Neuropsychological findings: myoclonic astatic epilepsy (MAE) and Lennox-Gastaut syndrome (LGS).
OBJECTIVE
To identify a specific neuropsychological profile associated with myoclonic astatic epilepsy (MAE) and Lennox-Gastaut syndrome (LGS).


METHODS
Seven patients diagnosed with MAE and four patients diagnosed with LGS were selected from patients referred to our Child Neurology Unit. The patients were assessed both clinically (awake, sleep, Holter EEG, seizures frequency, and semiology) and neuropsychologically (IQ, language, attention, visuospatial and visuomotor abilities, and behavior). One representative case of each syndrome is presented here.


RESULTS
The clinical picture of the MAE patient resembled that of an MAE condition associated with transitory epileptic encephalopathy. The neuropsychological findings suggest that electroclinical anomalies can temporarily affect cognitive and behavioral functioning. Early effective antiepileptic drug (AED) treatment was found to improve cognitive outcome. In contrast, LGS was associated with mental retardation, which persisted after seizure control.


CONCLUSIONS
At present, it remains difficult to delineate a precise neuropsychological profile associated with MAE and LGS. The cognitive outcome of MAE is variable and depends on the clinical pattern. With regard to LGS, the hypothesis of a genetic predisposition underlying both the epilepsy and the mental retardation is still valid. Alternatively, exposure to subclinical electrophysiological anomalies during a critical period of cerebral development may be responsible for the mental retardation. At the time the clinical manifestations appear, drug treatment, even if effective, would have only limited impact on cognitive outcome. However, early multidisciplinary intervention may help to improve behavior and communicative abilities, enhancing the quality of life of these children and their families.
Filippini
Melissa
M
Child Neurology Unit, Epilepsy Center, Neuropsychological Laboratory for Children with Epilepsy, Maggiore "C. A. Pizzardi" Hospital, Bologna, Italy. melissa.filippini@libero.it
Boni
Antonella
A
Dazzani
Gloria
G
Guerra
Angelo
A
Gobbi
Giuseppe
G
D000927:Anticonvulsants / Q000627:therapeutic use; D002648:Child; D003072:Cognition Disorders / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D015897:Comorbidity; D004569:Electroencephalography / Q000706:statistics & numerical data; D004831:Epilepsies, Myoclonic / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D004827:Epilepsy / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D006801:Humans; D008607:Intellectual Disability / Q000175:diagnosis / Q000453:epidemiology; D008297:Male; D001523:Mental Disorders / Q000175:diagnosis / Q000453:epidemiology; D009483:Neuropsychological Tests / Q000706:statistics & numerical data*; D011379:Prognosis
D000927:Anticonvulsants