Pediatrics
Final Professional MBBS — Pediatrics. Complete question bank: Long Questions (10 marks) and Short Notes (5 marks) across all 15 systems, with clinical pearls, drug doses, staging tables, mnemonics and key-point recaps.
DEFINITION
A febrile seizure is a seizure occurring with fever (temperature ≥ 38 °C) in a child aged 6 months to 5 years, in the absence of central nervous system infection, metabolic disturbance or a history of afebrile seizures. It is the commonest seizure disorder of childhood.
ETIOLOGY & RISK FACTORS
- Usually triggered by a viral infection (upper respiratory infection, roseola, otitis media).
- A rapid rise in temperature (rather than the absolute peak) is important.
- Strong genetic predisposition — a positive family history is common.
- Peak age is around 12–18 months.
CLASSIFICATION
Feature Simple (commonest) Complex Type Generalised Focal / with focal features Duration < 15 minutes > 15 minutes Recurrence in 24 h Single Recurrent within 24 h Post-ictal deficit None May have (Todd's paresis) CLINICAL FEATURES
- A brief generalised tonic-clonic seizure during a febrile illness, followed by post-ictal drowsiness.
- The child is neurologically normal before and after (in simple febrile seizures).
- Complex features (focal, prolonged, recurrent) warrant closer evaluation.
INVESTIGATIONS
- Investigations are directed at finding the source of fever, not the seizure itself.
- Lumbar puncture — if meningitis is suspected; consider strongly in infants < 12–18 months (in whom meningeal signs may be subtle) or if the child is not recovering.
- EEG and neuroimaging are NOT routinely indicated for simple febrile seizures; considered for complex/atypical cases.
- Blood glucose, electrolytes and calcium if clinically indicated.
ACUTE MANAGEMENT OF THE SEIZURE
- Protect the airway — place the child in the recovery position, do not restrain or put anything in the mouth.
- Most seizures are brief and self-terminating; if it lasts > 5 minutes, give a benzodiazepine — rectal diazepam or buccal/intranasal midazolam.
- Antipyretics (paracetamol) for comfort; treat the underlying infection.
- Ensure it is not meningitis/encephalitis before attributing it to a simple febrile seizure.
PREVENTION OF RECURRENCE
- Regular prophylactic antiepileptics are NOT recommended (risks outweigh benefits).
- Antipyretics reduce discomfort but do not reliably prevent recurrence.
- For prolonged/recurrent seizures, parents can be given intermittent/rescue benzodiazepine to use at home.
PROGNOSIS
- Excellent — simple febrile seizures do not cause brain damage or affect development/intelligence.
- Recurrence occurs in ~30% (higher if young age, low peak temperature, short fever-to-seizure interval, family history).
- The risk of later epilepsy is only slightly raised (higher after complex febrile seizures).
EPIDEMIOLOGY
- Affects 2–5% of children; the commonest seizure type of childhood.
- Peak incidence 12–18 months; a positive family history is frequent.
FEBRILE STATUS EPILEPTICUS
A febrile seizure lasting > 30 minutes (or recurrent without recovery) is febrile status epilepticus — it is managed as status epilepticus and warrants closer evaluation for an underlying CNS infection.
DIFFERENTIAL DIAGNOSIS
- CNS infection — meningitis/encephalitis (the crucial one to exclude).
- Rigors/shivering with fever; a first afebrile seizure that coincidentally occurs with fever; metabolic disturbance.
WHEN TO ADMIT / EVALUATE FURTHER
- Complex features, a first complex seizure, or a child who is not fully recovering.
- Age < 12–18 months, signs suggesting meningitis, or a toxic/unwell child.
- Diagnostic uncertainty or significant parental anxiety needing observation.
PARENT EDUCATION (a key part of management)
- Explain the benign nature and excellent prognosis; teach first-aid and seizure timing.
- Advise antipyretics for comfort (not as guaranteed prevention) and when to seek help.
RISK FACTORS FOR RECURRENCE
- Young age at first seizure (< 18 months); a lower peak temperature at the time of the seizure.
- A short interval between fever onset and the seizure; a family history of febrile seizures.
- The more risk factors present, the higher the recurrence rate.
RISK OF SUBSEQUENT EPILEPSY
The risk of later epilepsy after a simple febrile seizure is only marginally above the background population risk; it is higher after complex febrile seizures, or with a family history of epilepsy or pre-existing neurodevelopmental abnormality.
VACCINATION & FEBRILE SEIZURES
Some vaccines (e.g. MMR, DTP) can cause fever and, occasionally, an associated febrile seizure; this is not a contraindication to immunisation, and parents should be reassured and advised on antipyretics and first-aid.
📝CLINICAL / APPLIED POINTS- The single most important task is to exclude meningitis/encephalitis before diagnosing a febrile seizure.
- Have a low threshold for lumbar puncture in infants under 12–18 months, in whom meningeal signs are unreliable.
- Simple febrile seizures need no EEG or imaging — reassurance and parent education are the mainstay.
- Teach parents first-aid (recovery position, timing) and when to use rescue benzodiazepine and seek help.
- Emphasise the excellent prognosis to relieve the (often intense) parental anxiety.
💊KEY DRUG DOSES (viva)- If seizure > 5 min: rectal diazepam 0.5 mg/kg, or buccal/intranasal midazolam 0.3 mg/kg.
- Paracetamol 15 mg/kg for comfort (does not prevent recurrence).
🔑KEY POINTS TO REMEMBER- Seizure with fever, age 6 months–5 years, no CNS infection/metabolic cause.
- Simple (generalised, < 15 min, once/24 h) vs complex (focal, prolonged, recurrent).
- Investigations target the fever source; LP if meningitis suspected; no routine EEG/imaging.
- Acute: protect airway; benzodiazepine if > 5 min; antipyretics; treat infection.
- Excellent prognosis; ~30% recur; only slightly increased epilepsy risk; no routine prophylaxis.
