Morvan syndrome
Synonyms: Limbic encephalitis-neuromyotonia-hyperhidrosis-polyneuropathy syndrome · Morvan fibrillary chorea
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
Morvan syndrome is a rare, life-threatening, acquired neurologic disease characterized by neuromyotonia, dysautonomia and encephalopathy with severe insomnia. Signs involving central (e.g. hallucinations, confusion, amnesia, myoclonus), autonomic (e.g. variations in blood pressure, hyperhidrosis) and peripheral (e.g. painful cramps, myokymia) hyperactivity, as well as systemic manifestations (such as weight loss, pruritus, fever), are reported. Thymoma is present in some cases.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Morvan syndrome | Limbic encephalitis-neuromyotonia-hyperhidrosis-polyneuropathy syndrome · Morvan fibrillary chorea |
| fr | Syndrome de Morvan | Chorée fibrillaire de Morvan · Syndrome d'encéphalite limbique-neuromyotonie-hyperhidrose-polyneuropathie |
| de | Morvan-Syndrom | Fibrilläre Chorea Morvan · Syndrom der limbischen Enzephalitis mit Neuromyotonie, Hyperhidrose und Polyneuropathie |
| es | Síndrome de Morvan | Corea fibrilar de Morvan · Encefalitis límbica-neuromiotonía-hiperhidrosis-polineuropatía · Síndrome de encefalitis límbica-neuromiotonía-hiperhidrosis-polineuropatía |
| it | Sindrome di Morvan | Corea fibrillare di Morvan · Sindrome da encefalite limbica, neuromiotonia, iperidrosi e polineuropatia |
| nl | Syndroom van Morvan | Fibrillaire chorea van Morvan · Limbische encefalitis - neuromyotonie - hyperhidrose - polyneuropathie-syndroom |
| pt | Síndrome Morvan | Coreia fibrilhar Morvan · Síndrome de encefalite límbica-neuromiotonia-hiperhidrose-polineuropatia |
| pl | Zespół Morvana | Limbiczne zapalenie mózgu - neuromiotonia - hiperhydroza - polineuropatia · Włókienkowa pląsawiaca Morvana |
| cs | Morvanův syndrom | Syndrom zahrnující limbickou encefalitidu, neuromyotonii, hyperhidrózu a polyneuropatii · Morvanova fibrilární chorea |
| uk | Синдром Морвана | Синдром лімбічного енцефаліту-нейроміотонії-гіпергідрозу-полінейропатії · Фібрилярна хорея Морвана |
| zh | Morvan综合征 | 边缘叶脑炎-神经性肌强直-多汗症-多发性神经病综合征 |
| tr | Morvan sendromu | Morvan fibriler kore · Limbik ensefalit-nöromiyotoni-hiperhidroz-polinöropati sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G60.8 | NTBT | inclusion | validated |
| ICD-11 | 8E4A.3 | NTBT | index | validated |
| MONDO | 0008718 | E | validated | |
| UMLS | C3854373 | E | validated | |
| GARD | 9766 | E | validated | |
| MedDRA | 10075006 | 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 (3):
- ORPHA:221114Acquired peripheral movement disorder
rare neurological diseases - ORPHA:98750Autoimmune neurological channelopathy
rare neurological diseases - ORPHA:166484Inflammatory and autoimmune disease with epilepsy
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 | 60 cases | Worldwide | Case(s) | Validated | 27698847[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_27698847[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.
HPO phenotypes
1 annotations · validated 2024-03-04| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:5000005 | Anti-CASPR2 | |
Annotation source: 32651251[PMID]_29561731[PMID]_32783406[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/83467
- This record as JSON: /data/d/83467.json