Pediatric neurology notes

7. Lecture notes in Pediatric Neurology1
S. No. Contents
7.1 Seizures and Epilepsy
7.1.1 Status Epilepticus.
7.1.2 Febrile convulsions
7.2 Acute Bacterial Meningitis
7.3 Viral Meningoencephalitis
7.4 Tuberculous Meningitis
7.5 Microcephaly.
7.6 Craniosynostosis
7.7 Hydrocephalous
7.8 Cerebral Palsy. (Little’s Disease)
7.9 Mental Retardation
7.10 Guillain Barre’s Syndrome. (Post – infectious polyneuropathy)
7.11 Hemiplegia
7.12 Movement Disorders
7.12.1 Ataxia
7.12.2 Acute cerebellar Ataxia
7.12.3 Ataxia Telangiectasia
7.12.4 Friedreich’s Ataxia.
7.12.5 Tics
7.12.6 Chorea
7.12.7 Athetosis
7.12.8 Tremor
7.12.9 Myoclonus
7.12.10 Dystonia
7.12.11 Ballismus
7.12.12 Tardive Dyskinesia
7.13 Pseudotumor Cerebri
7.14 Bell’s Palsy
7.15 Neurocysticercosis

7.1.Seizures and Epilepsy.
1. Seizures.
1.1. Definition: it is a paroxysmal involuntary disturbance of brain function that manifest
as impairment of consciousness, motor or sensory activity, autonomic or behavior disturbances
due to abnormal electrical activity of brain
2. Epilepsy.
2.1. Definition: When two or more unprovoked seizures occur at an interval greater than 24
hours apart.
2.2. Classification of Epileptic Seizures:
2.2.1. Partial Seizures:
• Simple partial seizures – Consciousness maintained.
I. Motor.
II. Sensory.
III. Autonomic.
IV. Psychic.
• Complex partial seizures – Loss of consciousness.
2.2.2. Generalized Seizures:
• Absence seizures. (Petit mal Seizures)
 Typical.
 Atypical.
• Generalized seizures.
 Generalized tonic clonic seizures.
 Clonic seizures.
 Atonic.
 Myoclonic.
 Infantile spasm.
2.2.3. Unclassified Seizures:
2.2.4. Neonatal epileptic Seizures:
2.3. Mechanism of seizures:
2.3.1. Seizures arises from a focus of neuronal cell death. Theses areas develops hyper
excitability synapses.
2.3.2. Seizure discharge depends upon excitatory glutaminergic synapses.
2.3.3. Neuronal excitation may be by amino acids neurotransmitter like glutamate and
aspartate.
2.3.4. Stimulation of amygdala by kindling may produce seizures.
2.3.5. Underdeveloped brain is more susceptible to specific seizures.
2.3.6. Genetic factors.
2.3.7. Chromosomal abnormality:
• 20q 8q – Benign neonatal convulsions.
• 6p – Juvenile myoclonic epilepsy.
• 21q 22.3 – Progressive myoclonic epilepsy.
2.3.8. Substantia nigra has role in regulation and dissemination of seizures.
2.4. Etiology:
2.4.1. Perinatal conditions:
a) Cerebral malformation.
b) Intrauterine infections.
c) Hypoxic ischemia.
d) Hemorrhage.
e) Trauma.

2.4.2. Metabolic.
a) Hypoglycemia.
b) Hypocalcemia.
c) Hypomagnesemia.
d) Hyponatremia.
e) Reye’ Syndrome.
f) Porphyria.
g) Pyridoxine dependency/deficiency.
h) Hypernatremia.
i) Hyperbilirubinemia.
j) Alkalosis.
2.4.3. Intracranial infections.
a) Meningitis.
b) Encephalitis.
c) Brain abscess.
2.4.4. Poisoning.
a) Lead, cocaine.
b) Drugs and drug withdrawal.
2.4.5. Neuro – cutaneous syndromes.
a) Tuberculous sclerosis.
b) Neurofibromatosis.
c) Sturge – weber syndrome.
2.4.6. Systemic disorders:
a) Vasculitis.
b) SLE.
c) Hypertensive encephalopathy.
d) Renal failure.
e) Hepatic encephalopathy.
2.4.7. Others:
a) Trauma.
b) Tumor.
c) Febrile seizures.
d) Idiopathic.
e) Familial.
f) Degenerative disorders.
2.4.8. Epilepsy.
2.5. Treatment: Anticonvalscents drugs are:
S. No. Name of the Drug Dose
1. Carbamazepine 10 – 40mg /kg/day
2. Phenobarbitone 3 – 8 mg /kg/day
3. Phenytoin 3 – 8 mg /kg/day
4. Valproic acid 10 – 60mg /kg/day.
5. Clonazepam 0.05 – 0.2 mg/kg/day.
6. Clobazam 0.1 – 1mg /kg/day
7. Ethosuximide 20mg/kg/day.
8. Paraldehyde 0.1 to 0.2ml/kg/dose deep IM.
9. Gabapentin 15 - 45mg /kg/day.
10. Lamotrigine 2 – 10mg /kg/day.
11. Nitrazepam 0.2mg/kg/day.
12. Tiagapine 6 mg TDS

13. Vigabatrin 30 – 100mg/kg/day, 20 – 100mg/kg.
14. Primidone 10 -25mg /kg/day.
15. ACTH 20 – 40 Units IM/day.
2.6. Evaluation:
2.6.1 CBC.
2.6.2 Blood chemistry.
 Glucose.
 Ca⁺⁺⁺, Na⁺, K⁺, Cl⁺.
 Bicarbonate.
 Urea Nitrogen.
 Creatinine.
 Mg⁺2
2.6.3 Blood toxicology.
2.6.4 Urine toxicology.
2.6.5 Fundus.
2.6.6 CSF Examinations.
2.6.7 EEG.
2.6.8 Brain Imaging.
 CT.
 MRI.
 ECG.
2.7. Treatment of Epilepsy:
2.7.1 Medical treatment.
a) Preferably one drug should be used depending upon type of seizures (Clinically
and EEG based.
b) Dosage of drug can be increased up to maximum dose.
c) If seizures are not controlled second drug may be used.
d) Duration of treatment – seizures free period 2 – 3 yrs, then tapper the dose.
e) Periodic check up 3 monthly for side effects of drugs.
f) Life long treatment for juvenile myoclonic epilepsy.
g) Therapeutic drug monitoring.
2.7.2 Surgical treatment.
a) Indications:
 Intractable seizures.
 Focal seizures.
 Resection of epileptogenic foci.
2.7.3 Ketogenic diet.
2.7.4 Counselling of parents.
a) First aid measures.
b) Regularity of Drugs.
c) Child should avoid swimming, fires, machine work, swing.
d) Child should have identification card with him whenever he is out of home.
e) Child should not be permitted to go to terrace alone.
f) He should avoid playing with crackers.
g) Sleep should be complete and in proper time.

7.1.1. Status Epilepticus.
7.1.1.1 Definition: Continuous convulsion lasting longer than 30 mins. or occurrence of serial
convulsions between which there is no return of consciousness or brought to hospital seizing.
7.1.1.2 Classification:
a) Generalized.
• Tonic Clonic.
• Absence.
b) Partial.
• Simple.
• Complex.
• Secondary generalization.
c) Neonatal status.
7.1.1.3 Etiological classification: There are sub-major types
a) Prolonged febrile seizures.
b) Idiopathic status epilepticus.
c) Symptomatic status epilepticus.
7.1.1.4 Predisposing factors & Causes:
1) Sudden withdrawals of Anti-convalescents.
2) Irregular Anticonvalscents therapy.
3) Sleep deprivation.
4) Encephalitis.
5) Meningitis.
6) Congenital malformation of brain.
• Lissencephaly.
• Schizencephaly.
7) Inborn errors of metabolism.
8) Electrolyte imbalance.
9) Hypocalcemia & Hypoglycemia.
10) Drug intoxication.
11) Reyes syndrome.
12) Lead intoxication.
13) Extreme hyperpyrexia.
14) Brain tumor – especially frontal lobe.
7.1.1.5 Management: There are five Principles of Management:
A. Stabilize patient.
B. Rapid control of seizures.
C. Supportive treatment.
D. Search for cause.
E. Maintenance therapy.
7.1.1.6 Stepwise Approach:
Step 1: ABC (Airway, Breathing, Circulation). Suction, O2, Ventilation, Circulation IV line.
One I/V line for collecting blood sample, Second, I/V line for therapy. Investigation required
such as Blood sugar, Ca⁺, Mg⁺2
Electrolytes. If hypoglycemic then 25% dextrose 2ml/kg I/V.
Step 2: To modify as per IAP Guidelines:
a) Diazepam (Dose – 0.3mg/kg/I.V.)
Or
Lorazepam (Dose – 0.05-0.1mg/kg/I.V.) can be repeated after 10mins. If seizures not
controlled
b) Phenytoin or fosphenytoin (Dose – 15-20 mg/kg/I.V. in saline)
c) Further dose of phenytoin 10mg if seizures not controlled.