📚SOURCES: Ghai Essential Pediatrics; Nelson Textbook of Pediatrics; AAP febrile seizure guidelines.DEFINITION
Epilepsy is a chronic disorder characterised by an enduring predisposition to recurrent, unprovoked seizures. It is diagnosed after ≥ 2 unprovoked seizures > 24 hours apart, or one unprovoked seizure with a high probability of recurrence. A seizure is a transient event due to abnormal, excessive neuronal discharge.
CLASSIFICATION OF SEIZURES
- Focal seizures — arising in one part of the brain; with retained or impaired awareness (may become bilateral tonic-clonic).
- Generalised seizures — tonic-clonic, absence, myoclonic, atonic, tonic.
- Classified further by aetiology — structural, genetic, infectious, metabolic, immune or unknown.
COMMON PAEDIATRIC EPILEPSY SYNDROMES
Syndrome Key features Childhood absence epilepsy Brief blank stares with 3 Hz spike-wave EEG Benign rolandic (BECTS) Nocturnal focal facial seizures; good prognosis Juvenile myoclonic epilepsy Morning myoclonic jerks in adolescents West syndrome Infantile spasms + hypsarrhythmia + regression Lennox-Gastaut Multiple seizure types, cognitive impairment CLINICAL EVALUATION
- A detailed eyewitness account is the cornerstone (semiology, triggers, duration, post-ictal state).
- Distinguish epileptic seizures from mimics — breath-holding spells, syncope, and non-epileptic events.
- Developmental and neurological assessment; look for underlying causes.
INVESTIGATIONS
- EEG — supports the diagnosis, classifies the seizure/syndrome, and identifies characteristic patterns (e.g. 3 Hz spike-wave, hypsarrhythmia).
- Neuroimaging (MRI) — for focal seizures, focal deficits, or drug-resistant epilepsy (to find a structural cause).
- Metabolic/genetic tests when indicated (especially in infants with regression).
MANAGEMENT — Principles
- Start an antiepileptic drug (AED) after a confirmed diagnosis, choosing by seizure type; use monotherapy at the lowest effective dose.
- Titrate gradually; add a second drug or switch if the first fails.
- Aim for seizure freedom with minimal side-effects.
DRUG CHOICE BY SEIZURE TYPE
Seizure type First-line options Generalised tonic-clonic Valproate, levetiracetam Focal Carbamazepine, levetiracetam, oxcarbazepine Absence Ethosuximide, valproate Myoclonic Valproate, levetiracetam ⚠️DANGER / REMEMBER: Avoid carbamazepine/oxcarbazepine in absence and myoclonic seizures — they can worsen these generalised seizure types. Sodium valproate is avoided in adolescent girls where possible (teratogenicity).REFRACTORY EPILEPSY & OTHER MEASURES
- For drug-resistant epilepsy — reassess the diagnosis, then consider the ketogenic diet, epilepsy surgery, or vagus nerve stimulation.
- Address adherence, triggers (sleep deprivation), and lifestyle/safety (swimming, heights).
SEIZURE vs EPILEPSY
A single provoked seizure (e.g. febrile, hypoglycaemic) is not epilepsy. Epilepsy implies an enduring tendency to unprovoked seizures — the distinction determines whether long-term treatment is needed.
PRECIPITATING / TRIGGER FACTORS
- Sleep deprivation, missed medication, intercurrent illness/fever.
- Flickering lights (photosensitivity), stress, and (in adolescents) alcohol.
COMMON AED SIDE-EFFECTS (counsel about these)
Drug Notable side-effects Valproate Weight gain, hair loss, hepatotoxicity, teratogenic Carbamazepine Rash, hyponatraemia, marrow suppression Phenytoin Gum hypertrophy, hirsutism, ataxia Levetiracetam Behavioural/mood changes WITHDRAWAL OF THERAPY
AEDs can often be gradually withdrawn after a seizure-free period of about 2 years, taking into account the seizure type/syndrome, EEG and the family's wishes; tapering is done slowly to avoid withdrawal seizures.
PSYCHOSOCIAL ASPECTS
- Address stigma, schooling, and activity safety (swimming, cycling, heights).
- Screen for and manage associated learning and behavioural difficulties.
📝CLINICAL / APPLIED POINTS- The diagnosis is largely clinical — a good eyewitness description outweighs a single normal EEG.
- A normal EEG does not exclude epilepsy, and an abnormal EEG alone does not diagnose it.
- Match the drug to the seizure type; the wrong drug can worsen certain generalised seizures.
- Counsel on adherence, safety and the plan for eventual withdrawal after a seizure-free period.
- Reassess 'refractory' cases — non-adherence and misdiagnosis are common reasons for apparent drug failure.
💊KEY DRUG DOSES (viva)- Valproate 15–40 mg/kg/day; carbamazepine 10–20 mg/kg/day; levetiracetam 20–40 mg/kg/day.
- Ethosuximide (absence) 20 mg/kg/day; phenobarbitone 3–5 mg/kg/day.
- West syndrome: ACTH / vigabatrin.
🔑KEY POINTS TO REMEMBER- Epilepsy = ≥ 2 unprovoked seizures > 24 h apart (enduring predisposition).
- Seizures: focal vs generalised (tonic-clonic, absence, myoclonic, atonic).
- EEG classifies and supports; MRI for focal/refractory cases.
- Monotherapy chosen by seizure type; avoid carbamazepine in absence/myoclonic.
- Refractory → ketogenic diet, surgery, VNS; counsel on adherence, safety, withdrawal.
📚SOURCES: Ghai Essential Pediatrics; Nelson Textbook of Pediatrics; ILAE classification.DEFINITION
Acute bacterial (pyogenic) meningitis is inflammation of the meninges caused by pyogenic bacteria. It is a medical emergency with high mortality and a risk of serious neurological sequelae if treatment is delayed.
