Benign familial nocturnal alternating hemiplegia of childhood.
In infancy, two brothers developed recurrent attacks of alternating or bilateral hemiplegia arising exclusively out of sleep. The episodes were terminated by even brief sleep. Neither child had hypotonia, dystonic attacks, paroxysmal eye movement abnormalities, or other features characteristic of the now-classic form of alternating hemiplegia of childhood (AHC). The development of the brothers has so far remained normal. Both parents have a history of migraine. In the older boy, magnetic resonance spectroscopy (MRS) of muscle showed increased inorganic phosphate similar to what is found in children with AHC. In the younger brother and parents, MRS of muscle was normal. Other investigations were unrevealing. Flunarizine greatly reduced the duration of attacks. This genetically determined disorder represents a specific entity that is probably migraine-related and is easily misdiagnosed as AHC. Because of its benign course, particularly as far as mental development is concerned, it must be distinguished from classic AHC, which has a terrible prognosis.
Andermann
E
E
Department of Neurology and Neurosurgery, McGill University, Montreal, PQ, Canada.
Andermann
F
F
Silver
K
K
Levin
S
S
Arnold
D
D
D000328:Adult; D014151:Anti-Anxiety Agents; D000927:Anticonvulsants / Q000627:therapeutic use; D001569:Benzodiazepines; D001570:Benzodiazepinones / Q000627:therapeutic use; D000078306:Clobazam; D004569:Electroencephalography; D005260:Female; D005444:Flunarizine / Q000627:therapeutic use; D006429:Hemiplegia / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D006801:Humans; D007223:Infant; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008881:Migraine Disorders / Q000235:genetics; D018482:Muscle, Skeletal / Q000737:chemistry; D010710:Phosphates / Q000032:analysis; D012008:Recurrence; D012893:Sleep Wake Disorders / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*
D014151:Anti-Anxiety Agents; D000927:Anticonvulsants; D001570:Benzodiazepinones; D010710:Phosphates; D001569:Benzodiazepines; D000078306:Clobazam; D005444:Flunarizine