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Neurology Alim

Neurology Alim

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HEADACHE Headache is the most common neurological symptom. AETIOLOGY 1. Tension headache 2. Migraine headache 3. Cluster headache 4. Uncontrolled hypertension 5. Meningitis 6. Intracranial haemorrhage Subarachnoid haemorrhage Intracerebral haemorrhage Acute and chronic subdural haematoma 7. Raised intracranial pressure 8. Post traumatic 9. Temporal arteritis 10. Referred pain from Neck such as cervical spondylosis Eye Sinus Teeth Temporo mandibular joint SEVERE HEADACHE  Migraine and cluster headache  Meningitis  Subarachnoid haemorrhage TENSION HEADACHE Features  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

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Analgesics to control pain Patients may benefit with anti anxiety drugs and low dose amitriptyline

MIGRAINE Three times more common in women Pathogenesis  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 location Pulsating quality Moderate or severe intensity Aggravation by routine physical exercise During headache at least one of the following occurs Nausea and/or vomiting Photophobia Phonophobia At least 5 attacks occur fulfilling the above criteria History, physical examination and neurologic examination do not suggest any underlying organic disease

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Migraine may be precipitated by stress, food such as chocolate and alcohol, menstruation and drugs like oral contraceptives. Treatment Abortive  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 PRESSURE Aetiology  Intracranial space occupying lesions Tumor, abscess, tuberculoma, toxoplasmosis, lymphoma  Intracranial haemorrhage Subdural haematoma Intracerebral haemorrhage Subarachnoid haemorrhage

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Meningitis/meningo encephalitis Hypertensive encephalopathy Benign intracranial hypertension

Features Cardinal 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


INVESTIGATIONS Directed by history and physical examination. CT and MRI (with and without contrast) of the brain are the used to identify intracranial pathology. TREATMENT 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 neuron Starts 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 lesion
Muscle bulk Fasciculation Tone Tendon reflexes Plantar response Upper motor Normal or slightly reduced Absent Spastic (increased) Exaggerated Clonus may be present Extensor Lower motor Markedly reduced Present Flaccid (decreased) Decreased or absent Clonus absent Flexor or absent

Extensor plantar response (Babinski’s sign) Dorsiflexion of great toe and fanning out of the other toes Causes  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 limb Hemiplegia: paralysis of one half of the body including upper and lower limbs Paraplegia: paralysis of both lower limbs Quadriplegia: paralysis of all 4 limbs INVOLUNTARY MOVEMENTS TYPES        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 tremor Characteristically pill rolling, seen in patients with Parkinsonism and datura poisoning. 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, phenothiazine Chronic 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. 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) TICS Repetitive 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 SIGNS Clinical 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


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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 test Alternating 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) 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 NERVE Terms  Anosmia: absence of sense of smell  Parosmia: distorted smell  Hallucination: perception of smell in its absence Causes Anosmia  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 NERVE Visual acuity impairment (common causes)  Refractive error  Cataract  Vit A deficiency  Retinopathy  Glaucoma  Optic atrophy

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Visual field defect  Scotoma Central: optic neuritis Paracentral:choriod/retinal disorders  Homonymous hemianopia Stroke, mass lesion  Bitemporal hemianopia Pituitary Tumor Craniopharyngioma  Concentric contraction of visual field Chorioretinitis, glaucoma, long standing papilloedema Colour vision defect Common defect: red- green Less commonly: blue-yellow Most of these are inherited sex linked recessive disorders It can also occur in multiple sclerosis Fundus examination Papilloedema 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

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Space occupying lesions, hydrocephalus, meningitis/meningo encephalitis Benign intracranial hypertension Hypertension (grade IV retinopathy) Central retinal vein occlusion Cavernous sinus thrombosis Hypercapnia