ETIOLOGY (age-dependent)
- Neonates — Group B Streptococcus, E. coli, Klebsiella, Listeria monocytogenes.
- Beyond 3 months — Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae type b (Hib now rare where vaccinated).
- Predisposing factors — otitis media/sinusitis, head trauma/CSF leak, immunodeficiency, and neural-tube defects.
CLINICAL FEATURES
- Older children — fever, headache, vomiting, photophobia, neck stiffness, altered sensorium and seizures.
- Neonates/infants — non-specific: fever or hypothermia, poor feeding, lethargy, irritability, a high-pitched cry, bulging anterior fontanelle and seizures.
- Signs of meningeal irritation — Kernig's and Brudzinski's signs and neck rigidity (often absent in young infants).
- A petechial/purpuric rash suggests meningococcal disease.
INVESTIGATIONS
- Lumbar puncture & CSF examination — the key investigation.
- Blood culture, CBC, CRP, blood glucose (for CSF:blood glucose ratio), electrolytes.
- CT head before LP if there are signs of raised intracranial pressure, focal deficits or coma (to avoid herniation).
TYPICAL CSF FINDINGS
Parameter Pyogenic meningitis Appearance Turbid / cloudy Cells ↑↑ (neutrophils/polymorphs) Protein Raised Glucose Low (< ½ blood glucose) Gram stain/culture Organism seen/grown MANAGEMENT
- Prompt empirical IV antibiotics — do not delay for investigations: ceftriaxone/cefotaxime (± vancomycin); add ampicillin for Listeria in neonates.
- Dexamethasone (with or just before the first antibiotic dose) reduces hearing loss, particularly in Hib meningitis.
- Supportive care — airway/oxygen, careful fluids (watch for SIADH), control seizures and raised intracranial pressure, and treat shock.
- Isolation and chemoprophylaxis of close contacts for meningococcal (and Hib) disease.
COMPLICATIONS
- Acute — seizures, raised ICP, SIADH, subdural effusion/empyema, cerebral infarction, shock (meningococcaemia).
- Long-term — sensorineural hearing loss, hydrocephalus, epilepsy, cerebral palsy, and intellectual disability.
PREVENTION
- Vaccination — Hib, pneumococcal (PCV) and meningococcal vaccines.
- Chemoprophylaxis of close contacts (rifampicin/ciprofloxacin/ceftriaxone) for meningococcal and Hib.
- Early recognition and prompt treatment reduce mortality and sequelae.
PATHOPHYSIOLOGY
Bacteria reach the meninges (haematogenous / direct spread) → Inflammation of the meninges & subarachnoid space → Cerebral oedema, raised ICP, impaired CSF flow, vasculitis → Neuronal injury → deficits, seizures, sequelae
CSF IN DIFFERENT MENINGITIDES
Parameter Pyogenic Viral Tubercular Cells ↑↑ Neutrophils ↑ Lymphocytes ↑ Lymphocytes Protein High Mildly high Very high Glucose Low Normal Low Appearance Turbid Clear Cobweb clot MONITORING & MANAGEMENT OF COMPLICATIONS
- Watch for and treat SIADH (fluid restriction), raised ICP, and seizures.
- A persistent fever or focal signs → suspect subdural effusion/empyema (imaging).
- Arrange a hearing assessment on recovery — deafness is the commonest sequela.
EMPIRICAL ANTIBIOTIC BY AGE
Age Empirical regimen Neonate Ampicillin + cefotaxime (± gentamicin) > 3 months Ceftriaxone/cefotaxime (± vancomycin) PROGNOSIS
Outcome depends on the organism, the child's age, and — crucially — the speed of starting antibiotics. Delay increases mortality and the risk of permanent sequelae such as deafness, hydrocephalus and neurodevelopmental impairment; hence the emphasis on immediate treatment.
KEY SUPPORTIVE MEASURES
- Maintain airway, oxygenation and circulation; treat shock (especially meningococcaemia).
- Careful fluid management (neither over- nor under-hydrate; watch for SIADH).
- Control seizures and raised intracranial pressure; monitor neurological status closely.
📝CLINICAL / APPLIED POINTS- Meningitis is an emergency — give antibiotics immediately; never delay treatment for a CT or LP.
- In young infants the signs are non-specific — a bulging fontanelle and high-pitched cry are important clues.
- Do a CT before LP only if there are signs of raised ICP/focal deficit, but start antibiotics first.
- Give dexamethasone with the first dose of antibiotic to reduce hearing loss.
- Arrange a hearing assessment after recovery — deafness is the commonest sequela.
💊KEY DRUG DOSES (viva)- Ceftriaxone 100 mg/kg/day (meningitic dose) ± vancomycin 60 mg/kg/day; add ampicillin for neonatal Listeria.
- Dexamethasone 0.15 mg/kg/dose 6-hourly × 2–4 days (with/just before first antibiotic).
🔑KEY POINTS TO REMEMBER- Emergency; organisms are age-dependent (neonates: GBS/E. coli/Listeria; older: pneumococcus/meningococcus/Hib).
- Fever, neck stiffness, altered sensorium; non-specific in infants (bulging fontanelle, high-pitched cry).
- CSF: turbid, high neutrophils, high protein, LOW glucose.
- Immediate IV ceftriaxone (± vancomycin/ampicillin) + dexamethasone; don't delay for tests.
- Sequelae: deafness, hydrocephalus, epilepsy; prevent with vaccines + contact prophylaxis.
📚SOURCES: Ghai Essential Pediatrics; Nelson Textbook of Pediatrics; IAP/WHO meningitis guidelines.DEFINITION
Cerebral palsy (CP) is a group of permanent, non-progressive disorders of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances in the developing brain (occurring before, during, or in the first 2–3 years after birth). Although the brain lesion is static, the clinical picture may change as the child grows.
ETIOLOGY
- Prenatal (commonest overall) — congenital infections (TORCH), brain malformations, prematurity.