d) Pyridoxine B 6 (Dose – 200mg I.V. Stat)
Step 3: Anesthesia Phentobarbitol.
7.1.2. Febrile convulsions.
7.1.2.1 Definition: Seizures precipitated by rapid rise of fever in the age group of 6months to
6yrs. (Most commen between the age of 14 and 18 months)
7.1.2.2 Types: (Two types)
1) Typical febrile convulsions.
a) Generalized seizures.
b) Duration <15mins.
c) Occurs only once in 24 hours.
d) Family history may be there.
e) Normal EEG after 7days.
f) No CNS infections.
g) CSF is normal.
h) No Neurological deficit.
i) Immediate recovery from unconsciousness.
2) Atypical febrile convulsions
a) Age < 6months or >6yrs.
b) Duration more than >15 mins.
c) Focal convulsions.
d) <1 seizure in 24 hours.
e) Neurologic deficit before or after attack.
f) Abnormal EEG 7 days later.
7.1.2.3 Treatment:
1. Control fever by:
• Tepid sponging.
• Paracetamol
2. Control convulsion by:
• Lorazepam/Midazolam IM/ Nasal spray.
3. Treat the cause of fever.
4. Prophylaxis:
a) Short term prophylaxis: Anticonvalscents should be used during the period
of fever.
b) Long term prophylaxis:
• Indications:
1) Atypical febrile convulsions.
2) Fever with neurological diseases like C.P, M.R.
• Drugs:
1) Sodium valproate – Dose 3-5mg/kg/day.
2) Phenobarbitone – Dose 3-5mg/kg/day.

7.2.Acute Bacterial Meningitis.
7.2.1 Definition: Inflammation of the meninges caused by bacteria.
7.2.2 Etiology:
1) Organism during neonatal period:
a) E Coli.
b) Klebsiella.
c) Listeria monocytogenes.
d) Streptococcus group B.
2) Organism during 2months to 12 years:
a) N. Meningitis meningococci.
b) H influenza type B.
c) S. Pneumoniae.
d) Pseudomonas aeruginosa.
e) Staphylococcus aureus.
f) Coagulase negative staphylococci.
g) Salmonella.
h) L Monocytogenes.
7.2.3 Route of infection:
1) Hematogenous:
2) Direct inoculation: from:
o Mastoiditis.
o Meningomyelocele.
o Orbital Cellulitis.
o Sinuses.
o Cranial osteomyelitis.
o Vertebral osteomyelitis.
o Dermal sinus tract.
3) Through VP shunts.
7.2.4 Pathogenesis:
a) Meningitis commonly results from hematogenous dissemination.
b) Entry to CSF is through choroid plexus of lateral ventricles and meninges and then circulates
to extra cerebral CSF and subarachnoid space.
c) Bacteria rapidly multiply because CSF concentration of compliment and antibodies are
inadequate.
d) Inflammation is produced by endotoxins of gram-negative bacteria and pneumococcal cell
wall components like teichoic acid, peptidoglycans and also by inflammatory mediators like
tumor necrosis factors (TNF) interleukin I, prostaglandin E.
o Inflammatory response is characterized by:
a. Neutrophilic infiltration.
b. Increased vascular permeability.
c. Alteration of blood brain barrier.
d. Vascular thrombosis.
o Pathological changes seen are:
a. Vasculitis.
b. Thrombosis of small cortical veins.
c. Occlusion of major venous sinuses.
d. Necrotizing arteritis producing subarachnoid hemorrhages.
e. Cerebral cortical necrosis.
f. Cerebral infarction.
g. Hydrocephalous.

7.2.5 Pathophysiology:
1.Inflammation of spinal nerves and roots produces meningeal signs.
2.Inflammation of cranial nerves produces cranial neuropathies.
3.Increased intracranial pressure produces oculomotor nerve palsy due to presence of temporal lobe
compression of nerve during tentorial herniation.
• 6th
C.N. palsy non-localizing signs of increased ICP.
• Increased ICP is due to:
a) Cell death (Cytotoxic cerebral edema).
b) Cytokine induced increased capillary vascular permeability and vasogenic cerebral
edema.
c) Increased hydrostatic pressure after obstructed reabsorption of CSF in the arachnoid
villus or obstruction of the flow of fluid from ventricles.
4.Hydrocephalous can be occur as an acute complication of bacterial meningitis.
a. Communicating hydrocephalous is due to adhesive thickening of the arachnoid villi
around the cistern at the base of the brain.
b. Obstructive hydrocephalous develops after fibrosis and gliosis of the aqueduct of Silvius
or the foramina or magendie and Luschka.
5.Raised CSF proteins are due to:
a) Increased vascular permeability of BBB. (Blood Brain Barrier)
b) Loss of albumin rich fluid from the capillaries and vein travel across the subdural space.
6.Subdural effusion is due to continued transudation
7.Decreased CSF sugar due to decreased glucose transport by the cerebral tissue.
8.Cerebral cortex damage is due to vascular occlusion, hypoxia, cerebritis, and toxic encephalopathy,
due to bacterial toxins; increased ICP, ventriculitis, and subdural effusion.
9.Above pathological lesions result in the impaired consciousness, Seizures, C.N. palsies, motor deficit,
sensory deficit and later psychomotor retardation.
10. Seizures are due to:
o Cerebritis.
o Infarction.
o Electrolyte imbalance.
o Fever.
7.2.6 Clinical features:
1. Classical features:
a) Fever, Headache, Vomiting, irritability, high pitched cry, convulsions, bulging
fontanels.
b) Deranged consciousness, jitteriness, photophobia, focal neurological signs, diplopia,
ptosis, bradycardia.
2. Signs of meningococcal irritation:
o Nuchal rigidity.
o Back pain.
o Positive Kernig’s sign, Brudzinski sign.
7.2.7 Nonspecific features:
1) Lethargy, irritability, anorexia, poor feeding, myalgia, arthralgia, tachycardia, hypotension.
2) Various cutaneous signs – petechiae, purpura.
3) DIC, shock.
4) Tache Cerebrale – raised red streak within 30-60 seconds after stroking skin with blunt
object.
7.2.8 Diagnosis:
1. CSF Examination.
o Color turbid.

o Microorganism on Gram’s staining and culture.
o Neutrophilic leukocytosis.
o Elevated proteins.
o Decreased glucose concentration.
o CSF culture positive.
o Latex tests.
2. Blood count raised.
3. Blood culture – may be positive.
4. CT Scan.
5. MRI.
7.2.9 Treatment:
1) Specific therapy.
2) Symptomatic treatment.
3) Supportive care.
4) Treatment of complications.
I. Initial antibiotic therapy (Empirical)
a. Penicillin with 3rd
generation cephalosporin.
b. Cefotaxime 200mg/kg/day every 6hrly+ Aminoglycosides.
OR
c. Ceftriaxone 100mg/kg/day once per day+ Aminoglycosides.
d. Vancomycin with 3rd
generation cephalosporin if resistant to penicillin suspected.
Once culture and sensitivity report come antibiotics can be given according to report.
• Duration of Antibiotic therapy.
a) 7-14 days depends upon types of organism.
b) Gram negative bacillary meningitis should be treated for 3weeks.
II. Symptomatic treatment:
1. Dexamethasone – 0.15mg/kg/dose every 6hrly for 2days in children older than 6weeks in
meningitis caused by H. influenza type B.
o Corticosteroid will be given for maximum benefit. It can be given 1-2 hours before
antibiotic therapy.
o Corticosteroids limit production of inflammatory mediators there by preventing
auditory damage caused by H. influenza.
2. Seizure management: Initial Diazepam 0.1-0.2 mg/IV/kg/ dose followed by phenytoin
loading dose 10mg/kg.
3. Increased ICP: (Intracranial pressure)
o Mannitol 0.5mg/kg.
o IV furosemide 1mg/kg.
4. Metabolic complications like hyponatremia, hypoglycemia, must be treated by specific
therapy.
5. Control of fever by cold sponging and paracetamol.
III. Supportive treatment:
1. I/V should be 2/3 maintenance.
2. Patient with septic shock required fluid resuscitation therapy with vasoactive agent such as
dopamine, epinephrine.
IV. Monitoring:
1. Monitor pulse, BP. & Respiratory rate.
2. Neurological Assessment should be frequent during first 72 hours.
V. Treatment of complications.
a) Subdural empyema.
o Subdural tap.

o Antibiotics.
b) Hydrocephalus – V.P. Shunt.
7.2.10 Complication of Meningitis:
1. Subdural effusion.
2. Hydrocephalus.
3. Brain abscess.
4. Cranial nerve palsies.
5. Mental Retardation.
6. Cerebral infarct.
7. SIADH.

