1/3. Clinical outcomes after corpus callosotomy in patients with bihemispheric malformations of cortical development.OBJECT: epilepsy in patients with bihemispheric malformations of cortical development (MCD) is typically medically intractable. Focal resection has been reported to be ineffective. Corpus callosotomy has been advocated as a treatment option, but the results have been reported only in several case reports. The authors describe a series of 10 patients with bihemispheric MCDs who underwent total corpus callosotomy. methods: The MCDs in these patients included lissencephaly, band heterotopia, perisylvian polymicrogyria, and tuberous sclerosis. Preoperatively all patients suffered disabling drop attacks or intense head drop seizures that caused frequent physical injuries. The follow-up period ranged from 1.4 to 5.8 years (median 3.2 years). Seizure outcome, parental assessment of daily function, and parental satisfaction with outcome were assessed postoperatively. Drop attacks disappeared completely during the entire follow-up period in eight patients and decreased to less than 10% of baseline in one. Other types of seizures were resolved completely in one patient and decreased in seven. overall daily function improved and parents were satisfied with the surgery-related results in all patients except one who experienced a recurrence of drop attacks. There were no signs of significant and persistent neurological deficits in any case. CONCLUSIONS: Results of total corpus callosotomy in patients with bihemispheric MCDs were favorable in most cases. The procedure was particularly effective against drop attacks causing physical injuries and impaired quality of life in these patients.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/3. Epidermal nevus syndrome with hemimegalencephaly: a clinical report of a case with acanthosis nigricans-like nevi on the face and neck, hemimegalencephaly, and hemihypertrophy of the body.A 5-year-old boy with the epidermal nevus syndrome and hemimegalencephaly is reported. He had pigmented nevi on the forehead and neck, and hemihypertrophy of the body from the birth. He developed intractable seizures, mental retardation, and right hemiparesis. His seizure pattern changed from early infantile epileptic encephalopathy to infantile spasms at 2 months of age. Electroencephalograms showed a suppression-burst pattern in the neonatal period, subsequently changing to hypsarrhythmia. Computerized tomography of the brain disclosed slight dilatation of the posterior horn of the lateral ventricle at the age of 2 months. Later, hemimegalencephaly with calcification on the left side of the brain was noted. Histological examination of the pigmented nevus on the neck showed it to be an acanthosis nigricans-like lesion. Clinical differences between tuberous sclerosis and epidermal nevus syndrome with hemimegalencephaly are discussed.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
3/3. Familial osteosclerosis with abnormalities of the nervous system and meninges.A mother and daughter with osteosclerotic dysplasia are described. The daughter had generalized osteosclerosis, flattening of the angles of the mandibles, high-arched palate, mandibular and facial bone hypoplasia, a large sella turcica, and spacious foramen magnum, platybasia, basilar impression, widened spinal cord with enlarged intervertebral foramina, and scalloping of the posterior surfaces of the vertebral bodies. Radiographic contrast studies and operative intervention revealed multiple thoracic and lumbar meningoceles and an "empty" sella, as well as evidence of maldevelopment of the spinal cord, cerebellum, and cerebral cortex. Many of these skeletal features were noted to a lesser degree in the asymptomatic mother.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |