Neurosurg Psychiatry 2001; 71: 143-6.
10) Chaudhuri KR. Restless legs syndrome. N Engl J Med 2003; 349: 815.
11) Desautels A, Turecki G, Montplaisir J, Sequiera A, Verner A, Rouleau GA.
Identification of a major susceptibility locus for restless legs syndrome
on chromosome 12q. Am J Hum Genet 2001; 69: 1266-70.
12) Bonati MT, Ferini-Strambi L, Aridon P, Oldani A, Zucconi M, Casari G..
Autosomal dominant restless legs syndrome maps on chromosome 14q.
Brain 2003; 126: 1485-92.
13) Levchenko A, Montplaisir J, Dub M-P, Riviee J-B, St-Onge J, Turecki G, et al.
The 14q restless legs syndrome locus in the French Canadian population.
Ann Neurol 2004; 55: 887-91.
14) Desautels A, Turecki G, Montplaisir J, Ftouhi-Paquin N, Michaud M, Chouinard VA,
et al. Dopaminergic neurotransmission and restless legs syndrome: a genetic
association analysis. Neurology 2001; 57: 1304-6.
15) Ondo WG, Voung KD, Wang Q. Restless legs syndrome in monozygotic twins:
clinical correlates. Neurology 2000; 55: 1404-6.
16) Walters AS, Hickey K, Malzman J, Verrico T, Joseph D, Hening W, et al.
A questionnaire study of 138 patients with restless legs syndrome:
The 'Night-Walkers' survey. Neurology 1996; 46: 92-5.
17) Desautels A, Turecki G, Montplaisir J, Brisebois K, Sequeira A, Adam B, et al.
Evidence for a genetic association between monoamine oxidase A and restless
legs syndrome. Neurology 2002; 59: 215-9.
18) Allen RP, Barker PB, Wehrl F, Song HK, Earley CJ. MRI measurement of brain
iron in patients with restless legs syndrome. Neurology 2001; 56: 263-5.

19) Connor JR, Boyer PJ, Menzies SL, Dellinger B, Allen RP, Ondo WG, et al.
Neuropathological examination suggests impaired brain iron acquisition in
restless legs syndrome. Neurology 2003; 61: 304-9.
20) Connor JR, Wang XS, Patton SM, Menzies SL, Troncoso JC, Earley CJ, et al.
Decreased transferrin receptor expression by neuromelanin cells in restless legs
Syndrome. Neurology 2004; 62: 1563-7.
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1115-21.

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Restless legs syndrome and nocturnal myoclonus: initial clinical manifestation of
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 1 2 3

ATAXIA ( 9 2)
Worth PF. Sorting out ataxia in adults. Practical Neurology 2004; 4: 130-51.
ataxia
150,000 ( 60 )

Practical Neurology

BEHCETS DISEASE ( 11 1)
Sfikakis PP, Kaklamanis PH, Elezoglou A, Katsilambros N, Theodossiadis PG,
Papaefthimiou S, et al. Infliximab for recurrent, sight-threatening ocular
inflammation in Adamantiades Behet disease. Ann Intern Med 2004;
140: 404-6.
Anti-tumour necrosis factor (TNF) monoclonal antibody infliximab

BILIARY CIRRHOSIS ( 2 1)
Invernizzi P, Miozzo M, Battezzati PM, Bianch I, Grati FR, Simoni G, et al. Frequency of
monosomy X in women with primary biliary cirrhosis. Lancet 2004; 363: 533-4.
X monosomy primary biliary cirrhosis (PBC)
100
PBC
DOWNBEAT NYSTAGMUS ( 5 3)
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4-aminopyridine improves downbeat nystagmus, smooth pursuit and VOR gain.
Neurology 2004; 62: 1228-9.
2) Antonini G, Nemni R, Giubilei F, Gragnani F, Ceschin V, Morino S, et al.

Autoantibodies to glutamic acid decarboxylase in downbeat nystagmus.

J Neurol Neurosurg Psychiatry 2003; 74: 998-9.
HYPEREOSINOPHILIC SYNDROME ( 13 1)
1) Gleich GJ, Leiferman KM, Pardanani A, Tefferi A, Butterfield JH. Treatment of
hypereosinophilic syndrome with imatinib mesilate. Lancet 2002; 359: 1577-8.
imatinib tyrosine kinase chronic myeloid
leukaemia acute lymphoblastic leukaemia HES 5 4
2) Cool J, De Angelo DJ, Gotlib J, Stover EH, Legare RD, Cortes J, et al. A tyrosine
kinase
created by fusion of the PDGFRA and FIP 1L1 genes as a therapeutic target of
Imatinib
in idiopathic hypereosinophilic syndrome. N Engl J Med 2003; 348: 1201-14.
HES PDGFRA FIP 1L1 fuse Fusion protein tyrosine
kinase active imitinib HES haemopoietic cells
fusion protein
3) Schwartz RS. The hypereosinophilic syndrome and the biology of cancer. N Engl J
Med
2003; 348: 1199-1200.
MALARIA ( 17 2)
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A large focus of naturally acquired Plasmodium knowlesi infections in human beings.
Lancet 2004; 363: 1017-24.
P. knowlesi malaria Kapit Sarawak Singh
malaria
molecular genotyping malaria 58
P. knowlesi P. falciparum
P. malariae P. malariae 3 (tertian cycle)
P. knowlesi (quotidian cycle)

3) Abegunde AT. Monkey malaria in man. Lancet 2004; 364: 1217.

4) Barnes KI, Mwenechanya J, Tembo M, Mcllleron H, Folb PI, Ribeiro I, et al. Effect of

rectal artesunate compared with parenteral quinine in initial treatment of moderately

severe malaria in African children and adults: a randomised study. Lancet 2004;
363: 1598-605.
artesunate
24

5) Hien TT, Turner GDH, Mai NTH, Phu NH, Bethell D, Blakemore WF, et al.
Neuropathological assessment of artemether-treated severe malaria. Lancet
2003; 362: 395-6.
artemether cell nuclei

artemether quinine 21
6) Taylor-Robinson A. In-vitro model offers insight into the pathophysiology of severe
malaria. Lancet 2004; 363: 1661-2.
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nitric oxide production. Lancet 2003; 361: 676-8.
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artesunate 3
(recrudescence) gametocyte carriage
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Mefloquine resistance in Plasmodium falciparum and increased pfmdr 1 gene copy
number. Lancet 2004; 364: 438-47.
(P. falciparum)



pfmdr 1
encode transport protein
mefloquine pfmdr 1 copy number monitor

MELIOIDOSIS ( 4 1)
White NJ. Melioidosis. Lancet 2003; 361: 1715-22.

Septicaemia 20
ceftazidime
9 20 4 chloramphenicol,
doxycycline trimethoprim-sulpfamethoxazole ( amoxicillinclavulanate ) 10
4
brainstem encephalitis
MUSICOGENIC EPILEPSY ( 20 3)
Kaplan PW. Musicogenic epilepsy and epileptic music. A seizures song. Epilepsy Behavior
2003; 4: 464-73.
review literature 1605 ME reflex epilepsy
2
reticular
formation limbic system
Heschls gyrus

demyelination
head trauma
PULMONARY ALVEOLAR PROTEINOSIS ( 2 2)
Trapnell BC, Whitsett JA, Nakata K. Mechanisms of disease. Pulmonary alveolar proteinosis.
N Engl J Med 2003; 349: 2527-39.

2
lipids alveoli macrophages surfactants

granulocyte-macrophage colony-stimulating factor GM-CSF

autoantibodies GM-CSF pulmonary alveolar
proteinosis autoimmune
TOURETTES SYNDROME ( 19 2)
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Tourette Syndrome Association International Consortium for Genetics.
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obsessive compulsive disorder. Genomics 2003; 82: 1-9.
Tourettes syndrome 1: 2000 .. 1885 Tourette

 Erasmus Rotterdam Netherlands

OCD 2 OCD tics 3 Chromosome
2 C7 C7 CNTNAP2
TS

TS
2) Ojoo JC, Kastelik JA, Morice AH. Case Report. A boy with disabling cough.
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Psychiatry 2003; 74: 602-7.
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Neurology 2004; 62: 1626-7.
TOXOPLASMOSIS ( 3 3)
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Toxoplasma gondii antibody in Thai cats and their owners. Southeast Asian
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TRANSIENT GLOBAL AMNESIA ( 20 2)
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DWI.
Neurology 2004; 62: 2154-5.
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delayed focal MRI changes in the lateral hippocampus in transient global amnesia.
Neurology 2004; 62: 2165-70.
VERTEBRAL ARTERY DISSECTION ( 3 1)
1) Raupp SFA, Jellema K, Sluzewski M, de Kort PLM, Visser LH. Sudden unilateral
deafness
due to a right vertebral artery dissection. Neurology 2004; 62: 1442.
2) Lee H, Yi HA, Baloh HW. Sudden bilateral simultaneous deafness with vertigo as a sole
manifestation of vertebrobasilar insufficiency. J Neurol Neurosurg Psychiatry
2003; 74: 539-41.

3) Smith WS, Johnston SC, Skalabrin EJ, Weaver M, Azari P, Albers GW, et al.
Spinal manipulative therapy is an independent risk factor for vertebral artery
dissection. Neurology 2003; 60: 1424-8.
4) Williams LS, Biller J. Vertebrobasilar dissection and cervical spine manipulation.
A complex pain in the neck. Neurology 2003; 60: 1408-9.
5) Nazir FS, Muir KW. Prolonged interval between vertebral artery dissection and
ischaemic stroke. Neurology 2004; 62: 1646-7.

1
!
33 .

7
5-6
6 .
7 .

T 37.2C
PR 80 regular
BP 120/80
JVP not raised no leg oedema
prominent heaving over L parasternal border, increased S1 at apex and P2
S3 gallop at L parasternal border
no opening snap
grade III diastolic rumbling murmur at apex
clear lung fields
Liver : not palpable

mitral valve
diastole L atrium ventricle
ventricle P2
L parasternal border S1
opening snap (mitral stenosis MS)
myxoma atrium
opening snap (OS) MS OS diastole
apex aortic cusp
ventricle MS (mitral
regurgitation) (aortic regurgitation)

 ( 1) L atrial myxoma

diastolic murmur
murmur atrial myxoma myxoma
atrium ventricle (plop)
OS ( 2)

diastolic murmur echo

cardiogram 3 .
L atrial myxoma 5 x 4 x 4 cm.

Myxoma ( 1 5,000 3 4) ( 5)
83


( 6) atrium
ventricle ( 7)
( 8)
embolic stroke
erythrocyte sedimentation rate
( 9 10)
echocardiography MRI ( 11 12)

myxoma 20 Carney (
13) myxoma
Cushings syndrome Carney complex
chromosome 17
14
15
!
16 Edward Kaplan The deceitful heart Uses of
error The Lancet

14 rheumatic carditis
throat swab culture group A streptococci antistreptolysin O titre
atrial myxoma
!


1) Wood P. Diseases of the Heart and Circulation. Second, revised and enlarged
edition. Eyre and Spottiswoode. London. 1959; p.65 and p.530.
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Vol. 2 Eds. Weatherall DJ, Ledingham JGG, Warrell DA. Oxford Medical Publications.
Oxford. 1996; pp. 2472-4.
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of the literature and report of cases. Intern Med J. 2004; 34: 243-9.
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J Neurol Neurosurg Psychiatry. 2001; 70: 263.
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magnet or echo? Intern Med J. 2004; 34: 210-1.
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myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore).
1985; 64: 270-83.

