What is the mortality and morbidity associated with Charcot-Marie-Tooth (CMT) disease?

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

Answer

Life expectancy is normal in most patients with CMT. The degree of disability varies according to the CMT subtype, and it is unpredictable between and within families; even identical twins may be differently affected.

Typically, HNPP patients have a good quality of life between episodes of nerve damage. About 10% of patients have an incomplete recovery from episodes of nerve palsy. Cases of respiratory failure have been reported.

Rarely, patients with CMT may have laryngeal dysfunction with aspiration and voice problems.

Patients with DSS are often disabled in early childhood. CHN can lead to early death.

Whenever a patient is unexpectedly severely affected, a search for additional neurologic diseases is warranted.

Per definition, CMT is a disease of the peripheral nervous system (PNS), but CNS features can be found.


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