Category: Clinical Sciences/Health Conditions
16-year-old female presented with one week history of vertigo, gait instability, tremulousness, abnormal, eye movement, tremor, and anxiety. Imaging showed right ovarian teratoma, underwent right salpingo-oophorectomy and subsequently received IVIG, pulse steroid and Rituximab. Patient was admitted to inpatient rehabilitation with the diagnosis of NMDA-R negative opsoclonus-myoclonus-ataxia syndrome with marked improvement of symptoms and function.
The child with history of pseudoseizure since 5 years of age, was off medication and seizure free for 8 years, initially presented with vertigo, gait instability, tremulousness, abnormal eye movement (ocular flutter), tremor, weakness, pruritus, anxiety and panic attack. Whole body MRI work up revealed right ovarian teratoma. CSF study showed pleocytosis and elevated protein. She underwent right salpingo-oophorectomy and subsequently received IVIG, pulse steroid and Rituximab with some improvement of symptoms. On rehab admission, physical examination showed hypertension, impaired recall and cognition, vocal tremor, increased vocal pitch, rapid bilateral horizontal nystagmus worsened with focusing, titubation worsening on sitting or standing, improved on lying, dysmetria with heel shin test and finger nose test in late phase of trajectory, brisk patellar reflex in left knee, left ankle clonus, ataxic gait. Motor strength was 5/5 in all extremities. Patient’s Berg balance scale improved from 18/56 to 46/56.
Our case showed marked improvement of function and symptoms after tumor resection, treatment with IVIG, pulse steroid and rituximab along with 6 weeks of acute inpatient rehabilitation in a low stimulation environment setting. She was discharged with rolling walker, supervision in ambulation, stair negotiation with one hand rail, coordination of extremities without loss of balance, no titubation or ataxia.
Opsoclonus-myoclonus-ataxia syndrome is a rare clinical condition which may have better prognosis in NMDA-R antibody negative ovarian teratoma cases if combined with early whole body MRI work up, tumor resection, medical treatment and intensive rehabilitation therapy.