raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:623626

Paraneoplastic cerebellar degeneration

Synonyms: PCD · Paraneoplastic cerebellar ataxia · Rapidely progressive cerebellar syndrome · Subacute cerebellar degeneration

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
All ages
Status
active

Orphadata ships no English definition for this code.

1

Names in Orphadata languages

10 of 12 languages
LangNameSynonyms
enParaneoplastic cerebellar degenerationPCD · Paraneoplastic cerebellar ataxia · Rapidely progressive cerebellar syndrome · Subacute cerebellar degeneration
frDégénérescence cérébelleuse paranéoplasiqueAtaxie cérébelleuse paranéoplasique · Ataxie cérébelleuse à progression rapide · Dégénérescence cérébelleuse subaiguë · Syndrome cérébelleux à progression rapide
deParaneoplastische zerebelläre DegenerationPCD · Paraneoplastische zerebelläre Ataxie · Schnell fortschreitendes zerebelläres Syndrom · Subakute zerebelläre Degeneration
esDegeneración cerebelosa paraneoplásicaAtaxia cerebelosa paraneoplásica · Degeneración cerebelosa subaguda · Síndrome cerebeloso rápidamente progresivo
itDegenerazione cerebellare paraneoplasticaAtassia cerebellare paraneoplastica · Degenerazione cerebellare subacuta · PCD · Sindrome cerebellare a progressione rapida
nlParaneoplastische cerebellaire degeneratiePCD · Paraneoplastische cerebellaire ataxie · Snel progressief cerebellair syndroom · Subacute cerebellaire degeneratie
ptDegenerescência cerebelosa paraneoplásicaAtaxia cerebelosa paraneoplásica · Degenerescência cerebelosa subaguda · PCD · Síndrome cerebeloso com rápida progressão
plParanowotworowe zwyrodnienie móżdżku= en
csParaneoplastická cerebelární degenerace= en
ukПаранеопластична дегенерація мозочкаПаранеопластична мозочкова атаксія · PCD · Швидко прогресуючий мозочковий синдром · Підгостра дегенерація мозочка
2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10G31.8NTBTattributedvalidated
ICD-118E4A.0NTBTindexvalidated
MONDO0044877Evalidated
MeSHD020362Evalidated
UMLSC0393534Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (2):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

6 records
TypeClassValueAreaQualificationValidationSource
Point prevalence1-9 / 1 000 0000.9553 / 100 000EuropeValue and classValidated31552550[PMID]_32847939[PMID]_ORPHANET
Annual incidence1-9 / 1 000 0000.2225 / 100 000EuropeValue and classValidated31552550[PMID]_32847939[PMID]_ORPHANET
Point prevalence1-9 / 1 000 0000.6869 / 100 000FranceValue and classValidated32847939[PMID]_ORPHANET
Annual incidence1-9 / 1 000 0000.16 / 100 000FranceValue and classValidated32847939[PMID]_ORPHANET
Point prevalence1-9 / 100 0001.2236 / 100 000ItalyValue and classValidated31552550[PMID]
Annual incidence1-9 / 1 000 0000.285 / 100 000ItalyValue and classValidated31552550[PMID]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

24 annotations · validated 2024-10-30
HPOTerm (as annotated by Orphanet)Diagnostic
Frequent (79-30%)
HP:0001251Ataxia
HP:6000397CSF oligoclonal immunoglobulin G bands
HP:0012229CSF pleocytosis
HP:0002922Increased CSF protein concentration
Occasional (29-5%)
HP:5000006Anti-CV2/CRMP5 antibody positivity
HP:5000016Anti-Hu antibody positivity
HP:5000030Anti-Ri antibody
HP:5000034Anti-Tr/DNER antibody
HP:5000037Anti-Yo antibody positivity
HP:5000024Anti-mGluR1 antibody
HP:0001272Cerebellar atrophy
HP:0100543Cognitive impairment
HP:0000651Diplopia
HP:0001260Dysarthria
HP:0002015Dysphagia
HP:0002315Headache
HP:0012189Hodgkin lymphoma
HP:0011134Low-grade fever
HP:0033834Malaise
HP:0002017Nausea and vomiting
HP:0100013Neoplasm of the breast
HP:0000639Nystagmus
HP:0100615Ovarian neoplasm
HP:0002317Unsteady gait

Annotation source: 32809473[PMID]_34827413[PMID]

7

Sources for this card