LGI1 Encephalitis and IgG4-Related Disease: Rare Conditions Collide.

Autor:	Jesuthasan A; From the Charing Cross Hospital (A.J.); Department of Radiology (T.B.), Imperial College Healthcare NHS Trust, London, United Kingdom; Department of Surgery and Cancer (L.D.), Faculty of Medicine, Imperial College London, United Kingdom; London North West University Healthcare NHS Trust (S.M.); Department of Brain Sciences (M.R.J.), Faculty of Medicine, Imperial College London; and Renal Department (J.A.P.T.), Imperial College Healthcare NHS Trust, London, United Kingdom. aaronjesuthasan@gmail.com., Barwick T; From the Charing Cross Hospital (A.J.); Department of Radiology (T.B.), Imperial College Healthcare NHS Trust, London, United Kingdom; Department of Surgery and Cancer (L.D.), Faculty of Medicine, Imperial College London, United Kingdom; London North West University Healthcare NHS Trust (S.M.); Department of Brain Sciences (M.R.J.), Faculty of Medicine, Imperial College London; and Renal Department (J.A.P.T.), Imperial College Healthcare NHS Trust, London, United Kingdom., Dixon L; From the Charing Cross Hospital (A.J.); Department of Radiology (T.B.), Imperial College Healthcare NHS Trust, London, United Kingdom; Department of Surgery and Cancer (L.D.), Faculty of Medicine, Imperial College London, United Kingdom; London North West University Healthcare NHS Trust (S.M.); Department of Brain Sciences (M.R.J.), Faculty of Medicine, Imperial College London; and Renal Department (J.A.P.T.), Imperial College Healthcare NHS Trust, London, United Kingdom., Molloy S; From the Charing Cross Hospital (A.J.); Department of Radiology (T.B.), Imperial College Healthcare NHS Trust, London, United Kingdom; Department of Surgery and Cancer (L.D.), Faculty of Medicine, Imperial College London, United Kingdom; London North West University Healthcare NHS Trust (S.M.); Department of Brain Sciences (M.R.J.), Faculty of Medicine, Imperial College London; and Renal Department (J.A.P.T.), Imperial College Healthcare NHS Trust, London, United Kingdom., Johnson MR; From the Charing Cross Hospital (A.J.); Department of Radiology (T.B.), Imperial College Healthcare NHS Trust, London, United Kingdom; Department of Surgery and Cancer (L.D.), Faculty of Medicine, Imperial College London, United Kingdom; London North West University Healthcare NHS Trust (S.M.); Department of Brain Sciences (M.R.J.), Faculty of Medicine, Imperial College London; and Renal Department (J.A.P.T.), Imperial College Healthcare NHS Trust, London, United Kingdom., Tomlinson JAP; From the Charing Cross Hospital (A.J.); Department of Radiology (T.B.), Imperial College Healthcare NHS Trust, London, United Kingdom; Department of Surgery and Cancer (L.D.), Faculty of Medicine, Imperial College London, United Kingdom; London North West University Healthcare NHS Trust (S.M.); Department of Brain Sciences (M.R.J.), Faculty of Medicine, Imperial College London; and Renal Department (J.A.P.T.), Imperial College Healthcare NHS Trust, London, United Kingdom.
Jazyk:	angličtina
Zdroj:	Neurology(R) neuroimmunology & neuroinflammation [Neurol Neuroimmunol Neuroinflamm] 2023 Aug 15; Vol. 10 (6). Date of Electronic Publication: 2023 Aug 15 (Print Publication: 2023).
DOI:	10.1212/NXI.0000000000200158
Abstrakt:	Objectives: Leucine-rich glioma-inactivated 1 (LGI1) encephalitis and IgG4-related disease (IgG4RD) have traditionally been regarded as 2 distinct disease entities. Methods: We detail the presentation, investigations, and management of a patient who showed typical signs and symptoms of LGI1 encephalitis and also found to possess pancreatic changes and a serum profile in keeping with IgG4RD. Results: Serum and CSF analyses at presentation showed a significant hyponatraemia (117 mmol/L), elevated IgG4 concentration (1.73 g/L), and the presence of LGI1 antibodies. MRI revealed symmetrical diffuse T2-weighted hyperintensity and mild swelling throughout both medial temporal lobes. CT of the chest, abdomen and pelvis revealed an edematous, bulky pancreas with loss of lobulation, typical for IgG4RD. A glucocorticoid weaning regimen was commenced, facilitated by 2 rituximab infusions, with the patient showing an effective treatment response. HLA testing confirmed the presence of HLA DRB1 and HLA DQB1 risk alleles. Discussion: This case suggests that there may be shared mechanisms between LGI1 encephalitis and IgG4RD, supported by common risk HLA associations and treatment strategies/responses. To our knowledge, this represents the first instance that LGI1 encephalitis and IgG4RD have been reported in the same patient and emphasizes the continued development of our understanding of the wide range of IgG4-mediated conditions. (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)
Databáze:	MEDLINE
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