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Case Reports
. 2018 May 17:9:329.
doi: 10.3389/fneur.2018.00329. eCollection 2018.

IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid Changes

Massimiliano Montagna  1   2 , Rizvana Amir  3 , Ilse De Volder  1   4 , Martin Lammens  5 , Jef Huyskens  6 , Barbara Willekens  1   7
Affiliations
Case Reports

IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid Changes

Massimiliano Montagna et al. Front Neurol. .

Abstract

IgLON5-associated encephalitis is a syndrome with different clinical presentations consisting of sleep dysfunction, bulbar dysfunction, chorea, and progressive supranuclear palsy-like symptoms whereas dysautonomy and cognitive decline usually appear in later stages of the disease. We report a case of a patient with IgLON5-associated encephalitis presenting with rapidly progressive cognitive decline and atypical inflammatory lesions on brain magnetic resonance imaging, oligoclonal bands on cerebrospinal fluid, anti-IgLON5 antibodies exclusively of the IgG1 class, and a fierce inflammatory reaction on brain biopsy, who responded favorably to immunotherapy.

Keywords: IgLON5; akathisia; autoimmune encephalitis; brain inflammation; dyskinesia; rapidly evolving dementia.

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Figures

Figure 1
Figure 1
A timeline displaying synthetically the evolution of our case: in the red boxes, the clinical events have been reported; in the blue boxes, there is a list of the significant investigations that have been performed; and in the yellow boxes, we displayed the therapies and the relative effect.
Figure 2
Figure 2
Brain magnetic resonance imaging (Siemens Aera 1.5 T). Axial FLAIR (1, 2), axial ADC-maps (3), axial T1 after intravenous gadolinium (4, 5), and coronal T1 after intravenous gadolinium (6). Several lesions with high T2-signal on FLAIR in the right temporal lobe, bilateral in the frontal lobe, and the callosal body without signs of restricted diffusion on the ADC-maps, compatible with vasogenic edema. Several lesions in the right temporal lobe show patchy contrast enhancement after intravenous administration of gadolinium-based contrast (4–6). None of the lesions were hemorrhagic.
Figure 3
Figure 3
Important inflammation of the brain tissue with disseminated macrophages (B) and T-cells (C), no B-cells. Part of the T-cells is CD8-positive (D). Hematoxylin–eosin (A), immunohistochemical stain with antibodies against CD68 (B), CD3 (C), and CD8 (D). Magnification bar = 100 µm.
See this image and copyright information in PMC

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