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

Abstract

Opsoclonus-myoclonus-ataxia (OMA) syndrome is a debilitating autoimmune neurological disorder. Post-infectious opsoclonus-myoclonus-ataxia syndrome has been described with varying bacterial, spirochetal, and viral infections including several patients with HIV. However, specific immunopathological mechanisms that may lead to opsoclonus-myoclonus in HIV-positive patients are unknown.We report a case of HIV-associated opsoclonus-myoclonus and early HIV infection. A review of published literature shows opsoclonus-myoclonus can occur during early infection, in immune reconstitution syndrome or in association with other infections, especially tuberculosis.

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

“…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 …”

“…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.…”

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

“…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).…”

“…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].…”

“…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).…”