- Perinatal — birth asphyxia (HIE), prematurity/low birth weight, kernicterus.
- Postnatal — meningitis/encephalitis, head trauma, hypoglycaemia, severe hyperbilirubinaemia, intracranial haemorrhage.
CLASSIFICATION (by type of motor disorder)
Type Features Spastic (commonest, ~70%) Increased tone; hemiplegic, diplegic, or quadriplegic Dyskinetic (athetoid) Involuntary movements; often post-kernicterus Ataxic Incoordination, balance problems Mixed Combination of the above CLINICAL FEATURES
- Delayed motor milestones and abnormal tone (spasticity or, early on, hypotonia).
- Persistence of primitive reflexes beyond the expected age, and delayed postural reflexes.
- Abnormal posture and movement patterns; early hand preference (before 1 year) in hemiplegia.
- Feeding difficulties, drooling and, later, contractures/deformities.
ASSOCIATED PROBLEMS (must be actively sought)
- Intellectual disability and learning difficulties; epilepsy.
- Visual and hearing impairment; speech and language problems.
- Feeding/swallowing difficulties and malnutrition; behavioural problems; constipation.
DIAGNOSIS & INVESTIGATIONS
- Primarily a clinical diagnosis based on the history and neurological examination over time.
- MRI brain — to identify the underlying lesion/aetiology.
- Assess the associated problems — vision, hearing, EEG (if seizures), and developmental/cognitive evaluation.
MANAGEMENT (multidisciplinary)
- Physiotherapy, occupational therapy and speech therapy to maximise function.
- Spasticity management — orthoses, oral baclofen, botulinum toxin, and orthopaedic surgery for contractures.
- Treat associated conditions — epilepsy, feeding (may need gastrostomy), vision/hearing, and nutrition.
- Family support and education, assistive devices, and inclusive schooling; long-term, goal-directed care.
PREVENTION
- Good antenatal and perinatal care, skilled birth attendance and neonatal resuscitation.
- Prevention/prompt treatment of neonatal jaundice, hypoglycaemia, infections and birth asphyxia.
EARLY WARNING SIGNS (helping earlier diagnosis)
- Delayed motor milestones and abnormal tone (early hypotonia or evolving spasticity).
- Persistent primitive reflexes, fisting beyond 3 months, and early hand preference (< 1 year).
- Feeding difficulties, irritability and abnormal postures.
FUNCTIONAL CLASSIFICATION (GMFCS)
The Gross Motor Function Classification System (GMFCS, levels I–V) grades mobility from independent walking (I) to fully dependent (V), and is used to plan management and predict function.
DIFFERENTIAL DIAGNOSIS
- Progressive/degenerative neurological disorders — CP is non-progressive, so a child who is LOSING skills needs a workup for a metabolic/neurodegenerative disease.
- Spinal cord lesions, muscular dystrophies and hereditary neuropathies.
ORTHOPAEDIC & SPASTICITY CARE
- Prevent and treat contractures and hip subluxation (regular monitoring).
- Options — physiotherapy, orthoses, oral baclofen, botulinum toxin, intrathecal baclofen, and orthopaedic/selective dorsal rhizotomy surgery.
NUTRITION & GENERAL CARE
- Feeding/swallowing difficulties are common — assess nutrition; a gastrostomy may be needed for safe feeding.
- Manage drooling, constipation, dental care, and sleep; prevent aspiration.
ROLE OF THE FAMILY & COMMUNITY
The family is central to therapy — home exercises, positioning, and use of aids. Community-based rehabilitation, inclusive education, disability support and realistic goal-setting all improve participation and quality of life.
MULTIDISCIPLINARY TEAM
- Paediatrician, physiotherapist, occupational and speech therapists, orthopaedic surgeon.
- Ophthalmologist, audiologist, dietitian, psychologist, special educator and social worker.
- Coordinated, goal-directed care around the child and family.
📝CLINICAL / APPLIED POINTS- CP is a static lesion but a changing clinical picture — re-assess needs as the child grows.
- Persisting primitive reflexes and early hand preference are useful early clues.
- Always look for the treatable associated problems (epilepsy, hearing/vision, feeding, nutrition).
- Management is multidisciplinary and goal-directed — the aim is maximal function and participation, not 'cure'.
- Support and educate the family; realistic, compassionate counselling is central.
🔑KEY POINTS TO REMEMBER- Permanent, non-progressive motor/posture disorder from a lesion in the developing brain.
- Causes: prenatal (commonest), perinatal (asphyxia, prematurity, kernicterus), postnatal.
- Spastic type commonest; delayed milestones, abnormal tone, persistent primitive reflexes.
- Actively seek associated problems: epilepsy, intellectual disability, vision/hearing, feeding.
- Multidisciplinary therapy + spasticity management + family support; prevent via perinatal care.
📚SOURCES: Ghai Essential Pediatrics; Nelson Textbook of Pediatrics.DEFINITION
Guillain-Barré syndrome (GBS) is an acute, immune-mediated demyelinating polyradiculoneuropathy causing rapidly progressive ascending flaccid paralysis. In the post-polio era it is the commonest cause of acute flaccid paralysis (AFP) in children.
ETIOLOGY & PATHOGENESIS
- Typically post-infectious — 1–3 weeks after a respiratory or gastrointestinal infection (classically Campylobacter jejuni; also viral).
- Molecular mimicry — antibodies against the pathogen cross-react with peripheral nerve myelin/axons → immune-mediated nerve damage.
CLINICAL FEATURES
- Ascending, symmetrical flaccid weakness — starting in the legs and progressing upward over days.
- Areflexia / hyporeflexia (a key sign).
- Respiratory muscle involvement (may need ventilation) and bulbar weakness (swallowing/speech) — the dangerous features.
- Autonomic instability — labile blood pressure, arrhythmias, sweating.
- Sensory symptoms are usually mild; sphincter function is typically preserved.