Optic atrophy Loss 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 nerve TROCHLEAR 4th cranial nerve ABDUCENT 6th cranial nerve Testing  Eyelids  Squint  Nystagmus  Ocular movements  Pupillary light reflex and accomodation Eyelids Ptosis Drooping of the upper eyelid  Congenital  Acquired 3rd nerve palsy


Horner’s syndrome Myasthenia gravis Muscular dystrophy Exophthalmos Protrusion/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 Squint The visual axis of two eyes fail to converge to a point of focus  Concomitant (defective vision) No limitation of movement of eyes when tested individually No double vision  Paralytic (extra ocular muscle weakness) Limitation of movement of eyes when tested individually Double vision on looking towards the side of paresis/paralysis Nystagmus Involuntary oscillatory movement of eyeballs  Vertical brainstem disease  Horizontal brainstem disease vestibular disease cerebellar 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 Pupils Size Dilated: mydriatics 3rd nerve palsy datura poisoning brain death (not reactive to light) Pinpoint: pontine haemorrhage narcotic overdose pilocarpine Shape Irregular: old iritis Arygll Robertson pupil Direct/Consensual Light Reflex Absent in lesions of optic and oculomotor nerve Accommodation  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 nerve Difficulty 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 syndrome Paralysis of cervical sympathetic nerve. Features  Slight ptosis  Miosis  Anhidrosis  Enopthalmos Causes  Pancoast tumor of lung  Neck surgery  Trauma to neck  Spinal cord lesions TRIGEMINAL NERVE Motor Mandibular 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 jerk Exaggerated in upper motor neuron lesion such as cerebro vascular disease and motor neuron disease. Conjunctival and corneal reflexes Absent in lesions of Ophthalmic division lesion and brain death Sensory  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 sclerosis posterior cranial fossa tumor vascular- posterior inferior cerebellar artery lesion trauma base of skull Trigeminal neuralgia  Usually occurs in middle age and elderly


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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 NERVE Supplies the muscles of the face and stapedius It also carries taste fibres from the anterior 2/3rds of the tongue via the chorda tympami Aetiology Upper motor neuron lesion  Cerebro vascular disease  Mass lesion: tumor tuberculoma brain abscess  Demyelinating disease: multiple sclerosis Lower motor neuron lesion  Idiopathic (Bell’s Palsy)  Pontine lesion  Cerebello pontine angle tumor  Middle ear disease

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Herpes zoster of geniculate ganglion (Ramsay Hunt syndrome) Guillain Barre syndrome Sarcoidosis Malignant parotid tumors

Difference between upper motor and lower motor facial nerve palsy Upper Motor Lower Motor Opposite side of the face Same side of face involved (ipsilateral) involved (contralateral) Lower part of one Whole of one side of the face is affected side of the face is affected Able to wrinkle forehead Unable to wrinkle forehead Able to close eyelids Unable to close eyelids with upward rolling of the eyeball (Bell’s phenomenon) Taste sensation normal Loss of taste on one side of the Hyperacusis absent tongue and hyperacusis may be present May be associated with other features of upper Neurological features in upper and motor neuron lesion in upper and lower extremity lower limbs are usually absent Bell’s palsy Idiopathic lower motor facial nerve palsy. Clinical presentation  Affects patients of all ages and both sex  Symptoms develop over few hours

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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 impairment Treatment  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 Prognosis Most patients recover fully over a period of 3 to 6 months. Approximately 5 to 10 % do not recover.   VESTIBULOCOCHLEAR NERVE Cochlear: Innervates cochlea and subserves hearing Vestibular 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 Loss Conductive deafness  External canal: wax, foreign body, otitis externa  Tympanic membrane perforation  Middle ear disease Sensorineural deafness Cochlear or retrocochlear pathology

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Congenital rubella Meningitis Trauma Drugs: aminoglycosides, quinine, aspirin Acoustic neuroma Meniere’s disease

Vertigo Subjective spinning or rotatory sensation of the patient or the surrounding Peripheral  Benign positional vertigo  Acute viral labyrinthitis  Motion sickness  Middle ear disease

