Metabotropic glutamate receptor type 1 autoimmunity

A. Sebastian Lopez-Chiriboga, Lars Komorowski, Tania Kümpfel, Christian Probst, Shannon R. Hinson, Sean J Pittock, Andrew B McKeon

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

Objective: To describe retrospectively the clinical associations of immunoglobulin G (IgG) targeting metabotropic glutamate receptor 1 (mGluR1-IgG). Methods: Specimens of 9 patients evaluated on a service basis in the Mayo Clinic Neuroimmunology Laboratory by tissue-based immunofluorescence assay (IFA) yielded a robust, synaptic immunostaining pattern consistent with mGluR1-IgG (serum, 9; CSF, 2 available). Transfected HEK293 cell-based assay (CBA) confirmed mGluR1 specificity in all 11 specimens. A further 2 patients were detected in Germany primarily by CBA. Results: The median symptom onset age for the 11 patients was 58 years (range 33-81 years); 6 were male. All 9 Mayo Clinic patients had subacute onset of cerebellar ataxia, 4 had dysgeusia, 1 had psychiatric symptoms, and 1 had cognitive impairment. All were evaluated for malignancy, but only 1 was affected (cutaneous T-cell lymphoma). One developed ataxia post-herpes zoster infection. Head MRIs were generally atrophic or normal-appearing, and CSF was inflammatory in just 1 of 5 tested, though mGluR1-IgG was detected in both specimens submitted. Five patients improved (attributable to immunotherapy in 4, spontaneously in 1), 3 stabilized (attributable to immunotherapy in 2, cancer therapy in 1), and 1 progressively declined (untreated). The 2 German patients had ataxia, but fulfilled multiple sclerosis diagnostic criteria (1 relapsing-remitting, 1 progressive). However, both had histories of hematologic malignancy (acute lymphocytic leukemia and mantle cell lymphoma), and had mGluR1-IgG detected in serum by CBA (weakly positive on tissue-based IFA). Conclusions: mGluR1 autoimmunity represents a treatable form of cerebellar ataxia. Dysgeusia may be a diagnostic clue. Paraneoplastic, parainfectious, or idiopathic causes may occur.

Original languageEnglish (US)
Pages (from-to)1009-1013
Number of pages5
JournalNeurology
Volume86
Issue number11
DOIs
StatePublished - Mar 15 2016

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Autoimmunity
Immunoglobulin G
Dysgeusia
Cerebellar Ataxia
Ataxia
Immunotherapy
Fluorescent Antibody Technique
Mantle-Cell Lymphoma
Cutaneous T-Cell Lymphoma
HEK293 Cells
Herpes Zoster
Hematologic Neoplasms
Granulocyte-Macrophage Colony-Stimulating Factor
metabotropic glutamate receptor type 1
Serum
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Age of Onset
Multiple Sclerosis
Germany
Psychiatry

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Lopez-Chiriboga, A. S., Komorowski, L., Kümpfel, T., Probst, C., Hinson, S. R., Pittock, S. J., & McKeon, A. B. (2016). Metabotropic glutamate receptor type 1 autoimmunity. Neurology, 86(11), 1009-1013. https://doi.org/10.1212/WNL.0000000000002476

Metabotropic glutamate receptor type 1 autoimmunity. / Lopez-Chiriboga, A. Sebastian; Komorowski, Lars; Kümpfel, Tania; Probst, Christian; Hinson, Shannon R.; Pittock, Sean J; McKeon, Andrew B.

In: Neurology, Vol. 86, No. 11, 15.03.2016, p. 1009-1013.

Research output: Contribution to journalArticle

Lopez-Chiriboga, AS, Komorowski, L, Kümpfel, T, Probst, C, Hinson, SR, Pittock, SJ & McKeon, AB 2016, 'Metabotropic glutamate receptor type 1 autoimmunity', Neurology, vol. 86, no. 11, pp. 1009-1013. https://doi.org/10.1212/WNL.0000000000002476
Lopez-Chiriboga AS, Komorowski L, Kümpfel T, Probst C, Hinson SR, Pittock SJ et al. Metabotropic glutamate receptor type 1 autoimmunity. Neurology. 2016 Mar 15;86(11):1009-1013. https://doi.org/10.1212/WNL.0000000000002476
Lopez-Chiriboga, A. Sebastian ; Komorowski, Lars ; Kümpfel, Tania ; Probst, Christian ; Hinson, Shannon R. ; Pittock, Sean J ; McKeon, Andrew B. / Metabotropic glutamate receptor type 1 autoimmunity. In: Neurology. 2016 ; Vol. 86, No. 11. pp. 1009-1013.
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