ORPHA:217260

Progressive multifocal leukoencephalopathy

Synonyms: PML · Progressive multifocal leukoencephalitis

Orphadata ships no English definition for this code.

Names in Orphadata languages

12 of 12 languages

Lang	Name	Synonyms
en	Progressive multifocal leukoencephalopathy	PML · Progressive multifocal leukoencephalitis
fr	Leucoencéphalopathie multifocale progressive	LMP · Leucoencéphalite multifocale progressive
de	Leukoenzephalopathie, multifokale progressive	Leukoenzephalitis, multifokale progressive · PML
es	Leucoencefalopatía multifocal progresiva	LMP · Leucoencefalitis multifocal progresiva
it	Leucoencefalopatia multifocale progressiva	Leucoencefalite multifocale progressiva
nl	Progressieve multifocale leuko-encefalopathie	PML · Progressieve multifocale leuko-encefalitis
pt	Leucoencefalite multifocal progressiva
pl	Postępująca wieloogniskowa leukoencefalopatia	Postępujące wieloogniskowe zapalenie istoty białej mózgu
cs	Progresivní multifokální leukoencefalopatie	Progresivní multifokální leukoencefalitida
uk	Прогресуюча мультифокальна лейкоенцефалопатія	PML · Прогресуючий мультифокальний лейкоенцефаліт
zh	进行性多病灶脑白质病	进行性多病灶脑白质病
tr	İlerleyici multifokal lökoensefalopati	PML · İlerleyici multifokal lökoensefalit

qualifier = Orphanet mapping relation

Source	Code	Qualifier	ICD relation	Validation
ICD-10	A81.2	E	specific	validated
ICD-11	8A45.02	E	specific	validated
MONDO	0016318	E		validated
MeSH	D007968	E		validated
UMLS	C0023524	E		validated
GARD	7468	E		validated
MedDRA	10036807	E		validated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

ORPHA:98252Infectious encephalitis
rare neurological diseases; rare infectious diseases

No children.

0 direct

Orphanet records no gene directly on this code.

3 records

Type	Class	Value	Area	Qualification	Validation	Source
Point prevalence	Unknown	—	Worldwide	Class only	Not yet validated	ORPHANET
Annual incidence	<1 / 1 000 000	0.068 / 100 000	Sweden	Value and class	Validated	29321229[PMID]
Annual incidence	1-9 / 1 000 000	0.11 / 100 000	France	Value and class	Validated	35779271[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.

35 annotations · validated 2020-02-25

HPO	Term (as annotated by Orphanet)	Diagnostic
Very frequent (99-80%)
HP:0100707	Abnormal astrocyte morphology
HP:0031392	Abnormal proportion of CD4 T cells
HP:0002921	Abnormality of the cerebrospinal fluid
HP:0000707	Abnormality of the nervous system
HP:0100706	Abnormality of the oligodendroglia
HP:0007305	CNS demyelination
HP:0004302	Functional motor deficit
HP:0002721	Immunodeficiency
Frequent (79-30%)
HP:0002167	Abnormality of speech or vocalization
HP:0100543	Cognitive impairment
HP:0005415	Decreased proportion of CD8-positive T cells
HP:0001260	Dysarthria
HP:0002066	Gait ataxia
HP:0002315	Headache
HP:0004374	Hemiplegia/hemiparesis
HP:0003690	Limb muscle weakness
HP:0001268	Mental deterioration
HP:0003474	Somatic sensory dysfunction
HP:0000505	Visual impairment
HP:0010549	Weakness due to upper motor neuron dysfunction
Occasional (29-5%)
HP:0002381	Aphasia
HP:0004377	Hematological neoplasm
HP:0030516	Homonymous hemianopia
HP:0000639	Nystagmus
HP:0012246	Oculomotor nerve palsy
HP:0001300	Parkinsonism
HP:0000751	Personality changes
HP:0001250	Seizure
HP:0025479	Self-neglect
HP:0002321	Vertigo
HP:0001123	Visual field defect
Very rare (<4-1%)
HP:0000651	Diplopia
HP:0001310	Dysmetria
HP:0001287	Meningitis
HP:0003401	Paresthesia

Annotation source: 29231623[PMID]_31580233[PMID]_30366552[PMID]_26421780[PMID]