DIFFERENTIAL DIAGNOSIS OF ACUTE FLACCID PARALYSIS
Feature GBS Poliomyelitis Transverse myelitis Weakness Symmetrical, ascending Asymmetrical Para/quadriparesis, a sensory level Reflexes Absent Absent Increased later Sensory Mild Normal Sensory level Bladder/bowel Spared Spared Involved INVESTIGATIONS
- CSF — albuminocytological dissociation (raised protein with a normal/near-normal cell count) — characteristic (may be normal in the first week).
- Nerve conduction studies — features of demyelination (slowed conduction, conduction block).
- Exclude other causes of AFP (stool for poliovirus in surveillance; imaging for cord lesions).
MANAGEMENT
- Supportive care is paramount — monitor respiratory function (vital capacity) and provide ventilation if it deteriorates; protect the airway if bulbar weakness.
- Monitor autonomic function (cardiac monitoring); manage blood-pressure lability and arrhythmias.
- Immunotherapy — IV immunoglobulin (IVIG) or plasmapheresis (equally effective) to shorten the illness and improve recovery.
- Physiotherapy, DVT prophylaxis, pain management and nutritional support during recovery.
PROGNOSIS
Most children recover completely or with minimal deficit over weeks to months, as the peripheral nerves remyelinate. The main acute risks are respiratory failure and autonomic instability, which is why close monitoring in the acute phase is essential. GBS is a notifiable AFP for polio-surveillance purposes.
CLINICAL VARIANTS
- Miller Fisher syndrome — ophthalmoplegia, ataxia and areflexia.
- Acute motor axonal neuropathy (AMAN) and other axonal variants; pharyngeal-cervical-brachial variant.
MONITORING (the crux of care)
- Serial vital capacity / respiratory function — the key to timing ventilation.
- Continuous cardiac monitoring for autonomic arrhythmias; blood-pressure lability.
- Bulbar function (safe swallow) to prevent aspiration.
COMPLICATIONS
- Respiratory failure requiring ventilation; aspiration pneumonia.
- Autonomic instability — arrhythmias, labile BP; immobility complications (DVT, pressure sores).
FACTORS AFFECTING RECOVERY
Most children recover well; a rapid onset, need for ventilation, and axonal (rather than demyelinating) forms are associated with slower or less complete recovery. Because GBS is the main cause of AFP, every case must be reported and investigated to exclude poliomyelitis.
SUPPORTIVE CARE DURING RECOVERY
- Physiotherapy and graded mobilisation to prevent contractures and regain strength.
- DVT prophylaxis, pressure-area care, bladder/bowel care, and pain management (neuropathic pain is common).
WHY IT MATTERS FOR POLIO SURVEILLANCE
Because GBS is the leading cause of acute flaccid paralysis, every case in a child under 15 years must be notified and investigated (with stool samples for poliovirus) as part of the national polio-surveillance programme, even when GBS is the obvious clinical diagnosis.
KEY EXAM POINT
The classic picture is ascending symmetrical flaccid weakness with areflexia after a recent infection, with CSF albuminocytological dissociation; the priorities are close respiratory/autonomic monitoring and IVIG or plasmapheresis.
📝CLINICAL / APPLIED POINTS- The classic picture is ascending symmetrical weakness with areflexia after a recent infection.
- The two life-threats are respiratory failure and autonomic instability — monitor vital capacity and cardiac rhythm closely.
- CSF albuminocytological dissociation supports the diagnosis but may be normal in the first week.
- IVIG and plasmapheresis are equally effective; steroids alone are NOT beneficial.
- Any child with acute flaccid paralysis must be notified/investigated to exclude poliomyelitis.
💊KEY DRUG DOSES (viva)- IVIG 0.4 g/kg/day × 5 days (2 g/kg total), OR plasmapheresis.
- Supportive: respiratory monitoring/ventilation; NO steroids (ineffective alone).
🔑KEY POINTS TO REMEMBER- Acute immune-mediated demyelinating polyneuropathy; commonest cause of AFP.
- Post-infectious (Campylobacter); ascending symmetrical flaccid weakness with areflexia.
- Watch for respiratory failure, bulbar weakness and autonomic instability.
- CSF: albuminocytological dissociation; NCS shows demyelination.
- Supportive care (respiratory monitoring) + IVIG/plasmapheresis; good recovery usual.
📚SOURCES: Ghai Essential Pediatrics; Nelson Textbook of Pediatrics; national AFP surveillance guidelines.DEFINITION
Status epilepticus is a continuous seizure lasting ≥ 5 minutes, or recurrent seizures without full recovery of consciousness between them. It is a neurological emergency with a risk of permanent brain injury and death.
CAUSES
- Prolonged febrile seizure; CNS infection (meningitis/encephalitis).
- Poor antiepileptic-drug adherence or withdrawal; metabolic (hypoglycaemia, hyponatraemia, hypocalcaemia).
- Acute brain injury (trauma, stroke, hypoxia); poisoning.
STEPWISE MANAGEMENT
- ABC — airway, breathing, circulation; give oxygen; secure IV access; check blood glucose (treat hypoglycaemia).
- First-line (5 min): a benzodiazepine — IV lorazepam or IV/buccal/intranasal midazolam (may repeat once).
- Second-line (if continuing): IV phenytoin/fosphenytoin, levetiracetam or valproate.
- Refractory: anaesthetic agents (midazolam/thiopentone infusion) with intubation and ICU care.
- Treat the underlying cause throughout.
COMPLICATIONS
- Hypoxia, aspiration; hyperthermia and rhabdomyolysis.
- Cerebral injury; cardiovascular/metabolic derangements.
PRINCIPLES OF DRUG THERAPY
- Follow a time-based protocol — the longer a seizure continues, the harder it is to stop.
- Do not delay first-line benzodiazepine; prepare second-line and airway support early.
KEY EXAM POINT
Remember the sequence — ABC + glucose → benzodiazepine → second-line AED → anaesthesia — and always look for and treat the underlying cause (infection, metabolic, non-adherence).
