[Hereditary neuropathy with liability to pressure palsies. A contribution to the differential diagnosis of multiplex mononeuropathy]

Abstract

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.