NEUROLOGY

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HEADACHE

Headache is the most common neurological symptom.

AETIOLOGY1. Tension headache2. Migraine headache3. Cluster headache4. Uncontrolled hypertension5. Meningitis6. Intracranial haemorrhage

Subarachnoid haemorrhageIntracerebral haemorrhageAcute and chronic subdural haematoma

7. Raised intracranial pressure8. Post traumatic9. Temporal arteritis10. Referred pain from

Neck such as cervical spondylosisEyeSinusTeethTemporo mandibular joint

SEVERE HEADACHE Migraine and cluster headache Meningitis Subarachnoid haemorrhage

TENSION HEADACHEFeatures

Most common type of headache and experienced some time by most of the population Usually constant and generalised but often radiates forward from the occipital region,

described by the patient as dullness, tightness or pressure which may persist for days or weeks

Usually less severe during the early part of the day, worse as the day goes on and often does not respond well to analgesics and other drugs

Photophobia, phonophobia and nausea may be present Other features of neurological disease are absent Pathogenesis is unknown but emotional strain or anxiety is a common precipitant There may be underlying depressive illness or other psychiatric disorders

Management Patient assurance that there is no serious illness Analgesics to control pain Patients may benefit with anti anxiety drugs and low dose amitriptyline

MIGRAINEThree times more common in womenPathogenesis

Initial vasoconstriction followed by vasodilation Headache usually occurs due to vasodilatation of extracranial vessels

Types Classic: with aura Common: without aura Migraine variants: retinal, ophthalmoplegic, hemiplegic, basilar

Features Headache attack lasts 4 to 72 hours Headache has at least two of the following characteristics

Unilateral locationPulsating quality

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Moderate or severe intensityAggravation by routine physical exercise

During headache at least one of the following occursNausea and/or vomitingPhotophobiaPhonophobia

At least 5 attacks occur fulfilling the above criteria History, physical examination and neurologic examination do not suggest any underlying

organic disease

Migraine may be precipitated by stress, food such as chocolate and alcohol, menstruation and drugs like oral contraceptives.TreatmentAbortive

Rest in a quiet and dark room Analgesics + antiemetics (paracetamol/naproxen + metoclopramide/ Domperidone) Triptans (sumatriptan, zolmitriptan, rizatriptan) are potent vasoconstrictors of extracranial

vessels and may be started at the time of attack and repeated at 2 hours; maximum dose 5 to 6 times/week

Preventive Propronolol/ amitriptyline, used in alone or in combination Pizotifen Sodium valproate Verapamil Flunarezine

RAISED INTRACRANIAL PRESSUREAetiology

Intracranial space occupying lesionsTumor, abscess, tuberculoma, toxoplasmosis, lymphoma

Intracranial haemorrhageSubdural haematomaIntracerebral haemorrhageSubarachnoid haemorrhage

Meningitis/meningo encephalitis Hypertensive encephalopathy Benign intracranial hypertension

FeaturesCardinal features

Headache Vomiting Papilloedema

Other features: bradycardia, decreased respiratory rate, 6th cranial nerve palsy

Headache is Worse in the morning but gradually improves throughout the day May be associated with vomiting in the morning Dull ache in character Aggravated on bending forward Aggravated with coughing and straining May be relieved by analgesics

INVESTIGATIONSDirected by history and physical examination.CT and MRI (with and without contrast) of the brain are the used to identify intracranial pathology.

TREATMENT

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According to cause.

UPPER MOTOR AND LOWER MOTOR NEURON LESIONS

DEFINITION Upper motor neuron

Pyramidal cells and their fibres extending to motor nuclei of cranial nerves in the brainstem and the anterior horn cells of the spinal cord are known as upper motor neurons

Lower motor neuronStarts from the motor nuclei of the cranial nerves in the brainstem and anterior horn cells of the spinal cord and extend to the innervated muscles.

Differences between upper and lower motor neuron lesionUpper motor Lower motor

Muscle bulk Normal or slightly reduced Markedly reducedFasciculation Absent PresentTone Spastic (increased) Flaccid (decreased)Tendon reflexes Exaggerated

Clonus may be presentDecreased or absentClonus absent

Plantar response Extensor Flexor or absent

Extensor plantar response (Babinski’s sign)Dorsiflexion of great toe and fanning out of the other toesCauses

Upper motor neuron lesion Coma of any cause Following a seizure (post ictal period) Pes cavus Less than 12 months of age

TERMS Paresis: weakness Plegia: paralysis

Monoplegia: paralysis of one limbHemiplegia: paralysis of one half of the body including upper and lower limbsParaplegia: paralysis of both lower limbsQuadriplegia: paralysis of all 4 limbs

INVOLUNTARY MOVEMENTSTYPES

Tremor Chorea Athetosis Dystonia Myoclonus Ballism Tics

TREMOR

Rhythmic oscillating movement of a limb or a part of the limb or the head.

TYPES

Resting tremorCharacteristically pill rolling, seen in patients with Parkinsonism and datura poisoning.

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Action tremor Physiological tremor: (8 and 12 Hz) can be identified in normal subjects Exacerbated physiological tremor Essential tremor (familial, senile) Parkinsonism (resting tremor more common) Wilson’s disease Postural tremor Intention tremor (cerebellar disorders)

Physiological tremor may be exacerbated by Anxiety Fatigue Endocrine: thyrotoxicosis, hypoglycaemia, phaechromocytoma Drugs: salbutamol, aminophylline, caffeine, tricyclic antidepressants, sodium valproate Toxins: arsenic, lead, mercury Alcohol withdrawal

Flapping tremor Liver failure Renal failure Hypercapnia Drug toxicity (phenytoin) Acute focal parietal or thalamic lesion

CHOREA

Involuntary, sudden, non repetitive, quasi purposeful jerks or fragments of movements are termed as chorea. These may affect the face, tongue or limbs and are provoked by active movements.

AETIOLOGY Rheumatic fever Pregnancy (chorea gravidarum) Hereditary: Huntington’s chorea, Wilson’s disease Cerebral birth injury Drugs: oral contraceptives, phenothiazines, dopamine agonists Senile chorea Rare: SLE, thyrotoxicosis, polycythaemia rubra vera

ATHETOSIS

Slow, coarse, writhing movements of the limbs, face or tongue.

AETIOLOGY Basal ganglia damage: cerebral palsy, kernicterus Drugs: phenothiazine Wilson’s disease

DYSTONIA

Movement disorder in which a limb or the head involuntarily takes up an abnormal posture.

AETIOLOGY Drugs:

Acute dystonic reaction: metoclopromide, phenothiazineChronic tardive dystonia: chronic phenothiazine use

Cerebral palsy Wilson’s disease

MYOCLONUS

Myoclonus is sudden, involuntary jerking of a single muscle or a group of muscles.

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AETIOLOGY Benign essential myoclonus Epilepsy Static myoclonic encephalopathy (following cerebral anoxia)

BALLISM (HEMIBALLISMUS)

Wild flinging movement, usually of an upper limb following an attempt to move the limb. It is usually unilateral.

AETIOLOGY Lesion in the subthalamic nucleus (haemorrhage, infarction)

TICSRepetitive twitching movement of the face, neck or hands which are frequent and irritating. Tics can be temporarily controlled voluntarily.

AETIOLOGY Transient in children Stroke Trauma Drugs: levodopa, carbamazepine

CEREBELLAR DISORDERS

AETIOLOGY Vascular: infarction and hemorrhage Demyelinating -multiple sclerosis. Tumors- primary & secondary Infection -- chicken pox encephalitis in children.

-- cerebellar abscess. Alcohol. Drugs- anticonvulsants Non metastatic manifestations of tumors Hereditary ataxia. Myxoedema

PHYSICAL SIGNSClinical presentation is ipsilateral.

