Paediatrics Marrow Pg 181-190 (CNS)
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What is the first-line drug for managing Dravet Syndrome?

  • Carbamazepine
  • Lamotrigine
  • Stiripentol
  • Valproate (correct)

    • Lennox Gastaut Syndrome typically has an onset after the age of 10 years.

    False

    What genetic defect is associated with Dravet Syndrome?

    SCN1A gene

    The typical EEG pattern for Lennox Gastaut Syndrome is ____.

    <p>1.5 to 2.5 Hz spike &amp; wave pattern</p> Signup and view all the answers

    Match the following conditions with their respective features:

    <p>Dravet Syndrome = Multiple seizure types, genetic defect in SCN1A Lennox Gastaut Syndrome = Developmental delay, refractory seizures Status Epilepticus = Seizure duration &gt; 5 min requires treatment</p> Signup and view all the answers

    What is a major indication for intermittent prophylaxis in children?

    <p>History of more than 4 febrile seizures in 1 year</p> Signup and view all the answers

    Absence seizures can last longer than 30 seconds.

    <p>False</p> Signup and view all the answers

    Which medication is commonly used for the treatment of absence seizures in India?

    <p>Valproate</p> Signup and view all the answers

    Juvenile Myoclonic Epilepsy (JME) is characterized by sudden, jerky movements due to sudden muscle contraction called ______.

    <p>myoclonus</p> Signup and view all the answers

    Match the following types of seizures with their characteristics:

    <p>Typical absence seizures = 3 Hz spike &amp; wave pattern Atypical absence seizures = 10 Hz spike &amp; wave pattern Juvenile Myoclonic Epilepsy = Sudden jerky muscle contractions Febrile seizures = Frequent recurrent seizures due to fever</p> Signup and view all the answers

    What is the first line of treatment for seizures if blood glucose is normal?

    <p>Administer intravenous benzodiazepines (midazolam or lorazepam)</p> Signup and view all the answers

    A hypoglycemic child presenting with seizures should receive a 10% dextrose solution immediately.

    <p>True</p> Signup and view all the answers

    Name one medication that can be used in the second line of treatment for seizures.

    <p>Fosphenytoin</p> Signup and view all the answers

    The first step in the seizure treatment algorithm is the initial ______ which includes stabilizing airway, breathing, and circulation.

    <p>assessment</p> Signup and view all the answers

    Match the medications with their classification or purpose:

    <p>Thiopental = Anaesthetic for refractory seizures Midazolam = Benzodiazepine for seizure management Phenobarbital = Anaesthetic for refractory seizures Propofol = Anaesthetic for refractory seizures</p> Signup and view all the answers

    What is the most common type of Cerebral Palsy (CP)?

    <p>Spastic CP</p> Signup and view all the answers

    Cerebral Palsy is a progressive neurological condition.

    <p>False</p> Signup and view all the answers

    Name one prenatal factor that can lead to Cerebral Palsy.

    <p>Brain malformations</p> Signup and view all the answers

    Cerebral Palsy is classified based on motor manifestations and is most commonly caused by __________.

    <p>spasticity</p> Signup and view all the answers

    Match the types of Cerebral Palsy with their descriptions:

    <p>Diplegia = Both lower limbs affected Hemiplegia = One side of the body affected Quadriplegia = All four limbs affected Spastic CP = Characterized by increased tone/hyperreflexia</p> Signup and view all the answers

    Which of the following symptoms is part of the prodromal phase of meningitis?

    <p>Fever</p> Signup and view all the answers

    Coma is a condition associated with decreased mortality in untreated meningitis.

    <p>False</p> Signup and view all the answers

    What is the duration of the intensive phase of anti-tubercular treatment for meningitis?

    <p>2 months</p> Signup and view all the answers

    The feature called __________ identifies TB bacilli in CSF.

