D E Jacome1. 1. Department of Medicine, Franklin Medical Center, Greenfield, Massachusetts, USA.
Abstract
PURPOSE: Ehlers-Danlos syndrome (EDS) is a complex hereditary connective tissue disorder infrequently reported in association with epilepsy. Seven patients with ages ranging from 28 to 70 years with EDS and epilepsy are described. METHODS: Case review of clinical and diagnostic data. RESULTS: Two patients had occipital horn syndrome (EDS type IX) and partial seizures of probable supplementary motor area origin. Of these two, one had an area of frontal gliosis and was able to abate his seizures by hyperextending his neck; the other had a Dandy Walker malformation and also had pseudoseizures. The third patient of the series had complex partial seizures, pain asymbolia, and basilar artery hypoplasia. The fourth had ictal aphasia, left hemispheric hypotrophy, and distal right arm and left leg atrophy. The fifth patient had focal seizures, a venous parietal angioma, hyperekplexia, nocturnal head oscillations (jactatio capitis nocturna), monoclonal gammopathy-associated neuropathy, and Tourette syndrome. The sixth had affective illness, chronic fatigue, and complex partial seizures with autoscopic phenomena after intracranial bleed. The seventh patient had a previous stroke, peripheral neuropathy, and grand mal seizures. CONCLUSIONS: EDS may be accompanied by congenital or acquired central nervous system disorders and epilepsy. Additional neurologic conditions that are unrelated to EDS may be present.
PURPOSE:Ehlers-Danlos syndrome (EDS) is a complex hereditary connective tissue disorder infrequently reported in association with epilepsy. Seven patients with ages ranging from 28 to 70 years with EDS and epilepsy are described. METHODS: Case review of clinical and diagnostic data. RESULTS: Two patients had occipital horn syndrome (EDS type IX) and partial seizures of probable supplementary motor area origin. Of these two, one had an area of frontal gliosis and was able to abate his seizures by hyperextending his neck; the other had a Dandy Walker malformation and also had pseudoseizures. The third patient of the series had complex partial seizures, pain asymbolia, and basilar artery hypoplasia. The fourth had ictal aphasia, left hemispheric hypotrophy, and distal right arm and left leg atrophy. The fifth patient had focal seizures, a venous parietal angioma, hyperekplexia, nocturnal head oscillations (jactatio capitis nocturna), monoclonal gammopathy-associated neuropathy, and Tourette syndrome. The sixth had affective illness, chronic fatigue, and complex partial seizures with autoscopic phenomena after intracranial bleed. The seventh patient had a previous stroke, peripheral neuropathy, and grand mal seizures. CONCLUSIONS:EDS may be accompanied by congenital or acquired central nervous system disorders and epilepsy. Additional neurologic conditions that are unrelated to EDS may be present.