PLS
MCID: LTR001
MIFTS: 55

Lateral Sclerosis (PLS) malady

Neuronal diseases category

Summaries for Lateral Sclerosis

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Sources:
42NIH Rare Diseases, 43NINDS, 63Wikipedia, 46OMIM, 32MalaCards
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NIH Rare Diseases:42 Primary lateral sclerosis is a type of motor neuron disease, where nerve cells that control voluntary muscle movement breakdown and die. in primary lateral sclerosis only the upper motor neurons in the brain are affected. symptoms often begin with problems in the legs (e.g., weakness, stiffness, spasticity, and balance problems), but may also start with hand clumsiness and changes in speech. the symptoms worsen gradually over time, however people with this condition have a normal life expectancy. progression of symptoms varies from person to person, some people retain the ability to walk without assistance, others eventually require assistive devices such as canes or wheelchairs. diagnosis requires extensive testing to exclude other diseases. treatment may include baclofen and tizanidine to reduce spasticity, quinine or phenytoin to reduce cramps, as well as physical and speech therapy as required. last updated: 1/22/2010

MalaCards: Lateral Sclerosis, also known as primary lateral sclerosis, is related to amyotrophic lateral sclerosis and motor neuron disease. An important gene associated with Lateral Sclerosis is FIG4 (FIG4 homolog, SAC1 lipid phosphatase domain containing (S. cerevisiae)), and among its related pathways is Pathogenesis of ALS. Affiliated tissues include spinal cord, brain and testes.

NINDS:43 Primary lateral sclerosis (PLS) is a rare neuromuscular disease with slowly progressive weakness in voluntary muscle movement. PLS belongs to a group of disorders known as motor neuron diseases. PLS affects the upper motor neurons (also called corticospinal neurons) in the arms, legs, and face.

Wikipedia:63 Amyotrophic lateral sclerosis (ALS)—also referred to as motor neurone disease (MND) in most... more...

Description from OMIM:46 611637

Aliases & Classifications for Lateral Sclerosis

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Sources:
8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 10DISEASES, 44Novoseek, 48Orphanet, 46OMIM, 60UMLS, 27ICD9CM, 56SNOMED-CT, 34MeSH, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Neuronal diseases


Characteristics (Orphanet epidemiological data):

48
primary lateral sclerosis:
Inheritance: Autosomal recessive,Sporadic; Prevalence: 1-9/100000; Age of onset: Variable; Age of death: Normal


Aliases & Descriptions:

lateral sclerosis 8 10 60
primary lateral sclerosis 8 42 43 44 48 46
adult-onset primary lateral sclerosis 48
primary lateral sclerosis, adult, 1 60
papillon-lefevre disease 60
adult-onset pls 48
pls 48


External Ids:

Disease Ontology8 DOID:230
ICD9CM27 335.24
MeSH34 D016472
SNOMED-CT56 81211007
OMIM46 611637
ICD10 via Orphanet26 G12.2
SNOMED-CT via Orphanet57 81211007

Related Diseases for Lateral Sclerosis

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Sources:
17GeneCards, 18GeneDecks
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Diseases in the Juvenile Primary Lateral Sclerosis family:

lateral sclerosis Primary Lateral Sclerosis, Adult, 1

Diseases related to Lateral Sclerosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 209)
idRelated DiseaseScoreTop Affiliating Genes
1amyotrophic lateral sclerosis32.3NEFH, PRPH
2motor neuron disease31.3SOD1, NEFH, ALS2
3frontotemporal dementia31.2FUS, C9orf72, SOD1
4neuropathy30.6NEFH, FIG4, SETX
5neuronitis11.1
6dementia10.9
7juvenile primary lateral sclerosis10.7
8muscular atrophy10.6
9amyotrophic lateral sclerosis type 210.5
10multiple sclerosis10.5
11amyotrophic lateral sclerosis, type 410.5
12amyotrophic lateral sclerosis type 810.5
13alzheimer's disease10.4
14spinal muscular atrophy10.4
15parkinson's disease10.4
16progressive muscular atrophy10.4
17amyotrophic lateral sclerosis type 1110.4
18amyotrophic lateral sclerosis type 910.4
19amyotrophic lateral sclerosis type 310.4
20amyotrophic lateral sclerosis type 710.4
21werdnig-hoffmann disease10.4
22progressive supranuclear palsy10.4
23amyotrophic lateral sclerosis type 1010.4
24amyotrophic lateral sclerosis with frontotemporal dementia10.4
25cerebritis10.3
26hereditary spastic paraplegia10.3
27pick's disease10.3
28cervicitis10.3
29neuromuscular disease10.3
30paraplegia10.3
31neurologic diseases10.3
32amyotrophic lateral sclerosis type 510.3
33amyotrophic lateral sclerosis-parkinsonism/dementia complex 110.3
34myocardial infarction10.3
35poliomyelitis10.3
36tauopathy10.3
37amyotrophic lateral sclerosis type 610.3
38amyotrophic lateral sclerosis-parkinsonism/dementia complex10.3
39clear cell acanthoma10.3
40huntington's disease10.2
41muscular dystrophy10.2
42polyneuropathy10.2
43spinal cord disease10.2
44tardbp-related amyotrophic lateral sclerosis10.2
45anosognosia10.2
46anterior horn cell disease10.2
47aphasia10.2
48cerebral degeneration10.2
49leukemia10.2
50respiratory failure10.2

