Another promising treatment option for neuroblastoma-associated opsoclonus–myoclonus syndrome by oral high-dose dexamethasone pulse: Lymphocyte markers as disease activity

Oguma, Makiko; Morimoto, Akira; Takada, Ayako; Kashii, Yoshifumi; Fukuda, Tokiko; Mori, Masayuki; Yamagata, Takanori; Sugie, Hideo; Momoi, Mariko Y.

doi:10.1016/j.braindev.2011.04.005

Cited by 9 publications

(5 citation statements)

References 12 publications

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“…The reduced CD4 + T cells and CD4/CD8 ratio in the CSF of children with OMS 56,57 . partially supports the hypothesis of a neuroinflammatory process occurring.…”

Section: Immunopathogenesis Of Oms: Effects Of Antitumor Immunitysupporting

confidence: 71%

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Opsoclonus‐myoclonus syndrome associated with neuroblastoma: Insights into antitumor immunity

Cai

2022

Pediatric Blood & Cancer

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Opsoclonus‐myoclonus syndrome (OMS) is a rare neurological disorder. Half of these cases occur in children with neuroblastoma. Neuroblastoma patients with OMS usually have better oncological outcomes than those without OMS even after stratification by tumor stage and age, indicating that factors mediating OMS may also inhibit tumor cell proliferation. Although the mechanisms underlying OMS remain undefined, the cytokines and lymphocytes alterations in the cerebrospinal fluid support the concept that it is a pattern of neuroinflammation due to an autoimmune effect. The presence of lymphoid follicles consisting of follicular dendritic cells, CD20+ B lymphocytes, CD3+ T lymphocytes, and CD68+ macrophages in the tumor microenvironment in OMS‐associated neuroblastoma support the autoimmune nature of this disorder. This review focuses on the clinical and genetic features of OMS‐associated neuroblastoma, and we update readers on immune features of neuroblastoma with or without OMS to gain insights into antitumor immunity as it relates to tumor biology and prognosis.

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“…The reduced CD4 + T cells and CD4/CD8 ratio in the CSF of children with OMS 56,57 . partially supports the hypothesis of a neuroinflammatory process occurring.…”

Section: Immunopathogenesis Of Oms: Effects Of Antitumor Immunitysupporting

confidence: 71%

“…The reduced CD4 + T cells and CD4/CD8 ratio in the CSF of children with OMS. 56,57 partially supports the hypothesis of a neuroinflammatory process occurring. Accordingly, reductions in the CD4 + T-cell subset and CD4/CD8 ratio in the peripheral blood of children with OMS-associated neuroblastoma have also been reported.…”

Section: Amplification Of Omssupporting

confidence: 68%

Opsoclonus‐myoclonus syndrome associated with neuroblastoma: Insights into antitumor immunity

Cai

2022

Pediatric Blood & Cancer

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“…12 There are also anecdotal reports of benefit with topiramate, clonazepam, and oral high-dose dexamethasone pulses. 13–15…”

Section: Discussionmentioning

confidence: 99%

Clinical Profile and Outcome of Children With Opsoclonus-Myoclonus Syndrome

et al. 2013

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The opsoclonus-myoclonus syndrome is a distinct disorder characterized by opsoclonus, myoclonus, and ataxia, along with marked irritability and behavioral changes. Worldwide, data on its epidemiology, clinical features, and outcome are scarce. The aim of the study was to determine the clinical profile and outcome of children with this disorder. A retrospective study of all children admitted with a diagnosis of opsoclonus-myoclonus from 2000 to 2012 was done. Outcome was assessed on follow-up by direct assessment and by telephonic interview. Eleven patients with a diagnosis of opsoclonus-myoclonus were admitted over a 12-year period. Of the 11, 4 had paraneoplastic etiology. Children with paraneoplastic opsoclonus had more relapses and a poor outcome as compared to an idiopathic group. Paraneoplastic opsoclonus had a poor outcome in our experience.

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“…In some cases whereby OMAS is associated with neuroblastoma, tumor resection has led to the alleviation of symptoms, but, in others, the symptoms persisted [ 30 , 37 , 55 ]. Adrenocorticotropic hormone (ACTH) [ 54 ], steroid therapies [ 56 , 57 ], and IVIG are commonly used in the treatment of OMAS. A randomized controlled trial of 53 participants demonstrated a significantly superior response in patients who were given a combination of IVIG and prednisolone as compared to prednisolone alone [ 58 ].…”

Section: Reviewmentioning

confidence: 99%

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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Another promising treatment option for neuroblastoma-associated opsoclonus–myoclonus syndrome by oral high-dose dexamethasone pulse: Lymphocyte markers as disease activity

Cited by 9 publications

References 12 publications

Opsoclonus‐myoclonus syndrome associated with neuroblastoma: Insights into antitumor immunity

Opsoclonus‐myoclonus syndrome associated with neuroblastoma: Insights into antitumor immunity

Clinical Profile and Outcome of Children With Opsoclonus-Myoclonus Syndrome

Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients

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