ICNC Abstracts, ICNC 2018

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Multimodal immunotherapy in a patient with opsoclonus-myoclonus-ataxia syndrome - case report
Tomasz Mieszczanek

Last modified: 2018-09-09

Abstract


Introduction

Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rere autoimmun condition that presents clinically with acute or subacute onset of opsoclonus, myoclonus and ataxia.

Neuroblastom was confirmed in approximately half of the children with OMAS and predominantly affects young children(4).

Paraneoplastic OMAS are usually associated with lung, breast and ovarian cancers and account for about 20% of adult cases (2).

OMAS long term outcomes including motor and cognitive disabilities may be poor. Among United Kingdom's patients treated with corticosteroids monotherapy, 60% had ongoing motor abnormalities at last follow-up and 51% had learning disability (3).

Some investigations suggest that more aggressive immunotherapy might improve outcome in OMAS (1).

 

Methods:

Case report:  13 years old girl admitted with subacute onset of ataxia and opsoclonus. Clinical follow up was made.

 

Results:

Neurological examination - generelised ataxia and opsoclonus wtith rapid progression - in a few days patient was not able to to sit and eat by herself, she couldn't watch TV and read becouse of opsoclonus

Cerebral and medullary MRI was normal, cystic process in the small pelvis was found

Pleocytosis with 117 leucocytes was foud, normal protein and glucose

Laparotomy was made and ovarium with a policystical tumour was removed

Histology- mature teratoma without malignancy

CSF negative for neoplastic cells and autoimmunological encephalitis antibodies included NMDAR

Treatment with hig dosis corticosteroids combined with Immunoglobulins and plasma exchange was included

Total regression of opsoclonus and ataxia was obtained

 

Conclusions:

Multimodal immunotherapy of OMAS may be very effective


Keywords


opsoclonus-myoclonus-ataxia syndrome, multimodal immunotherapy, ovarian teratoma

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