Annals of neurology
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Annals of neurology · Nov 1995
Case ReportsFamilial hemiplegic migraine and autosomal dominant arteriopathy with leukoencephalopathy (CADASIL).
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a recently described familial cerebrovascular disorder shown to map to chromosome 19q12. Familial hemiplegic migraine has also been shown in some families to map close to the CADASIL locus. The fully developed CADASIL phenotype consists of recurrent strokes developing in the fourth decade, progressing to a pseudobulbar palsy, spastic quadriparesis, and subcortical dementia. ⋯ This disorder has been shown by linkage analysis to map to the CADASIL locus at chromosome 19. The phenotype at presentation of CADASIL in this family was variable and age related and included familial hemiplegic migraine, migraine with and without aura, transient ischemic attacks, strokes, and spinal cord infarction. This family study increases our understanding of the spectrum of clinical manifestations of this underrecognized familial cerebrovascular disorder.
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Annals of neurology · Nov 1995
Comparative StudySignificance of spikes recorded on electrocorticography in nonlesional medial temporal lobe epilepsy.
Whether spikes recorded by intraoperative electrocorticography imply active epileptogenicity has not been adequately addressed. We performed preresection and postresection electrocorticography on 47 patients with nonlesional medial temporal lobe epilepsy who were undergoing surgery for the treatment of medically refractory epilepsy. A standard anteromedial temporal lobectomy was performed on all patients, with no additional resection, regardless of electrocorticographic findings. ⋯ The majority of these had a low-frequency spike discharge rate and were localized to the margin of resection. We found no correlation between residual spikes on preresection and postresection electrocorticography and outcome. These findings do not support the role of intraoperative electrocorticography in guiding mesial temporal lobe resection.