14) N Engl J Med. Case records of the Massachusetts General Hospital. Case 112002. Eds. Harris NL, et al. 2002; 346: 1152-8.
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424-6.
16) Kaplan EL. The deceitful heart. Lancet. 2002; 359: 834.

2
40
49
.. 2546
2 2547

5
2
6
sciatica

levodopa & carbidopa

2
2
2

5
2 amlodipine 10

Parkinsonism
levodopa

1) Kayser-Fleischer ring
( 1)
2) Postencephalitic Parkinsonism
10-20
(oculogyric crisis)
levodopa
3) Multiple system atrophy (MSA) basal
ganglia, pons, cerebellum autonomic nervous system ( 2 3)
45-60 ( 2)

 orthostatic hypotension

4) Corticobasal degeneration (CBD) 60

( 3)
5) Progressive supranuclear palsy (PSP)

Hallervorden-Spatz Huntington
( 4)

Wt. 83 kgs
Ht. 157 cms
PR 72 regular
BP 140/90
rather immobile, expressionless face with frequent closure of eyelids
Palmomental reflexes ++ ++
no grasp reflex
Fundi
no K-F ring
EOMs : lack of upward and downward gaze
lateral gaze to either side full
no jaw jerk
Limbs : no tremor
moderately increased tone on both sides
SJ
++
++
BJ ++
++
TJ
++
++
KJ
++
++
AJ ++
++
P

Stance : unable to stand without support

()
tone

Tone
3
1) (spasticity) corticospinal
pyramidal tract upper motor neuron



clasp-knife
2

2) cogwheel


cogwheel rigidity basal
ganglia
substantia nigra
(vibration tremor)

3) plastic lead-pipe
basal ganglia (2)
(2)
Plastic lead-pipe rigidity Parkinsonism

Tone (1) (3) deep

tendon reflexes Babinski sign
supranuclear palsy
progressive supranuclear palsy (PSP)
ceruloplasmin 36 mg/dl ( 18-42) MRI PET
scan fluorine-18 L-DOPA L-DOPA uptake caudate nucleus putamen 2
(PD) PSP, MSA CBD
!
1950 PSP Clifford Richardson
Toronto
supranuclear ophthalmoplegia, pseudobulbar palsy, axial dystonia, limb rigidity dementia
cerebellar pyramidal signs ( 5 6)

.. 1904 ( 7)
Richardson .. 1962 7
9 Richardson Olszewski
John Steele
( 8) PSP
PSP SteeleRichardson-Olszewski syndrome
PSP
PSP 45 45
73 ( 55 )

2

blepharospasm
(apraxia of eyelid opening) ( 9 10)
(apraxia of eyelid closure) ( 11)

(anarthria)


3

 2
( 12)
PSP levodopa, dopamine agonist anticholinergic
pramipexole
PSP
MRI scan PSP (cognitive
domain) (executive function) (attention)
voxel based morphometry (VBM) MRI
PSP
( 13 14)
superior cerebellar peduncle MRI
( 15 16)
PSP

( 17)
PSP (age-adjusted prevalence) 6.4
MSA 4.4 PSP 1 15,000
MSA 1 25,000 ( 18) .. 1959
PSP 10 MSA 4-5
100 PSP 2
PSP
PSP
tau

microtubule

neurofibrillary tangles NFTs tauopathies (
19) PSP gene tau Saitohin gene
( 20 21)
PSP PSP
John Steele .. 1970 (
22) 54
John Steele ! John

(
Richardson )
John
2-3
John Guam John
2

1)
1 . .. 2544 47-58.

2) Quinn N. Multiple system atrophy the nature of the beast. J Neurol

Neurosurg Psychiatry. 1989; 52: 78-89.
3) Gibb WRG, Luthert PJ, Marsden CD. Corticobasal degeneration. Brain. 1989;
112: 1171-92.
4)
4 . .. 2547 101-19.
5) Richardson JC, Steele J, Olszewski J. Supranuclear ophthalmoplegia,
pseudobulbar palsy,
nuchal dystonia and dementia. Trans Am Neurol Ass. 1963; 88: 25-7.
6) Richardson JC. Progressive supranuclear palsy ocular, facial and bulbar. J
Neurosurg. 1966; 24: 281-3.
7) Steele JC. Progressive supranuclear palsy. Brain. 1972; 95: 693-704.
8) Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy. Archs
Neurol. 1964; 10: 333-59.
9) N Engl J Med. Case records of the Massachusetts General Hospital. Case 321975. 1975; 293: 346-52.
10) Dehaene I. Apraxia of eyelid opening in Progressive Supranuclear Palsy. Ann
Neurol. 1984; 15: 115-6.
11) Russell RWR. Supranuclear palsy of eyelid closure. Brain. 1980; 103: 71-82.
12) Nath U, Ben-Schlomo Y, Thomson RG, Lees AJ, Burn DJ. Clinical features and
natural history of progressive supranuclear palsy: a clinical cohort sudy. Neurology
2003; 60: 910-6.
13) Brenneis C, Seppi K, Schocke M, Benke T, Wenning GK, Poewe W. Voxel based
morphometry reveals a distinct pattern of frontal atrophy in progressive supranuclear
palsy. J Neurol Neurosurg Psychiatry. 2004; 75: 246-9.
14) Padovani A, Borroni B, Brambati SM, Agosti C, Broli M, Alonso R, et al.
Diffusion tensor imaging and voxel based morphometry study in early progressive
supranuclear palsy. J Neurol Neurosurg Psychiatry. 2006; 77: 457-63.

15) Paviour DC, Price SL, Stevens JM, Lees AJ, Fox NC. Quantitative MRI
measurement of superior cerebellar peduncle in progressive supranuclear palsy.
Neurology. 2005; 64: 675-9.
16) Oba H, Yagishita A, Terada H, Barkovich AJ, Kutomi K, Yamauchi T, et al. New
and reliable MRI diagnosis for progressive supranuclear palsy. Neurology. 2005;
64: 2050-5.
17) Nath U, Thomson R, Wood R, Ben-Schlomo Y, Lees A, Rooney C, et al.
Population based mortality and quality of death certification in progressive
supranuclear palsy (Steele-Richardson-Olszewski syndrome). J Neurol Neurosurg
Psychiatry. 2005; 76: 498-502.
18) Schrag A, Ben-Schlomo Y, Quinn NP. Prevalence of progressive supranuclear
palsy and multiple system atrophy : a cross-sectional study. Lancet. 1999; 354:
1771-5.
19) Golbe LI. Progressive supranuclear palsy in the molecular age. Lancet. 2000;
356: 870-1.
20) de Silva R, Hope A, Pittman A, Weale ME, Morris HR, Wood NW, et al.
Strong association of the Saitohin gene Q7 variant with progressive supranuclear
palsy. Neurology. 2003; 61: 407-9.
21) Pastor P, Ezquerra M, Perez JC, Chakraverty S, Norton J, Racette BA, et al.
Novel haplotypes in 17q21 are associated with progressive supranuclear palsy.
Ann Neurol. 2004; 56: 249-58.
22) Steele JC. Progressive supranuclear palsy. Report of a Thai patient. J Med
Assoc Thai. 1970; 53: 364-9.

3

19 .

1
3 .
1

autoimmune SLE

T 37.6C
PR 90 regular
RR 24
BP 110/70
slightly pale, puffy eyelids and periorbital swelling with slight chemosis of
both eyes
slight pitting oedema of both feet
shallow ulcer at hard palate
no liver stigmata
Abd : generalized distension
shifting dullness +
Liver & Spleen : not palpable
Bowel sounds normal
palpable small axillary lymph node about 2 cms. in diameter in L axilla
hyperpigmented papules and macules at both ears

()

SLE nephrotic syndrome

SLE


Urine : protein trace
(24 hr. urine protein 153 mg.)
Hb 10.4 PCV 30.6 MCV 82.7 MCH 28.2 MCHC 34.1 RBC
6
3.7x10
WBC 7,220 N 66 L 27 M 3 E 4
Platelets 225,000
ESR 62 mm/hr.
FBS 96
BUN 15
Cr 1.0
Cholesterol 207
TP 66.7/alb 24.4
Free T4 1.1 (1.0-2.8) TSH 1.4 g/dl (0.4)
Cortisol 12.5 at 8 am (normal 8-16)
Faeces : yellowish brown, no ova, cyst or parasite
no fat globules
SGOT 45
PT 69
CH50 12%
ANA +ve
peripheral pattern titre 1:64
homogeneous pattern titre > 1:256
anti-dsDNA (immunofluorescent)
+ve titre > 1:2560
anti Sm +ve
anti RNP -ve
Pleural fluid : slightly yellowish, RBC 4400, WBC 43/cu.mm all PMN
total protein 41.4
LDH 92
Pleural & peritoneal fluid : no growth
99mTc albumin scan : increased radiodensity in distal oesophagus,
stomach and colon

SLE pleural effusion

cholesterol
protein-losing enteropathy
(PLE)
PLE
(malabsorption syndrome) albumin
PLE lymphatic obstruction congestive heart
failure constrictive pericarditis Menetrier giant hypertrophic
gastritis PLE SLE 40 ( 1 2)
SLE


( 3)
SLE PLE
50 PLE primary sclerosing cholangitis
( 4)
SLE
4
1) mesenteric vasculitis
infarction PLE
2) chronic active hepatitis sclerosing cholangitis

3) pancreas pancreatitis

4) peritoneum
SLE (peritonitis) 1
( 5)
PLE
chromium-51, technetium-99 human serum albumin I131-PVP
(polyvinylpyrrolidine)
( 6) alpha-1-antitrypsin
( 7)
PLE
prednisolone azathioprine (
8) 16 5 14
11 5 6 14 1

PLE SLE
!

1) Waldmann TA. Protein-losing enteropathy. Gastroenterology 1966; 50: 422-43.

2) Pachas WN, Linscheer WG, Pinals RS. Protein-losing enteropathy in systemic
lupus erythematosus. Am J Gastroenterol 1971; 55: 162-7.
3) Asavatanabodee P, Surangsrirat S, Reungprateep J. Lupus-associated proteinlosing enteropathy: report of a case and literature review. Intern Med J Thai
2002; 18: 262-9.
4) Oh DC, Ng TM, Ho J, Leong KP. Systemic lupus erythematosus with concurrent
protein-losing enteropathy and primary sclerosing cholangitis: a unique association.
Lupus 2006; 15: 102-4.

5) Weinstein PJ, Noyer CM. Rapid onset of massive ascites as the initial
presentation of
systemic lupus erythematosus. Am J Gastroenterol 2000; 95: 302-3.
6) Waldmann TA, Wochner RD, Strober W. The role of the gastrointestinal tract in
plasma protein metabolism. Am J Med 1969; 46: 275-85.
7) Florent C, LHirondel C, Desmazures C, Aymes C, Bernier JJ. Intestinal clearance
of A-1 antitrypsin: a sensitive method for the detection of protein-losing enteropathy.
Gastroenterology 1981; 81: 777-80.
8) Mok CC, Ying KY, Mak A, To CH, Szeto ML. Outcome of protein-losing
gastroenteropathy in systemic lupus erythematosus treated with prednisolone
and azathioprine. Rheumatology (Oxford) 2006; 45: 425-9.

4

41 .

