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Panayiotopoulos syndrome

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Panayiotopoulos syndrome:

  • idiopathic susceptibility to early onset benign childhood seizures with electroencephalograhic occipital or extra occipital spikes, and manifests mainly with autonomic seizures. It is a benign childhood epilepsy
    • affects about 13% of children of 3-6 years old with one or more non-febrile seizures (peak age 4-5 years)
    • affects about 6% of the age group 1-15 years
    • more common in girls (2)
    • presenting in early childhood (mean 4-7 years) with rare seizures that are prolonged
      • characterised by autonomic features including vomiting, pallor and sweating followed by tonic eye deviation, impairment of consciousness with possible evolution into a secondarily generalised seizure
      • prognosis is excellent and treatment often unnecessary (3)

  • seizures can be prolonged, may mimic non-epileptic disorders, and may vary in severity from trivial to apparently life threatening

  • autonomic seizures are the hallmark of the Panayiotopoulos syndrome
    • autonomic symptoms and signs (mainly vomiting) occur from the onset in 80% of seizures, with half of them lasting for more than 30 minutes to hours, thus amounting to autonomic status epilepticus
    • about two thirds of the seizures occur during nocturnal sleep or brief daytime naps. "In a typical daytime seizure the child looks pale, complains, "I want to be sick," and vomits. If in sleep, the child wakes up with similar complaints or is found vomiting, confused, or unresponsive (1) ".Vomiting occurs in about 75% of seizures
    • other autonomic manifestations may occur either concurrently with vomiting or later in the course of the seizure, and include mydriasis, pallor, cardiorespiratory, gastrointestinal and thermoregulatory alterations, incontinence, and hypersalivation. In 20% or more of the seizures the child becomes pale, unresponsive and flaccid either before convulsing or in isolation
    • of nonautonomic manifestations, lateral eye deviation appears the most common (2)
    • seizures during sleep seem more common than those in wakefulness (2)
    • it is characteristic, even after the most severe seizures and status, for the the child to be normal after a few hours of sleep

  • EEG - generally shows multifocal spikes at various locations - normal in one third of patients

  • clinical course - benign - remission generally occurs within two years from onset
    • 33% of these children have a single seizure, and only 5-10% have more than 10 seizures that may be very frequent sometimes but the outcome is still favourable
    • Lengthy seizures do not appear to result in residual deficits or have adverse prognostic significance
    • about 20% of children with this syndrome may develop other types of infrequent, usually rolandic seizures, but these also remit before the age of 16 years

  • management
    • first-line treatment in children and young people with benign epilepsy with centrotemporal spikes, Panayiotopoulos syndrome or late-onset childhood occipital epilepsy (Gastaut type) (3)
      • discuss with the child or young person, and their family and/or carers, whether AED treatment for benign epilepsy with centrotemporal spikes, Panayiotopoulos syndrome or late-onset childhood occipital epilepsy (Gastaut type) is indicated
      • carbamazepine or lamotrigine should be offered as first-line treatment to children and young people with benign epilepsy with centrotemporal spikes, Panayiotopoulos syndrome or late-onset childhood occipital epilepsy (Gastaut type)

Reference:

  1. BMJ 2002;324:1228-1229.
  2. Epilepsia. 2003 Jan;44(1):81-8.
  3. NICE (April 2018). Epilepsies: diagnosis and management

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