Some problems of the systemic degeneration and atrophies.
Report on a family of three brothers in which the prevailing features of the clinical findings were those of Friedreich's "heredo-tabes". Histologically besides the degeneration of the peripheral proprioceptive neurone and of the ventral spinocerebellar tract, several other systems proved affected. For similar categories of cases, it does not seem useful to introduce a complicated terminology, and the a potiori denomination of the classical syndrome should be retained. Yet, both the clinical and the pathological examinations have to seek carefully in every individual case for the presence of pathological features which do not belong to the basic syndrome, and note them in the characterisation of the case. Although in systemic degeneration it is the most distal part of the neuronal unit where degeneration starts, the source of it lies in the originating nerve cell. For the pseudosystem-degenerations in the sense of Seitelberger this holds true only exceptionally, if at all.
D002490:Central Nervous System / Q000473:pathology*; D002493:Central Nervous System Diseases / Q000235:genetics*; D005621:Friedreich Ataxia / Q000235:genetics*; D006801:Humans; D008297:Male