好色先生

好色先生

Explore the latest content from across our publications
Join The AAN

Log In

Forgot Password?
Create New Account

Loading... please wait

Abstract Details

Identification of a Novel Phenotype of Opsoclonus-Myoclonus Syndrome (OMS) That Affects Teenagers and Young Adults and Responds to Treatment
Neuro-oncology
IN10 - (-)
004
Teratoma-associated encephalitis usually occurs with NMDAR antibodies. The spectrum of neurological symptoms in patients without these antibodies is unknown.
Review of our experience during the last 5 years with patients with teratoma-associated encephalitis without NMDAR antibodies. Immunological studies included rat brain immunohistochemistry, cultures of neurons, and cell-based assays.
Of 270 patients with encephalitis and teratoma, 36 (92% female) were negative for NMDAR antibodies. Of these, 22 (61%) developed a posterior fossa syndrome (ataxia, cranial nerve involvement, and/or OMS), 9 (25%) prominent psychiatric features with or without other neurological symptoms, 3 (8%) limbic encephalitis (1 with diencephalic dysfunction), and 2 other symptoms (seizures, decrease level of consciousness). Anti-neuronal antibodies were identified in 14 patients: 1 against Ma2 and 13 against neuronal cell-surface antigens of unknown identity. A notable finding was that 10 of 22 patients with posterior fossa symptoms had OMS. All patients with OMS were women with a median age of 27 years (range 15-32); in 7 patients neurological symptoms preceded tumor diagnosis. Six patients had CSF pleocytosis and increased protein concentration, and 3 had antibodies to unknown cell-surface antigens. In 9 patients, OMS showed complete response to tumor removal and/or immunotherapy, and in one patient a partial response.
This study reveals a novel phenotype of OMS that affects teenagers and young women, associates with ovarian teratoma, and responds to treatment better than previously known paraneoplastic OMS phenotypes (<3 year-old infants with neuroblastoma and older adults with cancer of the lung, breast or ovary). Other manifestations of teratoma-associated encephalitis without NMDAR antibodies include a posterior fossa syndrome and a disorder with predominant psychiatric symptoms. Teenagers and young women with new onset OMS should be examined for an underlying ovarian teratoma.
Authors/Disclosures
Thais Armangue, MD (IDIBAPS-HClinic)
PRESENTER 		Dr. Armangue has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. The institution of Dr. Armangue has received research support from ISCIII(Spanish institute of health) -PI21/00316, Marato TV3, La Caixa Research Foundadion, Pablove Foundation (689368), Torrons Vicens Foundation (PFNR0144), 2021 Invest AEP.
No disclosure on file
No disclosure on file
Francesc R. Graus, MD (IDIBAPS) Dr. Graus has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for MedLink. Dr. Graus has received intellectual property interests from a discovery or technology relating to health care.
Maarten J. Titulaer, MD, PhD (Erasmus Medical Center) The institution of Dr. Titulaer has received research support from Dutch Epilepsy Foundations (NEF 19-08). The institution of Dr. Titulaer has received research support from CSL Behring. The institution of Dr. Titulaer has received research support from UCB. The institution of Dr. Titulaer has received research support from Netherlands Organisation for Scientific Research (ZonMW, Memorabel initiative and E-RARE UltraAIE) . The institution of Dr. Titulaer has received research support from Horizon Therapeutics / Amgen. The institution of Dr. Titulaer has received research support from Dioraphte (charity). The institution of Dr. Titulaer has received research support from Guidepoint Global LLC. The institution of Dr. Titulaer has received research support from ArgenX. Dr. Titulaer has received publishing royalties from a publication relating to health care.
Richard J. Barohn, MD, FAAN (University of Missouri) Dr. Barohn has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for NuFactor. The institution of Dr. Barohn has received research support from FDA OPD R01. Dr. Barohn has received intellectual property interests from a discovery or technology relating to health care.
Josep O. Dalmau, MD, PhD, FAAN Dr. Dalmau has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astellas Research Institute of America. Dr. Dalmau has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen Research & Development . Dr. Dalmau has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for 好色先生. An immediate family member of Dr. Dalmau has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer Nature. The institution of Dr. Dalmau has received research support from Sage Therapeutics. The institution of Dr. Dalmau has received research support from Edmond J.Safra Foundation . The institution of Dr. Dalmau has received research support from La Caixa Foundation. The institution of Dr. Dalmau has received research support from Spanish Ministry of Health (ISCIII). The institution of Dr. Dalmau has received research support from Euroimmun, Inc. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care.