NEUROLOGYPYOGENIC MENINGITIS1. Common causes:Beyond the neonatal period, the 3 most common organisms that cause acute bacterial meningitis are:1. Immune competent children:1. Streptococcus pneumoniae,2. Neisseria meningitidis,3. Haemophilus influenzae type b (Hib).2. Immune deficiency and anatomic defects:1. Psuedomonas aeruginosa2. Staphylococcus aureus3. Coagulase - negative staphylococci4. Salmonella5. Listeria monocytogenes3. Immune deficiency and anatomic defects are:1. Altered immune defense:a. HIVb. Agammaglobulinemiac. Steroid therapyd. Post measles state2. Splenic dysfunction (sickle cell)3. Asplenia syndrome4. Cochlear implants5. CSF leak :a. Fracture skullb. Dermal sinusi. Pre auricular sinusii. Pilonidal sinusc. Spinal Meningocoel4. CSF shunt infection2. Clinical presentation:1. Clinical manifestations 1. Rarely dramatic onset and sudden death in 24 hrs2. Insidious onset:i. Feverii. Head acheiii. Photophobiaiv. Irritabilityv. Lethargyvi. Altered sensoriumvii. Convulsionsviii. Coma3. Clinical signs i. Nugal rigidityii. Kernig sign : Flexion of hip to 90 and extension of knee produce painiii. Brudzinki sign: Flexion of neck produces flexion of hip and kneesiv. Bulging fontannelv. Sutural wideningvi. Iv , vi, vii, viii cranial nerve palsyvii. Focal deficitsviii. Tache cerebrale: Stroke skin with a blunt instrument 30-60 sec raised red rash ix. Convulsions4. Invbestigations:Lumbar puncture a. Bacterial1. Pressure:>200 mm H2O2. Colour:opalescent3. Cells:200-2000 WBC/ml (pmn) 4. Protein:>130 mg/dl5. Glucose:24 hr apart suggest the presence of an epileptic disorder c. Incidence: Seizures occur in about 10% of children. 1-2% in general population2. Types of seizures:1. Focal seizures: Turning of the head and eyes to one side, unilateral clonic movements; Sensory disturbance localized to a specific area.2. Tonic seizures; Are characterized by increased tone or rigidity,3. Atonic seizures: Are characterized by flaccidity or lack of movement during a convulsion4. Clonic seizures: Consist of rhythmic muscle contraction and relaxation; 5. Myoclonus: Shocklike contraction of a muscle.3. Stages of a typical Seizure: pre ictus-ictus-post ictus 1. The prodrome: change in mood or behaviour or insomnia or feelings about the impending seizure 4. The aura: feeling a sense of unexplained fear, a strange smell or a feeling of numbness; dj vu phenomenon 3. Epileptic cry: Some people may cry out at the beginning of a seizure as the muscles around the vocal cords contract 4. Loss of consciousness5. Tonic phase: 6. Clonic phase:7. Loss of bowel and bladder control. 8. Postictal confusion .9. Postictal automatism10. Post ictal sleep 11. Somnambulism12. Severe headache. 13. Todds palsy 4. Common Epilepsy Triggers:1. Flickering or Flashing Light2. Hyperventilation 3. Lack of Sleep4. During ovulation, menstruation, pregnancy or menopause. This is known ascatamenial epilepsy.5. Especially foods that are rich inglutamate and aspartame 6. High fever7. Hot water- bath epilepsy8. TV epilepsy5. International classification of seizure types (1981) by the International League Against Epilepsy (ILAE)Classification Type Subtypes

1.Partial (focal seizures)a. Simple partial(Consciousness present)

1. Motor 2. Sensory 3. Autonomic 4. psychic

B. Complex partial (Consciousness impaired)

1. Simple partial onset, followed by impairment of consciousness 2. With impairment of consciousness at onset

2. Generalized

a. Absence

1. Absence seizures (petit mal)

b. Generalized

1. Myoclonic seizures2. Clonic seizures3. Tonic seizures4. Tonicclonic seizures(grand mal) 5. Atonic seizures 6. Infantile spasm

