NEUROLOGY & GENERAL PHYSICAL EXAMINATION

DR GANESHGOUDA MAJIGOUDRA
CONSULTANT NEUROLOGIST
NANJAPPA HOSPITALS DAVANAGERE
ganeshgoudam4@gmail.com
9380906082
 VITALS-
BP
• Determining the BP in both arms is useful in patients with suspected CVA.

• Significant asymmetries may reflect extracranial occlusive disease.

• Orthostatic hypotension is a frequent cause of syncope.

• Autonomic insufficiency due to peripheral causes, as in DM neuropathy, or due to failure of central

regulation, as in MSA.

• MC cause of orthostasis is as a side effect of antihypertensive therapy

 CUSHING RFLEX

• Its a late sign of increasing ICP and indicates that brainstem herniation is imminent.

• Triad of hypertension, bradycardia and irregular respirations in a patient with ICP.

• Increased BP occurs in some patients acutely with stroke or SAH before ICP has risen.

• Increased BP due to stroke is often due to peripheral attempts to compensate for cerebral ischemia and
usually resolves without treatment.

• Severe systemic hypotension is rarely due to a neurologic cause, except as a terminal event, and is
much more suggestive of a hemodynamic disturbance
 THE PULSE

• The pulse rate & character are important, especially if increased ICP is suspected.

• The pulse usually slows, but may occasionally accelerate.

• A bounding pulse occurs in AR or hyperthyroidism and a small, slow pulse in AS.

• Detecting the irregularly irregular pulse of AF is important in the evaluation of stroke patients.

• Both brady & tacharrhythmias produce cerebral hypoperfusion.

 RESPIRATION

• Neurologic complications of pulmonary disease are common. Note the respiratory rate, rhythm, depth,
and character of respirations.
• Abnormalities of respiration, such as Cheyne-Stokes, Biot, or Kussmaul breathing may be seen in coma
and other neurologic disorders.

• Either hyperpnea or periods of apnea may occur in increased ICP & in disturbances of the
hypothalamus.

• Use of accessory muscles of respiration may signal impending ventilatory failure in patients with
many NMJ disorders , GBS & MND.
 SMELL OF BREATH

• Acetone (DKA)

• Ethanol (intoxication)

• Fetor hepaticus, uriniferous (uremia)

• Garlic odor (arsenic poisoning)

• Household gas (carbon monoxide).

 TEMPERATURE
HYPERTHERMIA HYPOTHERMIA

• Infection • Exposure
• inflammation • sepsis
• neoplasms (rare), • shock
• Anticholinergics • myxedema coma
• SAH • Wernicke’s encephalopathy
• Hypothalamic Lesion •Drug Intoxication (especially
• Heatstroke barbiturates)
• thyroid storm • hypothalamic lesion
• malignant hyperthermia • Hypoglycemia
 GENERAL APPEARANCE

• Fever, pain, or distress;

• Evidence of weight loss;

• Abnormal posture of the trunk, head, or extremities; the general level of motor activity;

• Unusual mannerisms; bizarre activities; restlessness; or immobility

 GENERAL APPEARANCE

• Weight loss and evidence of malnutrition may indicate hyperthyroidism, Alzheimer’s disease,

Whipple’s disease, malabsorption syndrome , or amyloidosis.

• The body fat level & distribution, together with the hair distribution & the secondary sexual development

are important in the diagnosis of endocrinopathies & disorders of the hypothalamus

 GENERAL APPEARANCE

• Gigantism,

• Dwarfism,

• Gross deformities,

• Amputations, Contractures

• Disproportion or asymmetries between body parts.

 ABNORMAL POSTURES

• Spastic hemiparesis causes flexion of the upper extremity with flexion and adduction at the shoulder,

flexion at the elbow and wrist, and flexion and adduction of the fingers;

• In the lower extremity there is extension at the hip, knee, and ankle, with an equinus deformity of

the foot.
 ABNORMAL POSTURES

• In Parkinson’s disease and related syndromes, there is flexion of the neck, trunk, elbows, wrists, and

knees, with stooping, rigidity, masking, slowness of movement, and tremors.

• In myopathies there may be lordosis, protrusion of the abdomen, a waddling gait, and hypertrophy of

the calves.

• Peripheral nerve disease may cause wrist or foot drop or a claw hand or pes cavus.
PES CAVUS
 HEAD

• Shape, symmetry, and size of the head note any apparent abnormalities or irregularities.

• Premature closure of cranial sutures can produce wide variety of abnormally shaped skulls –

craniosynostosis.
 CRANIOSYNOSTOSIS

• Plagiocephaly.- involves fusion of either the right or left side of the coronal suture that runs from
ear to ear.

