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Mandatory seizures

Myoclonic-atonic seizures are mandatory seizure types for this syndrome. Atonic and myoclonic seizures are frequent. Myoclonic-atonic status epilepticus is common.

May have
  • Febrile seizures
  • Absence seizures - seen in half of patients, typically with myoclonic jerks, facial myoclonia and atonia (not just loss of awareness)
  • Atypical absence seizures
  • Tonic seizures are rare - the presence of tonic seizures is linked to a higher frequency of cognitive impairment
  • Generalized tonic-clonic seizures
  • Non convulsive status epilepticus is common, lasting for hours to days and manifests as cognitive impairment with repetitive myoclonic (affecting face, eyelids and limbs) and atonic seizures.
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