Opsoclonus–myoclonus–ataxia syndrome in an HIV-infected child

Pereira, Noella Maria Delia; Shah, Ira; Kulkarni, Shilpa

doi:10.1093/omcr/omw077

Cited by 10 publications

(5 citation statements)

References 7 publications

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“…[8910] There has been a case report of immune reconstitution syndrome in a child with HIV infection presenting with OMS. [11] There were 7 (31%) children with postinfectious etiology in our group though no organisms could be isolated in them. One child in our study who had symptoms of recent infection and initially classified as a probable postinfectious etiology turned out to have tumor at evaluation.…”

Section: Discussionmentioning

confidence: 84%

Clinical profile, prognostic indicators, and therapeutic outcomes of pediatric opsoclonus-myoclonus-ataxia syndrome: A single-center experience from South India

Muthusamy

Thomas

Yoganathan

et al. 2019

Ann Indian Acad Neurol

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Background: Opsoclonus myoclonus syndrome (OMS) is a neuroinflammatory disorder. Indian literature on its clinical profile and outcome is sparse. Objectives: The objective of this study is to describe the clinical profile and analyze outcomes and prognostic predictors in a cohort of children with OMS. Materials and Methods: This was a retrospective study of children with OMS between 2007 and 2017. Results: Twenty-two children were included in the study. The mean age at onset of symptom was 20.9 months (standard deviation [SD]: 7.5). The mean duration of delay in diagnosis was 8.4 months (SD 1.26) with acute cerebellitis being the most common misdiagnosis. Eleven children (50%) were diagnosed with tumor during evaluation and follow-up and 11 children (50%) belonged to idiopathic/postinfectious group. Magnetic resonance imaging brain was normal in all children except for one revealing cerebellar atrophy on follow-up. One child in the paraneoplastic group (neuroblastoma) had a positive PNMA2/Ta onconeural antibody. Children in the tumor group had an earlier age of onset (mean 15.5 vs. 26.3 months), shorter time to onset of opsoclonus from initial symptom (2.54 vs. 7.27 weeks), and higher severity score at presentation (13.7 vs. 11.3) compared to the nontumor group. Children in the nontumor group attained their first remission with treatment earlier (10.9 weeks, SD: 4.5) than the children with tumor (18.72 weeks, SD: 5.8). There was no significant difference in the outcome between the groups. Children with multiple relapses (>3) and late surgical intervention for tumor (>6 months after symptom onset) had a poor outcome. Discussion: A high index of suspicion coupled with early diagnosis and periodic tumor surveillance (even in the initially negative cases) along with aggressive combined multimechanistic immunotherapies is the key in improving outcomes. Conclusion: A high index of suspicion in appropriate clinical circumstances and early aggressive immunomodulation might lead to a better outcome.

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Section: Discussionmentioning

confidence: 84%

Clinical profile, prognostic indicators, and therapeutic outcomes of pediatric opsoclonus-myoclonus-ataxia syndrome: A single-center experience from South India

Muthusamy

Thomas

Yoganathan

et al. 2019

Ann Indian Acad Neurol

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“…Even though rare, myoclonus as a clinical sign of neuronal injury has been described in HIV-infected individuals, It was linked to HIV itself in acute infection ( Denning, 1988 ), HIV-associated dementia ( Canafoglia et al, 2003 ), to HIV immune reconstitution syndrome ( Kanjanasut et al, 2010 , Pereira et al, 2016 ) or HIV viral escape in CSF ( Cabaraux et al, 2020 ). Neurological HIV-associated opportunistic infections such as cerebral toxoplasmosis ( Mattos et al, 2002 ), progressive multifocal leukoencephalopathy ( Bettoni et al, 1986 ), or brain tuberculosis ( Guedes et al, 2018 ) could also present with myoclonus.…”

Section: Discussionmentioning

confidence: 99%

Subacute myoclonic measles encephalitis – An opportunistic HIV-associated infection

Ene

Duiculescu

Rădoi

et al. 2023

Front. Cell. Neurosci.

