CNS

Stroke
WHO Def (Global) cerebral function
Rapidly developing clinical signs of
'
focal disturbance of

with symptoms lasting for 24 hours or longer or leading to death ,

with no apparent
cause other than of vascular origin .

-
Types stroke

V V V

Ischemic Haemorrhage others

[80%] [17%3 631]

V
V V

-
Cryptogenictsoi ) ' Intracranial Haemorrhage .
Arterial dissection

Intraparenchyma 't . Venous sinus thrombosis

-

. Small penetrating artery thrombosis -

subarachnoid -
Vasculitis
( lacuna stroke) [ 2513
-

Subdural . Intracranial aneurysms

'
larger artery thrombosis 1201 ) Extra dural Arteriovenous malformations
-

cardiogenicembolick.it
'
Haemorrhage ischemic

infarction

L5D
-
Others

'
Risk factors . All age groups :

High Risk low Risk

'
Hypertension .
migraine
.
Smoking .
Oral contraceptives
.
DM .
Alcohol
-
Atrial fibrillation .
patent foramen ovale
.
Drugs : cocaine amphetamine
,
.
Recent myocardial infarction
'
Dilated cardiomyopathy
'
Prosthetic valve
.
^
Endocarditis Sleep apnea

High cholesterol
'

Obesity
'

- Vasculitis

.
Additional Risk factors more common in young patients :

-
Protein lands deficiency .
Sickle cell anaemia
.
Antithrombin II deficiency .
Hyperhomocystinemia
.
Antiphospholipid syndrome -
thrombotic thrombocytopenia Purpura
. Factor v Leiden mutation . Arterial dissection
.
Prothrombin 620210A heterozygous mutation . Infections ( syphilis IH1V)

-
Systemic Malignancy
 Syndrome localization clinical Features
middle cerebral Artery (mi ) Internal capsule contralateral Pure motor or sensory motor
-

. -

Syndrome
-

Caudate Nucleus features

Data men Parkinson ian features [ if putamenlpallidus
-
-

pallidus ischemia presents

-
Middle cerebral Artery (ma ) .
Superior division of MCA -

Mz
-

Brachial Syndrome : weakness of hand 4 arm

Syndrome -

frontal Operator syndrome :Brocha 's aphasia

with facial weakness

'
Inferior division of MCA -

Mz
-

If dominant hemisphere :
Wernicke 's Aphasia &
Contralateral Homonymous Superior quadrantanopia
-

If Non dominant -

hemisphere : Hemispatial Neglect;

Spatial agnosia without weakness

.
Complete MCA syndrome -

contralateral Hemiplegia
-

Contralateral Hemi anaesthesia

-

Contralateral Homonymous hemianopia

-

Global Aphasia ( if dominant hemisphere inn )

i
Unpaired ACA syndrome
-

frontal lobe -

profound Abulia
-

Entire medial part of cerebral hemispheres -

Bilateral pyramidal signs with quadriparesis

Urinary incontinence
-

'
Claude 's syndrome Red Nucleus 1 Cerebral peduncle 3rd nerve palsy + contralateral ataxia

-
Weber 's syndrome medial midbrain 1 Cerebral peduncle 3rd nerve palsy + Contralateral hemiplegia

'
Benedict's syndrome Red nucleus 1 medial midbrain Kleber 's -1 Tremors + Ataxia

thalamus contralateral hemisensory loss ; Agonising pain

-

Dejerine .

Roussy syndrome

'
Perinaud 's syndrome Dorsal Midbrain -
loss of up gaze

Downward ocular deviation (setting sun sign)

-

lid Retraction Nystagmus( Collier 's sign )

-

Mydriasis

Milord Gublar syndrome ventral Pons Ipsilateral lateral LVI palsy)

'
gaze nerve
-

LMN facial Palsy

-

contralateral hemiparesis
-

Ipsilateral
-
Medial medullary syndrome Medial Medulla -

XII nerve palsy

-

Contralateral hemiparesis
-

Intra nuclear optnalmoplegia

-

Loss of posterior column sensation

tea List of C1F s is highlighted .

 -
DX .
Clinical signs : BE FAST

B- Balance loss

E- Eye - Blurred vision

F- Face -
Weakness

A- Arms -
sudden weakness
5- Speech -

difficulty or slurred speech

T -

Time -

Sooner the treatment starts ,

better the outcome

-
Investigations :

' 9 scans -

Identity and exclude haemorrhage as cause of stroke .

'
MRI -

Reliably documents the extent & location of infarction .

.
Diffusion weighted imaging -

more sensitive for early brain infarction .

FLAIR ( fluid Attenuated recovery)

-
inversion
.
MR ICT angiography of cerebral vessels
. Intra arterial contrast radiography
.
Cardiac investigations -

EC4 .
Echocardiography .

'
complications .
cerebral edema
-
Cardiac arrytnmias
-
Myocardial Infarction
'
seizures
-
DV71Pulmonary embolism
'
pressure sores

Urinary infections
'

91 bleed (stress ulcers)

.

.
constipation

. DID, structural stroke mimickers functional stroke mimickers

.
Cerebral Tumors . Focal seizures
Subdural Haematoma
-

Hypoglycemia
.

'
Cerebral Abscess .
Encephalitis
Multiple sclerosis
. '
migraine with Aura

Transient global amnesia

.

. maniere 's disease

'
psychiatric disorders .
 .MX

① General measures . maintain Airway and breathing

.
Circulation -

check Pulse BP.

.

Treat abnormalities with N fluids

.
Look for hypoglycemia and treat accordingly

② Intravenous thrombosis .
Iv r -
TDA ( Recombinant tissue plasminogen Activator) .

At rate 09 mg 1kg ( max 90mg )

-

No other antithrombotic px for 24 hrs .

'
UI :
BP 71851110mm Hgi Platelet 51,00 0001µL ;
, Haematooritsds.to ; Prior Intracranial Haemorrhage

③ Endo vascular techniques .

Endovascular Mechanical thrombedomy : As alternate ( adjuncts're to patients who are

ineligible for thrombolytic -

④ Anti platelet therapy Aspirin 1150mg ) + Clopidogrel L75 mg] daily

⑤ Anticoagulation therapy . Not routinely recommended .

- Indications -

Cerebral venous thrombosis

known Hyperooagu table states

-

⑥ Medical support . Prevention of complications :

Infections :
Prophylactic Antibiotics
-

DVT : subcutaneous heparin

catheterisation Ryle 's tube feeding

-

- Maintain ence of Blood pressure

-
Fever -

Anlipyretics
'
serum glucose should be monitored .
keep 1110 mg 1dL .

Infuse Insulin if needed .

ICT water Restriction Iv mannitol

-
Decrease :
,

'

Hemicraniectomy
prophylactic sub occipital decompression of large cerebellar infarcts
'

⑦ statins Atorvastatin ,
Rosalia statin 110 -20mg )

⑧ Neuro protection Excitatory Amino acid pathway inhibitors :

magnesium labelvale ,
Nimodipine
L
Have not been proven beneficial in humans .

⑨ Rehabilitation .
Early physical occupational , and speech therapy .

.
Restrain therapy (immobilising unaffected side) has been shown to improve hemiparesis .

*a
secondary Prevention : ABCD 's score helps in identifying risk .

ABCD ' score ( Us minimal risk ; score 7 6 →

high risk of stroke

treatment of Atherosclerosis ,
Carotid atherosclerosis and Intracranial atherosclerosis .

Atorvastatin 180mg Hay )

-

Antiplatelets ( Aspirin ,
opidogrel )
Anticoagulant therapy ( warfarin apixaban )
-

,
 Ischaemic Stroke
'
Aetiology . Thrombotic occlusion small vessel Clacunar)

Large vessel thrombosis

'
Embolic occlusion

. Venous sinus thrombosis

.
Subarachnoid haemorrhage due to Vaso spasm -

Embolic Stroke/Acute Cerebral Embolism :

Artery to stroke
-

artery embolic
-

Any vessel may be source of emboli ( Aortic arch , vertebral & Basilar arteries)
-

Atherosclerosis within carotid artery (carotid bifurcation is most common source )

Intracranial Atherosclerosis
-

'
Cardioembolic stroke
-

Heart is most common source of emboli

-

Non Rheumatic Atrial fibrillation is most common cause of cerebral embolism .

tfmboli most often lodge in Middle cerebral artery of posterior Cerebral artery .

