Hereditaty Sensory & Motor Neuropathy
- CMT is the prototype
CMT (HMSN 1)
- Autosomal dominant
- Demylinating disease characterized by peroneal muscle weakness
- Absent DTR
- Slow nerve conduction velocity
- May effect hands
- HMSN 2 – similar but mild
Treatment
Pes cavovarus
- Deformity includes claw toes, plantar flexed 1st metatarsal, adduction & inversion of the metatarsals, midfoot malposition of the cuboid, navicular & metatarsals
- Leads to a very high arch
- Invertors are stronger than everters
- Block test is useful to see if the cavus is flexible
Early
- < 10 years of age – plantar release
- flexible consider transfer of the tibialis anterior but it is often effected as well, other options include closing wedge of the first metatarsal, transfer of the peroneus longus & lateral closing wedge osteotomy
- late – triple arthrodesis
Hip Dysplasia
- 6-8%
- typical acetabular dysplasia, coxa valga & subluxation
- usually early soft tissue release & late bony reallignment
Hands
- later
- 2/3 of patients
- intrinsic weakness
- numbness
