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Opsoclonus-myoclonus syndrome (OMS) is a rare neuroinflammatory disorder characterized by ataxia, opsoclonus, and myoclonus. Clinical diagnosis of OMS has been challenging; therefore, we sought to determine the clinical and treatment profiles of patients with OMS at the largest pediatric hospital in Latin America. We analyzed the data of patients diagnosed with OMS between 2010 and 2020 at Pequeno Principe Hospital (Brazil) to determine the corresponding clinical profile more accurately. Of the approximately 50,000 visitors to our pediatric neurology department from 2010 to 2020, 10 patients with OMS were observed. Five nontumor cases included three parainfectious and two idiopathic cases. The median time from symptom onset to diagnosis was 34 days. All patients with diagnostic OMS criteria in the idiopathic, nontumor group underwent whole-exome sequencing, with potentially pathogenic mutations identified in two cases. Nine patients were treated with methylprednisolone pulse, followed by oral steroids; eight received one or more intravenous immunoglobulin treatments; and six received azathioprine and cyclophosphamide. Complete symptomatic recovery was observed in only one patient. OMS diagnosis remains challenging. Diagnostic suspicion is necessary to improve the management of these patients and allow early immunosuppressive treatment. Paraneoplastic etiology is the most prevalent. In idiopathic patients who do not respond to immunosuppressive treatment, tests, such as whole-exome sequencing, may reveal a differential diagnosis. Genetic alterations that increase the risk of tumors may be an important clue to the pathophysiology of OMS. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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.

Kinsbourne syndrome or opsoclonus-myoclonus syndrome (OMS), also called dancing feet syndrome, manifests in opsoclonus, myoclonus, and ataxia. It occurs more frequently in pediatric patients, and around 50% of cases are caused by neuroblastoma (NBL). This investigation aimed to determine the incidence of OMS in children diagnosed with neuroblastic tumors. Data collected from 2004 to 2019 at the Department of Pediatric Oncology, Hematology, and Transplantology of Poznań University of Medical Sciences (Poznań, Poland) were analyzed. The research group included 119 patients under 18 years of age. There were only 3 cases (2.52%) of OMS in the research group, all younger than 3 years. The tumor was localized in the adrenal gland in 1 patient and paravertebrally in the other 2. The presented symptoms included nystagmus, balance disturbances, and tremors. All cases of OMS in children require imaging to determine whether it is caused by NBL. However, among patients with NBL, OMS is a rare manifestation.

Opsoclonus-myoclonus syndrome is a rare neurological condition characterized by opsoclonus, myoclonus, ataxia, irritability, and sleep disturbances. In pediatric patients, symptoms usually start between 16 and 18 months of age; opsoclonus-myoclonus syndrome presentation in children under 6 months is rare. Approximately 50% of cases are associated with neuroblastoma. We report an early onset presentation of opsoclonus-myoclonus syndrome in a previously healthy, 3-month-old female infant. The diagnostic workup revealed no abnormalities. The patient underwent monthly cycles of dexamethasone pulses and intravenous immunoglobulin with a favorable response. After a few months, the patient presented intermittent opsoclonus before the next scheduled pulse so from the 9th cycle onwards, the intravenous immunoglobulin dose was increased to 2 g/kg. After 9 months of treatment, she was diagnosed with a latent Mycobacterium tuberculosis infection. Due to this infection, dexamethasone pulses were discontinued, and intravenous immunoglobulin treatment was maintained with clinical improvement The patient received 18 intravenous immunoglobulin cycles, leaving her with a score of one on the Mitchell-Pike scale. Developmental milestones have been attained according to age. Despite the range of therapeutic options for managing opsoclonus-myoclonus syndrome described in the literature, the efficacy of these available therapies needs to be better established. A modified upfront approach with dexamethasone and intravenous immunoglobulin could be an option in settings where rituximab is unavailable., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

BACKGROUND: Opsoclono-myoclonus syndrome or Kinsbourne syndrome is a rare neurological disorder in children, often associated with occult neuroblastoma. This can also be triggered by infections and is often associated with medium to long-term sequelae, the severity of which will depend on the delay in diagnosis and initiation of treatment. CLINICAL CASE: We present the case of a 22-month-old infant who presented with opsoclonus-myoclonus-ataxia. At first, the diagnosis of Enterovirus rhomboencephalitis was established and later of adrenal neuroblastoma. CONCLUSION: Opsoclonus-myoclonus syndrome represents a diagnostic challenge for pediatricians. The appearance of this paraneoplastic neurological syndrome requires the search for a hidden neuroblastoma to promptly treat a life-threatening event such as the tumor and to prevent the neurological sequelae related to this syndrome. [ABSTRACT FROM AUTHOR]