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IntroductionAn unusual cluster of myoclonic epilepsy was observed in a Romanian pediatric HIV cohort concurrent with measles outbreaks. We describe this particular form of subacute measles encephalitis (SME) in a group of HIV-infected children and adolescents with severe immunosuppression.MethodsThis is a single-center study, starting in 1997 and covering 4 measles outbreaks in Romania. The presumptive diagnosis of subacute myoclonic measles encephalitis (SMME) was based on: (1) epidemiological data, previous measles episode or presumed contact with measles virus (MV), (2) clinical presentation with initial localized myoclonic jerks with rapid extension and subsequent motor deficit with preserved mental status, and (3) neuroimaging studies revealing cortical gray matter lesions. Definitive diagnosis was based on a neuropathological exam and immunohistochemistry of brain tissues, and measles RNA detection in the cerebrospinal fluid (CSF).ResultsThirty-six patients were diagnosed with a particular form of SME during consecutive measles outbreaks in Romania: 1996–1998 (22); 2005–2008 (12); 2010–2011 (1) and 2016-2018 (1). Most children were born in the late 80s and had parenterally acquired HIV infection in early childhood. Before the episode of SMME, 11 patients had confirmed measles, while the rest, without typical rash, had a respiratory tract infection and/or presumed previous measles contact. In all patients, the clinical onset was sudden, with unilateral myoclonus. MRI findings revealed mainly focal cortical gray matter lesions. Neurologic symptoms progressed rapidly to coma and death in most patients. Three patients survived SMME, they had higher CD4 count at onset, slower progression of neurological symptoms, and benefit of immune recovery with cART. Immunocytochemistry studies revealed MV in the brain with a pattern suggesting an ascending viral neural infection. MV was isolated from CSF in 7 out of 8 patients. Sequence analysis of MV RNA from both nasopharyngeal swabs and CSF was available for one patient with similar N-450 strain characteristics.ConclusionDuring an outbreak of measles, neurological manifestations, especially myoclonus in immunosuppressed patients, can be related to measles even in the absence of an acute episode. This particular form of subacute myoclonic measles encephalitis is an opportunistic fatal disease. Immune recovery due to effective antiretroviral treatment might increase survival.

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“…Apart from this, a rapid increase in CD4 cell counts may explain the appearance of OMS during HIV antiretroviral treatment. [ 22 – 24 ]…”

Section: Discussionmentioning

confidence: 99%

Opsoclonus-myoclonus syndrome with severe clinical course and beneficial outcome

Koziorowska-Gawron¹,

Koszewicz²,

Bladowska

et al. 2021

Medicine

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Rationale: Opsoclonus-myoclonus syndrome (OMS) is a rare immune-mediated movement disorder, mostly of paraneoplastic or idiopathic origin. The disease usually has an acute onset, serious course and leads rapidly to disability in adult patients. To the best of our knowledge, this is the fourth presented case of OMS with a severe course and complete reversibility of neurological symptoms in a pregnant woman. This report includes videos and a literature review. Patient concerns: A 30-year-old woman in the 12th week of pregnancy developed severe nausea and vomiting, after several days balance and gait disorders appeared. On admission to hospital, neurological examination revealed opsoclonus, dysarthria, myoclonic jerks with ataxia of the trunk and limbs with inability to sit, stand or walk. Diagnosis: Well-known causes of OMS were excluded. Although in our patient the idiopathic origin of the disorder was taken under consideration, diagnosis of opsoclonus-myoclonus related to the pregnancy was highly likely. Interventions: After administration of steroids and benzodiazepines the patient improved. Outcomes: In the 6th month of pregnancy, after termination of immunotherapy, she recovered completely and was able to sit, stand and walk independently. In the 39th week of pregnancy, she delivered a healthy child. Lessons: We confirm that understanding of clinical symptoms and rare causes of OMS contributes to early diagnosis and therapy, which ensures an optimal outcome. One probable cause of OMS could be a physiological change to immune system regulation during pregnancy. The relationship between OMS and pregnancy remains uncertain and needs further investigation.

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Opsoclonus–myoclonus–ataxia syndrome in an HIV-infected child

Cited by 10 publications

References 7 publications

Clinical profile, prognostic indicators, and therapeutic outcomes of pediatric opsoclonus-myoclonus-ataxia syndrome: A single-center experience from South India

Clinical profile, prognostic indicators, and therapeutic outcomes of pediatric opsoclonus-myoclonus-ataxia syndrome: A single-center experience from South India

Subacute myoclonic measles encephalitis – An opportunistic HIV-associated infection

Opsoclonus-myoclonus syndrome with severe clinical course and beneficial outcome

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