Hyperekplexia: A syndrome of pathological startle responeses
Hyperekplexia: A syndrome of pathological startle responeses
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DOI：
10.1002/ana.410150107
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发表时间：
1984-01
影响因子：
11.2
通讯作者：
MD E. Saenz-Lope;MD F. J. Herranz-Tanarro;MD J. C. Masdeu;Mds J. R. Chacon Pefia;Neurology Monrefiore;Madrid Spain Cruz Roja
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文献类型：
--
作者：
MD E. Saenz-Lope;MD F. J. Herranz-Tanarro;MD J. C. Masdeu;Mds J. R. Chacon Pefia;Neurology Monrefiore;Madrid Spain Cruz Roja
我们描述了一个家庭和三个零星的惊厥病或过敏症病例。突如其来的意外噪音导致患者僵硬地摔倒，经常受伤但仍保持清醒。这一不寻常的实体不同于惊厥性癫痫和猝倒。氯硝西潘在三名患者中被证明无效。丙戊酸、5-羟色氨酸或吡拉西坦显著减轻了三名患者的异常惊厥。
We describe a family and three sporadic cases of startle disease, or hyperekplexia. Sudden unexpected noises caused the patients to fall rigidly, often injuring themselves but retaining consciousness. This unusual entity differs from startle epilepsy and cataplexy. Clonazepam proved ineffective in three patients. Valproic acid, 5‐hydroxytryptophan, or piracetam markedly reduced the abnormal startle in three patients.