7.3.Viral Meningoencephalitis.
7.3.1. Definition: Acute inflammation of meninges and brain by viruses.
7.3.2. Etiology:
Viruses:
1. Enteroviruses >80% cases.
2. Other 20% unclear
a) Arbo virus.
b) Herpes virus.
o HSV type I – focal involvement.
o HSV type II – diffuse involvement.
c) Varicella zoster.
d) Influenza.
e) Para influenza.
f) Measles, Mumps, Rubella.
g) Epstein-Barr virus.
h) Cytomegalo virus.
i) Rabies.
7.3.3. Pathogenesis:
a) Virus enter CNS through
1. Lymphatic system by
o Ingestion – Enterovirus.
o Inoculation of mucous membrane – Measles, Rubella, Herpes & Varicella.
2. Direct through nervous
3. Hematogenous – Entry by insects, bite, mosquito bite.
b) Neurologic damage by
1. Direct invasion of brain tissue.
2. By host reaction to viral antigen. (Immunological)
c) Pathologic lesions.
1. Meningeal congestion: Infiltration by mononuclear cells perivascular cuffs of
lymphocytes and plasma cells.
2. Tissue necrosis.
3. Myelin breakdown.
7.3.4. Clinical manifestation:
a) Diffuse cerebral involvement features:
1. Altered sensorium.
2. Convulsions.
3. Abnormal movements.
4. Abnormal behavior.
5. Fever.
b) Increased ICP – Headache, Vomiting.
c) Altered tone.
d) Altered reflexes.
7.3.5. Diagnosis:
1. Clinical:
a) Japanese B encephalitis occurs in epidemic.
b) Preceding rash suggest: Measles, mumps, rubella & Varicella.
c) Keratoconjunctivitis suggests: Adenovirus.
d) Cold & cough suggests: Respiratory virus.
e) Parotitis, pancreatitis, orchitis, suggests: Mumps virus.
2. Disease pattern specific to virus:

a) Japanese B encephalitis: Rapidly changing CNS signs.
b) Herpes simplex virus: Temporal lobe lesion – visual and auditory aphasia,
behavioral changes.
c) Rabies: Brain stem involvement
d) Coxsackie & Varicella zoster: Cerebellar involvement, ataxia, nystagmus.
3. CSF Examination:
a) Absence of organism on stain.
b) Pleocytosis initially polymorphs later lymphocyte few to thousands
c) Increased protein later but initially normal. Higher in HSV.
d) CSF sugar normal.
e) CSF culture sterile.
4. Virologic studies:
a) Virus isolation from CSF, urine, feces, nasopharynx.
b) Detection of viral antigen.
c) Detection of antibodies by ELISA, PCR and RIA.
o IgM in early sample.
o IgG in later sample.
5. CT Scan.
6. MRI.
7. ECG – Diffuse slow wave activity.
7.3.6. Treatment:
1. Supportive care:
a) Maintain airway patency by suction.
b) O2
c) Control of pyrexia by antipyretic.
d) Secondary infection control by antibiotics.
2. Treatment of raised ICP:
a) Raising head above heart level.
b) Restriction of fluid to 2/3 or ½ of daily requirement.
c) 3% saline.
20% mannitol 0.5-1gm/kg.
d) Furosemide – 1mg/kg.
3. Treatment seizures:
a) Hypoglycemia
10% Glucose 2ml/kg.
b) Hypocalcemia
Calcium: 10% Calcium gluconate. 1-2ml/kg
c) Correct electrolyte balance.
d) Anticonvalscents – phenytoin – 3-8mg/kg.
4. Specific therapy:
a) Herpes simplex – Acyclovir – 30 mg/kg/day. I/V for 10days.
7.3.7. Prevention by vaccination:
a) Polio vaccine.
b) MMR vaccine.
c) JE vaccine during epidemics.
Sequelae of mumps encephalitis.
Deafness (8th
CN involvement)

7.4.Tuberculous Meningitis.
7.4.1 Definition: Meningitis caused by organism mycobacterium tuberculae.
7.4.2 Pathogenesis:
1. It is secondary to some primary tubercular infection in body occurs within 3to6 months.
2. It develops during lymphohematogenous dissemination of primary infection.
3. There is metastatic caseous lesion in cerebral cortex called Rich focus or lesions may
localize in meninges.
4. Lesion increases in size and discharges tubercle bacilli into subarachnoid space.
Occasionally subependymal tubercles discharges tubercle bacilli into subarachnoid space.
5. Gelatinous exudate infiltration in cortico meningeal blood vessels causing vasculitis. Site
circle of Willis, cerebral arteries, performing vessel of basal ganglia.
6. Vasculitis leads to vascular obstruction causing infarction of cerebral cortex.
7. Ventriculitis.
8. Inflammatory exudate interferes with CSF flow causing communicating hydrocephalus.
Obstruction is at the level of basal cistern or interventricular foramina.
9. Generalized cerebral edema due to macroangiopathy.
10. Combination of vasculitis, infarction, cerebral edema, hydrocephalus, hemorrhage,
leukoencephalopathy results in severe brain damage.
11. Destruction of 2,3,4,6,7 cranial nerves is common.
12. Electrolyte abnormalities due to salt wasting or syndrome of inappropriate antidiuretic
hormone secretion is also contributes to pathophysiology of TBM.
13. Motor root damage by exudates immunological mechanism is also seen.
1) Age 6monyhs to 6years (9months to 3year is common)
2) Onset – Usually indious rarely may be acute or chronic.
3) Three stages:
a) Stage I – Stage of invasion (Prodromal stage)
A. Duration usually 1-3weeks.
B. Non-specific symptoms like fever, headache, irritability, drowsiness,
malaise, photophobia, (Lies curled up in bed, recent handling) loss of
developmental milestones, altered behavior.
b) Stage II (Stage of meningeal irritation)
A. Fever, headache, vomiting, seizures.
B. Signs of meningeal irritation, neck rigidity, Kernig sign, Brudzinski sign.
C. Cranial nerve palsies: Usually II, III, IV, VI, VII. 6th
cranial nerve
commonly affected.
D. Focal neurological signs like hemiplegia, monoplegia, paraplegia and
quadriplegia.
E. Movement disorders: Hemiballismus, tremors and myoclonic jerks.
F. Speech impairment.
G. Fundus examination: Papilledema, choroid tubercles.
H. Hydrocephalus.
c) Stage III – Coma (Stage of cerebral involvement)
A. Coma.
B. Hemiplegia or paraplegia.
C. Decerebrate posturing.
D. Deterioration of vital signs – Pupil dilated and fixed, pulse rapid respiration
irregular.
E. Hypertension.
F. Eventually death.

G. Fundus Examination: Optic atrophy
Clinicopathological correlation
1 Meningeal Exudates Meningeal sign: Cranial nerve involvement,
Hydrocephalus.
2 Brain parenchymal involvement Clouding of consciousness, seizures, hypothalamic and
brainstem signs.
3 Arteritis, vascular obstruction Focal neurological deficit
4 Hypersensitivity response Massive brain edema, increased ICP.
7.4.4 Diagnosis:
1) High clinical suspicion.
2) CSF examination (LP). Microscopically – may be xanthochromic.
a) Leukocyte count increased 10-500 cells/mm3
lymphocytes predominates but
initially polymorphonuclear leukocytes may be present.
b) CSF glucose – less than 40mg% rarely bellow 20mg%
c) Protein markedly high 400-500mg% secondary to hydrocephalic block.
d) Acid fast CSF stains may be positive in 30% of cases.
e) Cobweb formation positive.
f) CSF – ADA (adenosine deaminase assay) level raised >10 units/L
3) Mantoux test. May be positive in 50%
4) X-Ray chest. positive in 50%
5) Culture of gastric aspirate and urine may be positive.
6) Radiographic studies – CT or MRI of Brain may show basilar enhancement,
communicating hydrocephalous, cerebral edema, early focal ischemia, tuberculoma and
cerebral infarction.
7) Serum & CSF ADA or Biochemical markers:
a) Gas liquid chromatography to detect tuberculostearic acid. Result in 24hrs.
b) ADA level > 10unit/L of CSF ADA.
c) Bromide partition test.
8) Molecular Biology PCR useful test.
7.4.5 Differential Diagnoses:
1. Pyogenic meningitis especially partially treated pyogenic meningitis.
a) CSF ADA level is more than 10 U/L in TBM.
b) Cob web formation in CSF in TBM
2. Cryptococcal meningitis – culture diagnostic.
3. Encephalitis.
4. Enteric encephalopathy.
5. Brain abscess.
6. Brain tumor.
7. Chronic subdural hematoma.
7.4.6 Treatment:
1. Specific treatment for – 1year.
a) Four drugs for 2 months HRZE.
1) Isoniazid – 10mg/kg.
2) Rifampicin – 10mg/kg.
3) Pyrazinamide – 25mg/kg.
4) Streptomycin - 30mg/kg.
5) Ethambutol - 20mg/kg.
b) Three drugs for 9months.
1) Isoniazid – 10mg/kg.

