What is the prognosis of Charcot-Marie-Tooth (CMT) disease?

Updated: Feb 19, 2019
  • Author: Francisco de Assis Aquino Gondim, MD, PhD, MSc, FAAN; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Answer

Answer

In most inherited neuropathies, a person's life span is not altered. Disability is highly variable and difficult to predict in young individuals, even among siblings. In general, CMT is slowly progressive. If progression accelerates, other causes, such as acquired neuropathies or other inherited neuromuscular conditions, should be sought. Often, males are affected more than females, possibly because of a greater likelihood of nerve trauma. Most patients need some kind of ankle support at some time in their life. Weakness rarely spreads to proximal leg or arm muscles. Patients with DSS are more likely to lose their ability to ambulate independently. One study reported significant disability in 44% of patients with CMT1A and depression in 18% of patients with CMT1A. In addition, 36% of patients opted against childbearing.

In general, patients with HNPP have excellent quality of life. About 10% of patients make an incomplete recovery from episodes of nerve palsy. The above-mentioned fulminant forms are extremely rare.


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