• Produces a flattening of the forehead and the brow on the affected side, with the forehead tending

to be excessively prominent on the opposite side.

• Trigonocephaly is a fusion of the metopic (forehead) suture

• Early closure of this suture may result in a prominent ridge running down the forehead. The forehead
looks quite pointed, like a triangle, with closely placed eyes (hypotelorism).
• Scaphocephaly is an early closure of fusion of the sagittal suture. This is the most common type of
synostosis. This suture runs front to back, down the middle of the top of the head. This fusion
causes a long, narrow skull.
 CRANIOSYNOSTOSIS

• Severe craniosynostosis involving multiple sutures may cause increased ICP .

• Craniosynostosis usually occurs as an isolated condition, but there are numerous syndromes in

Crouzon’s, Apert’s, and Carpenter’s syndromes.

 HEAD

• Other deformities or developmental anomalies include:

• Hydrocephaly, macrocephaly, microcephaly, asymmetries or abnormalities of contour, disproportion

between the facial and the cerebral portions, scars, signs of recent trauma.
 FACE

• Dilated veins, telangiectatic areas, or port- wine angiomas on the scalp or face.

• In those with head trauma, ecchymosis over the mastoid (Battle sign) or around the eyes but not

extending beyond the orbital rim (“raccoon eyes”) suggests basilar skull fracture.
 SKULL

• Palpation of the skull may disclose deformities due to old trauma, burr hole, or craniotomy defects,

tenderness, or scars.

• The size and patency of the fontanelles is important in infants as bulging of the fontanelles & suture

separation can occur with increased ICP in children.

• Neurofibromas of the scalp occur in von Recklinghausen’s disease.

• Localized swelling of the scalp may occur with osteomyelitis of the skull.

• Exostoses may indicate an underlying meningioma.

• Giant cell arteritis may cause induration and tenderness of the superfi cial temporal arteries.
POTTS PUFFY TUMOUR
 PERCUSSION OF SKULL

• Percussion of the skull may disclose dullness on the side of a tumor or subdural

hematoma

• Tympanitic percussion note in hydrocephalus & increased ICP in infants and children

(Macewen’s sign, or “cracked pot” resonance).

 AUSCULTATION OF THE HEAD
• Bruits may be heard best over the temporal regions of the skull, the eyeballs, & the mastoids

• Cephalic bruits may occur with angiomas, aneurysms, arteriovenou malformations, neoplasms that

compress large arteries, and in the presence of atherosclerotic plaques that partially occlude cerebral

or carotid arteries.
• Ocular bruits usually signify occlusive intracranial cerebrovascular disease. A carotid bruit
may be transmitted to the mastoid
• An ocular bruit in a patient with a arteriovenous aneurysm may disappear on carotid compression.
• Murmurs may be transmitted from the heart or large vessels
 FACIAL EXPRESSION

• Acromegaly,
• Myxedema,
• Hyperthyroidism,
• Down’s syndrome,
• Mucopolysacchroidosis.
RISUS SARDONICUS IN TETANUS
MASK LIKE FACE
PERPETUAL SURPRISE - PROCERUS SIGN IN PSP
MYOTONIC DYSTROPHY
 EYES

• Ophthalmologic abnormalities can provide many clues to the etiology of neurologic disease as well

as to the presence of underlying systemic disease that may be causing neurologic symptomatology.
 EYES

• Bilateral exophthalmos due to thyroid eye disease;

• Carotidcavernous Fistula,
• meningocele,
• encephalocele,
• corneal clouding from mucopolysaccharidosis;
 EYES

• Brushfield spots on the iris due to down’s syndrome

• Lisch nodules in neurofibromatosis;

• Keratoconjunctivitis sicca due to sjögren’s syndrome

• Depositions of amyloid in the conjunctiva;
• Herpes zoster ophthalmicus;
• Kayser-fleischer rings in wilson’s disease;
 HYPERTELORISM- MEANS THE PRESENCE OF WIDE SPACED EYES. THIS IS

DIAGNOSED WHEN THE INTER INNER CANTHAL DISTANCE B E T W E E N THE

TWO EYES IS MORE THAN HALF OF THE INTER PUPILLARY DISTANCE.

BILATERAL EXOPHTHALMOS DUE TO THYROID EYE
DISEASE
 CAROTIDOCAVERNOUS FISTULA,

• A carotid-cavernous fistula (CCF) results from an abnormal communication between the arterial

and venous systems within the cavernous sinus in the skull.

