CLIPPERS
Synonyms: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
CLIPPERS is a rare neuroinflammatory disorder characterized by brainstem-predominant encephalomyelitis which typically presents with cerebellar and cranial nerve manifestations (gait ataxia, dysarthria, visual disorders, parasthesias), as well as brainstem, myelopathy and cognitive findings, that respond to steroid treatment. Punctate curvilinear post-gadolinium contrast enhancement predominantly in the pons and cerebellum is observed on brain MRI and prominent, perivascular, CD3+ T-cell predominantly lymphocytic inflammation in neuropathology.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | CLIPPERS | Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids |
| fr | CLIPPERS | Inflammation chronique lymphocytaire avec rehaussement périvasculaire pontique cortico-sensible · Inflammation chronique lymphocytaire avec rehaussement périvasculaire pontique corticosensible |
| de | CLIPPERS | |
| es | CLIPPERS | Inflamación linfocítica crónica con mejora perivascular pontina sensible a esteroides |
| it | CLIPPERS | Infiammazione linfocitica cronica con rialzamento perivascolare pontino sensibile agli steroidi |
| nl | CLIPPERS | Chronische lymfocytische ontsteking met pontiene perivasculaire verbetering als respons op steroïden |
| pt | CLIPPERS | |
| pl | CLIPPERS | = en |
| cs | CLIPPERS | Chronický lymfocytární zánět s pontinním perivaskulárním zvýrazněním, odpovídající na steroidy |
| uk | CLIPPERS | Хронічне лімфоцитарне запалення з понтинним периваскулярним посиленням, що реагує на стероїди |
| zh | 慢性淋巴细胞性炎症伴脑桥血管周围强化对类固醇的反应 | 慢性淋巴细胞性炎症伴脑桥周围血管类固醇敏感 |
| tr | CLIPPERS | Steroidlere yanıt veren pontin perivasküler gelişim ile seyreden kronik lenfositik inflamasyon |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G04.8 | NTBT | attributed | validated |
| ICD-11 | 8E4A.0 | NTBT | index | validated |
| MONDO | 0017297 | E | validated | |
| UMLS | C3854437 | E | validated | |
| GARD | 10779 | E | validated | |
| MedDRA | 10075197 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:182064Rare neuroinflammatory or neuroimmunological disease
rare neurological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 50 cases | Worldwide | Case(s) | Validated | 24028073[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/284448
- This record as JSON: /data/d/284448.json