Panayiotopoulos syndrome

A benign childhood focal epilepsy (also called early-onset occipital epilepsy) characterized by seizures with prominent autonomic symptoms (especially vomiting) arising from an occipital focus.

• Panayiotopoulos syndrome is an important benign epilepsy of childhood (≈6% of kids with epilepsy). Its dramatic autonomic seizures are often misdiagnosed (as fainting, migraine, GI illness, etc.). Recognizing this syndrome prevents unnecessary invasive workups and highlights the broad manifestations of seizures on exams.

• Occurs in a child (typical onset 3–6 years) with otherwise normal development and neurologic exam.
• Seizures often happen at night or during sleep. Episodes start with autonomic symptoms: the child looks pale, feels nauseated, and usually vomits (classic sign).
• Eyes may deviate to one side; the child can appear to faint (sudden limp unresponsiveness). In about half of episodes a brief convulsion (tonic–clonic) follows the autonomic phase.
• Seizures are often prolonged (lasting several minutes, sometimes >30 min) yet the child fully recovers afterward with no deficits. Seizure frequency is low (many patients have ≤4 seizures total).

1. Suspect PS when a child has episodes of vomiting with altered awareness or convulsive activity, especially if occurring out of sleep (mimicking syncope or gastroenteritis).
2. Obtain an EEG (include sleep): interictal EEG often shows high-amplitude occipital spikes (which may intensify with eyes closed due to "fixation-off" sensitivity). Background is normal and MRI is typically normal.
3. Distinguish from mimics: e.g. vasovagal syncope (usually brief, with a trigger and immediate recovery) or migraine/cyclic vomiting (recurrent vomiting without EEG changes or seizure activity).
4. If exam or imaging shows focal neurologic findings (e.g. hemiparesis or a cortical lesion) or if seizures are atypical (very frequent, progressive, or not resolving), then reconsider the diagnosis – PS is idiopathic and self-limited.

1. Mainly reassurance and education – it's self-limited. Often no daily medication is needed if seizures are infrequent. Provide a rescue benzodiazepine (e.g. rectal diazepam or intranasal midazolam) for any seizure lasting >5 minutes.
2. If seizures are frequent or very prolonged, consider daily therapy. First-line choices include levetiracetam or valproate (broad-spectrum antiseizure meds).
3. Avoid carbamazepine and similar focal AEDs in PS – some reports suggest they can aggravate the seizures.
4. Ensure caregivers know seizure first-aid and have an action plan. Regular follow-up is important, but prognosis is excellent (most patients become seizure-free within a few years).

• Remember the P's of Panayiotopoulos: pale, puke, and prolonged (pallor, vomiting, and long seizures).
• Unique EEG clue: fixation-off sensitivity – occipital spikes appear when the child's eyes are closed (not fixating).
• Despite dramatic events, it's benign: kids typically outgrow the seizures within a few years and have normal development.

• Focal neurologic deficits or developmental regression – not typical for PS (consider structural causes like cortical dysplasia). Children with PS should be neurologically normal between seizures.
• Atypical EEG findings (e.g. diffuse slowing or persistent focal slowing) – suggests another diagnosis (e.g. encephalitis or symptomatic epilepsy) rather than benign PS.
• Unusually refractory or very frequent seizures, or failure to remit by adolescence – reconsider the diagnosis (PS should be self-limited with infrequent seizures).

1. Child with episodic vomiting + possible seizures → get detailed history and EEG (with sleep) to look for occipital spikes consistent with PS.
2. If features fit PS and neuro exam/MRI are normal → diagnose PS (no extensive workup needed); educate family on benign course and provide rescue med for prolonged seizures.
3. If seizures are rare and short → no daily meds (just observation). If seizures are frequent/prolonged → start an appropriate antiseizure medication (avoid those that may worsen PS).
4. Follow periodically with neurology. Seizures typically remit within a few years. Maintain safety precautions and school planning until seizures have fully resolved.

• Preschooler with nocturnal episodes of vomiting and unresponsiveness (initially thought to be gastroenteritis or syncope) → Panayiotopoulos syndrome (autonomic childhood epilepsy).
• 5‑year‑old turns pale, vomits, and has eyes deviated for several minutes at night, then a brief generalized seizure with quick recovery → classic Panayiotopoulos syndrome seizure.
• EEG showing occipital spike-wave discharges that increase with eye closure in a child with infrequent nighttime seizures → characteristic of Panayiotopoulos syndrome.