'
paradoxical Embolus
-

Atrial septal defect or patent foramen ovale may allow passage of fragments
Of thromboemboli ( eg from DV7 ) from Right atrium to left .

-
Fat embolism ,
air embolism ,
tumor embolism rarely alw neurological deficits .

'
UF i Mx :
written under stroke .
 Lacunar Infarct
Intro Small penetrating arterial branches of 200 -800µm diameter supply the deep brain
-
.
,

parenchyma .

. Thrombosis of these vessels cause small infarcts .

referred to as lacuna .

-
Infarcts range from 0.2 to 15mm in diameter .

.
Small vessel strokes account for 20% of strokes .

. Risk factor .
Hypertension
.
Age

- C1F syndrome signs 1 Symptoms

.
pure motor -
Contralateral hemiparesis hemiplegia

-
Pure sensory -

contralateral hemisensory loss

-

Persistent transient numbness of tingling sensation

on one side of body .

- Mixed sensorimotor -

contralateral weakness & numbness

Ipsilateral sensory impairment
-

Hemiparesis 1Hemiplegia

'
Dysartnria -

Clumsy hand
-

slurred speech
-

Weakness of contralateral hand (fine motor)

'
Ataxia -

hemiparesis
-

Cerebellar and motor symptoms

-

contralateral hemi paresis

Ataxia out of proportion to weakness
-

'
Hemiballismusltlemichored -

contralateral limb flailing Idyskinesis

.
Investigation & Mx written under stroke .
 Subarachnoid Haemorrhage
Intro SAH is which blood subarachnoid space
pathological condition in enters
- .
a
-

.
Less common than ischemic stroke on intra cerebral haemorrhage .

Accounts
'
for 5% of strokes .

.
Etiology ① Trauma : -

closed

penetrating
-

Electric
-

② Vascular Aneurysms ( Berry

: -

aneurysm )
-

Arteriovenous malformations
-

Vasailitides

③ Idiopathic :
Benign perimesen cephalic SAH

④ Blood dyscarias : -
leukemia

Haemophilia
-

thrombocytopenia

⑤ Infections : -

Dengue
Leptospirosis
-

Bacterial meningitis

⑥ Drugs : -

Amphetamines
cocaine
-

-
Nicotine

Anticoagulants
-

⑦ Neoplasms : -

Gliomas
-

meningiomas
-

Haemangioblastoma

•
Risk factors .
Hypertension
smoking
-

cigarette
.
0C pills
.
pregnancy
'

Increasing age
•
polycystic kidneys
.
fibro muscular dysplasia
'
AV malformations
.
slight med risk during LP & cerebral angiography
 -
C1F .
Headache CMK symptom)
-

sudden onset ( thunderclap )

mild warning headaches (Sentinel headaches) 2- Burks

preceding major haemorrhage .

'
vomiting
-
Decreased consciousness and alertness
'
seizure Got )
-
stiff neck may be present ( positive Koenig 's sign )
.
fundoscopy shows Subhyaloid haemorrhage (canoe shaped) produced due to tracking
-

Of blood along the subarachnoid space around the optic nerve

investigations . a scan
'
lumbar puncture : Done it probability of SAH is high and 9 does not reveal blood in

subarachnoid space .

CSF -

Uniformly blood stained .

-
Xanthochromia -

appears within 6 hrs of SAH (persists for 2 weeks) .

Spectraphotometry to estimate bilirubin confirms the diagnosis .

Angiography
'

-
MR angiography 19 angiography
'
ECG : prolonged GRS i tsed QT interval ; prominently peaked or deeply invented 1-waves
( cerebral T waves )
-
DIDS .
Migraine
. Acute Bacterial meningitis
- Cervical arterial dissection

-
Complications .
Obstructive Hydrocephalus : -

May be asymptomatic
May cause deteriorating consciousness following SAH
[
.

4- drainage via shunt

^
Arterial spasm : -

Causes delayed Cerebral ischemia resulting in Hemiparesis Koma .

It is a poor prognostic feature

-

B : Vasco dilators

.
Hyponatremia -

Managed by fluid Restriction

-
MX ① Absolute bed rest 14 weeks)

② Supportive measures :

protect airway
Manage blood pressure
-

pain management
-

sedation
 ③ control Hypertension :

Target mean arterial pressure mmHg

30

Drugs ! La beta lol .

Esmolol , Nicardipine
* Avoid sodium Nitropreside ( may cause 9 sed top)

④ things to strictly avoid :

Hypovolemia , hypotension ,
hyperthermia , Hyperglycemia . Hyponatremia .

⑤ Calcium channel Blocker :

Nimodipine [ 30 -60mg N for 14 days ,

followed by 360mg oral for next 1 days)
Prevents delayed cerebral ischemia .

⑥ Prophylactic Anticonvulsants

⑦ Interventional management Early aneurysm repair :

prevents Rerupturel Re bleeding .

Aneurysm Repair clipping by neurosurgeon

-

coiling by Endo vascular surgeon

-

Insertion of Platinum coils into

Aneurysm sac .

⑧ Treatment of complications

Circle of Willis
Anterior Communicating Artery

< Anterior Cerebral Artery

V

Internal carotid Artery

V
^

Middle Cerebral Artery

a Posterior communicating Artery

^
Posterior cerebral
Artery

< Basilar Artery

<
Vertebral Artery
'
Constituents of circle of Willis :

'

2 Internal carotid art ( Rt El Lt) Posterior Cerebral Artery (Rt d Lt )

. .
.

'
Anterior cerebral Art (Rt . .
4lb .
posterior communicating artery
(formed by joining )
.
Anterior Art Basilar artery of a vertebral art
-

communicating
 Transient Ischemic Attacks
Definition defined transient episode of neurologic dysfunction caused by focal brain
'
T1A is as ,

spinal cord or retinal ischaemia without acute infarction .

TIA is characterised by a brief episode of neurological dysfunction ( sudden loss

of function ) in which symptoms and signs resolve completely after a brief

period within 24 hours ( usually within 30 minutes ) .

Types Large artery low flow Recurrent short tasting episodes [ thrombotic / shotgun TIAJ
-
. TIA -

'
Embolic TIA :
Longer tasting less frequent episodes with varied symptoms

.
Lacunar TIA

-
C1F . Hemi paresis
-
Aphasia
. Amaurosis Fugax ( sudden transient loss of vision in one eye)
. Hemi sensory loss
'
Hemianopic visual loss
.
Diplopia
'
Vertigo
Vomiting
-

Choking
'

- Ataxia

. Risk factors . Atheroma

.
Hypertension
. Diabetes mellitus

' DX Clinical evidence of source of embolus ( valvular heart disease ,

M1 ,
endocartitis )

.mx . medical therapy and surgical Treatment if required and appropriate .

. If ABCDZ score 74 or patient has had 2 recent T1A i He should be

investigated and advised for secondary prevention of stroke

.

( Anti platelets Anticoagulation

, ,
statins )
 Pyogenic Meningitis
-
Organisms . Neonate : E. coli ,
Listeria monocytogenes ,
group B streptococci
. Infants 1 Children :
Haemophilus influenzae ,
Neisseria meningitis ( meningococcal's )
.
Adolescents :
meningococcal ,
Pneumococcus
- Extremes of age : Streptococcus pneumoniae ,
Listeria monocytogenes
most common organism causing community acquired bacterial meningitis Pneumococcus
-

Organism that causes epidemics of bacterial meningitis meningococcal -

Staphylococcus Klebsiella
.
Uncommon :
aureus ,

C1F classical triad Fever rigidity

.
. :
,
Headache , nuchal

Vomiting
.

-
Photophobia

varies from lethargy to

-
consciousness :
coma

. Seizures focal -

due to arterial ischaemia ,

infarction ,
cortical venous thrombosis

Generalised -

due to hyponatremia
. occasional cranial nerve palsies with visual defects , dysphagia .
hemi paresis

. For meningococcal -

Diffuse erythematous Macedo popular rash found on trunk ,

lower limb ,
mucous membranes .

