| خلاصه مقاله | Severe brain disorders in which the epileptic electrical discharges during brain maturation may contribute to progressive psychomotor dysfunction
-Common characteristics:
-Related to early age
-Aggressive EEG paroxysmal activity
-Multiple and often drug-resistant seizure types
-Cognitive, behavioral, and neurological deficits
The most common epileptic syndrome
in infancy
-Charactenstics (one element may be missing):
Epileptic (infantile) spasms: in all patients
Hypsarrhythmia in EEG
Arrest or regression of psychomotor development
Onset 3-12 months (2 wks to 18 mo), peak at 5 mo
-Incidence, 3-5 per 10,000 live births 1.3% of all epilepsies
-Clusters of sudden, brief (0.2-2 sec), bilateral (symmetrical > asymmetrical) tonic contractions of the axial and limb muscles. Mixed > flexor (salaam spasms) > extensor
-Slower than myoclonus and faster than tonic seizures
-Eye deviation or nystagmoid movements occur in 60% and may be an isolated ictal symptom
-Often followed by motionlessness (akinesia) and diminished responsiveness (arrest) for up to 90 sec
-A cry or laughter often follows the end of the attacks
Symptomatic (Genetic, structural-metabolic): 70%. Pre-, peri- and postnatal insuits, maiformations, neurocutaneous disorders (TS), Down syndrome. infections and rarely inborn errors of metabolism. Poor prognosis
Probably symptomatic or Cryptogenic (Unknown) 25%. The main inclusion criteria is psychomotor deficits prior to development of seizures in infants with normal imaging and metabolic and other
work-ups
Idiopathic 5%. Normal pre-morbid development and possible hereditary predisposition (family history of seizures). May have a better prognosis |