2) Rifampicin – 10mg/kg.
3) Ethambutol - 20mg/kg.
c) Corticosteroids – Initial parenteral dexamethasone 0.2 – 0.4 mg/kg/I.V.
Prednisolone 2mg/kg/day/orally for 4wk and then taper over the next 4wks.
Advantages:
o Reduces vasculitis.
o Reduces inflammation.
o Lowers intracranial pressure (ICP)
2. Symptomatic treatment.
a) Seizures: Diazepam, Phenytoin.
b) Fever: Cold sponging, paracetamol.
3. Supportive treatment.
a) Increase ICP treated by:
I. 2/3 of maintenance fluids/day 3% saline.
II. Mannitol – 0.5-2 gm/day/I.V. for three days
III. Dexamethasone 0.25 – 0.6mg/kg/day in 2 divided doses for 3days.
IV. Orally glycerol – 1 gm/kg/dose 2to6 hourly.
V. Acetazolamide – 50to 70mg/kg/day 8hourly for 2to 3 days.
b) Nutritional support, fluid therapy.
c) Tube feeding of comatose patient.
d) Care of bowel and bladder functions.
4. Surgery: V.P. shunt for hydrocephalus.
5. Physiotherapy:
6. Follow up: Assessment for 1yr for:
a) Improvement in disease.
b) Side effects of drugs.
7.4.7 Prognoses:
I. Good in stage I.
II. Most of patients of stage III may have permanent disabilities like blindness, deafness,
paraplegia, diabetes insipidus, and mental retardation.
III. Prognosis in younger infants is worst.
7.4.8 Complications & Sequelae:
2. Psychiatric disorders.
3. Seizures & Epilepsy.
4. Blindness.
5. Deafness.
6. Hemiparesis.
7. Ophthalmoplegia.
8. Endocrinopathies.
a) Obesity, Frohlich syndrome.
b) Hypogonadism.
c) Sexual precocity.
d) Diabetes insipidus.
e) Growth retardation.

7.5. Microcephaly.
7.5.1. Definition: Head circumference bellow 3 SD of the normal for the age and sex.
Head circumference normal values:
S. No. Stage of growth & development Normal Finding
1 Birth 33 – 35cm
2 3 months 40 cm
3 6 months 43 cm
4 1 year 46 cm
5 2 years 48 cm
6 3 years 49 cm
7 7 years. 50 cm
8 Above 12 years. 52 – 54 cm
7.5.2. Types:
1. Primary:
a) It is genetic in origin.
b) Occurs during first 7months of gestation.
c) Can have slanted forehead.
Causes:
1) Familial (Autosomal) recessive has slanted forehead.
2) Autosomal dominant.
3) Chromosomal abnormalities:
o Down syndrome.
o Cri du chat syndrome.
o Cornelia de lange syndrome.
o Rubinstein – Taybi syndrome.
o Smith Lemli – opitz syndrome.
o Edward syndrome.
2. Secondary microcephaly:
a) It is not genetic in origin.
b) Result of insult to brain during last 2 months of pregnancy or perinatal period.
7.5.3. Causes:
a) Irradiation of mother pelvis during first trimester.
b) Maternal alcoholism.
c) Drugs:
1. Cocaine.
2. Heroin.
3. Antiepileptic drug.
4. Anticancerous drugs.
5. Mega dosage of Vit A.
6. Mercury.
d) Intrauterine infection – TORCH
1. Cytomegalo virus.
2. Rubella.
3. Toxoplasmosis.
4. Herpes simplex.
5. Zika virus infection.
e) Meningitis, Encephalitis.
f) Malnutrition:
g) Metabolic (Maternal diabetes mellites)
h) Hypoxic ischemic encephalopathy

i) Hyperthermia.
o Damage to secondary microcephaly is:
1. Gliosis.
2. Calcification.
3. Encephalomengitis.
4. Porencephaly.
7.5.4. Investigation/ Diagnosis:
1. Serial measurement of head.
2. Karyotyping.
3. CT/MRI.
4. Serum and Urine amino acid level.
5. TORCH screening of mother and child IgG/IgM antigen, antibody.
6. Urine culture for cytomegalovirus.
7.5.5. Treatment:
1. Genetic and family counselling.
2. Management of mental retardation.
7.5.6. Differential diagnosis:
Craniosynostosis.

7.6. Craniosynostosis.
7.6.1. Definition: Premature closure at birth of one or more cranial sutures.
7.6.2. Features:
1. Overriding of sutures.
2. Small head.
3. Signs of increased intracranial pressure.
o Early diagnosis is must.
7.6.3. Diagnosis:
1. X-Ray skull.
2. CT Scan.
7.6.4. Treatment:
Surgical (coronal incision – ear to ear)

7.7. Hydrocephalous.
7.7.1. Definition:
Dilatation of ventricles leading to large head circumference above 97th
percentile for the age, caused
by impaired circulation or absorption of CSF or increased production by choroid plexus.
Amount of CSF
o Infant 50ml.
o Adult 150 ml.
7.7.2. Types:
1. Obstructive hydrocephalous.
1) Aqueductal stenosis.
2) Intrauterine viral infections – mumps, meningoencephalitis.
3) Vein of Galan malformation.
4) Posterior fossa brain tumors.
5) Arnold – Chiari malformation.
6) Dany – walker syndrome.
2. Communicating hydrocephalous.
1) Subarachnoid hemorrhage.
2) Tubercular meningitis.
3) Bacterial meningitis.
4) Intrauterine infections.
5) Arnold – Chiari malformation.
7.7.3. Clinical features: Clinically
1. Large head.
2. Bulging fontanel.
3. Sutural separation.
4. Dilated scalp vein.
5. Sun setting signs. (Sclera become visible above cornea).
6. Spastic diplegia.
7. McEwin sign/ cracked pot sign (Resonant note on percussion of skull)
8. Ataxia.
9. Featured of increased ICP.
a) Irritability.
b) Vomiting.
c) Cranial nerve palsies.
d) Papilledema.
e) Personality changes.
f) Decrease school performance.
• Prominent occiput suggests Dandy – walker syndrome.
• Small occiput with myelomeningocele suggests Arnold – Chiari malformation.
1. Hydranencephaly/ Hydraencephaly.
2. Hemolytic Anemia.
3. Megalencephaly.
4. Thick cranium in
a) Rickets.
b) Osteopetrosis.
7.7.5. Investigations:
1. Serial measurement of head circumference.
2. Cranial USG.

3. CT Scan.
4. MRI.
7.7.6. Treatment:
1. Find out the cause and treat it.
2. Medical treatment:
• Indication:
1. Subarachnoid hemorrhage.
2. Meningitis.
• Treatment:
1. Acetazolamide.
2. Furosemide.
3. Oral glycerol.
3. Surgical treatment:
• Indication.
1. Tumor.
2. Cysts.
3. A.V. malformation.
• Treatment.
1. Ventriculoperitoneal shunt.
2. Ventriculoarterial shunt.
• Complication of shunt.
1. Shunt infection.
2. Shunt nephritis.
3. Blockage.
Dandy – walker malformation: It is due to agenesis of cerebellar vermis with obstruction of
foramina of magendie and Luschka.
o A cystic dilation of 4th
ventricle due to development of failure of the root of the IV
ventricle
Arnold Chiari malformation: (Small posterior fossa)
Two types:
Type I Adolescent (not associated with hydrocephalus)
Type II There is displacement of cerebellar tonsil into cervical canal characterized by:
a) Progressive hydrocephalus.
b) Meningomyelocele.
c) Anomaly of hind brain.
Cause: Failure of pontine flexure during embryogenesis leading to a elongation of IV
ventricle, kinking of brainstem, displacement of inferior vermis, pons, medulla into the
cervical canal.

7.8. Cerebral palsy. (Little Disease)
7.8.1 Definition:
• Group of motor syndrome.
• Resulting from insult to developing brain antenatally and postnatally.
• Manifesting as disorder of posture and movement.
7.8.2 Etiology: Cerebral anoxia is most important etiological factor leading to chronic motor disability.
Causes:
a) Antenatal causes (80%)
1. Congenital anomalies.
2. Intrapartum asphyxia.
3. Intrauterine maternal infection.
b) Natal causes: Birth asphyxia.
c) Postnatal causes:
1. Low birth wt. babies especially less than 1000gm of birth weight.
2. Prematurity.
3. Vascular – intracerebral hemorrhage.
4. Cerebral ischemia.
5. Endocrine – Hypothyroidism.
6. Postnatal asphyxia.
7. Kernicterus.
8. Metabolic – Hypoglycemia.
9. Postnatal infections.
7.8.3 Risk factors of CP:
1. Before pregnancy.
a) History of fetal wastage.
b) Maternal thyroid disorder.
c) Family history of mental retardation.
2. During pregnancy.
a) Treatment with estrogen, progesterone, thyroid drugs.
b) Maternal seizures.
c) Polyhydramnios.
d) Eclampsia.
e) Twin pregnancy.
f) Congenital malformation.
g) Fetal growth restriction.
h) Abnormal fetal presentation.
3. During labor – Premature, Separation of placenta.
4. Postnatal period. – Newborn encephalopathy.
7.8.4 Clinical manifestation:
i. Motor handicap. – Spasticity, Hypotonia, Athetosis.
ii. Delayed milestone.
iii. Associate features:
1. Learning disabilities, feeding difficulties.
2. Involuntary movements like athetosis, chorea, seizures.
3. Motor speech disability.
4. Hearing impairment, Visual impairment, squint.
5. Abnormalities of muscle tones, Reflexes.
6. Gait abnormalities.
7.8.5 Classification:

