⚡ Chapter 30: Paroxysmal Disorders

Nelson's Pediatric Symptom-Based Diagnosis | Seizures · Epilepsy syndromes · Febrile convulsions · Infantile spasms · Non-epileptic paroxysmal events · Status epilepticus

🔍 Paroxysmal Disorders in Children: Key Concepts

🧠 Epileptic vs Non-Epileptic
Epileptic: excessive synchronous neuronal discharge. Non-epileptic: syncope, breath-holding, tics, psychogenic, migraine, sleep disorders.
🔥 Febrile Seizures
6m-5y, fever without CNS infection. Simple (<15 min, generalized, once per 24h). Complex (focal, prolonged, recurrent). No treatment needed for simple.
⚠️ Infantile Spasms (West syndrome)
Clusters of brief flexor/extensor spasms, hypsarrhythmia on EEG, developmental regression. Emergency: ACTH or vigabatrin.
🧬 Epilepsy Syndromes
Benign Rolandic (centrotemporal spikes), Childhood absence (3Hz spike-wave), Lennox-Gastaut (drop attacks), Dravet (SCN1A).
💊 Status Epilepticus
Seizure >5 min or recurrent without recovery. Emergency: benzodiazepines (lorazepam IV, diazepam rectal), then fosphenytoin/levetiracetam, then phenobarbital or propofol.
🔄 Non-epileptic mimics
Breath-holding spells (cyanotic/pallid), syncope, night terrors, tics, shuddering attacks, benign neonatal sleep myoclonus.

💡 Key takeaway: Obtain detailed history and video. Distinguish epileptic seizures from mimics. Febrile seizures are common but reassure families. Infantile spasms are a medical emergency — early treatment improves neurodevelopmental outcome.

🩺 Clinical Approach to Paroxysmal Events (Spells) in Children

🔹 Step 1: Is it a seizure? Red flags for epilepsy
• Stereotyped, recurrent events, often with alteration of consciousness.
• Postictal state (confusion, fatigue, Todd paralysis).
• Eye deviation, automatisms, tonic-clonic movements.
• Provoked by fever, sleep deprivation, flashing lights.
🔹 Step 2: Differentiate epileptic from non-epileptic paroxysmal events
• Syncope: pallor, prodrome (lightheadedness), precipitated by standing, rapid recovery.
• Breath-holding spells: triggered by frustration/pain, cyanotic or pallid, child regains quickly.
• Night terrors: non-REM sleep, inconsolable, no recall, often mid-night.
• Psychogenic non-epileptic seizures (PNES): gradual onset, pelvic thrusting, eyes closed, long duration.
🔹 Step 3: Seizure classification (focal vs generalized)
• Focal: originates in one hemisphere (aura, automatisms, unilateral clonic).
• Generalized: both hemispheres (absence, tonic-clonic, myoclonic, atonic).
• EEG is essential to classify and guide treatment.
🔹 Step 4: Age-specific epilepsy syndromes
• Neonatal: benign familial neonatal (KCNQ2), Ohtahara, early myoclonic.
• Infancy: West syndrome (spasms), Dravet syndrome (SCN1A).
• Childhood: absence (3Hz spike-wave), Rolandic (centrotemporal spikes), Lennox-Gastaut.
• Adolescence: juvenile myoclonic epilepsy (JME).
🔹 Step 5: Management of first seizure & status epilepticus
• First unprovoked seizure: EEG, MRI if focal, risk of recurrence ~40%. Treat after 2nd seizure in most.
• Status epilepticus: 0-5 min: ABCs, lorazepam IV (0.1mg/kg). 5-15 min: fosphenytoin or levetiracetam. >15 min: phenobarbital, intubation, ICU.