Sympathetic nerve outflow to skin in a case with dentatorubral-pallidoluysian atrophy.
J Clin Neurosci
; 87: 80-83, 2021 May.
Article
em En
| MEDLINE
| ID: mdl-33863540
ABSTRACT
Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disorder characterized by slowly progressive cerebellar ataxia. Previously, autonomic symptoms or dysfunction have not been reported. To evaluate subclinical autonomic dysfunction regarding thermoregulatory function in SCA, we recorded sympathetic outflow to skin in a DRPLA patient confirmed by genetic analysis. We recorded skin sympathetic nerve activity (SSNA), which was elicited and recorded by using the microneurographical technique. In results, the resting frequency of SSNA bursts was very low (8.2 ± 0.4 bursts/min [institutional normal range 20.8 ± 2.4 bursts/min]). However, acceleration of SSNA bursts induced by mental arithmetic stress was confirmed. The amplitude of reflex bursts induced by electrical stimuli was slightly low (9.6 ± 1.6 µV [institutional normal range 10.9 ± 2.2 µV]), and the reflex latency was mildly prolonged (872 ± 23.7 msec [institutional normal range 761.9 ± 51.7 msec]). These results suggest potentially central autonomic dysfunction in this patient with DRPLA. To our knowledge, this is the first report to record SSNA and confirm subclinical autonomic dysfunction in a case with DRPLA.
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Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Pele
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Fenômenos Fisiológicos da Pele
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Fibras Adrenérgicas
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Epilepsias Mioclônicas Progressivas
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Condução Nervosa
Limite:
Female
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Humans
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Middle aged
Idioma:
En
Revista:
J Clin Neurosci
Assunto da revista:
NEUROLOGIA
Ano de publicação:
2021
Tipo de documento:
Article