Hyperekplexia and stiff-man syndrome: abnormal brainstem reflexes suggest a physiological relationship
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چکیده
منابع مشابه
Hyperekplexia and stiff-man syndrome: abnormal brainstem reflexes suggest a physiological relationship.
BACKGROUND AND OBJECTIVES Hyperekplexia and the stiff-man syndrome (SMS) are both conditions with exaggerated startle suggesting abnormal brainstem function. Investigation of brainstem reflexes may provide insight into disturbed reflex excitation and inhibition underlying these movement disorders. PATIENTS AND METHODS Using four-channel EMG, we examined four trigeminal brainstem reflexes (mon...
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Neonatal hyperekplexia is a rare autosomal dominant startle disorder. Presenting soon after birth, it is often mistakenly diagnosed as spastic quadriparesis, epilepsy etc. While the long-term prognosis is relatively benign, sudden death due to severe spasms have been seen in sporadic cases. We report a case of hyperekplexia with some typical and some unusual findings.
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A patient suffering from severe and continuous muscular spasms is presented. The condition has been classified as the stiff man syndrome and is the first case recorded of the condition in a young African girl. The electrophysiological, biochemical, histochemical, and ultramicroscopic aspects of this disorder have been studied. The spasm appears to be related to overproduction of brain noradrena...
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ژورنال
عنوان ژورنال: Journal of Neurology, Neurosurgery & Psychiatry
سال: 2004
ISSN: 0022-3050
DOI: 10.1136/jnnp.2003.018135