Hereditary Motor Sensory Neuropathy

Hereditary Motor Sensory Neuropathy

 

Incidence

1 in 2500

Types

Many but broadly, types 1 and 2
1: Hypertrophic (excess myelination)
AD; age 25; legs moderate; hand changes also
2: Neuronal (nerve atrophy)
AR; age 5-15 boys; stalk legs; fast progress
Sensory loss: patchy, possibly just proprioception loss. When severe: ulcers and Charcot

Muscle atrophy in this order:
Intrinsics
Peroneus brevis
Toe extensors
Tibialis anterior

Intrinsic wasting in the hands