Background: Opsoclonus-myoclonus syndrome (OMS) is an immune-mediated disorder that affects the central nervous system. It is recognized as a paraneoplastic syndrome highly associated with neuroblastic tumors. In Mexico, accurate epidemiological data are lacking and there are no well-structured protocols aimed at standardizing a treatment regimen in patients with OMS. Aim: This study aims to describe the clinical features, etiological factors, and treatment related to OMS in Mexican children. Patients and Methods: A retrospective study was performed over a 10-year period (2006-2016), using the database of the "Hospital Infantil de México Federico Gómez". The patients were divided into two groups (tumor vs. no tumor) to determine if there were significant demographic differences between the subgroups. The demographic variables were compared between groups using the Student's t-test. Results: Eleven patients were identified. The median age at the onset of the first clinical manifestation was 23 ± 14 months and the median age at diagnosis was 26 ± 15 months. The time delay between the onset of symptoms and diagnosis was approximately 3 months. Conclusion: In Mexico, there is a paucity of experience regarding the clinical presentation of OMS, and due to the infrequency of this condition, it is rarely diagnosed in our population since most pediatricians are unaware of the existence of the syndrome. The findings of this paper are consistent with the previous studies in terms of a closely equal sex ratio, predominance of Stage I tumors, a greater number of female patients with a paraneoplastic etiology, high relapse rates, and frequent neuropsychiatric morbidity. [ABSTRACT FROM AUTHOR]

Kinsbourne syndrome, also known as “Opsoclonus-myoclonus syndrome” or “Dancing eyes syndrome” is a rare condition characterized by rapid, irregular, multi-directional eye movements (opsoclonus), myoclonic movements in the trunk, face and/or limbs and ataxia. It occurs in children aged between 6 and 36 months. Its etiology may be paraneoplasic (neuroblastoma), non-paraneoplasic (infectious processes) or idiopathic. Regardless of its etiology, immunosuppressive drugs have been used in order to reduce the formation of antibodies possibly involved in the pathophysiology. We report the case of a 21 month-old girl with this syndrome secondary to an infectious respiratory illness. She had ataxia, opsoclonus, upper limbs myoclonus, irritability and altered sleep pattern. Neuroblastoma was ruled out. Initial management was done with methylprednisolone pulses, followed by oral prednisolone. She had progressive clinical improvement, and is currently asymptomatic with no sequelae. In these patients a paraneoplasic syndrome should always be ruled out.

Kinsbourne syndrome is a rare neurological paraneoplastic syndrome associated with neuroblastic tumors. There are very few literatures on its anesthetic management and interaction with anesthetic agents. The epileptogenic potential of certain anesthetic agents such as ketamine, etomidate, and meperidine might trigger opsoclonus and myoclonus and have an impact on the long-term neurological outcome. The objective of this case report is to discuss the safety of anesthetic agents and their relationship in a patient with Kinsbourne syndrome. We discuss our experience in the anesthetic management of a child with Kinsbourne syndrome with ganglioneuroblastoma in the thoracic paravertebral space.

Opsoclonus myoclonus ataxia syndrome (OMAS) is a rare inflammatory neurological disorder characterized by ocular, motor, behavioral, language, and sleep disturbances. It usually affects infants and young children but may affect adults. A 28-year-old male was brought to our emergency ward with complaints of involuntary spontaneous eye movements and jerky movements of limbs with imbalance while walking. He had a history of short febrile illness 10 days prior. His magnetic resonance imaging (MRI) of the brain, cerebrospinal fluid (CSF) analysis, and other routine investigations were normal. The patient was treated with injectable methylprednisolone (1 g) given for five days along with other supportive therapy. A significant reduction in the opsoclonus, myoclonus, and ataxia was seen on a six-month follow-up. OMAS should be identified early to avoid the use of inappropriate medications, and immunotherapy must be provided as early as possible in order to prevent irreversible neurological damage., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Shah et al.)

Opsoclonus-myoclonus syndrome (OMS), also known as Kinsbourne syndrome or dancing eyes syndrome, is an extremely rare neurological condition that comprises a heterogenous constellation of symptoms including opsoclonus along with diffuse or focal body myoclonus. It is usually referred to as a paraneoplastic entity, but it may also be associated to an infectious, metabolic, or idiopathic cause. Small-cell carcinoma of the lung is the most commonly reported malignancy associated with OMS. The authors describe a case of a 69-year-old male that presented with ataxic gait, phono- and photophobia, vertigo, dizziness, lethargy, nausea, and vomiting. During examination, rapid, multidirectional eye movements; slight dysarthria; and facial myoclonus were noted. He was admitted to the hospital, and after a thorough study, a diagnosis of OMS was established. Intravenous corticosteroids were started, alongside physiotherapy, and a slight improvement of his symptoms was noted. Imaging revealed a suspicious lesion in the left lung, along with lymphadenopathies and bone metastases. Histology confirmed the diagnosis of stage IV small-cell lung cancer (SCLC). Chemotherapy (ChT) with carboplatin and etoposide was started, and a gradual improvement of his neurological complaints was noted. After six cycles, the disease progressed, and second-line ChT with topotecan was started. After two cycles, the patient experienced significant clinical deterioration and eventually died. In conclusion, OMS is a poorly understood condition with uncertain neurological prognosis. The treatment of the primary neoplasm may improve neurological symptoms. The recognition of paraneoplastic syndromes is of utmost importance since early diagnosis of a malignancy relates to better outcomes., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Moreira et al.)

