[Hereditary neuropathy with liability to pressure palsies. A contribution to the differential diagnosis of multiplex mononeuropathy].
Hereditary neuropathy with liability to pressure palsies could be diagnosed in two families. This little-known, dominantly inherited disorder is clinically characterised by recurring, spontaneously regressive palsies of peripheral nerves, mostly after minimal mechanical compression of the nerve concerned. It can be clearly differentiated from mononeuropathies of different pathogenesis even in clinically not involved nerves, by means of pathological neurographic findings and by the detection of pathognomonic myelinic thickenings in nerve biopsies. Prognosis is favourable if recurrences are avoided. This means that counselling of the patients and their families is of prophylactic significance.
D000328:Adult; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D010243:Paralysis / Q000235:genetics* / Q000473:pathology; D010375:Pedigree; D010523:Peripheral Nervous System Diseases / Q000235:genetics* / Q000473:pathology; D011312:Pressure; D013497:Sural Nerve / Q000473:pathology
Hereditäre Neuropathie mit Neigung zu Druckläsionen. Ein Beitrag zur Differentialdiagnose der Mononeuropathia multiplex.