    <p>Ziehl Neelsen staining</p> Signup and view all the answers

    Match the symptoms to their respective conditions:

    <p>Fever = Meningitis Seizures = Viral Meningoenchephalitis Photophobia = Meningitis Speech defects = Viral Meningoenchephalitis</p> Signup and view all the answers

    Which of the following describes Dyskinetic CP?

    <p>Characterized by motor control difficulties and abnormal limb movements.</p> Signup and view all the answers

    Ataxic CP results from damage to the cerebellum.

    <p>True</p> Signup and view all the answers

    List two symptoms associated with spasticity in Cerebral Palsy.

    <p>Scissoring posture and toe walking</p> Signup and view all the answers

    The persistent neonatal reflex, the __________ reflex, is an example of Global Developmental Delay.

    <p>Moro's</p> Signup and view all the answers

    Match the types of cerebral palsy with their descriptions:

    <p>Dyskinetic CP = Abnormal movements of limbs Ataxic CP = Cerebellar dysfunction symptoms Mixed CP = Combination of all three types Spastic CP = Increased muscle tone and reflexes</p> Signup and view all the answers

    Which of the following statements about Lennox Gastaut Syndrome is true?

    <p>It typically has an onset in early childhood.</p> Signup and view all the answers

    The typical age of onset for Dravet Syndrome is before the age of 1 year.

    <p>True</p> Signup and view all the answers

    What type of seizures are characterized by sudden, jerky movements due to muscle contractions?

    <p>Myoclonic seizures</p> Signup and view all the answers

    Juvenile Myoclonic Epilepsy (JME) is characterized by sudden muscle contractions called ______.

    <p>myoclonic jerks</p> Signup and view all the answers

    Match the following seizure types with their characteristics:

    <p>Absence Seizures = Brief lapses in consciousness Tonic Seizures = Stiffening of body muscles Atonic Seizures = Sudden loss of muscle tone Clonic Seizures = Rhythmic jerking movements</p> Signup and view all the answers

    What is the primary goal of administering steroids alongside the first dose of IV antibiotics in treating CNS infections?

    <p>Reduce inflammation and incidence of sensorineural hearing loss</p> Signup and view all the answers

    Long-term complications of CNS infections can include seizures.

    <p>False</p> Signup and view all the answers

    What is the typical duration of antibiotic treatment for children suffering from CNS infections?

    <p>7–10 days</p> Signup and view all the answers

    In TB meningitis, a __________ focus develops leading to severe symptoms.

    <p>CNS</p> Signup and view all the answers

    Match the following diagnostic methods with their application in diagnosing CNS infections:

    <p>Gram's staining = Identifying bacterial presence Culture &amp; sensitivity (C&amp;S) = Determining effective antibiotics PCR = Detecting causative bacteria CT or MRI = Assessing structural brain changes</p> Signup and view all the answers

    Which organism is the most common cause of bacterial meningitis in children older than 2 years?

    <p>Streptococcus pneumoniae</p> Signup and view all the answers

    Projectile vomiting is a sign of increased intracranial pressure in older children.

    <p>True</p> Signup and view all the answers

    What sign is characterized by flexion of the hips and knees in response to neck flexion?

    <p>Brudzinski's sign</p> Signup and view all the answers

    The appearance of CSF in bacterial meningitis is typically __________.

    <p>turbid/cloudy</p> Signup and view all the answers

    Match the following clinical features with their corresponding conditions in bacterial meningitis:

    <p>Neck stiffness = Physical exam finding for meningeal irritation Kernig's sign = Resistance to leg extension in hip flexion Brudzinski's sign = Flexion response in hips and knees Bulged anterior fontanelle = Sign of increased intracranial pressure in infants</p> Signup and view all the answers

    What is the primary EEG finding associated with myoclonic episodes?

    <p>4 – 6 Hz polyspike &amp; wave pattern</p> Signup and view all the answers

    Symptomatic West Syndrome is not associated with any CNS abnormalities.

    <p>False</p> Signup and view all the answers

    What is the first-line treatment of choice for West Syndrome?

    <p>Inj. ACTH</p> Signup and view all the answers

    The main precipitating factor for seizures in patients is ______.

