Opsoclonus–myoclonus syndrome following rotavirus gastroenteritis

Gürkaş, Esra; Gücüyener, Kıvılcım; Yılmaz, Ünsal; Havalı, Cengiz; Demir, Ercan

doi:10.1111/ped.12433

Cited by 15 publications

(10 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The requisite types of diagnostic scans and frequency of scanning are not standardized. Also, an argument for a para-/post-infectious designation has been made for specific infectious associations with OMS, such as Epstein–Barr virus ( 43 ), Mycoplasma pneumoniae ( 44 ), hepatitis C ( 45 ), adenovirus C3 ( 46 ), rotavirus ( 47 ), among others ( 4 ). In our study, however, mostly non-specific illnesses were reported, there was no consistent pathogen identified, and the majority of patients appeared to have no “infectious” prodrome.…”

Section: Discussionmentioning

confidence: 99%

Demographic, Clinical, and Immunologic Features of 389 Children with Opsoclonus-Myoclonus Syndrome: A Cross-sectional Study

2017

View full text Add to dashboard Cite

Pediatric-onset opsoclonus-myoclonus syndrome (OMS) is a devastating neuroinflammatory, often paraneoplastic, disorder. The objective was to characterize demographic, clinical, and immunologic aspects in the largest cohort reported to date. Cross-sectional data were collected on 389 children in an IRB-approved, observational study at the National Pediatric Myoclonus Center. Non-parametric statistical analysis was used. OMS manifested in major racial/ethnic groups, paralleling US population densities. Median onset age was 1.5 years (1.2–2 interquartile range), inclusive of infants (14%), toddlers (61%), and youngsters (25%). The higher female sex ratio of 1.2 was already evident in toddlers. Time to diagnosis was 1.2 months (0.7–3); to treatment, 1.4 months (0.4–4). Irritability/crying dominated prodromal symptomatology (60%); overt infections in <35%. Acute cerebellar ataxia was the most common misdiagnosis; staggering appeared earliest among 10 ranked neurological signs (P < 0.0001). Some untreated youngsters had no words (33%) or sentences (73%). Remote neuroblastic tumors were detected in 50%; resection was insufficient OMS treatment (58%). Age at tumor diagnosis related to tumor type (P = 0.004) and stage (P = 0.002). A novel observation was that paraneoplastic frequency varied with patient age—not a mere function of the frequency of neuroblastoma, which was lowest in the first 6 months of life, when that of neuroblastoma without OMS was highest. The cerebrospinal fluid (CSF) leukocyte count was minimally elevated in 14% (≤11/mm3) with normal differential, and commercially screened serum autoantibodies were negative, but CSF oligoclonal bands (OCB) and B cells frequency were positive (58 and 93%). Analysis of patients presenting on immunotherapy revealed a shift in physician treatment practice patterns from monotherapy toward multi-agent immunotherapy (P < 0.001); the number of agents/sequences varied. In sum, a major clinical challenge is to increase OMS recognition, prevent initial misdiagnosis, and shorten time to diagnosis/treatment. The index of suspicion for an underlying tumor must remain high despite symptoms of infection. The disparity in onset age of neuroblastoma frequency with that of neuroblastoma with OMS warrants further studies of potential host/tumor factors. OMS neuroinflammation is best diagnosed by CSF OCB and B cells, not by routine CSF or commercial antibody studies.

show abstract

Section: Discussionmentioning

confidence: 99%

Demographic, Clinical, and Immunologic Features of 389 Children with Opsoclonus-Myoclonus Syndrome: A Cross-sectional Study

2017

View full text Add to dashboard Cite

show abstract

“…Imaging‐negative cases have been designated as “parainfectious,” “post‐infectious,” or “idiopathic,” usually on the basis of whether “prodromal” (preneurological) symptoms were interpreted as suggestive of infection . Despite a litany of infections reported in case studies, some with intrathecal inflammation, no consistent pathogen has been identified, the infection is usually nondeclaratory (nondemonstrable), and patients may have no “infectious” prodrome …”

Section: Introductionmentioning

confidence: 99%

“…Imaging-negative cases have been designated as "parainfectious," 13,14 "post-infectious," [15][16][17] or "idiopathic," 18,19 usually on the basis of whether "prodromal" (preneurological) symptoms were interpreted as suggestive of infection. 20 Despite a litany of infections reported in case studies, 14,16,[21][22][23][24][25][26][27][28][29][30][31][32][33][34][35] some with intrathecal inflammation, no consistent pathogen has been identified, 3 the infection is usually nondeclaratory (nondemonstrable), 10 and patients may have no "infectious" prodrome. 20 The biological validity of OMS etiological designations has been enigmatic and difficult to address.…”