A. Physiological:
a) Spastic cerebral palsy:
i. Most common C.P. (70 – 80%)
ii. Cause – injury to UMN of pyramidal tract.
iii. Features – Truncal hypotonia in first year.
Spasticity in 2nd
year with increased deep tendon reflex and clonus.
b) Dyskinetic C. P.:
i. 10 – 15% cases.
ii. Cause – Injury to basal ganglia (Kernicterus)
iii. Features –
a) Variable tone of whole body.
b) Involuntary movement
c) Hearing impairment is common
d) Motor speech disability.
e) Ataxic C.P.:
i. 5% cases.
ii. Causes – Injury to cerebellum.
iii. Features:
a) Abnormalities of voluntary movement.
b) Abnormalities of basal ganglia.
c) Gait wide based, Unsteady gait.
d) Abnormalities of muscle tone.
f) Mixed C.P.:
B. Classification by distribution:
a) Spastic diplegia.
1. Spasticity of large greater than arms.
2. Drags legs while crawling (Commando crawl)
3. Associated with LBW, Birth asphyxia, ICH.
4. Features:
1) Seizures minimal.
2) Learning disabilities.
3) Language problem less common.
4) Contractures.
5) Mental Retardation less common.
6) Neuropathological (most severe form) finding: Periventricular
Leukomalacia.
b) Spastic quadriplegia.
1. Involves all four limbs.
2. Associated with LBW, Severe Birth asphyxia.
3. Features:
• Mental retardation – more commen.
• Seizures – more commen.
• Feeding difficulty.
• Scoliosis.
• Orthopedic problems.
• Speech and visual abnormalities are commen.
• MRI – Periventricular leukomalacia and multicystic cortical
encephalomalacia.
c) Spastic Hemiplegia.

1) Causes – Cerebrovascular insult, embolic phenomenon, and vascular
malformation.
2) Features:
• Seizures.
• Cognitive function spared.
• Motor impairment.
• Language difficulties.
d) Extrapyramidal. Athetoid, dyskinetic.
1) Hypotonic infant.
2) Feeding difficulties with tongue thrust, drooling.
3) Speech difficulties
4) Seizures uncommon.
5) UMN lesion not present.
6) Mostly intellect is preserved.
7) Lesion – posterior putamen and ventrolateral thalamus.
7.8.6 Markers for early identification of CP:
1. Decreased alertness.
2. Decreased spontaneous activity.
3. Constant fisting with adduction of thumb beyond 2mths of age.
4. Delayed social smile.
5. Primitive reflexes persisting beyond 4-6months.
6. Delayed milestones.
7. Persistent tone abnormalities.
8. Persistent asymmetry in posture, movement and reflexes.
7.8.7 Diagnosis:
1. History.
2. Physical examination persistent – plantar extensor beyond 2 yrs.
3. MRI,
4. Additional studies.
a) Test of hearing.
b) Test of visual function.
c) Genetic evaluation of patient for congenital malformation or metastatic disorders.
7.8.8 Treatment:
1. Physiotherapy: Exercises to prevent contractures.
a) Hydrotherapy.
b) Massage.
2. Symptomatic treatment.
• Anticonvalscents for seizures.
3. Spasticity management.
a) Implantation of intrathecal baclofen pump.
b) Rhizotomy – Spinal nerve roots are cut.
c) Tenotomy of Achilles tendon.
d) Botulinum toxin injection in muscles.
e) Drugs:
• Dantrolene sodium 1mg/kg/BD.
• Benzodiazepines.
• Levodopa.
 Rigidity, dystonia.
 Spastic quadriplegia.
• Carbamazepine. For dystonia

4. Orthopedic management.
5. Family support.
6. Hyperbaric oxygen – no importance.
7. Adaptive Equipment. For spastic diplegia such as walker, poles, standing frames.
8. Quadriplegia. Management by
a) Motorized wheel chairs.
b) Special feeding devices.
c) Modified typewriters.
d) Special seating arrangements.
9. Enhancement of communication skills.
a) Use of bliss symbols.
b) Talking typewriters.
c) Adapting computers.
10. Behavior problems.
Early identification and management of psychological problems
11. Ophthalmologic management.
Squint, Nystagmus, Optic atrophy – management by ophthalmologists.

7.9.Mental Retardation.
7.9.1 Definition: Group of disorder that have in common deficit of:
a) Adaptive functions.
b) Intellectual function before maturity is reached (18years).
7.9.2 Diagnostic criteria.
a) Onset before – 18years.
b) Subaverage IQ ,70.
c) Defect in adaptive functioning – deficit of two or more of following ten adaptive skills.
7.9.3 Diagnosis:
Deficit of two or more of following ten adaptive skills.
1. Self-care.
2. Communication.
3. Home living.
4. Social skills/ Interpersonal skills.
5. Community resources use.
6. Self-direction.
7. Health and safety.
8. Functional academic skills.
9. Leisure.
10. Work.
IQ is tested by following tests:
1. Baily scale of infant development.
2. Stanford – Binet test.
3. Wechsler intelligence scale.
Test for adaptive functioning: Assess Adaptive behavior in four areas:
1. Communication.
2. Daily living skills.
3. Socialization.
4. Motor skills.
7.9.4 Classification:
i. Classification according to severity.
S No. Severity Range
1 Mild 55 – 70
2 Moderate 35 – 55
3 Severe 20 – 35
4 Profound < 20
ii. Educational Classification.
S No. Particulars Range
1 Educable 50 – 70
2 Trainable 35 – 50
3 Custodian < 35
7.9.5 Etiology: Causes more in boys 2:1
1. Hereditary disorders:
a) Inborn errors of metabolism.
• Phenylketonuria.
• Hurler disease.

• Taysach’s disease.
b) Single gene abnormality.
• Muscular dystrophy.
• Neurofibromatosis.
• Tuberous sclerosis.
c) Chromosomal aberration.
• Translocation.
• Fragile X Syndrome.
2. Early alteration of embryonic development:
a) Down’s Syndrome.
b) Prenatal causes.
o Intrauterine infection.
o Drugs.
o Alcohol.
3. Other pregnancy related problems:
a) Perinatal problem.
b) Fetal malformation.
c) Placental insufficiency.
d) Prematurity.
e) Hypoxia.
f) Trauma.
4. Acquired childhood diseases:
a) Encephalitis.
b) Meningitis.
c) Cardiac arrests.
d) Drowning.
e) Intoxication.
5. Unknown.
6. Environmental & Social problem;
a) Deprivation.
b) Childhood psychosis.
c) Neurosis.
7.9.6 Pathogenesis: CNS maturation is influenced by following factors:
i. Genetic.
ii. Molecular.
iii. Autocrine.
iv. Paracrine.
v. Endocrine.
vi. Receptor in brain.
vii. Signaling molecule in brain.
Any defect in above factors can affect mental development.
7.9.7 Clinical Manifestation:
i. Dysmorphism: Genetic syndrome like microcephaly.
ii. Associate dysfunction:
a) Cerebral palsies.
b) Mutism.
iii. Failure to meet age appropriate expectation.
iv. Lack:
a) Lack of visual response.
b) Lack of auditory response.

c) Unusual tone.
d) Unusual posture.
v. Motor delay.
vi. Language delay.
vii. Behavior problems.
viii. Academic under achievement.
ix. Failure to interact with environment.
7.9.8 Laboratory tests:
1. Neuro imaging.
2. Metabolic screening.
a) Lysosomal enzyme in lymphocyte.
b) Urinary organic acid.
c) Plasma amino acids
d) Blood lactate.
3. Molecular testing. For fragile X syndrome
4. Chromosomal testing.
5. EEG – Specially in seizures disorders.
6. Karyotyping.
7.9.9 Differential diagnoses:
i. Autism: Language and social adaptation skills affected more in autism.
ii. Poorly controlled seizures
iii. C.P.
a) Motor skill more affected than cognitive skills.
b) Pathological reflex.
c) Tone changes.
iv. Hearing loss, vision loss.
v. Communication disorders
7.9.10 Management:
1. Mostly mental retardation is not treatable.
2. Associated impairments are treatable.
3. Medicational therapy: helpful in treating:
4. Supportive care:
5. Interdisciplinary management:
a) Psychological therapy.
b) Speech/language therapy.
c) Physical therapy.
d) Occupational therapy.
e) Audiology.
6. Nutrition.
7. Nursing care.
8. Social work.
9. Educational services.
10. Recreational activity.
11. Family counselling.
12. Periodic re – evaluation.
7.9.11 Prevention
1. Awareness of bad effects of alcoholism and smoking in mothers.
2. Promote folic acid supplements in early prenatal period.
3. Encourage use of railing guards to prevent accidents.
4. Safe driving.