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Drugs: anticonvulsants, antihypertensive Acoustic neuroma Brainstem disease: vascular, demyelination, space occupying lesion

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GLOSSOPHARYNGEAL  Motor: stylopharyngeus muscle Sensory: general and special sensation to posterior 3rd of tongue and mucous membrane of pharynx Disorder Glossopharyngeal neuralgia


Pain similar to trigeminal neuralgia in the glossopharyngeal nerve distribution. Carbamazepine may be helpful In combination with other lower cranial nerves disorders VAGUS  Motor and sensory to palate, pharynx and larynx  Dorsal nuclues: parasympathetic Aetiology Upper 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 aorta Clinical features  Dysphagia  Dysphonia: nasal intonation, hoarseness of voice due to left vocal cord paralysis  Nasal regurgitation ACCESSORY Motor supply to sternocleidomastoid and trapezius Aetiology  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 HYPOGLOSSAL Motor supply to ipsilateral part of tongue Aetiology  Lower brainstem disease: vascular, tumors, motor neuron disease, bulbar polio  Extramedullary: basal meningitis, occipital bone disorders


COMA DEFINITIONS  Sleep Normal physical and mental state of inactivity from which the patient can be aroused. Confusion Mental and behavioural state of impaired comprehension, coherence and capacity to reason. Delirium A state of confusion accompanied by agitation, hallucinations, tremor and illusions. Stupor A 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. Coma It is a deep sleep like state from which the patient cannot be aroused.  Brain death Severe irreversible brain damage with functioning cardiovascular system seen in patients on ventilators. COMA Aetiology  Metabolic and endocrine disorders Hyponatreamia Hypoglycaemia and hyperglycaemia (DKA and hyperosmolar coma) Adrenocortical insufficiency Hypothyroidism  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 scale Eye opening Motor response Verbal response Spontaneous 4 Obeys 6 Oriented 5 To speech 3 Localises 5 Confused conversation 4 To pain 2 Withdraws 4 Inappropriate 3 Nil 1 Abnormal flexion 3 Incomprehensible sounds 2 Extensor response 2 Nil 1 Nil 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 Management Immediate 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 necessary Circulation: 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


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 Examination General 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 Investigations Directed 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 brain Chest x-ray  Lumbar puncture for CSF examination Treatment  General management of unconscious patient (please see ischaemic stroke)  Treatment of underlying cause



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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 ATTACK DEFINITION 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 FEATURES Determine 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 loss 4. Slurred speech/aphasia 5. Ipsilateral headache of vascular type 6. Carotid bruit may be present over the carotid bifurcation 7. 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, tinnitus


3. Slurred speech (dysarthria), ataxia, numbness around lips or face 4. Gait dysequilibrium/ instability


In approximately 5-10% of TIAs, patients experience symptoms that reflect abnormalities in both carotid and vertebrobasilar territories. INVESTIGATIONS 1. 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 TIA Clopidogrel: 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 PROGNOSIS Within 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

TYPES Cerebrovascular disease comprises of  Ischaemic stroke (Infarction) Thrombosis Embolism  Haemorrhage Cerebral and cerebellar haemorrhage Subarachnoid haemorrhage  Others

Cortical venous and dural venous sinus thrombosis ISCHAEMIC STROKE AETIOLOGY Cardiac emboli: as in TIA Carotid or vertebral artery disease: as in TIA Haematologic cause: as in TIA Others: cocaine abuse RISK FACTORS As in TIA CLINICAL PRESENTATION Clinical presentation varies with the cerebral vessels involved Artery Involved Neurologic Deficit Middle 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  Usually seen in elderly hypertensive patients and diabetics of major cerebral arteries  Four characteristic syndromes are possible: (lacunar infarction) Pure motor hemiplegia (66%) Dysarthria-clumsy hand syndrome (20%) Pure sensory stroke (10%) Ataxic hemiparesis syndrome with pyramidal tract signs INVESTIGATIONS CT 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 TREATMENT General measures Admit to hospital Care 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 it If patient is not a known case of hypertension, treat if: Blood pressure > 220/120 mm Hg Hypertensive encephalopathy is present Blood 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 HAEMORRHAGE AETIOLOGY  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) INVESTIGATION CT scan of brain  Haemorrhage appears as a zone of increased density  Shifts of intracranial contents and compression of the ventricles may be present TREATMENT Medical 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 it Maintain 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 therapy Evacuation 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