Dysmetria: the movement is imprecise in direction, force and distance Dyssynergia: movement is broken up into components Intention tremor: increased tremor as the finger approaches the target Dysdiadochokinesis: inability to perform rapidly alternating movements Nystagmus: coarse horizontal nystagmus in lateral cerebellar lesion Dysarthria: scanning speech Hypotonia Pendular tendon jerk (knee) Cerebellar ataxia: broad based gait and the patient tends to fall towards the side of lesion,

equally severe with eyes open or closed

Tests commonly used Upper extremity:

Finger nose testAlternating pronation and supination of the hands

Lower extremity: heel shin test

GAIT DISORDERS Hemiplegic gait: the leg describes a semicircle with toes scraping the floor (circumduction)

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Paraplegic:spastic gait affecting both legs (scissors) Parkinson’s gait(festinant gait): slow shuffling gait with small steps; unable to stop when

pushed forward (propulsion) or backwards (retropulsion) Cerebellar (drunken gait or reeling gait): broad base gait and the patient tends fall towards the

side of lesion, equally severe with eyes open or closed Sensory: stamping gait; the patient walks with wide base continuously looking at the ground

raising his feet high in the air and stamping them onto ground which is greatly increased with eyes closed

Waddling gait: feet planted wide apart, body is tilted backwards and sways from side to side like a duck and occurs in muscular disease

Foot drop:foot is lifted in the air and slapped onto the ground seen in peroneal nerve palsy and peripheral neuropathy

CRANIAL NERVES

OLFACTORY NERVETerms

Anosmia: absence of sense of smell Parosmia: distorted smell Hallucination: perception of smell in its absence

CausesAnosmia

Local disorder in the nose: common cold, allergic rhinitis, foreign body Fracture cribriform plate ethmoid bone Frontal lobe tumor Basal meningitis

Parosmia Pregnancy Hysteria

Hallucination Temporal lobe epilepsy

OPTIC NERVEVisual acuity impairment (common causes)

Refractive error Cataract Vit A deficiency Retinopathy Glaucoma Optic atrophy

Visual field defect Scotoma

Central: optic neuritisParacentral:choriod/retinal disorders

Homonymous hemianopiaStroke, mass lesion

Bitemporal hemianopiaPituitary TumorCraniopharyngioma

Concentric contraction of visual fieldChorioretinitis, glaucoma, long standing papilloedema

Colour vision defectCommon defect: red- greenLess commonly: blue-yellowMost of these are inherited sex linked recessive disordersIt can also occur in multiple sclerosis

Fundus examinationPapilloedema

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Initial sign is the loss of normal venous pulsation at the optic disc followed by hyperaemia. The margin of the disc then becomes indistinct, the whole disc is raised and there may be retinal haemorrhage.Causes

Raised intracranial pressure Space occupying lesions, hydrocephalus, meningitis/meningo encephalitis

Benign intracranial hypertension Hypertension (grade IV retinopathy) Central retinal vein occlusion Cavernous sinus thrombosis Hypercapnia

Optic atrophyLoss of optic nerve fibre makes the disc appear pale Causes

Optic neuritis Ischaemia: central retinal artery occlusion Long standing papilloedema Compression of the optic nerve Toxins: methanol Degenerative condition

For hypertensive and diabetic retinopathy please look at respective chapters

OCULOMOTOR 3rd cranial nerveTROCHLEAR 4th cranial nerveABDUCENT 6th cranial nerveTesting

Eyelids Squint Nystagmus Ocular movements Pupillary light reflex and accomodation

EyelidsPtosisDrooping of the upper eyelid

Congenital Acquired

3rd nerve palsyHorner’s syndromeMyasthenia gravisMuscular dystrophy

ExophthalmosProtrusion/prominence of the eyeball; the sclera is visible between the eyelids and corneal limbus

Graves’ disease Retro orbital tumor deposit Cavernous sinus thrombosis and carotico cavernous fistula

Lid Lag and lid retraction Hyperthyroidism

SquintThe visual axis of two eyes fail to converge to a point of focus

Concomitant (defective vision)No limitation of movement of eyes when tested individuallyNo double vision

Paralytic (extra ocular muscle weakness)Limitation of movement of eyes when tested individuallyDouble vision on looking towards the side of paresis/paralysis

NystagmusInvoluntary oscillatory movement of eyeballs

Vertical brainstem disease Horizontal brainstem disease

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vestibular diseasecerebellar disease

Rotary eye ball disease Ataxic medial longitudinal bundle in brainstem lesion

Eye movements 6th nerve: abduction of the eyeball 4th nerve: downwards and inwards All other movements: 3rd cranial nerve

PupilsSizeDilated: mydriatics

3rd nerve palsydatura poisoningbrain death (not reactive to light)

Pinpoint: pontine haemorrhagenarcotic overdosepilocarpine

Shape Irregular: old iritis

Arygll Robertson pupilDirect/Consensual Light ReflexAbsent in lesions of optic and oculomotor nerveAccommodation

Convergence of eyeballs Constriction of pupils Increase in anterior convexity of lens

Absent in case of 3rd nerve palsy, autonomic neuropathy and acute glaucoma

Arygll Robertson pupil (neurosyphilis) Accommodation normal Direct/consensual absent

3rd Cranial nerve lesion Ptosis Lateral and downward deviation of eye ball Limitation of movement + diplopia Pupil: dilated

4th Cranial nerveDifficulty in looking downwards and inwards

6th Cranial nerve Medial deviation of the eyeball Limitation of lateral movement + diplopia

Aetiology of 3rd, 4th and 6th cranial nerve palsy Idiopathic Diabetes mellitus Vasculitis Brainstem disease Basal meningitis Cavernous sinus disease Tumor/fracture greater wing of sphenoid bone Orbital tumors Raised intracranial pressure (6th cranial nerve palsy as a false localizing sign)

Horner’s syndromeParalysis of cervical sympathetic nerve.Features

Slight ptosis Miosis Anhidrosis Enopthalmos

Causes Pancoast tumor of lung

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Neck surgery Trauma to neck Spinal cord lesions

TRIGEMINAL NERVEMotorMandibular division, supplies the following muscles

Temporalis muscle Masseter muscle Medial and lateral pterygoid muscles

Loss of function leads to deviation of the jaw to the same side and difficulty in mastication.Jaw jerkExaggerated in upper motor neuron lesion such as cerebro vascular disease and motor neuron disease.Conjunctival and corneal reflexesAbsent in lesions of Ophthalmic division lesion and brain deathSensory

Ophthalmic division: scalp, forehead, upper eyelid, side of nose Maxillary division: cheek and upper lip Mandibular division: lower part of the face, general sensation over tongue

Aetiology Ophthalmic division: herpes zoster Maxillary division: trigeminal neuralgia Mandibular division: trigeminal neuralgia

loss of sensation over lower part of face due to incorrect lower molar tooth extraction

Others: multiple sclerosisposterior cranial fossa tumorvascular- posterior inferior cerebellar artery lesiontrauma base of skull

Trigeminal neuralgia Usually occurs in middle age and elderly Paroxysmal lancinating pain along the distribution of maxillary and mandibular division of

trigeminal nerve lasting few seconds or 1 to 2 minutes Pain may be spontaneous or precipitated by chewing, speaking or smiling Pain tends to recur during day or night several weeks at a time Physical examination is normal Treatment is with carbamazepine; alternative drugs are gabapentin and phenytoin

FACIAL NERVESupplies the muscles of the face and stapediusIt also carries taste fibres from the anterior 2/3rds of the tongue via the chorda tympami

AetiologyUpper motor neuron lesion

Cerebro vascular disease Mass lesion: tumor

tuberculomabrain abscess

Demyelinating disease: multiple sclerosisLower motor neuron lesion

Idiopathic (Bell’s Palsy) Pontine lesion Cerebello pontine angle tumor Middle ear disease Herpes zoster of geniculate ganglion (Ramsay Hunt syndrome) Guillain Barre syndrome Sarcoidosis Malignant parotid tumors

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Difference between upper motor and lower motor facial nerve palsyUpper Motor Lower MotorOpposite side of the face involved (contralateral)

Same side of face involved (ipsilateral)

Lower part of one side of the face is affected

Whole of one side of the face is affected

Able to wrinkle forehead Unable to wrinkle foreheadAble to close eyelids Unable to close eyelids with upward

rolling of the eyeball (Bell’s phenomenon)Taste sensation normalHyperacusis absent

Loss of taste on one side of the tongue and hyperacusis may be present

May be associated with other features of upper motor neuron lesion in upper and lower extremity

Neurological features in upper and lower limbs are usually absent

Bell’s palsyIdiopathic lower motor facial nerve palsy.Clinical presentation

Affects patients of all ages and both sex Symptoms develop over few hours Patient may complain of pain behind the ears prior to weakness Ipsilateral weakness of whole of one side of the face Difficulty in closing the eyelids Dribbling of saliva from the angle of the mouth on the affected side Accumulation of food in the mouth on the affected side Loss of taste from anterior 2/3rd on one side of the tongue Hyperacusis may be present Occasionally patients complain of numbness of the affected side of the face On examination: lower motor facial palsy

no sensory impairmentTreatment

Oral prednisolone 60 mg daily for 5 days then tapered during the next 5 days Oral acyclovir combined with prednisolone may be better than prednisolone alone Physiotherapy of facial muscle Paper tape to depress eyelid during sleep and to prevent corneal drying

PrognosisMost patients recover fully over a period of 3 to 6 months. Approximately 5 to 10 % do not recover.