Graphical network of the top 20 diseases related to Lateral Sclerosis:



Diseases related to lateral sclerosis

Clinical Features for Lateral Sclerosis

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Sources:
46OMIM
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Clinical features from OMIM:

611637

Clinical synopsis from OMIM:

611637

Drugs & Therapeutics for Lateral Sclerosis

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Lateral Sclerosis

Drug clinical trials:

Search ClinicalTrials for Lateral Sclerosis

Search NIH Clinical Center for Lateral Sclerosis

Search CenterWatch for Lateral Sclerosis

Genetic Tests for Lateral Sclerosis

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Anatomical Context for Lateral Sclerosis

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Sources:
32MalaCards
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MalaCards organs/tissues related to Lateral Sclerosis:

32
Spinal cord, Brain, Testes, Cortex, Skin, Endothelial, Skeletal muscle, Temporal lobe, Tongue, Monocytes, Eye, Bone, Salivary gland, Pituitary, Bone marrow, Prostate, Amygdala, Liver, Breast, Whole blood, Cerebellum

Animal Models for Lateral Sclerosis or affiliated genes

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Publications for Lateral Sclerosis

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Sources:
50PubMed
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Articles related to Lateral Sclerosis:

(show top 50)    (show all 1877)
idTitleAuthorsYear
1
Clinicopathologic features of autosomal recessive amyotrophic lateral sclerosis associated with optineurin mutation. (23889540)
2014
2
Screening of VCP mutations in Chinese amyotrophic lateral sclerosis patients. (23102936)
2013
3
Cerebrospinal fluid of newly diagnosed amyotrophic lateral sclerosis patients exhibits abnormal levels of selenium species including elevated selenite. (23732511)
2013
4
Facial nuclear degeneration on MRI in bulbar onset amyotrophic lateral sclerosis. (23681222)
2013
5
Epidemiological surveillance of amyotrophic lateral sclerosis in Saguenay region. (23968945)
2013
6
A novel phosphorylation site mutation in profilin 1 revealed in a large screen of US, Nordic, and German amyotrophic lateral sclerosis/frontotemporal dementia cohorts. (23141414)
2013
7
BMAALS: A French national project searching for a link between amyotrophic lateral sclerosis and the neurotoxic amino acid L-BMAA. (24098935)
2013
8
Spatial analysis of amyotrophic lateral sclerosis in Northern New England, USA, 1997-2009. (23881670)
2013
9
Investigating the contribution of VAPB/ALS8 loss of function in amyotrophic lateral sclerosis. (23446633)
2013
10
Chitotriosidase - a putative biomarker for sporadic amyotrophic lateral sclerosis. (24295388)
2013
11
Cardiac sympathetic function in the patients with amyotrophic lateral sclerosis: analysis using cardiac [123I] MIBG scintigraphy. (23784610)
2013
12
Novel deletion mutations of OPTN in amyotrophic lateral sclerosis in Japanese. (22402017)
2012
13
Primary lateral sclerosis as progressive supranuclear palsy: diagnosis by diffusion tensor imaging. (22517038)
2012
14
Clinical heterogeneity in Italian patients with amyotrophic lateral sclerosis. (21651514)
2012
15
Screening of the FUS gene in familial and sporadic amyotrophic lateral sclerosis patients of Chinese origin. (22340366)
2012
16
Motor neuron disease: Functional screening identifies novel candidate risk genes in amyotrophic lateral sclerosis. (22143366)
2012
17
Motor nerve conduction study in cauda equina with high-voltage electrical stimulation in multifocal motor neuropathy and amyotrophic lateral sclerosis. (21254095)
2011
18
FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration. (21408206)
2011
19
Familial frontotemporal dementia with amyotrophic lateral sclerosis and a shared haplotype on chromosomeA 9p. (21072532)
2011
20
Novel FUS/TLS mutations and pathology in familial and sporadic amyotrophic lateral sclerosis. (20385912)
2010
21
Amyotrophic lateral sclerosis and excitotoxicity: from pathological mechanism to therapeutic target. (20406181)
2010
22
SOD1 G93D mutation presenting as paucisymptomatic amyotrophic lateral sclerosis. (19922144)
2009
23
Thioredoxin reductase 1 haplotypes modify familial amyotrophic lateral sclerosis onset. (18996185)
2009
24
Update on fundamental and clinical research in amyotrophic lateral sclerosis]. (19419744)
2009
25
XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy. (19762508)
2009
26
The syndromes of frontotemporal dysfunction in amyotrophic lateral sclerosis. (18752088)
2008
27
Superoxide dismutase 1 mutants related to amyotrophic lateral sclerosis induce endoplasmic stress in neuro2a cells. (18233996)
2008
28
