raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:139417

Acute transverse myelitis

Type
Disease
Group
Disorder
Age of onset
Adolescent, Adult, Childhood, Elderly, Infancy
Status
active

A rare inflammatory demyelinating disorder of the spinal cord that can be either idiopathic (IATM) or secondary to a known cause (SATM).

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enAcute transverse myelitis
frMyélite transverse aiguë
deMyelitis, akute transverseQuerschnittsmyelitis
esMielitis transversa aguda
itMielite trasversa acuta
nlAcute transversale myelitisAcute myelitis transversa
ptMielite transversa, aguda
plOstre poprzeczne zapalenie rdzenia
csAkutní transverzální myelitida
ukГострий поперечний мієліт
zh急性横贯性脊髓炎
trAkut transvers miyelit

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10G37.3NTBTattributedvalidated
ICD-118A41.0NTBTindexvalidated
MONDO0015342Evalidated
UMLSC0270627Evalidated
GARD19913Evalidated

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):

Children (2):

Codes that Orphanet moved or referred to this one:

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

3 records
TypeClassValueAreaQualificationValidationSource
Annual incidence1-9 / 100 0008.6 / 100 000United StatesValue and classValidated31235660[PMID]
Point prevalence1-9 / 100 0007.9 / 100 000United StatesValue and classValidated31235660[PMID]
Annual incidence1-9 / 100 0004.72 / 100 000WorldwideValue and classValidated19812117[PMID]_19896453[PMID]_31235660[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

53 annotations · validated 2021-11-18
HPOTerm (as annotated by Orphanet)Diagnostic
Frequent (79-30%)
HP:0012332Abnormal autonomic nervous system physiology
HP:0002071Abnormality of extrapyramidal motor function
HP:5000013Anti-GFAP antibody
HP:0012229CSF pleocytosis
HP:0002922Increased CSF protein concentration
HP:0001324Muscle weakness
HP:0003474Somatic sensory dysfunction
Occasional (29-5%)
HP:0031845Abnormal libido
HP:0004370Abnormality of temperature regulation
HP:0025615Abscess
HP:0032492Anti-myelin oligodendrocyte glycoprotein antibody positivity
HP:0002960Autoimmunity
HP:0005341Autonomic bladder dysfunction
HP:0003487Babinski sign
HP:0003418Back pain
HP:0007305CNS demyelination
HP:0002019Constipation
HP:0011967Decreased circulating copper concentration
HP:0100502Decreased circulating vitamin B12 concentration
HP:0032283Disseminated nontuberculous mycobacterial infection
HP:0010835Dissociated sensory loss
HP:0009053Distal lower limb muscle weakness
HP:0012534Dysesthesia
HP:0032271Extrapulmonary tuberculosis
HP:0012378Fatigue
HP:0001945Fever
HP:0001288Gait disturbance
HP:0002578Gastroparesis
HP:0001347Hyperreflexia
HP:0000822Hypertension
HP:0011972Hypoglycorrhachia
HP:0002721Immunodeficiency
HP:0010831Impaired proprioception
HP:0002495Impaired vibratory sensation
HP:0031700Invasive parasitic infection
HP:0001287Meningitis
HP:0010547Muscle flaccidity
HP:0031179Nuchal rigidity
HP:0001278Orthostatic hypotension
HP:0002590Paralytic ileus
HP:0002385Paraparesis
HP:0010550Paraplegia
HP:0003401Paresthesia
HP:0031691Severe viral infection
HP:0001257Spasticity
HP:0002138Subarachnoid hemorrhage
HP:0002725Systemic lupus erythematosus
HP:0003484Upper limb muscle weakness
HP:0002493Upper motor neuron dysfunction
HP:0002839Urinary bladder sphincter dysfunction
HP:0000020Urinary incontinence
HP:0000016Urinary retention
Very rare (<4-1%)
HP:0200023Priapism

Annotation source: 29375121[PMID]_23186897[PMID]_24099672[PMID]

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Sources for this card