3. Unclassified Febrile seizures

6. Pathophysiology 1. Seizures can be viewed as resulting from an imbalance between excitatory and inhibitory processes in the brain2. Abnormalities in the membrane properties of neurons, 3. Decreased inhibitory neurotransmission which is primarily by gamma-amino butyric acid (GABA), or4. Enhanced excitatory neurotransmission which is primarily mediated by glutamate.5. Small group of abnormal neurons undergo prolonged depolarizations associated with the rapid firing of repeated action potentials.6. Epileptic neurons recruit adjacent neurons creating a storm of electrical activity in the brain leading to clinical seizure 7. Evaluation 1. Dysmorphic features are evaluated2. Adenoma sebaceum, shagreen patch, multiple cafeau-lait spots, a nevus flammeus, and the presence of retinal phakoma indicate a neurocutaneous disorder3. Electrolytes, toxicology screening, urine and serum metabolic testing4. Serum glucose5. ECG: heart blocks; prolonged QT syndrome 6. LP: meningitis, encephalitis, sepsis, subarachnoid hemorrhage, or a demyelinating disorder7. Neuroimaging: for structural brain abnormalities including malformations, injuries, infections, or tumors.8. The EEG is abnormal in about 15% of healthy subjects and normal in about 20% of patients with epilepsy; Spikes and waves occurring in the , and ranges in the interictal EEG record are the hallmark of epilepsy8. Treatment of Epilepsy Antiepileptic drugs:Drug Dose Side effects Rx: epilespy

Phenobarbitone 3-8 mg/kg Hyperactivity, academic deterioration, reversal of sleep cycles Generalized tonic-clonic Partial Status epilepticus

Phenytoin 5-15 mg/kg Poor seizure control due to fluctuating drug levels, cosmetic side effects,hirsutism, ataxia Generalizedtonic-clonic PartialStatusepilepticus

Valparin 10-60 mg/kg Nausea, vomiting, loss of appetite, weight gain, irregular menstruation, alopecia, Somnolence Generalizedtonic-clonic AbsenceMyoclonic

Carbamazepine 10-30 mg/kg Drug rash, worsening seizures, rarely worsening school performance Generalized tonic-clonic Partial

Clobazam 0.4-1.2 mg/kg Behaviour changes, aggression, sleep disturbances, constipation, weight gain Adjunctive therapy whenSeizures poorlyControlled

Nitrazepam (Mogadon) 0.2mg/kg/24 hr Absence

Paraldehyde 150200mg/kgMaintenance,20 mg/kg/hr Generalized statusEpilepticus

diazepam and lorazepam IV / rectal CNS depression GeneralizedstatusEpilepticus

9. Ketogenic Diet in Epilepsy1. High fat and low protein/carbohydrate diet given with/without a restricted fluid intake to maintain ketosis on a long term basis2. For controlling refractory seizures3. It exerts an anticonvulsant effect secondary to elevated levels of -hydroxybutyrate and acetoacetate resulting from the ketosis 4. The use of valproic acid is contraindicated because the risk of hepatotoxicity is enhanced.10. Surgery for epilepsy: For focal seizuresLeft vagal nerve stimulation produced by an implanted device reduced seizures by 3142% over 18 mo