'
signs :

1) cervical rigidity 1 Neck stiffness

a) Hennig 's sign -

knee pain with hip flexion
3) Brudzinski 's sign -

knee / hip flexion when neck is flexed .

signs of Raised ICP :

Reduced level of consciousness

-

Papi'll edema
-

Dilated poorly reactive pupil

-

Decerebnate posture
-

Vith cranial nerve palsy

-

Cushing 's Reflex :

Bradycardia ,
HTN , irregular respiration

. DX 1) CSF Examination :

-
Appearance :
Turbid ; forms coagulum
.
pressure :
Raised ( above 180mm H20 )
.
proteins : Raised 1750 mgldl )
Decreased ( mgldl )
'
Glucose : 0-20

-
Chlorides :
Decreased
-
microscopy :
9ham stain → Bacteria t

M sed and
polymorphs lymphocytes
-

" Blood
culture and sensitivity
'
14 .
Empirical therapy :

. Infants 53 months :
Ampicillin tcefotaxi.me

- Infants 73 month ¢ Adult 555 years :

cefotaxi.me/Ceftriaxone +
Vancomycin
. Adults 755 years with detonating conditions : Ampicillin -1 cefotaxi.me/ceftriaxone- vancomycin
' Hospital acquired 1 post neurosurgery 1 impaired cell mediated immunity : Ampicillin + vancomycin
+ ceftazidi.me/meropenem

-
Specific therapy :

it Neisseria meningitis : Ampicillin 3g N T1D

>
penicillin resistant -

Ceftriaxone 2g N BIDI Cefotaxime 2g N BID

-
N doses for 7 days is adequate
-

Close contacts >

Chemo prophylaxis -

Azilhromycin 500mg once

ii
) Streptococcus pneumoniae :

Penicillin sensitive : Penicillin G

Intermediate :
ceftriaxonelcofotaxi.me
-

Resistant :
ceftriaxonellefotaxi.me + Vancomycin
→
N antibiotics to be continued for 2 weeks

→
Intraventriuelar vancomycin is more effective than IV.

Iii) staphylococci -

Meth icillin sensitive Nafcillin

methicillin resistant : vancomycin 1g N TID

iv ) Gram -

ve (except pseudomonas) -

cettriaxonefcefotaxi.me for 3 weeks .

↳ Pseudomonas :
leftazidi.me/meropenem

vi ) Listeria monocytogenes :
Ampicillin × 3 weeks

vii ) U .

influenzae : ceftriaxonelcefotaxi.me

viii ) Bacteroides fragilis : Metronidazole

. Adjunct've therapy :
Dexamethasone -
inhibits synthesis of TNFd and IT1B

+ CSF outflow resistance

stabilise BBB

Given 20 minutes before antibiotic therapy .

T¥tofRk
'
:
Elevation of head to 450

mannitol
 -
Complications .
Obstructive hydrocephalus
' thrombi phlebitis of Iepto meningeal veins
- Chronic adhesive arachnoid its
•
cerebral abscess
-
Subdural empyema
'
focal Neurological deficits CCN palsy ,
hemi paresis )

.
sensorineural hearing loss
- Seizures
. Waterhouse -

Fred erichsen syndrome ( in meningococcal El pneumococcal meningitis)

. Cerebral herniation due to Raised KP

Tubercular meningitis
-
Investigations 1) CSF study .

. cobweb formation on standing ( indicates fibrinogen)

-
Cytology :
100-500 leukocytes tell i Lymphocyte predominant , Polymorphs in acute stage

Biochemical
-
:

Protein :
IO0-1OO mgldl ( Raised )
-

Glucose : 20-50 mgldl ( Reduced )

-

Chloride : Reduced
-

Adenosine deaminase elevated

.
microbiology -

Gram stain :
Negative
2N stain : Acid fast bacilli seen

Centrifuged CSF thick smear from pellicle and repeated enhance detection
'
:
culture

- OF culture :
in Lowenstein -
Jensen media 4-8 weeks )

2) Radiology
- chest x ray
' CT scan -

thickening of meninges in basal cistern

Hydrocephalus (50%-801)

3) Immunology :

Antibody detection
-

Antigen detection IELISA )

-

PCR
-
complications .
Raised KP
,
cerebral oedema
- Basal meningitis with CN I , II ,
#
,
VI. VI palsy
.
Focal neurological deficits
'
seizures
'
Hydrocephalus

TUberdl oma.opticochiasmaticpachymenigiti.si
'

Diabetes insipid us ,
SIADH

Hypothalamic disorders
.

.mx . ATT for 15 years in uncomplicated cases .

Steroids during initial 6 weeks adhesion formation

. to decrease possibility of .

-
Surgical intervention : If hydrocephalus ,
tubercular ma .
abscess develops .
 Brain Abscess
-
Aetiology - Bacterial

Site source of infection organisms

- Frontal lobe Parana sat sinuses , teeth streptococci ,

Anaerobes
-
Temporal lobe middle ear streptococci ,
Enter bacteria ceae

. Cerebellum sphenoid sinus ,

mastoid Pseudomonas . Anaerobes
.
Any site penetrating trauma Staphylococcus

'
Other organisms
:

Mycobacterium tuberculosis
-
No cardio
-

Fungi :
Crypto aus , Histo plasma , Aspergillus
-

protozoa :
TOXO plasma gondii ,
Schistosoma , trypanosoma

-
C1F . Fever
-
Headache
'
Neck stiffness
. Nausea , vomiting
.
photo phobia

. Drowsiness
.
Raised KT
'
seizure
focal neurological
'
deficits

.
DX CT scan IMRI

'
B .
Appropriate antimicrobial therapy

.
Surgical -

Drainage by burr hole aspiration

-

CT guided stereotactic aspiration

 Neurosyphilis
'
Aetiology :
treponema Pallidum -

Neuro syphilis may take years to develop after primary infection .

- C1F .
Asymptomatic :
Occurs with few months of infection
primary
.

symptomatic
- :

1) meningeal Syphilis : symptoms within 2 years infection

occur of
primary
.

Headache .
Neck stiffness ,
No fever
-

Seizures ,
Altered sensorium
-

Rashes on palms and soles

2) meningovasculdr syphilis : usually presents 6-7 years after infection

can develop early (6 months ) or late 110-12 years) after infection .

Stroke : Hemi paresis , aphasia , visual loss

Headache vertigo
-

psychological abnormalities

3) Parenchymatous syphilis :

A) General paresis of insane : 20 years after primary infection

-

Personality changes ,
illusions ,
delusions

- Hallucinations ,
Dementia
-

Hyperactive reflexes
-

Argyll Robertson pupil

B) Takes Dorsalis :
Demyelination of posterior column dorsal ,
root 9 dorsal root ganglia
-

severe lightening pain in trunk 4 eitremitiesi Urinary incontinence

-

patchy tactile sensory loss ,

severe impairment of proprioception
-

sensory Ataxia
-

Perforating ulcers of feet

-

Charcot Joints
-

Visceral crisis :
Epigastric pain with vomiting Intestinal
,
crisis with

diarrhea .
Rectal crisis with tenesmus , genitourinary crisis with strangling

-
DX CSF exam :

- Pressure : Normal I Raised

Appearance
-
: clear
- Protein : Raised 1100-200 mgldl )
'
Glucose : Normal
'
Cell count : 4 leukocytes ,
predominantly lymphocytes
'
VDRL i
tve ( Gold standard )
'
FTA ABS lrtuore scent treponemal antibody absorption) : t ( High sensitivity ,
low specificity )

"
Rt : .
Penicillin 18-24 million units Iday × 20 days

. For penicillin sensitive patients : Erythromycin 4 tetracycline

-
Re-examine patient every 3 months

- CSF examination every 6 months

. If CSF is abnormal patient is treated with another full course of penicillin

,
 Transverse Myelitis
- Definition Acute usually monophonic , demyelination g inflammatory disorder affecting spinal cord

myelitis refers to inflammation of spinal cord and transverse signifies involvement

-

across one spinal cord level .

Causes 1) Bacterial boreliosis

-
infections -

Mycoplasma pneumoniae , lyme , syphilis .

2) Viral infections .

Herpes simplex , Herpes zoster , cytomegalovirus ,

Epstein bar virus

Enteroviruses.HN Rabies ,

3) Post vaccine -

cow pox , Rabies

4) Autoimmune diseases -
SLE . Sarcoidosis ,
sjogren 's syndrome

5) Daraneo plastic syndromes

6) Vascular
-

thrombosis of spinal arteries .

- C1F . Lower limb weakness

.
Sensory : sensations diminished or absent below level of involved spinal cord .

Pain and temperature sensations are diminished .

'
Bladder and bowel dysfunction :
Disturbed sphincter control
-
thermite 's sign : Radiation of parestnesias down the spine or limbs with neck flexion
>
Suggests intrinsic cervical spinal cord lesion .

