Secondary syringomyelia
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
Secondary syringomyelia is a rare medullar disease defined as a development of a fluid-filled cavity or syrinx within the spinal cord due to blockage of CSF circulation (e.g., due to basal archnoiditis, meningeal carcinomatosis, various mass lesions), spinal cord injury (e.g., due to trauma, radiation necrosis, hemorrhage, spinal abscess), spinal dysraphism or intramedullary tumours. It presents with neuropathic pain, numbness, muscular weakness, changes in tone or spasticity or autonomic changes (hyperhidrosis, heart rate or blood pressure instability). Selective loss of pain and temperature with relative preservation of dorsal column function (touch and pressure) are classic findings.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Secondary syringomyelia | |
| fr | Syringomyélie secondaire | |
| de | Syringomyelie, sekundäre | |
| es | Siringomielia secundaria | |
| it | Siringomielia secondaria | |
| nl | Secundaire syringomyelie | |
| pt | Siringomielia, secundária | |
| pl | Wtórna jamistość rdzenia | |
| cs | Sekundární syringomyelie | |
| uk | Вторинна сирингомієлія | |
| zh | 继发性脊髓空洞症 | |
| tr | Sekonder siringomiyeli |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G95.0 | NTBT | attributed | validated |
| ICD-11 | 8D66.1 | E | specific | validated |
| MONDO | 0020509 | E | validated | |
| UMLS | C4749399 | 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:3280Syringomyelia
rare neurological diseases
No children.
Codes that Orphanet moved or referred to this one:
- ORPHA:99859OBSOLETE: Posttraumatic syringomyelia referred to · inactive
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
26 annotations · validated 2017-06-28| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0003396 | Syringomyelia | |
| Very frequent (99-80%) | ||
| HP:0040272 | Hyperintensity of MRI T2 signal of the spinal cord | |
| Frequent (79-30%) | ||
| HP:0003418 | Back pain | |
| HP:0012229 | CSF pleocytosis | |
| HP:0006824 | Cranial nerve paralysis | |
| HP:0000224 | Decreased taste sensation | |
| HP:0001618 | Dysphonia | |
| HP:0007209 | Facial paralysis | |
| HP:0003473 | Fatigable weakness | |
| HP:0001288 | Gait disturbance | |
| HP:0002922 | Increased CSF protein concentration | |
| HP:0010550 | Paraplegia | |
| HP:0003401 | Paresthesia | |
| HP:0010532 | Paroxysmal vertigo | |
| HP:0010871 | Sensory ataxia | |
| HP:0003474 | Somatic sensory dysfunction | |
| Occasional (29-5%) | ||
| HP:0000622 | Blurred vision | |
| HP:0001283 | Bulbar palsy | |
| HP:0007305 | CNS demyelination | |
| HP:0003409 | Distal sensory impairment of all modalities | |
| HP:0100518 | Dysuria | |
| HP:0002858 | Meningioma | |
| HP:0000639 | Nystagmus | |
| HP:0002073 | Progressive cerebellar ataxia | |
| HP:0007024 | Pseudobulbar paralysis | |
| HP:0001250 | Seizure | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 19991056[PMID]_2377746[PMID]_10967183[PMID]_11239955[PMID]_12181709[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/99857
- This record as JSON: /data/d/99857.json