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23. Startle reaction and startle epilepsy

We present 3-year old girl with clinical picture of spastic cerebral palsy with inadequate motor reaction (reflex myoclonus) provoked by sudden and unexpected sounds beginning immediately after birth, lasting for seconds, sometimes with loss of postural tone and fallings especially in the morning. Repetition of the stimulus would induce transient habituation (refractory period of 1 up to 3min). The girl’s mental status was normal. Standard and video EEG recordings were nonspecific. Antiepileptic drugs (clonazepam, levetiracetam) were unsuccessful in treating this condition. Because of their clinical and therapeutical similarities startle disease (hyperekplexia) and startle epilepsy usually can be confused, especially in early childhood period. EEG and particularly video EEG recordings ar…

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