title: Corticosteroid- responsive asymmetric neuropathy with a myelin protein zero gene mutation.
abstract: A patient with hereditary neuropathy presented with asymmetric distal weakness. On nerve biopsy, there was demyelination and onion-bulb formation, and molecular analysis revealed that the patient was heterozygous for an MPZ mutation. The patient improved with corticosteroid treatment.
authors.affiliation: Department of Neurology, Institute of Clinical Medicine, University of Tsukuba, Ibaraki, Japan. masa.wat@igaku.md.tsukuba.ac.jp
authors.forename: M
authors.initials: M
authors.lastname: Watanabe
authors.forename: N
authors.initials: N
authors.lastname: Yamamoto
authors.forename: N
authors.initials: N
authors.lastname: Ohkoshi
authors.forename: H
authors.initials: H
authors.lastname: Nagata
authors.forename: Y
authors.initials: Y
authors.lastname: Kohno
authors.forename: A
authors.initials: A
authors.lastname: Hayashi
authors.forename: A
authors.initials: A
authors.lastname: Tamaoka
authors.forename: S
authors.initials: S
authors.lastname: Shoji
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D001706:Biopsy; D005260:Female; D015417:Hereditary Sensory and Motor Neuropathy / Q000188:drug therapy* / Q000235:genetics* / Q000473:pathology; D006801:Humans; D018993:Myelin P0 Protein / Q000235:genetics*; D017354:Point Mutation; D013497:Sural Nerve / Q000473:pathology
chemical_list: D000305:Adrenal Cortex Hormones; D018993:Myelin P0 Protein