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Mutations in the X-linked gene doublecortin, which encodes a protein with no dear structural homologues, are found in pedigrees in which affected females show "double cortex" syndrome (DC; also known as subcortical band heterotopia or laminar heterotopia) and affected males show X-linked lissencephaly. Mutations in doublecortin also cause sporadic DC in(More)
OBJECTIVE To develop a multivariate risk factor model for predicting postoperative verbal memory decline in an individual patient following dominant or nondominant anterior temporal lobectomy (ATL). METHODS The authors studied 132 consecutive ATL patients who 1). were older than 16 years at surgery, 2). had estimated preoperative Full Scale IQ score of(More)
Mutations in the X-linked doublecortin gene appear in many sporadic cases of double cortex (DC; also known as subcortical band heterotopia), a neuronal migration disorder causing epilepsy and mental retardation. The purpose of this study was to examine why a significant percentage of sporadic DC patients had been found not to harbor doublecortin mutations(More)
Cerebral cavernous malformations (CCMs) are vascular abnormalities of the brain that can result in a variety of neurological disabilities, including hemorrhagic stroke and seizures. Mutations in the gene KRIT1 are responsible for CCM1, mutations in the gene MGC4607 are responsible for CCM2, and mutations in the gene PDCD10 are responsible for CCM3. DNA(More)
OBJECTIVE There are concerns that generic and brand antiepileptic drugs (AEDs) may not be therapeutically equivalent. This study investigated how generic AED substitution may have negative consequences. METHODS Sixty-nine of 150 physicians who participated in a large survey on generic AED substitution completed a case review form regarding a patient who(More)
Certain behaviors that occur during a complex partial seizure (CPS) are useful in lateralizing the side of seizure onset. In 5 (5.3%) of 94 consecutive patients with partial epilepsy, we observed ictal unilateral arm and hand paresis during 27 of 34 CPS. In all these seizures, this behavior occurred contralateral to an epileptogenic temporal lobe, as(More)
While disorders of neuronal migration are associated with as much as 25% of recurrent childhood seizures, few of the genes required to establish neuronal position in cerebral cortex are known. Subcortical band heterotopia (SBH) and lissencephaly (LIS), two distinct neuronal migration disorders producing epilepsy and variable cognitive impairment, can be(More)
OBJECT A gene contributing to the autosomal-dominant cerebral cavernous malformation (CCM) phenotype, KRIT1 (an acronym for Krev Interaction Trapped 1), has been identified through linkage analysis and mutation screening. The authors collected blood samples from 68 patients with familial CCM and 138 patients with apparently sporadic CCM as well as from(More)
OBJECTIVE To compare the sensitivity of standard magnetic resonance imaging (MRI) scans done outside an epilepsy center with that of special protocol MRI scans done at an epilepsy center in delineating relevant lesions of the temporal lobe. SUBJECTS Eighty-four consecutive patients who had temporal lobe resections for refractory temporal lobe epilepsy(More)
We report a family with band heterotopia in a mother and daughter and lissencephaly in a son (X-linked inheritance pattern). Postmortem examination of the boy revealed classical lissencephaly and, among other findings, simplified and discontinuous inferior olives without inferior olivary heterotopia. The absence of inferior olivary heterotopia may(More)