The Double Whammy—Opsoclonus–Myoclonus–Ataxia Syndrome and COVID-19

• DOI: 10.4103/aian.aian_1110_21
• Publication Date: 2022-04-06T09:52:12Z
• Authors: Revathy Vijayarag...; Vijayashankar Par...; Muthukani Sankara...; Madhuri Kankanala; Pendem Hima Bindu; Safinaaz; Neetu Mariam Alex
• Keywords: Opsoclonus; Neurological examination; Myoclonic Jerk; Prednisolone

Since the emergence of coronavirus disease 2019 (COVID-19), a plethora neurological syndromes and complications have been unravelled. Opsoclonus–myoclonus–ataxia syndrome (OMAS) is rare disorder that was first described in 1962 infants by Kinsbourne much more common children than adults. The diagnosed presence three out following four clinical manifestations: opsoclonus; myoclonus and/or ataxia; behavioural changes sleep disturbances; tumours or anti-neuronal antibodies.[1] While paraneoplastic OMAS common, parainfectious has also reported with various infections. We hereby report case postinfectious COVID-19 infection. A 64-year-old woman developed infection presented fever breathlessness. She treated oral steroids minimal oxygen for 5 days. diabetic had undergone renal transplantation 20 years ago. Her function stable prednisolone 10 mg mycophenolate mofetil. On day 11 after recovery from COVID-19, she altered behaviour irrelevant talks followed one episode generalized seizure. examination, disoriented no focal signs. Random blood sugar 600 mg/dL. Laboratory workup revealed normal liver parameters, electrolytes urine ketones. started on levetiracetam insulin infusion. Two days later, severe myoclonic jerks involving all extremities trunk worsened stimulus such as sound movements. marked multidirectional, high amplitude fast frequency, irregular conjugate eye movements suggestive opsoclonus. speech slurred associated ataxia limbs [https://links.lww.com/AIAN/A7]. Magnetic resonance imaging (MRI) brain cerebrospinal fluid (CSF) analysis were normal. intravenous (methylprednisolone 1 gm/day days). detailed antibodies (ANNA-1, ANNA-2, PCA-1, MA2, CV2, amphiphysin, SOX1, recoverin, titin, Zic4, GAD65, Tr) autoimmune (NMDA, AMPA, GABA-B, LGI-1, CASPR2) serum CSF negative. Whole-body FDG-PET CT moderate improvement opsoclonus pulse steroids. Subsequently few her OMA symptoms became drowsy. Hence, immunoglobulin (400 mg/kg/day) rituximab gm responded well, completely improved follow-up 2 weeks [https://links.lww.com/AIAN/A8]. Our patient acute onset opsoclonus–myoclonus–ataxia during phase Other causes like paraneoplastic, drugs considered, but autoimmune, PET scan whole body Even though hyperglycaemia are risk factors to trigger myoclonus, sudden temporal course favourable aetiology. adults commonly malignancies small cell lung carcinoma breast, ovarian nasopharyngeal carcinoma. Idiopathic patients less 40 better prognosis.[2] should not preclude any presenting OMAS. To best our knowledge, 16 cases COVID-19-related myoclonus–ataxia published literature. All them mild-to-moderate pneumonia except who Neurological symptom ranged early 3 post-COVID-19 up 6 weeks. Nine components OMAS, opsoclonus, ataxia. Confusion two cases. analysis. Paraneoplastic antibody conducted patients, it Ten IVIg, which received both IVIg steroid. One alone. Twelve significant immunotherapy.[34] Exact pathogenesis known. probable mechanism behind an immune-mediated dysfunction cerebellum brainstem.[5] Inflammatory activation nervous system unidentified SARS-CoV-2 antigens could be cause symptomatology. transient response systemic steroid therapy antiepileptic drugs; however, remarkable rituximab. In conclusion, complication initiation aggressive immunotherapy may provide complete remission. Informed written consent authors attest necessary permission papers acquired. consented form photographs other information journal. aware name initial will every effort taken keep identity hidden, anonymity cannot guaranteed. Financial support sponsorship Nil. Conflicts interest There conflicts interest. Acknowledgements thank Ms. Greeshma, Research Assistant, DME Office, Apollo Hospitals, Chennai, helping publishing this report.