CMT/Bulbar Palsy

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----Date: Saturday, May 06, 2000 12:37 AM

Subject: CMT/Bulbar Palsy

Title

Varying occurrence of vocal cord paralysis in a family with autosomal

dominant hereditary motor and sensory

neuropathy.

Author

Donaghy M; Kennett R

Address

University Department of Clinical Neurology, Radcliffe Infirmary, Oxford,

UK.

Source

J Neurol, 246(7):552-5 1999 Jul

Abstract

A white British family with the axonal form of hereditary motor and sensory

neuropathy (HMSN, type II) contained one

member who developed a recurrent laryngeal nerve palsy at the age of 41

years, in addition to 4 years of symptomatic

polyneuropathy and an abducens nerve palsy. Neither of the other family

members (the mother and sister) with

electrophysiologically confirmed polyneuropathy had any neuropathic

symptoms in the limbs or laryngeal or respiratory

muscle involvement. An autosomal dominant pattern of inheritance is likely.

This is a second report of this rare form of

HMSN (type IIC) in which there is associated laryngeal or respiratory

muscle weakness. This family differs from the two

previously reported pedigrees in which laryngeal or diaphragm weakness had

commenced within the first two decades.

The discovery of asymptomatic family members attests to the diagnostic

value of clinical and electrophysiological study of

first-degree relatives when laryngeal or bulbar symptoms develop in the

context of chronic axonal polyneuropathy.

HMSN type IIC should be distinguished from the more common forms of HMSN -

type IIA, in which axonal

polyneuropathy is restricted to the limbs, and type IIB, which is of early

onset and associated with foot ulceration.

This can be read at,

http://www.infotrieve.com/healthworld/std_format.cgi?med96-97+424913+(bulbar+pal\

sy) >Becky M.