2
2

2

6
false localizing sign

T 37.2C
PR 60
RR 20 BP 110/60
stiff neck present, slightly drowsy, but conscious
Fundi : bilateral disc swelling c flame shaped haemorrhage
venous pulsation +
VAR 20/50 VAL 20/50
lateral rectus paresis
no weakness or definite sensory impairment

()
subacute meningitis
2 cryptococcus neofermans CT
MRI


MRI : no hydrocephalus
slight leptomeningeal enhancement
CSF :
opening pressure 330, clear
cells 80, mononuclear almost 100%
sugar 31 mg%
protein 49 mg%
Blood : HIV -ve

MRI cryptococcal meningitis (CM) (TBM)

TB 3 hydrocephalus protein
CM TBM
CSF
India-ink stain -ve 2 +ve

30 TBM
CM 10 CM Helbok
Neuroepidemiology ( 1) Reimund Helbok
research fellow
114 ..
1993 1999 CM 54 TBM 37
TBM CM CT MRI cerebral infarct
TBM
CM 79 HIV
TBM HIV 3 43

CM HIV ( 2)

.. 1993-1994 .. 1977-1988 CM 10
HIV
HIV India
ink 88 100
CM
TBM TBM
lymphocytes neutrophil CM eosinophil
( 3 4) eosinophil
amphotericin B ( 5) ( 6) CM
HIV 17 36

 18 TBM CM TB cryptococci
India ink 2
!
amphotericin B fluconazole 9

fluconazole
20
Amphotericin B

liposomal amphotericin B
flucytosine 2 fluconazole 8-10
HIV ( 7) HIV

India ink (budding)

HIV

(HAART)
CD4 100 fluconazole ( 8)

HIV fluconazole 400
( 9)
Jeremy Day

( 10)

1) Helbok R, Pongpakdee S, Yenjun S, Dent W, Beer R, Lackner P, et al. Chronic

meningitis in Thailand. Clinical characteristics, laboratory data and outcome in
patients with specific reference to tuberculosis and cryptococcosis.
Neuroepidemiology. 2006;
26: 37-44.
2) Tunlayadechanont S, Viranuvatti K, Phuapradit P, Sathapatayavong B, Tantirittisak
T, Boongird P. Cryptococcal meningitis in patients with non-HIV and HIV infection :
a clinical study. Neurol J Southeast Asia. 1997; 2: 45-50.
3) Anderson P, Macklis J, Brown M, Ory D. Eosinophilic cerebrospinal fluid
pleocytosis and cryptococcal meningitis. Ann Intern Med. 1985; 103: 306-7.
4) Grosse P, Schultz J, Schmierer K. Diagnostic pitfalls in eosinophilic cryptococcal
meningoencephalitis. Lancet Neurol. 2003; 2: 512.

5) Schmutzhard E, Jitpimolmard S, Boongird P, Vejjajiva A. Peripheral eosinophilia

in the course of treatment of cryptococcal meningitis. Mykosen. 1987; 30: 601-4.
6) Moosa MY, Coovadia YM. Cryptococcal meningitis in Durban South Africa :
a comparison of clinical features, laboratory findings, and outcome for human
immunodeficiency virus (HIV)-positive and HIV-negative patients. Clin Infect Dis.
1997; 24: 131-4.
7) Saag MS, Graybill RJ, Larsen RA, Pappes PG, Perfect JR, Powderly WG, et al.
Practice guidelines for the management of cryptococcal disease. Infectious Diseases
Society of America. Clin Infect Dis. 2000; 30: 710-8.
8) Vibhagool A, Sungkanuparph S, Mootsikapun P, Chetchotisakd P,
Tansuphaswasdikul S, Bowonwatanuwong C, et al. Discontinuation of secondary
prophylaxis for cryptococcal meningitis in human immunodeficiency virus-infected
patients treated with highly active antiretroviral therapy : a prospective, multicenter,
randomized study. Clin Infect Dis. 2003; 36: 1329-31.
9) Chetchotisakd P, Sungkanuparph S, Thinkhamrop B, Mootsikapun P, Boonyaprawit
P. A multicentre, randomized, double-blind, placebo-controlled trial of primary
cryptococcal meningitis prophylaxis in HIV-infected patients with severe immune
deficiency. HIV Medicine. 2004; 5: 140-3.
10) Day JN. Cryptococcal meningitis. Pract Neurol. 2004; 4: 274-85.

5
!
19 .

2
6 . 2

1
4-5

T 39.9C

RR 36
PR 120 BP 110/70
pale, discrete brownish maculopapular skin lesions at both cheeks,
shagreen patch like lesion at both buttocks and hypopigmented patches on the arm and
L leg periungual fibroma in both big toes
Eyeground : normal in both eyes
grade 2/6 systolic ejection murmur at L parasternal border no diastolic
murmur
Chest : diminished movements R > L
breath sounds diminished lower zone
Abd : large, slightly tender mass on the extending from the
hypochondrium
to lower quadrant with ill-defined border but not crossing the
midline

()




Tuberous Sclerosis Complex (TSC) MRI

systolic murmur functional

Hb 5.5 PCV 18.4

RBC 3.26 x 106 ( 4.2-54)
MCV 56.5
MCH 16.9
MCHC 30
RDW 27.3 ( 11.5-14.5)
aniso 2+ micro 2+ hypo 2+ platelet clumping 3+ count 235,000
WBC 16,800 N 86 L 8
M6
Hb typing normal
serum Fe 0.9
TIBC 28.1
High Resolution CT lungs : multiple small cystic lesions 2
pulmonary lymphangioleiomyomatoin
(LAM) slight pneumothorax
consolidation middle lobe
CT brain multiple calcified, subependymal nodules lateral ventricles
tuberous sclerosis chordothalamic groove astrocytoma
enhancement 2 calcified cortical tubers 2
MDCT scan abdomen retroperitoneal mass multiple fatty
lesions soft tissue density
angiomyolipoma (AML)

TSC 40C

24 retroperitoneum
hypernephroma lymphoma
pneumothorax
pleurodesis
giant-cell
astrocytoma tuber harmatoma
Tuberous sclerosis 17
AML

33 ( 1)


- 9 ( 2)
Tuberous sclerosis
Bourneville .. 1880
Pringle disease
Pringle Middlesex London
Bourneville-Pringle disease 40
neuro-cutaneous diseases 4
neurofibromatosis, tuberous sclerosis, Sturge-Weber disease syndrome von HippelLindau disease
4
tuberous sclerosis ( 3) TSC
(harmatoma)
2 3
2 TSC1 9q34
hamartin TSC2 16p13.3
tuberin TSC autosomal dominant
( 4)
1 25,000 90 70
95 TSC2 3
4 TSC

( 5)
( 6) pneumothorax LAM ( 7)
Bourneville
Desir-Magloire Bourneville
Charcot ( 8)

Charcot Pierre Marie

Charcot-Marie-Tooth Josef Babinski ! Gilles de la
Tourette - ( 9)
Sigmund Freud !

1) Gojaseni P, Khamapirad T, Muangman V. Renal angiomyolipoma. A report of

two cases with angiographic studies and a note on associated hypertension. J Med
Assoc Thai. 1973; 56: 609-13.
2) Chuahiran S, Visudhiphand P, Vimolchalao M. Tuberous sclerosis : The first
case report in Thailand with a review of various related pathological findings in the
literature. J Med Assoc Thai. 1978; 61: 187-93.

3) Narayanan V. Tuberous sclerosis complex : genetics to pathogenesis. Pediatr

Neurol. 2003; 29: 404-9.
4) Devlin LA, Shepherd CH, Crawford H, Morrison PJ. Tuberous sclerosis complex :
clinical features, diagnosis, and prevalence within Northern Ireland. Dev Med
Child Neurol. 2006; 48: 495-9.
5) Curatolo P, Bombardieri R, Cerminara C. Current management for epilepsy in
tuberous sclerosis complex. Curr Opin Neurol. 2006; 19: 119-23.
6) Goh S, Butler W, Thiele EA. Subependymal giant cell tumors in tuberous
sclerosis complex. Neurology. 2004; 63: 1457-61.
7) Young LR, Almoosa KF, Pollock-Barziv S, Coutinho M, McCormack FX, Sahn SA.
Patient perspectives on management of pneumothorax in lymphangioleiomyomatosis.
Chest. 2006; 129: 1267-73.
8) Pryse-Phillips W. Companion to Clinical Neurology. Little, Brown and Company,
Boston, USA. 1995; pp.113.
9) . 2
2 .. 2547 189-94.

32

1
1
2 1
.

1 3

1) 1
10 Thunderclap headache (TCH) !
primary subarachnoid haemorrhage (SAH) aneurysm vertebral
carotid artery dissection pituitary
(sex or orgasmal headache) (cough headache) primary
TCH ( 1)
2) (1)

(cyst) (ventricle) colloid cyst
third ventricle cysticercosis
spontaneous intracranial hypotension
(SIH)

T 36.6C
RR 20
alert, well oriented
no neck stiffness
Fundi : normal
no abnormal signs

CT scan : no SAH

PR 80

BP 100/60


opening pressure
CSF : clear, colourless, crenated RBC 5,000
protein 55 mg%
glucose 65 mg% (Blood glucose 101)
Wright stain and India ink stain : negative
MDCT scan diffuse mild leptomeningeal
enhancement along cerebral hemispheres cerebellar folia
()

(SIH)
20 venous sinus
dura mater

Harvey Cushing ..
1904 ( 2) SIH
New Daily Persistent Headache (NDPH) ( 3) SIH
dural sac
elastin fibrillin
(Marfan syndrome)



6
3 4

100
MRI
SIH Bahram
Mokri Mayo Clinic (
4)
1 ( 5) SIH

(meningeal biopsy) 6
MRI MRI
MRI ( 7)
( 8)
SIH
theophylline
corticosteroids

 epidural blood patch

(fibrin glue)




Heinrich Irenaeus Quincke
(.. 1842-1922)

angioneurotic oedema Quincke oedema defect C1

esterase inhibitor poikilocytosis pernicious anaemia
Entamoeba histolytica E Coli!

( 9 10)

1) Schwedt TJ, Matharu MS, Dodick DW. Thunderclap headache. Lancet Neurol.
2006; 5: 621-31.
2) Cushing H. The sensory distribution of the fifth cranial nerve. Bull John Hopkins
Hosp. 1904; 15: 213-32.
3) Goadsby PJ, Boes C, Sudlow CLM. Low CSF volume (pressure) headache.
Pract Neurol. 2002, 2: 192-7.
4) Mokri B. Headache caused by decreased intracranial pressure : diagnosis and
management. Curr Opin Neurol. 2003; 16: 319-26.
5) Unnwongse K, Phanthumchinda K. Spontaneous intracranial hypotension : a
case with extensive intraspinal dural enhancement and delayed sixth cranial nerve
paresis. Intern Med J Thai. 2004; 20: 49-55.
6) N Engl J Med. Case records of the Massachusettes General Hospital. Eds.
Scully RE, et al. 1998; 338: 180-8.
7) Schievink WI, Maya MM, Louy C. Cranial MRI predicts outcome of spontaneous
intracranial hypotension. Neurology. 2005; 64: 1282-4.
8) Hfner K, Koch W, Stnder M, Tonn J-C, Tatsch K, Meindl T, et al. Three sites
of high flow CSF leakage in spontaneous intracranial hypotension. Neurology.
2006; 66: 775-6.

