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Abstract Details

Paraneoplastic Cerebellar Ataxia and Antibodies to Metabotropic Glutamate Receptor 2
Autoimmune Neurology
S39 - Autoimmune and Paraneoplastic Neurological Disorders: Clinical Characteristics and Diagnosis (2:24 PM-2:36 PM)
008
To report the presence of a new neuronal-surface antibody against the metabotropic glutamate receptor 2 (mGluR2-Ab) in two patients with paraneoplastic cerebellar ataxia.
In autoimmune encephalitis there is an extensive number of autoantibodies against neuronal cell surface or synaptic receptors that associate with a good response to immunotherapy. However, in autoimmune cerebellar ataxias the occurrence of similar autoantibodies is rare.
mGluR2-Ab were initially characterized by immunohistochemistry on rat brain and confirmed by immunofluorescence on HEK293 cells transfected with mGluR2. Additional studies included analysis of potential cross-reactivity with other mGluR’s, expression of mGluR2 in patients’ tumors, and the effects of mGluR2-Ab on cultures of rat hippocampal neurons.
Patient 1 was a 78-year-old woman with chronic cerebellar ataxia with an initial relapsing-remitting course who developed a small-cell tumor of unknown origin. Patient 2 was a 3-year-old girl who presented a steroid-responsive acute cerebellitis preceding the diagnosis of an alveolar rhabdomyosarcoma. Patients’ serum and CSF showed a characteristic immunostaining of hippocampus and cerebellum in rat brain sections and immunolabeled the cell surface of live rat hippocampal neurons. HEK 293 cells transfected with mGluR1, 2, 3, and 5 confirmed that patients’ antibodies only recognized mGluR2.
mGluR2-Ab were not detected in 160 controls, 120 with paraneoplastic, autoimmune, or degenerative ataxias and 40 with autoimmune encephalitis and antibodies against mGluR5 or unknown antigens. Expression of mGluR2 in tumors was confirmed by immunohistochemistry using a commercial mGluR2-Ab. Incubation of live rat hippocampal neurons with CSF of patient 2 did not modify the density of surface mGluR2 clusters.
mGluR2-Ab are a novel biomarker of a treatable type of paraneoplastic cerebellar ataxia. The potential pathogenic effect of the antibodies is not mediated by down regulation or internalization of neuronal surface mGluR2.
Authors/Disclosures

PRESENTER 		No disclosure on file
Eugenia Martinez-Hernandez, MD (Hospital Clinic Barcelona) Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. The institution of Dr. Martinez-Hernandez has received research support from Instituto de Salud Carlos III. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a support for attending meetings with Sanofi. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a support for attending meetings and speaker honoraria with UCB. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a speaker honoraria with Alexion. Dr. Martinez-Hernandez has received personal compensation in the range of $0-$499 for serving as a speaker honoraria with Terumo. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a support for attending meetings with Janssen. Dr. Martinez-Hernandez has received personal compensation in the range of $500-$4,999 for serving as a support for attending meetings with CSL Behring.
Bastien Joubert Bastien Joubert has nothing to disclose.
No disclosure on file
No disclosure on file
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.
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.