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Multimodal Evoked Potentials of Kennedy's Disease

Published online by Cambridge University Press:  02 December 2014

Tsu-Hsien Lai ,
Bing-Wen Soong ,
Jen-Tse Chen ,
Yen-Yu Chen ,
Kuan-Lin Lai ,
Zin-An Wu  and
Kwong-Kum Liao
Tsu-Hsien Lai
Affiliation:
Department of Neurology, Taipei Veterans General Hospital
Bing-Wen Soong
Affiliation:
Department of Neurology, Taipei Veterans General Hospital
Jen-Tse Chen
Affiliation:
Department of Neurology, Taipei Veterans General Hospital
Yen-Yu Chen
Affiliation:
Department of Neurology, Changhua Christian Hospital, Changhua, Taiwan
Kuan-Lin Lai
Affiliation:
Department of Neurology, Taipei Veterans General Hospital
Zin-An Wu
Affiliation:
Department of Neurology, Taipei Veterans General Hospital
Kwong-Kum Liao
Affiliation:
Department of Neurology, Taipei Veterans General Hospital
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Abstract

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Background:

Kennedy's disease (KD) is an X-linked recessive polyglutamine disease. Traditionally, it is a lower motor neuron syndrome with additional features such as gynecomastia and tremor. Sensory symptoms are minimal if ever present. We used multimodal evoked potential (EPs) tests to study the distribution of the involvement of the disease.

Methods:

Visual, brainstem auditory, somatosensory and motor EPs were studied in six KD patients. All of them had typical presentations and had been proved genetically.

Results:

Abnormal findings were noted as follows: prolonged peak latencies of visual EPs, increased hearing threshold level, inconsistent brainstem auditory EPs, decreased amplitudes of cortical potentials of somatosensory EPs, and increased motor threshold to transcranial magnetic stimulation.

Conclusions:

Our multimodal EP studies showed that KD involved multiple levels of the nervous system. It implies the widespread effects of the mutant androgen receptors.

Résumé:

RÉSUMÉ:

Potentiels évoqués multimodaux dans la maladie de Kennedy.

Contexte:

La maladie de Kennedy (MK) est une maladie récessive à polyglutamines, liée au chromosome X. Il s'agit traditionnellement d'un syndrome du neurone moteur périphérique accompagné d'autres manifestations comme de la gynécomastie et du tremblement. Si des symptômes sensitifs sont présents, ils sont minimes. Nous avons utilisé les potentiels évoqués (PÉs) multimodaux pour étudier la distribution de l'atteinte dans cette maladie. Méthodes : Nous avons étudié les PÉs visuels, les PÉs auditifs du tronc cérébral, les PÉs somesthésiques et les PÉs moteurs chez six patients atteints de MK. Chez tous, le tableau était typique et la maladie avait été confirmée par un test génétique. Résultats : Les anomalies suivantes ont été constatées : des latences prolongées du pic des PÉs visuels, un seuil auditif plus élevé, des PÉs auditifs du tronc cérébral discordants, une amplitude diminuée des potentiels corticaux des PÉs somesthésiques et un seuil moteur plus élevé à la stimulation magnétique transcrânienne. Conclusions : Nos études des PÉs multimodaux démontre que plusieurs niveaux du système nerveux sont atteints dans la MK, ce qui témoigne des effets diffus des récepteurs androgéniques mutants.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 34 , Issue 3 , August 2007 , pp. 328 - 332
Copyright
Copyright © The Canadian Journal of Neurological 2007

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