MYOCLONIC ENCEPHALOPATHY IN NON-PROGRESSIVE DISORDERS

Mandatory seizures

Recurrent periods of myoclonic status epilepticus (subcontinuous myoclonic seizures interspersed with absence or myoclonic absence seizures) occur, with each episode lasting days-weeks. Myoclonic jerks involve the eyelids, face and limbs and are erratic (and may be subtle in young infants). The child may have reduced responsiveness, drooling, reduced mobility and inco-ordination at these times. Atonic seizures typically co-exist resulting in head nodding, or falls.

May have

• Focal motor seizures
• Hemiclonic seizures (rare, during febrile illness)
• Massive myoclonias
• Generalized tonic-clonic seizures (rare, during febrile illness)