5. Avoid adolescent pregnancy.
6. Immunization.
a) Congenital infection.
b) Meningitis.
c) Encephalitis.
7. Newborn screening – for metabolic disorders.
8. Prevent lead poisoning.
9. Lock – up medicine, poison and firearms.
Preventable causes of mental retardation.
1. Neonatal hypoglycemia.
2. Neonatal hypothyroidism.
3. Galactosemia.
4. Phenylketonuria.
5. Hypoxia.

7.10. Guillain Barre’s Syndrome. (Post – infectious polyneuropathy)
7.10.1 Definition: It is a post-infectious polyneuropathy.
7.10.2 Etiopathology:
1. Cause not understood.
2. There is demyelinating neuropathy.
3. Most probably caused by autoimmune mechanism triggered by viruses or other agents like
mycoplasma, campylobacter infection about 2to3 weeks prior to illness.
4. There may be axonal degeneration.
5. Mainly involves motor nerves.
6. May involve sensory and autonomic nerve.
1. Age –
• Affects all ages.
• More commen in 5to14yrs.
2. Pain in muscles is earliest symptoms.
3. Weakness in limbs follows non-specific viral infection of gut, respiratory system by about
10 days.
4. Landry’s ascendinding paralysis:
a) Weakness usually begins with lower extremities and ascend upward – involving
trunk, upper limbs and finally bulbar muscles.
b) Paralysis is symmetrical in most of cases.
c) Present in both proximal and distal groups.
d) Paralysis is flaccid type (LMN Paralysis)
e) Tendon reflexes are lost (Areflexia).
f) Onset is gradual and progresses over days to weeks
5. Paresthesia may occur. Sensory deficit is frequent. Glove stoking distribution is present.
6. Bulbar involvement occurs in 5% cases.
o Intercostal muscles weakness causes respiratory insufficiency.
o There may be dysphagia.
o There may be respiratory failure.
o Sometimes there may be symptoms of meningoencephalitis or meningitis.
o Extraocular muscle involvement is rare.
o 3rd
cranial nerve palsies may be present.
7. Autonomic nervous system involvement: Monitor B.P. and cardiac rate for:
o Postural hypotension.
o Episodes of profound bradycardia.
o Occasional asystole.
8. Urinary incontinence or retention:
o It is transient complication in 70% cases.
9. Chronic relapsing polyradiculoneuropathy:
Definition:
• Recurs intermittently.
• Does not improve over months to year.
10. Congenital G.B. Syndrome:
Present in neonate as:
o Generalized hypotonia.
o Weakness.
o Areflexia.
o CSF slows albuminocytological dissociation.
o Electrophysiological criteria positive.

11. Miller fisher syndrome:
• Internal ophthalmoplegia.
• Ataxia.
• Areflexia.
12. Poor prognostic features.:
o Cranial nerve involvement.
o Patient requiring intubation.
o Maximum disability at the time of presentation.
13. Course of disease:
• Spontaneous recovery starts in 2 to 3 weeks. Complete recovery in 6months to
2years.
• Improvement begins inverse to direction of involvement.
• Bladder function improve first.
• Lower extremities improve last.
• Tendon reflexes recover lasts.
7.10.4 Diagnosis:
1. CSF Examination.
a) Albuminocytological dissociation is diagnostic.
b) CSF protein rises late in first week reaches maximum in 2nd
to 3rd
week.
c) No pleocytosis or few cells count above 50 is strong evidence against GBS.
d) CSF glucose is normal.
2. Motor nerve conduction – Reduced.
3. Sensory nerve conduction – Slow.
4. Electromyogram – Acute degeneration of muscles.
5. CPK – Normal or mildly raised.
6. Antiganglioside antibodies – Against GM1 & GD1 positive.
7. Serological tests for campylobacter jejune helps in establishing cause.
7.10.5 Differential Diagnoses:
a) Poliomyelitis.
b) Polymyositis.
c) Transverse myelitis.
d) Cerebellar ataxia.
e) Traumatic neuritis.

S. No. Sign & Symptoms Polio GBS Transverse Myelitis Traumatic neuritis
1 Progression of
paralysis
24 – 48 hrs. onset to full paralysis. From hour to 10days. From hour to 4days. From hour to 4days.
2 Fever onset High always present at onset of
flaccid paralysis, gone the following
day
Not common Rarely present. Commonly present before,
during and after flaccid
paralysis.
3 Flaccidity Acute asymmetrical, paroxysmal
symmetrical.
Acute symmetrical distal. Acute, lower limbs. Acute asymmetric limb
4 Muscle tone Diminished Diminished Diminished in lower
limbs
Diminished in limb
5 DTRs Decreased or Absent Absent Absent in lower limbs
early hyper reflexia late.
Decreased or absent
6 Sensation Severe myalgia and backache, no
sensory changes
Cramps, tingling,
hypoanaesthesia of palms
and soles
Anesthesia of lower limbs
with sensory level.
Pain in gluteal region.
7 Cranial nerve Only when bulbar and bulbospinal Often present affecting
nerves VII, IX, X, XI, XII
Absent Absent
8 Respiratory
insufficiency
Only when bulbar and bulbospinal In severe cases Sometimes Absent
9 CSF: WBCs
Protein
High WBCs
Normal or slightly increased.
<10WBCs high Normal
Normal or slightly
elevated
Normal.
Normal.
10 Bladder dysfunction. Absent Transient Present Never
11 Nerve conduction
velocity 3rd
wk.
Abnormal anterior horn cell disease
(normal during the first two wk0
Abnormal demyelination Normal or abnormal, no
diagnostic value
Abnormal in sciatic nerve
12 EMG – 3wk. Abnormal Normal Normal Normal
13 Sequelae at 3
months and up to a
year.
Severe asymmetrical atrophy,
skeletal deformities developing
later.
Symmetrical atrophy of
distal muscle
Flaccid diplegia, atrophy
after years
Moderate atrophy, only in
affected lower limb.

7.10.6 Treatment:
1. Patient with mild disease showing no progression should be observed without treatment
in hospital.
Disease is self-limiting.
2. Patient should be admitted – if the disease is severe and progressive, for observation for
the involvement of respiratory muscles.
3. Drug therapy:
i. Immunoglobulin I.V.
Dose: 400mg/kg/day.
Route: I.V.
Duration: 5 days.
ii. Steroids:
1) Prednisolone:
Dose: 1-2mg/kg/day for 3wks then tapered off.
2) Methyl Prednisolone:
Dose: 30mg/kg/day in single dose.
Duration: 3days.
iii. Plasma pheresis:
1) Ten exchanges daily.
2) Removes antibodies.
iv. Immunosuppressive agents: for chronic cases are under trial.
v. I.V. Immunoglobulin with interferon: Effective in refractory cases.
vi. Plasmapheresis:
4. Treatment of secondary bacterial infection by antibiotics:
5. Nursing care:
a) Monitoring of pulse, B.P. and Respiration.
b) Care of bowel and bladder.
c) Prevention of bed sores.
d) Nasogastric intubation if dysphagia.
6. Supportive treatment.
a) Assisting ventilation by ventilator if respiratory failure.
b) Tracheostomy if required.
7. Physiotherapy.
7.10.7 Prognosis:
o Good.
o Most of the cases improve completely.

7.11. Hemiplegia.
7.11.1 Definition: It is paralysis of one side of body with or without facial nerve involvement.
7.11.2 Etiology:
1. Infections:
a) Meningitis.
b) Encephalitis.
c) Retropharyngeal abscess causing thrombosis of carotid artery or its compression.
2. Vascular:
a) Vascular malformations.
b) Moyamoya disease.
c) Takayasu disease.
d) Migraine.
e) Fibromuscular dysplasia.
f) Ehlers – Danios syndrome.
g) Marfan syndrome.
3. Trauma:
a) Intraoral trauma to peritonsillar area can lead to internal carotid artery thrombosis.
b) Head injury
• Subdural hematoma.
• Intracerebral bleed.
4. Cardiac Disease:
1) Cyanotic CHD.
a) Arterial / venous thrombosis.
b) Embolism.
c) Brain abscess.
d) Polycythemia.
5. Hematological:
a) Sickle cell disease.
b) Polycythemia Vera.
c) Leukemia.
d) Antithrombin C deficiency.
e) Dysfibrinogenemia
6. Metabolic disorders:
a) Juvenile diabetes mellitus.
b) Hypoglycemia.
c) Mellaus syndrome.
d) Homocystinuria.
e) Pyruvate dehydrogenase deficiency.
7. Collagen vascular diseases. And vasculitis.
8. Congenital porencephaly.
9. Brain tumors.
10. Status epilepticus or Todds paralysis.
11. Lipid abnormalities.
12. Alternate hemiplegia of childhood.
a) Age 2 – 8 months.
b) Intermittent Hemiplegia for minutes to weeks.
c) Associated choreoathetosis or dystonic movement.
d) Progressive mental retardation.
e) Prognosis poor.
13. Acute stroke syndrome.