VESTIBULOCOCHLEAR NERVECochlear: Innervates cochlea and subserves hearingVestibular Supplies labyrinth and semicircular canals

Responsible for posture, balance and equilibrium Cochlear dysfunction

Hearing loss Tinnitus

Vestibular dysfunction Vertigo Difficulty in gait and balance

Hearing LossConductive deafness

External canal: wax, foreign body, otitis externa Tympanic membrane perforation Middle ear disease

Sensorineural deafnessCochlear or retrocochlear pathology

Congenital rubella Meningitis Trauma Drugs: aminoglycosides, quinine, aspirin

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Acoustic neuroma Meniere’s disease

VertigoSubjective spinning or rotatory sensation of the patient or the surroundingPeripheral

Benign positional vertigo Acute viral labyrinthitis Motion sickness Middle ear disease Drugs: anticonvulsants, antihypertensive Acoustic neuroma

Central Brainstem disease: vascular, demyelination, space occupying lesion

GLOSSOPHARYNGEAL Motor: stylopharyngeus muscle Sensory: general and special sensation to posterior 3 rd of tongue and mucous membrane of

pharynxDisorderGlossopharyngeal neuralgiaPain similar to trigeminal neuralgia in the glossopharyngeal nerve distribution. Carbamazepine may be helpfulIn combination with other lower cranial nerves disorders

VAGUS Motor and sensory to palate, pharynx and larynx Dorsal nuclues: parasympathetic

AetiologyUpper motor

Cerebro vascular disease Motor neuron disease Multiple sclerosis

Lower motor Brainstem disease: vascular lesion (lateral medullary syndrome), bulbar polio, motor neuron

disease, syringobulbia Neoplasm: jugular foramen, posterior condylar space, retro parotid space Meningitis Diphtheria Guillain Barre syndrome Left recurrent laryngeal nerve: lesions within the chest such as bronchogenic carcinoma,

lymphoma, aneurysm of arch of aortaClinical features

Dysphagia Dysphonia: nasal intonation, hoarseness of voice due to left vocal cord paralysis Nasal regurgitation

ACCESSORYMotor supply to sternocleidomastoid and trapeziusAetiology

Lesions of the medulla and upper spinal cord:Vascular, syringomyelia/syringobulbia, polio, motor neuron disease

Jugular foramen, posterior laterocondylar space, posterior retroparotid space: tumors in combination with IX,X or XII cranial nerves

Neck: trauma, surgery

HYPOGLOSSALMotor supply to ipsilateral part of tongueAetiology

Lower brainstem disease: vascular, tumors, motor neuron disease, bulbar polio

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Extramedullary: basal meningitis, occipital bone disorders

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COMADEFINITIONS

SleepNormal physical and mental state of inactivity from which the patient can be aroused.

ConfusionMental and behavioural state of impaired comprehension, coherence and capacity to reason.

DeliriumA state of confusion accompanied by agitation, hallucinations, tremor and illusions.

StuporA state of decreased arousability in which the patient can be awakened only by vigorous stimuli, accompanied by motor behaviour that leads to avoidance of uncomfortable stimuli.

ComaIt is a deep sleep like state from which the patient cannot be aroused.

Brain deathSevere irreversible brain damage with functioning cardiovascular system seen in patients on ventilators.

COMAAetiology

Metabolic and endocrine disordersHyponatreamiaHypoglycaemia and hyperglycaemia (DKA and hyperosmolar coma)Adrenocortical insufficiencyHypothyroidism

Shock Organ failure: hepatic failure, respiratory failure, uraemia (renal failure) Cerebrovascular disease and brain tumors Infections: septicaemia, cerebral malaria, meningitis, encephalitis, brain abscess Drug and alcohol overdose Following seizure Head injury

Glasgow coma scaleEye opening Motor response Verbal responseSpontaneous 4 Obeys 6 Oriented 5To speech 3 Localises 5 Confused conversation 4To pain 2 Withdraws 4 Inappropriate 3Nil 1 Abnormal flexion 3 Incomprehensible sounds 2

Extensor response 2 Nil 1Nil 1

Coma score: E+M+V Mechanism

Diffuse brain dysfunction Brain stem lesion: haemorrhage, infarction, tumors, which inhibits reticular activating system Brain stem compression: inhibits reticular activating system

ManagementImmediate management

Ensure ABC (airway, breathing and circulation)Clear airway if secretions are present Breathing: if absent start artificial respiration with mouth to mouth breathing and intubate if necessaryCirculation: if pulse is absent start cardiopulmonary resuscitation (CPR)

Check pupils: whether dilated and fixed Check for evidence of trauma/head injury

Assessment Obtain history from accompanying relatives or friends General examination of the patient Neurological examination including assessment of depth of coma according to Glasgow Coma

Scale

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History Fever, headache, diabetes mellitus, diarrhoea and vomiting, jaundice, history of travel to

malaria endemic region, weakness of one side of the body, trauma, seizure, drug and alcohol intake

ExaminationGeneral examination

Vital signs: pulse, blood pressure, temperature, respiratory rate and type Jaundice, anaemia, cyanosis Dehydration

Neurological examination Mental status assessment (including response to painful stimuli) Trauma to head, neck and cervical spine Fundi: papilloedema and retinal haemorrhages Ocular movements and gaze Pupils: reacting/non reacting, size and symmetry Corneal reflex present or absent Facial nerve palsy present or absent Gag reflex Motor function and reflexes Neck stiffness and Kernig’s sign

InvestigationsDirected by history and physical examination

Blood sugar CBC, blood for malarial parasite, ICT for malaria Serum electrolytes, blood urea and serum creatinine, liver function tests Urine R/M/E and toxicology Blood culture Arterial blood gas analysis (if available) Serum TSH and T4, cortisol level Imaging

CT scan/MRI scan of brainChest x-ray

Lumbar puncture for CSF examinationTreatment

General management of unconscious patient (please see ischaemic stroke) Treatment of underlying cause

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CEREBRO VASCULAR DISEASE TYPES

Transient if the neurological deficit recovers within 24 hours Completed if the focal deficit is persistent and not worsening Evolving if the focal deficit continues to worsen after about 6 hours from onset

TRANSIENT ISCHAEMIC ATTACKDEFINITION Transient ischaemic attack (TIA) is a sudden or rapid onset of neurologic deficit caused by cerebral ischaemia. It may last for a few minutes or up to 24 hours and clears without residual signs.

AETIOLOGY Cardiac emboli

Cardiac arrhythmias: atrial fibrillation, sick sinus syndrome (SSS) Mitral valve disease (MS, MR, MVP) After myocardial infarction (mural thrombus) Infective endocarditis Prosthetic heart valves

Carotid or vertebral artery disease Atherosclerosis Dissecting aortic aneurysm Arteritis (Takayasu’s, giant cell) Vasculitis

Haematologic cause RBC disorders

Polycythemia rubra vera, sickle cell anemia, erythrocytosis, severe anemia Platelet disorders: thrombocytosis Myeloproliferative disorders: leukemias with white cell counts > 150,000/mm3 Increased viscosity (Waldenstrom’s macroglobulinemia)

Others Transient hypotension Compression of neck vessels by osteophytes Kinking of neck vessels during rotation of the head Cocaine abuse

RISK FACTORS Advanced age Hypertension Smoking

Diabetes mellitus Hyperlipidemias Obesity

The risk for stroke is highest in months immediately following the initial TIA and decreases thereafter.

