9 - Pediatric Neurology 2025

Epilepsy

A 5 y/o M presented with two or more unprovoked seizures occurring in a time frame of >24 hrs. Impression?

Seizure type

What is the primary determinant of the type of

medications the patient with seizure disorder is likely to respond to?

Epilepsy syndrome

What determines the type of prognosis one could expect in a patient with seizure disorder?

Focal seizures

This category of seizure is characterized by an initial activation of a system of neurons limited to part of cerebral hemisphere. Previously known as "partial" seizure.

• Focal seizures without impairment of consciousness

• Focal seizures with impairment of consciousness

Focal seizures are subdivided into which categories?

Genetic epilepsy

A condition where direct result of a known or presumed genetic defect(s) in

which the genetic defect is not causative of a brain structural or metabolic disorder other than the epilepsy

Benign childhood epilepsy with centrotemporal spike

Most common type of benign epilepsy syndrome with focal seizure?

Benign childhood epilepsy with centrotemporal spike

A child coming in with focal seizure with buccal and throat tingling and tonic or

clonic contractions of 1 side of the face, with drooling and

inability to speak but with preserved consciousness and

comprehension

Panayiotopoulos type

A child coming in with complex partial seizures with ictal vomiting. Noted with occipital spikes on EEG. Impression?

Gastaut type

A child coming in with complex partial seizure, visual auras, migraine headache. On EEG, noted with occipital spikes. Impression?

Early myoclonic infantile encephalopathy

This epilepsy type starts during the 1st 2 months of life with severe myoclonic seizures and burst suppression pattern on EEG.

Inborn error of metabolism

Usual cause of Early myoclonic infantile encephalopathy?

Early infantile epileptic encephalopathy

(Ohtahara syndrome)

This epilepsy type starts during the 1st 2 months of life with tonic seizures and burst suppression pattern on EEG

Brain malformations or Syntaxin binding protein 1 mutations

Usual causes of Ohtahara syndrome?

Severe myoclonic epilepsy of infancy

(Dravet syndrome)

This epilepsy type starts as focal febrile status epilepticus or focal febrile seizures and later manifests myoclonic and other seizure types

West syndrome

This epilepsy type is characterized by a triad of:

• Infantile epileptic spasms

• Developmental regression

• Hypsarrhythmia on EEG

Lennox-Gastaut Syndrome

This epilepsy type is characterized by a triad of:

• Developmental delay

• Multiple seizure type: absence, myoclonic, astatic, and tonic

• EEG = 1-2Hz spike and slow waves, poly-spike bursts in sleep and slow background in wakefulness

Absence seizure

Formerly ‘petit mal’

This seizure type involves brief staring spells that usually last for less than 15 seconds; usually resolves 2-5 years after its onset, usually at puberty

Partial seizures

Antiepileptic drugs acting on sodium channels are effective against?

Absence seizures

Antiepileptic drugs acting on T-type calcium channels are effective against?

Oxcarbazepine

Carbamazepine

First line AED for Focal seizures and epilepsies?

Ethosuximide

First line AED for absence seizures?

Valproate (males) and

Lamotrigine (females)

First line AED for Juvenile

Myoclonic Epilepsy (Janz syndrome)?

Clobazam, Valproate,

Topiramate,

Lamotrigine, ± Rufinamide

Lennox-Gastaut syndrome is treated with this 1st line AED

Adrenocorticotropic hormone (ACTH) & Vigabatrin

Infantile spasms are treated with this 1st line drug

Valproate + benzodiazepine

(Clobazam or Clonazepam);

Topiramate

First-line AEDs for Dravet syndrome?

Valproate

First-line AED for benign myoclonic epilepsy

Simple febrile seizure

Febrile seizure that is generalized and occurs for less than 15 minutes. There is no recurrence in the 1st 24 hours and there is no focal sign in the post-ictal period.

Complex febrile seizure

Febrile seizure that is focal and lasts for more than 15 minutes. It may recur in the 1st 24 hours with focal signs in the postictal period.

< 6 mos of age or if the child is ill-appearing or at ANY AGE if there are clinical signs or symptoms of concern

Based on Nelson's, when should a Lumbar puncture done in patients with febrile seizure?

If the patient is deficient in

Haemophilus influenzae type b and Streptococcus pneumoniae immunizations or for whom immunization status is unknown

When is lumbar tap an option for a patient 6-12 mos old presenting with febrile seizure?

• Age <1 year

• Duration of fever <24 hours

• Fever 38-39 C

What are the 3 major risk factors for the recurrence of febrile seizures?

• Family history of febrile seizures

• Family history of epilepsy

• Complex febrile seizure

• Daycare

• Male gender

• Lower serum sodium at time of presentation

What are known minor risk factors for the recurrence of febrile seizures?