    <p>sleep deprivation</p> Signup and view all the answers

    Match the seizure type with its associated feature:

    <p>Infantile spasms = Associated with West Syndrome Jack-knife seizures = Abrupt contraction of limbs Salaam spells = Repetitive neck flexion Myoclonic episodes = Consciousness unaffected</p> Signup and view all the answers

    Study Notes

    Dravet Syndrome

    • Onset: Less than 1 year old
    • Genetic defect: SCN1A gene (I alpha subunit of sodium channel)
    • Clinical features: Multiple seizure types, difficult to manage
    • Management:
      • First-line drug: Valproate (unsatisfactory treatment response)
      • Newer drug: Stiripentol - increases GABA activity, reduces seizures

    Lennox-Gastaut Syndrome

    • Onset: 2 to 10 years old
    • Clinical features:
      • Developmental delay
      • Multiple refractory seizures
    • Triad: Developmental delay, multiple refractory seizures, typical EEG pattern (1.5 to 2.5 Hz spike and wave)
    • Treatment: Valproate (DOC) – Drug of Choice

    Status Epilepticus

    • Emergency characterized by a long duration seizure
    • Timelines:
      • T1: Time when treatment should be initiated (greater than 5 minutes)
      • T2: Time after which seizure activity may cause long-term consequences (neuronal injury) (greater than 30 minutes)

    Intermittent Prophylaxis

    • Given during the first few days of fever at home
    • Indications: Major risk factors for recurrent febrile seizures
    • Criteria: More than 3 episodes in 6 months OR more than 4 episodes in 1 year
    • Medication: Oral clobazam (in India) / Oral diazepam

    Absence Seizures (Petit Mal Seizures)

    • Minimal manifestations
    • Age affected: School-going children (greater than 5 years)
    • Clinical features:
      • Duration: Up to 30 seconds
      • Precipitating factors: Hyperventilation, stress
      • Symptoms: Vacant stares, eye flutters
    • Types:
      • Typical: Minimal manifestations, EEG finding: 3 Hz spike and wave pattern
      • Atypical: Can have generalized tonic-clonic seizures (GTCS), myoclonic seizures, EEG finding: 10 Hz spike and wave pattern

    Absence Seizure Treatment

    • India: Valproate
    • Other drug: Lamotrigine
    • Ethosuximide: Limited availability and use in India

    Juvenile Myoclonic Epilepsy (JME) (JANZ syndrome)

    • Myoclonus: Sudden, jerky movements due to sudden muscle contraction
    • Age affected: Adolescents
    • Inheritance: Polygenic Inheritance
    • Associated with mutations in:
      • CLCN2
      • GABRAI
      • CACNB4

    Treatment Algorithm for Seizures

    • Step 1: Initial Assessment
      • Stabilize airway, breathing, and circulation
      • Neurological evaluation
      • Attempt IV access
      • Blood glucose management
    • Step 2: Seizure management
      • If blood glucose is normal:
        • First-line: Administer intravenous benzodiazepines (midazolam or lorazepam)
        • No response: Proceed to second-line treatment
      • If hypoglycemic (blood glucose less than 54 mg/dL):
        • Administer 5 mL/kg of 10% dextrose solution
        • If seizure resolves: Continue monitoring
        • If seizure persists: Proceed to second-line
      • Second-line: Administer one of the following anti-epileptic drugs:
        • Fosphenytoin
        • Valproate
        • Levetiracetam
      • No response: Identify and treat refractory status epilepticus
    • Step 3: Refractory Status Epilepticus:
      • Anaesthetic dose of one of the following:
        • Thiopental
        • Midazolam
        • Phenobarbital
        • Propofol
      • Continuous IV infusions
      • EEG monitoring

    Cerebral Palsy (CP)

    • AKA: Static Encephalopathy - Non-progressive damage to developing brain
    • Most common chronic motor disability in childhood
    • Motor disabilities: Abnormality in posture and movement
    • Intellectual disability: Possible
    • Manifestations: Evolve over time
    • Management: Multidisciplinary

