NMDA receptor antibody in teratoma-related opsoclonus-myoclonus syndrome

Urriola, Nicolás; Helou, Jacob; Maamary, Joel; Pogson, Jacob M.; Lee, Frederick; Parratt, Kaitlyn; Gillis, David; Fulham, Michael; Hálmagyi, G. Michael

doi:10.1016/j.jocn.2018.10.011

Cited by 9 publications

(3 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Together, the constellation of an immunotherapyresponsive brainstem-cerebellar syndrome in the setting of a teratoma points strongly to an immune-mediated paraneoplastic process, although specific antibody-antigen characterization has yet to be elucidated. 8,9 This literature review illustrates that our patient's presentation fits within the framework of published cases of opsoclonus associated with ovarian teratoma, and that the prognosis with immunosuppressive therapy is favorable. From bench to bedside: neuroimmunology and opsoclonus-ataxia syndrome Flow cytometry analysis of our patient's CSF provides an interesting perspective on future management.…”

Section: Ovarian Teratoma-associated Opsoclonussupporting

confidence: 61%

Clinical Reasoning: A 16-year-old girl with ataxia, oscillopsia, and behavioral changes

et al. 2020

View full text Add to dashboard Cite

A 16-year-old girl with no relevant medical history presented with 1 week of vertigo, tremulousness, and gait instability. She described the room as "moving and unsteady." She reported anxiety and had a panic attack in the emergency department. In the months prior to presentation, she displayed atypical irritability and aggressiveness toward her siblings. One week before the onset of vertigo, she completed a 5-day course of azithromycin for presumed sinusitis. She had not traveled in the prior year. The patient denied drug or alcohol use, sexual activity, sick contacts, or any recent tick or mosquito bites. On examination, the patient had slow, soft, and deliberate speech but was alert and oriented to person, place, time, and situation. She had intact naming, comprehension, and repetition. She was able to register and recall 3/3 words. Her ocular ductions were full. However, there were constant, rapid eye movements in both horizontal and vertical directions, mostly observed in primary gaze and with fixation. The movements were conjugate and of moderate amplitude and high frequency (video 1, part 1). There was no slow phase. Dix-Hallpike and head impulse testing were negative. There was no intention tremor, dysdiadochokinesia, or dysmetria on initial examination, although the patient later developed bilateral dysmetria on day 3 of her hospitalization. She had prominent truncal ataxia, which limited gait assessment. The remainder of her examination was unremarkable. Questions for consideration: 1. How would you localize this patient's presentation? 2. How would you characterize her eye movements? GO TO SECTION 2

show abstract

Section: Ovarian Teratoma-associated Opsoclonussupporting

confidence: 61%

Clinical Reasoning: A 16-year-old girl with ataxia, oscillopsia, and behavioral changes

et al. 2020

View full text Add to dashboard Cite

show abstract

“…OMS is a rare syndrome characterized by opsoclonus, which is spontaneous, irregular, multivectorial saccadic eye movements, along with focal or diffuse myoclonus and sometimes ataxia ( 55 ). In addition to KLHL antibodies as described above, one case of anti-NMDAR antibody-positive OT-associated OMS has been reported ( 29 ). In a large series with OMS (n = 114), 39% (45/114) of patients had paraneoplastic OMS.…”

Section: The Neurological Syndromes Of Ot-related Pnssmentioning

confidence: 95%

Ovarian Teratoma-Related Paraneoplastic Neurological Syndromes

Lin

Wang

2022

Front. Oncol.

View full text Add to dashboard Cite

Paraneoplastic neurological syndromes (PNSs) are a group of neurological disorders triggered by an underlying remote tumor. Ovarian teratoma (OT) is the most common histologic type of germ cell tumor in females. The most common PNSs associated with OT is anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. However, with the increasing number of new antibodies reported over the last decade, the clinical spectrum of OT-related PNSs is also expanding. Our knowledge of OT-related PNSs is still far from complete. Here, we provide a comprehensive review of the most recent findings in the field of OT-related PNSs, with a particular focus on their clinical and pathological characteristics. Overall, the description of neuronal antibodies in PNSs associated with OT strongly suggests that antibodies may be responsible for the clinical symptoms in some cases. OT-related PNSs are associated with various clinical manifestations, including anti-NMDAR encephalitis, limbic encephalitis, encephalomyelitis, progressive cerebellar syndrome and opsoclonus-myoclonus syndrome. The pathological characteristics of the OT suggest that the mechanism of PNSs is probably due to heteromorphic neurons in the tumor tissue, the ectopic expression of the antigens in neural tissue within the teratomas and patients’ unusual immune response. Despite the severity of the neurological syndromes, most patients with OT-related PNSs showed good neurologic response to early tumor resection combined with immunotherapy. To further advance the management of OT-related PNSs, additional studies are needed to explore this complex topic.

show abstract

“…Most commonly observed in the pediatric population, this syndrome and constellation of symptoms is often associated with neuroblastoma [3]. In rare cases, other lesions such as a benign teratoma can lead to OMS, which can be reversed with tumor removal [1,4,5]. Therefore, prompt detection and treatment of these tumors is paramount to optimal outcome.…”

Section: Introductionmentioning

confidence: 99%

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

Park

Aljabban

Fanburg‐Smith

et al. 2020

Radiology Case Reports

View full text Add to dashboard Cite

Opsoclonus Myoclonus Syndrome (OMS, or Opsoclonus Myoclonus Ataxia) is a rare condition that presents with saccadic movements of the eyes, cerebellar ataxia, and choreiform movements of the limbs. While previous reports have described the use of ultrasound, CT, FDG-PET and traditional focused MRI for localization of OMS-associated masses, whole body MRI has not previously been reported for this purpose. Here we describe a 16-year-old patient who exhibited OMS and underwent whole body MRI to rule out the more commonly associated neuroblastoma. An ovarian mass was discovered, resected, and pathology confirmed benign teratoma - there was subsequent resolution of symptoms after complete surgical resection. Whole body MRI should be considered in pediatric cases of OMS due to the paraneoplastic nature of the disease with associated tumor, high sensitivity of disease detection, lack of ionizing radiation, excellent tissue resolution and demonstrated effectiveness in pediatric imaging.

show abstract

NMDA receptor antibody in teratoma-related opsoclonus-myoclonus syndrome

Cited by 9 publications

References 8 publications

Clinical Reasoning: A 16-year-old girl with ataxia, oscillopsia, and behavioral changes

Clinical Reasoning: A 16-year-old girl with ataxia, oscillopsia, and behavioral changes

Ovarian Teratoma-Related Paraneoplastic Neurological Syndromes

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

Contact Info

Product

Resources

About