HIV-associated opsoclonus-myoclonus-ataxia syndrome: early infection, immune reconstitution syndrome or secondary to other diseases? Case report and literature review

Guedes, Bruno Fukelmann; Filho, Márcio A. A. Vieira; Listik, Clarice; Carra, Rafael Bernhart; Pereira, Cristiane B.; Silva, Emanuelle R. da; Gomes, Hélio Rodrigues; Vidal, José Ernesto

doi:10.1007/s13365-017-0603-3

Cited by 19 publications

(19 citation statements)

References 13 publications

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“…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%

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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

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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.

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Section: Introductionmentioning

confidence: 99%

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

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“…HIV-related OMAS has been observed so far in primo-infection, immune reconstitution or when another infection is associated (5). Eighteen case reports including ours have been reported [ (5,9); Supplementary Files]. Only one report has shown a high CSF compared to serum viral load despite HIV infection control under antiretroviral therapy (ART) in the serum (9).…”

Section: Discussionmentioning

confidence: 90%

“…We found no association reported between sinus thrombosis and OMAS. HIV-related OMAS has been observed so far in primo-infection, immune reconstitution or when another infection is associated (5). Eighteen case reports including ours have been reported [ (5,9); Supplementary Files].…”

Section: Discussionmentioning

confidence: 93%

“…Opsoclonus-Myoclonus-Ataxia syndrome (OMAS) is a rare auto-immune cerebellar disorder. The etiology is paraneoplastic, para-infectious, toxic-metabolic, or idiopathic (4)(5)(6). OMAS has been reported in association with HIV under three conditions: when the infection is newly diagnosed, during immune reconstitution or when another infection occurs (5).…”

Section: Introductionmentioning

confidence: 99%

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CSF HIV RNA Escape in Opsoclonus-Myoclonus-Ataxia Syndrome: Case Report and Review of the Literature

et al. 2020

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Background: Human immunodeficiency viruses (HIV) infection is associated with a broad range of neurological manifestations, including opsoclonus-myoclonus ataxia syndrome (OMAS) occurring in primary infection, immune reconstitution syndrome or in case of opportunistic co-infection.Case: We report the exceptional case of a 43-year-old female under HIV treatment for 10 years who presented initially with suspected epileptic seizure. Although the clinical picture slightly improved under anti-epileptic treatment, it was rapidly attributed to OMAS. The patient exhibited marked opsoclonus, mild dysarthria, upper limbs intermittent myoclonus, ataxia in 4 limbs, truncal ataxia, and a severe gait ataxia (SARA score: 34). The diagnostic work-up showed radiological and biological signs of central nervous system (CNS) inflammation and cerebral venous sinus thromboses. The HIV viral load was higher in cerebrospinal fluid (CSF) than in the blood (4,560 copies/ml vs. 76 copies/ml). She was treated for 5 days with pulsed corticotherapy. Dolutegravir and anticoagulation administration were initiated. Follow-ups at 2 and 4 months showed a dramatic improvement of clinical neurologic status (SARA score at 4 months: 1), reduction of CNS inflammation and revealed undetectable CSF and serum viral loads.Conclusion: This case underlines the importance of the evaluation of the CSF viral load in HIV patients developing OMAS and suggests CSF HIV RNA escape as a novel cause for OMAS.

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Neuro-Ophthalmologic Emergencies in Movement Disorders

Conway

Seay

Rucker

2021

Movement Disorder Emergencies

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HIV-associated opsoclonus-myoclonus-ataxia syndrome: early infection, immune reconstitution syndrome or secondary to other diseases? Case report and literature review

Cited by 19 publications

References 13 publications

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

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

CSF HIV RNA Escape in Opsoclonus-Myoclonus-Ataxia Syndrome: Case Report and Review of the Literature

Neuro-Ophthalmologic Emergencies in Movement Disorders

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