Tuesday, September 25, 2012

Hereditary Neuropathies of the Charcot-Marie-Tooth Disease Type (Includes HNA)




Maybe I gave up on CMT too easily earlier in the process. I had seen earlier that there was a subset of CMT refered to as “Hereditary neuropathy with predisposition to pressure palsy”, but I do not remember seeing that they went on to describe HNA as a subset of that. Maybe it is possible to have both HNA and CMT because it appears here at least that the two are related as described.

Form this website:

Hereditary Neuropathies of the Charcot-Marie-Tooth Disease Type

http://emedicine.medscape.com/article/1173484-overview#aw2aab6b3


“Neuritis with brachial predilection/hereditary neuralgic amyotrophy/hereditary brachial plexus neuropathy
Dreschfeld in 1886 may have been the first to recognize this condition when he described a woman with 3 episodes of painful arm weakness, whose sister had experienced 7 such attacks. Jacob et al in 1961 described in 7 patients of 2 unrelated families 14 similar episodes of recurrent brachial neuritis with incapacitating pain, weakness, wasting, depression of reflexes, and sensory loss. The legs were involved, and arm involvement was severe.[20] In 1973, Guillozet and Mercer described a similar condition in 3 generations of a family.[21] At times, the lower cranial nerves and sympathetic nervous system are involved as well.

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.”

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