CMT (Subscribe)  

Hereditary Motor Sensory Neuropathies I-III
Epidemiology 1: 3000
Definition
Group of inherited peripheral sensory & motor neuropathies
- Includes Charcot MarieTooth D

Classification
Hypertrophic vs Neuronal HMSN
I Hypertrophic CMT
II Neuronal CMT
III Infantile Hypertrophic Neuropathy - Dejerine-Sottas
IV Refsum's disease

Charcot-Marie-Tooth disease (CMT) is the most common inherited neurologic disorder. CMT is characterized by inherited neuropathies without known metabolic derangements.
Synonyms and related keywords: CMT, CMT 1, CMT 2, hereditary motor and sensory neuropathy, HMSN, peroneal muscular atrophy, PMA, peroneal progressive muscular atrophy, peroneal muscular atrophy with thickened nerves, Hoffman disease,...

This is an inherited sensorineural peripheral polyneuropathy first described independently by Charcot and Marie in France and Tooth in England in 1886.
CMT1 or hereditary motor and sensory neuropathy type 1, HMSNI. This is the most common form. Onset usually by age 10 with:
* Weakness
...

Objectives
1. Define hereditary motor sensory neuropathy and list several types
2. Describe presenting symptoms and signs of type 1 Charcot-Marie-Tooth disease
3. Discuss orthopaedic problems associated with HMSN, and an approach to management

muscle weakness primarily affects peroneus brevis muscle and the tibialis anterior, w/ relative sparing of the peroneus longus;
- results in planatar flexion of first ray, resulting in hindfoot varus ...