A progressive and hereditary degeneration of peripheral nerves and roots that leads to wasting of the
distal muscles of the extremities. CMT involves feet and legs initially (peroneal nerve paralysis), and hands
and arms later. It rarely involves muscles more central than the elbows, or above the mid-third of the thighs.
The first sign of the disease is often pes cavus (an exaggeration of the normal foot arch due to
involvement of the extensor and everter muscles of the foot). The foot becomes plantarflexed, inverted, and
adducted, producing a typical equinovarus deformity.
Calluses and perforating ulcers are also common. Later, all muscles below the middle third of the thighs
may atrophy, causing the patient’s lower extremities to resemble a “stork leg.” Deficits may involve touch and pain (usually in feet earlier than hands), as well as proprioception. Deep tendon reflexes of the involved
limbs are usually absent.
distal muscles of the extremities. CMT involves feet and legs initially (peroneal nerve paralysis), and hands
and arms later. It rarely involves muscles more central than the elbows, or above the mid-third of the thighs.
The first sign of the disease is often pes cavus (an exaggeration of the normal foot arch due to
involvement of the extensor and everter muscles of the foot). The foot becomes plantarflexed, inverted, and
adducted, producing a typical equinovarus deformity.
Calluses and perforating ulcers are also common. Later, all muscles below the middle third of the thighs
may atrophy, causing the patient’s lower extremities to resemble a “stork leg.” Deficits may involve touch and pain (usually in feet earlier than hands), as well as proprioception. Deep tendon reflexes of the involved
limbs are usually absent.