Abstract
BackgroundBrain magnetic resonance imaging (MRI) rarely reveals structural changes in patients with suspected anti-Tr/DNER encephalitis and thus provides very limited information. Here, we combined structural MRI, functional MRI, and positron emission tomography-computed tomography (PET-CT) findings to characterize this rare disorder in a patient.Case presentationA 43-year-old woman presented with progressive cerebellar ataxia, memory impairment, anxiety, and depression. Anti-Tr antibodies were detected in both her serum (1:10) and cerebrospinal fluid (1:10). A diagnosis of anti-Tr-positive autoimmune cerebellar ataxia was established. The patient’s symptoms were worse, but her brain MRI was normal. Meanwhile, voxel-based morphometry analysis showed bilateral reduced cerebellar volume, especially in the posterior lobe and uvula of the cerebellum and the middle of the left temporal lobe compared with 6 sex- and age-matched healthy subjects (6 females, 43 ± 2 years; p < 0.05). Using seed-based functional connectivity analysis, decreased connectivity between the posterior cingulate cortex/precuneus and left frontal lobe compared to the control group (p < 0.05) was detected. PET-CT revealed bilateral hypometabolism in the cerebellum and relative hypermetabolism in the cerebellar vermis and bilateral frontal lobe, but no malignant changes.ConclusionsA combination of structural MRI, functional MRI, and brain PET-CT has higher diagnostic and prognostic value than conventional MRI in patients with suspected anti-Tr/DNER encephalitis.
Highlights
Brain magnetic resonance imaging (MRI) rarely reveals structural changes in patients with suspected anti-Tr/Delta/Notch-like epidermal growth factor-related receptor (DNER) encephalitis and provides very limited information
A: The symptoms and treatment measures of the patient; B: Voxel-based morphometry (VBM) analysis showed reduced cerebellar volume bilaterally, especially in the posterior lobe and uvula of cerebellum, and the middle of the left temporal lobe compared with controls; C: The seed-based functional connectivity (FC) investigation showed lower connectivity between posterior cingulate cortex (PCC)/precuneus and left frontal lobe than that of the control group; D: Brain positron emission tomography-computed tomography (PET-CT) showed obvious decreased fluorodeoxyglucose (FGD) uptake in bilateral cerebellum; E: whole-body PET-CT showed no malignant changes were found performed at the whole-brain level in addition to PETCT
We report a confirmed case of anti-Tr positive autoimmune cerebellar ataxia with no signs of malignancy at 1 year follow up
Summary
Brain magnetic resonance imaging (MRI) rarely reveals structural changes in patients with suspected anti-Tr/DNER encephalitis and provides very limited information. We hypothesize that multimodal imaging analyses may reveal structural and functional changes in the brains of patients with anti-Tr/DNER cerebellar ataxia, increasing the pathophysiological and prognostic value of these assessments. A diagnosis of anti-Tr positive autoimmune cerebellar ataxia in the absence of malignancy was established and the patient received immune therapy successively.
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