STATUS EPILEPTICUS

Definition:Status epilepticus is defined as a continuous convulsion lasting longer than 2030 min or the occurrence of serial convulsions between which there is no return of consciousness. Classification:a. generalized (tonic-clonic, absence) b. partial (simple, complex, or with secondary generalization). c. Status epilepticus is a medical emergency Etiology:There are three major subtypes of status epilepticus in children: 1. Prolonged febrile seizures 2. Idiopathic epilepsy leading to status epilepticus 3. Symptomatic epilepsy leading to status epilepticus:Eg: encephalitis, meningitis, Inborn errors Initial treatment:1. The oral airway is secured; 2. Suction and oxygen 3. If necessary intubation and assisted ventilation. 4. A nasogastric tube is placed in position, and an IV catheter is inserted. 5. If hypoglycemia is confirmed by Dextrostix, a rapid infusion of 5 mL/kg of 10% dextrose is provided. Baseline investigations:1. Blood is obtained for a CBC and electrolytes (including calcium, phosphorus, and magnesium), glucose, creatinine, lactate, and anticonvulsant levels, if indicated. 2. Blood and urine may be obtained for metabolic studies and toxicology, 3. Arterial blood gases should be determined, 4. Examination of the CSF. Baseline neurologic examination: A physical and neurologic examination should be carried out concurrently to assess the following: 1. evidence of trauma; 2. papilledema, a bulging anterior fontanel, or lateralizing neurologic signs suggesting increased ICP; 3. manifestations of sepsis or meningitis;4. retinal hemorrhages that may indicate a subdural hematoma; 5. Kussmaul breathing and dehydration suggestive of metabolic acidosis or irregular respirations signifying brainstem dysfunction; 6. evidence of failure to thrive, a peculiar body odor, or abnormal hair pigmentation that suggests an inborn error of metabolism; 7. Constriction or dilatation of pupils suggesting a toxin or drugs as the cause of the status epilepticus. Management:1. Steps in drug therapy:Step I:a. Diazepam: should be given IV directly into the vein (not the tubing) in a dose of 0.1-0.3 mg/kg at a rate no greater than 2 mg/min for a maximum of three doses.ORb. Lorazepam 0.05-0.1 mg/kg IV administered slowly. ORc. midazolam is 0.15-0.3 mg/kg IV. d. Alternatives: If an IV line cannot be established or the child is some distance from a medical center:i. Buccal or nasal midazolam (0.5 mg/kg) is another optionii. Rectal diazepam or lorazepam can be used safely. 0.3-0.5 mg/kg. lorazepam 0.05-0.1 mg/kg. iii. Sublingual lorazepam 0.05-0.1 mg/kg. Step II: if the seizures persist:a. Phenytoin 15 up to 30 mg/kg IV (given in 10 mg/kg increments) at the rate of 1 mg/kg/min. ORb. The phenytoin prodrug fosphenytoin has advantages over the older formulation because it is water soluble, less irritating after IV injection, and well absorbed after intramuscular injection. The dosage in PE units is the same as phenytoin preparation. ORc. Phenobarbital is given in a loading dose of 15-20 mg/kg or in neonates 20-30 mg/kg IV during 10-30 min. With control of the seizures, the maintenance dose is 3-5 mg/kg/24 hr divided into two equal dosesStep III: a. Constant IV infusion of either midazolam (0.20 mg/kg bolus, 20-400 g/kg/hr infusion) or propofol (1-2 mg/kg, 2-10 mg/kg/hr infusion) ORb. If seizures continue, induction of barbiturate coma. The initial IV loading dose of thiopental is 2-4 mg/kg and is then titrated to achieve a burst suppression EEG pattern. ORc. A 5% solution of paraldehyde is prepared by adding 1.75 mL of paraldehyde (1 g/mL) to D5W to a total volume of 35 mL. The loading dose is 150-200 mg/kg IV slowly for 15-20 min, and then seizure control is maintained with an infusion of 20 mg/kg/hr in a 5% concentration in a glass bottle, because the drug is incompatible with plastic.ORd. Valproic acid loading dose 10-15 mg/kg. IV may become a useful drug If still refractory:e. General anesthesia 2. Continuation of anticonvulsant therapy:a. a long-term antiepileptic should be maintained in children with a progressive neurologic disorder or with a history of recurrent seizures before the onset of status epilepticus. b. In a prolonged febrile seizure was the cause, anticonvulsant therapy is maintained arbitrarily for 3 mo in this case and is discontinued if the child remains asymptomatic.Prognosis: Status epilepticus produces potentially life-threatening disturbances in physiologic function, and the mortality rate of status epilepticus is 5%.

FEBRILE CONVULSIONS

1. Febrile seizures are age dependent and are rare before 9 mo and after 5 yr of age.2. The peak age of onset is approximately 14-18 mo of age, and 3. The incidence approaches 3-4% of young children. 4. An autosomal dominant inheritance pattern is demonstrated in some families.

5. Clinical Manifestations.

Simple febrile seizure Complex febrile seizure

Lasts less than 15 minutes Lasts 15 minutes or longer

Occurs once in a 24-hour period Occurs more than once in a24-hour period

Generalized Focal

No previous neurologic problemsPatient has known neurologic problems, such as cerebral palsy

6. Recurrence Of febrile seizures: a. Approximately 30-50% of children have recurrent seizures b. Risk factors for recurrence:i. Atypical seizure or postictal period, ii. A positive family history of epilepsy, iii. An initial febrile seizure before 9 mo of age, iv. Delayed developmental milestones, or a v. Pre-existing neurologic disorder. 7. Risk of epilepsy:a. 9% when several risk factors are present, b. 1% in children who have no risk factors

8. Acute evaluation:a. Determine the cause of the fever and to rule out meningitis.b. If any doubt exists about the possibility of meningitis, a lumbar puncture with examination of the cerebrospinal fluid (CSF) is indicated. c. The possibility of viral meningoencephalitis should also be kept in mind, especially that caused by herpes simplex.d. Viral infections of the upper respiratory tract, measles, roseola, and acute otitis media are most frequently the causes of febrile convulsions.