The article presents a case study of a 20-month old female infant who underwent insertion of an implanted central venous access device for the administration of intravenous immunoglobulin (IVIg) and cyclophosphamid. It indicates that the infant was diagnosed with opsoclonus-myoclonus syndrome (OMS). It indicates that the patient did not suffer any adverse effects during the surgical procedure which was maintained with anesthesia.

To test the efficacy and safety of corticotropin-based immunotherapies in pediatric opsoclonus-myoclonus syndrome, 74 children received corticotropin alone or with intravenous immunoglobulin (groups 1 and 2, active controls); or both with rituximab (group 3) or cyclophosphamide (group 4); or with rituximab plus chemotherapy (group 5) or steroid sparers (group 6). There was 65% improvement in motor severity score across groups (P < .0001), but treatment combinations were more effective than corticotropin alone (P = .0009). Groups 3, 4, and 5 responded better than group 1; groups 3 and 5 responded better than group 2. The response frequency to corticotropin was higher than to prior corticosteroids (P < .0001). Fifty-five percent had adverse events (corticosteroid excess), more so with multiagents (P = .03); and 10% had serious adverse events. This study demonstrates greater efficacy of corticotropin-based multimodal therapy compared with conventional therapy, greater response to corticotropin than corticosteroid-based therapy, and overall tolerability. [ABSTRACT FROM AUTHOR]

Opsoclonus-myoclonus syndrome is characterized by abnormal lymphocyte trafficking into brain. The authors hypothesized that mycophenolate mofetil, a lymphocyte proliferation inhibitor, might be therapeutic. The cerebrospinal fluid and blood immunophenotypes of 15 children with predominantly chronic-relapsing opsoclonus-myoclonus syndrome were compared before and after treatment by flow cytometry. Mycophenolate mofetil reduced the cerebrospinal fluid expansion of HLA-DR+ activated T cells (-40%); the frequency of other T-cell or natural killer cell subsets remained unchanged, but cerebrospinal fluid B cells increased significantly. Adrenocorticotropic hormone dose was lowered by 64% over an average of 1.5 years, yet 73% eventually relapsed despite therapeutic drug levels. Prior treatment with rituximab prevented relapse-associated increase in cerebrospinal fluid B cells, without hindering mycophenolate mofetil-induced reduction in T-cell activation. These data demonstrate resistant immunologic problems in chronic-relapsing opsoclonus-myoclonus syndrome. Mycophenolate mofetil did not prevent relapse. The novel effect of mycophenolate mofetil on chronically activated T cells may contribute to its efficacy in T-cell mediated neurological disorders. [ABSTRACT FROM AUTHOR]

Presents several abstracts of various pediatric research in 2005. "Prevalence of Scalp Scaling in Prepubertal Children," by Judith V. Williams; "Severity of Respiratory Syncytial Virus Bronchiolitis is Affected by Cigarette Smoke Exposure and Atopy," by Joseph P. Bradley; "Frequency of Infective Endocarditis Among Infants and Children With Staphylococcus aureus Bacteremia," by Anne Marie Valente.

Opsoclonus-myoclonus syndrome (OMS) is a rare, autoimmune neurological disorder that is poorly recognized and undertreated. Neuroblastoma is found in one half of the cases. Because of the high incidence of spontaneous regression of neuroblastoma, it is unknown whether not finding a tumor means there was none. To define demographic trends and the standard of care in the first large series of OMS, 105 children were recruited over a 13-year period in a retrospective questionnaire survey. Children with and without a tumor differed little in viral-like prodrome and neurological symptoms. Earliest neurological symptoms were staggering and falling, leading to a misdiagnosis of acute cerebellitis. Later symptoms included body jerks, drooling, refusal to walk or sit, speech problems, decreased muscle tone, opsoclonus, and inability to sleep. Tumor resection alone did not provide adequate therapy for most. Adrenocorticotropic hormone (ACTH), prednisone, and intravenous immunoglobulin were used with equal frequency, but ACTH was associated with the best early response. More than one half of the children had relapses. Residual behavioral, language, and cognitive problems occurred in the majority. The delay in diagnosis (11 weeks) and initiation of treatment (17 weeks) is unacceptably long. [ABSTRACT FROM PUBLISHER]

Opsoclonus-myoclonus syndrome is a rare neurological disorder. In children, the etiology varies, although it is a paraneoplastic manifestation (mainly of neuroblastoma) in 40% to 80% of cases. Whole-body MRI promises to be a powerful tool in the search for a possible primary tumor in this condition for which the diagnostic algorithm is yet to be established. We present the case of a two-year-old boy with signs of opsoclonus-myoclonus syndrome in whom a retroperitoneal neuroblastoma was detected by whole-body MRI., (Copyright © 2012 SERAM. Published by Elsevier Espana. All rights reserved.)