Cerebellar Ataxia with Anti-mGluR1 Auto-Antibody in a Pediatric Patient: A Case Report

Wang, Hongyan; Yi, Bin; Wang, Meng; Wang, Yadan; Fu, Juan; Wang, Xindi; Cao, Ling

doi:10.2139/ssrn.4356441

Cited by 1 publication

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“…Since the first description of two patients with ACA, metabotropic glutamate receptor type 1 (mGluR1) antibodies, and remote history of Hodgkin lymphoma in 2000, 37 patients, median age at diagnosis 55 years, have been reported but only three with an associated tumor [12,13]. Thirty-five patients developed cerebellar ataxia with gait and trunk instability, dysarthria, oculomotor abnormalities, and limb dysmetria.…”

Section: Metabotropic Glutamate Receptormentioning

confidence: 99%

Neuronal antibodies in nonparaneoplastic autoimmune cerebellar ataxias

Saiz,

Graus

2024

Current Opinion in Neurology

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Purpose of review To describe relevant advances in nonparaneoplastic autoimmune cerebellar ataxias (ACA) with neuronal antibodies. Recent findings Apart from metabotropic glutamate receptor 1(mGluR1) antibodies, in recent years, the number of neuronal antibodies against surface antigens in ACA has increased with the description of glutamate kainate receptor subunit 2 (GluK2) antibodies in young patients with cerebellitis. Around 20% of patients with contactin-associated protein-like 2 (CASPR2) encephalitis also present prominent cerebellar ataxia. However, isolate cerebellar ataxia is unusual (<4%). Outcome in patients with neuronal antibodies against surface antigens remains suboptimal despite the cerebellar ataxia probably is antibody-mediated. Concerning neuronal antibodies against intracellular antigens, up to 25% of patients with glutamic acid decarboxylase (GAD) antibodies present transient episodes of vertigo or diplopia that antedate the development of the ACA. There is in-vitro evidence that septin-5 is partially exposed to the membrane and the antibodies may interfere with septin-5 function. The clinical significance of the remaining antibodies against intracellular antigens remains unclear. Summary The number of antibodies against surface antigens is increasing in ACA, but the response to the immunotherapy remains suboptimal. More studies are needed to clarify the role of most of the antibodies against intracellular antigens described in these patients.

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Section: Metabotropic Glutamate Receptormentioning

confidence: 99%