CMT: Type II



- See: CMT Menu:

- Discussion:
    - autosomal dominant inheritance;
    - type II is the neuronal form of CMT disease and is seen infrequently;
    - onset is delayed until the second or third decade of life;
    - sensory and motor nerve-conduction times that are only mildly abnormal, and a variable inheritance pattern;

- Clinical Manifestations:
    - characterized by persistently normal reflexes;
    - there is more profound distal lower extremity weakness than encountered in Type I;
    - characteristic stork leg appearance frequently is seen caused by atrophy of the distal third or the quadriceps and hamstrings;
    - flail foot develops a calcaneovarus (? valgus) deformity;
    - fixed bony deformity is better managed by a combination of calcaneal and metatarsal osteotomies and may require the use of AFOs;
           - posterior displacement calcaneal osteotomy is effective in correcting calcaneocavus deformity of the type II neuropathy



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Friday, September 9, 2011 9:54 am