Pediatric opsoclonus-myoclonus-ataxia syndrome: Experience from a tertiary care university hospital.
Neurol India
; 66(5): 1332-1337, 2018.
Article
em En
| MEDLINE
| ID: mdl-30232999
ABSTRACT
BACKGROUND:
Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare disorder; there is limited experience regarding its clinical course and therapeutic response. AIMS ANDOBJECTIVES:
To describe the clinical profile, investigations, and therapeutic outcome in pediatric OMAS. PATIENTS ANDMETHODS:
Fourteen children (age 27.1 ± 7 months; male female = 12.3) suffering from OMAS seen over a period of 10 years (2006-2015) were included in the study. Their clinicodemographic profile, investigations, therapeutic outcome at follow-up, and relapses were reviewed.RESULTS:
Ten children reported antecedent events (respiratory infection 7; gastrointestinal infection 1; vaccination 2). The most common referral diagnosis was acute cerebellitis (n = 8). Hypotonia (n = 9), abnormal behavior (n = 10), and neuroregression (n = 6) were also the frequent manifestations. Brain magnetic resonance imaging, cerebrospinal fluid, and urinary vanillylmandelic acid were normal in all the patients. Seven patients had an underlying tumor (abdomen 4; thorax 2; neck 1) detected by ultrasound (n = 2/14), computed tomography (CT) (n = 6/12), and fluorodeoxyglucose - positron emission tomography (n = 2/2). CT scan identified the tumor in 2 patients where metaiodobenzylguanidine scintigraphy was negative. All patients received steroids for 22.3 ± 20 months (3 months to 5 years). Eight required prolonged immunomodulation (>12 months). Complete remission after follow-up of 31.3 ± 19 months (7 months to 5 years) was noted in 5 patients, whereas the rest had persisting behavioral and cognitive abnormalities. Relapses were noted in 6 patients related to intercurrent infections (n = 5) and discontinuation of steroids (n = 1). The patients presented with isolated symptoms of the full-blown syndrome during their relapses.CONCLUSION:
OMAS in children runs an indolent course requiring careful monitoring and long-term immunomodulation. An abnormal behavior is common and the outcome is variable.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndromes Paraneoplásicas
/
Síndrome de Opsoclonia-Mioclonia
Tipo de estudo:
Prognostic_studies
Limite:
Child, preschool
/
Female
/
Humans
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Infant
/
Male
Idioma:
En
Revista:
Neurol India
Ano de publicação:
2018
Tipo de documento:
Article
País de afiliação:
Índia