9. Evaluation:a. Glucose determination, serum electrolytes and toxicology screening b. An electroencephalogram (EEG) for atypical features c. Neuroimaging for children with atypical features 10. Treatment:a. Treat primary cause; control fever; reassure of the parents. b. Prolonged anticonvulsant prophylaxis: i. Controversial and no longer recommended. ii. Antiepileptics such as phenytoin and carbamazepine have no effect on febrile seizures. c. Oral diazepam is an effective and safe method of reducing the risk of recurrence of febrile seizures. At the onset of each febrile illness, oral diazepam, 0.3 mg/kg q8h (1 mg/kg/24 hr), is administered for the duration of the illness (usually 2-3 days). This strategy may be useful when parental anxiety associated with febrile seizures is severe.

SUBDURAL EFFUSION:

I. Collections of fluid in the subdural space develop in 10-30% of patients with meningitis and are asymptomatic in 85-90% of patients. II. Subdural effusions are especially common in infants. III. Symptomatic subdural effusions may result in:1. a bulging fontanel, 2. diastasis of sutures,3. enlarging head circumference, 4. emesis, 5. seizures, 6. fever, and 7. Abnormal results of cranial transillumination. IV. CT or MRI scanning confirms the presence of a subdural effusion. V. In the presence of increased ICP or a depressed level of consciousness, symptomatic subdural effusion should be treated by aspiration through the open fontanel. Fever alone is not an indication for aspirationPSEUDO TUMOR CEREBRI:

