Morphological and biochemical studies were performed on a brain biopsy from a patient with typical Lafora disease. Qualitative morphological investigation of the cortex showed that the Lafora bodies were most abundant in layers III and V of the cerebral cortex. They were exclusively located in the neurons and their processes. Quantitative morphological investigation of the cerebral cortex revealed abnormalities of the pyramidal cells of layers III and V. The neurons were often slightly atrophic. There was a reduction in the number of dendrites, in maximal dendritic length, and in the number of spines on the apical dendrites and on the side branches. Because of the biochemical study of the pyruvate metabolism of the gray and white matter of the cortex did not show abnormalities, a mitochondrial dysfunction is not likely.

Adolescent↗

PubMedDate not supplied

Myelopathy due to cervical spine rheumatoid arthritis. A case report.

A 62-year-old woman with cervical myelopathy due to cervical spine rheumatoid arthritis is presented. Transoral decompression of the spinal cord, followed by posterior spine fusion in the same session was performed with good results. Unfortunately she died 3 months later due to cardiovascular disease. Clinical, radiological, surgical and autopsy data are presented.

Arthritis, Rheumatoid↗

PubMedDate not supplied

Two cases of progressive multifocal leuko-encephalopathy with unusual aspects.

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Adult↗

PubMedDate not supplied

Polyglucosan bodies in brain tissue: a systematic study.

The relation between age, sex and presence of polyglucosan bodies in the brain has been systematically studied in 64 patients who did not suffer from neurological brain disorders and in 2 cases with Lafora's disease. In the normal brain the number of polyglucosan bodies is related to increasing age. Under the age of 40, polyglucosan bodies can be found sporadically in cases without a neurological disorder, but in the cases of Lafora's disease their number is uncomparatively higher. A sex relation has not been found.

Adolescent↗