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Movement disorder emergencies in childhood

Lookup NU author(s): Dr Rob ForsythORCiD


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The literature on paediatric acute-onset movement disorders is scattered. In a prospective cohort of 52 children (21 male; age range 2mo-15y), the commonest were chorea, dystonia, tremor, myoclonus, and Parkinsonism in descending order of frequency. In this series of mainly previously well children with cryptogenic acute movement disorders, three groups were recognised: (1) Psychogenic disorders (n = 12), typically >10 years of age, more likely to be female and to have tremor and myoclonus (2) Inflammatory or autoimmune disorders (n = 22), including N-methyl-D-aspartate receptor encephalitis, opsoclonus-myoclonus, Sydenham chorea, systemic lupus erythematosus, acute necrotizing encephalopathy (which may be autosomal dominant), and other encephalitides and (3) Non-inflammatory disorders (n = 18), including drug-induced movement disorder, post-pump chorea, metabolic, e.g. glutaric aciduria, and vascular disease, e.g. moyamoya. Other important noninflammatory movement disorders, typically seen in symptomatic children with underlying aetiologies such as trauma, severe cerebral palsy, epileptic encephalopathy, Down syndrome and Rett syndrome, include dystonic posturing secondary to gastro-oesophageal reflux (Sandifer syndrome) and Paroxysmal Autonomic Instability with Dystonia (PAID) or autonomic 'storming'. Status dystonicus may present in children with known extrapyramidal disorders, such as cerebral palsy or during changes in management e.g. introduction or withdrawal of neuroleptic drugs or failure of intrathecal baclofen infusion; the main risk in terms of mortality is renal failure from rhabdomyolysis. Although the evidence base is weak, as many of the inflammatory/autoimmune conditions are treatable with steroids, immunoglobulin, plasmapheresis, or cyclophosphamide, it is important to make an early diagnosis where possible. Outcome in survivors is variable. Using illustrative case histories, this review draws attention to the practical difficulties in diagnosis and management of this important group of patients. (C) 2011 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Publication metadata

Author(s): Kirkham FJ, Haywood P, Kashyape P, Borbone J, Lording A, Pryde K, Cox M, Keslake J, Smith M, Cuthbertson L, Murugan V, Mackie S, Thomas NH, Whitney A, Forrest KM, Parker A, Forsyth R, Kipps CM

Publication type: Conference Proceedings (inc. Abstract)

Publication status: Published

Conference Name: European Journal of Paediatric Neurology: 9th European Pediatric Neurology Society Congress

Year of Conference: 2011

Pages: 390-404

ISSN: 1090-3798

Publisher: Elsevier Ltd


DOI: 10.1016/j.ejpn.2011.04.005

Library holdings: Search Newcastle University Library for this item

ISBN: 15322130