Clinical features, prognostic factors, and antibody effects in anti-mGluR1 encephalitis.
Adult
Aged
Animals
Atrophy
/ pathology
Autoantibodies
/ immunology
Autoimmune Diseases of the Nervous System
/ complications
Cells, Cultured
Cerebellar Diseases
/ diagnosis
Child
Embryo, Mammalian
Encephalitis
/ complications
Female
Follow-Up Studies
Hippocampus
/ cytology
Humans
Immunoglobulin G
/ classification
Immunotherapy
Magnetic Resonance Imaging
Male
Middle Aged
Neurons
Prognosis
Rats
Receptors, Metabotropic Glutamate
/ immunology
Journal
Neurology
ISSN: 1526-632X
Titre abrégé: Neurology
Pays: United States
ID NLM: 0401060
Informations de publication
Date de publication:
01 12 2020
01 12 2020
Historique:
received:
22
04
2020
accepted:
14
07
2020
pubmed:
16
9
2020
medline:
17
12
2020
entrez:
15
9
2020
Statut:
ppublish
Résumé
To clinically characterize patients with anti-metabotropic glutamate receptor (mGluR) 1 encephalitis, to identify prognostic factors, and to study the immunoglobulin G (IgG) subclasses and effects of antibodies on neuronal mGluR1 clusters. Clinical information on new and previously reported patients was reviewed. Antibodies to mGluR1 and IgG subclasses were determined with brain immunohistochemistry and cell-based assays, and their effects on mGluR1 clusters were studied on rat hippocampal neurons. Eleven new patients were identified (10 adults, 1 child);4 were female. In these and 19 previously reported cases (n = 30, median age 55 years), the main clinical manifestation was a subacute cerebellar syndrome that in 25 (86%) patients was associated with behavioral/cognitive changes or other neurologic symptoms. A tumor was found in 3 of 26 (11%). Brain MRI was abnormal in 7 of 19 (37%) at onset and showed cerebellar atrophy in 10 of 12 (83%) at follow-up. Twenty-five of 30 (83%) patients received immunotherapy. Follow-up was available for 25: 13 (52%) had clinical stabilization; 10 (40%) showed significant improvement; and 2 died. At the peak of the disease, patients with bad outcome at 2 years (modified Rankin Scale score > 2, n = 7) were more likely to have higher degree of initial disability, as reflected by a worse Scale for Assessment and Rating of Ataxia score, and more frequent need of assistance to walk. Antibodies to mGluR1 were mainly IgG1 and caused a significant decrease of mGluR1 clusters in cultured neurons. Anti-mGluR1 encephalitis manifests as a severe cerebellar syndrome, often resulting in long-term disability and cerebellar atrophy. The antibodies are pathogenic and cause significant decrease of mGluR1 clusters in cultured neurons.
Identifiants
pubmed: 32928978
pii: WNL.0000000000010854
doi: 10.1212/WNL.0000000000010854
pmc: PMC7734921
doi:
Substances chimiques
Autoantibodies
0
Immunoglobulin G
0
Receptors, Metabotropic Glutamate
0
metabotropic glutamate receptor type 1
0
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
e3012-e3025Informations de copyright
© 2020 American Academy of Neurology.
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