CLINICAL FEATURESDetermine if the TIA involves the carotid or vertebrobasilar territory. Prognosis and therapeutic approach depend on the vascular territory involved.Characteristics of carotid artery syndrome

1. Amaurosis fugax: ipsilateral transient monocular vision loss due to small emboli in ophthalmic artery

2. Hemiparesis/hemiplegia 3. Hemisensory loss4. Slurred speech/aphasia5. Ipsilateral headache of vascular type 6. Carotid bruit may be present over the carotid bifurcation7. Microemboli, hemorrhages, and exudates may be noted in the ipsilateral retina

Characteristics of vertebrobasilar artery syndrome (4 Ds)1. Binocular visual disturbances (diplopia, blurred vision, total blindness) 2. Dizziness/vertigo, nausea, vomiting, tinnitus3. Slurred speech (dysarthria), ataxia, numbness around lips or face4. Gait dysequilibrium/ instability

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In approximately 5-10% of TIAs, patients experience symptoms that reflect abnormalities in both carotid and vertebrobasilar territories.

INVESTIGATIONS1. Routine investigations

CBC, ESR, PT, APTT, blood chemistry, fasting lipid profile VDRL, ANA (in special cases)

2. Imaging Chest x-ray: to exclude cardiac and pulmonary pathology CT/MRI scan of the brain: to exclude hemorrhage/infarct and subdural haematoma

3. Cardiac evaluation ECG: to exclude arrhythmias and recent MI Echocardiography: to exclude cardiac source of emboli 24 hour Holter monitor: to exclude arrhythmias

4. Vascular evaluation Duplex ultrasonography is the preferred noninvasive study in symptomatic patients with

TIAs in the anterior circulation and can detect ulcers, plaques, and plaque hemorrhages Carotid Doppler, alone or combined with periorbital Doppler, is useful when duplex

ultrasonography is not available Cerebral arteriography/ Magnetic Resonance Angiogram (MRA)

TREATMENT Medical therapy

Anticoagulation: initially with enoxaparin/heparin, then followed by warfarin In patients with contraindications to oral anticoagulants: (history of GI bleeding, bleeding

tendencies, severe hypertension, elderly patients with frequent falls, uncooperative patients):Aspirin: dose 75 to 150 mg/day decreases the risk of subsequent stroke by 15-30% in patients with TIAClopidogrel: 75mg once daily

Surgical therapy Carotid endarterectomy is indicated in the following settings

High grade stenosis (>70%) in symptomatic patients Multiple TIAs despite medical therapy

PROGNOSISWithin 5 years of an attack of TIA

Stroke in 30% of patients Myocardial infarction in 15% of patients

STROKE

DEFINITION Stroke can be defined as the rapid onset of a neurologic deficit involving a certain vascular territory and lasting longer then 24 hour.

CLASSIFICATION

TYPESCerebrovascular disease comprises of

Ischaemic stroke (Infarction) ThrombosisEmbolism

HaemorrhageCerebral and cerebellar haemorrhageSubarachnoid haemorrhage

OthersCortical venous and dural venous sinus thrombosis

ISCHAEMIC STROKEAETIOLOGY

Cardiac emboli: as in TIA Carotid or vertebral artery disease: as in TIA Haematologic cause: as in TIA Others: cocaine abuse

RISK FACTORSAs in TIA

CLINICAL PRESENTATIONClinical presentation varies with the cerebral vessels involvedArtery Involved Neurologic DeficitMiddle cerebral artery Hemiplegia (upper extremity and face are usually more

involved than lower extremities) Hemianesthesia (hemisensory loss) Hemianopia (homonymous) Aphasia (if dominant hemisphere is involved)

Anterior cerebral artery Hemiplegia (lower extremities more involved than upper extremities and face)

Primitive reflexes present (e. g., grasp and suck) Urinary incontinence

Vertebral and basilar arteries Ipsilateral cranial nerve findings and contralateral (or bilateral) sensory or motor deficits

Deep penetrating branches of major cerebral arteries (lacunar infarction)

Usually seen in elderly hypertensive patients and diabetics Four characteristic syndromes are possible:

Pure motor hemiplegia (66%) Dysarthria-clumsy hand syndrome (20%) Pure sensory stroke (10%)Ataxic hemiparesis syndrome with pyramidal tract signs

INVESTIGATIONSCT scan of the brain

To identify infarct or haemorrhage, assess size and extent of stroke To exclude brain abscess, tumor, subdural haematoma and subarachnoid haemorrhage

Cerebral infarction is seen on CT scan as an area of decreased density. Initial CT scan may be negative and infarct may not be evident for 2-3 days after the infarction. Routine investigations

CBC, ESR, plasma glucose, serum electrolytes, blood urea, serum creatinine, lipid profile ECG Chest x-ray

TREATMENTGeneral measuresAdmit to hospitalCare of the unconscious patient (in unconscious patients)

Check ABC (airway, breathing, circulation) IV access Nasogastric tube Urinary catheterization; avoid constipation Regular posture change every 2 hours Oral hygiene: suction and mouth wash Care of eyes: taping of eyelids, prevention of corneal damage Maintain adequate hydration and nutrition

Specific measures Enoxaparin or heparin followed by warfarin if source of embolism is present Aspirin or clopidogrel if no source of embolism is present Thrombolysis is used in early cases in advanced medical centers Blood pressure control

Continue antihypertensive medication is patient is already on itIf patient is not a known case of hypertension, treat if:Blood pressure > 220/120 mm HgHypertensive encephalopathy is presentBlood pressure generally should not drop below 150/100 since cerebral perfusion could be impaired

Treat coexisting medical conditions if present such as diabetes mellitus, infections and hyperlipidaemia

Physiotherapy/occupational therapy/speech therapy Rehabilitation

Rehabilitation should be a combined effort by the attending physician, physical therapist, speech therapist, nursing staff, social service, and the patient’s family

CEREBRAL HAEMORRHAGEAETIOLOGY

Hypertension Bleeding disorders and anticoagulation therapy Amyloid angiopathy in elderly patients Haemorrhagic conversion of ischaemic stroke

CLINICAL PRESENTATION

Usually occurs during periods of activity, often manifesting with headache, vomiting, and sudden onset of neurological deficits that can rapidly progress to coma and death

Neurologic deficits depend on the area involved (please see ischaemic stroke) Signs of raised intracranial pressure may be present (bradycardia, decreased respiratory rate,

6th nerve palsy)

INVESTIGATIONCT scan of brain

Haemorrhage appears as a zone of increased density Shifts of intracranial contents and compression of the ventricles may be present

TREATMENTMedical therapy

Admit to hospital Care of the unconscious patient, if unconscious (please see ischaemic stroke) Control of hypertension:

Continue antihypertensive medication if patient is already on itMaintain systolic blood pressure between 140-160 mm Hg preferably using shorter acting drugs Lower blood pressure may reduce cerebral edema, but it risks promoting border zone ischaemia (penumbra)

Treatment of cerebral edema with mannitol, 1-1.5 g/kg of a 20% solution given IV over 30 min

Maintain adequate hydration and nutrition Treat coexisting conditions such as diabetes mellitus and infections Physiotherapy

Surgical therapyEvacuation of hematoma is indicated in the following situations: Non-comatose patients with cerebellar hemorrhage > 3 cm in diameter Patients with surgically accessible cerebral hematoma that produces progressive signs of

temporal lobe herniation or refractory intracranial hypertension Signs of brainstem compression Progressive deterioration in the clinical status of the patients

SUBARACHNOID HAEMORRHAGE (SAH)

AETIOLOGY Ruptured congenital saccular (berry) aneurysm on the circle of Willis: commonest cause

Ruptured AV malformation Bleeding disorders Ruptured mycotic aneurysm

CLINICAL PRESENTATIONSymptoms

Generalized sudden severe headache that radiates into the posterior neck region, and is worsened by neck and head movements

Altered mental status/loss of consciousness Vomiting and photophobia

Signs Level of consciousness varies from normal to deeply comatose Fever and neck rigidity are present or usually develop with 24 hours Fundi may show papilloedema and/or retinal hemorrhage Cranial nerve abnormalities may be present (diplopia, dilated pupil) Focal neurologic signs usually are absent

INVESTIGATIONS CT scan of head confirms the presence of subarachnoid blood localized to the basal cisterns or

extending intracerebrally or in the ventricles; a fresh haemorrhage produces an area of increased density. The scan may be normal if done more than 48 hr after the SAH or if the hemorrhage is small

Lumbar puncture (LP) is indicated if a CT scan of the head is not available or if CT is negative and the index of suspicion is high

