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A 57-year-old male patient developed within 2 weeks rapid progressive gait instability, memory deficits, hypomania, and recurrent complex tic-like involuntary outbursts of vocalization 15 and 18 months after double autologous hematopoietic stem-cell transplantation for multiple myeloma. CSF showed 19 mononuclear cells/μL, and MRI revealed progressive multifocal cortico-subcortical T2/FLAIR-hyperintensities with high metabolic activity in FDG-PET/CT (Figures 1 and 2). Histopathology of a biopsied lesion revealed sparse inflammatory changes of cortico-subcortical areas. Within the next 2 weeks, he developed continuous rhythmic myoclonic jerks in the left hand (“myoclonia continua”), initially with and later without epileptic activity in EEG.1 After excluding differential diagnoses, that is, intracerebral viral, malignant, demyelinating or other autoimmune disease, we diagnosed anti-GABAA receptor antibody-positive autoimmune encephalitis,2 presumably triggered by aberrant posttransplant immune reconstitution. After corticosteroids, plasma exchange, and rituximab, he showed near-complete recovery within several months. Encephalitis with severe seizures and multifocal brain lesions should prompt consideration of anti-GABAA receptor encephalitis.
Figure 1 Evolution of MRI Findings
Axial MRI (A) on admission (day 0) with multiple, asymmetric, cortical/subcortical FLAIR lesions without contrast enhancement; (B) on day 20 with progression of lesions; (C) on day 33 with incomplete regression; and (D) on day 159 with complete remission of all lesions.
Figure 2 FDG-PET/CT and Histopathologic Findings
The lesions showed (A) markedly increased metabolic activity on FDG-PET/CT (day 8) and (B–D) reactive gliosis and conspicuous nodular aggregates (B, hematoxylin-eosin staining), which stained positive for macrophages and microglia on immunohistochemistry (C, CD68 staining; D, HLA-DR2 staining) of brain biopsy (day 13).

References

1.
Cockerell OC, Rothwell J, Thompson PD, Marsden CD, Shorvon SD. Clinical and physiological features of epilepsia partialis continua. Cases ascertained in the UK. Brain. 1996;119(Pt 2):393-407.
2.
Hoshina Y, Galli J, Wong KH, et al. GABA-A receptor encephalitis after autologous hematopoietic stem cell transplant for multiple myeloma: three cases and literature review. Neurol Neuroimmunol Neuroinflamm. 2022;9(6):e200024.

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Published In

Neurology®
Volume 102Number 7April 9, 2024
PubMed: 38452326

Publication History

Received: November 20, 2023
Accepted: January 24, 2024
Published online: March 7, 2024
Published in print: April 9, 2024

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The authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.

Study Funding

This study received no specific funding. The Section of Neuroimmunology and Multiple Sclerosis Research was supported by the Clinical Research Priority Program (CRPP-MS) of the University of Zurich.

Authors

Affiliations & Disclosures

From the Department of Neurology (C.L.T., L.L.I., I.J.); Institute of Neuropathology (K.F.), University Hospital Zurich and University of Zurich, Switzerland; Institute of Neurology (K.F.), Queen Square Brain Bank, University College London, United Kingdom; Cantonal Hospital of Zug (M.S.-G.), Baar; Swiss Epilepsy Center (L.L.I.), Klinik Lengg, Zurich; Neuroimmunology and MS Research (NIMS) (I.J.), Department of Neurology and University of Zurich, University Hospital Zurich; and Neuroimmunology Outpatient Clinic (I.J.), Center for Multiple Sclerosis, Neurocenter, Bellevue, Zurich, Switzerland.
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From the Department of Neurology (C.L.T., L.L.I., I.J.); Institute of Neuropathology (K.F.), University Hospital Zurich and University of Zurich, Switzerland; Institute of Neurology (K.F.), Queen Square Brain Bank, University College London, United Kingdom; Cantonal Hospital of Zug (M.S.-G.), Baar; Swiss Epilepsy Center (L.L.I.), Klinik Lengg, Zurich; Neuroimmunology and MS Research (NIMS) (I.J.), Department of Neurology and University of Zurich, University Hospital Zurich; and Neuroimmunology Outpatient Clinic (I.J.), Center for Multiple Sclerosis, Neurocenter, Bellevue, Zurich, Switzerland.
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Maximilian Schubring-Giese, MD
From the Department of Neurology (C.L.T., L.L.I., I.J.); Institute of Neuropathology (K.F.), University Hospital Zurich and University of Zurich, Switzerland; Institute of Neurology (K.F.), Queen Square Brain Bank, University College London, United Kingdom; Cantonal Hospital of Zug (M.S.-G.), Baar; Swiss Epilepsy Center (L.L.I.), Klinik Lengg, Zurich; Neuroimmunology and MS Research (NIMS) (I.J.), Department of Neurology and University of Zurich, University Hospital Zurich; and Neuroimmunology Outpatient Clinic (I.J.), Center for Multiple Sclerosis, Neurocenter, Bellevue, Zurich, Switzerland.
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From the Department of Neurology (C.L.T., L.L.I., I.J.); Institute of Neuropathology (K.F.), University Hospital Zurich and University of Zurich, Switzerland; Institute of Neurology (K.F.), Queen Square Brain Bank, University College London, United Kingdom; Cantonal Hospital of Zug (M.S.-G.), Baar; Swiss Epilepsy Center (L.L.I.), Klinik Lengg, Zurich; Neuroimmunology and MS Research (NIMS) (I.J.), Department of Neurology and University of Zurich, University Hospital Zurich; and Neuroimmunology Outpatient Clinic (I.J.), Center for Multiple Sclerosis, Neurocenter, Bellevue, Zurich, Switzerland.
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Funding for travel - Medtronic
Consultant Fee - Eisai, Jazz, Angelini
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NONE
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From the Department of Neurology (C.L.T., L.L.I., I.J.); Institute of Neuropathology (K.F.), University Hospital Zurich and University of Zurich, Switzerland; Institute of Neurology (K.F.), Queen Square Brain Bank, University College London, United Kingdom; Cantonal Hospital of Zug (M.S.-G.), Baar; Swiss Epilepsy Center (L.L.I.), Klinik Lengg, Zurich; Neuroimmunology and MS Research (NIMS) (I.J.), Department of Neurology and University of Zurich, University Hospital Zurich; and Neuroimmunology Outpatient Clinic (I.J.), Center for Multiple Sclerosis, Neurocenter, Bellevue, Zurich, Switzerland.
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Served on a scientific advisory board - Alexion, Biogen, Bristol Myers Squibb, Celgene, Janssen-Cilag, Neuway, Merck, Novartis, Roche and Sanofi Genzyme; none of these are related to this study
Has Patents issued - Polyoma virus jc peptides and proteins in vaccination and diagnostic applications (WO2013014134A3)
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Commercial - Biogen: 2008 and 2013
Commercial - Novartis: 2013
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Notes

Correspondence Dr. Jelcic [email protected]
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Submitted and externally peer reviewed. The handling editor was Editor-in-Chief José Merino, MD, MPhil, FAAN.

Author Contributions

C.L. Togni: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; analysis or interpretation of data. K. Frontzek: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; analysis or interpretation of data. M. Schubring-Giese: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; analysis or interpretation of data. L.L. Imbach: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; analysis or interpretation of data. I. Jelcic: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; analysis or interpretation of data.

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