Abstract |
We report a case of non- familial hyperekplexia which characteristically developed apnea and feeding difficulties in the neonatal period. The abnormal startle response was evident from the second week of life onwards. The infant showed a marked improvement of the startle response and muscle hypertonia with clonazepam. Clobazam was also tried with no apparent response. A prominent long latency C response was observed on EMG examination, suggesting a possible cortical neuronal hyperexcitability origin for the abnormal startle response observed in hyperekplexia.
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Authors | J L Gherpelli, A R Nogueira Jr, E J Troster, A D Deutsch, C R Leoné, M W Brotto, A Diament, J L Ramos |
Journal | Brain & development
(Brain Dev)
1995 Mar-Apr
Vol. 17
Issue 2
Pg. 114-6
ISSN: 0387-7604 [Print] Netherlands |
PMID | 7625544
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Anti-Anxiety Agents
- Benzodiazepinones
- Benzodiazepines
- Clobazam
- Clonazepam
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Topics |
- Anti-Anxiety Agents
- Apnea
(etiology)
- Benzodiazepines
- Benzodiazepinones
(therapeutic use)
- Clobazam
- Clonazepam
(therapeutic use)
- Electromyography
- Female
- Humans
- Infant, Newborn
- Movement Disorders
(diagnosis, physiopathology)
- Reflex
- Reflex, Startle
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