Document Detail

Evidence of a non-progressive course of alternating hemiplegia of childhood: study of a large cohort of children and adults.
MedLine Citation:
PMID:  20974617     Owner:  NLM     Status:  MEDLINE    
Alternating hemiplegia of childhood is a neurological disorder characterized by episodes of hemiplegia, various non-epileptic paroxysmal events and global neurological impairment. Characterization of the evolution and outcome into adulthood has not been sufficiently investigated. The goal of this study was to elucidate the natural history of alternating hemiplegia within a large cohort of 157 patients, as part of the European Network for Research on Alternating Hemiplegia project. A questionnaire was formulated to determine the severity of both paroxysmal and global neurological impairment and address progression of the disorder by allocating data to specific age epochs up to and over 24 years of age. Patients in early age groups were consistently present in subsequent later age groups and for each patient, data were collected for each corresponding age epoch. The study was based on predominantly retrospective and, for a period of 2 years, prospective data. At inclusion, patients were aged from 9 months to 52 years. The median age at diagnosis was 20 months. All patients experienced hemiplegic attacks; 86.5% reported episodes of bilateral weakness, 88% dystonic attacks, 53% epileptic seizures, 72% developed chorea and/or dystonia and 92% mental retardation. When data over the course of the illness were examined for the whole cohort, the severity of symptoms did not appear to change, with the exception of abnormal ocular movements and hypotonia that regressed, but did not disappear into adulthood (from 86 to 36% and 76 to 36%, respectively). No statistically significant correlation between a history of severe paroxysmal hemiplegic/dystonic episodes and a worse neurological outcome was identified. Seven patients died, some of whom experienced severe plegic attacks or epileptic seizures at the time of death. History of severe plegic/dystonic attacks was not found to be an aggravating factor for deceased patients. Our results provide evidence that the natural history of alternating hemiplegia is highly variable and unpredictable for individual patients. However, we did not find evidence to support a steadily progressive and degenerative course of the disorder when patients were analysed as a group. For a minority of patients, a risk of sudden death was associated with more severe neurological impairment. The European Network for Research on Alternating Hemiplegia Registry, validated by our study, includes all major neurological signs and symptoms of alternating hemiplegia and may thus be used as a precedent for the progressive inclusion and follow-up of patients as well as a reference for genetic studies and treatment trials.
Eleni Panagiotakaki; Giuseppe Gobbi; Brian Neville; Friedrich Ebinger; Jaume Campistol; Sona Nevsímalová; Laura Laan; Paul Casaer; Georg Spiel; Melania Giannotta; Carmen Fons; Miriam Ninan; Guenter Sange; Tsveta Schyns; Rosaria Vavassori; Dominique Poncelin; ; Alexis Arzimanoglou
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-10-24
Journal Detail:
Title:  Brain : a journal of neurology     Volume:  133     ISSN:  1460-2156     ISO Abbreviation:  Brain     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-12-03     Completed Date:  2011-01-07     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372537     Medline TA:  Brain     Country:  England    
Other Details:
Languages:  eng     Pagination:  3598-610     Citation Subset:  AIM; IM    
Institute for Children and Adolescents with Epilepsy (IDEE), Hôpital Femme Mère Enfant, University Hospitals of Lyon (HCL), France.
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MeSH Terms
Aging / physiology
Autonomic Nervous System Diseases / etiology,  physiopathology
Child, Preschool
Cohort Studies
Data Collection
Data Interpretation, Statistical
Disability Evaluation
Disease Progression
Epilepsy / etiology
Functional Laterality / physiology
Headache / etiology
Hemiplegia / diagnosis,  mortality,  pathology*
Middle Aged
Ocular Motility Disorders / etiology
Retrospective Studies
Seizures / etiology
Sleep Disorders / etiology
Treatment Outcome
Young Adult
Alexis Arzimanoglou / ; Jaume Campistol / ; Paul Casaer / ; Friedrich Ebinger / ; Carmen Fons / ; Melania Giannotta / ; Giuseppe Gobbi / ; David Kemlink / ; Laura Laan / ; Brian Neville / ; Sona Nevsímalová / ; Miriam Ninan / ; Claudia Oechsler / ; Eleni Panagiotakaki / ; Georg Spiel / ; Maria Teresa Bassi / ; Giorgio Casari / ; Francis Crawley / ; Boneschi Filippo Martinelli / ; Sophie Nicole / ; Dominique Poncelin / ; Guenter Sange / ; Tsveta Schyns / ; Arn van den Maagdenberg / ; Rosaria Vavassori / ; Claudio Zucca /

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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