    Cerebral Palsy Etiology

    • Prenatal period (most common):
      • Brain malformations
      • Maternal factors (toxins)
      • TORCH infection
    • Trauma during:
      • Perinatal period: Birth asphyxia, Trauma to head
      • Postnatal period: Infections (meningitis), Trauma to the developing brain
    • Intracranial hemorrhages

    Cerebral Palsy Classification

    • Based on motor manifestations:
      • Spastic CP (most common): Characterized by increased tone/hyperreflexia
    • Topographical Classification:
      • Diplegia: Both lower limbs affected
      • Hemiplegia: One side of the body affected
      • Quadriplegia: All four limbs affected, most common in India

    Cerebral Palsy - Seen In:

    • Preterm babies
    • Neonates
    • Sequelae of Periventricular Leukomalacia
    • Neonatal stroke

    Cerebral Palsy: Additional Information

    • Most common type in India: Spastic CP
    • Birth asphyxia of term babies
    • Parasagittal infarction

    Types of Cerebral Palsy

    • Dyskinetic/Choreo-athetoid CP:
      • Dyskinesia: Abnormal movements of limbs
      • Asphyxia/increased level of jaundice: Damage to Basal ganglia - Choreo-athetoid/extrapyramidal movements
      • Kernicterus
    • Ataxic CP:
      • Malformations in/toxin-damage to cerebellum vermis - Cerebellar dysfunction symptoms
    • Mixed CP:
      • Combination of all three types

    Cerebral Palsy - Clinical Presentation

    • Global Developmental Delay (GDD): Delay in at least two domains of milestones (gross motor, fine motor, language, social)
    • Persistence of neonatal reflexes (brain remains immature)
      • Example: Moro's reflex persists after 6 months
    • Spasticity:
      • Scissoring posture seen in spastic diplegia type of CP
      • Due to increased tone of adductors in the lower limb
      • Toe walking: Involuntary
      • Cortical thumb: Due to increased tone of thumb.
    • Vision abnormalities
    • Hearing abnormalities

    Cerebral Palsy - Management

    • MRI Brain (To localize area of damage)
    • Treat symptomatically with a multidisciplinary approach
      • Seizures: Anti-epileptics
      • Spasticity: Muscle relaxants (Baclofen, Botulinum toxin A)
      • Contractures: Tendon release procedures
      • Dystonia/Dyskinesia: Trihexyphenidyl, Levodopa

    TB Meningitis

    • Prodromal phase: 1-4 weeks
      • Fever/Irritability/Vomiting
      • Not conclusive of TB meningitis
    • If undiagnosed/untreated:
      • Meningitis (can mimic bacterial meningitis): Features of meningeal irritation (photophobia, seizures), coma (increased mortality)
    • Diagnosis:
      • CT Brain: Thick exudates in basal cistern (characteristic feature)
      • CSF Findings:
        • Cell: Lymphocyte predominance
        • Protein: Very high (greater than 1000 mg/dL) (Normal: 20-45 mg/dL)
        • ADA (Adenosine deaminase): Increased
        • Ziehl Neelsen staining: Identifies TB bacilli
        • CB-NAAT (Cartridge Based - Nucleic acid Amplification Testing)
        • Cobweb appearance of CSF (when CSF is left undisturbed in a test tube)

    TB Meningitis Treatment

    • Anti-tubercular treatment: 9-12 months
    • Intensive phase (2 months): HRZE regimen (Isoniazid, Rifampicin, Pyrazinamide, Ethambutol)
    • Maintenance phase (7-10 months): HR regimen (Isoniazid, Rifampicin)
    • Steroids: For 6-8 weeks
      • IV Dexamethasone (few days)
      • Oral steroids

    Viral Meningoencephalitis

    • Encephalitis: Cortical lobe involvement
    • Signs of involved lobe (e.g., speech defect, focal seizures)