DX Shows swelling
-
. MR1 -

spinal cord with gadolinium enhancing lesions .

Exclude compressive causes of spinal cord dysfunction

.
CSF examination -

Shows cellular pleocytosis

-

Protein is slightly increased

-

IgG index is elevated

'
Chest x ray ,
tuberculin test , ESR -
Exclude TB .

-
Serology ,
PCR , cultures ( viral causes , mycoplasma )

'
B
High dose corticosteroids Prednisone 60mg oral aid 5 days
. -

'
IV immunoglobulins
.
plasma phones is
- Supportive therapy
-
Treat underlying cause .

For viral TM Acyclovir every 8 hours 7 days

:
10mg 1kg IV X
-

-
For severe , refractory cases -

Azatnioprine , methotrexate ,
oral cyclophosphamide

.
Prognosis : there may be no recovery .
In cases of recovery most recovery occurs within 1st 3 months .

complete recovery may take up to 2 years or longer .

 Guillain-Barre syndrome -

'
Definition It mediated
is a heterogeneous group of immune conditions which is most commonly
acute , severe fulminantpolyradi.ae/opathy/poly neuropathy .

-
Etiology .
Preceding infections -

Campylobacter jejuni
-

Epstein-Barr virus

Mycoplasma pneumoniae Herpes virus

-

Cytomegalovirus

prior vaccination (old types)

-
-

Swine flu ,
influenza .
Rabies

. In patients with lymphoma ,

SLE .

Pathogenesis Immune self (infections Ivaccines ) that misdirect

to antigens to
-
responses non

Host nerve tissue through resemblance of epitope [ molecular mimicry ]

- Variants 1) Acute inflammatory demyelinating poly neuropathy ( AIDP) - T cell mediated

-
Anti GM1 ab in 150% cases

seen in Adults

2) Acute motor Axonal neuropathy ( AMAN ) -

Anti GN11 , GD1A antibodies

in children ¢
seen young adults
-

3) Acute motor sensory axonal neuropathy IAMSAN ) -

Anti GD1A ab .

mostly seen in adults

4) Miller Fisher syndrome IMFS ) -

Anti GQ1B , GT1A antibodies

-

may be axonal or demyelination

-

seen in adults and children

presents with that moplegia ataxia and are Hella

-

' C1F .
Acute rapid onset of paralysis evolving over days 1 weeks
-

Predominantly motor with 1 without sensory

-

Ascending paralysis :
weakness begins in K and rapidly ascends to UL .

-
Pain -

Acute deep aching pain in weak muscle -

.
No fever or constitutional symptoms on onset of weakness

Sensory minimal 4
.
involvement -

may precede weakness

presents as distal pareslhesia ¢ loss of pain sensation

 - Involvement of facial 9 respiratory muscles 130% cases ) requiring ventilatory support .

'
Autonomic disturbances -

fluctuation of BP , postural hypotension

-
Bladder dysfunction in late and severe cases

- On examination ,
loss of deep tendon reflexes ( A reflex ia )

Clinical 4 weeks plateau and

worsening period of reaching
-

occurs over a , a

No further progression .

-
Investigations .
CSF studies :

i. Raised proteins 400-1000 mgldl )

2. Normal sugar
3. Cell count C 10 cells 1mm 's ( Upto 50 cells in patients with concurrent HN )

Albumin cytologl.cat dissociation ( Raised proteins with no pleocytos.is) is seen .

-
Nerve conduction studies :

mild 1 early : Normal

-

Demyelinating :
prolong distal latency ,
slow conduction ,
conduction block ,

prolonged F wave latency ; Absent H -

reflex
-

Primary Axonal : Reduced amplitude of compound action potential without

slow conduction .

-
Antibody detection tests -

Detection of Anti GM1 , GD1A , GQ1B GT1A antibodies .

-
B .
Early treatment :
Immunotherapy after 72 weeks of 1st motor symptom is not
effective

Immunoglobulins glkgl day for

'
Intravenous 0.4 infusion 5 days .

IV
Ig neutralises autoantibodies .

Screen patient for IgA deficiency .

In patients with IgA deficiency ,

severe allergic reactions may develop due to IgG .

.
Dlasmapheresis ( Plasma exchange) 50 mhlkg on 5 separate occasions over 1-2 weeks
-
.

.
No role of glucocorticoids .

'
Full occurs in 50-701 in 1 year
recovery cases .

Worsening
-
case :
monitor in ICU ; ventilators support may be needed

-
Supportive measures : DVT prophylaxis , prevention & R, of bedsores .

Physiotherapy
 Herpes zoster
Herpes zoster is responsible for a variety of neurological disorders .

- Disorders . Herpes zoster opthalmicus with involvement of trigeminal nerve

'
Involvement of geniculate ganglion of CN VII.
-
Involvement of CNS I ,
VIII ,
II and I

meningitis
'
Aseptic
-
peripheral motor neuropathy
. viral transverse myelitis
'
viral encephalitis

cerebellitis.vasuulopdlhi.es
'
Acute
& stroke
.
Acute retinal necrosis
.
Sacral meningoradiculitis ( Ekberg syndrome)
'
Temporal arthritis
'
post hermetic neuralgia
.
Bell 's palsy -

major cause in patients who are HSV seronegative

'
DX ( DCRI
. csf exam -

Detection of Herpes zoster DNA

- MX .
Val acyclovir Ifamicydovir -

for uncomplicated
therapy should be started within 72 hours and should be continued for 7- 10 days
-

'
Corticosteroids -

Tapering starting at 60mg 1 day

course X 2-3 weeks
L safe in immunocompetent patients .

'
Intravenous acyclovir -

for complicated VZV infections

.
Fosca met -
for acyclovir resistant VZV infections
 Cerebellar disorder signs
I. Ataxia - Ataxia may be result of cerebellar lesions or combination of cerebellar and

extra cerebellar lesions .

-
Due to defective timing of contraction of agonist 1 antagonist muscles
- Lesions of vermis of cerebellum cause truncal ataxia , while lesions of hemispheres
cause ipsilateral limb ataxia

Causes of cerebellar Ataxia

'
'
.

Sporadic I Acquired Genetic

i. Vascular : stroke ,
AV malformations . Autosomal dominant : spin cerebellar ataxia

ii. Infectious : cere.be/lilIs cerebellar abscess

, ,
HIV

iii. Toxins : Alcohol . Autosomal Recessive : Friedrich ataxia

Heavy metal →
mercury
Drugs
-

Lithium
-

Metronidazole

Cyclosporin @
-

Antiepileptics
iv. structural & Neoplastic
-

Glioma
-

cerebellar tumor
-

meningioma
v. Immune mediated :

multiple sclerosis
-

Parana plastic syndrome

-

Gluten ataxia
vi. Deficiency
-

vitamin Bi

vitamin B12
-

-
Truncal Ataxia :
patient can not sit or stand unsupported and tend to fall backwards .

.
Gait Ataxia :
patient tends to stand with feet well apart ( wide based gait) .

Patient tends to reel to side unilateral lesion or from side to side

in cases of bilateral lesion ( drunken gait)

2. Hypotonia .
Usually accompanies acute hemispheric lesions .

-
Ipsilateral to side of cerebellar lesion .

-
Pendula r knee jerk : leg keeps swinging after knee jerk more than 3 times .
 3. Dysdiadochokinesia .
Impaired performance of rapidly alternating movements .

- Method to demonstrate :

it Alternating movements ( supination & promotion ) of forearm and hand quickly .

ii
) Stewart Holmes rebound sign

4. Asynergia - Lack of synergy of various muscles while performing complex movements .

-
movements are broken up into isolated ,
successive parts .

5. Dysmetria . Abnormal excursions in movements .

. methods to demonstrate :

i.
Finger to Nose test
2 Heel to Shin test

6. Past Pointing overshooting of limb while reaching target .

Demonstrated by finger to nose test .

7. cerebellar Dysartnria .
Abnormalities in articulation and prosody .

.
scanning , slurring ,
staccato , garbled speech .

-
Hemispheric lesions are alw speech diorders more often ly than vernal lesions .

8. Intention tremor . tremors occurring during a goal directed movement .

'
Antagonist activation that normally stops a goal directed movement is inappropriately
sized or timed .

9. Oculomotor dysfunction Gaze evoked nystagmus , upbeat nystagmus ,

rebound nystagmus .
'

all are possible in

midline cerebellar lesions .