Neurodevelopmental abnormalities

Which factor has the greatest risk for occurrence of subsequent epilepsy in a child?

Simple febrile seizure

What is the most common seizure disorder during childhood?

Status epilepticus

This clinical condition is defined as continuous seizure activity or recurrent seizure activity without regaining of consciousness lasting for more than 5 min

Refractory status epilepticus

Status epilepticus that has

failed to respond to therapy, usually with at least 2 medications (such as a BZD and another medication)

Securing airway, breathing, and circulation

Priority in the management of status epilepticus

Diazepam or lorazepam, followed by Phenytoin /

Fosphenytoin and phenobarbital, then valproate and Levetiracetam

Progression of treatment option in status epilepticus according to Nelson's

HHV-6 and HHV-7

One third of the cases of febrile status epilepticus are due to what viral infection?

• Communicating Hydrocephalus

• Basal Enhancements

• Infarcts/Arteritis

Triad of cranial imaging findings in TB meningitis?

Choroid plexus epithelium

CSF production occurs in?

Arachnoid villus cells in the superior sagittal sinus

CSF reabsorption occurs in?

• Signs of increased ICP

• Local infection at desired puncture site

• Radiological signs of obstructive hydrocephalus, cerebral edema, or hernation

What are absolute contraindications to Lumbar puncture?

• Signs of shock, sepsis or hypotension

• Coagulation defects

• Focal neurological deficit

• GCS < 8

• Epileptic seizures

What are relative contraindications to lumbar puncture?

Leukocytosis (predominantly PMNs)

Elevated CHON

Decreased glucose (usually < 40 or <50% serum glucose)

Expected finding in the CSF of patients with acute bacterial meningitis?

• Leukocytosis (predominantly mononuclear cells)

• Slightly elevated CHON

• Normal to decreased glucose

Expected finding in the CSF of patients with acute viral meningitis?

• Leukocytosis (predominantly Lymphocytes)

• Elevated CHON

• Decreased glucose

Expected finding in the CSF of patients with TB meningitis?

Procalcitonin

In cases where CSF analysis can't be done, which laboratory test may help differentiate viral from bacterial meningitis?

CNSI from N. fowleri

Child with a history of swimming in warm water came in for prefrontal headache, high fever, and anosmia. Impression?

Borrelia burgdorferi

Child with a history of systemic disease and cutaneous lesions came in for Bell's palsy. Suspected etiology?

C. jejuni

Child with a history of GIT infection came in for ascending paralysis. Suspected etiology?

HiB

Child with a history of respiratory infection came in for ascending paralysis. Suspected etiology?

Reye syndrome

Child with a history of viral infection and aspirin use came in for lethargy and irritability. Impression?

Cerebrum

80% of brain abscess occurs in what area of the brain?

CHD (embolization), CSOM, face and scalp infections, dental infections, orbital cellulitis, penetrating head injuries, VP shunt infections

Usual causes of brain abscess

3rd generation cephalosporin + metronidazole

Antibiotic of choice for brain abscess from an unknown cause?

Oxacillin/Vancomycin

+

3rd gen Cephalosporin

+

Metronidazole

Antibiotic of choice for brain abscess from head trauma or neurosurgery?

Penicillin + Metronidazole

Antibiotic of choice for brain abscess from suspected CHD?

Vancomycin + Ceftazidime

Antibiotic of choice for brain abscess from infected VP shunt

Broad spectrum + Amphotericin B

Antibiotic of choice for brain abscess in an immunocompromised host?

• (+) gas in the abscess

• Multiloculated abscesses

• Posterior fossa location

• Fungal cause

• Associated infections like mastoiditis, periorbital abscess, sinusitis

What are indications for surgery for brain abscess?

4-6 weeks

Antibiotic therapy for brain abscess requires a duration of?

Aerosol droplets

Contact with respi secretions

Mode of transmission of meningococcemia?

Until 24 hours after initiating

effective treatment

What is the period of communicability of meningococcemia?

Waterhouse-Friderichsen syndrome

Characterized by diffuse adrenal hemorrhage without vasculitis, DIC, and coma with high mortality rate

Penicillin G 250k-300k U/kg/day IV in 4-6 divided doses x 5-7 days (at least)

Alternatives:

Ceftriaxone IM or IV (100 mkDay) OD or BID

or

Cefotaxime IM or IV (200-300 mkDay) q6 or 8 hrs

Drug of choice for Meningococcemia?

Cefotaxime

Drug of choice for neonates with N. meningitidis invasive infection?

Household, school, or daycare contacts during the 7 days befoe onset of illness

Medical personnel with intimate exposure (intubation, suctioning)

Exposure to meningococcemia that requires antibiotic prophylaxis is defined as?