CAUSES TO ALWAYS EXCLUDE
- Hypoglycaemia (check glucose in every case) and electrolyte disturbances.
- CNS infection (meningitis/encephalitis); AED non-adherence; toxins/poisoning.
COMPLICATIONS OF PROLONGED SEIZURES
- Hypoxic brain injury, aspiration, hyperthermia and rhabdomyolysis.
- Cardiorespiratory compromise — hence the priority on airway, oxygen and prompt drug therapy.
KEY EXAM POINT
Status epilepticus is defined by a seizure lasting ≥ 5 minutes (the older '30-minute' definition is outdated for treatment purposes); early, protocol-driven therapy prevents refractoriness and brain injury.
💊KEY DRUG DOSES (viva)- Benzodiazepine: IV lorazepam 0.1 mg/kg, or midazolam 0.15 mg/kg IV / 0.3 mg/kg buccal.
- Second-line: phenytoin/fosphenytoin 20 mg/kg, levetiracetam 40 mg/kg, or valproate 20–40 mg/kg IV.
- Refractory: midazolam/thiopentone infusion + intubation.
🔑KEY POINTS TO REMEMBER- Seizure ≥ 5 min or recurrent seizures without recovery — an emergency.
- ABC + oxygen + glucose check first.
- Benzodiazepine → phenytoin/levetiracetam/valproate → anaesthesia (refractory).
- Always identify and treat the underlying cause.
📚SOURCES: Ghai Essential Pediatrics; Nelson Textbook of Pediatrics.DEFINITION
Tubercular meningitis (TBM) is meningitis caused by Mycobacterium tuberculosis. It is a subacute, basal meningitis and the most serious form of extrapulmonary tuberculosis in children, with high morbidity if treatment is delayed.
CLINICAL STAGES
- Stage I (prodromal) — non-specific: low-grade fever, malaise, irritability, anorexia; no focal signs.
- Stage II — meningeal signs, cranial nerve palsies (basal exudate), altered sensorium, raised ICP.
- Stage III — coma, dense deficits, decerebration.
INVESTIGATIONS
- CSF — lymphocytic pleocytosis, high protein, low glucose, and a fine 'cobweb' clot; AFB stain/culture/GeneXpert.
- Neuroimaging — basal exudates, hydrocephalus, infarcts, tuberculomas.
- Evidence of TB elsewhere (chest X-ray, Mantoux, contact history).
MANAGEMENT
- Anti-tubercular therapy (ATT) — an intensive multidrug regimen followed by prolonged continuation (longer than for pulmonary TB).
- Corticosteroids — reduce inflammation, oedema and mortality.
- Manage hydrocephalus (may need a shunt) and raised ICP; supportive care.
COMPLICATIONS
- Hydrocephalus (basal exudates blocking CSF flow), cranial-nerve palsies, and vasculitic infarcts (stroke).
- Tuberculoma, SIADH, and long-term neurological deficits.
KEY EXAM POINT
Prognosis depends heavily on the stage at which treatment starts — stage I disease does well, whereas stage III (coma) has high mortality and morbidity, so early suspicion and ATT are vital.
DIAGNOSIS — SUPPORTING EVIDENCE
- Evidence of TB elsewhere — chest X-ray (miliary/primary complex), a positive Mantoux, and a contact history.
- CSF GeneXpert/AFB improves confirmation; imaging shows basal enhancement and hydrocephalus.
PREVENTION
BCG vaccination reduces severe forms such as TBM and miliary TB in young children; early detection and treatment of infectious adult contacts and chemoprophylaxis of exposed young children are also important.
KEY POINTS ON MANAGEMENT
- Start ATT on strong clinical/CSF suspicion — do not wait for culture confirmation.
- Add corticosteroids; treat hydrocephalus and raised ICP; ensure adherence for the full prolonged course.
💊KEY DRUG DOSES (viva)- ATT — intensive phase (HRZE) then prolonged continuation (total 9–12 months).
- Prednisolone 2 mg/kg/day × 4 weeks then taper; manage hydrocephalus.
🔑KEY POINTS TO REMEMBER- Subacute basal meningitis from M. tuberculosis; staged I–III.
- CSF: lymphocytic, high protein, low glucose, cobweb clot.
- Imaging: basal exudates, hydrocephalus, infarcts.
- ATT + steroids; treat hydrocephalus; prognosis depends on the stage at treatment.
📚SOURCES: Ghai Essential Pediatrics; Nelson Textbook of Pediatrics.DEFINITION
Breath-holding spells are benign, involuntary paroxysmal events in young children (6 months–5 years) in which the child stops breathing (in expiration) during crying, leading to colour change and sometimes brief loss of consciousness. They are NOT epilepsy and are always triggered.
TYPES
Type Trigger Colour Cyanotic (commonest) Anger, frustration, crying Blue Pallid Pain, fright (vagal) Pale CLINICAL FEATURES
- A precipitant (upset/pain) → vigorous cry → breath-holding → colour change → brief limpness/loss of consciousness (± a few clonic jerks).
- Rapid, complete recovery; the child is entirely normal between episodes.
EVALUATION & MANAGEMENT
- A clinical diagnosis; exclude iron-deficiency anaemia (associated and treatable) and cardiac arrhythmia if atypical.
- Reassurance — explain the benign, self-limiting nature; avoid reinforcing the behaviour.
- Treat iron deficiency if present (often reduces spells); no antiepileptic drugs needed.
DIFFERENTIATION FROM EPILEPTIC SEIZURES
Feature Breath-holding spell Epileptic seizure Trigger Always present (cry/pain) Usually none Colour change Before the event After/during Recovery Rapid, complete Post-ictal drowsiness KEY EXAM POINT
The always-present trigger and rapid full recovery distinguish breath-holding spells from epilepsy; check for and treat iron-deficiency anaemia, and reassure the family that they are benign.