Romberg 's test : it is not a test of cerebellar disorder .

Romberg 's sign is positive in cases of sensory ataxia

.

However ,
if ataxia does not improve after opening of eyes ,
it is suggestive
of a cerebellar lesion .
 Amyotrophic Lateral Sclerosis
-
sporadic (90-951) -
Idiopathic . Familial (5-101)-7 Autosomal dominant

-
Introduction .
Amyotrophic lateral sclerosis CALS ) is most common form of progressive motor

neuron disease -

-
Caused by degeneration of motor neurons at all levels of CNS , including
anterior horn cells ,
brainstem motor nuclei and motor cortex .

:
Amyotrophic :
Atrophy of muscle fibre consequent to denotation due to anterior horn

degeneration
Lateral sclerosis :
sclerosis of anterior d lateral corticospinal tracts which are replaced

by progressive gliosis .

. Incidence : I -3 100,000
per
'
Epidemiology .
Age : Risk increases with age up to 70 years of age [ Peak in 6th 7th decade]
-

. sex : male ) Female 121 to 7 :D

' C1F . Muscle weakness

Often presents with focal motor weakness of proximal 1 distal

-

one limb ( upper or lower ) .

the focal motor weakness spreads to contiguous muscles in same region

-

before involving another region .

then ,
other limbs and trunk muscles are involved .

- Muscle wasting
-
Stiffness and cramping
Twitching and fasa.cn/ations wasted muscle
'
of

Symptoms of brainstem involvement :

'
Dysphagia ( may lead to aspiration pneumonia )
-
prominent wasting of tongue
.
Difficulty in articulation and phonation
-
Weakness of ventilators muscles
. No sensory abnormalities ,
No bowel 4 bladder dysfunction

"
DX Diagnosis by series of clinical neurological
largely and
.
is examinations .

'
Myelogram of cervical spine :
Detects lesions in selected areas of spinal cord .

.
EMG and Nerve conduction velocity to measure muscle response to nerve stimulation .

- MRI :
coronal TLWI shows bit hyperintensity along corticospinal tract forming
wine glass appearance or Garland sign .
 '
DIDS Since untreatable should be excluded
ALS is all secondary causes .

. other motor neuron disease Primary lateral sclerosis ( UMN only )

Progressive bulbar palsy
progressive muscle atrophy CLMN only)

.
structural lesions cervical spondylosis
Spinal cord AV malformation

Neuropathic Chronic

demyelinatingpolyneuropatny.myopathi.es
inflammatory
'
:

:
Dolymyositis ,
Inclusion body myosin's

- NMJ disorders :
myasthenia gravis

.
Neurodegenerative diseases :
Parkinson 's , multiple sclerosis

-
Infectious : HIV ,
CMV , Lyme 's disease

- Endocrine :
Hyperthyroidism , Hyperparathyroidism

. Toxins :
Heavy metals

. Malignancy : Primary ; metastasis to CNS ,

motor neuron syndromes with multiple myeloma

'
R There is no treatment that arrests the underlying pathological process of

ALS .

. Riluzole :
Iv glutamate release and thus decreases excitotoxic cell death .

> Dose :
100mg 1 day
>
ADRS :
Dizziness , weight loss ,
elevation of liver enzymes

'
Edaravarohe :
Acts as antioxidant

-
For spasticity : Badofen 5-
10mg bid

Tizanidine I -4mg bid

memantine 5mg 1 day
Tetrazepam at bedtime
50mg

'
Rehabilitation : .
foot drop splint
finger extension splint
'

-
Respiratory support :
tracheotomy , positive pressure ventilation
-
But bar involvement : Gastronomy for nutrition
.
physiotherapy , speech 4 occupational therapy

prognosis most cases progress to death in about 3-5 years .

. :
 Epilepsy and Seizures
of
'
seizure : Paroxysmal event due to abnormal ,
excessive , hypersynchro nus discharges from an aggregate
CNS neurons
-

-
Epilepsy : Condition in which an individual has recurrent seizures due to chronic underlying process .

. Classification :

>
Focal
y
Aware I impaired awareness I 7 motor onset Non motor onset
Onset unknown awareness
- Automatism . Autonomic
.
Atomic .
Behaviours arrest
'
. Clonk cognitive
'
Epileptic spasms
'
Emotional
-
-
Hyper kinetic sensory
'
Myodonic
- Tonic
'
Focal to Bilateral Tonic clonk

Generalised 7 motor
) Non motor Absence)
onset Tonic Clinic
. .
Typical
Clinic
.
.
Atypical
Tonic
seizures .
. Myodonic
Myodonic

myodonia.myodonic-tonic-donic.my
.
. Eyelid

odonic -
Atomic
-
Atomic

. Epileptic spasms

Unknown Motor Non Motor

,
> I
onset ' tonic Clonk .
Behaviour Arrest
- Epileptic spasms

Unclassified
>
seizures
- Causes . Neonates : .
Infants 4 Children :

Perinatal hypoxia & ischaemia -

Febrile seizure

Intracranial haemorrhage 4 trauma -

Metabolic
-
CNS infections -

Degenerative
metabolic primary Epilepsy syndromes
- -

( Hypoglycemia , Hyperltlypocakemia ,
Hyponatremia) -

CNS infections

- Adolescents
.
Young adult (18-35) . Older adults (735 yrs)
-

trauma
-

Trauma -

cerebrovascular disease

Genetic disorders
-

Alcohol withdraw I -
Trauma

-
Infection
-

Brain tumor
-

Alcohol withdraw 1
-

Degenerative disease

.
C1F Generalised, Tonic-Clonic Seizures
Main type of seizure in 10% people with epilepsy
.

.
Usually begins abruptly without warning
,
.

.
Initially -

Tonic contraction of muscle throughout body

leads to
✓

-
Loud moan or total cry

Cyanosis
-

Biting of tongue
-
Marked enhancement of sympathetic tone > 4 HR it BP ; 4
pupillary size

. After 20 seconds : clonk phase starts with superimposed relaxation which

progressively increases until end of ictal period

. Post total phase : .

Unresponsiveness
-
muscular flaccidity
'
Excessive salivation

/ Bladder
'
Bowel incontinence

-
Patient gradually regains consciousness over minutes to hours with accompanying
post total contusion ,
Headache ,
muscle ache .

.
EEG : .
Generalised High amplitude
-
Poly spike discharges in tonic phase
.
Spikes interrupted by slow waves in clinic phase
-
This creates spike and -
-

wave pattern in GTCS .

-
Investigations -
Routine :
-

serum glucose ,
calcium and electrolytes
-

liver function tests

Kidney function tests

-

-
EEG i
May help in diagnosis of epilepsy ,
classification of seizure type and
evidence of particular epilepsy syndrome .

presence of electro graphic seizure activity clearly establishes diagnosis

-

EEG may also be helpful in inter ictal period

-

by showing certain abnormalities

that are strongly suggestive of epilepsy .

EEG is normal in 401 of epileptic patients

'
Neuroimaging : MR1 7CT

'
lumbar puncture -

if indicated

'
B . First seizure :

-
Establish whether seizure was a paroxysmal episode or epileptic .

Determine of identifying factors

-

the cause seizure by risk , precipitating

Factors , general examination and investigations .

Decide whether anticonvulsant therapy is required .

RenceOfas①zwe :
-

Identification of underlying cause and precipitating factors .

Determination of adequacy of patient 's current therapy .

'
Antiepileptic drug therapy should be started in
any patient with recurrent
seizures of unknown etiology or a known cause that cannot be reversed
with in short time .

- Risk Factors alw recurrent seizures :

An abnormal neurological examination

-

seizures presenting as status epileptic us

-
post -
ictal Todd 's paralysis
strong family history of seizures
-

Abnormal EEG
-

Abnormal CT or MR1

.
Monotherapy for GTSC :

Valproate 15 mglkg be increased maximum of 60mg 1kg

every week to
:
can a
-

Topiramate : 25 -50mg 1 day

2nd choice Zonisamide Ievetiracetam
Lamotrigine carbamazepine phenytoin
:
, , , ,
- Indications to discontinue therapy :

Complete medical control of seizures for 1-5 years

-

Single ,
seizure type , either focal or generalised
Normal neurological examination ( including intelligence)
-

Normal EEG

'
In most cases , it is preferable to reduce the dose of drug gradually over

2- 3 months .

