Autoimmune Encephalitis With mGluR1 Antibodies Presenting With Epilepsy, but Without Cerebellar Signs: A Case Report

Anita M. Vinke, Shenghua Zong, Josien H. Janssen, Carolin Correia-Hoffmann, Marina Mané-Damas, Jan G.M.C. Damoiseaux, J. M. de Vries, Dirk Pröpper, Peter Molenaar, Mario Losen, Pilar Martinez Martinez, Rob P.W. Rouhl

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OBJECTIVE: To describe the unique case history of a patient with mGluR1 antibodies, with mainly limbic and without cerebellar symptoms. METHODS: A 50-year-old woman initially presented with focal seizures with epigastric rising and déjà-vu sensations, next to cognitive complaints, and musical auditory hallucinations. MRI, EEG, and neuronal autoantibody tests were performed. RESULTS: EEG findings showed slow and sharp activity (sharp waves and sharp-wave-slow-wave complex) in the left temporal lobe. A test for autoantibodies was negative initially. Because of persistent symptoms, serum and CSF were tested 4 years later and found positive for mGluR1 antibodies. Treatment started with monthly IV immunoglobulins and azathioprine that was replaced by mycophenolate mofetil later. Especially cognitive symptoms and hallucinations did not respond well to the treatment. During treatment, mGluR1 antibodies remained present in CSF. DISCUSSION: Whereas cerebellar symptoms are present in 97% of mGluR1-positive cases, our patient presented without ataxia. Therefore, we suggest that the clinical presentation of patients with mGluR1 antibodies is probably more diverse than previously described. Testing for mGluR1 antibodies should be considered in patients with limbic encephalitis and epilepsy, especially when negative for more common antibodies.

Original languageEnglish
Article numbere1171
JournalNeurology(R) neuroimmunology & neuroinflammation
Issue number4
Publication statusPublished - 21 Jul 2022

Bibliographical note

Funding Information:
The authors are thankful for the financial support received from the following funding organizations: P. Martinez-Martinez was supported by an Aspasia/NWO grant (015.011.033), and S. Zong, C. Correia-Hoffmann, and M. Mané-Damas were supported by Kootstra Talent Fellowships. The authors also thank Vivianne van Kranen-Mastenbroek (Academic Center for Epileptology Kempenhaeghe/MUMC + Heeze and Maastricht) for providing the EEG image and Josep Dalmau from Instituto de Investigación Biomédica August Pi i Sunyer (IDIBAPS), Barcelona, for providing the mGluR1-EGFP plasmids and for his help with the detection of the autoantibodies.

Publisher Copyright:
© American Academy of Neurology.


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