• It is a type of arteriovenous fistula.
ENCEPHALOCELE
CORNEAL CLOUDING
KF RING
BRUSHFIELD SPOTS IN DOWNS SYNDROME
LISCH NODULES
 EARS

• Examination of the ears is particularly important in patients with hearing loss or vertigo.

• Examination of the ear canal may reveal a glomus tumor in a patient with jugular foramen syndrome, vesicles

due to herpes zoster infection, or evidence of a posterior fossa cholesteatoma.

• CSF otorrhea may cause a clear or bloody ear discharge.

• Hemorrhage into the middle ear may cause a bulging, blue-red tympanic membrane in patients with basilar

skull fracture.
 HITZELBERGER’S SIGN

• Seen in acoustic neuroma.

• It occurs due to involvement of the 7th cranial nerve.

• There is early involvement of the sensory fibers which causes hypoesthesia of the posterior

meatal wall.
 NOSE

• Perforation of the nasal septum may be a clue to cocaine abuse.

• A saddle nose may be a sign of congenital syphilis, leprosy , wegeners

• Evidence of bacterial infection in dangerous area of face may be a sign of cavernous sinus thrombosis;

• Watery drainage may be due to csf rhinorrhea.

 TONGUE AND ORAL CAVITY
• A triple furrowed tongue is seen in myasthenia gravis;

• Lingua plicata in Melkersson-Rosenthal syndrome;

• Macroglossia in amyloid, myxedema, and Down’s syndrome.

• Other potential findings include xerostomia in Sjögren’s ,a lead line along the gums in lead toxicity, trismus in
tetanus or polymyositis; and mucosal ulceration in Behçet’s disease.

• Notched teeth are a sign of congenital syphilis (Hutchinson teeth).

 NECK

• Note any adenopathy, thyroid masses or enlargement, deformities, tenderness, rigidity, tilting or other

abnormalities of posture, asymmetries, changes in contour, or pain on movement.

• Meningeal irritation may cause nuchal rigidity, head retraction, and opisthotonos. Neck movement may

also be restricted with cervical spondylosis cervical radiculopathy, and dystonias.

• In meningeal irritation the primary limitation is in neck flexion; in spondylosis the limitation is either

global or primarily in rotation and lateral bending

• In the Klippel-Feil syndrome, syringomyelia, and platybasia, the neck may be short and broad,

movement limited, and the hairline low.

• The carotid arteries should be cautiously and lightly palpated bilaterally, one at a time, and, followed by

auscultation for carotid bruits.

 EXTREMITIES
• Note any limb deformities, contractures, edema, or color changes.

• Any variation from the normal in the size or shape of the hands, feet, or digits, as well as
deformities, joint changes, contractures, pain or limitation of movement, localized tenderness,
wasting, clubbed fingers, or ulcerations may be significant.

• Edema may be evidence of CCF or cardiomyopathy.

• Arthropathy may be a sign of connective tissue disease, sarcoidosis, or Whipple’s disease.

• Painless arthropathy (Charcot joint) occurs when a joint is deafferented; painless enlargement of the
shoulder has been reported as the presenting manifestation of syringomyelia.

• Palmar erythema may be a clue to alcohol abuse.

• Diseases of the nervous system are found in association with such skeletal and developmental
anomalies as syndactyly, polydactyly, and arachnodactyly.
 NERVE
THICKENING
 NERVE
THICKENING
 SKIN

• Spider angiomas in alcohol abuse;

• Erythema chronicum migrans in lyme disease;

• Purpura and petechiae in ttp , meningococcemia, and rocky mountain spotted fever.

• Livedo reticularis in apla and cryoglobulinemia;

• GPE IN NEUROPATHY CASE
• GPE IN ATAXIA CASE
• GPE IN MYOPATHY CASE
 SKIN

• Hyperpigmentation in nelson’s syndrome, addison’s disease;

• Other important findings include signs of scleroderma;

• Scars, needle marks or other evidence of intravenous substance.

• Numerous dermatologic manifestations of the neurocutaneous syndromes.