9) Firkin BG, Whitworth JA. Dictionary of Medical Eponyms. The Parthenon

Publishing Group, Carnforth, UK. 1987; pp. 428.
10) Pryse-Phillips W. Companion to Clinical Neurology. Little, Brown and Company,
Boston, USA. 1995; pp. 732.

7

59

6
2
3
1 .

polymyalgia rheumatica ( 1)
50
polymyalgia rheumatica
polymyositis

autoimmune disease

T 38C
PR 96 BP 130/80
alert, ill-looking
Skin : no petechiae or purpura
wasting of anterior tibial muscles
Limbs :
Power

deltoids
3/5
wrist extensors
4/5
finger extensors
4/5
hand grip
5/5
hip flexors
3/5
extensors
quadriceps
hamstrings
foot dorsi flexor
3/5
evertors

L
3/5
4/5
4/5
5/5
3/5

3/5
4/5
4/5

3/5
4/5
4/5
5/5

3/5

5/5

DTRs all present

no Babinski response
impaired pain sensation over dorsum of foot up to ankle
()
(proximal myopathy)
DTRs mononeuropathy
autoimmune disease vasculitis arteritis
ESR ANA profile

Hb 10.5
WBC 7,500, normal differential count
PCV 30.7
MCV 73.8
MCHC 26.7
MCHC 33.9
Platelets 286,000 ESR 107 Creactive protein (CRP) 20.6 (normal 0-5)
ANA -ve
CH50 100%
C3 & C4 normal
Coombs test -ve
ANCA +ve p-ANCA > 1:320 (ethanol fixed)
Urine : protein 3+ RBC : numerous WBC 3-5/HPF
kidney biopsy crescentic glomerulonephritis
immunofluorescent study -ve

ESR CRP anti-neutrophil cytoplasmic antibody

(ANCA) crescentic glomerulonephritis
microscopic polyangiitis (MPA)
vasculitis arteritis 3

1 aorta
temporal artery Takayasu ( 2) giant cell arteritis (
1)
2 polyarteritis
nodosa PAN ( 3)
Wegeners granulomatosis
( 4) Churg-Strauss Hench-Schonlein purpura MPA
1 2 3
ANCA
.. 1982 segmental necrotizing glomerulonephritis
ANCA+ p-ANCA myeloperoxidase MPA
crescentic necrotizing glomerulonephritis cANCA

serine proteinase 3 Wegeners granulomatosis

neutrophil elastase lysozyme - p c-ANCA 3
58 propylthiouracil
crescentic proliferative glomerulonephritis p-ANCA titre 1:320
thionamide .. 1993 (
5)

pulse methylprednisolone cyclophosphamide
focal seizure MRI small
cerebral infarct vasculitis
transverse colon
vasculitis cyclophosphamide
Aspergillasis 9

MPA
10
vasculitic neuropathy

hypertrophic
pachymeningitis ( 7)


( 8, 9 10) 30
Takayasu microscopic vasculitis
cranial neuropathy ( 6)
systemic vasculitis
20 1

11

ANCA 12
Lancet 29 2006

1) . 50
3 2 ..
2547 159-74.

2) . ?
3 2
.. 2547 11-20.
3) .
3 2
.. 2547 112-22.
4) .
2 2
.. 2546 109-18.
5) Dolman KM, Gans ROB, Vervaat TJ, Zevenbergen G, Maingay D, Nikkels RE, et
al. Vasculitis and antineutrophil cytoplasmic autoantibodies associated with
propylthiouracil therapy. Lancet 1993; 342: 651-2.
6) Amnueilaph R, Charoenvej P, Vejjajiva A. Pulseless disease presenting with
isolated abducens nerve palsy and recurrent cutaneous angiitis. Br Med J 1973; 3:
27-8.
7) Furukawa Y, Matsumoto Y, Yamada M. Hypertrophic pachymeningitis as an initial
and cardinal manifestation of microscopic polyangiitis. Neurology 2004; 63: 1722-4.
8) Phanthumchinda K, Sinsawaiwong S, Haemachudha T, Yodnophaklao P.
Idiopathic hypertrophic cranial pachymeningitis: an unusual cause of subacute and
chronic headache. Headache 1997; 37: 249-52.
9) Phabphal K, Poungvarin N. Idiopathic hypertrophic cranial pachymeningitis
presenting with Tolosa-Hunt syndrome. A report of two cases. Neurology J Thai
2003; 3: 33-8.
10) Boonyawiroj S, Phanthumchinda K. Extensive anterior cranial fossa idiopathic
hypertrophic pachymeningitis: a case report and review of the literature.
J Med Assoc Thai 2005; 88: 1934-40.
11) Scott D. Primary systemic vasculitis. In: Horizons in Medicine 15. Ed. Weber J.
Royal College of Physicians of London, London. 2003; pp. 183-91.
12) Bosch X, Guilabert A, Font J. Antineutrophil cytoplasmic antibodies. Lancet
2006; 368: 404-18.

8

65

2
medulla

() system disease cerebellar
system, extrapyramidal system pyramidal system
20 subacute combined degeneration spinal cord
combined system disease lateral column (pyramidal tract)
posterior dorsal column postero-lateral sclerosis
pyramidal tract 2

slightly obese, short neck and nasal speech

PR 72
BP 150/90
no jaw jerk
Face
Palate moved normally on phonation
Tongue - no wasting or fasciculation
- moved sideways slowly
Limbs : no muscle wasting or fasciculation
spasticity 2 to 3+
L > R legs > arms
SJ
+++ +++
BJ +++ +++
TJ
+++ +++
KJ
+++ +++
AJ
++
++
P

no sensory impairment

spastic gait
()
spastic quadriparesis spastic
tongue
cervical myelopathy
spondylosis

MRI

(EMG) fibrillation
giant motor unit potentials anterior horn cell

primary lateral sclerosis (PLS)
.. 2546
6

PLS
PLS
amyotrophic lateral sclerosis (ALS) Charcot Joffroy ALS
.. 1869 ALS Charcots disease
(Lou Gehrig) eponym!
ALS 0.6-2.6
( 1 2) .. 1992 Pringle
PLS
23
ALS 96 30
( 3) ALS 40 20
( 4)
PLS
PLS ( 5)
PLS
lower motor neuron (anterior horn cells cranial motor nuclei)
cervical spondylotic myelopathy,
(cranio-vertebral junction),
12 Lathyrism

( 6)
Gordon (
7) PLS 39
lower motor neuron 4

ALS
( 8)
PLS ALS
4 ( 9)

 PLS riluzole ALS

100 PLS
Professor
Swash
ALS PLS
ALS riluzole
PLS

ALS !

1) Chancellor AM, Warlow CP. Adult onset motor neuron disease: worldwide
mortality,
incidence and distribution since 1950. J Neurol Neurosurg Psychiatry.
1992; 55: 1106-15.
2) Swash M. Clinical features and diagnosis of amyotrophic lateral sclerosis.
In: Amyotrophic Lateral Sclerosis. Eds. Brown Jr RH, Meininger V, Swash M.
Martin Dunitz, London. 2000; pp. 3-30.
3) Vejjajiva A, Foster JB, Miller H. Motor neuron disease. A clinical study. J
Neurol Sci 1967; 4: 299-314.
4) Vejjajiva A. Motor neuron disease a short analysis of 40 patients from
Thailand. In: Amyotrophic lateral sclerosis in Asia and Oceania. Eds.Chen KY, Yase Y.
National Taiwan University, Taipei. 1984; pp. 125-30.
5) Swash M, Desai J, Misra VP. What is primary lateral sclerosis? J Neurol Sci
1999; 170: 5-10.
6) Spencer PS, Allen CN, Kisby GE, Ludolph AC, Ross SM, Roy DN. Lathyrism and
western Pacific amyotrophic lateral sclerosis: etiology of short and long latency
motor system disorders. In: Amyotrophic lateral sclerosis and other motor neuron
diseases. Ed. Rowland LP. Advances in Neurology. Vol. 56, New York,
Raven Press. 1991; pp. 287-310.
7) Gordon PH, Cheng B, Katz IB, Pinto M, Hays AP, Mitsumoto H, et al. The
natural history of primary lateral sclerosis Neurology 2006; 66: 647-53.
8) Rosenfeld J, Swash M. Whats in a name? Lumping or splitting ALS, PLS,
PMA, and the other motor neuron diseases? Neurology 2006; 66: 624-5.
9) .
1 2 ..
2545 11-21.
3

9

23

2 12
2

2
.

T 37.3C
PR 88
BP 130/70
Legs : arterial pulses all present
very red, shiny skin of both feet - slight swelling of both legs up to
ankle
Tone
DTRs 2+ symmetrical
P
no sensory impairment

2

erythromelalgia

MRI mild atrophic spinal
cord epidural analgesia
chronic regional pain syndrome
gabapentin carbamazepine
autonomic dysfunction
causalgia lumbar sympathectomy
2 lumbar sympathetic block
lumbar sympathectomy ( )
2

 Carbamazepine
800
Erythromelalgia
2

erythermalgia ( 1)
small-fibre neuropathy
( 2 3)
causalgia complex regional pain syndrome (CRPS) 2
CRPS I II
CRPS I reflex sympathetic dystrophy

CRPS II causalgia
( 4) sympathetic
catecholamine
norepinephrine
sympathectomy

( 5)
erythromelalgia Weir
Mitchells disease Primary erythermalgia autosomal dominant
warm stimuli
sodium channel (channelopathy) gene encode Na+ channel (Nav 1.7)
dorsal root ganglion sympathetic
ganglion
( 6) primary erythromelalgia
gene encode ion channel !
12
primary erythromelalgia gene
sodium channel Stephen Waxman
15 gene gene erythromelalgia
erythermalgia! ( 7)

1) Smith LA, Allen EV. Erythermalgia (erythromelalgia) of extremities : a syndrome

characterized by redness, heat and pain. Am Heart J 1938; 16: 175-88.
2) Davis MDP, Sandroni P, Rooke TW, Low PA. Erythromelalgia : vasculopathy,
neuropathy or both? A prospective study of vascular and neurophysiologic
studies in erythromelalgia. Arch Dermatol 2003; 139: 1337-43.
3) Cohen JS. Erythromelalgia : New theories and new therapies. J Am Acad
Dermatol 2000; 43: 841-7.

4) Jnig W, Baron R. Complex regional pain syndrome : mystery explained?

Lancet Neurol 2003; 2: 687-97.
5) Kemler MA, DeVet HCW, Berendse GAM, Van Den Wildenberg FA, Van Kleef M.
The effect of spinal cord stimulation in patients with chronic reflex sympathetic
dystrophy : Two years follow-up of the randomized control trial. Ann Neurol
2004; 55: 13-8.
6) Waxman SG, Dib-Hajj SD. Erythromelalgia : A hereditary pain syndrome enters
the molecular era. Ann Neurol 2005; 57: 785-8.
7) Han C, Rush AM, Dib-Hajj SD, Li S, Xu Z, Wang Y, Waxman SG. Sporadic onset
of erythermalgia: A gain-of-function mutation in Nav 1.7. Ann Neurol 2006;
59: 553-8.

10

40

2 1
4 .
2
6 2
2
6 .

