Electromyography and clinical neurophysiology · 2007
Rigid spine syndrome: vacuolar variant multimodal evoked potentials.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Stübgen Joerg-Patrick
Аннотация
BACKGROUND: Overt or covert central nervous system (CNS) abnormalities have been reported in various primary muscle diseases, including congenital muscular dystrophies.
PURPOSE: To ascertain by neurophysiologic techniques evidence of CNS dysfunction in a relatively large, homogeneous group of patients with the "vacuolar variant" of the rigid spine syndrome (RSS).
METHODS: Standard evoked potential (EP) techniques were used to study the visual, auditory and somatosensory pathways in patients with RSS.
RESULTS: Abnormal values were recorded in 78% (7/9) of patients, specifically of visual pathways (2/9), brainstem auditory pathways (2/9), and somatosensory pathways (5/9), and the retina (1/9). Abnormal findings were not correlated with clinical measures of patient age, disease duration nor degree of weakness.
CONCLUSION: Electrophysiological studies showed that the CNS is not primarily involved in any disease process that underlies this congenital myopathy. There were reasonable other explanations for most abnormal measurements.
PURPOSE: To ascertain by neurophysiologic techniques evidence of CNS dysfunction in a relatively large, homogeneous group of patients with the "vacuolar variant" of the rigid spine syndrome (RSS).
METHODS: Standard evoked potential (EP) techniques were used to study the visual, auditory and somatosensory pathways in patients with RSS.
RESULTS: Abnormal values were recorded in 78% (7/9) of patients, specifically of visual pathways (2/9), brainstem auditory pathways (2/9), and somatosensory pathways (5/9), and the retina (1/9). Abnormal findings were not correlated with clinical measures of patient age, disease duration nor degree of weakness.
CONCLUSION: Electrophysiological studies showed that the CNS is not primarily involved in any disease process that underlies this congenital myopathy. There were reasonable other explanations for most abnormal measurements.