Fundus examination to exclude papilloedema must be done before LP In SAH the CSF will be uniformly grossly bloody, whereas in a traumatic LP the number of RBCs decreases progressively from tube 1 to tube 4. More reliable, is the presence of xanthochromia in the CSF

Four vessel conventional angiography (both carotids and vertebral) is necessary to determine the best therapeutic approach, medical or surgical

TREATMENTTreatment of SAH varies with the patient’s clinical status, the location and surgical access of the aneurysm Medical management

Strict bed rest in a quiet darkened private room with cardiac monitoring (frequent cardiac arrhythmias)

Control of headache with paracetamol and codeine The patient should avoid all forms of straining (stool softeners and mild laxatives are

indicated to prevent constipation) Maintain systolic pressure in the range of 140-160 mm Hg Start nimodipine within 96 hours, 60 mg every 4 hours for 21 days to reduce vasospasm Reduce cerebral edema with mannitol Care of the unconscious patient, if unconscious (please see ischaemic stroke)

Surgical management Aneurysm repair by

Surgical clip across the neck of aneurysm by neurosurgeon Placement of platinum coils within the aneurysm by neurointerventional radiologist

Stereotactic radiosurgery Symptomatic surgically inaccessible intracranial arteriovenous malformations

COMPLICATIONS Rerupture: in untreated patients in the first month is 30% with peak in the first 7 days Hydrocephalus: progressive drowsiness or slowed mentation Vasospasm: symptomatic ischaemia or infarction occurs between 4 to 14 days, most often at 7

days characterized by decrease in mental status and focal symptoms Hyponatraemia: occurs in the first 2 weeks from excess vasopressin secretion Seizures Cardiac abnormalities: ECG changes and arrhythmias

EPILEPSY

DEFINITIONEpilepsy is a condition characterized by recurrent seizures.

A seizure is an abnormal clinical event caused by paroxysmal excessive discharge of cerebral neurons. This may result in alteration in consciousness, behavioural disturbance, abnormal motor activity, sensory and autonomic dysfunction occurring alone or in combination.

CLASSIFICATIONINTERNATIONAL CLASSIFICATION OF EPILEPSY

1. Partial seizuresA. Simple partial seizures B. Complex partial seizures C. Partial seizures evolving to secondarily generalized seizures 2. Generalized seizures A. Absence seizures

1. Typical absence 2. Atypical absence

B. Myoclonic seizures C. Clonic seizures D. Tonic seizures E. Tonic-clonic seizures F. Atonic seizures

3.Epilepsy syndromeA. A. Febrile convulsion

B. Infantile spasmC. Lennox- Gastaut.

AETIOLOGY Idiopathic: 70% Congenital anomalies Genetic disorders Cerebral palsy and perinatal injuries Space occupying lesions- primary and secondary tumors Cerebrovascular disease CNS infections: meningitis, encephalitis, tuberculoma, cerebral abscess, toxoplasmosis Head injury Metabolic disorders Drugs and toxins

A single seizure may also result from metabolic disorders such as: Hypoglycemia Hypo and hypercalcemia, hypomagnesaemia Hypo and hypernatremia Hepatic encephalopathy and uraemia

GENERALIZED SEIZURES In generalized seizures, there is diffuse disturbance of cortical function, and seizures begin more or less simultaneously in both hemispheres. Consciousness is usually impaired, and motor manifestations are usually bilateral. The interictal and ictal EEG abnormalities are bilateral and reflect a neuronal discharge that is widespread in both hemispheres.

Generalized tonic-clonic seizureProdrome

There is sometimes a nonspecific prodrome, beginning hours before onset This prodrome may consist of headache, insomnia, mood change, and irritability that occurs

before the seizureTonic phase

The initial phase is tonic contraction of the muscles of the body Patient loses consciousness Tonic contractions of the muscles of expiration and larynx produce ‘ictal cry’ Rotatory movement of the eye ball

Jerky movement of one or more limbsClonic phase

Alternating contraction and relaxation of limbs, face and trunk Frothing around the mouth Tongue biting

Post ictal Patient remains in flaccid state and is unconscious Urinary incontinence and faecal incontinence Excessive salivation with stridorous breathing and partial airway obstruction

PARTIAL SEIZURES Partial seizures begin focally in a restricted area of cortex. Partial seizures may be subdivided into three types: Simple partial seizurePatients present with

1. No alteration of consciousness2. Motor symptoms and signs 3. Sensory symptoms and signs 4. Autonomic symptoms or signs5. Psychic symptoms6. These may occur either alone or in combination

Complex partial seizure (also known temporal lobe epilepsy)Patients may present with:

1. Impaired consciousness2. Automatism: chewing, crying, laughing3. Quasi purposeful activity characterized by eating, walking, playing cards, drawing4. Ictal and post ictal amnesia

Partial seizure with secondary generalization Patients present with

1. Impaired consciousness 2. Tonic-clonic (convulsive) movements

ABSENCE SEIZURES These seizures were formerly termed petit mal seizures.Two types

Typical Atypical

Numerous attacks can occur in a day.

Typical absence seizures are characteristic of the idiopathic (primary) generalized epilepsies, characterized by lapses of consciousness that rarely last longer than 10 seconds. They may be associated with clonic movements, changes in postural tone, automatisms, and autonomic changes.

Atypical absences almost always produce motor signs, especially changes in tone. They last from 10 to 25 seconds and may be followed by postictal confusion, unlike typical absence seizures. Atypical absences more often occur on awakening and in drowsiness but are not provoked by hyperventilation.

During a typical absence seizure, the EEG shows generalized symmetric spike and slow-wave complexes repeating at a frequency of 3 to 4 cycles per second.

INVESTIGATIONSElectroenephalography (EEG)EEG is the single most informative diagnostic test and should be performed routinely. EEG helps in

Classifying the seizure type Localization of seizure focus

Neuroimaging Magnetic Resonance Imaging MRI is the preferred imaging modality for evaluating a patient with epilepsy. It is highly sensitive to identify structural abnormalities in the brain

Computed Tomography Although MRI is clearly superior, the computed tomography (CT) is still useful. Tumors larger than 1 or 2 cm in size can usually be identified. However, lesions in the temporal lobe can be missed with CT because of bony artifact. Metabolic tests

Plasma glucose, serum electrolytes, serum calcium, phosphate and magnesium Blood urea, serum creatinine and LFT CBC Lumbar puncture for CSF analysis, if necessary

TREATMENT The main goal of epilepsy treatment is to enable affected individuals to live as normal a life as possible. Anti epileptic drugs (AEDs) are given to prevent seizures and is not a cure for epilepsy. When choosing an AED, one should select an effective agent for the epilepsy syndrome.

Medical treatment should always begin with the use of a single AED. A treatment is deemed adequate provided there is a favourable response. Therapeutic serum drug levels should be targeted when starting therapy, and these levels help to assess medication compliance and dosage.

Table 1. Choice of anti epileptic drugsSeizure type First-choice antiepileptic

drugSecond choice antiepileptic drug

Simple partial, complex partial, secondarily generalized

Carbamazepine, phenytoin Lamotrigine, phenobarbital, valproic acid, gabapentin, topiramate

Primary generalized tonic clonic seizure (GTCS)

Valproic acid, clonazepam Carbamazepine, phenytoin and lamotrigine

Absence Ethosuximide Valproic acid

Myoclonic Valproic acid Clonazepan

Table 2. Pharmacokinetic data common antiepileptic drugsAntiepileptic drug Maintenance

daily dose (mg/day)

Number of

daily doses

Therapeutic

serum levels (mg/ml)

Phenytoin 300-600 1-3 10-20

Carbamazepine 400-2400 2-4 4-12

Phenobarbital 90-300 1-2 15-40

Primidone 750-2000 3-4 5-12

Valproic acid 600-4000 2-4 50-110

Ethosuximide 500-2000 3-4 40-150

Clonazepam 1-6 1-3 20-80

Felbamate 2400-3600 3-4 22-137

Gabapentin 900-4800 3-6 > 1

Lamotrigine 300-800 2 > 2

Status Epilepticus Status epilepticus (SE) is among the most frequent and serious neurologic emergencies encountered by the neurologist.

