Hereditary Neuropathies

What are Hereditary Neuropathies?

Hereditary neuropathies are a group of inherited disorders of the peripheral nervous system. Within the group there are 4 subcategories of disorders, including hereditary motor and sensory neuropathy, hereditary sensory neuropathy, hereditary motor neuropathy, and hereditary sensory and autonomic neuropathy. Symptoms of these disorders vary and may include numbness and tingling in the feet and hands, muscle weakness (especially in the distal muscles), scoliosis, thin lower legs, foot deformities, insensitivity to pain, and autonomic symptoms such as impaired sweating, postural hypotension, and skin blotching. Nerve conduction studies, nerve biopsies, and genetic testing help with diagnosis. These disorders may be diagnosed as early as birth or as late as middle or late age. Charcot-Marie-Tooth disorder accounts for the majority of all hereditary neuropathies.

Is there any treatment?

There is no standard course of treatment for hereditary neuropathies. Treatment is symptomatic and supportive and may include orthopaedic surgery and bracing to improve mobility, and measures to protect against stress fractures-particularly in the feet and legs. Genetic counselling is available.

What is the prognosis?

The prognosis for individuals with hereditary neuropathies depends upon the type of neuropathy. Some types progress slower than others.