Posttransplant acute limbic encephalitis
Synonyms: PALE
- Type
- Particular clinical situation in a disease or syndrome
- Group
- Disorder
- Age of onset
- All ages
- Status
- active
Posttransplant acute limbic encephalitis is a rare, acquired, non-paraneoplastic limbic encephalitis disorder, that develops in the setting of treatment-related immunosuppression, typically after allogeneic hemapoietic stem cell transplantation, characterized by onset of confusion, headache, anterograde amnesia, seizures and/or loss of consciousness 2-6 weeks following transplantation. Bilateral, non-enhancing T2 hyperintensities in limbic structures are observed on magnetic resonance imaging. Mild cerebrospinal fluid pleocytosis and syndrome of inappropriate antidiuretic hormone secretion may also be associated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Posttransplant acute limbic encephalitis | PALE |
| fr | Encéphalite limbique aiguë post-transplantation | = en |
| de | Limbische Enzephalitis, akute, nach Transplantation | = en |
| es | Encefalitis límbica aguda postrasplante | = en |
| it | Encefalite limbica acuta post-trapianto | |
| nl | Posttransplantie acute limbische encefalitis | = en |
| pt | Encefalite límbica aguda pós-tranplante | |
| pl | Potransplantacyjne ostre zapalenie układu limbicznego | = en |
| cs | Posttransplantační akutní limbická encefalitida | = en |
| uk | Посттрансплантаційний гострий лімбічний енцефаліт | = en |
| zh | 移植后急性边缘脑炎 | = en |
| tr | Nakil sonrası akut limbik ensefalit | SOLUK |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G04.8 | NTBT | attributed | validated |
| MONDO | 0015595 | E | validated | |
| UMLS | C4750744 | 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 (2):
- ORPHA:97275Encephalitis
rare neurological diseases - ORPHA:565779Rare disorder potentially indicated for transplant or complication after transplantation
rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet 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
23 annotations · validated 2021-11-30| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0200149 | CSF lymphocytic pleiocytosis | |
| HP:0012756 | CSF polymorphonuclear pleocytosis | |
| HP:0011185 | EEG with focal epileptiform discharges | |
| HP:0031885 | Hyperglycorrhachia | |
| HP:0002902 | Hyponatremia | |
| HP:0002922 | Increased CSF protein concentration | |
| HP:0002354 | Memory impairment | |
| HP:0001250 | Seizure | |
| HP:0100806 | Sepsis | |
| HP:0020071 | Viremia | |
| Occasional (29-5%) | ||
| HP:0012332 | Abnormal autonomic nervous system physiology | |
| HP:0025100 | Abnormal hippocampus morphology | |
| HP:0100022 | Abnormality of movement | |
| HP:0000739 | Anxiety | |
| HP:0001251 | Ataxia | |
| HP:0000708 | Atypical behavior | |
| HP:0100543 | Cognitive impairment | |
| HP:0001289 | Confusion | |
| HP:0000716 | Depression | |
| HP:0001332 | Dystonia | |
| HP:0011203 | EEG with abnormally slow frequencies | |
| HP:0001336 | Myoclonus | |
| HP:0000709 | Psychosis | |
Annotation source: 33396564[PMID]_33548682[PMID]_30256500[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/163921
- This record as JSON: /data/d/163921.json