SE is defined as clinical or electrographic seizures lasting at least 30 minutes, or serial seizures during which consciousness is not regained between seizures. However, for practical purposes, seizures that last long enough to raise concern about an altered physiologic state (for example, tonic-clonic seizures

lasting more than 5 minutes) should be treated as SE. Although any seizure type can develop into SE, tonic clonic seizures most often occur in SE.

Status epilepticus is caused by acute CNS insults, such as stroke, infection, hemorrhage, and traumatic brain injury, and systemic derangements from intoxications and metabolic abnormalities. Morbidity and mortality chiefly depend on the underlying cause of the SE; for example, SE caused by AED withdrawal has a more benign prognosis than SE due to anoxic injury.

Treatment protocol for Status Epilepticus Start intravenous line containing isotonic saline at a low infusion rate. Call EEG laboratory (if

available) to start recording as soon as feasible Administer lorazepam 0.1-0.15 mg/kg IV (2 mg/min); if seizures persist, administer phenytoin, 18

mg/kg IV (50 mg/min) or fosphenytoin 15-20 mg/kg IV (150 mg/min). If seizures persist, give additional 7 mg/kg phenytoin or fosphenytoin IV

If seizures persist, intubate; insert bladder catheter; start EEG recording (if available); check temperature

Administer phenobarbital, loading dose of 20 mg/kg IV (100 mg/min). If seizures persist, midazolam or propofol drip can be used

PARKINSONISM

CLASSIFICATION Idiopathic: Parkinson’s disease Secondary

Drugs Neuroleptics: haloperidol, fluphenazine, prochlorperazine Anti-emetic: metoclopramide Rarely: methyldopa and lithium carbonate

Repeated head trauma Post encephalitis Toxins and industrial exposure (especially consider in the younger patient): carbon

tetrachloride, carbon monoxide, carbon disulfide, cyanide, manganese, methanol, and MPTP

PARKINSON’S DISEASE

PATHOLOGYIdiopathic degenerative process in which there is loss of pigmented dopaminergic neurons in the substantia nigra. Decreased dopaminergic inhibitory output from substantia nigra to the basal ganglia is thought to account for much of the hypokinetic motor symptoms of Parkinson’s disease (PD).

CLINICAL FEATURESCardinal features

Tremor Rigidity Hypokinesia (bradykinesia) Loss of postural reflex

Age of onset: 40 to 70 years

SymptomsTremorUsually starts as unilateral resting tremor in the upper limb decreased by action, absent in sleep and increased by emotionHypokinesia (bradykinesia)Slowness of movement which leads to difficulties in writing, dressing, and feedingRigidityStiffness affects limbs, neck and back with patients complaining that they feel “ wooden” or are moving against great resistance

Posture /gait abnormalities Patients assume a stooped posture and develop a disturbance of equilibrium leading to frequent falls

Signs General

Expressionless faceIndistinct speechFlexed postureExaggerated glabellar reflex (Myerson’s sign: blinking that does not reduce or stop with repeated tapping on the forehead)Mild orthostatic hypotension Sialorrhea: moist, greasy face with seborrheic dermatitis

TremorResting tremor, initially unilateral in the upper limb with pill rolling movement between thumb and the fingers

RigidityCog wheel mainly in the upper limb and lead pipe in the lower limb

Hypokinesia (bradykinesia)Slowness in initiating and repeating movementsImpairment of fine movements such as writing, fastening buttons

Sensory system is normal and mental function is initially intact

INVESTIGATIONS PD is a clinical diagnosis with no definitive test CT/MRI scan of head are either normal or show nonspecific atrophy Electroencephalogram, blood, and cerebrospinal studies are typically normal

DIFFERENTIAL DIAGNOSES Isolated depression: manifested mainly as psychomotor retardation, major depression may be

confused with PD, with which a reactive depression is not uncommon. Essential tremor: characterized by a postural and action tremor (not resting) of the hands and

head without bradykinesia and rigidity. It frequently decreases temporarily with ingestion of ethanol, and more than 50 per cent of patients respond to propranolol or primidone. Frequently positive family history is present.

Parkinson plus syndromes: differentiated from idiopathic PD by the presence of various associated symptoms and signParkinsonism + disturbances of ocular motility: Progressive supranuclear palsy Parkinsonism + cerebellar symptoms and signs: Olivopontocerebellar degenerations Parkinsonism + severe autonomic insufficiency: Shy-Drager syndrome

TREATMENT PD is relentlessly progressive; no treatment modalities have been shown to categorically slow disease progression. Treatment is palliative and directed at relieving symptoms.

Drug therapyLevodopa combined with carbidopa Levodopa crosses the blood-brain barrier, is converted to dopamine and acts by enhancing activity at central dopamine receptors. Carbidopa prevents peripheral conversion of levodopa and reduces side effects.Dosing: Initial dose carbidopa/levodopa 25 mg/100 mg tablet three times daily. Extended release preparations are helpful in patients who show excessive symptomatic fluctuations.

Initially drugs are used after meals to reduce side effects. Later drugs are used before meals to increase efficacy.

Other drugs Anticholinergic agents: first choice in treatment of patients with primarily tremor Bromocriptine: direct receptor agonist (primarily D2 dopamine receptors) Pergolide: direct receptor agonist (D1 and D2 dopamine receptors) Selegiline: MAO-B inhibitor

Ropinirole: newer dopamine agonist Amantadine COMT inhibitor (entacapone, tolcapone)

DietEfficacy of drugs may be improved by reducing dietary protein at breakfast and lunch in advanced stage of PD. Physiotherapy Regular exercise is helpful to the patients.

SurgerySeveral surgical procedures are currently under evaluation. Thalamotomy (reduces tremor) and pallidotomy (reduces tremor, rigidity, and bradykinesia) are in revival. Implantation of human fetal nigral cells and installation of trophic factors are under investigation.

MULTIPLE SCLEROSIS

DEFINITIONMost common demyelinating disease of the central nervous system of autoimmune etiology.

PATHOLOGY Histology shows a plaque of inflammatory demyelination most commonly located in the

periventricular areas of the brain, optic nerves and spinal cord. Initially there is an area of disintegration of myelin sheath with infiltration by lymphocytes and macrophages, followed by gliosis leaving a shrunken scar

CLINICAL FEATURESCommon features

Optic neuritis: impairment of visual acuity Relapsing and remitting sensory symptoms: paraesthesia Weakness of the lower limbs (paraparesis or paraplegia) Cerebellar ataxia Bladder and bowel dysfunction Less commonly, subacute loss of function of upper limb 6th nerve palsy, inter nuclear ophthalmoplegia, facial palsy

Diagnostic Criteria Age <60 years Symptoms or signs of deficits in two or more anatomical sites in CNS Abnormal signs are present on CNS examination which indicate white matter involvement CNS involvement in one or two patterns

Relapsing and remitting: two or more episodes lasting at least 24 hours and > one month apartProgressive: slow and / or stepwise progression over at least 6 months

No other explanation of symptoms

INVESTIGATIONSImaging

MRI is the investigation of choice and shows areas of demyelination in brain and cervical followed by thoracic spinal cord

CSF examination Cell count: increased lymphocytes in the acute phase Protein electrophoresis: oligoclonal bands of IgG in 70-90% patients between attacks

Evoked Potentials (EP) Visual, auditory and somatosensory evoked potentials show prolonged latency of specific EP.

Subclinical MS can be diagnosed by EP studies

TREATMENTSpecific treatmentRelapsing-remitting (RR) MS

Acute attacks are treated with short course of corticosteroids such as IV methylprednisolone. Steroids reduce the severity but do not influence long term outcome

Interferon 1a, Interferon 1b and glatiramer acetate may be used in stable diseaseProgressive MS

Interferon 1a, Interferon 1b and glatiramer acetate Mitoxantrone, azathioprine, methotrexate and IVIg may be used in intolerant cases

Supportive Spasticity: physiotherapy and baclofen Ataxia: clonazepam Urinary: self catheterization and ditropan Rehabilitation and social support

PARAPLEGIADEFINITION

Paraplegia is the complete paralysis of both lower limbs Paraparesis is the partial weakness of both lower limbs

AETIOLOGYSpinal cord disease is the usual cause but occasionally peripheral nerve and cerebral lesions can produce paraplegia/paraparesis.

