PMID: 8741078May 1, 1996

Sensory involvement in X-linked spino-bulbar muscular atrophy (Kennedy's syndrome): an electrophysiological study

Journal of Neurology
A PoloN Rizzuto

Abstract

Electrophysiological findings were studied in a family with spino-bulbar muscular atrophy (SBMA): the subjects were three male patients aged 58, 38 and 34 years and two female carriers aged 63 and 28 years. Diagnosis was proven at the molecular genetic level. Electromyography in the males showed spontaneous activity and neurogenic reorganization of the motor unit; motor nerve conduction was normal. Sensory action potentials were variably reduced in amplitude, but some were completely normal. Somatosensory evoked potentials, from both the upper and lower limbs, were invariably abnormal because involvement of the central pathways was observed. These findings are in agreement with histological investigations documenting lesions in the posterior columns. Brain-stem acoustic evoked potentials showed an increase in wave I latency. The electrophysiological data provide further evidence of the extent of sensory damage either in the central or the peripheral nervous system in SBMA patients, who otherwise have a constant clinical presentation of progressive motor neuron disease.

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Citations

Sep 7, 2007·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·Tsu-Hsien LaiKwong-Kum Liao
Nov 18, 2015·Journal of Molecular Neuroscience : MN·Paola RusminiAngelo Poletti
May 21, 2004·Internal Medicine Journal·K J Greenland, J D Zajac
Feb 4, 2009·Journal of Neuroimaging : Official Journal of the American Society of Neuroimaging·Sonja SuntrupRainer Dziewas
Sep 24, 2019·Endocrine Reviews·Elisabetta VegetoAngelo Poletti
Oct 28, 2015·Journal of Neurology, Neurosurgery, and Psychiatry·Giorgia QuerinItalian Study Group on Kennedy's disease
Apr 20, 2020·Acta Neuropathologica·Samir R NathAndrew P Lieberman
Aug 15, 2020·Frontiers in Aging Neuroscience·Riccardo CristofaniAngelo Poletti
Aug 26, 2020·Acta neurologica Belgica·Elia G MalekAchraf Makki
Mar 5, 1998·Muscle & Nerve·M J Aminoff, A Eisen
Nov 7, 2019·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Frederick J Arnold, Diane E Merry

Related Concepts

Carrier of Disorder
Surface Electromyography
Somatosensory Evoked Potentials
Genetic Linkage Analysis
Hereditary Motor Neuronopathy
Genealogical Tree
X Chromosome
Auditory Brainstem Responses

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