What is hereditary neuralgic amyotrophy (HNA) in 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

HNA is an autosomal dominant form of recurrent focal neuropathy. Individuals experience episodic brachial plexus neuropathy with weakness, atrophy, and sensory disturbances, preceded almost always by severe pain in the affected arm. Age of onset is in the second and third decades of life and rarely in the first. Recovery is usually complete and begins weeks to months after the onset of symptoms. Recurrent episodes may affect either arm. The right arm is involved more often.

Phenotypic variation may occur, with some patients following the classic relapsing-remitting course and others following a chronic undulating course. Common dysmorphic features include hypotelorism, short stature, cleft palate, unusual skin folds, and creases in the neck or scalp referred to as cutis verticis gyrata.


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