. Most recurrences occur in first 3 months after discontinuing therapy and

patients should be advised to avoid potentially dangerous situations .

'
R, of
Refractory epilepsy :

- 1st line drugs :

carbamazepine , phenytoin , valproic acid , Lamotrigine
.
Addition of Ievetiracetam and topiramate if 1st line unsuccessful .

.
ketogenic diet

-
surgical 14 of refractory seizure :

Antero medial temporal lobotomy

-

Amygdala hippocampectomy
Hemispherectomy
-

corpus Callisto my
-

Lesion @ ctomy For Focal seizures .

- D0C For Other seizures :

- Absence seizures -

Ethosuximide.ua/proate
monotherapy for partial seizures carbamazepine
topiramate.myodonic.ua/proate,Ievetiracetam.cIonazepam
-
phenytoin
-

, ,

.
febrile convulsion -

Diazepam rectal 0.5mg 1kg

 Status Epilepticus
- Definition . Status epilepticus refers to continuous seizures of repetitive discrete seizures
,

with impaired consciousness in internal period .

'
status epileptic seizures of greater than 5 minutes or more than one seizure

within 5 minute period without the patient returning to normal between them .

types Generalised
'
.
convulsive status epileptics
'
Non convulsive status epileptics

' C1F 4 sed cardiac

.
Initial compensatory phase
:
sympathetic overdrive ,
output BP,

.
Dewmpensation
-

Hemostat's failure
-

cardio respiratory collapse

-

Electrolyte imbalance
-

Raised KD and cerebral edema

-

Multi organ failure

-
Complications Aspiration , Hypotension ,
cardiac arrhythmias ,
renal 1 Hepatic Failure

.MX .
First 5 minutes :

maintain airway , breathing and circulation .

Give 02
-

Obtain IV access
-

Begin EC4 monitoring

-

Check finger stick glucose

-

Draw blood For serum electrolytes ,

RFT ,
magnesium ,
calcium
-

toxicology screen ( urine and blood )

- 6-10 minutes :

-
Thiamine 100mg N ,
50mL Of D50 N

lorazepam 4mg N Over 2 minutes , if still seizing , repeat in 5 minutes

If no rapid IV access give diazepam 20mg I midazolam 10mg oral or 1M

-

'
10-20 minutes :

Begin As phenytoin 20mg 1kg N at 150 mglmin with BD & ECG monitoring
-

Phenytoin 15-20
mg/kg at 30
-50mg Im in
- 20 -

60 minutes :

11 Continuous N midazolam
-

Loading dose :
0.2mg 1kg ; Repeat 0.2 mg/kg boluses every 5 minutes till seizures stop

Max loading dose :

2
mg/kg
Continuous IV infusion dose 0.05 2.9mg Ikglhr
-

: -

OR

2) IV propofol 1mg 1kg i repeat boluses every 5 minutes till seizures stop

OR

3) N Valproate 40 minutes
mg/kg over 10

.
760 minutes :

Continuous N phenobarbital
-

Loading 5mg 1kg

:
, repeat 5mg 1kg untill seizures stop .

Continuous N dose : 0.5-10 mglkglhr

'
Perform convulsive controlled Begin continuous
neuroimaging when
activity is EEG -
.

.
Treat metabolic abnormalities and hypothermia
.
 Parkinsonism
i
Parkinsonism is a syndrome consisting of a variable combination of tremor ,

rigidity . bradykinesia and a characteristic disturbance of gait and posture .

'
classification Parkinson ian disorders

v v
✓

Primary Secondary Parkinsonism plus

-
Syndrome
Parkinson's disease - Toxins -
MAP , manganese mercury ,

-
Viral -

Encephalitis lethargica

-
Metabolic -
Wilson 's disease
V ~

familial Head injury punch drunk syndrome

Sporadic
-

-
Infectious -

HIV , postencephalitic
-
Drugs -

dopamine receptor blockers , reserpine ,

lithium

- Vascular -

Multi infarct
-

, Binswanger disease
- Trauma -

Pugilistic encephalopathy
- Others -

Parathyroid disorders Normal , pressure hydrocephalus;

psychogenic , para neoplastic , tumors

-
Etiology 1) primary -

Idiopathic
Environment risk factors :

pesticide exposure
-

oxidative stress
-

Non smokers have higher chance than smokers .

-
About 5- 1st .

cases are familial in origin .

Associated genes : PARK -

SNCAIDARKI) ,
PARK -
LR RK2 ( PARK 8) ,
DARK Parkin ( PARK
-
2)
and various other PARK genes CPARKI -

20)

secondary Causes mentioned above

' -

- CIF . Motor :

1) Tremor -

Early and presenting symptom in 90% patients .

Typically starts with fingers and hands at rest .

( Resting tremors )
-

worsens with emotional stress

-

Disappear on voluntary movement and sleep .

Often begins with rhythmic flexion -

extension of fingers ! hand / Foot or

with rhythmic promotion summation -

of forearm .
 2) Stooped posture

3) Gait slow short shuffling gait with reduced

: arm
swing .

f- estimating gait

41 Brady kinesia I Akinesia :

Difficulty in initiating voluntary movement

-

Fixity of facial expression ( mask like face )

-

Repetitive tapping over glabella produces sustained blink response ( Myerson 's sign )

4) speech -

Soft , quiet ,
montanus and stuttering

5) Rigidity :

increased resistance to passive movement

-

Increase in muscle tone present throughout movement ( lead pipe rigidity ) .

-
Ratchet jerkiness is felt when tremor is superimposed on rigidity .

fog wheel rigidity )

6) Combination of tremor , rigidity and brady kinesia results in small ,

tremulous ,

illegible handwriting ( micro graphic ) .

- Non motor features :

'
Autonomic dysfunction : orthostatic hypotension , Urinary incontinence . compilation

Sleep disorders
'
: Insomnia , daytime somnolence

Sensory
.
: Pain ,
Hyposmia

Rheumatological Frozen shoulder Swan neck deformity

-
:
,

'
Neuropsychiatric :
Anxiety ,
depression , apathy ,
psychosis ,
dementia

. Others : Restless leg syndrome ,

seborrhoea .

DX is made clinical
Diagnosis grounds
- . on .

-
CTIMRI Usually normal
-

-
Functional dopaminergic imaging :
by SPECT or PET -

shows reduced uptake of striata

dopaminergic marker ( particularly in posterior putamen)

-
Dopamine transporter imaging ( DAT)

'
staging : Hoehn and Udhr staging
.MX of curative
Drug treatment Parkinsonism is symptomatic rather than .

1) Levodopa : precursor of dopamine .

Single most effective drug

-

Provides symptomatic relief ,

particularly brady kinesia .

2) Peripheral dopa carboxylase inhibitors : Reduce peripheral metabolism of levodopa .

Carbidoba , Benseraside

3) Dopamine facilitator :
Amantadine

4) Dopamine receptor agonists : Bromocriptine , Cabergoline -

Ergot derivatives

Ropinirole , Rotigotine , Apo morphine -

Non ergot derivatives

5) MAO -

B inhibitors : Facilitate breakdown of excess dopamine in synapse

.

Enhance efficacy of levodopa Carbidopa formulations

-
.

selegelli.no Rasagiline
-

b) 0Mt inhibitors : Prolong action of levodopa by decreasing peripheral metabolism .

Tokapone , Entacapone

7) Anticholinergic ! Helpful relieving tremors in

trihexyphenidyl.benztropi.no
-

-
Surgical Mx : Indications are Intractable tremor and Drug induced motor fluctuations

i.
stereotactic surgery ( ventrolateral Ihalmotomyl
2. pallid otomy
3. Deep brain stimulation -

Best site : sub thalamic nucleus

MA0 B Monoamine oxidase type B

'

-
-

CONT : Catechol -

O -

methyltransferase
 Chorea
'
Irregular ,
semi purposeful , abrupt . rapid ,
brief ,
jerky . unsustained movements that

flow randomly From one part of body to other .

.
these movements disappear during sleep
-
When choreic movements are severe , assuming a flinging ,
sometimes

violent character ,
they are called Ballism .

