Postural tremor in X‐linked spinal and bulbar muscular atrophy
Identifieur interne : 002129 ( Main/Exploration ); précédent : 002128; suivant : 002130Postural tremor in X‐linked spinal and bulbar muscular atrophy
Auteurs : Ritsuko Hanajima [Japon] ; Yasuo Terao [Japon] ; Setsu Nakatani-Enomoto [Japon] ; Masashi Hamada [Japon] ; Akihiro Yugeta [Japon] ; Hideyuki Matsumoto [Japon] ; Tomotaka Yamamoto [Japon] ; Shoji Tsuji [Japon] ; Yoshikazu Ugawa [Japon]Source :
- Movement Disorders [ 0885-3185 ] ; 2009-10-30.
Descripteurs français
- Pascal (Inist)
English descriptors
- KwdEn :
- Adult, Age of Onset, Coherence, DNA Repeat Expansion, Electromyography, Fourier Analysis, Genes, X-Linked (genetics), Humans, Kennedy's disease, Male, Median Nerve (physiopathology), Middle Aged, Muscular Disorders, Atrophic (complications), Muscular Disorders, Atrophic (genetics), Muscular Disorders, Atrophic (physiopathology), Nervous system diseases, Posture, Reflex, SBMA, Tremor, Tremor (etiology), Tremor (physiopathology), Ulnar Nerve (physiopathology), Upper Extremity (physiopathology), coherence, mechanical tremor, peripheral tremor, reflex tremor.
- MESH :
- complications : Muscular Disorders, Atrophic.
- etiology : Tremor.
- genetics : Genes, X-Linked, Muscular Disorders, Atrophic.
- physiopathology : Median Nerve, Muscular Disorders, Atrophic, Tremor, Ulnar Nerve, Upper Extremity.
- Adult, Age of Onset, DNA Repeat Expansion, Electromyography, Fourier Analysis, Humans, Male, Middle Aged, Posture.
Abstract
Postural tremor is a common initial symptom in spinal and bulbar muscular atrophy (SBMA), but its pathophysiological mechanisms remain to be studied. This study was undertaken to examine the physiological mechanisms underlying postural tremor in SBMA. For eight patients (36–63 years old) with genetically confirmed SBMA, we recorded surface electromyograms (EMGs) from the forearm muscles and hand movements with an accelerometer (ACC) while maintaining a posture with and without a weight load. We then analyzed their power spectra and coherence. The peak tremor frequency was 6–9 Hz in seven patients and 2–3 Hz in one patient. Oscillatory movements were associated with EMG activity in five patients, but not in three patients. Weight loads and postural changes affected the tremor frequency in all patients. Tremor was classified as “reflex tremor” in five patients and “mechanical tremor” in three patients. These results suggest that peripheral factors play important roles in tremor genesis in SBMA, although its clinical features resemble essential tremor. Subclinical sensory disturbance or a decrease of motor unit numbers might be candidates for such peripheral factors contributing to tremor genesis in SBMA. © 2009 Movement Disorder Society
Url:
DOI: 10.1002/mds.22566
Affiliations:
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Le document en format XML
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<front><div type="abstract" xml:lang="en">Postural tremor is a common initial symptom in spinal and bulbar muscular atrophy (SBMA), but its pathophysiological mechanisms remain to be studied. This study was undertaken to examine the physiological mechanisms underlying postural tremor in SBMA. For eight patients (36–63 years old) with genetically confirmed SBMA, we recorded surface electromyograms (EMGs) from the forearm muscles and hand movements with an accelerometer (ACC) while maintaining a posture with and without a weight load. We then analyzed their power spectra and coherence. The peak tremor frequency was 6–9 Hz in seven patients and 2–3 Hz in one patient. Oscillatory movements were associated with EMG activity in five patients, but not in three patients. Weight loads and postural changes affected the tremor frequency in all patients. Tremor was classified as “reflex tremor” in five patients and “mechanical tremor” in three patients. These results suggest that peripheral factors play important roles in tremor genesis in SBMA, although its clinical features resemble essential tremor. Subclinical sensory disturbance or a decrease of motor unit numbers might be candidates for such peripheral factors contributing to tremor genesis in SBMA. © 2009 Movement Disorder Society</div>
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<name sortKey="Ugawa, Yoshikazu" sort="Ugawa, Yoshikazu" uniqKey="Ugawa Y" first="Yoshikazu" last="Ugawa">Yoshikazu Ugawa</name>
<name sortKey="Yamamoto, Tomotaka" sort="Yamamoto, Tomotaka" uniqKey="Yamamoto T" first="Tomotaka" last="Yamamoto">Tomotaka Yamamoto</name>
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