[Continuous intrathecal baclofen therapy using an implantable pump system in severe spasticity]. / Kontinuierliche intrathecale Baclofen-Therapie mittels implantierbarer Pumpensysteme bei schwerer Spastik.

Spasticity is an expression of a damage of the motor neurone associated with velocity dependent increase of the muscle tone. Since 1986 21 patients with severe spasticity mainly in multiple sclerosis were treated with an implantable pump system to administrate continuously Baclofen intrathecally. Even with a very small dosage of Baclofen patients who were previously treated until intoxication without success there was a favorable reduction in spasticity. Therefore we propose to perform this procedure before using surgical methods or stimulating methods.

Myoadenylate deaminase deficiency.

This report concerns two unrelated males; one had sarcoidosis, sarcoid myopathy and muscle weakness, and the other had exercise-induced weakness and myalgia. Both patients had a lack of ammonia rise in their serum after an ischemic work test, minimal histochemical activity of myoadenylate deaminase in repeated muscle biopsies, and less than 5% of normal biochemical activity of myoadenylate deaminase in their skeletal muscles. These three criteria establish primary myoadenylate deaminase deficiency as a separate primary metabolic muscle disease which merits differential diagnostic consideration when patients complain of muscle weakness and cramps.

Neuromyopathy and vitamin E deficiency in man.

A 12-year-old boy, born of a consanguineous marriage, had ataxia, sensory neuropathy, generalized muscle hypotrophy and a lower serum vitamin E level. Two of his relatives had died of a clinically similar disorder in their late adolescence. Morphologically, his striated muscle fibers and Schwann cells of his sural nerve contained numerous autofluorescent acid phosphatase-positive lipopigments which, by electron microscopy, consisted of a finely granular matrix surrounded by a trilaminar membrane. These lysosomal lipopigments were similar to those observed in muscle fibers of a patient afflicted with abeta-lipoproteinemia. They probably represent the morphological sequelae of long-standing vitamin E deficiency in this child, the extract origin of which has not been fully elucidated.

Cranial computertomography in children with tuberous sclerosis.

20 children with tuberous sclerosis were examined by computertomography (CCT). The test is useful to detect cerebral involvement with great certainty. The method is especially helpful in the early diagnosis of the disease. This offers the possibility of giving an early genetic advice. Characteristic CCT scan abnormalities in tuberous sclerosis are multiple densities in the walls and the roof of lateral ventricles. This specific finding can already be seen prior to the occurrence of calcifications in standard roentgenograms.

[The nosological position and histogenic origin of the so-called cerebellar astrocytomas (author's transl)]. / Zur nosologischen Stellung und histologischen Herleitung des sogenannten Kleinhirnastrozytoms

The unusual status of the cerebellar astrocytoma is based upon clinical and histological evidence. "Bergstrand Tumour" is proposed as a new name for it. A new concept of its origin is put forward, namely that it is derived from the "tanycytes" (Horstmann) of the periventricular structures.