1/3. Scleroderma cerebritis, an unusual manifestation of progressive systemic sclerosis.A 42-year-old female with scleroderma experienced two exacerbations in which behavioral changes were the main clinical features. On both occasions she presented with paranoid delusions, perceptual aberrations, and disorientation. After treatment with corticosteroids, the patient's mental status returned to normal, and her electroencephalogram showed an increase in alpha wave frequency, which is consistent with a resolving delirium. Unlike systemic lupus erythematosus, scleroderma rarely involves the central nervous system. This case illustrates an unusual manifestation of progressive systemic sclerosis, primary cerebral involvement which presented as an acute organic brain syndrome. connective tissue diseases, notably systemic lupus erythematosus, often present neuropsychiatric symptoms. Despite the fact that there appears to be a clinical and pathological continuum among the connective tissue diseases, an organic psychosis rarely occurs in progressive systemic sclerosis (scleroderma. Described here is a patient with scleroderma in whom behavioral abnormalities were the main features of two exacerbations of the disease.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/3. Persistent neurological deficit precipitated by hot bath test in multiple sclerosis.For a half century, the hot bath test has been used as a "diagnostic test" in multiple sclerosis. The appearance of new neurological signs or aggravation of preexisting signs generally is transient, with resolution on return of body temperature to normal. We have observed four patients, however, with considerable and prolonged neurological debilitation after hot bath testing. We suggest caution in the application of such testing.- - - - - - - - - - ranking = 0.83333333333333keywords = sclerosis (Clic here for more details about this article) |
3/3. Late onset spinal muscle atrophy--a sex linked variant of Kugelberg-Welander.A syndrome of progressive muscular atrophy in reported in male members of a Caucasian family. Two affected members were examined in detail, one with post mortem. Fasciculations and atrophy of tongue as well as of the proximal limb muscles were observed, and there was profound weakness of the proximal muscles. The EMG and muscle biopsy were consistent with a neurogenic disease. The most pronounced lesion was in the lateral part of the anterior horn, with minimal involvement of the ventral portion of the anterior horns and sparing of the neurons of Clarke's column. Two earlier families with possible Kugelberg-Welander syndrome have been reported in which a sex-linked form also seems probable, and the varied inheritance pattern and uncertain pathological correlations suggest that the Kugelberg-Welander and familial amyotrophic lateral sclerosis both represent heterogenous neurological disorders.- - - - - - - - - - ranking = 0.16666666666667keywords = sclerosis (Clic here for more details about this article) |