REASSURANCE & COURSE
Breath-holding spells resolve spontaneously by around 4–5 years of age; parents should be reassured, advised not to reinforce the behaviour, and told that the child comes to no harm despite the alarming appearance.
🔑KEY POINTS TO REMEMBER- Benign, triggered paroxysmal events in 6 months–5 years; NOT epilepsy.
- Cyanotic (anger) vs pallid (pain/fright, vagal); brief loss of consciousness with full recovery.
- Exclude iron-deficiency anaemia; treat it if present.
- Reassurance is the mainstay; self-limiting with age.
📚SOURCES: Ghai Essential Pediatrics; Nelson Textbook of Pediatrics.DEFINITION
Neurocutaneous syndromes (phakomatoses) are a group of disorders with combined skin and nervous-system involvement, arising from abnormal development of tissues of ectodermal origin. Most are inherited in an autosomal dominant pattern.
NEUROFIBROMATOSIS TYPE 1
- ≥ 6 café-au-lait macules, axillary/inguinal freckling, cutaneous neurofibromas, Lisch nodules (iris hamartomas), optic glioma.
TUBEROUS SCLEROSIS
- Ash-leaf (hypopigmented) macules, adenoma sebaceum (facial angiofibromas), shagreen patch.
- Seizures (infantile spasms), developmental delay; cortical tubers; renal and cardiac (rhabdomyoma) lesions.
STURGE-WEBER SYNDROME
- Facial port-wine stain (trigeminal distribution) + leptomeningeal angioma.
- Seizures, hemiparesis, glaucoma and intellectual disability.
INHERITANCE & COMPLICATIONS
- Most are autosomal dominant with variable expression; genetic counselling is important.
- Complications include epilepsy, learning difficulties, tumours (optic glioma in NF1; renal/cardiac in tuberous sclerosis) and glaucoma (Sturge-Weber).
KEY EXAM POINT
Recognise the classic skin marker of each — café-au-lait macules (NF1), ash-leaf macules/adenoma sebaceum (tuberous sclerosis), and a facial port-wine stain (Sturge-Weber) — each with its associated neurological features.
MANAGEMENT PRINCIPLES
- No cure — care is multidisciplinary and surveillance-based: control seizures, monitor for the associated tumours, and support development/learning.
- Regular ophthalmology (glaucoma in Sturge-Weber; optic glioma in NF1) and imaging as indicated.
- Genetic counselling for the family.
SCREENING & FOLLOW-UP
- Regular developmental, ophthalmological and (as indicated) imaging surveillance for the associated tumours and complications.
- Blood-pressure monitoring (renal lesions in tuberous sclerosis; phaeochromocytoma in NF1).
NF1 DIAGNOSTIC CRITERIA (≥ 2 of 7)
- ≥ 6 café-au-lait macules (> 5 mm pre-pubertal / > 15 mm post-pubertal).
- ≥ 2 neurofibromas or 1 plexiform neurofibroma; axillary/inguinal freckling.
- ≥ 2 Lisch nodules; optic glioma; a distinctive bony lesion; a first-degree relative with NF1.
🔑KEY POINTS TO REMEMBER- Phakomatoses = combined skin + CNS disease; mostly autosomal dominant.
- NF1: café-au-lait macules, neurofibromas, Lisch nodules.
- Tuberous sclerosis: ash-leaf macules, adenoma sebaceum, seizures, delay.
- Sturge-Weber: port-wine stain, leptomeningeal angioma, seizures, glaucoma.
📚SOURCES: Ghai Essential Pediatrics; Nelson Textbook of Pediatrics.DEFINITION
Encephalitis is inflammation of the brain parenchyma, usually viral, presenting with fever and altered brain function (as opposed to meningitis, which primarily inflames the meninges).
ETIOLOGY
- Herpes simplex virus (HSV) — treatable and important (temporal-lobe involvement).
- Japanese encephalitis — a major cause in India (mosquito-borne, epidemic).
- Enteroviruses, measles, mumps, dengue and others.
CLINICAL FEATURES
- Fever with altered sensorium/behaviour, headache and vomiting.
- Seizures, focal neurological deficits, and features of raised intracranial pressure.
INVESTIGATIONS & MANAGEMENT
- CSF — lymphocytic pleocytosis, mildly raised protein, normal glucose; PCR for the virus (HSV).
- MRI (temporal lobes in HSV) and EEG.
- Empirical IV acyclovir (for possible HSV) pending results; supportive care — manage seizures, raised ICP and fluids.
- Prevention — Japanese encephalitis vaccine in endemic areas; vector control.
MENINGITIS vs ENCEPHALITIS
Meningitis primarily inflames the meninges (neck stiffness, photophobia, preserved cerebration early), whereas encephalitis inflames the brain parenchyma (early altered sensorium, behavioural change, seizures, focal deficits). Overlap ('meningoencephalitis') is common.
KEY EXAM POINT
Start empirical acyclovir in any child with suspected encephalitis until HSV is excluded — early treatment of HSV encephalitis greatly improves the outcome.
COMPLICATIONS & PROGNOSIS
- Raised intracranial pressure, status epilepticus, and long-term neurological sequelae (motor, cognitive, epilepsy).
- Japanese encephalitis in particular carries a high risk of death or disability; HSV encephalitis outcome improves markedly with early acyclovir.
KEY EXAM POINT
Any child with fever plus altered sensorium or seizures has encephalitis until proven otherwise — start empirical acyclovir for possible HSV immediately, and remember Japanese encephalitis as a major, vaccine-preventable cause in India.
🔑KEY POINTS TO REMEMBER- Inflammation of brain parenchyma → fever + altered brain function ± seizures/deficits.
- HSV (treatable) and Japanese encephalitis (major in India) are key causes.
- CSF lymphocytic + PCR; MRI (temporal lobes in HSV); EEG.
- Start empirical acyclovir + supportive care; JE vaccine for prevention.