10

2 peripheral neuropathy
C fibre
A-fibre symmetrical
sensori-motor polyneuropathy reflex joint position
sense vibration sense

thin looking, dark skin complexion

PR 72
BP 120/80
Abd : Liver span 12 cms.
Spleen : just palpable
CNS : wasting of calf muscles c weakness of DF & PF
no fasciculation
absent DTRs in all limbs
JPS (& VS) impaired in both big toes
decreased pain sensation in both feet up to ankles
()

symmetrical
sensori-motor
polyneuropathy 2
3 3-4

1)
paraneoplastic syndrome bronchogenic
carcinoma
2) systemic amyloidosis 50
autonomic neuropathy

3) POEMS paraneosplastic syndrome

serum protein electrophoresis bone survey osteosclerotic myeloma

Hb 15
Hct 45.3
WBC 17600
N 78
L 13
MCV 87.7
MCHC 33
ESR 45
Urine : no protein, Bence-Jones protein absent
Blood : Cr 0.9
Ca++ 9.2
Alb 30.6 (normal 42-52), TP 80.3 (normal 66-84)
Protein electrophoresis : M protein + (IgG monoclonal gammopathy)
serum testosterone 0.7 ng/ml (normal 2.6-12.1)
cortisol 9.2 g/dl (8-16)
Free T4 0.9 (0.7-1.7 ng/dl)
T3 50 (90-250)
TSH 8.9 (0-4)
thyroid autoantibodies -ve
LH 4.2 mIu/ml (5-10)
FSH 3.3 (4-25)
Prolactin 16.4 ng/ml (up to 25)
Bone marrow aspiration : plasmacytosis 5-10%
Bone survey : no osteosclerotic lesion

M3

POEMS syndrome M protein

POEMS acronyms P
Polyneuropathy O Organomegaly E = Endocrinopathy M = M protein
S = skin changes
Bardwick POEMS ..
1980 ( 1) Crow-Fukase syndrome .. 1956 RS Crow
Bristol 2 peripheral neuritis multiple
myeloma 54 67 Crow
( 2)
.. 1969 Fukase ( 3) 1 solitary
plasmacytoma polyneuropathy

100 Nakanishi 1984 (
4) multiple sclerosis
2

 Tokyo Fukuoka POEMS

50 polyneuropathy
papilloedema 2
.... .. 1984 (
5) 3-4

POEMS

P - Polyneuropathy polyneuropathy symmetrical
sensori-motor chronic inflammatory demyelinating polyneuropathy
CIDP sensory
proprioception vibration pain temperature
autonomic

Papilloedema 2
venous sinus thrombosis
( 6)
O - organomegaly 50
Castleman
E - endocrinopathy POEMS
gynaecomastia

M - monoclonal gammopathy (M-spike) M-protein 80
osteosclerotic myeloma lambda light chain IgA IgG
S - skin changes - (hypertrichosis)
(hyperpigmentation), finger clubbing
glomeruloid haemangiomas POEMS
44 (
7)
POEMS
necrotizing vasculitis ( 8), proliferative vasculopathy
( 9),
(inflammatory myopathy) 10 pulmonary hypertension (
11)
POEMS vascular endothelial growth
factor (VEGF) ( 12 13) prednisolone
mycophenolate mofetil thyroxine
testosterone ( 14
15)
16


1) Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL.
Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy,
M protein and skin changes : the POEMS syndrome. Report of two cases
and a review of the literature. Medicine (Baltimore) 1980; 559: 311-22.
2) Crow RS. Peripheral neuritis in myelomatosis. Br Med J 1956; 2: 802-4.
3) Fukase M, Kakimatsu T, Nishitani H, et al. Report of a case of solitary
plasmacytoma
in the abdomen presenting with polyneuropathy and endocrinological disorders.
Clin Neurol 1969; 9: 657.
4) Nakanishi T, Sobue I, Toyokura Y, Nishitani H, Kuroiwa Y, Satoyoshi E, et al.
The Crow-Fukase syndrome. A study of 102 cases in Japan. Neurology
1984; 34: 712-20.
5) Witoonpanich R, Jootar S, Vejjajiva A, Chuahirun S, Nitiyanant P. The POEMS
(Crow-Fukase) syndrome : a case report. J Med Assoc Thai 1988; 71: 406-11.
6) Witoonpanich R, Phankian S, Jootar S, Poonyathalang A, Worapongpaiboon S,
Phudhichareonrat S, et al. POEMS syndrome with venous sinus thrombosis
and visual failure : a case report. J Med Assoc Thai 2005; 88: 690-4.
7) Weimer T, Norton A, Gutmann L. Glomeruloid hemangiomas : A marker for
POEMS. Neurology 2006; 66: 453-4.
8) Shibata M, Yamada T, Tanahashi N, Koto A, Kuramochi S, Fukushima S, et al.
POEMS syndrome with necrotizing vasculitis : A novel feature of vascular
abnormalities. Neurology 2000; 54: 772-3.
9) Intragumtornchai T, Phanthumchinda K, Lerdlum S, Sumpathanukul P,
Sakulramrung R. POEMS syndrome : a case with proliferative vasculopathy and a
review of cases in Thailand. J Med Assoc Thai 1993; 76: 585-90.
10) Goebels N, Walther EU, Schaller M, Pongratz D, Mueller-Felber W. Inflammatory
myopathy in POEMS syndrome. Neurology 2000; 55: 1413-4.
11) Mukerjee D, Kingdon E, Vanderpump M, Coghlan JG. Pathophysiological insights
from a case of reversible pulmonary arterial hypertension. J R Soc Med 2003;
96: 403-4.
4

12) Watanabe O, Arimura K, Kitajima I, Osame M, Maruyama I. Greatly raised

vascular endothelial growth factor (VEGF) in POEMS syndrome. Lancet 1996; 347:
702.
13) Dyck PJ, Engelstad JN, Dispenzieri A. Vascular endothelial growth factor and
POEMS. Neurology 2006; 66: 10-12.
14) Hogan WJ, Lacy MQ, Wiseman GA, Fealey RD, Dispenzieri A, Gertz MA.
Successful treatment of POEMS syndrome with autologous hematopoietic
progenitor cell transplantation. Bone Marrow Transplantation. 2001; 28: 305-9.
15) Dispenzieri A. POEMS syndrome. Hematology (Am Soc Hematol Educ Program)
2005; 360-7.
16) Caswell R, Warner T, Mehta A, Ginsberg L. Neurological rarity. POEMS
syndrome. Practical Neurology 2006; 6: 111-6.

11

38
21 2545

8 .
2
6

1 2

50
8


endometriosis
catamenial haemoptysis

Wt. 51.2 kgs.

Ht. 156 cms.
PR 80 BP 130/80
CVS & Chest: no abnormality

T 37.2C

()
x ray & MRI
CBC bleeding, clotting time coagulation factor

Hb 10.4
PCV 32
MCV 77.2
MCH 25
6
RBC 4.15 x 10
RDW 15.7
Platelets 267,000
WBC 7630 N 67 L 20 M 3 E 10
PTT 40 PT 14.1 INR 1.25
anti-HIV -ve
Stool exam: no ova, cyst or parasite
CXR normal

MCHC 32.4

High Resolution CT thorax: small RLL lesion

Bronchoscopy: no abnormality

endometriosis Danazol (200 mg) 1

- 6 2
Danazol 1
15

(catamenial epilepsy) Sir William Gowers
.. 1885 ( 1)
recurrent pneumothorax 100 ( 2),
pneumomediastinum, haemothorax catamenial haemoptysis
( 3, 4, 5) 35
( 19-54 ) 2
CT
danazol
ethisterone pituitary-gonadal axis pituitary
gonadotrophins endometrium danazol
Endometriosis

( 6) Alcocks canal pudendal
(perineum) (vulvodynia)
( 7)
endometriosis

( 8) endometriosis
metastasis
9

endometriosis

1) Rodriguez Macias KA. Catamenial epilepsy. Gynecological and hormonal

implications. Five case reports. Gynaecol Endocrinol 1996; 10: 139-42.

2) Shiraishi T. Catamenial pneumothorax: report of a case and a review of the

Japanese and non-Japanese literature. Thorac Cardiovasc Surg 1991; 219: 1328-32.
3) Weber F. Catamenial hemoptysis. Ann Thorac Surg 2001; 72: 1750-1.
4) Yu Z, Fleischman JK, Rahman HM, Mesia AF, Rosner F. Catamenial hemoptysis
and pulmonary endometriosis. A case report. Mt Sinai J Med 2002; 69: 261-3.
5) Yisa SB, Okenwa AA, Husemeyer RP. Treatment of endometriotic catamenial
haemoptysis with etonogestrel subdermal implant. Br J Obstet Gynaecol 2004;
111: 385-6.
6) N Engl J Med. Case Records of the Massachusetts General Hospital. Case 51987. 1987; 316: 261-8.
7) Nehme-Schuster H, Youssef C, Roy C, Brettes J-P, Martin T, Pasquali J-L, et al.
Alcocks canal syndrome revealing endometriosis. Lancet. 2005; 366: 1238.
8) Nap AW, Groothuis PG, Demir AY, Evers JL, Dunselman GA. Pathogenesis of
endometriosis. Best Pract Res Clin Obstet Gynaecol 2004; 18: 233-44.
9) Giudice L, Kao LC. Endometriosis. Lancet 2004; 364: 1789-99.

12

43 . .

4
. 20
1-2 2 disulfiram
4

1 3

24
catecholamine

delirium tremens
disulfiram
aldehyde dehydrogenase ethyl alcohol
acetaldehyde /

T 37.8C
PR 80
RR 20
BP 150/70
drowsy, confused but followed simple command unsteady on standing,
no jaundice, no neck stiffness
Fundi : normal
Pupils
EOMs :
100

100

80

80

100

80

80

100

bilateral horizontal nystagmus on lateral gaze + vertical nystagmus on upward

gaze
Limbs : no tremor, ataxic gait
DTRs + +
no Babinski sign

Hb 11.5
Hct 37
WBC 7100
N 72
E 1 B 1 L 26
Urinalysis, Chest x ray ECG
MRI : hypodense lesion at temporal lobe L occipital lobe
generalized brain atrophy, no subdural haematoma
()


(nystagmus)
2 abducens
nerve cranial nerve 6 2 ataxia
Wernickes encephalopathy

Wernickes encephalopathy thiamine

100 Risperidal 1 mg 48
2

Wernickes encephalopathy (WE) thiamine 1

Karl Wernicke (.. 1848-1905) 3
.. 1841 2
pyloric stenosis Wernicke acute superior haemorrhagic
polioencephalitis .. 1936 Sir Rudolf Peters
1
Wernickes encephalopathy B1 ( 1, 2 3)
symmetrical polyneuropathy 50
acute beri beri 1
carbohydrate thiaminase 1
1
2

 acute beri beri

thiamine 30 (
4) 1
( 5)
40
WE
acute beri beri Denny-Brown

2 beri beri
WE
transketolase protein ( 6) WE
1
1 acute beri beri WE

( 7, 8 9) (hyperemesis gravidarum)
( 10) ( 11)
HIV 12 13
WE

(nystagmus)
rotatory nystagmus
latral rectus palsy 2 ptosis internuclear ophthalmoplegia

Ataxia WE superior cerebellar vermis vestibular

nuclei trunk ataxia
Finger-nose heel-shin tests dysarthria
Deep tendon reflexes polyneuropathy

WE
(retrograde + anterograde)
confabulation Korsakoff psychosis
Sergei Sergeivich Korsakoff (.. 1854-1900)

amnestic syndrome .. 1887-1891

1
Korsakoff cerebropathia psychica toxemica Friedrich Jolly
Korsakoffs syndrome (
14) Maurice Victor Raymond Adams William
Collins
1950-1969 Wernicke-Korsakoff

