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[A case of Charcot-Marie-Tooth disease type 2 caused by homozygous MME gene mutation].

Authors :
Yamashiro M
Ohnari K
Higuchi Y
Hashiguchi H
Takashima H
Okada K
Source :
Rinsho shinkeigaku = Clinical neurology [Rinsho Shinkeigaku] 2023 Nov 23; Vol. 63 (11), pp. 743-747. Date of Electronic Publication: 2023 Oct 25.
Publication Year :
2023

Abstract

The patient is a 44-year-old man. His parents are consanguineous. He experienced muscle weakness in his toe and distal tingling sensation in his feet at 42 years of age, which gradually progressed. Additionally, a marked cyanotic discoloration of the feet appeared and worsened progressively. Neurological examination revealed loss of tendon reflexes and distal muscle weakness in the lower extremities. Findings from nerve conduction studies indicated axonal polyneuropathy. Upon detection of the MME gene mutation, the patient was diagnosed with autosomal-recessive Charcot-Marie-Tooth disease 2T (ARCMT2T). In this case, cyanosis of the lower extremities possibly was associated with ARCMT2T, and it was suggested to be due to neprilysin deletion linked with the MME mutation. This represents the first documented occurrence of cyanosis as a distinctive feature of CMT with MME mutation.

Details

Language :
Japanese
ISSN :
1882-0654
Volume :
63
Issue :
11
Database :
MEDLINE
Journal :
Rinsho shinkeigaku = Clinical neurology
Publication Type :
Academic Journal
Accession number :
37880116
Full Text :
https://doi.org/10.5692/clinicalneurol.cn-001870