Acute disseminated encephalomyelitis

Summary

Acute disseminated encephalomyelitis (MONDO:0019383) is a disease and 15 clinical trials. A subtype of demyelinating disease — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: Unknown (Worldwide) [Orphanet-validated]
• Phenotypes (HPO): 53
• Clinical trials: 15

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 1 000 000	0.6	Europe	Validated

Signs & symptoms

Clinical features (HPO)

53 HPO clinical features (Orphanet curated; top 50 by frequency):

HPO ID	Term	Frequency
HP:0001298	Encephalopathy	Obligate (100%)
HP:0007204	Diffuse white matter abnormalities	Very frequent (80-99%)
HP:0007305	CNS demyelination	Very frequent (80-99%)
HP:0010845	EEG with generalized slow activity	Very frequent (80-99%)
HP:0000708	Atypical behavior	Frequent (30-79%)
HP:0000737	Irritability	Frequent (30-79%)
HP:0001250	Seizure	Frequent (30-79%)
HP:0001251	Ataxia	Frequent (30-79%)
HP:0001268	Mental deterioration	Frequent (30-79%)
HP:0001317	Abnormal cerebellum morphology	Frequent (30-79%)
HP:0001945	Fever	Frequent (30-79%)
HP:0002018	Nausea	Frequent (30-79%)
HP:0002134	Abnormality of the basal ganglia	Frequent (30-79%)
HP:0002143	Abnormality of the spinal cord	Frequent (30-79%)
HP:0002181	Cerebral edema	Frequent (30-79%)
HP:0002315	Headache	Frequent (30-79%)
HP:0002518	Abnormal periventricular white matter morphology	Frequent (30-79%)
HP:0002922	Increased CSF protein concentration	Frequent (30-79%)
HP:0007256	Abnormal pyramidal sign	Frequent (30-79%)
HP:0012486	Myelitis	Frequent (30-79%)
HP:0012696	Abnormal thalamic MRI signal intensity	Frequent (30-79%)
HP:0012747	Abnormal brainstem MRI signal intensity	Frequent (30-79%)
HP:0025373	Interictal EEG abnormality	Frequent (30-79%)
HP:0031696	Disseminated viral infection	Frequent (30-79%)
HP:0032492	Anti-myelin oligodendrocyte glycoprotein antibody positivity	Frequent (30-79%)
HP:0100786	Hypersomnia	Frequent (30-79%)
HP:0200149	CSF lymphocytic pleiocytosis	Frequent (30-79%)
HP:0000572	Visual loss	Occasional (5-29%)
HP:0000718	Aggressive behavior	Occasional (5-29%)
HP:0001259	Coma	Occasional (5-29%)
HP:0001269	Hemiparesis	Occasional (5-29%)
HP:0001289	Confusion	Occasional (5-29%)
HP:0001324	Muscle weakness	Occasional (5-29%)
HP:0002013	Vomiting	Occasional (5-29%)
HP:0002167	Abnormality of speech or vocalization	Occasional (5-29%)
HP:0003474	Somatic sensory dysfunction	Occasional (5-29%)
HP:0004887	Respiratory failure requiring assisted ventilation	Occasional (5-29%)
HP:0006824	Cranial nerve paralysis	Occasional (5-29%)
HP:0010843	EEG with focal slow activity	Occasional (5-29%)
HP:0011151	Obtundation status	Occasional (5-29%)
HP:0011193	EEG with focal spikes	Occasional (5-29%)
HP:0030857	Eye movement-induced pain	Occasional (5-29%)
HP:0031179	Nuchal rigidity	Occasional (5-29%)
HP:0100653	Optic neuritis	Occasional (5-29%)
HP:0004305	Involuntary movements	Very rare (<1-4%)
HP:0006562	Viral hepatitis	Very rare (<1-4%)
HP:0006597	Diaphragmatic paralysis	Very rare (<1-4%)
HP:0007103	Hypointensity of cerebral white matter on MRI	Very rare (<1-4%)
HP:0012302	Herpes simplex encephalitis	Very rare (<1-4%)
HP:0020088	Post-vaccination measles	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	acute disseminated encephalomyelitis
Mondo ID	MONDO:0019383
EFO	EFO:0007130
MeSH	D004673
Orphanet	83597
DOID	DOID:639
ICD-11	1390433308
NCIT	C34578
SNOMED CT	83942000
UMLS	C0014059
MedGen	4033
GARD	0008639
NORD	727
Is cancer (heuristic)	no

Also known as: acute disseminated encephalitis · acute disseminated encephalomyelitis · ADE · ADEM · post-infectious encephalomyelitis

Disease family

This is a subtype of demyelinating disease. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › nervous system disorder › central nervous system disorder › neurodegenerative disease › demyelinating disease › acute disseminated encephalomyelitis

Related subtypes (7): demyelinating polyneuropathy, central pontine myelinolysis, polyradiculoneuropathy, Schilder disease, Balo concentric sclerosis, demyelinating disease of central nervous system, boylan dew greco syndrome

Subtypes (3): acute hemorrhagic leukoencephalitis, acute disseminated encephalomyelitis with anti-MOG antibodies, acute disseminated encephalomyelitis without anti-MOG antibodies

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 15.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	14
PHASE3	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT00004645	PHASE3	UNKNOWN	Phase III Randomized, Double-Blind, Sham-Controlled Study of Plasma Exchange for Acute Severe Attacks of Inflammatory Demyelinating Disease Refractory to Intravenous Methylprednisolone
NCT00445367	Not specified	ACTIVE_NOT_RECRUITING	Biobank For MS And Other Demyelinating Diseases
NCT05017142	Not specified	RECRUITING	Swiss Pediatric Inflammatory Brain Disease Registry (Swiss-Ped-IBrainD)
NCT05154370	Not specified	RECRUITING	China National Registry of Neuro-Inflammatory Diseases
NCT06474520	Not specified	RECRUITING	Efficacy and Safety of Calculus Bovis Sativus (CBS) for Idiopathic Inflammatory Demyelinating Disease (CBSinIIDD)
NCT06502015	Not specified	RECRUITING	Biomarkers in Autoimmune Disease of Nervous System
NCT06541626	Not specified	RECRUITING	Sun Yat-Sen Cohort of CNS Idiopathic Inflammatory Demyelinating Diseases
NCT06863974	Not specified	RECRUITING	High-throughput Omic Technology for Identification of Biomarkers of Relapsing Acute Disseminated Encephalomyelitis in Immune Cell Network
NCT07087873	Not specified	RECRUITING	Assessment of Transcranial Alternating Current Stimulation’s Clinical Efficacy in Treating Cognitive Impairment of Idiopathic Inflammatory Demyelinating Diseases
NCT07188194	Not specified	NOT_YET_RECRUITING	ADAM’S Prognostic Markers
NCT07540182	Not specified	NOT_YET_RECRUITING	The Aim of the Present Study is to Determine Outcome Predictors in Children Who Were Diagnosed as Acute Disseminated Encephalomyelitis (ADEM).
NCT03284801	Not specified	UNKNOWN	Management Of Acute Disseminating Encephalomyelitis
NCT03942952	Not specified	COMPLETED	PEDIATRIC SONICS: Pediatric Study of Neuropsychology and Imaging in CNS Demyelinating Syndromes.
NCT05630313	Not specified	UNKNOWN	Gene Sequencing as a Strategy for Identifying Genetic Factors Associated With Serious Adverse Events After Covid-19 Vaccines in Use in Brazil
NCT06443333	Not specified	UNKNOWN	National, Multicentric Registry Study on Neuroimmunological Diseases in China