• Children: Rifampicin 10 mkDose PO q12 hrs for a total of 4 doses (max 600 mg)

• < 1 month old: Rifampicin 5 mkDose q12 hrs x 2 days

OR

• Ceftriaxone 125 mg SD IM for < 12 yrs old

• > 18 yrs old: Ciprofloxacin 500 mg PO SD

What is the DOC for prophylaxis for meningococcemia?

Gram Negative Bacteria / GBS

Infant < 2 mos old with sepsis, seizure, irritability, lethargy, bulging of fontanelles, rigidity. Organism involved?

HSV

Child presenting with headache, fever, confusion, lethargy, nuchal rigidity, and vomiting. CSF shows RBCs. Organism involved?

3rd and 4th weeks in utero

Neural tube defects arise from failure of neural tube to close spontaneously during what week of embryological development?

Myelomeningocoele

Most severe form of neural tube defect (dysraphism)?

Periconceptual Folic acid (before conception until at

least the 12th wk of gestation)

– 0.4 mg OD

Most effective treatment to prevent myelomeningocele?

Lumbosacral

75% of myelomeningocoeles occur at what segment of the spine?

Chiari Type I

Chiari malformation characterized by elongation of cerebellar tonsils extending in

vertebral canal

Chiari Type II

Chiari malformation with elongation of inferior vermis and brainstem with their displacement in cervical spinal canal with myelomeningocele and hydrocephalus.

Chiari Type III

Bony defect at occipito-cervical level with herniation of cerebellum into encephalocele.

• 1. Abnormal neurologic signs

• 2. Behavioral changes, recent school failure, fall-off in linear growth rate

• 3. Headache awakens the child during sleep

• 4. Migraine & seizure occur in the same episode

• 5. Focal neurologic signs

• 6. Cluster headaches esp. in <5 yrs old

SIX indications to do cranial imaging in headache?

• More than 2-4 severe episodes monthly

• Unable to attend school regularly

• PedMIDAS > 20

When to give prophylactic therapy for migraine?

Analgesics or antiemetics

DOC for migraine

Prochlorperazine IV 0.15 mg/kg max. 10 mg

DOC for status migrainosus

Tuerous sclerosis

Child with history of infantile spasms presents with generalized seizure disorder & skin lesions (shagreen patch, ash leaf). Impression?

TSC1 and TSC2

Genes mutated in Tuberous sclerosis?

(+) Tubers in the

convolutions of the cerebral hemispheres. (+)

calcifications projecting

into the ventricular cavity (candle-dripping appearance)

Characteristic cranial CT scan findings in TS?

Chromosome 17

Neurofibromatosis gene is located in what chromosome?

• Six or more café au lait macules >5mm in diameter in prepubertals and >15mm in postpubertal individuals

• Axillary or inguinal freckling 2-3 mm in diameter

• Two or more iris Lisch nodules

• Two or more neurofibromas (along the skin, PNS, blood vessels & within viscera) or one plexiform neurofibroma

• Distinctive osseous lesion

• Optic glioma

Neurofibromatosis 1 is diagnosed when any 2 are present of which clinical features?

• 1. Bilateral 8th nerve masses (acoustic neuroma)

• 2. Parent, sibling, or child with NF-2 and either unilateral 8th nerve masses or any 2 of the ff: neurofibroma, meningioma, glioma, schwannoma

Neurofibromatosis type 2 is diagnosed when 1 of which features are present?

Guillain-Barre syndrome

Weakness begins in the lower extremities & progressively involves the trunk, upper limbs & bulbar muscles (Landry ascending paralysis) following a nonspecific viral infection (GIT or RT) by 10 days (Campylobacter jejuni and herpesvirus)

Miller-Fisher syndrome

GBS with acute ophthalmoplegia, ataxia, areflexia

• Cranial nerve involvement

• Need for intubation

• Maximum disability at the time of presentation

Features predictive of poor outcome in GBS

Tendon reflexes

Last function to recover in GBS?

IVIG (0.4 grams / kg / day for 5 consecutive days)

Management of rapidly progressive ascending paralysis in GBS?

Medulloblastoma

Most common malignant brain tumor in children

Homer-Wright rosettes

Histologic pattern of medulloblastoma on biopsy

Cerebellar astrocytoma

Most common infratentorial tumor and with the best prognosis?

Craniopharyngoma

Solid & cystic supratentorial tumor that tend to calcify. Patients may present with

short stature and pressure to optic chiasm produces bitemporal visual field cuts.

Optic nerve glioma

Supratentorial tumor presenting with decreased visual acuity & pallor of the

Discs. 25% of the cases have neurofibromatosis.

Stroke

For an acute onset of a focal neurologic deficit in a child. First differential to rule out is?