Neurological complications of peripheral and cutaneous T-cell lymphomas.

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.

Involuntary tonic spasms of a limb due to a brain stem lacunar infarction.

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 ventral pons. There was no evidence for carotid occlusive disease. An electroencephalogram recorded during the movements showed no abnormality. The abnormal movements responded well to treatment with oral diazepam. CONCLUSIONS: A brain stem lacunar infarction may be associated with involuntary tonic limb spasms clinically similar to those reported as paroxysmal symptoms of multiple sclerosis.

A familial syndrome with cutaneous malignant melanoma and cerebral astrocytoma.

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.

Frequency and significance of antibody to double-stranded DNA in chronic active hepatitis.

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.

How lupoid is lupoid hepatitis?

The records of 89 patients with hepatitis B surface antigen, negative severe chronic active hepatitis and antinuclear antibody (ANA) positivity were reviewed to assess the degree of clinical similarity between this disorder and systemic lupus erythematosus (SLE). Although immunologic abnormalities, such as antibodies to native DNA and positive lupus erythematosus cell preparations were common, few patients with ANA positive chronic active hepatitis had clinical features to suggest SLE. The 1971 preliminary criteria and the 1982 revision of the American Rheumatism Association criteria for classification of SLE were 93 and 91% specific, respectively. We conclude that despite laboratory similarities, ANA positive chronic active hepatitis and SLE differ substantially in their clinical presentations.

Do the effects of adventitious deafness include disordered speech?

The popular clinical prediction is that adventitious deafness will result in deterioration of speech sound production; consequently, a recommendation of speech conservation training is thought to be warranted in these cases. This prediction usually follows from a theory that counts feedback (particularly auditory feedback) as important in the maintenance of normal speech. The results of this study, designed to examine the speech of five adventitiously deafened adults matched with a group of normal controls, do not support the prediction of speech deterioration. Even so, judges were able to differentiate deafened from normally hearing speakers. Some possibilities are considered that could account for the identity of the deafened speakers in the absence of clinically significant speech disorder, and clinicians are encouraged to reevaluate their assumptions concerning this population.

The effect of a phase transition on penetration of phospholipid monolayers by melittin and glucagon.

The penetration of melittin and glucagon into phospholipid monolayers was studied by measuring compression isotherms of phospholipids in the absence and presence of various concentrations of protein in the subphase. Differences in molecular area were calculated as a function of protein concentration at constant pressure. Area change as a function of surface pressure at constant protein concentration was also calculated. Melittin showed greater affinity for penetration into phosphatidylglycerol (PG) than into phosphatidylcholine (PC) monolayers. The cutoff pressure for melittin penetration was 45 mN/m with PC and 60 mN/m (extrapolated) with PG. Dipalmitoyl PC and PG monolayers show phase transitions upon compression at 25 degrees C. Both melittin and glucagon showed increased penetration as measured by area change within the region of the phase transition with both lipids. Glucagon showed a cutoff pressure of 25 mN/m for penetration into dimyristoyl PC. The preference of glucagon for interaction with lipid bilayers in the gel phase is discussed with respect to monolayer penetration as a function of surface pressure.