a) Definition: Pseudotumor cerebri is a clinical syndrome that mimics brain tumors and is characterized by increased intracranial pressure (ICP) with a normal cerebrospinal fluid (CSF) cell count and protein content and normal ventricular size, anatomy, and position.b) Etiology:1. There are many explanations for the development of pseudotumor cerebri, including alterations in CSF absorption and production, cerebral edema, abnormalities in vasomotor control and cerebral blood flow, and venous obstruction. 2. The causes of pseudotumor are numerous and include:1) metabolic disorders: 1. galactosemia, 2. hypoparathyroidism, 3. pseudohypoparathyroidism, 4. hypophosphatasia, 2) prolonged corticosteroid therapy or too rapid corticosteroid withdrawal, 3) refeeding of a significantly malnourished child,4) hypervitaminosis A, 5) Addison disease, 6) obesity, 7) menarche, 8) oral contraceptives 9) Drugs:1. nalidixic acid, 2. tetracycline, 10) hematologic disorders:1. polycythemia, 2. venous thrombosis (lateral sinus or posterior sagittal sinus thrombosis), c) Clinical fatures:1. The most frequent symptom is headache, and although vomiting also occurs, it is rarely as persistent and pernicious as that associated with a tumor. 2. Diplopia secondary to paralysis of the abducens nerve is a frequent complaint.3. Most patients are alert and lack constitutional symptoms. 4. Infant may reveals a bulging fontanel and a cracked-pot sound or Macewen sign (percussion of the skull produces a resonant sound) due to separation of the cranial sutures.5. Papilledema 6. Early optic nerve edema may be noted with ultrasonography. 7. An inferior nasal defect may be detected on formal tangent screen testing. 8. No focal neurologic signs d) Pseudotumor cerebri is mainly a self-limited condition, but optic atrophy and blindness are the most significant complications. e) Sinus thrombosis should be treated with anticoagulation. f) L.P:1. For others, the initial lumbar tap that follows a CT or MRI scan is both diagnostic and therapeutic. 2. The spinal needle produces a small rent in the dura that allows CSF to escape the subarachnoid space, thus reducing the ICP. 3. Several additional lumbar taps and the removal of sufficient CSF to reduce the opening pressure by 50% occasionally lead to resolution of the process. g) Drugs:1. Acetazolamide, 10-30 mg/kg/24 hr, and corticosteroids have been effective for some patients. h) Surgery:1. Rarely, a lumboperitoneal shunt or subtemporal decompression is necessary if the aforementioned approaches are unsuccessful and optic nerve atrophy supervenes. 2. Some centers perform optic nerve sheath fenestration. HYDROCEPHALUS1. Definition: It represents a diverse group of conditions that result from impaired circulation and absorption of CSF or rarely increased production as in choroid plexus papilloma2. CSF pressure:a. Ventricles: 180 mm of waterb. Superior sagittal sinus: 903. CSF flow Lateral ventricles Foramina of monro 3rd ventricle Aqueduct of sylvius 4th ventricle Foramina of luschka (lateral) and magendie (midline) Cistern at the base of the brain Subarachnoid space surrounding the brain, spinal cord and cauda equina Reenters circulation through the arachnoid granulations 4. Types of hydrocephalus 1. Non Communicating or obstructive: CSF blockage within in the ventricular system 2. Communicating or non obstructive: obliteration of subarachnoid cysterns or malformation of the arachnoid villi 5. Etiology 1. Non Communicating or obstructive hydrocephalus Abnormality of aqueduct of Sylvius:1. Stenosis: TORCH infection2. Developmental defect: Branching and forking3. Sex linked recessive trait in some associated with spina bifida occulta 4. Gliosis: Intrauterine viral infections; Neonatal Meningitis ; Subarachnoid hemorrhage 5. Vein of Galen malformation in the midline6. Posterior fossa tumors7. Chiari malformation8. Dandy-Walker syndrome2. Communicating or non obstructive hydrocephalus 1. Subarachnoid hemorrhage2. Exudate due to Meningitis : pneumococcal and TBM3. Intrauterine infections4. Leukemic infiltrates 6. Clinical 1. Increase in head cicumference2. Open and bulging AF3. Dilated scalp veins4. Broad forehead5. Sunset sign: compression of tectum by suprapineal recess7. Irritability, lethargy and vomiting8. Headache9. Personality changes and deterioration in academic performance10. Skull percussion: cracked pot or Macewan sign11. Small occiput Chiari malformation12. Prominent occiput: Dande - Walker syndrome7. Clinical signs 1. Papilledema 2. Abducent nerve palsy3. Long tract signs:i. Hyperreflexia; ii. Hypertonia; iii. Clonus; iv. Babinsky 8. Chiari Malformation-Type I 4. Cerebellar downward displacement into cervical canal1. Adolescent age2. No hydrocephalus3. Headache 4. Neck pain5. Urinary frequency6. Progressive lower limb spasticityChiari- I 9. Chiari Malformation Type II 1. Progressive hydrocephalus2. Menigomyelocoel3. Embryology: 1. failure of pontine flexure 2. Elongation of 4th ventricle3. Downward displacement of vermis, pons and medulla into Cx canal4. Stridor week cry in infancy5. Spasticity and incoordination in childhood 10. Dandy-Walker malformation1. Developmental failure of roof of 4th ventricle2. Agenesis of posterior cerebellar vermis with agenesis of corpus callosum3. Cystic dilatation of 4th ventricle in posterior fossa4. Hydrocephalus with prominent occiput5. Cerebellar ataxia6. Long tract signs11. Diagnosis 1. Progressive increase in occipitofrontal circumference2. Wide bulging AF3. Prominent occiput 4. Flatened occiput 5. Frontlal bossing6. Sunset sign 7. Sutural seperation 8. Mecewen sign9. Pappiledema 12. DD 1. Cranial wault thickening: anemia, rickets, osteogenesis imperfecta, epipyseal dysplasia2. Subdural effusion: parietal prominence3. Megalencephaly (increased brain substance): Taysachs, Mucopoly, leukodystrophy, maple syrup urine disease 4. Cerebral gigantism5. Familial megalencephaly- autosomal dominant6. Hydranencephaly 13. Treatment 1. Medical:1. Acetazolamide and furosemide rduce rate of CSF production2. Shunt:1. Ventriculo peritoneal3. Shunt complications:1. Shunt occlusion: headache and vomiting2. Shunt infection: staph epidermidis-meningitis 14. Prognosis 1. Determined by associated complications2. Lower IQ3. Visual defects4. Most are pleasant mannered; some aggressive5. Chronic ICT can produce increased gonadotropins and precocious puberty