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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 PRESENTATION Symptoms  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 TREATMENT Treatment 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 DEFINITION Epilepsy 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. CLASSIFICATION INTERNATIONAL CLASSIFICATION OF EPILEPSY 1. Partial seizures A. 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 syndrome A. A. Febrile convulsion B. Infantile spasm C. 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 seizure Prodrome  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 seizure Tonic 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 limbs Clonic 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 seizure Patients present with 1. No alteration of consciousness 2. Motor symptoms and signs 3. Sensory symptoms and signs 4. Autonomic symptoms or signs 5. Psychic symptoms 6. These may occur either alone or in combination Complex partial seizure (also known temporal lobe epilepsy) Patients may present with: 1. Impaired consciousness 2. Automatism: chewing, crying, laughing 3. Quasi purposeful activity characterized by eating, walking, playing cards, drawing 4. 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. INVESTIGATIONS Electroenephalography (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 drugs First-choice antiepileptic Seizure type drug Simple partial, complex partial, Carbamazepine, phenytoin secondarily generalized Primary generalized tonic Valproic acid, clonazepam clonic seizure (GTCS) Absence Ethosuximide Valproic acid Myoclonic Second choice antiepileptic drug Lamotrigine, phenobarbital, valproic acid, gabapentin, topiramate Carbamazepine, phenytoin and lamotrigine Valproic acid Clonazepan

Table 2. Pharmacokinetic data common antiepileptic drugs Antiepileptic drug Maintenance Number of Therapeutic daily dose daily doses serum levels (mg/day) (mg/ml) Phenytoin Carbamazepine Phenobarbital Primidone Valproic acid 300-600 400-2400 90-300 750-2000 600-4000 1-3 2-4 1-2 3-4 2-4 10-20 4-12 15-40 5-12 50-110

Ethosuximide Clonazepam Felbamate Gabapentin Lamotrigine

500-2000 1-6 2400-3600 900-4800 300-800

3-4 1-3 3-4 3-6 2

40-150 20-80 22-137 >1 >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 PATHOLOGY Idiopathic 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 FEATURES Cardinal features  Tremor  Rigidity  Hypokinesia (bradykinesia)  Loss of postural reflex Age of onset: 40 to 70 years Symptoms Tremor Usually starts as unilateral resting tremor in the upper limb decreased by action, absent in sleep and increased by emotion Hypokinesia (bradykinesia) Slowness of movement which leads to difficulties in writing, dressing, and feeding Rigidity Stiffness 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 face Indistinct speech Flexed posture Exaggerated 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 Tremor Resting tremor, initially unilateral in the upper limb with pill rolling movement between thumb and the fingers Rigidity  Cog wheel mainly in the upper limb and lead pipe in the lower limb Hypokinesia (bradykinesia) Slowness in initiating and repeating movements Impairment 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 sign Parkinsonism + 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 therapy Levodopa 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) Diet Efficacy 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. Surgery Several 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 DEFINITION Most 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 FEATURES Common 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

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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 apart Progressive: slow and / or stepwise progression over at least 6 months No other explanation of symptoms

INVESTIGATIONS Imaging  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 TREATMENT Specific treatment Relapsing-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 β1a, Interferon β1b and glatiramer acetate may be used in stable disease Progressive MS  Interferon β1a, Interferon β1b 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

