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Cognitive and Behavioral Correlates of Tuberous Sclerosis
Complex
The Journal of Child Neurology 2004 Nov;19(11):847-52.
Zaroff CM, Devinsky O, Miles D, Barr WB
Few studies have examined the clinical features of neocortical temporal
lobe epilepsy (NTLE) in carefully selected patients. We reviewed records
from 21 patients with NTLE, defined by intracranial electroencephalogram
(EEG), who have been seizure free for 1 year or more following temporal
lobectomy. The mean age of onset at the time of first seizure was
14 years (range, 1-41 years). Febrile seizures were reported in only
2 patients (9.5%). In contrast to prior mesial temporal lobe epilepsy
(MTLE) studies, seizure-free intervals between the initial cerebral
insult or first seizure and habitual seizures were uncommon. Possible
or known risk factors for epilepsy were reported in 13 of 21 patients
(62%). Fifteen (71%) patients reported auras, with experiential phenomena
being the most common type. Magnetic resonance imaging was normal
or nonspecific in 15 patients, revealed mild hippocampal atrophy in
2, tumors in 2, and heterotopic gray matter and hippocampal atrophy
in 1, and cortical dysgenesis in 1. Neuropsychological testing showed
deficits consistent with the seizure focus in 13 patients (62%), and
Wada test showed ipsilateral memory deficits in 10 (48%). The most
common behavioral manifestation was a motionless stare at ictal onset
(48%). In contrast to prior studies of MTLE, only 1 NTLE patient had
frequent independent, contralateral temporal lobe epileptiform spikes
on scalp EEG.
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