MICROCEPHALY1. Definition: Microcephaly is defined as a head circumference that measures more than three standard deviations below the mean for age and sex. 2. Microcephaly may be subdivided into two main groups: i. Primary (genetic) microcephaly and ii. Secondary (nongenetic) microcephaly. 3. Etiology:a. Primary (Genetic)

1. Familial a. Autosomal recessive:

b. Autosomal dominant:2. Syndromes:

a. Down (21-trisomy)b. Edward (18-trisomy)c. Cri-du-chat (5 p-)

d. Cornelia de Langee. Rubinstein-Taybif. Smith-Lemli-Opit4. Secondary (Nongenetic)

1. Radiation:

2. Congenital infections: TORCHS

3. Drugs:

i. Fetal alcohol:ii. Fetal hydantoin:4. Meningitis/encephalitis:5. Hyperthermia: Significant fever during 1st 4-6 wk6. Hypoxic-ischemic encephalopathy:7. Maternal phnylketonuria 5. Clinical Manifestations and Diagnosis:a. A very small head circumference implies a process that began early in embryonic or fetal development.b. An insult to the brain that occurs later in life, particularly beyond the age of 2 yr, is less likely to produce severe microcephaly. 6. Laboratory investigation:a. mother's serum phenylalanine level b. A karyotype is obtained if a chromosomal syndrome is suspected c. CT scanning or MRI may be useful in identifying structural abnormalities of the brain or intracerebral calcification. d. Additional studies include: TORCH screening test7. Treatment:a. Genetic and family counseling.

ENCEPHALITIS

DefinitionEncephalitis is an inflammation or infection of the parenchyma of the brain sometimes accompanied by infection of the surrounding tissues (meningoencephelitis), the pia mater, the arachnoid, and CSF.Etiology:1. Primary encephalitis is caused by a direct viral infection of the spinal cord and brain. 2. Secondary encephalitis as result of complications of a current viral infection Etiology:1. Enterovirus: 80% feco oral 1. Poliomyelitis2. Coxsackie3. Echo (ev9)2. Arbo virus: mosquito and ticks1. Japanease encephalitis2. Westnile3. St.louiz4. Tick-borne virus3. Herpez:3. Hsv i, ii4. Varicella zoster4. Cmv5. Epstein bar virus6. Mumbs7. Rabies8. Rubella9. Vaccine virus3. Polio, mumps,measles10. Acanthamoeba3. Swimming pool ; contact lens

