[A family of hereditary neuropathy with liability to pressure palsies with a proband who developed right and left foot drop successively following the left radial nerve palsy].
A 41-year-old man developed multifocal mononeuropathies manifesting right and left foot drop successively, following the left radial nerve palsy as an initial symptom. Based on the neurological findings and the results of the genetic study of peripheral myelin protein (PMP) 22 gene and the histological study of the sural nerve on biopsy, the diagnosis of hereditary neuropathy with liability to pressure palsies (HNPP) was made. Two asymptomatic carriers were found among his family members based on the genetic study. The diagnosis of HNPP can be definitely established by the genetic study and this disease is relatively rare. In this report it is important to note that there are a few patients who show radial nerve palsy as an initial symptom, that we should carefully study the family members to obtain the prevalence of HNPP because asymptomatic carriers may be present, and that the carriers should be advised to avoid strenuous exercises and works which may produce excessive extension or compression of nerve trunks with the subsequent development of clinical symptoms.
Ohnishi
A
A
Department of Neurology, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.
Yamamoto
T
T
Suenaga
A
A
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005534:Foot Diseases / Q000209:etiology*; D017353:Gene Deletion; D015417:Hereditary Sensory and Motor Neuropathy / Q000150:complications* / Q000235:genetics; D006579:Heterozygote; D006801:Humans; D008297:Male; D008875:Middle Aged; D009185:Myelin Proteins / Q000235:genetics; D010243:Paralysis / Q000209:etiology*; D020425:Radial Neuropathy / Q000209:etiology*; D013497:Sural Nerve / Q000473:pathology
D009185:Myelin Proteins