Shubhdeep Ahden1, Benjamin George1, Lawrence Samkoff1
1University of Rochester - Strong Memorial Hospital
Objective:
To present a case of opsoclonus-myoclonus-ataxia syndrome in an adult patient following Epstein Barr Virus infection.
Background:
Opsoclonus-myoclonus-ataxia syndrome (OMAS), also called disorder of dancing eyes and feet, is a condition that causes opsoclonus along with diffuse or focal body myoclonus and truncal titubation with or without ataxia and other cerebellar signs. It is commonly seen as a paraneoplastic syndrome in children with neuroblastoma; however, there are several associated post-infectious and paraneoplastic etiologies in children and adults.
Results:
A 26-year-old woman developed generalized malaise, abdominal discomfort, and right upper quadrant abdominal swelling. One week later, she developed blurred vision and gait impairment, for which she presented to the emergency room. On physical exam, she had opsoclonus, left appendicular ataxia, and left hemi-ataxic gait. Brain magnetic resonance imaging was normal. Serologic studies revealed elevated liver function tests and positive Epstein Barr Virus (EBV) IgM and IgG. Evaluation of cerebrospinal fluid revealed normal cells, glucose, and protein, negative oligoclonal bands as well as negative meningitis/encephalitis polymerase chain reaction panel and negative autoimmune encephalopathy panel. Workup for occult neoplasms was unrevealing. She was diagnosed with post-infectious OMAS due to EBV. She was treated with high-dose steroids with taper and intravenous immunoglobulin, which led to further improvement. Gabapentin, clonazepam, and topiramate were used for symptomatic treatment of opsoclonus.
Conclusions:
We describe clinical features, diagnostic workup, and treatment of a case of Epstein Barr Virus-associated post-infectious opsoclonus-myoclonus-ataxia syndrome in an adult. Despite recovery from the viral illness, the patient continued to have symptoms for several weeks which were ultimately improved with steroids and IVIG.