Case Report: Presence of Anti-MAG in the CSF Can Be Associated With a Neurodegenerative Process With Frontal Involvement

Frontiers in Neurology

Marie Benaiteau¹  Fabienne Ory-Magne¹  Jérémie Pariente²  Guillaume Dorcet³  Chloé Bost⁴  Antoine Blancher⁴  Françoise Fortenfant⁴ 

[1] Département de Neurologie, Hôpital Pierre Paul Riquet, CHU de Toulouse, Toulouse, France;INSERM ToNIC, Toulouse NeuroImaging Center, Université de Toulouse, Université Paul Sabatier, Toulouse, France;INSERM U1043 – CNRS UMR 5282, INFINITY, Toulouse, France;Laboratoire d'Immunologie, Institut Fédératif de Biologie, CHU de Toulouse, Toulouse, France;

关键词: MAG;    autoantibodies;    neurodegenerative diseases;    differential diagnosis;    myelin alteration;   

DOI  :  10.3389/fneur.2022.847798

来源: DOAJ

【 摘 要 】

BackgroundAutoimmune encephalitis (AIE) is an increasingly broad nosological framework that may clinically mimic neurodegenerative diseases (NDDs).Cases ReportedWe describe here the clinical, radiological, electrophysiological, and biological evolution of three patients. Two women aged 73 and 72 years and a 69-year-old man presented with complex cognitive and focal neurological symptoms and each had a predominant frontal dysexecutive involvement and an unexpectedly high titer of anti-MAG antibodies in the serum and cerebrospinal fluid (CSF). The question of an autoimmune cause was raised. After 2 years of follow-up and, for two of them, without improvement despite immunosuppressive treatments, diagnoses of NDD were eventually retained: post-radiation encephalopathy, progressive supranuclear palsy (PSP), and Alzheimer's disease.ConclusionThe presence of a high titer of anti-MAG antibodies may be found in NDD. It could reflect cerebral tissue damages, particularly in the case of significant frontal involvement. Atypical presentations may lead to a search for a paraneoplastic neurologic syndrome or AIE. However, the indirect immunofluorescence staining positivity on a monkey cerebellum section linked with anti-MAG antibodies should not lead to those diagnoses being retained.

【 授权许可】

Unknown