a) Arterial thrombosis.
b) Venous thrombosis.
c) Intracranial hemorrhage.
d) Arterial embolism.
7.11.3 Localization of site lesion:
1. Cerebral cortex: Contralateral features.
a) Arm is weak than leg.
b) Cortical sensory loss.
c) Aphasia.
d) Apraxia.
e) Seizures
f) UMN 7th
C.N. palsies.
2. Internal capsule contralateral.
a) Dense Hemiplegia.
b) 7th
CN palsy. – UMN type.
c) Hemianesthesia.
3. Mid brain (Weber syndrome)
a) Contralateral Hemiplegia.
b) Ipsilateral 3rd
CN palsy.
4. Pons. (Millard Gubler syndrome)
b) Ipsilateral VI&VII CN palsies (LMN)
5. Medulla (Jackson syndrome)
b) Ipsilateral IX. X &XI CN palsies (LMN)
6. Spinal cord lesion
a) Ipsilateral Hemiplegia
b) Post column sensory loss.
c) Contralateral temperature and pin prick loss.
7.11.4 Diagnosis:
1. History:
2. Physical Examination: Searching for underlying disease process.
a) Evidence of trauma.
b) Evidence of infection.
c) Metabolic disease.
d) Hematological disorder.
e) Neurocutaneous syndrome.
f) Increased ICP or hydrocephalous.
3. CT Scan.
4. EEG.
5. MRI.
6. Cerebral Angiography.
7. Electrocardiography.
8. Echocardiography.
9. Test for vasculitis & connective tissue Disorders:
a) ESR.
b) C3 C4
c) RA factor.
d) ANA (Antinuclear antibodies.
10. Lipid disorder test.

11. Coagulation disorder test.
12. Hematological disorder test
a) Sickle cell disease.
b) Thrombocytopenia.
13. Metabolic disorder test.
14. Infection etiology test.
a) Blood count.
b) CSF examination.
7.11.5 Treatment:
1. Treatment according to cause.
2. Infections are treated with antibiotic.
3. Increased ICP – treated by:
a) Mannitol.
b) Dexamethasone.
4. Specific therapies.
a) Low molecular weight heparin for venous sinus thrombosis.
b) Thrombolytic therapies within 3hrs after stroke.
c) Regular blood transfusion for sickle cell disease.
d) Immunosuppressant for vasculitis.
e) Surgical evacuation for large blood clot.
5. Rehabilitation;
a) Speech therapy.
b) Physical therapy.
c) Occupational therapy.
d) Psychological services.
e) Special education.

7.12. Movement Disorders. They can be:
1) Involuntary movements and/or
2) Abnormally in posture, tone, balance and fine motor control.
7.12.1. Ataxia.
1. Definition: Inability to make smooth, accurate and coordinated movements, may be:
a. Generalized or
b. Primarily affect gait, hand-arms.
2. Etiology: It is caused by disorders of,
a. Cerebellum.
b. Post column of spinal cord.
c. Peripheral nerves.
d. Labyrinth.
3. Causes:
a. Congenital.
1) Agenesis of vermis or cerebellum.
2) Arnold Chari malformation.
3) Dandy-walker syndrome.
4) Encephalocele
b. Degenerative.
1) Friedreich ataxia.
2) Ataxia – telangiectasia.
3) Familial ataxia.
c. Infection.
1) Acute cerebellar ataxia.
2) Cerebellar abscess.
3) Labyrinthitis.
4) Coxsackie virus infection.
5) Diphtheria.
6) Echo virus.
7) Infectious mononucleosis.
8) Infectious – polyneuritis.
9) Japanese B encephalitis.
10) Mumps encephalitis.
11) Mycoplasma pneumonia.
12) Pertussis.
13) Poliomyelitis.
14) Varicella.
15) Typhoid.
d. Drug – Toxic effects.
1) Carbamazepine.
2) Clonazepam.
3) Phenobarbitone.
4) Phenytoin.
5) Primidone.
6) Alcohol
7) Thallium.
e. Metabolic.
1) Abetalipoproteinemia.
2) Maple syrup disease.
3) Refsum disease.

f. Neoplastic.
1) Frontal lobe tumor.
2) Cerebellar tumor.
3) Neuroblastoma.
4) Pontine tumor.
g. Tic paralysis.
h. Traumatic.
i. Vascular migraine.
4. Clinical features:
1) Acute onset – infection, trauma, toxic agents.
2) Indigenous onset – Brainstem tumor, Cerebellar tumor.
3) Family history – positive in hereditary ataxia.
4) Visual and auditory disability points towards inherited ataxias.
5) Horner syndrome and cranial nerve palsies indicate brainstem disease.
6) Cerebellar defect – classical findings:
a. Hypotonia.
b. Nystagmus.
c. Staggering gait.
d. Titubation.
7) Findings of dysfunction of post column of spinal cord – Romberg sign positive
with eye closed.
8) Sensory ataxia findings:
a. Standing difficulty.
b. Walking difficulty.
c. Wide based gait.
d. Romberg sign positive.
e. Loss of position sense.
f. Loss of vibration sense.
g. Pain, light, touch, temperature – not affected.
7.12.2. Acute cerebellar Ataxia.
1. Age 1-3 years.
2. Diagnosis by exclusion.
3. Often follows viral illness especially varicella, coxsackie virus, echo virus by 2-3 weeks.
4. Etiology – Auto immune response to viral agent affecting cerebellum.
5. Features – acute onset, vomiting, truncal ataxia, horizontal nystagmus, dysarthria.
CSF: Normal initially, Protein increased.
6. Improvement in few weeks to two months.
7. Prognosis excellent, few may have sequalae:
a. Behavior disorders.
b. Speech disorders.
c. Ataxia, incoordination.
7.12.3. Ataxia Telangiectasia.
1. Autosomal recessive degenerative disorder.
2. Causes – Mutation in ATM gene located at 11q22 – q23. Additional chromosomal breaks.
3. Features:
a. Ataxia begin at about 2 years of age, progress to loss of ambulation by
adolescents.

b. Oculomotor apraxia: Difficulty in fixing smoothly on an object. There by
overshooting the target with lateral movement of head followed by refixating the
eyes.
c. Telangiectasia evident by middle childhood – found on bulbar conjunctiva,
bridge of nose, ear, exposed surface of extremities.
d. Skin changes – loss of elasticity.
e. Immunological abnormalities.:
1) Serum secretory IgA decrease.
2) Decrease IgG2 and IgG4
3) IgE decrease.
4) Leading to frequent sinopulmonary infection.
f. More prone to develop:
i. Lymphoreticular tumors:
• Lymphoma, Leukemia.
• Hodgkin’s disease.
ii. Brain tumor.
g. Increase α feto proteins.
h. Death from infection, tumor dissemination.
7.12.4. Friedreich’s Ataxia.
1. Inherited as autosomal recessive trait.
2. Genetic defect –
o FXN gene in chromosome 9q13,
o Mutant gene contains expanded GAA triplet repeats.
o Gene encodes 210 amino acid – FRATAXIN.
o Degeneration of post. Column of spinal cord.
3. Age – Before 10yrs of age.
4. Ataxia – Progressive slowly and involve lower extremities lower than upper.
5. Romberg test positive.
6. Deep tendon reflex – absent.
7. Plantar – Extensor.
8. Speech – Dysarthria.
9. Nystagmus present.
10. Apathetic but Intelligence preserved.
11. Weakness of hands and feet.
12. Loss of vibration and position sense.
13. Skeletal abnormalities
14. Electrophysiology – visual, auditory, brainstem, somatosensory, evoked potentials are
abnormal.
15. CVS – Hypertrophic cardiomyopathy.
7.12.5. Tics.
1. Definition: TICS are spasmodic, involuntary repetitive, stereotyped movements that
are non-rhythmic often exacerbated by stress and may affect any muscle group.
2. Types: Three types
A. Transient TICS of childhood:
• Eye blinking, facial movement, throat clearing.
• Persist for one year.
• No treatment required.

B. Chronic motor TICS disorder:
• Occurs in children.
• Persists throughout life.
• Involves three muscle group.
• Genetic in origin.
C. Gilles de La Tourette syndrome:
o Lifelong condition.
o Age of onset 2-21 years.
o Etiology – Autosomal dominant.
o Gene – 18q22.1
o Clinically multiple motor TICS in different parts of body with at least one vocal
TIC.
o Associated with.
→ Obsessive compulsive behavior.
→ ADHD.
o Examples- movement of face, neck, shoulder, eyelid.
Ultimately, the TICS are accompanied by vocalization (vocal TICS) including throat
clearing, sniffing, barking, coprolalia, echolalia, palilalia, echo kinesis.
3. Treatment:
→ Haloperidol – 0.25 mg/24 hours.
→ Penfluridol, pimozide, clonidine.
7.12.6. Chorea.
1. Definition: Irregular, rapid, semi purposeful, uncontrolled involuntary random
movements
2. Etiology: Functional overactivity of dopaminergic system.
3. Causes:
a) Acquired – Sydenham chorea (Rheumatic fever).
o Diagnostic triad:
 Chorea.
 Hypotonia.
 Emotional liability.
b) SLE
c) Huntington disease – inherited degenerative disorder of CNS.
d) Atypical seizures.
e) Drug intoxication:
→ Phenytoin.
→ Fluphenazine.
→ Hormonal induced.
f) Lyme disease.
g) Hypoparathyroidism
h) Hyperthyroidism.
i) Wilson’s disease.

