Primary lateral sclerosis, adult, 1

Preferred Label : Primary lateral sclerosis, adult, 1;

Symbol : PLSA1;

CISMeF acronym : PLSA; PLSA1;

Type : Phenotype or locus, molecular basis unknown;

Alternative titles and symbols : PLSA; Pls, adult;

Description : Although primary lateral sclerosis (PLS) is similar to amyotrophic lateral sclerosis (ALS; 105400), they are considered to be clinically distinct progressive paralytic neurodegenerative disorders. Following a period of diagnostic confusion, the clinical distinction between ALS and PLS became clear and diagnostic criteria were established (Pringle et al., 1992). PLS is characterized by degeneration of the upper motor neurons and the corticospinal and corticobulbar tracts, whereas ALS is a more severe disorder characterized by degeneration of both the upper and lower motor neurons. See 606353 for autosomal recessive juvenile-onset PLS, which is caused by mutations in the ALS2 gene (606352).;

Inheritance : Autosomal dominant;

Prefixed ID : %611637;

Details

Origin ID

611637;

UMLS CUI

C1968845;

Automatic exact mappings (from CISMeF team)
- Primary lateral sclerosis [ORDO Disease]
Currated CISMeF NLP mapping
- primary lateral sclerosis, adult, 1 [MeSH concept]
- primary lateral sclerosis, adult, 1 [MeSH Supplementary Concept]
DO Cross reference
- lateral sclerosis [DO Concept]
HPO term(s)
- Abnormal upper motor neuron morphology [HPO term]
- Adult onset [HPO term]
- Autosomal dominant inheritance [HPO term]
- Babinski sign [HPO term]
- Dysphagia [HPO term]
- Hyperreflexia [HPO term]
- Slowly progressive [HPO term]
- Spastic dysarthria [HPO term]
- Spastic gait [HPO term]
- Spastic tetraparesis [HPO term]
ORDO concept(s)
- Primary lateral sclerosis [ORDO Disease]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
UMLS correspondences (same concept)
- primary lateral sclerosis, adult, 1 [MeSH Supplementary Concept]
- primary lateral sclerosis, adult, 1 [MeSH concept]

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