Orphanet Rare Disease Ontology

Last uploaded: December 13, 2023
Preferred Name

Encephalitis lethargica

Synonyms

Von Economo encephalitis

Definitions

A rare brain inflammatory disease characterized by acute or subacute encephalitis with involvement of the midbrain and basal ganglia occurring in children as well as adults. Initial symptoms are pharyngitis and fever, followed by progressive lethargy, sleep disturbances, extrapyramidal symptoms (parkinsonism, chorea, dystonia), neuropsychiatric manifestations (obsessive-compulsive behavior, mutism, catatonia), and ocular features (oculogyric crises). Autoantibodies against human basal ganglia are often positive. Survivors may develop post-encephalitic syndromes, most prominently parkinsonism.

ID

http://www.orpha.net/ORDO/Orphanet_83600

alternative_term

Von Economo encephalitis

definition

A rare brain inflammatory disease characterized by acute or subacute encephalitis with involvement of the midbrain and basal ganglia occurring in children as well as adults. Initial symptoms are pharyngitis and fever, followed by progressive lethargy, sleep disturbances, extrapyramidal symptoms (parkinsonism, chorea, dystonia), neuropsychiatric manifestations (obsessive-compulsive behavior, mutism, catatonia), and ocular features (oculogyric crises). Autoantibodies against human basal ganglia are often positive. Survivors may develop post-encephalitic syndromes, most prominently parkinsonism.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=83600

has_age_of_onset

Adult

has_inheritance

Not applicable

hasDbXref

ICD-10:A85.8

UMLS:C0014040

MedDRA:10052369

ICD-11:1C80

label

Encephalitis lethargica

notation

ORPHA:83600

part_of

http://www.orpha.net/ORDO/Orphanet_306666

http://www.orpha.net/ORDO/Orphanet_166490

http://www.orpha.net/ORDO/Orphanet_98255

prefixIRI

ORDO:Orphanet_83600

prefLabel

Encephalitis lethargica

present_in

Europe AND has_point_prevalence_range : <1 / 1 000 000

treeView

http://www.orpha.net/ORDO/Orphanet_306666

http://www.orpha.net/ORDO/Orphanet_166490

http://www.orpha.net/ORDO/Orphanet_98255

subClassOf

http://www.orpha.net/ORDO/Orphanet_377788

http://www.orpha.net/ORDO/Orphanet_557493

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