1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-7-05-b 10.15844/pedneurbriefs-7-1-8 Hemiplegic Syndromes Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Autosomal Dominant Alternating Hemiplegia http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 01 1993 01 07 2016 7 1 5 6 Copyright: © 1993 The Author(s) 1993 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. A syndrome of autosomal dominant alternating hemiplegia: clinical presentation mimicking intractable epilepsy; chromosomal studies; and physiologic investigations

The familial occurrence and autosomal dominant inheritance of alternating hemiplegia of childhood is reported from Children’s Hospital, and Massachusetts General Hospital, Harvard Medical School, Boston.

 Autosomal Dominant Inheritance Alternating Hemiplegia Neurodegenerative Disease

The familial occurrence and autosomal dominant inheritance of alternating hemiplegia of childhood is reported from Children’s Hospital, and Massachusetts General Hospital, Harvard Medical School, Boston. Hemiplegie attacks without preceding seizure activity occurred in the proband, a 9-year-old, developmentally retarded boy, during the first year of life. Choreo-athetosis and dystonic posturing were noted between attacks. Father, brother, paternal uncle, and paternal grandmother had histories of alternating hemiplegia. Stroke, neurodegenerative disease, and other causes were excluded. The karyotype showed a translocation, 46,XY,t(3;9)(p26;q34) in the patient and affected relatives. Flunarizine decreased attack frequency >70%. [1]

COMMENT. This is the first description of truly familial alternating hemiplegia, a syndrome of unknown etiology. The authors favor neurovascular or metabolic, rather than epileptic mechanisms.

Sakuragawa N reports 23 cases in an excellent review article from the National Institute of Neuroscience, Ogawa Higashi-cho, Kodaira-shi, Tokyo 187, Japan [2]. The classification of alternating hemiplegia as a migraine variant is discussed.

 Mikati MA Maguire H Barlow CF Ozelius L Breakefield XO Klauck SM A syndrome of autosomal dominant alternating hemiplegia: clinical presentation mimicking intractable epilepsy; chromosomal studies; and physiologic investigations Neurology 1992 Dec 42 12 2251 7 10.1212/WNL.42.12.2251 1361034 Sakuragawa N Alternating hemiplegia in childhood: 23 cases in Japan Brain Dev 1992 Sep 14 5 283 8 10.1016/S0387-7604(12)80144-6 1456380