( 1) ( 15)
Korsakoff syndrome 44 75 WE
1 WE
Wernicke-Korsakoff
transketolase

1
MRI 16,
17, 18, 19 20 MRI MRI

WE 1 MRI ( 21)
Karl Wernicke
aphasia ( 22) Wernickes aphasia
fluent sensory aphasia Brocas motor non-fluent aphasia

1) Victor M, Adams RD, Collins GH. The Wernicke-Korsakoff Syndrome and Related
Neurologic Disorders Due to Alcoholism and Malnutrition. Second Edition.
Contemporary Neurology Series 30. FA Davis Co. Philadelphia. 1989; 231 pp.
2) Pryse-Phillips W. Companion to Clinical Neurology. Little Brown and Company.
Boston. 1995; pp. 909-10.
3) Peters RA. The biochemical lesion in vitamin B1 deficiency. Lancet 1936; i:
1161-5.
4) Vimokesant S, Kunjara S, Rungruangsak K. Beriberi caused by antithiamin factors
in food and its prevention. In : Thiamin: Twenty years of progress. Eds. Sable
HZ and Gluber CJ. Ann NY Acad Sci 1982; 378: 123-36.
5) Vimokesant SL, Nakornchai S, Dhanamitta S, Hilker DM. Effect of tea
consumption on thiamin status in man. Nutr Rep Int 1974; 9: 371-6.
6) Heap LC, Pratt OE, Ward RJ, Waller S, Thomson AD, Shaw GK, et al. Individual
susceptibility to Wernicke-Korsakoff syndrome and alcoholism-induced cognitive
deficit : impaired thiamine utilization found in alcoholic and alcohol abusers.
Psych Genetics 2002; 12: 217-24.
7) Nadel AM, Burger PC. Wernicke encephalopathy following prolonged intravenous
therapy. JAMA 1976; 235: 2403-5.

8) Vortmeyer AO, Hagel C, Laas R. Haemorrhagic thiamine deficient

encephalopathy following prolonged parenteral nutrition. J Neurol Neurosurg
Psychiatry 1992; 55: 826-9.
9) Ogershok PR, Rahman A, Nestor S, Bricke J. Wernicke encephalopathy in
nonalcoholic patients. Am J Med Sci 2002; 323: 107-11.
10) Hillboom M, Pyhtinen J, Pylvnen V, Sotaniemi K. Case report. Pregnant,
vomiting and coma. Lancet 1999; 353: 1584.
11) Jagadha V, Dock JHN, Halliday WC, Smyth HS. Wernickes encephalopathy in
patients on peritoneal dialysis or hemodialysis. Ann Neurol 1987; 21: 78-84.
12) Alcaide ML, Jayaweera D, Espinoza L, Kolber M. Wernickes encephalopathy in
AIDS : a preventable cause of fatal neurological deficit. Int J STD AIDS 2003; 14:
712-3.
13) Tattevin P, Sauala F, Revest M, Taussig D, Michelet C. Confusion, memory
disorders and ophthalmoparesis in a patient with AIDS. Lancet 2006; 367: 368.
14) Blansjaar BA. Korsakoffs syndrome. In : Neurological Eponyms. Eds. Koehler
PJ, Bruyn GW, Pearce JMS. Oxford University Press, Oxford. 2000; pp. 234-8.
15) Blansjaar BA, Van Dijk JG. Korsakorff minus Wernicke syndrome. Alcohol
Alcohol 1992; 27: 435-7.
16) Kashihara K, Irisawa M. Neurological Picture. Diffusion weighted magnetic
resonance imaging in a case of acute Wernickes encephalopathy. J Neurol
Neurosurg Psychiatry 2002; 73: 181.
17) Doherty MJ, Waston NF, Uchino K, Hallam DK, Cramer SC. Diffusion
abnormalities in patients with Wernicke encephalopathy. Neurology 2002; 58: 6557.
18) Ploner M, Schnitzler A. Clinical picture. Wernickes encephalopathy. Lancet
2003; 361: 1000.
19) Koontz DK, Filho JAF, Sagar SM, Rucker JC. NeuroImages. Wernicke
encephalopathy. Neurology 2004; 63: 394.
20) Kaineg B, Hudgins PA. Images in clinical medicine. Wernickes encephalopathy.
N Engl J Med 2005; 352: e18.

21) Chotmongkol V, Limpawattana P. Wernickes encephalopathy : report of a case.

J Med Assoc Thai 2005; 88: 855-8.
22) Dalman AJE, Eling P. Wernickes aphasia. In : Neurological Eponyms. Eds.
Koehler PJ, Bruyn GW, Pearce JMS. Oxford University Press. Oxford. 2000; pp.
244-9.

13
50
55 .

7
7

temporal parietal



2
chronic subdural haematoma, frontal
meningioma normal pressure hydrocephalus
neurosyphilis, hypothyroidism 12

alert, cooperative, well oriented in time, place and person

100-7 : 93,
Memory : recall 2/3
recent
remote
Abstract thinking :

Judgement

-
-
-
-
-
-

MMSE 17
VAR 20/30
VAL 20/30
Fundi & Pupils : normal
Face & Tongue
Limbs :
SJ
+++ +++
BJ +++ +++
TJ
+++ +++
KJ
+++ +++
AJ ++
++
P

no abnormal movements
()
VDRL FTA-AbS anti HIV
MRI

Blood : anti-HIV non-reactive
VDRL +ve 1:32
FTA-AbS and TPHA : reactive
CSF cells : 15 mononuclear cells, RBC 20
2

protein 115
sugar 68 / blood sugar 148
VDRL -ve, TPHA : reactive, FTA IgG +ve
MRI brain : multiple non-specific ischaemic foci

HIV

25
( 1 2)

12.24 procaine penicillin 2.4

probenecid 2
20


( 3 16)

1) Polnikorn N, Witoonpanich R, Vorachit M, Vejjajiva S, Vejjajiva A. Penicillin

concentrations in cerebrospinal fluid after different treatment regimens for syphilis.
Br J Vener Dis. 1980; 56: 363-7
2) Polnikorn N, Witoonpanich R, Vorachit M, Vejjajiva S, Vejjajiva A. Penicillin
concentrations in the cerebrospinal fluid after benzathine penicillin and probenecid
in the treatment of syphilis. Br J Vener Dis. 1982; 58: 342
3)
2500 2513; 53: 117-23.
4) :
.. 2527 150-9.
5) :
.. 2532 153-62.

6) Vejjajiva A, Vejjajiva S, Polnikorn N, Rajsiri N, Bunyaratavej P. Cerebrospinal

fluid immunoglobulins : Their diagnostic role in neurosyphilis. Asian Pacific J Allerg
Immun. 1983; 1: 140-2.
7) Polnikorn N, Rajsiri N, Luptikultum S, Vejjajiva S, Vejjajiva A. Treponemal tests
and immunoglobulins in the cerebrospinal fluid of patients with secondary syphilis.
J Med Assoc Thai. 1983; 66: 604-10.
8) Golden MR, Marra CM, Holmes KK. Update on syphilis : resurgence of an old
problem. JAMA. 2003; 290: 1510-4.
9) Carr J. Neurosyphilis. Pract Neurol. 2003; 3: 328-41.
10) Marra CM, Maxwell CL, Smith SL, Lukehart SA, Rompalo AM, Eaton M, et al.
Cerebrospinal fluid abnormalities in patients with syphilis : association with clinical
and laboratory features. J Infect Dis. 2004; 189: 369-76.
11) Marra CM, Tantalo LC, Maxwell CL, Dougherty K, Wood B. Alternative
cerebrospinal fluid tests to diagnose neurosyphilis in HIV - infected individuals.
Neurology. 2004; 63: 85-8.
12) Lukehart SA, Godornes C, Molini BJ, Sonnett P, Hopkins S, Mulcahy F, et al.
Macrolide resistance in Treponema pallidum in the United States and Ireland.
N Engl J Med. 2004; 351: 154-8.
13) Moyer P. Neurosyphilis : Not gone but often forgotten. Neurology Today.
2004; 4: 80-2.
14) Timmermans M, Carr J. Neurosyphilis in the modern era. J Neurol Neurosurg
Psychiatry. 2004; 75: 1727-30.
15) Lair L, Naidech AM. Modern neuropsychiatric presentation of neurosyphilis.
Neurology. 2004; 63: 1331-3.
16) Odonnell JA, Emery CL. Neurosyphilis : a current review. Curr Infect Dis
Rep. 2005; 7: 277-84.

14

53 .

2 1 .

2 .
Paracetamol norfloxacin, domperidone

.

20 1 .

haemoglobinuria myoglobinuria
bilirubin (haematuria)

haemoglobinuria intravascular haemolysis
haematuria
intravascular haemolysis glucose-6phosphate dehydrogenase (G-6PD)

Hb

Wt. 51 kgs. T 38C

marked jaundice, pale

PR 92 regular RR 20

BP 130/60

Heart: grade 1/6 ejection systolic murmur at right upper sternal border
Abdomen: no mass felt
slight tenderness over hypochondrium

Urine: brownish black
protein 4+
RBC 0-1/HPF
Hb 5 RBC 2.79x106 WBC 18,500 N 78 L 11 M 4 B 1 MPV
9.54
spherocyte 1+
polychromasia 1+
Band 5
nucleated RBC 4
Platelets 313,000 no malaria parasite
Heinz body 15%
TB 11.5
DB 0.6 SGOT 178 PT 40 AP 67 Chol 198
FBG 117 BUN 25 Cr 0.7 Na+ 138 K+ 4.04 Cl- 100 CO2 22
Coombes test -ve
HBsAg ve
anti HBs +ve
anti HBcIgM ve
anti HAV +
anti HAVIgM ve
anti HCV ve
Methaemoglobin Reduction test: Met Hb 3.25
Hb 5.0
Meth. Reduction = 65% (Normal 0 5%)
()
TB
SGOT acute intravascular haemolytic anaemia Heinz bodies
Heinz bodies cell inclusions
methaemoglobin reduction
G6PD 2
IV fluid 0.45% normal saline solution 3,000 ml. 7.5%
NaHCO3 50 ml. 24
G6PDD
400
( 1)
X q28 recessive
homozygote gene
carrier G6PD G6PD
Mahidol ()
163 serine glycine
( 2)

 G6PD Mahidol (
3)
(RBC)

glycolysis
nicotinamide adenine dinucleotide phosphate (NADP) NADPH NADPH

cell membrane

reduced Hb
oxidized MetHb NADPH
G6PD G6PD intravascular
haemolysis


sulfa,
thiazide, sulphonylurea tolbutamide,
phenacetin, quinidine, primaquine
nitrofurantoin nitrate
(fava beans) sodium nitrite
acute haemolysis
G6PD

G6PD

G6PD

G6PD
ketosis gene ( 4)

( 5) clinical
problem-solving G6PD

1) Luzzatto L. Glucose 6-phosphate dehydrogenase (G6PD) deficiency. In:

Oxford textbook of Medicine. Third Edition. Vol. 3. Eds: Weatherall DJ,
Ledingham JGG, Warrell DA. Oxford Medical Publications, Oxford. 1996; pp. 353741.
2) Vulliamy TJ, Wanachiwanawin W, Mason PJ, Luzzatto L. G6PD Mahidol, a
common deficient variant in South East Asia is caused by a (163) glycine-serine
mutation. Nucleic Acids Res 1989; 17: 5868.
3) Iwai K, Hirono A, Matsuoka H, Kawamoto F, Horie T, Lin K, et al. Distribution
of glucose-6-phosphate dehydrogenase mutations in Southeast Asia. Hum Genet
2001; 108: 445-9.

