Panayiotopoulos syndrome

 

Presentation

It is a benign and self-limiting epilepsy in young 3 – 6y aged children (range is 1 – 13y age) with occipital paroxysms.

 

Episodes of feeling nauseous, looking pale with wide pupils, and flushed face followed by episodes of vomiting. There may be profuse sweating, drooling or incontinence; all described as ‘autonomic’ features.

 

There is change in behaviour with unresponsiveness, focal deviation of eyes and face to one side, with clonic jerking of limbs for several minutes, then post-ictal drowsiness. Most episodes are nocturnal, awakening from sleep; and could last 30 to 60 minutes.

Seizures are infrequent (e.g. once a month)

 

These are different from the ‘benign epilepsy of childhood with occipital paroxysms (BECOP)’, which is seen, in older (8 – 15y) children. BECOP has brief episodes of visual disturbance, seeing flashing lights and headaches.

 

Investigation

The diagnosis is made by ‘history’ with autonomic features during nocturnal episodes in a young child. Video evidence of episodes is very helpful.

 

EEG has bilateral occipital spike discharges, especially during sleep.

 

Management

Since seizures are infrequent and most children only have 3 to 5 episodes in total, so medication is often not necessary.

Seizures often go in to remission spontaneously after a few months or within 3 years from onset. Seizures are not known to return in adulthood.

Development and cognition is not affected.

 

Frequent episodes may concern some families & treatment may sometimes be offered with Carbamazepine, Lamotrigine or Levetiracetam.