Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
Preferred Name

Primary lateral sclerosis
Synonyms

PLS

Adult-onset PLS

Adult-onset primary lateral sclerosis

Definitions

Primary lateral sclerosis (PLS) is an idiopathic non-familial motor neuron disease characterized by slowly progressive upper motor neuron dysfunction leading to spasticity, mild weakness in voluntary muscle movement, hyperreflexia, and loss of motor speech production.

ID

http://www.orpha.net/ORDO/Orphanet_35689

alternative_term

PLS

Adult-onset PLS

Adult-onset primary lateral sclerosis

definition

Primary lateral sclerosis (PLS) is an idiopathic non-familial motor neuron disease characterized by slowly progressive upper motor neuron dysfunction leading to spasticity, mild weakness in voluntary muscle movement, hyperreflexia, and loss of motor speech production.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=35689

has_age_of_onset

Adult

Elderly

has_inheritance

Not applicable

Autosomal recessive

Autosomal dominant

hasDbXref

ICD-10:G12.2

ICD-11:8B60.4

UMLS:C0154682

MedDRA:10036704

OMIM:611637

label

Primary lateral sclerosis

notation

ORPHA:35689

part_of

http://www.orpha.net/ORDO/Orphanet_183500

http://www.orpha.net/ORDO/Orphanet_182070

http://www.orpha.net/ORDO/Orphanet_98503

prefixIRI

ORDO:Orphanet_35689

prefLabel

Primary lateral sclerosis

present_in

Europe AND has_point_prevalence_average_value : 1.5 AND has_point_prevalence_range : 1-9 / 100 000

treeView

http://www.orpha.net/ORDO/Orphanet_183500

http://www.orpha.net/ORDO/Orphanet_182070

http://www.orpha.net/ORDO/Orphanet_98503

subClassOf

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

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

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