Oxidant-antioxidant imbalance in the erythrocytes of sporadic amyotrophic lateral sclerosis patients correlates with the progression of disease. (18308427)
2008
29
A caspase-3-cleaved fragment of the glial glutamate transporter EAAT2 is sumoylated and targeted to promyelocytic leukemia nuclear bodies in mutant SOD1-linked amyotrophic lateral sclerosis. (17823119)
2007
30
Identification of a novel D109Y mutation in Cu/Zn superoxide dismutase (sod1) gene associated with amyotrophic lateral sclerosis. (17257622)
2007
31
Histological recovery of the hepatocytes is based on the redox system upregulation in the animal models of mutant superoxide dismutase (SOD)1-linked amyotrophic lateral sclerosis. (16598672)
2006
32
Alterations in anti-oxidative defence enzymes in erythrocytes from sporadic amyotrophic lateral sclerosis (SALS) and familial ALS patients. (16681429)
2006
33
Amyotrophic lateral sclerosis 2-deficiency leads to neuronal degeneration in amyotrophic lateral sclerosis through altered AMPA receptor trafficking. (17093100)
2006
34
Expression of ubiquitin and proteasome in motorneurons and astrocytes of spinal cords from patients with amyotrophic lateral sclerosis. (16806703)
2006
35
Sporadic amyotrophic lateral sclerosis and breast cancer: Hyaline conglomerate inclusions lead to identification of SOD1 mutation. (16423367)
2006
36
Unsaturated fatty acids induce cytotoxic aggregate formation of amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutants. (15799963)
2005
37
Astrocytic production of nerve growth factor in motor neuron apoptosis: implications for amyotrophic lateral sclerosis. (15056289)
2004
38
Treatment for spasticity in amyotrophic lateral sclerosis/motor neuron disease. (14974059)
2004
39
Decreased galectin-1 immunoreactivity of the skin in amyotrophic lateral sclerosis. (12639727)
2003
40
Selective loss of trans-acting instability determinants of neurofilament mRNA in amyotrophic lateral sclerosis spinal cord. (12730211)
2003
41
S100A6, a calcium- and zinc-binding protein, is overexpressed in SOD1 mutant mice, a model for amyotrophic lateral sclerosis. (11108968)
2000
42
Amyotrophic lateral sclerosis in an adult following acute paralytic poliomyelitis in early childhood. (10090681)
1999
43
Eosinophilic intranuclear inclusions in the hippocampal pyramidal neurons of a patient with amyotrophic lateral sclerosis. (9144593)
1997
44
Difficulties in distinguishing sporadic from familial amyotrophic lateral sclerosis. (8651656)
1996
45
Patients with amyotrophic lateral sclerosis and other neurodegenerative diseases have increased levels of neurofilament protein in CSF. (8863508)
1996
46
Investigation of a null mutation of the CNTF gene in familial amyotrophic lateral sclerosis. (8543936)
1995
47
Coexistence of amyotrophic lateral sclerosis and Werdnig-Hoffmann disease within a family. (7807154)
1994
48
Analysis of the functional effects of a mutation in SOD1 associated with familial amyotrophic lateral sclerosis. (7917302)
1994
49
Lathyrism and western Pacific amyotrophic lateral sclerosis: etiology of short and long latency motor system disorders. (1906674)
1991
50
Effect of thyroliberin treatment on the thyrotropin and prolactin levels in patients with amyotrophic lateral sclerosis]. (2132051)
1990

Genetic Variations for Lateral Sclerosis

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Expression for genes affiliated with Lateral Sclerosis

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Lateral Sclerosis

Search GEO for disease gene expression data for Lateral Sclerosis.

Pathways for genes affiliated with Lateral Sclerosis

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51QIAGEN, 29KEGG
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Pathways related to Lateral Sclerosis according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
11.1ALS2, PRPH, NEFH, SOD1

Compounds for genes affiliated with Lateral Sclerosis

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GO Terms for genes affiliated with Lateral Sclerosis

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16Gene Ontology
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Cellular components related to Lateral Sclerosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1neurofilamentGO:00588310.8PRPH, NEFH

Biological processes related to Lateral Sclerosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cell deathGO:00821911.4ALS2, NEFH, C9orf72, FIG4, TRPM7, SETX
2locomotory behaviorGO:00762610.9ALS2, FIG4, SOD1
3neurofilament cytoskeleton organizationGO:06005210.5NEFH, SOD1

Products for genes affiliated with Lateral Sclerosis

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Sources for Lateral Sclerosis

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet