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paraneoplastic cerebellar degeneration - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

paraneoplastic cerebellar degeneration

MONDO:0044877

Overview

A rare, immune-mediated disorder characterized by cerebellar degeneration due to the presence of an often undetected malignancy (usually carcinoma or lymphoma) in an anatomic site other than the cerebellum. Signs and symptoms include progressive ataxia, dysarthria, and nystagmus.

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include common findings: Ataxia, Increased CSF protein concentration, CSF pleocytosis, and CSF oligoclonal immunoglobulin G bands; and sometimes findings: Nystagmus, Diplopia, Dysarthria, and Shrinkage of the cerebellum (cerebellar atrophy) and others.

Organ System	Phenotype Count	Example Features
Lab test results	7	Increased CSF protein concentration, Anti-CV2/CRMP5 antibody positivity, Anti-Hu antibody positivity

Diagnosis

Biomarker and diagnostic research for paraneoplastic cerebellar degeneration has been reported in the published literature.

Prognosis

Phenotype severity distribution: 4 common features.

Estimated prevalence: 1-9 in 1,000,000 (Rare).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for paraneoplastic cerebellar degeneration.

48 publications have been identified in PubMed for paraneoplastic cerebellar degeneration. Research spans Case Report / Case Series (63%), Review / Meta-Analysis (21%), and Basic Science / Preclinical (6%).

Research Type	Count	% of Total
Patient case studies	30	63%

Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 6:47 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:623626

GARD

Genetic and Rare Diseases Info Center

GARD:22493

Questions for Your Care Team

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Kisho

paraneoplastic cerebellar degeneration

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

paraneoplastic cerebellar degeneration

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications