Favorable Outcomes in a Case of Non-paraneoplastic DNER Ataxia Treated with Immunotherapy

Anti-DNER antibody is associated with paraneoplastic cerebellar degeneration (PCD) and Hodgkin’s disease (HD). However, recent studies reported cases absence of HD and that non-tumor anti-DNER antibody-associated ataxia was not well characterized. We present a case of acute cerebellar ataxia and nys...

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Published inCerebellum (London, England) Vol. 23; no. 4; pp. 1697 - 1701
Main Authors Duan, Ruo-Nan, Si, Wei-Yue, Cao, Li-Li
Format Journal Article
LanguageEnglish
Published New York Springer US 01.08.2024
Springer Nature B.V
Subjects
Antibodies
Antigens
Ataxia
Atrophy
Autoantibodies - blood
Biomedical and Life Sciences
Biomedicine
Brief Report
Cerebellar ataxia
Cerebellar Ataxia - diagnostic imaging
Cerebellar Ataxia - drug therapy
Cerebellar Ataxia - immunology
Cerebellum
Cerebrospinal fluid
Dysarthria
Female
Females
Gait
Humans
Immunosuppressive agents
Immunotherapy
Immunotherapy - methods
Magnetic Resonance Imaging
Male
Malignancy
Middle Aged
Neurobiology
Neurodegeneration
Neuroimaging
Neurology
Neurosciences
Patients
Positron emission tomography
Rituximab
Rituximab - therapeutic use
Steroid hormones
Tomography
Treatment Outcome
Tumors
Non-paraneoplastic cerebellar degeneration
Hodgkin’s disease
Immunotherapy
DNER
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Summary:Anti-DNER antibody is associated with paraneoplastic cerebellar degeneration (PCD) and Hodgkin’s disease (HD). However, recent studies reported cases absence of HD and that non-tumor anti-DNER antibody-associated ataxia was not well characterized. We present a case of acute cerebellar ataxia and nystagmus with detected anti-DNER antibody. Brain magnetic resonance imaging (MRI) showed cerebellar atrophy. High titer of anti-DNER antibody was detected in CSF and serum. Positron emission tomography (PET) scanning was unremarkable at a 10-month follow up. The patient improved significantly after immunosuppressive therapy with intravenous steroids, immunoglobulin followed by rituximab. Our study suggest that the presence of such anti-neuronal antibodies might not come along with malignancy and that early onset non-tumor patients are more likely to have a better outcome after immunotherapy. Early diagnosis and timely immunosuppressive therapy may prove beneficial for these patients.
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ISSN:1473-4230
1473-4222
1473-4230
DOI:10.1007/s12311-023-01636-z