Evidence map›Paper›PMID 41746313›Full record

ArticleNeuroradiology2026

A rare case of crescent-shaped peridentate hyperintensity preceding cerebellar atrophy in GAD65 antibody-associated ataxia.

Hiroyuki Maki, Noriko Sato, Risa Kagaya, Kazuhiro Murayama, Yunosuke Kumazawa, Yuji Tsujimura, Hirohisa Watanabe, Masanori Inoue

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Article in Neuroradiology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

8 authors.

Hiroyuki MakiFujita Health University School of Medicine, Toyoake, Japan. machikita205@gmail.com.
Noriko SatoNational Centre Hospital, National Centre of Neurology and Psychiatry, Kodaira, Japan.
Risa KagayaNational Centre Hospital, National Centre of Neurology and Psychiatry, Kodaira, Japan.
Kazuhiro MurayamaFujita Health University School of Medicine, Toyoake, Japan.
Yunosuke KumazawaFujita Health University School of Medicine, Toyoake, Japan.
Yuji TsujimuraFujita Health University School of Medicine, Toyoake, Japan.
Hirohisa WatanabeFujita Health University School of Medicine, Toyoake, Japan.
Masanori InoueFujita Health University School of Medicine, Toyoake, Japan.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Glutamic acid decarboxylase 65 (GAD65) antibody-associated cerebellar ataxia (GAD65-CA) is a rare autoimmune neurological disorder wherein antibodies against the GAD65 enzyme impair GABAergic neurotransmission, ultimately causing Purkinje cell dysfunction. In this report, a 33-year-old woman exhibited right-dominant limb ataxia and dysarthria with high-titer anti-GAD65 antibodies (> 2,000 U/mL) in serum and cerebrospinal fluid. Three months after symptom onset, magnetic resonance imaging (MRI) revealed a crescent-shaped hyperintensity in the white matter surrounding the right dentate nucleus, along with hilum hyperintensity on T2-weighted imaging and fluid-attenuated inversion recovery sequences, without cerebellar atrophy. However, during follow-up, progressive right cerebellar atrophy developed, and iron accumulated within the dentate nucleus. These early MRI signal changes around the dentate nucleus suggested secondary axonal degeneration resulting from Purkinje cell injury. Therefore, this case highlights a distinctive imaging pattern, providing new insights into autoantibody-induced cerebellar synaptic impairment.

Indexed as

AutoantibodiesCerebellar AtaxiaCerebellumGlutamate DecarboxylaseMagnetic Resonance ImagingAdultAtrophyCerebellar NucleiDiagnosis, DifferentialFemaleHumansanti-GAD65 autoantibodyAutoantibodiesGlutamate DecarboxylaseCerebellar ataxiaDentate nucleusGAD65Purkinje cells

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.