Hyperekplexia, a cause of neonatal apnea: a case report.
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.
Gherpelli
J L
JL
Departamento de Neurologia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Brazil.
Nogueira
A R
AR
Troster
E J
EJ
Deutsch
A D
AD
Leoné
C R
CR
Brotto
M W
MW
Diament
A
A
Ramos
J L
JL
D014151:Anti-Anxiety Agents; D001049:Apnea / Q000209:etiology*; D001569:Benzodiazepines; D001570:Benzodiazepinones / Q000627:therapeutic use; D000078306:Clobazam; D002998:Clonazepam / Q000627:therapeutic use; D004576:Electromyography; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D009069:Movement Disorders / Q000175:diagnosis / Q000503:physiopathology*; D012018:Reflex; D013216:Reflex, Startle
D014151:Anti-Anxiety Agents; D001570:Benzodiazepinones; D001569:Benzodiazepines; D000078306:Clobazam; D002998:Clonazepam