4) Sobngwi E, Gautier J-F, Kevorkian J-P, Villette J-M, Riveline J-P, Zhang S, et al.
High prevalence of glucose-6-phosphate dehydrogenase deficiency without
gene mutation suggests a novel genetic mechanism predisposing to ketosis-prone
diabetes. J Clin Endoc Metab 2005; 90: 4446-51.
5) Janssen WJ, Dhaliwal G, Collard HR, Saint S. Clinical Problem-solving. Why
why matters. N Engl J Med 2004; 351: 2429-34.

15

55 .
29 (27 2519) 26

1
25

7
2

alert & intelligent

speech: normal
EOMs full, no nystagmus
Limbs: myoclonic jerking of arms when elevated
Finger to nose test impaired, interrupted by myoclonic jerking of arms.
Heel-shin test: similarly impaired by sudden jerking of legs.
Tone
DTRs + +
P

Gait: unsteady, broad-based, interrupted by jerking

action myoclonus cerebral anoxia

Lance-Adams syndrome
James Lance

Raymond Adams ( 1) Lance
Raymond Adams
myoclonus

Lance Adams syndrome 40
2 60
scoline apnoea

10

Lance-Adams syndrome
myoclonus
action myoclonus
Lance Adams Lhermitte
Marsden action myoclonus
cerebellum
5-hydroxytryptophan
( 2 3) Lance-Adams syndrome
2 Parkinsonism
!

diazepam,
phenobarbital,
carbamazepine haloperidol 1 5 hydroxytryptophan
clonazepam (
4 5) sodium valproate ( 6)
clonazepam

2-3 55
clonazepam 6 valproate 1200
Lance-Adams syndrome


( 7, 8 9)
cerebellum inferior olive (medulla)
impulses spino-olivary tract pallidum,
midbrain thalamus central tegmental tract impulses inferior
olive cerebellar vermis cerebellar hemispheres (Purkinje cells)
inferior cerebellar peduncle
Lance-Adams syndrome serotonin 5hydroxytryptamine inferior olive serotonin
action myoclonus serotonin inferior olive
olivocerebellar rhymicity ( 10)
Purkinje cells cerebellum
aldolase C EAAT4 ( 11)

posthypoxic action myoclonus valproic acid clonazepam

piracetam ( 12)

Action myoclonus heatstroke (
8) coeliac disease gluten sensitivity ( 13)

( 14)
posthypoxic myoclonus
(
15 18)


1) Lance JW, Adams RD. The syndrome of intention or action myoclonus as a
sequel to hypoxic encephalopathy. Brain. 1963; 86: 111-36.
2) Lhermitte P, Peterfalvi M, Marteau R, Gazengel J, Serdaru M. Analyse
pharmacologique d'un cas de myoclonies d'intention et d'action post-anoxiques. Rev
Neurol. 1971; 124: 21-31.
3) Chadwick D, Reynolds EH, Marsden CD. Relief of action myoclonus by
5 hydroxytryptophan. Lancet. 1974; ii: 111-2.
4) Van Woert MH, Sethy VH. Therapy of intention myoclonus with 1-5
hydroxytryptophan and a peripheral decarboxylase inhibitor MK486. Neurology.
1975; 25; 135-40.
5) Chadwick D, Hallett M, Harris R, Jenner P, Reynolds EH, Marsden CD. Clinical,
biochemical and physiological factors distinguishing myoclonus responsive to
5-hydroxytryptophan, tryptophan plus a monoamine oxidase inhibitor and clonazepam.
Brain. 1977; 100: 455-87.
6) Fahn S. Posthypoxic action myoclonus: review of the literature and report of
two cases with response to valproate and estrogen. Adv Neurol. 1979; 26: 4984.
7) Richardson JC, Rewcastle NB, De Lan J. Hypoxic myoclonus: clinical and
pathological observations. In Physiological Aspects of Clinical Neurology. Ed. F.
Clifford Rose. Blackwell Scientific Publications. Oxford. 1977; pp. 231-45.
8) Marsden CD, Hallett M, Fahn S. The nosology and pathophysiology of
myoclonus. In Neurology 2 Movement Disorders. Eds. Marsden CD, Fahn S.
Butterworth Scientific. London. 1982; pp. 196-248.
9) Fahn S. Posthypoxic action myoclonus: literature review update. Adv Neurol.
1986; 43: 157-69.
10) Welsh JP, Placantonakis DG, Warsetsky SI, Marquez RG, Bernstein L, Aicher SA.
The serotonin hypothesis of myoclonus from the perspective of neuronal rhythmicity.
Adv Neurol. 2002; 89: 307-29.

11) Welsh JP, Yuen G, Placantonakis DG, Vu TQ, Haiss F, OHearn E, et al. Why do
Purkinje cells die so easily after global brain ischemia? Aldolase C, EAAT4 and
the cerebellar contribution to posthypoxic myoclonus. Adv Neurol. 2002;
89: 331-59.
12) Agarwal P, Frucht SJ. Myoclonus. Curr Opin Neurol. 2003; 16: 515-21.
13) Lu CS, Thompson PD, Quinn NP, Parkes PD, Marsden CD. Ramsay Hunt
syndrome and coeliac disease: a new association. Mov Disord. 1993; 8: 252.
14) Werhahn KJ, Brown P, Thompson PD, Marsden CD. The clinical features and
prognosis of chronic posthypoxic myoclonus. Mov Disord. 1997; 12: 216-20.
15) Frucht SJ. The clinical challenge of posthypoxic myoclonus. Adv Neurol. 2002;
89: 85-8.
16) Jundt F, Lempert T, Dorken B, Pezzutto A. Trimethoprim-sulfamethoxazole
exacerbates posthypoxic action myoclonus in a patient with suspicion of
Pneumocystis jiroveci infection. Infection. 2004; 32: 176-8.
17) Frucht SJ, Trost M, Ma Y, Eidelberg D. The metabolic topography of
posthypoxic myoclonus. Neurology. 2004; 62: 1879-81.
18) Frucht SJ, Bordelon Y, Houghton WH. Marked amelioration of alcohol-responsive
posthypoxic myoclonus by gammahydroxybutyric acid (Xyrem). Mov Disord.
2005; 20: 745-51.

16

63

4
6 . 2

2 2
4-5

congestive cardiac
failure 2 sensory polyneuropathy

Wt. 60.5 kgs. Ht. 155 cms. T. 37.2C RR 40

PR 60 regular B.P.
170/90
pitting oedema of legs 2+
CVS: apex beat 6th ICS outside MCL
no heaving no thrill
Heart sounds S3 gallop no murmur
Lungs: crepitations both sides L > R
Abd: Liver enlarged 10 cms. below right costal margin. Spleen not palpable
NS. absent AJs
diminished pin prick in both legs from knees downwards

congestive cardiac failure orthopnoea (

) murmur 3 left ventricular failure
3 (triple rhythm) (gallop) left ventricular
diastolic gallop left atrial pressure rapid left ventricular filling (
mitral stenosis )
(cardiomyopathy) hypertrophic cardiomyopathy ( 1)
amyloidosis sarcoidosis amyloidosis

heart failure amyloidosis

sensory polyneuropathy
(macroglossia)

()

proteinuria 4+ amyloidosis nephrotic syndrome

Urine: protein 4+
pH 6.5
WBC 0-1, RBC 3-5/HPF
fatty cast 1-2, waxy cast 0-1, granular cast 1-2
24 hour total protein 7,107 gms.
Hb 8.5 PCV 25.6 MCV 74.7 MCH 25.8 MCHC 34.6
RBC 3.42 x 106 RDW 21
WBC 20,500 N 80 L 10 M 8 E 2
ESR 34
VDRL non reactive anti HIV -ve
T.P 49.5 alb 24.3 Protein electrophoresis alb 49.3 1 6 2 14
14.3 16.4
no monoclonal gammopathy
BUN 19
Cr 0.6
Chol 296
TG 293
TB 0.7 DB 0.4 SGOT 46 PT 65 GT 1,082 Alk Phos 296
Chest x ray: cardiomegaly
High Resolution CT Lungs: ground glass opacities c associated interstitial
thickening, small lung cysts and small
nodules
in both lungs, mild mediastinal node enlargement
ECG: non-specific ST-T wave abnormality
Echocardiogram: concentric LVH, mild MR, mild TR, no pericardial effusion
amyloidosis biopsy
Bone Marrow: interstitial deposits of amorphous eosinophilic materials
Liver biopsy:
deposits of homogeneous eosinophilic materials in
sinusoids with
characteristic apple-green bi-refringence by polarized light
on
Congo-red stained section diagnostic of amyloidosis
Kidney biopsy: amyloidosis (AL)


Amyloidosis soluble protein
insoluble fibrils
20 amyloid beta
pleated sheets Congo Red polarized
( 2)
amyloid amylum (starch) Amyloidosis

chronic osteomyelitis
rheumatoid arthritis secondary amyloidosis amyloid A protein
(AA) ( 3)
2 AL (A Amyloidosis L Lightchain) amyloid
50 multiple myeloma
2 8 ( 4)
3 ( 25 systemic amyloidosis )
autosomal dominant amyloid transthyretin (TTR)
transport thyroxine retinol-binding protein TTR
gene chromosome 18 gene
30 amino acid, methionine valine Amyloidosis
Familial amyloid polyneuropathy (FAP) ( 5
6)
AL
50 restrictive cardiomyopathy
arrhythmia conducting system sensitive digoxin
sensory polyneuropathy
autonomic neuropathy
(
3, 6, 7 8)
AL amyloidosis multiple myeloma
mephalan prednisolone
(stem cell transplantation)
amyloid
amyloidosis FAP
TTR
Amyloidosis
amyloid

3
Pepys 9
Mayo 229 10 .. 1970-1980
11
monoclonal protein AL 77 congestive heart
failure 6
3

 7
6 8
10 11 amyloidosis !

1)
4 .. 2547 31-7.
2) Kazatchkine MD, Husby G, Araki S, Benditt EP, Benson MD, Cohen AS, et al.
Nomenclature of amyloid and amyloidosis WHO-IUIS Nomenclature
Sub-Committee. Bulletin of the WHO. 1993; 71: 105.
3) Pepys MB. Amyloidosis. In Oxford Textbook of Medicine. Third Edition. Vol. 2.
Eds. Weatherall DJ, Ledingham JGG, Warrell DA. Oxford Medical Publications.
Oxford 1996; pp. 1512-24.
4) Commenzo RL, Gertz MA. Autologous stem cell transplantation for primary
systemic amyloidosis. Blood 2002; 99: 4276-82.
5) Hund E, Linke RP, Willig F, Grau A. Transthyretin-associated neuropathic
amyloidosis: pathogenesis and treatment. Neurology 2001; 56: 431-35.
6) Freeman R. Autonomic peripheral neuropathy. Lancet 2005; 365: 1259-70.
7) Maredia N, Ray SG. Cardiac amyloidosis. Clin Med 2005; 5: 504-9.
8) Andrews TC, Bodi I, Reilly MM, Harwood G, Al-Sarraj S. An unusual cause of
speech and swallowing difficulty. Practical Neurology 2003; 3: 358-65.
9) Kyle RA, Greipp PR. Amyloidosis (AL). Clinical and laboratory features in 229
cases. Mayo Clin Proc 1983; 58: 665-83
10) Merlini G, Bellotti V. Molecular mechanisms of amyloidosis. N Engl J Med
2003; 349: 583-96.
11) Kelly JW. Clinical Implications of Basic Research: Attacking Amyloid. N Engl J
Med 2005; 352: 722-23.