 DERMATOMYOSITIS- SKIN MANISFESTATIONS
• Grotton’s Sign:

– An erythematous, scaly eruption over the extensor surfaces of the metacarpophalangeal joints and
digits
 HELIOTROPE RASH:

– A reddish-purple eruption on the upper

eyelid
– accompanied by swelling of the eyelid
– Most specific rash in DM
– Only present in a minority of patients.
SHAWL SIGN AND V SIGN
 “MECHANIC’S HANDS”

• Roughened, cracked skin at tips and

lateral aspects of the fingers resulting
in irregular, dirty-appearing lines
 NAIL CHANGES

Capillary Loop Dilatation

 NAIL CHANGES

Periungual Erythema
 PHAKOMATOSES - NEUROCUTANEOUS SYNDROMES

• The phakomatoses or neurocutaneous syndromes are a phenotypically and genetically diverse group

of multisystem disorders that primarily affect the skin and central nervous system.
 LIST OF PHACOMATOSES

• Neurofibromatosis type1
• Neurofibromatosis type 2

• Noonan syndrome with multiple lentigines(LEOPARD syndrome)

• Tuberous sclerosis complex
• Ataxia telangiectasia
• Incontinentia pigmenti
 SKIN – NEUROCUTANEOUS MARKERS

1. Café- au- lait spots

• Dark brown hypermelanotic macules with smooth or irregular borders, commonly seen in back,
buttocks and trunk.
• Seen in neurofibromatosis.
• Also seen in Albright's synd, Fanconi anemia, Bloom syndrome.
• - Axillary Freckling - similar small round spots in axilla and inguinal areas are pathognomonic in NF.
CAFÉ AU LAIT SPOTS. PIGMENTED MACULES(>1 CM) ON THE TRUNK.

PLEXIFORM NEUROMA SEEN.

 CRITERIA 2

6/> CALMs : > 5 mm in diameter: Prepubertal individuals

> 15mm in greatest diameter in Postpubertal individuals
Café – au – lait spots or patches
 Criteria 3 (Skin)

Freckling in the axillary or inguinal regions

 2. NEUROFIBROMAS

• These are multiple subcutaneous papules and nodules in NF.

• Two types-

• Fusiform neurofibromas – Firm discrete subcutaneous nodules attached to a nerve.

• Plexiform neurofibromas – Subcutaneous elastic tumors, feel like a bag of worms over face, scalp,
neck and chest.
NEUROFIBROMA
 3. LISCH NODULES

• Slightly raised well circumscribed melanotic iris nodules seen by slit lamp in 90% of NF pts.
 4. ADENOMA SEBACEUM (FACIAL
ANGIOFIBROMA
• Numerous discrete smooth glistening round rubbery papules pinhead to pea size, over butterfly area
in the face and nasolabial folds, cheeks, nose, chin.

• These are slow growing angiofibroma.

• Seen in 90% of pts in Tuberous Sclerosis (TS) by 4 yrs of age.

• DD – Acne vulgaris.
ADENOMA SEBACEUM:MULTIPLE ANGIFIBROMAS OF THE
FACE .ERYTHEMATOUS PAPULES ON THE LOWER HALF OF
FACE-CHEEKS,NASOLABIAL FOLDS, SIDES OF THE NOSE
AND CHIN.
ADENOMA SEBACEUM
 5. SHAGREEN PATCH

• These are Connective tissue naevi.

• Skin colored irregular lumpy cobblestone- like plaques in lumbosacral areas which grow to the
size of a palm.
• Satellite papules grow around a central plaque with orange peel like indentation.
• Seen in 70% of pts in TS.
• Also seen in MEN1, Cowden syndrome and Hunter's syndrome.
SHAGREEN PATCH- Leathery plaques of
subepidermal fibrosis,usually situated on lumbosacral
trunk.
SHAGREEN PATCH.
 6. SUBUNGUAL & PERIUNGUAL FIBROMAS

• Firm pink to skin colored papules, nodules from under the nail bed in toe & finger nails. Seen in

90% of pts in TS.

SUBUNGUAL FIBROMA.
 7. ASH LEAF MACULES

• Hypomelanotic polygonal macules grow upto 5 cm over trunk and buttocks.

• >3 lesions are seen in 90% of TS patients.

• Also seen in MEN1, vitiligo, nevus anemicus, koyanagi syndrome

ASH LEAF SPOTS. Hypopigmented macules. The average size is 1 to
3 cm, and the common shapes are oval, polygonal, and lance-ovate,
whereas the less common shapes are dermatomal and confettilike.
Ash leaf spots. "confetti" macules. Multiple, discrete, small, confetti- like, hypopigmented
macules of variable size on the leg.
Detection of ash leaf spots may require Wood's lamp
examination(UVA), especially in fair- skinned individuals. An ash
leaf spot on the scalp will result in poliosis, which is a
circumscribed patch of gray-white hair.
 8. PORT WINE STAIN (FACIAL NEVUS FLEMMENS

• Congenital macular lesions light pink initially, can progress to red purple nodules over the

skin, choroidal and leptomeningeal vessels and other body areas.

• Seen in Sturge Weber syndrome.

