Forty‐one year follow‐up of childhood‐onset opsoclonus‐myoclonus‐ataxia: Cerebellar atrophy, multiphasic relapses, and response to IVIG

Pranzatelli, Michael R.; Tate, Elizabeth D.; Kinsbourne, Marcel; Caviness, Verne S.; Mishra, Braja Gopal

doi:10.1002/mds.10283

Cited by 29 publications

(15 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Most studies had patient numbers between 10-21, but the largest series (by Tate et al [24] and Pohl et al [16]) had 105 and 54 patients, respectively. The longest follow-up in a single report was 41 years [18]. Complete recovery was seen in only 12%-38% of the children [15,16].…”

Section: Discussionmentioning

confidence: 98%

Long-term outcome of ten children with opsoclonus-myoclonus syndrome

2006

View full text Add to dashboard Cite

Opsoclonus-myoclonus syndrome (OMS) in children is a rare neurological condition with opsoclonus, myoclonus, ataxia and irritability in the first 2 years of life. It can be idiopathic, parainfectious, or paraneoplastic with tumours of the neural crest. Few studies of long-term follow-up after OMS have been published. We investigated the motor, cognitive and behavioural outcome of ten patients (eight girls and two boys) seen between 1987 and 2002. We reviewed the records and reassessed the patients. A ganglioneuroma was found in one patient and a neuroblastoma in another. Tumour resection did not influence the OMS. The age at diagnosis was 10-24 months and the follow-up period 1-17 years (average 6.5 years). The interval between the first signs and symptoms and starting treatment was 2-12 weeks: treatment consisted of different immunosupressants. Remission was achieved within 5 months in seven, and relapses were present in seven of ten. At follow-up, only one child had mild ataxia. IQ testing was performed in nine with scores below 75 in four and above 85 in four. Attention deficit and visuomotor difficulties led to school problems with special needs, also in those three children with normal IQs. Only two children were attending regular schools. Behavioural problems were reported in seven, and speech difficulties were present in five. In conclusion, the long-term outcome in our patients with OMS was dominated by cognitive and behavioural problems and not by ataxia. Compared with previous reports, our patients were treated earlier. Larger studies and uniform treatment protocols are needed to demonstrate whether early and prolonged immunosupressant therapy has a favourable influence on outcome.

show abstract

Section: Discussionmentioning

confidence: 98%

Long-term outcome of ten children with opsoclonus-myoclonus syndrome

2006

View full text Add to dashboard Cite

show abstract

“…IVIg has been shown to improve function acutely, but long-term benefit has to be validated further. 2,32 More recently, rituximab, a monoclonal anti-B cell antibody, has been used successfully to treat OMS as adjunctive therapy to adrenocorticotrophic hormone (ACTH), IVIg, or both. 18 Rituximab reduces cerebrospinal fluid expansion of B cells, a marker of disease activity, thereby decreasing autoimmune activation.…”

Section: Discussionmentioning

confidence: 99%

Outcome and Prognostic Features in Opsoclonus-Myoclonus Syndrome From Infancy to Adult Life

et al. 2011

View full text Add to dashboard Cite

show abstract

“…3,4 The brainstem and cerebellum are thought to give rise to the principal motor features. 3,[5][6][7] In children, neuroblastoma 8 and viral infections 9,10 are the most common causes. 11 The immunopathophysiology of childhood OMS has remained rather elusive.…”

mentioning

confidence: 99%