We describe a family in which cutaneous malignant melanoma or cerebral astrocytoma, or both, developed in eight members over three generations. Other malignancies also occurred with a lesser frequency. In two patients with both malignant melanoma and astrocytoma, the brain tumor followed the diagnosis of melanoma by a period of 2 and 10 years and was the primary cause of morbidity and mortality. The findings in this family may represent a newly described genetic disorder.
To determine the frequency and pattern of neurological complications of T-cell lymphoma (TCL), we retrospectively reviewed the medical records of 316 patients with TCL diagnosed between January 1984 and May 1991. Disease entities not included in this study were lymphoblastic lymphoma, primary central nervous system lymphoma, CD30-positive anaplastic large cell lymphoma, and lymphomas secondary to human immunodeficiency virus or human T-cell lymphotropic virus type I. Cases were classified as having direct complications (parenchymal, leptomeningeal, epidural, or peripheral) or indirect complications (paraneoplastic, disease related, or treatment related). Preexisting neurological conditions were excluded. The overall rate of neurological complications was 7.9%. The frequency of neurological complications in peripheral TCL and cutaneous TCL was 17% and 3%, respectively, with at least half of the neurological complications in both conditions due to direct involvement of the nervous system. Direct neurological complications of TCL were primarily due to leptomeningeal and parenchymal involvement. There were no cases of epidural spinal cord disease.
To assess the frequency and significance of immunoglobulin G antibody to double-stranded DNA in chronic active hepatitis, 99 patients with severe disease were tested for the antibody by an enzyme-linked immunosorbent assay of established sensitivity and specificity. Antibody was detected in 56 patients (57%) and occurred with similar frequency in patients with autoimmune (64%), idiopathic (46%) and type B (43%) disease. The mean serum level of antibody was higher in autoimmune disease but it was not significantly different from that in the other diagnostic categories. Patients with and without the antibody could not be distinguished by clinical, laboratory or histologic findings. Responses to corticosteroid therapy and mortality were unrelated to antibody status. In five patients, the antibody disappeared after corticosteroid treatment and induction of histologic remission. We conclude that immunoglobulin G antibody to double-stranded DNA is frequently present in patients with severe chronic active hepatitis. Patients with autoimmune disease may have higher antibody levels than others, but the presence of antibody does not connote a disease of specific etiology, unique presentation or different behavior. The prevalence of the finding and its disappearance after corticosteroid therapy suggest that it is a nonspecific manifestation of inflammatory activity.
Background and Purpose Although repetitive involuntary movements are a well-recognized manifestation of carotid occlusive disease, similar movements have not been reported with a lacunar infarction outside of the basal ganglia or subthalamic nucleus. We describe a man with tonic spasms associated with a lacunar infarction in the right ventral pons.Case Description Involuntary tonic spasms of a paretic limb developed acutely in a 69-year-old hypertensive man with a clinical presentation of pure motor hemiparesis. Magnetic resonance imaging demonstrated a lacunar infarction of the
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