- Causes 1) Rheumatic ( Sydenham 's chorea )

a) Huntington 's chorea [ Autosomal dominant]

3) Encephalitis -

Japanese encephalitis ,
measles
4) vascular
5) HIV related : HIV encephalitis , Ioxoplasmosis
6) Drugs : L -

dopa , phenytoin
7) Degenerative disorders of brain

b) pregnancy ( chorea gravidarum)

a) Endocrine -
metabolic dysfunction : Adrenal insufficiency Hyponatremia Hyperltlypocakemia
, ,

10 ) Miscellaneous : Anoxic encephalopathy , cerebral palsy ,

Kernicterus , Multiple sclerosis

'
signs and
1) Involuntary profusion retraction of the tongue Jack in the box

2) Milking action of patient 's if asked to grasp the

fingers physician 's
fingers [ Milk -

maid sign]

3) Inability to hold the hands above head with palms facing each other as

it results in promotion of arms so that palms face outwards fpronator sign )

.mx symptomatic

chorea ( Saint Vitus Dance )

-
Sydenham 's :

Sodium Valproate 200 -600 mg T1D

-

Risperidone ( if valproate is not effective)

-

Ualoperidol occasionally

Huntington 's chorea

.
:

-
HD chorea is self limited and is usually not disabling
-

pschosis treated with atypical narcoleptic .

Depression & anxiety treated with antidepressants and anti anxiety drugs .

Experimental trials Ubiquinone creatine as disease modifying therapy

.

,
.
 Cluster Headache
- cluster Headache ( Migrainous neuralgia ) is distinct from migraine and is

much less common

.

. cause and precise mechanism is unknown .

. It differs from migraine in its character , absence of genetic predisposition ,

lack

of triggering dietary Factors

observed Functional imaging during

-
Abnormal hypothalamic activity is on studies an

attack

-
patients are usually smokers and consume more than average school .

' C1F .
periodic with recurrent bouts of identical headaches beginning at same hour
For weeks at a time
-
severe , stabbing 1 boring ,
Unilateral periorbital / Retro orbital pain with parasympathetic
autonomic features is same eye I Lacrimation ,
conjunctiva 1 redness )

DX Criteria attack fulfilling Following

-
At least 5 :

'
severe unilateral orbital , supraorbital , temporal pain tasting Is to 180 minutes
'
Headache is accompanied by at least one of following :
-

Restlessness or agitation
-

Autonomic features
1) Conjunctiva redness or Iacrimation
2) Nasal congestion and rhinorrhea

3) Edema of eyelid

4) Sweating on the forehead

5) Mia's and/or ptosis
-
frequency of attacks : from one every other day to eighth day .

-
Mx . Acute attacks :

subcutaneous injection of Sumatriptan 16mg )

Oral sumatriptan is not effective .

Sumatriptan 120mg ) and 201mi triptan 15mg ) nasal sprays are also effective .

Inhalation of 100%
Oxygen at 10-124 min For 15-20 min .

Octreotide is effective in acute attacks .

-
Prophylaxis :
-

sodium valproate ,
lithium ,
verapamil
And I or

-
Oral corticosteroids
 Abnormalities of Gait
-
circum auction pyramidal 1 Hemiplegia :

Lesions of upper motor neuron produce characteristic extension of affected leg .

There is tendency For toes to strike ground on walking and outward

swing / throwing of lower limbs .

( circumlocution at Hip joint)

There is leaning towards opposite normal side
-

Arm of affected side is addicted at shoulder 9 Flexed at elbow wrist 4

, Fingers

'
High stepping slapping Gait / Foot drop :

Seen in common perineal nerve palsy .

-
Weakness of ankle dorsiftexion , results in a less controlled descent of foot , which

makes slapping noise as it hits ground .

In severe cases ,
foot will be lifted higher at knee joint to allow room for

inadequately dorsiflexed foot to swing through ( High stepping )

-
waddling Gait myopalhic Gait :
caused due to weakness of proximal lower limb muscles ( Dolymyositis ,

muscular dystrophy ) .

and
the hips are not properly fixed by muscles trunk movements are exaggerated
-
.

. Trendelenburg Gait .
weakness of abductor muscles of lower limb [ Gluteus medius and minimus]

During stance phase or while standing one leg there is tilting 1drooping of
-

on ,

pelvis to opposite side .

. Ataxic Gait -

seen in lesions of cerebellum or vestibular apparatus .

the patient is unstable tremulous and reels in any direction and walks
-

on a broad base .

-
Shuffling Gait f- estimating 1 Extra pyramidal gait : seen in parkinsonism

-
scissoring Gait seen in cerebral palsy due to bilateral spasticity

. Apraxia Gait .
The power , cerebellar functions and proprioception are normal on examination but

the patient can't initiate and organise motor act of walking .

The feet appear to be stuck to floor .

. Seen in diffuse bilateral hemisphere disease or diffuse frontal lobe disease

[ Tumor , Hydrocephalus ,
infarction)

-
stomping Gait sensory Ataxia :

Broad based , high stepping stamping gait ,

due to loss of proprioception .

seen in poly neuropathic taboo dorsalis

-

.
Hyper kinetic Gait :
Chorei Form gait :
irregular , jerky .
involuntary movements in all extremities .

seen in forms of chorea ,

athetosis or dystonia .
 Myasthenia Gravis
'
myasthenia gravis is an autoimmune NNI junction disorder characterised by
weakness and fatigue of skeletal muscles .

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Pathophysiology .
Antibodies :
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Anti ACHR antibodies detected in 80 -

85% cases

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Anti Musk antibodies in Anti ACHR negative cases .

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Changes in thymus :

thymus is abnormal in 751 patients ( Hyperplastic or tnymic tumors )

i C1F .
More common in males kill
peak incidence is around 30 years of age

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Fluctuating weakness ( that worsens on exertion) and fatigueability of muscles .

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Shows more focal muscle involvement ( ocular ,
facial ,
But bar ,
Neck , jaw , respiratory )

.
Diplopia and ptosis

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Respiratory weakness

. No sensory signs or signs of involvement of CNS .

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Aggravating factors .
Exertion
. Hot climate

. Emotional
. Pregnancy
- Menstruation
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Drugs -

Aminoglycosides , phenytoin

.MyasthenkCri Rapid severe deterioration of myasthenia that can bring patient to brink of

respiratory failure and quadnbaresis in hours .

Generally caused by a respiratory infection or Sedative with NM blockade

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.
 '
DI 1) Serology : Detection of antibodies against ACHR or musk .

2) Pharmacological tests :
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Anti cholinesterase test :
Administration of anticholinesterase improves strength in

myasthenia muscles .

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Edrophonium test 1 Tension tests :
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Test dose of edrophonium 12mg ) is given IV .

If there is improve ht , drop Further test .

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If negative ,
further 5 -8mg IV is given ; test is considered positive if there is substantial

improvement in weakness .

positive test is suggestive of MG ( sensitivity 801 )

3) Electro diagnostic :

Repeated nerve stimulation test

Single fiber electro myogram

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4) Other tests :

CBC ,
ESR ,
RA Factor
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CT ,
X -

ray , MR1 :
to exclude thy mama
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screening for associated autoimmune disorders .

. A 1) Oral anti cholinesterase :

Pyndostig mine 30 -60mg ,

3-4 times a day for]
Neoslig mine IS 30 mg 1 day
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2) Immunosuppression :

Immediate : Iv Ig
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Intermediate :
prednisolone 110mg Hay ) , cyclosporin @ ,
tacrolimus
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long term : Azalhioprine

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Refractory : High dose cyclophosphamide .

3) Plasma pheresis

4) thymectomy
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Indications : 1) Patients with generalised MG between 15-55 years of age .

2) thy mom a
Advantages 1) long term benefit
'
!

2) Improves prognosis
3) Negligible medication

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Mx Myasthenia crisis :
1) Icu admission

2) Respiratory assistance (preferably Non invasive )

3) Pulmonary physiotherapy
4) Plasma pheresis or IV Ig .
 Compressive Myelopathy
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Causes . Extradural :
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Spondylosis
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Disk prolapse
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Trauma

Tumor :
Multiple Myeloma Metastasis
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CVJ abnormalities
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Fluorosis
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TB spine
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Epidural abscess
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Epidural haematoma

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Intra dural :

Tumor :
Neuro fibro ma , meningioma , lipoma ,
sarcoma
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Metastasis
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Arachnoid its
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Sarcoidosis
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cervical meningitis
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Arteriovenous malformations
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leukemic infiltration
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Arachnoid cyst

. Intra medullary :

Syrinx
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Tumor : Ependyoma , astrooytoma Haemangioblastoma

, , Haematomyelia

Symptoms
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1) pain -

occurs early
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Localised over spine or in a root distribution

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may be aggravated by coughing or sneezing

2) Sensory -

paresthesia
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Numbness specially in lower limb spread proximally to level of trunk .

3) Motor -
Occurs late
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Weakness ,
heaviness or stiffness of limbs .

4) Sphincters -

Occurs late
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Urgency 1 Hesitancy of micturition

 -
signs vary according to level of cord compression .

1) Cervical :

Above Cy
- :
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Upper motor neuron signs

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Sensory loss in all 4 limbs

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Weakness of diaphragm
.
4- Cs :
Quadriplegia with preserved respiratory function
.
G- IT : -
Lower motor Neuron signs 9 segmental sensory loss in arms

Upper motor neuron signs in legs

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weakness of respiratory ( intercostal ) muscles .

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2) Thoracic cord !

Spastic paraplegia with a sensory level on trunk

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Weakness of legs , sacral loss of sensation and Extensor plantar response

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midline back pain

3) lumbar cord 9 Cauda equina :

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4- L2 :
Cremasteric reflex abolished
.
↳ Ly - : -

Paralyses flexion and abduction of thighs

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weakness of extension at knee joint

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Abolished patellar reflex .

Is G paralyse movements of foot and ankle flexion at knee

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: -

Abolished ankle jerk .

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Localisation of level of lesion in compressive My elopathy :

1) Distribution root pain : Ask for specific dermatomes involved

2) Examine patient from below upwards for demonstration of Upper border of

sensory loss -

lspinolhalamic tract )

3)
Girdle like sensation or constriction at level of lesion .
( involvement )
of Posterior column

4) Zone of hyperaesthesia ( just above level of girdle like sensation) or hyperalgeria is

due to compression of posterior Nerve Roots

5) Analysis of Abdominal Reflex :

If upper abdominal reflex is intact with loss of middle
and lower reflexes ,
site of lesion is probably T10 spinal segment .

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Beevor 's sign : -

H lesion lies above to ,

entire rectus abdo minus is weak ,
and there is no contraction of muscle
- If lesion is at or below T10 , upper abdominal muscle function is preserved & lower muscles

are weak .
So ,
when head is flexed against resistance ,
the upper abdominal muscles pull the
umbilicus upward
.

shift of umbilicus by 3cm when head is Flexed is significant .

. Causes of Beevor 's sign : 1) Spinal Cord Injury 131 Adult form of acid maltose
deficiency
2) ALS
 6) Atrophy of muscles in segmental distribution ( due to Anterior Horn Cells )

7) loss of deep reflexes below the level of segmental involvement

8) Deformity or swelling in vertebra 4 tenderness of vertebra .

a) Radiological Investigations

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Investigations . MRI of spine
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Investigation of choice

localise compression and define extent of compression

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Plain X-ray spine :
May show destruction of bone and soft tissue .

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Myelography

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Serum B12

- CSI examination

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Biopsy : if a
secondary tumor is causing cord compression .

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R Treatment depends on underlying lesion .

main aim is decompression of cord .

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Benign tumors -

surgical excision

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TB -

ATT and surgical decompression

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Traumatic lesions -
Need treatment by neurosurgeon

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Extra dural compression due to malignancy has poor prognosis .

- It is important to diagnose and treat early because damage

may be reversible in early stages .
 Peripheral Neuropathy
Classification 1) Monon empathy Involvement of
simplex
- :
single nerve

2) Mononeuropathy multiplex : simultaneous or sequential involvement of severe individual

( multiple mononeuropatny ) nerves

3) Dolyneuropathy : Diffuse involvement of numerous peripheral nerves .

Causes 1) neuropathy
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mono
-

simplex :
Peripheral nerve entrapment or compression .
eg
: carpal Tunnel syndrome
-

multiplex :
1) Ischaemia due to vasculitis

a) microangiopatny
3) HIV

4) Hepatitis C

5) Neurofibromatosis

6) Neoplastic infiltration .

2) Poly neuropathy :

Axonal Demyelinationg

Acute ( axonal ) 1) GBS CAIDP)

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1) GBS -

AMANIAMSAN

2) Lead 2) Diphtheria
3) Cisplatin
4) lyme 's disease

Subacute 1) Alcohol DH1V

1)
.
.
Damone Amiodarone

2) Toxins s . Isoniazid 2) Vasculitis 2) para protein emia

3) porphyria - lithium 3) Siogren 'S 3) Arsenic
4) DM . Phenytoin 4) Chloro quince
Disu Hiram
5) multifocal Neuropathy
.
5) metabolic
cimetidine
6) Nutritional '
6) Gold

- Chronic 1) CKD 1) CIDP

1) CMT 11 2) CMTI and II

3) Secondary amyloidosis 3) Hypothyroidism

4) DM 41 Leprosy
 - C1F 1) Motor
i.
weakness Diseases with only motor symptoms :
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wasting -
Motor Neuron disease

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Loss of dexterity -
GBS
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Cramps . Lead toxicity
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Tremors .
CIDP

fascinations
.
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Acute porphyria
.
spasms

2) sensory :

. Numbness ; loss of sensations in hands and Feet

- Pain
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Burning sensation
.
Tingling
. Pins and needles sensation
.
sensory ataxia ( in large fiber neuropathic s )

3) Autonomic :

. Postural hypotension
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Sweating abnormalities
.
Bowel and bladder dysfunction
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sexual dysfunction

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Investigations Routine Special

1) Blood : -

CBC , peripheral smear

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Nerve conduction studies
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ESR .
Vitamins A and E
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C reactive
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protein . Nerve biopsy

. CSF examination
2) Biochemical : -
Blood glucose . Serum ACE
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LDH . Serum amyloid

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vitamin Biz , folate .

Genetic testing
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Serum protein
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LFT , RFT

3) serology : ANA ,
ANCA ,
HIV testing

4) Others : chest X
ray ,
USG abdomen

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A :

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Depends on underlying cause .

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Symptomatic treatment
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Pain -
NSAIDs , Opiods , tricyclic antidepressants
Parestnesia carbamazepine 1300 -1200mg Hay ) Amitripty line ( 25mg Hay ) gabapentine , Duloxetine
- -

, ,

- weakness -

physiotherapy
 Upper motor Neuron lesion lower motor Neuron lesion

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type of paralysis spastic Hadid

- Muscle tone
Hypertonic ( Rigidity ) Hypotonia

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Deep tendon Reflexes Exaggerated 1 Hyper reflexion Hyporeftexl.ci/AreHeXia
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Atrophy late ( due to disuse ) muscle wasting is seen

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Fascinations None present

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Distribution of weakness Distal predominant 1 Regional Proximal predominant I segmental

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Babin ski sign present Absent

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Flexor spasms looms present Absent
 Trigeminal Neuralgia
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Trigeminal neuralgia is also known as Prosopalgia or Fothergill 's disease

- It is a neuropathic disorder defined as sudden , episodes of usually unilateral

severe ,
brief , stabbing , recurring pain in distribution of one or more branches

of Ith cranial nerve .

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Usually seen in 6th and 7th decade of life .

Major risk factor is Hypertension .

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Etiology .
Idiopathic
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Demyelination of nerve

.
Multiple sclerosis
.
Petrus ridge compression
.
posttraumatic
.
Intracranial tumors
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Intracranial vascular abnormalities
viral etiology
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. CIF . pain
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Sudden .
unilateral ,
intermittent . sharp shooting 1 Laminating .

pain is short duration and recurrent .

Attacks do not occur in sleep .

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Pain occurs along distribution of 5th nerve i starts at mandibular division and
then spreads to maxillary and oplhalmic divisions .

It be bilateral in cases of multiple sclerosis or

expanding cranial tumor
may
- .

pain is precipitated by minor trauma to trigger zones ( touch chewing shaving

-

, , ,

rinsing mouth) .

Trigger area on face is so sensitive that even air currents

may trigger an episode .

No signs of sensory loss or vtn nerve dysfunction can be demonstrated .

'
th - 1st line : Carbamazepine 100-200 mglday it to 200 -400mg over 2-3 weeks
. 2nd line ! Badofen.lamotrigin@iphenytoin.ga bapentine
.
Long acting anaesthetics : Alcohol injection peripheral glycerol injection ,
into particular branch

of nerve

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Surgery : If drug therapy Fails or not tolerated .

Peripheral neureotomy
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Open procedures ( Intracranial procedures)

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Micro vascular decompression
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Percutaneous rhizotomies

Gamma knife radiosurgery

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