7.12.7. Athetosis.
1. Definition: Writhing, irregular movement of extremities, predominantly distal
portion often associated with chorea.
2. Causes:
a) C.P. caused by:
• Kernicterus.
• Asphyxia.
• Genetic.
• Metabolic disorders.
• Prematurity.
b) After circulatory arrest.
c) Drug – Phenothiazine.
7.12.8. Tremor.
1. Definition: Involuntary movement characterized by rhythmic oscillations of a part of
body, which may be more prominent during rest or with movement.
Jitteriness: Rhythmic tremors of equal amplitude and around a fix axis.
2. Causes of tremor:
a) Physiological tremor.
b) Essential tremor.
c) Primary writing tremor.
d) Drugs.
→ Amphetamine.
→ Valproic acid.
→ Neuroleptics.
→ Tricyclic antidepressants.
e) Metabolic disorders.
o Hypoglycemia.
o Thyrotoxicosis.
o Neuroblastoma.
o Pheochromocytoma.
f) Following recovery from head injury
g) Wilson’s disease.
h) Parkinsonism.
i) Cerebellar tumor.
j) Midbrain tumor.
7.12.9. Myoclonus.
1. Definition: Sudden involuntary shock like jerks caused by a muscle activity, lasting
50 – 300m/sec. – Disrupt voluntary movement.
2. Pathophysiology:
a) Cortical – Myoclonus.
b) Reticular – Reflex myoclonus.
c) Subcortical – Myoclonus.
d) Peripheral – Myoclonus.
3. Etiology:
a) Essential myoclonus.
b) Primary epilepsy syndrome.
c) Acquired metabolic syndrome.
o Uremia.

o Hyponatremia.
o Hepatic encephalopathy.
d) Progressive cerebral disorders.
o Alzheimer’s disease.
o SSPE
o Huntington disease.
e) Post hypoxic myoclonus.
f) Focal lesions of C. cortex, tumor.
g) Encephalitis.
h) Trauma.
4. Treatment:
a) Treatment of cause.
b) Drugs to control myotonic movements:
→ Clonazepam.
→ Valproate.
→ Primidone.
→ Piracetam.
7.12.10. Dystonia.
1. Definition: Syndrome of sustained simultaneous contractions of agonists and
antagonists’ group of muscle resulting in transient twisting movements or repetitive
movement or abnormal posture.
2. Causes:
a) Perinatal asphyxia.
b) Kernicterus.
c) Generalized primary dystonia.
d) Drugs.
e) Wilson Disease.
f) Hallervorden spatz disease.
g) Genetic mutation.
h) C.P. – Extrapyramidal type.
7.12.11. Ballismus.
→ Definition: Rapid movement of shoulder or hip, which are violent and irregular, and
are absent during sleep.
7.12.12. Tardive Dyskinesia.
→ Definition: Characterized by stereotypical, facial movement, particularly by lip
smacking and protrusion and retraction of tongue.
→ Causes: Drugs like – Phenothiazine, Haloperidol.

7.13. Pseudotumor Cerebri
7.13.1. Definition: Clinical syndrome characterized by:
a) Increased intracranial pressure.
b) Normal CSF.
c) Normal ventricular size, Anatomy and position.
7.13.2. Etiology:
i. Drugs:
a) Hypervitaminosis A.
b) Vitamin A deficiency.
c) Prolonged corticosteroid.
d) Rapid corticosteroid withdrawal.
e) Nalidixic acid.
f) Growth hormone treatment.
g) Tetracycline.
h) Nitrofurantoin
i) Isotretinoin.
ii. Infections:
a) Mastoiditis.
b) Chronic otitis media.
c) Roseola Infantum.
d) Guillain Barre syndrome.
iii. Hematological disorders:
a) Polycythemia.
b) Iron deficiency anemia.
c) Hemolytic anemia.
iv. Metabolic disorders:
a) Galactosemia.
b) Hypoparathyroidism.
c) Pseudohypoparathyroidism.
d) Hypophosphatasia.
v. Obstruction of intracranial drainage system by venous thrombosis due to:
a) Head injury.
b) Superior vena cava operation.
vi. Recovery phase PEM
vii. Wiskott Aldrich syndrome.
7.13.3. Clinical features:
a) Headache, Vomiting.
b) Diplopia (6th
nerve palsy)
c) Alert and lack constitutional symptoms.
d) Bulging fontanel.
e) Cracked pot sound.
f) Papilledema with enlarged blind spot.
g) Ultrasonography – optic nerve edema.
7.13.4. Treatment:
a) Find out the cause and treat it.
b) Repeated lumbar puncture.
c) Drugs:
→ Acetazolamide 10 – 30 mg/kg/day.
→ Corticosteroids.
→ Rarely:

o Lumboperitonial shunt.
o Sub temporal Decompression.
d) Refractory cases – need reevaluation of diagnoses.

7.14. Bell’s palsy.
7.14.1. Definition: It is an acute unilateral fascial palsy that is not associated with other cranial
neuropathies or brain stem dysfunctions.
7.14.2. Etiology:
1. It is post infections allergic or immune demyelinating facial neuritis.
2. Usually develops abruptly 2wks after systemic viral infections.
3. Preceding infection may be Epstein Barr virus, Lyme disease, Herpes simplex virus, Mumps
virus.
7.14.3. Clinical features:
1) Age – Any age.
2) There is lower neuron motor paralysis of facial nerve on one side manifesting as:
a) Patient is unable to close the eye on one side.
b) Corner of mouth droops.
c) Loss of taste sensation on anterior 2/3 of tongue.
d) Complete half of face is paralyzed.
7.14.4. Treatment:
1) Ocular lubricant to prevent exposure keratitis.
2) Supportive treatment.
3) Corticosteroid – Helpful if given earlier in course.
4) Surgical decompression of facial canal not recommended.
5) Acyclovir – useful if given early.
7.14.5. Prognosis:
1) It is excellent.
2) Recovery spontaneously in >85% cases.
3) 10% have mild facial weakness as sequel.
4) 5% can have permanent severe facial weakness.

7.15. Neurocysticercosis.
7.15.1. Etiology: Intermediate stage of taenia solium (Pork tapeworm)
7.15.2. Transmission:
1. Consumption of infected undercooked pork.
2. Ingestion of food or water contaminated with eggs of T. Solium.
7.15.3. Clinical features: Basis of anatomical location, clinical presentation and radiologic appearance.
1. Seizures (Present in >70% cases)
2. Any cognitive or neurologic abnormality from psychosis to stroke.
7.15.4. Classification:
1. Parenchymal: Seizures, Intellectual deterioration, focal signs.
2. Intraventricular: Sign of increase ICP, Hydrocephalus.
3. Meningeal: Sign of meningeal irritation and increase ICP.
4. Racemose: Sign of meningeal irritation and increase ICP.
5. Spinal: Nerve root pain, sign of spinal cord compression, transverse myelitis.
6. Ocular: Decreased visual acuity.
7.15.5. Diagnosis:
1. Imaging studies: CT, MRI (Better)
2. Serological studies: Enzyme linked immunotransfer Biot (EITB), CSF for eosinophilia
(not reliable.)
o Tuberculoma.
o Toxoplasmosis.
o Vasculitis.
o Tumor.
o Sarcoidosis.
o Encephalitis.
o Stroke & Meningitis.
7.15.7. Treatment:
1. Inactive lesions on CT: Not require therapy other than Anticonvalscents.
2. Active parenchymal lesion:
a) Corticosteroids: 2-3 days before and during Anticysticercal therapy.
b) Anticysticercal therapy (Two drugs)
o Albendazole: (Better outcome) 15mg/kg/day in two divided doses for 28
days.
o Praziquantel: 50-100 mg/kg/day in three divided doses for 30 days.
3. Surgery: (Limited value)
a) Placements of shunts.
b) Removal of large solitary cysts for decompression.
c) Removal of mobile cyst.
d) Some cases fail to respond to medical therapy.
Difference between Tuberculoma & Neurocysticercosis on CT head.
S. No. Tuberculosis Neurocysticercosis
1 Size >20mm, irregular outline with marked edema Smaller, Less cerebral edema.
2 More likely to cause midline shift Unlikely to cause midline shift
3 Progressive focal deficit
4 CT target sign more suggestive
5 MRI features as described
6 Morphological type – enplague Viable cyst/ring/disk/nodule calcification.
7 MR Spectroscopy (Lipid peak) No Lipid peaks.

References:
1. Parthasarathy, K Nedunchalian, Gowri Shankar HC, Textbook of Balram chowdhary’s Pediatrics Lecture
notes, PEE PEE Publication, 2nd edition, Pg no. 163 – 195.

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