Opsoclonus–ataxia syndrome associated with ovarian mature teratoma

Abstract

A 16-year-old girl with no prior medical history developed vertigo and nausea following alimentary infection. Neurological examination showed limb and truncal ataxia, opsoclonus, myoclonus, and hyperreflexia. Brain magnetic resonance imaging and cerebrospinal fluid analysis showed no abnormalities. Treatment with i.v. high-dose methylprednisolone and immunoglobulin was started, but this proved ineffective. The clinical course was unusual, so whole-body computed tomography was done to evaluate other differential diagnoses. Imaging identified right ovarian mature teratoma. Paraneoplastic opsoclonus-ataxia syndrome was suspected, therefore single-incision laparoscopic ovarian cystectomy was done 10 days after admission. Two months after therapy, the patient had complete recovery and remained asymptomatic at 1 year after onset. Serum testing for anti-neuronal antibodies was negative, including for anti-N-methyl-d-aspartate-receptor antibody. Young women with ataxia and opsoclonus of unclear etiology should be examined for the presence of ovarian teratoma, then intensive immunotherapy and prompt tumor resection can lead to good clinical outcome.

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

“…This conundrum is not limited to NB and OMS. An apparent duality of causation also upends a uniform model of autoimmune encephalitis, in which ovarian teratoma—rare in OMS—is found in a percentage of females that increases with age: 9% in those < 14 years old, 30% in those < 18 years old, and 56% of those > 18 years old . In contrast, teratoma‐associated N ‐methyl‐D‐aspartate receptor (NMDAR) encephalitis has not been reported in young males, and testicular teratoma is rare in men.…”

Pediatric whole body MRI detects causative ovarian teratoma in opsoclonus myoclonus syndrome

“…While there have been many case reports of OMS as a paraneoplastic syndrome associated with many tumors such as breast cancer, melanoma, and nonsmall cell lung cancer, neuroblastoma is the most common cause in pediatric patients. In adolescent and adult females, ovarian teratomas have also been reported with some frequency [10,11]. In our patient, laparoscopic resection was accomplished promptly, with recovery not otherwise affecting her clinical picture.…”

“…As the main concern was detection of the lesion without exposure to ionizing radiation and superior soft tissue resolution of the sympathetic chain and adrenal glands, a whole body MRI was performed using a 3T magnet with STIR focused sequence. This sequence not only allows for excellent signal to background contrast ratio, but is also preferred to other fat suppression sequences for imaging across large body parts [6,10,16].…”

Clinical Reasoning: An adolescent girl presenting with worsening vertigo, headache, and ataxia

“…6 Cases of OMS associated with ovarian teratomas have been reported in both adolescents and adults. 7,8 The pathophysiology of OMS is thought to be autoimmune in nature. Though many patients do not have identified autoantibodies, those with gynecologic malignancies and OMS may test positive for ANNA-2 (Anti-Ri) antibodies.…”