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Movement disorder emergencies in childhood

Movement disorder emergencies in childhood
Movement disorder emergencies in childhood

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 non-inflammatory 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.

Biotin, Cardiopulmonary bypass, Chorea, Creatine, Drugs, Dystonia, Infection, Metabolic, Myoclonus, Neuroleptic malignant syndrome, Opsoclonus, Organic aciduria, Parkinsonism, Paroxysmal Autonomic Instability with Dystonia, Sandifer syndrome, Status dystonicus, Sydenham's chorea, Systemic lupus erythematosus, Tremor, Wilson's disease
1090-3798
390-404
Kirkham, F. J.
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Haywood, P.
039dccd6-ea7b-4d60-924a-68ec04e8e38e
Kashyape, P.
53b118a0-2b39-4898-b52e-d136f72f5a16
Borbone, J.
a1b36759-5aa8-443d-8db8-8d1138b90eb7
Lording, A.
ba4e5596-5081-4d63-8046-152388aaa476
Pryde, K.
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Cox, M.
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Keslake, J.
386531c2-a0bf-4e80-97df-d54cae4d8b9f
Smith, M.
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Cuthbertson, L.
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Murugan, V.
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MacKie, S.
a27ea771-3cd8-438f-9c71-75454eafd999
Thomas, N. H.
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Whitney, A.
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Forrest, K. M.
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Parker, A.
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Forsyth, R.
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Kipps, C. M.
e43be016-2dc2-45e6-9a02-ab2a0e0208d5
Kirkham, F. J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Haywood, P.
039dccd6-ea7b-4d60-924a-68ec04e8e38e
Kashyape, P.
53b118a0-2b39-4898-b52e-d136f72f5a16
Borbone, J.
a1b36759-5aa8-443d-8db8-8d1138b90eb7
Lording, A.
ba4e5596-5081-4d63-8046-152388aaa476
Pryde, K.
cd3b90c0-f119-44d4-b3e9-9ce5d33c2efb
Cox, M.
40f0c88f-0295-47ba-a1db-df693592ba73
Keslake, J.
386531c2-a0bf-4e80-97df-d54cae4d8b9f
Smith, M.
558e1c6b-5c57-444a-9508-509c50128f91
Cuthbertson, L.
d0b57198-6e5e-4522-8bfb-b0360ae2b8eb
Murugan, V.
ce24f428-77c4-4033-9718-b982d2dedaca
MacKie, S.
a27ea771-3cd8-438f-9c71-75454eafd999
Thomas, N. H.
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Whitney, A.
e98cdee2-33d2-41d9-bbd4-8ba44e4b537b
Forrest, K. M.
93f2bb17-da4d-43a3-9f52-1842f558b85e
Parker, A.
2060d83a-15d6-444c-82f3-a0e4e549dcaa
Forsyth, R.
08f5e1d2-8247-485d-98b1-d9c8a8d2ed99
Kipps, C. M.
e43be016-2dc2-45e6-9a02-ab2a0e0208d5

Kirkham, F. J., Haywood, P., Kashyape, P., Borbone, J., Lording, A., Pryde, K., Cox, M., Keslake, J., Smith, M., Cuthbertson, L., Murugan, V., MacKie, S., Thomas, N. H., Whitney, A., Forrest, K. M., Parker, A., Forsyth, R. and Kipps, C. M. (2011) Movement disorder emergencies in childhood. European Journal of Paediatric Neurology, 15 (5), 390-404. (doi:10.1016/j.ejpn.2011.04.005).

Record type: Article

Abstract

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 non-inflammatory 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.

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More information

Published date: September 2011
Keywords: Biotin, Cardiopulmonary bypass, Chorea, Creatine, Drugs, Dystonia, Infection, Metabolic, Myoclonus, Neuroleptic malignant syndrome, Opsoclonus, Organic aciduria, Parkinsonism, Paroxysmal Autonomic Instability with Dystonia, Sandifer syndrome, Status dystonicus, Sydenham's chorea, Systemic lupus erythematosus, Tremor, Wilson's disease

Identifiers

Local EPrints ID: 428348
URI: http://eprints.soton.ac.uk/id/eprint/428348
ISSN: 1090-3798
PURE UUID: 56a36c95-a799-4297-bdae-08a76135d07c
ORCID for F. J. Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

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Date deposited: 21 Feb 2019 17:30
Last modified: 30 Jan 2020 01:31

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Contributors

Author: F. J. Kirkham ORCID iD
Author: P. Haywood
Author: P. Kashyape
Author: J. Borbone
Author: A. Lording
Author: K. Pryde
Author: M. Cox
Author: J. Keslake
Author: M. Smith
Author: L. Cuthbertson
Author: V. Murugan
Author: S. MacKie
Author: N. H. Thomas
Author: A. Whitney
Author: K. M. Forrest
Author: A. Parker
Author: R. Forsyth
Author: C. M. Kipps

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