JAPANESE ENCEPHALITISIntroduction 1. It is a disease caused by the mosquito-borne Japanese encephalitis virus2. The virus is an enveloped virus of the genus flavivirus; Single stranded RNA virus Mode of transmission1. The infection is transmitted through the bite of an infected Culicine mosquito. Reservoir of infection 1. The animal hosts include pigs, cattle and horses, Water birds such as pond herons, cattle egrets, poultry birds and ducks2. The pigs are considered amplifying hosts. Vectors 1. Culicine mosquitoes:1. Culex tritaeniorhynchus: most important vector in south India2. Culex vishnui 3. Culex gelidus Seasonality 1. JE is a seasonal disease. Epidemics coincide with the monsoon and post monsoon period (August to December)Clinical manifestation 1. Incubation period: 4-14 days.2. Ratio of overt disease to inapparent infection varies from 1:300 to 1:1000. 3. The course of disease in man may be divided into prodromal, acute, late stage and sequelae phase. 4. The fatality varies between 20-40 percent, but may reach over 58 percent.5. The symptoms and signs of encephalitis under 4 headings: 1. Symptoms and Signs of Infection: High grade fever, headache & Malaise. 2. Symptoms and Signs of Brain damage due to infection:1. Confusion, irritability, loss of consciousness;2. Seizures and/or other abnormal movements, 3. Focal neurological deficits 4. Doll's eye movements (DEM),5. Absent corneal reflex, 6. Absent pupillary light reflex,7. Deviation of angle of mouth, 8. Decorticate or decerebrate rigidity, 9. Irregular respiration.3. Symptoms and Signs of Raised Intracranial Tension: 1. Headache, vomiting, up going plantars 2. Abducent nerve palsy: false localizing signs 3. Absent pupillary light reflex on one side: temporal lobe herniation and compression of III cranial nerve4. Hemiplegia: late sign of temporal lobe herniation and compression of the brain stem5. Bradycardia, hyperthermia & hypertension : hypothalamic dysfunction6. Irregular breathing : brainstem damage, 7. Squint : III or IV or VI cranial nerve palsy4. Symptoms and signs of meningeal irritation:1. Neck rigidity, 2. Kernigs sign (limitation of knee extension when the hip is flexed to 90)Diagnosis: 1. CSF lymphocytic pleocytosis with normal glucose level is diagnostic of viral encephalitis. 2. Serological tests can confirm the diagnosis in only about25% of viral encephalitis cases. Workup 1. ELISA for detection of anitiviral antibodies2. Antibody titres:1. Ig M antibodies 2. Ig G rising titer3. Polymerase chain reaction (PCR) for detection of viral components 4. Neuroimaging: MRI : 1. Reveals either mixed intensity or hypo intense lesion in thalami. 2. Thalamic changes may be helpful in the diagnosis of JE Differential diagnosis 1. Bacterial meningitis 2. Other encephalitis3. Intracranial abscess4. Tuberculous Meningitis5. Parasitic infections (e.g., Toxoplasmosis, cysticercosis)6. Active demyelinating disease.7. Cerebral Malaria8. Reye's Syndrome9. Bulbar PoliomyelitisManagement 1. There is no specific treatment for JE2. Main causes of Mortality and Morbidity are:1. Pulmonary aspiration of saliva or vomitus2. Hypoxia3. Hypoglycemia4. Uncontrolled Seizures5. Hyperpyrexia6. Raised ICT7. Pulmonary Edema8. Secondary Infections9. Brainstem involvement10. SIADH. Management 1. Establish an adequate airway. Suction throat secretions as and when necessary.2. Administer oxygen; reduces cerebral edema3. Fluids, plasma, blood or even a dopamine drip (5-20 mg/kg/min) for hypotension.4. Avoid fluid overload5. Give IV 10% dextrose; suppresses gluconeogenesis Seizure management 1. IV Diazepam 0.1 - 0.3 mg/Kg in 1-5 minutes. The dose may be repeated in 5 - 20 minutes.2. Phenytoin 10 - 20 mg /Kg over 10 - 20 minutes ; repeat dose of 5 - 10 mg /Kg IV may be given after 1 hour3. Inj. Paraldehyde 4%, 0.1 - 0.3 ml/Kg, IM4. Oral maintenance through nasogastric tube:For raised Intracranial Tension 1. Normalize temperature. Hyperthermia increases cerebral blood flow (CBF), cerebral blood volume (CBV) and intracranial tension/pressure (ICP).2. For cerebral edema:1. 20 % Mannitol IV infusion:1. Loading dose: 5ml/kg in 20 minutes, followed by 2. Maintenance: 1.25 ml/kg every 6-12 hours 2. Oral Glycerol 0.5 ml/Kg diluted in twice the volume of water or fruit juice 3 times daily3. Inj. Frusemide 1 mg/Kg/dose IM 2 times daily4. Hyperventilation with an Ambu bag or hyperbaric oxygenation can be effective in reducing intracranial pressure by decreasing cerebral blood flow.5. IV dexamethazone3. Other measures 1. Urinary catheterization2. Prevent aspiration3. Prevent bed sores4. Dark and sound proof room to minimize external stimulation5. Oral Hygiene6. Correction of serum sodium abnormalities7. IV / Enteral feeding8. Stress ulcers: oral or inj. Ranitidine Poor prognostic signs 1. Dilated nonreacting pupils,2. Rapid breathing,3. Accumulation of bronchial secretions,4. Appearance of excessive sweating,5. Dolls eye movements, 6. Persistence of fever,7. Refractory seizures,8. Decerebrate posture,9. Decorticate posture.Long Term Therapeutic Measures 1. Physiotherapy and rehabilitation2. Spasticity: Benzodiazepines, Baclofen3. Hemiballismus chlorpromazine4. Choreoathetosis Haloperidol5. Myoclonus Valproic acidPrognosis 1. Earlier statistics indicate that for JE the rule of thirds applies. 33.3% recover well. 33.3% suffer significant morbidity and 33.3% die.2. The mortality and morbidity can be brought down significantly (mortality