 STURGE WEBER SYNDROME

Port Wine Stain / Nevus flammeus

 RIGHT FACIAL PORT-WINE BIRTHMARK IN THE
DISTRIBUTION OF THE FIRST AND SECOND DIVISIONS OF
CRANIAL NERVE V,CAUSING FACIAL HEMIHYPERTROPH
 9. OCULAR TELANGIECTASIA

• Dilated blood vessels over sclera.

• Seen after development of ataxia, around 3 – 6 yrs of age in patients with Ataxia Telangiectasia..
 10. CUTANEOUS TELANGIECTASIA

• Venous angiomas over the bridge of nose, butterfly area of the face, ante cubital area, neck, popliteal

fossa.

• Seen in 90% of pts by 5-6 yrs in AT (louis- Bar disease) and also in Von Hippel Lindau disease
IN INCONTINENTIA PIGMENTI, CUTANEOUS HYPERPIGMENTED
MARKINGS FOLLOW THE LINES OF BLASCHKO
 SPINE

• Note any deformity, abnormality of posture or motility, localized tenderness, or muscle spasm.

• Tuberculosis and neoplasms of the spine may cause a marked kyphosis (gibbus)

• Muscular dystrophy often results in an increased lumbar lordosis.

• Scoliosis is common in syringomyelia and friedreich’s ataxia.

 SPINE

• Localized rigidity with a slight scoliosis and absence of the normal lordosis are frequent symptoms of

lumbosacral radiculopathy.

• Tenderness to percussion over the spinous processes, using either the fist or a reflex hammer, can

occur with localized processes such as spinal epidural hematoma or abscess.

DIMPLING OF THE SKIN OR UNUSUAL HAIR GROWTH OVER
THE
SACRUM SUGGEST SPINAL DYSRAPHISM
INDICATORS OF SPINAL
DYSRAPHISM
 CVJ ANOMALIES

• The craniovertebral (or craniocervical) junction(CVJ) is a collective term that refers to the occiput(posterior

skull base), atlas, axis, and supporting ligaments.

• It is a transition zone b/w a mobile cranium & spinal column.

 PARTS OF CV JUNCTION INCLUDE

 The Occiput
 First Cervical Vertebra (Atlas)
 Second Cervical Vertebra (Axis)
 Their articulations and
 Connecting ligaments
NEURAL TRUCTURES RELATED
ARE –
CAUDAL BRAINSTEM
(MEDULLA)

Fourth ventricle
Rostral part of spinal cord
Lower cranial (9,10,11 {only N that passes through the FM}, 12) & upper cervical
nerves (The C1(SUBOCCIPITAL NERVE) , C2, and C3 nerves with both rami).
In cerebellum, only the tonsils, biventral lobules &the lower part of the vermis (nodule,
uvula & pyramid) ,
 CLASSIFICATION

•Bony Anomalies Major Anomalies

1. Occipitalization
2. Basilar Invagination
3. Dens Dysplasia
4.Atlanto- axial dislocation.
5. Platybasia Minor Anomalies
6. Dysplasia of Atlas
7. Dysplasia of occipital condyles, clivus, etc.
8. Soft Tissue anomalies

9.Arnold-Chiari Malformation Syringomyelia Syringobulbia

 CLINICAL FEATURES

Cervical symptoms and signs- pain suboccipital region rediating

vertex,stifness in 85%

Myelopathic Features- long tract involvement and wasting

CN involvement- IX, X,XI,XI inI 20%

Vascular in15% Transient Attack of V-B insufficiency

Sensory symptom of post.column involvement.
Cerebellar symptoms/signs- Nystagmus, Ataxia,intention tremor, dysarthria

Features of Raised ICT- usually seen in Pts. Having basilar impresssion

and/or ACM
ATAXIA,ATROPHY,SYRINX –POOR PROGNOSIS
 KLIPPEL- FEIL SYNDROME

• Described patients who had a short, webbed neck; decreased range of motion (ROM) in the cervical

spine; and a low hairline.

• In majority of normal persons, hairline stops at or above the C4 spine.

• Type 1- Massive fusion of cervical and often upper thoracic vertebra. Associated with short neck,

low hairline and restricted neck movements

• Type 2- associated with fusion of several cervical vertebra

• Type 3 – associated with fusion of 2 cervical vertebra.

KLIPPEL FEIL SYNDROME
 REFERENCES
• Dejongs neurologic examination , 7th edition

• Bickerstaffs neurology examination, 6th edition

• Tena Rosser, MD . Neurocutaneous Disorders , review CONTINUUM AAN.

 THANK YOU

ganeshgoudam4@gmail.com

9380906082