Section: Introductionmentioning

confidence: 99%

Multifactorial analysis of opsoclonus‐myoclonus syndrome etiology (“Tumor” vs. “No tumor”) in a cohort of 356 US children

Pranzatelli

Tate

McGee

2018

Pediatric Blood & Cancer

View full text Add to dashboard Cite

Background: Pediatric opsoclonus-myoclonus syndrome (OMS) presents a paradox of etiopathogenesis: A neuroblastic tumor (NB) is found in only one half of the cases, the others are ascribed to infections or designated as idiopathic. Method:From an IRB-approved observational study of 356 US children with OMS, secondary analysis of "etiology" and related factors was performed on a well-characterized cohort. The "Tumor" (n = 173) and "No Tumor" groups (n = 183), as defined radiologically, were compared according to multiple factors considered potentially differentiating. Data were analyzed retrospectively using parametric and nonparametric tests as indicated.Results: Patients with NB were not distinguishable by prodromal symptoms, OMS onset age, gender, race/ethnicity, OMS severity, rank order of neurological sign appearance, or geographic distribution. Various CSF immunologic biomarker abnormalities of OMS did not vary in the presence or absence of a detectable tumor: frequency of six lymphocyte subsets, or concentrations of 18 cytokines/chemokines, cytokine antagonists, chemokine receptors, cell adhesion molecules, or neuronal/glial markers. Prior responsiveness to conventional immunotherapy was not contingent on tumor/no tumor designation. Conclusions:Multiple convergent factors provide compelling empirical evidence and rationalize the concept that OMS is one neurological disorder, regardless of apparent etiology. Limitations to the current clinical etiologic classifications as paraneoplastic, parainfectious/post-infectious, and idiopathic etiology require antigen-based biological solutions to tease out the molecular pathophysiology of viral/tumoral mechanisms. Systematic studies, regardless of presumed etiology, will be necessary to find the highest-yield combination of imaging approaches, screening for infectious agents, and new biomarkers. Two testable hypotheses for future research are presented.

show abstract

“…Se han informado los siguientes patógenos como asociados al SOM: Mycoplasma pneumoniae, Salmonella entérica, rotavirus, citomegalovirus, herpes virus tipo 6 y hepatitis C (1,13). Recientemente se han descrito casos asociados a virus del dengue y mixovirus (14)(15)(16). Otros mecanismos asociados son infección por VIH, toxinas, embarazo, enfermedad de Lyme, virus varicela zóster, vacunas (influenza, triple viral, rubeola) (17).…”

Section: Discussionunclassified

Síndrome de Kinsbourne: reporte de un caso

Paredes-Ebratt

Espinosa

2017

iatreia

View full text Add to dashboard Cite

El síndrome de Kinsbourne, conocido también como "síndrome de opsoclonus-mioclonus" o "síndrome de los ojos danzantes", es una enfermedad rara caracterizada por movimientos oculares rápidos, irregulares, multidireccionales (opsoclonus), movimientos mioclónicos en tronco, cara y/o extremidades y ataxia; se presenta entre los 6 y 36 meses de edad. Su etiología puede ser paraneoplásica (neuroblastoma), no paraneoplásica (infecciosa) o idiopática. Independientemente de la causa, se han utilizado inmunosupresores para reducir la formación de anticuerpos posiblemente involucrados en su fisiopatología.Presentamos el caso de una niña lactante de 21 meses con este síndrome secundario a un cuadro respiratorio viral. Tuvo ataxia, opsoclonus, mioclonías de miembros superiores, irritabilidad y alteración en el patrón de sueño. Se descartó el diagnóstico de neuroblastoma. El tratamiento inicial se hizo con pulsos de metilprednisolona seguida de prednisolona oral. Sus síntomas desaparecieron progresivamente y recuperó la marcha; actualmente está asintomática y sin secuelas. En estos pacientes se debe descartar siempre un síndrome paraneoplásico.

show abstract

Opsoclonus–myoclonus syndrome following rotavirus gastroenteritis

Cited by 15 publications

References 11 publications

Demographic, Clinical, and Immunologic Features of 389 Children with Opsoclonus-Myoclonus Syndrome: A Cross-sectional Study

Demographic, Clinical, and Immunologic Features of 389 Children with Opsoclonus-Myoclonus Syndrome: A Cross-sectional Study

Multifactorial analysis of opsoclonus‐myoclonus syndrome etiology (“Tumor” vs. “No tumor”) in a cohort of 356 US children

Síndrome de Kinsbourne: reporte de un caso

Contact Info

Product

Resources

About