ABSTRACT
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.
Subject(s)
Baclofen/administration & dosage , Infusion Pumps, Implantable , Paraplegia/drug therapy , Quadriplegia/drug therapy , Dose-Response Relationship, Drug , Humans , Motor Neurons/drug effects , Muscle Spasticity/drug therapy , Neurologic ExaminationSubject(s)
Congresses as Topic , Cultural Characteristics , Culture , Verbal Behavior , Humans , LanguageABSTRACT
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.
Subject(s)
AMP Deaminase/deficiency , Neuromuscular Diseases/enzymology , Nucleotide Deaminases/deficiency , Adult , Ammonia/blood , Biopsy , Electromyography , Exercise Test , Humans , Lactates/blood , Lactic Acid , Male , Middle Aged , Muscles/enzymology , Muscles/pathology , Neuromuscular Diseases/pathology , Sarcoidosis/enzymologySubject(s)
Absenteeism , Disability Evaluation , Disabled Persons , Morbidity , Germany, West , HumansABSTRACT
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.
Subject(s)
Hereditary Sensory and Autonomic Neuropathies/complications , Vitamin E Deficiency/complications , Child , Hereditary Sensory and Autonomic Neuropathies/pathology , Humans , Leg/innervation , Male , Muscles/pathology , Muscles/ultrastructure , Sural Nerve/pathology , Sural Nerve/ultrastructure , Thigh/innervation , Vitamin E Deficiency/pathologySubject(s)
Confidentiality , Ethics, Medical , Adolescent , Adult , Child , Child Abuse , Germany, West , Humans , Insurance, Health , Occupational Diseases/diagnosisABSTRACT
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.
Subject(s)
Tomography, X-Ray Computed/methods , Tuberous Sclerosis/diagnostic imaging , Adolescent , Calcinosis/diagnostic imaging , Cerebral Ventriculography , Child , Child, Preschool , Female , Humans , Male , Time Factors , Tuberous Sclerosis/geneticsABSTRACT
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.
