Dancing Eyes, Dancing Feet

Badaki, Oluwakemi; Schapiro, Emily

doi:10.1097/pec.0b013e31815c9d66

Cited by 6 publications

(9 citation statements)

References 15 publications

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“…Later, neuropsychological sequellae, such as attention-deficit disorder, learning and memory disorders, and cognitive impairment, may emerge ( 6 , 7 ). The challenge is to consider OMS early in the differential diagnosis ( 17 ), exclude look-alike diseases ( 4 , 18 – 20 ), and shorten the time to treatment ( 12 ). Through such measures, the quality of life for children with OMS can be improved and the burden of chronic disease reduced.…”

Section: Introductionmentioning

confidence: 99%

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

2017

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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.

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Section: Introductionmentioning

confidence: 99%

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

2017

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“…An atypical presentation of OMAS can lead to a misdiagnosis of cerebellar ataxia or acute cerebellitis [ 37 , 38 , 39 ], leading to a delay in diagnosis. This delay averages about 8.4 months after the initial presentation [ 40 ].…”

Section: Reviewmentioning

confidence: 99%

“…Routine tests such as a CBC, renal and liver function tests, clotting studies, and inflammatory markers (erythrocyte sedimentation rate and C reactive protein) are typically obtained [ 8 ]. A lumbar puncture followed by the analysis of CSF is also usually performed, which can show elevated white blood cell counts and the presence of oligoclonal bands [ 1 , 37 , 45 ]. Recent studies have also shown B-cell expansion in the CSF of OMAS patients [ 46 , 47 ].…”

Section: Reviewmentioning

confidence: 99%

Section: Reviewmentioning

confidence: 99%

“…To screen OMAS patients for neuroblastoma, imaging modalities such as whole-body CT, MRI, or MIBG scintigraphy should be applied [ 37 , 49 ]. Urine catecholamine metabolites and specific autoantibodies associated with OMAS and neuroblastoma can also be measured [ 8 , 37 ]. Brunklaus et al reported that CT/MRI imaging of the chest and abdomen was the most accurate tool to detect occult neuroblastoma, while MIBG and urine catecholamines were less sensitive, especially in tumors with low metabolic activity [ 50 ].…”

Section: Reviewmentioning

confidence: 99%

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Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients

Hsu,

Tejani,

Shah

et al. 2024

Children

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Opsoclonus-myoclonus ataxia syndrome (OMAS), also known as Kinsbourne syndrome, is a rare disorder that presents with myoclonus, ataxia, abnormal eye movements, irritability, and sleep disruptions, often in young children. We report a case of an infant barely 6 months old, with no significant past medical history, who presented to the emergency department with tremors, jerking motions of the head and arms, and rapid eye movements. After an extensive workup, she was found to have a neuroblastoma, which was subsequently surgically removed via thoracotomy. Despite an initial improvement in symptoms post-resection, the patient’s symptoms recurred. She was subsequently treated with dexamethasone, intravenous immunoglobulin (IVIG), and rituximab. After treatment, the patient was noted to have mild global developmental delays but was otherwise well. This case report highlights the rare occurrence of OMAS in an infant barely 6 months old at diagnosis. Using the PubMed database, a systematic review was conducted to highlight the clinical presentation, diagnosis, and management of OMAS.

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