17

37 .

1 .
3 schizophrenia
haloperidol, trihexyphenidyl diazepam 1 .
.

antipsychotic
neuroleptic malignant syndrome (NMS)

T 39C
RR 30
PR 120
BP 140/90
stuporose, no response to verbal command
Fundi
Pupils
rigid neck and generalized cogwheel rigidity of limbs
DTRs +++ +++
P

Urine : brownish
sp gr 1.030
pH 6
protein 3+ RBC +++
WBC > 100/HPF
Hb 12.8
Hct 38
WBC 13500
N 95 L 3
261000
BUN 122
Cr 7.0
Uric Acid 10.5
+
CPK 1443 Na 129 K+ 6.4 Cl- 91 Co2 12.8

M2

Platelets

()
cogwheel rigidity
protein RBC CPK BUN Cr NMS

 (Rhabdomyolysis) myoglobin
myoglobin
haemodialysis bromocriptine & clonazepam
6 1
bromocriptine levodopa
& benserazide

Neuroleptic Malignant Syndrone (NMS)

Delay .. 1960 ( 1)
.
( 2)
NMS acute dopamine deficiency syndrome
neuroleptic 0.1 2.5 ( 3)
haloperidol


clozapine, olanzapine, risperidone, lithium, carbamazepine ( 4), tricyclic
antidepressants metoclopramide selective serotonin
reuptake inhibitors SSRI venlafaxine ( 5) donepezil
( 6)
levodopa dopamineagonist NMS (
7)
NMS 3 9
NMS (
8) 200 ! NMS

NMS
gene NMS 2 gene CYP2D6 gene
metabolize NMS
( 9)
NMS Malignant Hyperthermia (MH)
MH gene
succinylcholine
ryonidine receptor gene chromosome 19q gene calcium
channel chromosome 3q 5q 7q
calcium ions sarcoplasmic reticulum
( 11 12)
Serotonin (SS)
neurotransmitter, serotonin
receptor 5HT1A 5HT2
antidepressant
NMS NMS
( 13, 14 15)
Creatine phosphokinase (CPK) CPK
enzyme CPK M B
2

 CK-MM 40
CPK ( 16)
haloperidol CPK NMS

( 17)
NMS SGOT, PT CPK CPK
( 18)
CPK 2-3 NMS ( 19)
NMS acute renal failure
myoglobinuria haemodialysis bromocriptine dopamine
receptor agonist 20 ( 20)
dantrolene bromocriptine
methylprednisolone pulse therapy
( 21)
Rhabdomyolysis
( 22)

1) Delay J, Deniker P. Drug-induced extrapyramidal syndromes. In : Handbook of

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Arch Intern Med 1991; 151: 794-6.
3

8) Pearlman CA. Neuroleptic malignant syndrome : a review of the literature.

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18

24

7
5

7 .
gentamicin ampicillin 2
2
2

3-4
1. epileptic convulsion
2. (mental confusion)
3.
4.
4 acute polyradiculoneuropathy
acute intermittent porphyria
acute polyradiculoneuropathy Guillain-Barr syndrome
acute porphyria
SIADH
(syndrome of inappropriate secretion of antidiuretic hormone)
porphyria

tendon reflex

T 38C
RR 24
PR 104
no neck stiffness
Abdomen: mildly distended, not tender
CNS
bilateral LMN facial palsy

BP 130/100

flaccid limbs power 2/5

absent deep tendon reflexes in all limbs
no Babinski response
sensation could not be evaluated
loose and sphincter tone
()

Urine: amber colour pH 6.5 sp.gr 1.020

ketone trace
porphobilinogen +
24 hr. uroporphyrin 349/ng (normal 0-50)
serum Na+ 132 K+ 4.2 Cl- 97
SGOT 55
PT 129
Alk Phos 150
Cholesterol 363
Alb 28.7
TP 60.6
Hb 12.4
WBC 8260
N 58
L 20
M 15
E4
B3
EMG nerve conduction velocity sensori motor
axonal polyneuropathy
. Na+ 116 CT
wedge shaped non-enhanced hypodense lesions both frontal lobes
SIADH Carbamazepine

acute
sensori motor polyradiculoneuropathy + epileptic convulsion

3
acute porphyria
rigor 4-5
lobar pneumonia, acute ascending cholangitis, acute pyelonephritis malaria
39 Kayser-Fleischer (KF) ring
Wilsons disease ( 1) KF ring
(pathognomonic sign)
porphobilinogen WatsonSchwartz test ( 2) benzaldehyde HCL
saturated sodium acetate solution 3-4
porphobilinogen chloroform chloroform
Ehrlichs aldehyde reagent 5 ml.
porphobilinogen reagent benzaldehyde

 Watson-Schwartz test Ehrlichs reagent

sensitivity
porphobilinogen 10 ( 3)
screening
tests porphyrin spectrophotometry sensitive
fluorescence ( 4)
porphyria

Porphyria haem
acute
intermittent porphyria (AIP) porphobilinogendeaminase (PBG-D) gene
(mutation)

colic
rebound tenderness AIP

AIP 2
( 5) (
6)




7
diazepam carbamazepine
gabapentin
haem (heme arginate) infusion
( 7)

gonadotrophin releasing hormone analogue
( 8)
Porphyria

3
(King George III) (.. 1738-1820) (Hanover)
3 (arsenic)

haem metabolism porphyria (
9) !

1)
1 2 ..
2545 47-58

2) Watson CJ, Schwartz S. A simple test for urinary porphobilinogen. Proc Soc
exp Biol (NY) 1941; 47: 393-4.
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Edition. London. J & A Churchill Ltd. 1964; pp. 848-64.
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diagnosis of
porphyrias. Siriraj Med J 2005; 57: 530-5.
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PJ, Bruyn GW. North-Holland Publishing Company. Amsterdam. 1976; pp. 429-47.
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Practical Neurology 2006; 6: 255-8.
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19

70
. 21 2540

3
10
. haloperidol 15 thioridazine
100 benzhexol 6
6 .

20
BUN 153 Cr 9.7

BUN Cr
acute stroke, subdural
haematoma

antipsychotic neuroleptic malignant syndrome

haloperidol

T 36.4C
PR 72
RR 24
BP 150/90
drowsy, no neck stiffness
Pupils : normal
Limbs : Tone : normal
Power : slight generalized weakness of both legs (power grade
3/5)
DTRs all present
Flexor plantar response
()
localising sign metabolic
encephalopathy renal electrolyte abnormality

30

 tapazole
Na+ 110 mEq
Na+ tapazole

Hb 11.2
PCV 35
MCV 93
RBC 3.77x106
WBC 9,500
N 84
L 11
M5
Platelets 163,000
Urine : protein 3+
RBC numerous WBC 10-20/HPF myoglobin -ve
BUN 153
Cr 11.3
+
Cl 86
K+ 5.22
CO2 10
Na 116
CPK 4,578
SGOT 480
SGPT 95
serum myoglobin -ve

3% NaCl infusion 21
10 Na+ 131 mEq BUN Cr 61
3.9 CPK 625 3
spastic quadriparesis DTRs Babinski
response 2
()
CPK rhabdomyolysis acute renal
failure
myoglobin spastic
quadriparesis central pontine myelinolysis (CPM)
Na+
CPM .. 1959
Raymond Adams Maurice Victor Mancall
( 1)

( 2-6) CPM
.. 1983 ( 7)
CPM ( 8 9) (
10) ( 11 12)
! ( 13)
Na+ 8 mmol ( 14)
MRI CPM ( 15 16)
MRI

CPM

( 17)
orofacial dyskinesia
CPM 34 11 11
10 2 ( 18)
12 CPM

( 19)



( 20)

1) Adams RA, Victor M, Mancall EL. Central pontine myelinolysis: a hitherto

undescribed disease occurring in alcoholics and malnourished patients. Arch Neurol
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20) .
: .
: .. 2549 419-25.

20

35
7 2514

2
7


4-5

21 4-5



2-3 39 8
8

focal
dystonia
emotional lability
emotional incontinence
corticobulbar tracts pons pseudo-bulbar palsy

pugilistic (boxing) encephalopathy

alert and cooperative - no evidence of dementia

PR 72 regular
BP 130/80
slight dysarthria
Fundi : normal
JJ + +
palmomental reflexes + +
L +
slight increased tone in arm - more on attempted writing

coordination
SJ
BJ
TJ
KJ
AJ
P

+++
+++
+++
++
+

+++
+++
+++
++
+

EEG mild diffuse abnormality

Air Encephalogram mild diffuse cerebral atrophy
Blood VDRL + ve (1:1) (yaws)
()
jaw jerk bilateral corticobulbar tract
lesion palmomental reflex diffuse cerebral
dysfunction Marinesco-Radovici reflex

(mentalis
muscle) ( 1)
Palmomental reflex ( 2)
basal
ganglia punch drunk syndrome Martland
.. 1928 ( 3)
pugilistic boxing encephalopathy Parkinsonism

cerebellum pyramidal tract (
4-7)

Macdonald Critchley
( 8) Critchley
5

CT scan air encephalogram cerebral atrophy
cerebral ventricles septum pellucidum
lateral ventricles 2
cavum septum pellucidum ( 6)
cerebral cerebellar cortex
basal ganglia ( 9) Neurofibrillary tangles (NFT) (AD)
senile plaques NFT
ubiquitin AD ( 10)
93
(mild to moderate closed head injury)

10 30 ( 11)

 35 6
74
scan



1
chronic pancreatitis
( 12)

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3

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16 ( 1.8) plasma aldosterone 400 pmol/l aldosterone/renin ratio
(ARR)
800 119 ( 14.1) ARR plasma
aldosterone
spironolactone 69 26/11
. K+
c adrenal adenoma d K+ renin
spironolactone
e aldosterone K+
renin
f aldosterone 400 pmol/l renin
activity 0.5 pmol/ml/h adrenal adenoma

Amyotrophic Lateral Sclerosis (ALS) Motor Neuron Disease ( 1

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2

ALS

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Pamela Shaw

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Hypoventilation
tracheostomy
noninvasive ventilation (NIV) ALS bulbar function

riluzole
NIV
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2

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MRI CVT
SLE
thunderclap
headache CVT
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gene G allele variant DGSIGNI 336 DGSIGNI 336

CD 209 promotor gene variant CD 209 gene
receptor dendritic cells

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1 20,000

George Huntington (.. 1850-1916)
Johan
Christian Lund(.. 1830-1906) 1860 George
Huntington 9 Lund ..
1959!
Ben Harper
setesdalryyja!
Setesdal
!
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Motor neuron disease (MND)
Amyotrophic Lateral Sclerosis (ALS)

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MS 90

oligoclonal IgG band (OCGB) MRI

OCGB
sensitivity 91.4 specificity 94.1 MRI sensitivity 74.23
specificity 88.2 OCGB MRI
sensitivity 97.1 specificity 88.2
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5

Murine Typhus ( 4 2)
1) . .. 2544-2546
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130 3

Vogt-Koyanagi-Harada disease ( 7 2)
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Eponym ( )

Friedrich Wegener
(Nazi)
!
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Wilsons disease
serum ceruloplasmin


2

1
Kayser-Fleischer ring
ceruloplasmin