Charcot Marie Tooth (CMT)
Charcot Marie Tooth Disease (CMT) disease is a hereditary disorder with progressive peripheral motor neuropathy. This syndrome leads to progressive distal and anterior deformities, especially in the feet and ankles. Predominantly, the distal leg muscles, peroneal and anterior tibial muscles are affected causing progressive weakness with gait disturbances (clumsiness, frequent falls, and tripping over feet).
As Charcot Marie Tooth Disease (CMT) progresses, the deformity of pes cavus worsens and eventually foot drop may occur. Currently there is no cure for Charcot Marie Tooth Disease (CMT). As the feet of the patients with Charcot Marie Tooth Disease (CMT) become more pes cavus, the soft tissue of the plantar surface become abnormally short. The metatarsal heads are lower in relation to the hind foot at the tarsometatarsal joints. This foot deformity becomes rigid and lacks the ability to absorb shock.
Rocker bottom foot does not match the shape of any standard shoes so breakdown of the tissue will occur. Since there is LOPS or loss of protective sensation, activity is generally not curbed and the deformed midfoot will break down with repeated micro-traumas of tissues that are not designed to sustain the repeated stress of steps, such as the heel and forefoot.
The danger is that an open wound is susceptible to bacteria that can lead to infection, dangerous since blood supply may be compromised for healing.
Charcot foot disease can affect the forefoot or rearfoot. Osteomyelitis, arthritis, and soft tissue infections are conditions which can be confused with Charcot Foot Disease.
Unfortunately, due to the lack of knowledge of Charcot Marie Tooth Disease (CMT), patients tend to seek out and are guided toward very cushioned and flexible footwear. This type of footwear actually causes more instability to a cavus foot. These patients, in fact, need footwear that has strong rear foot control with lateral stability.
Custom made shoes are a very good choice for patients with advanced stages of Charcot Marie Tooth Disease (CMT). Cushioned custom orthotics provide the needed shock absorption and support for the pes cavus foot but these need to be contained in controlling footwear, generally high tops. Metatarsal support will unload the metatarsal heads (the balls of the feet). The combination of cushioned orthotic devices and stable and controlling footwear will give the patient optimum support and comfort to be able to walk without pain and reduce callusing, ulcers, ankle sprains and falls. Due to the high medial longitudinal arch, the extensor tendons and ligaments are shortened causing dorsiflexion of the distal phalanges (i.e. claw and hammer toes). Therefore these patients need shoes with deep toe boxes.
In today’s pedorthic facilities, there are many choices of footwear with these features.
Charcot Marie Tooth was named for three physicians who first identified the disease in 1886. There are now known to be at least five genetic causes of Charcot Marie Tooth Disease (CMT) that lead to two distinct types of nerve disease.
- HMSN Type 1 is demyelinating or damage to nerve fiber insulation.
- HMSN Type 2 is neuronal or damage to nerve fibers themselves.
Both types of Charcot Marie Tooth Disease (CMT) expect normal life length, limited disability, and typically very slow progression of the disease. Severity varies greatly.
Around age 20, symptoms may begin as the result of demyelinating types. In the neuronal, symptoms usually develop later in life, from early adult to middle age. Weakness in the ankles and feet is generally more severe in the neuronal type.
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