Spinal CordNon compressiveCompressive

Peripheral nervesGuillain Barre syndrome

BrainParasagital cortical lesions Meningioma Venous sinus thrombosisHydrocephalusMultiple cortical infarcts

Spinal cordNoncompressive

Multiple sclerosis Syringomyelia Myelitis Syphilis Motor neurone disease Vascular disease of the spinal cord Subacute degeneration of the spinal cord

Compression Inflammatory lesion Vertebral neoplasms

Tuberculosis MetastasesEpidural abscess Multiple myeloma

Disc and vertebral lesions Spinal cord tumorsTrauma MeningiomaChronic degenerative lesion Neurofibroma

Intramedullary spinal cord tumors

CLINICAL FEATURES Paraparesis or paraplegia is often accompanied by sensory loss and loss of bladder and bowel

control Lesions in the cervical and thoracic part of the spinal cord are associated with features of

upper motor neuron lesion Lesions in the lumbar part of the spinal cord are associated with features of lower motor

neuron lesion

Spinal cord compressionPrincipal features of spinal cord compressionSymptoms

Radicular pain at the site of compression Weakness or paralysis (paraparesis/paraplegia) Sensory loss Sphincter disturbance

Signs Upper motor neuron lesion Sensory loss which rises to the level of compression Vertebral column tenderness on palpation initially, later there may be gibbus formation

Compression at the level of T10 causes a band of pain that radiates around the abdomen at the level of umbilicus worse on coughing and straining. Spastic paraparesis develops the rate of which depends on the underlying pathology. Numbness which commences distally in the lower limbs and rises to the level of compression is known as the sensory level. Sphincter disturbance develops, principally retention of urine and loss of bladder control.

Principal features of spinal cord compressionSymptoms

Radicular pain at the site of compression Weakness or paralysis (paraparesis/paraplegia) Sensory loss Sphincter disturbance

Signs Upper motor neuron lesion Sensory loss which rises to the level of compression Vertebral column tenderness on palpation initially, later there may be gibbus formation

Compression at the level of T10 causes a band of pain that radiates around the abdomen at the level of umbilicus worse on coughing and straining. Spastic paraparesis develops the rate of which depends on the underlying pathology. Numbness which commences distally in the lower limbs and rises to the level of compression is known as the sensory level. Sphincter disturbance develops, principally retention of urine and loss of bladder control.

Non compressive lesions of spinal cord Radicular pain is absent Sensory level may or may not be present depending on the aetiology

Transverse myelitisThe broad and somewhat vague term is used to describe acute inflammation of the cord and paraplegia/paraparesis occurring with viral infections, MS, other inflammatory and vascular conditions.

INVESTIGATIONS X-rays of the spine MRI imaging of the spine Myelogram Specific investigations according to possible cause

TREATMENTGeneral considerations

Health and morale of the patient should be considered carefully Urine and respiratory infection should be recognized and treated early Chronic renal failure is the single most common cause of death in paraplegia

Specific treatment Treatment of underlying disorder, such as relief of spinal cord compression Bladder: initially catheterization is often necessary. Many patients manage to self catheterize

or a reflex emptying develops. Free drainage is essential to avoid the complications of urinary stasis-infection, renal and bladder calculi

Bowel: constipation and faecal impaction must be avoided. Manual evacuation may be necessary in the initial stage, but later reflex emptying develops

Skin care: the risk of pressure sore is great. Meticulous care should be paid to cleanliness and turning the patient every two hours. If pressure sores develop plastic surgery repair should be considered

Lower limbs: in paralysed limbs, passive physiotherapy helps to prevent contractures. Severe spasticity may be helped by baclofen, and diazepam

Rehabilitation Many patients with traumatic paraplegia return to full or partial self sufficiency and a

wheelchair existence. With appropriate support, patients can return to active role in society

PERIPHERAL NEUROPATHY

DEFINITIONDisorder of peripheral nerves characterized by symmetrical distal involvement, manifested by sensory disturbance and/or motor weakness.

AETIOLOGY Infections: leprosy, diphtheria Metabolic disorders: diabetes mellitus, uraemia, porphyria, amyloidosis Nutritional : vitamins B1, B6, B12 deficiency Drugs and toxins: INH, ddI, vincristine, cisplatin, arsenic overdose Alcohol Autoimmune disorders: Guillain Barre syndrome, critical illness, SLE, rheumatoid arthritis,

polyarteritis nodosa, monoglonal gammopathy of unknown significance (MGUS) Neoplastic disorders: bronchogenic carcinoma Inherited: Charcot Marie Tooth disease, Refsum’s disease IdiopathicMost common Leprosy Diabetes mellitus

PATHOLOGYDemyelination and/ or axonal damage of peripheral nerves.

CLINICAL FEATURESClassic presentation: sensory/motor features in the stocking and glove areas in symmetrical distributionSensoryInitially patients develop tingling, pins and needles in the toes which progresses upwards. Later the upper extremity may be affected. There is impairment of sensation of all modalities without a dermatomal distribution. MotorLower extremityMuscle wasting and weakness with foot drop, pes cavus, and impairment of dorsiflexion of footUpper extremityMuscle wasting and weakness especially of the handsGait abnormalityResults from impairment of proprioception and motor weakness.

INVESTIGATION Nerve conduction study (motor, sensory and mixed nerve study)

Demyelination reduction in nerve conduction velocity (NCV) along the nerve, increased latency and sometimes associated with conduction block

Axonal damage reduction in amplitude of compound action potential EMG: features of acute and chronic denervation Nerve biopsy: sural nerve is preferred Investigation directed towards cause of disorder

MONONEUROPATHYDEFINITIONFocal involvement of a single nerve trunk

AETIOLOGY Trauma Compression Entrapment

Diabetes mellitus Leprosy Vasculitis Sarcoidosis

COMMON SITES OF INVOLVEMENT Cranial nerves: extra ocular nerves and facial nerve are commonly involved Ulnar nerve: produces claw hand Median nerve: carpal tunnel syndrome Posterior tibial: loss of sensation in the foot, weakness of small muscles of

foot Radial nerve wrist drop Common peroneal nerve: foot drop

CLINICAL FEATURES Motor weakness in the muscles supplied by the nerve Sensory loss in the area of distribution of the nerve There may be localized nerve thickening

MONONEURITIS MULTIPLEXDEFINITIONSimultaneous or sequential involvement of individual non contiguous nerve trunks which occurs over days to weeks.

AETIOLOGY

Diabetes mellitus Leprosy Vasculitis

Sarcoidosis AIDS/ HTLV related Hypereosinophilic syndrome

The disease may progress to produce a more confluent and symmetric involvement resembling distal symmetric neuropathy

GUILLAIN BARRE SYNDROME (GBS)

Acute, often severe polyneuropathy that is autoimmune in nature.

In about 75% of cases, GBS occurs 1 to 4 weeks after respiratory or gastrointestinal tract infection. GBS is more common in lymphoma including Hodgkin’s disease, HIV seropostive individuals and SLE.

PATHOLOGY Demyelination of peripheral nerves In severe cases, there is secondary axonal damage

PRESENTATION Duration < 4 weeks Progressive motor weakness of 2 or more limbs, of flaccid type Absent tendon reflexes Mild sensory impairment Involvement of facial and bulbar muscles is common Respiratory muscle weakness leading to respiratory failure requiring ventilatory support

occurs in 20 to 30 % of cases Autonomic dysfunction is common with cardiac arrhythmias and fluctuating blood pressure

INVESTIGATIONS CSF

Normal or slightly raised WBCElevated protein (commonly between 100 to 1000 mg/dL)Classically described as albumino-cytologic dissociationCSF is often normal in patients with symptoms < 48 hours but by the end of the week protein is usually elevated

Electrophysiologic study (nerve conduction study) shows evidence of demyelination and/or axonal damage

TREATMENTSpecific treatementInitiate specific treatment as soon as possible. Treatment after 2 weeks of diagnosis is not effective.

Both IVIg and plasmapheresis are nearly equally effective.IVIg: given in a dose of 0.4 gm/kg body weight for 5 days.Plasmapheresis: 40 to 50 ml/kg plasma exchange 4 to 5 times on alternate days (8 to 10 days) to a total of 250 ml/kg body weight

Supportive and symptomatic treatmentPatients with deteriorating condition, require monitoring in the Intensive Care Unit with attention to vital capacity, cardiovascular status, and chest physiotherapy. About 20 to 30 percents patients require ventilatory assistance.