    Pyogenic/Bacterial Meningitis

    • Etiology:
      • 0-2 months: Escherichia coli
      • 2-24 months: Haemophilus influenzae (most common) > Streptococcus pneumoniae
      • Greater than 2 years: Streptococcus pneumoniae (overall most common cause)

    Bacterial Meningitis Clinical Features

    • Fever
    • Others: Grand mal seizures, irritability
    • Signs of increased intracranial pressure:
      • Infant: Bulged anterior fontanelle
      • Older Child: Projectile vomiting, occipital headache, visual defects (papilledema)
    • Signs of meningeal irritation (uncommon less than 2 years)

    Bacterial Meningitis - Physical Examination Findings

    • Neck stiffness/Nuchal rigidity: Painful flexion at the neck, keeps neck stiff
    • Brudzinski's sign: Flexion of hips and knees in response to neck flexion
    • Kernig's sign: Resistance to extension of the leg while the hip is flexed (Flex hip & Knee, Extend the knee, pain in the spine region – due to stretching of inflamed meninges)

    Bacterial Meningitis - Investigations

    • CSF analysis: Lumbar puncture
    • Contraindication (C/1): Severe signs of increased intracranial tension (ICT) - can lead to herniation
    • Contrast Enhanced CT (CECT): Shows meningeal enhancement

    Bacterial Meningitis - CSF Parameters

    CSF parameter Findings Normal CSF
    Appearance of CSF Turbid/Cloudy (Abundant neutrophils) Clear
    Cell count Increased neutrophils Less than 5
    Protein Increased (greater than 100 mg/dL) 20-45 mg/dL
    Glucose Decreased 45-80 mg/dL
    Lactate Increased 1.0-2.2 mmol/L

    Bacterial Meningitis - Diagnosis Methods

    • Gram's staining
    • Culture & sensitivity (C&S)
    • PCR (more sensitive)

    Bacterial Meningitis - Complications

    • Acute: Seizures, brain abscess, subdural effusion
    • Long term/chronic: Sensorineural hearing loss (SNHL) - particularly with Streptococcus pneumoniae associated meningitis

    Bacterial Meningitis - Treatment

    • Antibiotics: 3rd generation cephalosporins (Ceftriaxone, Cefotaxime) - If no response in 48 hours, add vancomycin
    • Steroids: Along with the first dose of IV antibiotics. Dexamethasone x 2 days
    • Duration of Rx:
      • Child: 7–10 days
      • Neonate: 3 weeks
    • Can change antibiotic post C&S results

    Myoclonic Seizures

    • Myoclonic episodes: Occur upon awakening (e.g., inability to hold toothbrush due to jerks)
    • Consciousness unaffected
    • Associated with other seizure patterns in some patients (e.g., GTCS, Absence seizures)

    Myoclonic Seizures - Precipitating Factors

    • Sleep deprivation
    • Alcohol intake

    Myoclonic Seizures EEG Findings

    • 4 – 6 Hz polyspike and wave pattern

    Myoclonic Seizures - Treatment

    • Drug of Choice (DOC): Valproate
    • Life-long treatment - due to increased risk of adulthood seizures

    West Syndrome

    • Onset: Less than 1 year old
    • Types:
      • Idiopathic (30%): Not associated with other CNS abnormalities
        • Clinical features: Infantile spasms, jackknife seizures, Salaam spells
      • Symptomatic (70%): Associated with worst outcome - CNS defects present
    • Triad (Onset < 1 yr):
      • Developmental regression (loss of previously acquired milestones)
      • High-voltage spikes in chaotic background
    • Treatment: Initiate at the earliest (delay = irreversible brain damage)
    • DOC: Inj.ACTH
    • Second-line drug: Vigabatrin (DOC if associated with tuberous sclerosis)

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    Description

    This quiz explores essential aspects of Dravet Syndrome, Lennox-Gastaut Syndrome, and Status Epilepticus, focusing on their onset, clinical features, and management strategies. Test your knowledge on key treatments and how to address seizures effectively.

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