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Reprasentative Enzymaktivitaten des energieliefernden Stoffwechsels wurden in Muskelproben aus dem M. biceps brachii, M. deltoideus und M. tibialis anterior bei Patienten mit umschriebenen Lasionen peripherer Nerven gemessen. Als Normalkollektiv dienten die gleichzeitig gemessenen Enzymaktivitaten in den normalen Muskeln der Gegenseite. 5 Patienten litten an einer Armplexusschadigung, 7 Patienten hatten sich eine Axillarisparalyse und 8 Patienten eine Peronaeusparalyse zugezogen. In allen denervierten Muskeln waren elektromyographisch keine Zeichen einer Reinnervation nachweisbar.

A child is reported with Behcet's disease who presented with skin and joint manifestations, oral ulcers, brainstem syndrome and neurogenic muscular atrophy. The neurogenic muscular atrophy was confirmed by electrophysiologic, histologic and histochemical studies. Electron microscopy of muscle showed a vasculopathy. The possible etiology of the muscle lesions is discussed. The relevant literature on muscle and peripheral nerve involvement in Behcet's disease is reviewed. It is proposed that neuromyopathy be added to the neurological manifestations of Behcet's disease. To our knowledge, this is the first case of neurogenic atrophy reported in Behcet's disease.

A man of 68, who died from a second heart stroke had neurogenic atrophy of the legs. The anterior tibial muscle showed abundant target and targetoid fibres. Half a year prior to death he had increasing weakness and wasting of the legs. The morphometric evaluation of the target and targetoid fibres showed a considerable independence of the plane size of the single zones from the total gauge of the fibres on cross section. Only a small part of the variation in size of zone 2 (4%) depended on the size of the target, indicating that zone 2 cannot just be a passive product of a shrinkage and disappearance of the target, but that there must be an active process, which leads to the change in zone 2. The regressive development of the centrally demarcated fibrillar bundle (target) is regarded as secondary degeneration following interruption of the nutritional supply.

The study of some muscular biopsies has shown modifications of the rough endoplasmic reticulum with dilatations of cisternae which contain heaps of an osmiophilic, non structured material. These vesicles, the “flecked vesicles”, are localized in atrophic muscular fibers and are particularly frequent in neurogenic atrophies, permitting so a diagnosis orientation. The authors discuss a possible relation with disturbances of the turn-over of proteins and more specially of acetylcholine-esterase.

Since the age of 4, a young man suffered from a slowly progressive neurogenic atrophy of muscles of the distal type. At the age of 24 he unexpectedly died in alcoholic intoxication. Anatomical findings: No damage of the large nerve-cells in the anterior horn. Generalised neuroaxonal dystrophy in the central and peripheral nervous system, which differs from all the forms of naD known up to now by their combination with uncountable Rosenthal fibers in the brainstem and the periventricular and perivascular borderregions. We suspect a dysmetabolical process concerning the neuronal as well as the glial metabolism, which in spite of tendency of generalisation has clinically (hitherto) only shown up its secondary effect of neurogenic atrophy of the distal muscles.