PROGNOSISFull functional recovery occurs in about 85% patients within several months to a year. Death occurs in less than 5% patients, usually due to pulmonary complications and cardiac arrhythmias.

MENINGITIS

Meningitis means inflammation of the meninges. It may be caused by

1. InfectionVirusBacteriaFungi

2. Malignant cells3. Drugs and contrast media4. Blood (following subarachnoid haemorrhage)

Meningitis is usually used to describe inflammation due to infection.

AETIOLOGYVirus

EnterovirusCoxsackieEchoPolio

Mumps Epstein-Barr virus HIV Herpes simplex

Bacteria S. pneumoniae N. meningitidis H. influenzae type b ( in children) Listeria monocytogenes Gram negative organism Staphylococcus aureus Mycobacterium tuberculosis Treponema pallidum

Fungi Cryptococcus neoformans Coccidioides immitis Histoplasma capsulatum

ACUTE BACTERIAL MENINGITIS

S. pneumoniae is the most common cause of meningitis in adult > 20 years of age. Predisposing risk factors include pneumococcal pneumonia, acute and chronic otitis media, diabetes mellitus, alcoholism, splenectomy, head injury with basilar fracture and cerebrospinal fluid rhinorrhoea

N. meningitides occurs most commonly between the ages of 2 and 20 years. There is an initial colonisation of nasopharynx which is followed by invasive meningococcal disease

H. influenzae type b occurs primarily in unvaccinated children and adults L. monocytogenes occurs in pregnancy, diabetes mellitus, immunosuppressive therapy,

organ transplants and is often associated with impaired cell mediated immunity Enteric gram negative organisms are associated with chronic illness such as diabetes

mellitus, cirrhosis, regular alcohol intake, urinary tract infection and neurosurgical procedures

Staphylococcus aureus is associated with neurosurgical procedures

PATHOLOGYThe most common bacteria that cause meningitis, S. pneumoniae and N. meningitidis initially colonize the nasopharynx and then gain access to bloodstream. Pneumococcal pneumonia may also predispose to bacteraemia. Once in the bloodstream, the bacteria reach intraventricular choroid plexus, which allows direct access to the CSF.

In acute bacterial meningitis, the pia-arachnoid is congested with polymorphs. A layer of pus forms that may organize to form adhesions. Infection is primarily in the subarachnoid space.

CLINICAL FEATURESTriad

Fever, headache and neck stiffness (>90% cases)Usually a short history of 1 to 3 days

Others Alteration in mental status occurs in 75% cases Photophobia and vomiting are often present Kernig’s and Brudzinski’s signs are also classic signs of meningeal irritation Petechial rash often occurs in patients with meningococcal meningitis Septicaemic shock may occur

Typically sudden onset, with high fever and rigor, severe headache, photophobia and vomiting.

Causes of neck stiffness Meningitis Meningism Subarachnoid haemorrhage Cervical spondylosis Torticolis Tetanus HCR Uncooperative patient

VIRAL MENINGITIS

This is usually a benign, self limiting condition lasting 4-10 days.

FEATURES Fever Headache-frontal or retroorbital with photopbobia and pain on moving the eyes Meningeal irritation Fever may be accompanied by malaise, myalgia, anorexia, nausea, vomiting, abdominal

pain and/or diarrhoea. Mild degree of lethargy or drowsiness may be present Headache may follow for some weeks but there are no serious sequelae In viral meningitis, there is predominantly lymphocytic inflammatory reaction in the CSF

without pus formation and adhesions

TUBERCULOUS MENINGITIS

PATHOLOGY

Results from haematogenous spread of primary or post primary pulmonary disease or rupture of a subependymal tubercle into the subarachnoid space

The brain is covered in viscous exudates with numerous meningeal tubercles. Adhesions are typically seen

CLINICAL FEATURESClassically described in three stages

Prodromal stage Meningitic stage Stage of paralysis and coma

Prodromal stage: patients may have low grade fever lasting for days to weeks associated with, headache, anorexia, malaise, weight loss and general ill health.Meningitic stage: patients have features characteristic of meningitis with fever, headache, neck stiffness and altered mental status.Paralysis and coma: develops in untreated patients.

CHRONIC MENINGITIS

Chronic meningitis is defined as clinical features of meningitis lasting for 4 weeks or more.Examples: tuberculous meningitis, cryptococcal meningitis, syphilis

INVESTIGATIONS CSF CT scan of head (if required) CBC Blood culture Chest x-ray Routine chemistry

Before doing lumbar puncture, it is essential to check for papilloedema. Patients with papilloedema or focal neurological problems should have CT scan of head performed prior to lumbar puncture. Lumbar puncture should be avoided in the case of obstructive hydrocephalus or mass lesions to prevent herniation of brain.CSFCondition Appearance Cell

typecount

Glucose Protein Gram stain/ culture

Normal Clear Lymphocytes<5/mm3

>60% of blood sugar40-80 mg/dL

<45mg/dL

Viral Clear Lymphocytes10-2000

Normal 40-80mg/dL

Bacterial Turbid/purulent

Polymorphs1000-5000/mm3

Very low 50-200 mg/dL May be positive

Tuberculous Cob web formation on standing

Lymphocytes50-5000/ mm3

Low 50-300mg/dL(very high)

Fungal Clear/turbid

Lymphocytes50-500/ mm3

Low Elevated

Malignant Clear Lymphocytes0-100/ mm3

Low Normal/elevated

CBCIn acute bacterial meningitis, CBC shows polymorpho leucocytosis

Blood culturesBlood cultures should be done in acute meningitis and may be positive

Chest X-rayMay show infiltrate of pneumococcal pneumonia

TREATMENTACUTE BACTERIAL MENINGITIS

Bacterial meningitis is a medical emergency Early initiation of therapy with antibiotics is essential Hospitalize the patient, preferably in the Intensive Care Unit

Choice of antibiotic Simple regimenCeftriaxone 2gm IV 12 hourly + Ampicillin 2gm IV every 4 hours until gram stain and C/S results of CSF are available

Start with Ceftriaxone 2gm IV immediately If there is no papilloedema or focal neurological deficit, perform lumbar puncture

immediately If there is papilloedema or focal neurological deficit, do CT scan of brain to exclude mass

lesion or obstructive hydrocephalus to prevent brainstem herniation If CSF gram stain is negative continue with Ceftriaxone 2gm IV twice daily and add

Ampicillin 2gm IV every 4 hours (to cover Listeria monocytogenes) until culture and sensitivity are available. Ampicillin should be continued even if culture is negative in patients with high risk for Listeria

If CSF gram stain shows gram negative organism, continue with Ceftriaxone until culture and sensitivity results are available; change antibiotics later if required

If CSF gram stain shows gram positive diplococci (pneumococcus), add Vancomycin 1 gm IV 12 hourly until culture and sensitivity results are available. If culture shows pneumococcus resistant to Penicillin continue Vancomycin; if pneumococcus is sensitive to Penicillin, then continue with Ceftriaxone or switch to Penicillin G 4 million units every 4 hours

Dexamethasone 0.4mg/kg every 12 hours for 2 days produces better results in pneumococcal meningitis and should ideally be started 15 to 20 minutes before the 1st dose of antibiotic therapy

Duration of antibiotic therapy Meningococcal meningitis: usually 7 to 10 days Pneumococcal meningitis: usually 10 to 14 days

Supportive and symptomatic care of the patient.

PreventionMeningococcal infectionIndex case and other close contacts require prophylaxis:Rifampicin 600mg twice daily for 2 days or Ciprofloxacin 750 mg single doseAzithromycin 500 mg single doseCeftriaxone 250 mg single dose

VIRAL MENINGITISTreatment Supportive and symptomatic

TUBERCULOUS MENINGITIS9-12 months

4 drugs for two months: INH (H), Rifampicin (R), Pyrazinamide (Z) Ethambutol (E) 2 drugs for the next 7-10 months: HR Add pyridoxine to prevent INH induced peripheral neuropathy

Add oral steroids for the first 4 to 6 weeks.

COMPLICATIONS OF MENINGITISCNS

Cranial nerve palsy Hydrocephalus Focal neurological deficit Epilepsy Mental retardation and behavioural disturbance Persistent headache

Acute bacterial meningitis may also produce Shock Disseminated intravascular coagulation Renal failure Peripheral gangrene Arthritis Pericarditis