PARAPLEGIA DEFINITION  Paraplegia is the complete paralysis of both lower limbs  Paraparesis is the partial weakness of both lower limbs AETIOLOGY Spinal cord disease is the usual cause but occasionally peripheral nerve and cerebral lesions can produce paraplegia/paraparesis.  Spinal Cord Non compressive Compressive  Peripheral nerves Guillain Barre syndrome  Brain Parasagital cortical lesions Meningioma Venous sinus thrombosis Hydrocephalus Multiple cortical infarcts Spinal cord Noncompressive  Multiple sclerosis  Myelitis  Motor neurone disease  Subacute degeneration of the spinal cord Compression  Inflammatory lesion Tuberculosis Epidural abscess  Disc and vertebral lesions Trauma Chronic degenerative lesion

 Syringomyelia  Syphilis  Vascular disease of the spinal cord

 Vertebral neoplasms Metastases Multiple myeloma  Spinal cord tumors Meningioma 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 compression Principal features of spinal cord compression Symptoms  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 compression Symptoms  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 myelitis The 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 TREATMENT General 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 DEFINITION Disorder 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

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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  Idiopathic Most common  Leprosy  Diabetes mellitus PATHOLOGY Demyelination and/ or axonal damage of peripheral nerves. CLINICAL FEATURES Classic presentation: sensory/motor features in the stocking and glove areas in symmetrical distribution Sensory Initially 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. Motor Lower extremity Muscle wasting and weakness with foot drop, pes cavus, and impairment of dorsiflexion of foot Upper extremity Muscle wasting and weakness especially of the hands Gait abnormality Results 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 MONONEUROPATHY DEFINITION Focal involvement of a single nerve trunk AETIOLOGY  Trauma  Compression  Entrapment Diabetes mellitus Leprosy Vasculitis Sarcoidosis

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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

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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 MULTIPLEX DEFINITION Simultaneous 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 WBC Elevated protein (commonly between 100 to 1000 mg/dL) Classically described as albumino-cytologic dissociation CSF 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

TREATMENT Specific treatement Initiate 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 treatment Patients 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. PROGNOSIS Full 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. Infection Virus Bacteria Fungi 2. Malignant cells 3. Drugs and contrast media 4. Blood (following subarachnoid haemorrhage) Meningitis is usually used to describe inflammation due to infection. AETIOLOGY Virus  Enterovirus Coxsackie Echo Polio  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


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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

PATHOLOGY The 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 FEATURES Triad  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

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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


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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 FEATURES Classically 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.

Condition Normal Viral Bacterial Tuberculous Appearance Clear Clear Turbid/ purulent Cob web formation on standing Cell type count Lymphocytes <5/mm3 Lymphocytes 10-2000 Polymorphs 1000-5000/mm3 Lymphocytes 50-5000/ mm3 Glucose >60% of blood sugar 40-80 mg/dL Normal Very low Low Protein <45mg/dL 40-80 mg/dL 50-200 mg/dL 50-300 mg/dL (very high) Gram stain/ culture

May be positive

Fungal Malignant

Clear/ turbid Clear

Lymphocytes 50-500/ mm3 Lymphocytes 0-100/ mm3

Low Low

Elevated Normal/ elevated

CBC In acute bacterial meningitis, CBC shows polymorpho leucocytosis Blood cultures Blood cultures should be done in acute meningitis and may be positive Chest X-ray May show infiltrate of pneumococcal pneumonia 

ACUTE 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 regimen Ceftriaxone 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. Prevention Meningococcal infection Index case and other close contacts require prophylaxis: Rifampicin 600mg twice daily for 2 days or Ciprofloxacin 750 mg single dose Azithromycin 500 mg single dose Ceftriaxone 250 mg single dose VIRAL MENINGITIS Treatment

Supportive and symptomatic TUBERCULOUS MENINGITIS 9-12 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 MENINGITIS CNS  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

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