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CMT Type II



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- 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