📚SOURCES: Ghai Essential Pediatrics; Nelson Textbook of Pediatrics.DEFINITION
Hydrocephalus is an abnormal accumulation of cerebrospinal fluid (CSF) within the ventricles, causing ventricular dilatation and raised intracranial pressure, due to obstruction of flow, impaired absorption, or (rarely) overproduction of CSF.
TYPES & CAUSES
- Obstructive (non-communicating) — block within the ventricular system: aqueductal stenosis, Dandy-Walker malformation, tumour.
- Communicating — impaired CSF absorption: post-meningitis, post-haemorrhage.
- Associated with neural-tube defects (spina bifida / Arnold-Chiari).
CLINICAL FEATURES
- Infants — rapidly increasing head circumference, tense/bulging fontanelle, widely split sutures, 'sunset' eyes, dilated scalp veins, irritability and vomiting.
- Older children (fused sutures) — headache, vomiting, papilloedema and other signs of raised ICP.
INVESTIGATIONS & MANAGEMENT
- Cranial ultrasound (through the open fontanelle in infants); CT/MRI to define the cause and level of obstruction.
- Ventriculoperitoneal (VP) shunt or endoscopic third ventriculostomy (ETV); treat the underlying cause.
- Monitor for shunt complications — blockage and infection.
COMPLICATIONS OF SHUNTS
- Blockage (recurrent raised-ICP symptoms) and infection (fever, shunt-tract signs).
- Over-drainage, and the need for revisions as the child grows.
KEY EXAM POINT
In an infant, serial head-circumference measurement crossing centiles plus a bulging fontanelle and 'sunset eyes' is the classic presentation; imaging defines the type and cause, and a VP shunt or ETV is the treatment.
PATHOPHYSIOLOGY (CSF circulation)
CSF is produced by the choroid plexus, flows through the ventricles and is absorbed by the arachnoid villi. Hydrocephalus results when this pathway is obstructed or absorption is impaired, so CSF accumulates and raises intracranial pressure.
ASSOCIATED CONDITIONS
- Neural-tube defects (spina bifida, Arnold-Chiari malformation); intraventricular haemorrhage of prematurity.
- Post-meningitic and post-haemorrhagic hydrocephalus.
KEY EXAM POINT
In an infant, an abnormally increasing head circumference is the earliest and most important sign; measure and plot it serially, and image (ultrasound through the fontanelle, or CT/MRI) to confirm and define the cause before shunting.
🔑KEY POINTS TO REMEMBER- Excess CSF → ventricular dilatation + raised ICP.
- Obstructive (aqueductal stenosis) vs communicating (post-meningitis/haemorrhage).
- Infants: increasing head circumference, bulging fontanelle, sunset eyes.
- USG/CT/MRI; treat with VP shunt or ETV; watch for shunt block/infection.
📚SOURCES: Ghai Essential Pediatrics; Nelson Textbook of Pediatrics.DEFINITION
Duchenne muscular dystrophy (DMD) is a severe, progressive X-linked recessive muscle disorder caused by a mutation in the dystrophin gene (absent dystrophin), leading to progressive muscle degeneration. It affects boys.
CLINICAL FEATURES
- Onset in early childhood — delayed walking, frequent falls, difficulty running/climbing stairs.
- Proximal muscle weakness; Gower's sign (using hands to 'climb up' the legs when rising).
- Calf pseudohypertrophy (fat/fibrous replacement), waddling (lordotic) gait.
- Progressive — usually wheelchair-bound by ~12 years; later cardiomyopathy and respiratory failure.
INVESTIGATIONS
- Markedly raised serum creatine kinase (CK).
- Genetic testing (dystrophin gene deletion) — confirmatory; muscle biopsy (absent dystrophin) if needed.
MANAGEMENT
- Corticosteroids — slow the decline in muscle strength and prolong ambulation.
- Multidisciplinary supportive care — physiotherapy, prevention of contractures, and cardiac and respiratory monitoring.
- Genetic counselling and carrier detection for the family.
GENETICS & CARRIER DETECTION
- X-linked recessive — affected boys, carrier mothers; ~⅓ are new mutations.
- Female carriers may have a raised CK and, occasionally, mild symptoms; offer genetic counselling and prenatal testing.
KEY EXAM POINT
Suspect DMD in a boy with delayed walking, a waddling gait, calf pseudohypertrophy and Gower's sign; a very high CK and dystrophin gene testing confirm it, and steroids plus multidisciplinary care are the mainstay.
BECKER MUSCULAR DYSTROPHY (contrast)
Becker muscular dystrophy is caused by mutations in the same dystrophin gene but with reduced (not absent) dystrophin — it is milder, with a later onset and slower progression than DMD.
COMPLICATIONS
- Progressive loss of ambulation; joint contractures and scoliosis.
- Dilated cardiomyopathy and respiratory failure are the major causes of death — hence regular cardiac and respiratory surveillance.
SUPPORTIVE & MULTIDISCIPLINARY CARE
- Physiotherapy and stretching to delay contractures; assistive devices and wheelchair when needed.
- Cardiac (echocardiography) and respiratory (lung function, night-time support) surveillance; bone health.
- Steroids to prolong ambulation, plus psychological and educational support for child and family.
💊KEY DRUG DOSES (viva)- Prednisolone 0.75 mg/kg/day (or deflazacort) — slows decline, prolongs ambulation.
- Cardiac (ACE-I/beta-blocker) and respiratory support as disease progresses.
🔑KEY POINTS TO REMEMBER- X-linked recessive dystrophin defect; affects boys; progressive.
- Delayed walking, proximal weakness, Gower's sign, calf pseudohypertrophy.
- Wheelchair by ~12 yr; later cardiomyopathy & respiratory failure.
- Very high CK; confirm by genetic testing; steroids + supportive care + counselling.
📚SOURCES: Ghai Essential Pediatrics; Nelson Textbook of Pediatrics.