PLS
MCID: LTR001
MIFTS: 56

Lateral Sclerosis (PLS)

Categories: Genetic diseases, Muscle diseases, Neuronal diseases, Rare diseases
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Aliases & Classifications for Lateral Sclerosis

MalaCards integrated aliases for Lateral Sclerosis:

Name: Lateral Sclerosis 11 14 71 33
Primary Lateral Sclerosis 11 19 52 58 75 53 33
Adult-Onset Primary Lateral Sclerosis 11 19 58
Motor Neuron Disease 43 71
Adult-Onset Pls 19 58
Pls - [primary Lateral Sclerosis] 33
Lateral Incomplete Paralysis 33
Lateral Complete Paralysis 33
Lateral Spinal Sclerosis 33
Lateral Paralysis 33
Pls 58

Characteristics:


Inheritance:

Primary Lateral Sclerosis: Autosomal dominant,Autosomal recessive 58

Prevelance:

Primary Lateral Sclerosis: 1-9/100000 (Europe) 58

Age Of Onset:

Primary Lateral Sclerosis: Adult,Elderly 58

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Disease Ontology 11 DOID:230
ICD9CM 34 335.24
MeSH 43 D016472
NCIt 49 C129933
SNOMED-CT 68 81211007
ICD10 via Orphanet 32 G12.2
UMLS via Orphanet 72 C0154682 C1968845
Orphanet 58 ORPHA35689
ICD11 33 1686688462
UMLS 71 C0085084 C0154682

Summaries for Lateral Sclerosis

NINDS: 52 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.  It occurs when nerve cells in the motor regions of the cerebral cortex (the thin layer of cells covering the brain which is responsible for most higher level mental functions) gradually degenerate, causing movements to be slow and effortful.  The disorder often affects the legs first, followed by the body, trunk, arms and hands, and, finally the bulbar muscles (muscles that control speech, swallowing, and chewing).  Symptoms include weakness, muscle stiffness and spasticity, clumsiness, slowing of movement, and problems with balance and speech. PLS is more common in men than in women, with a varied gradual onset that generally occurs between ages 40 and 60. PLS progresses gradually over a number of years, or even decades. Scientists do not believe PLS has a simple hereditary cause.  The diagnosis of PLS requires extensive testing to exclude other diseases. When symptoms begin, PLS may be mistaken for amyotrophic lateral sclerosis (ALS) or spastic paraplegia.  Most neurologists follow an affected individual's clinical course for at least 3 to 4 years before making a diagnosis of PLS.

MalaCards based summary: Lateral Sclerosis, also known as primary lateral sclerosis, is related to frontotemporal dementia and/or amyotrophic lateral sclerosis 2 and frontotemporal dementia and/or amyotrophic lateral sclerosis 3, and has symptoms including ataxia, myoclonus and hemiplegia. An important gene associated with Lateral Sclerosis is SPG7 (SPG7 Matrix AAA Peptidase Subunit, Paraplegin), and among its related pathways/superpathways is Amyotrophic lateral sclerosis (ALS). The drugs Mexiletine and Dronabinol have been mentioned in the context of this disorder. Affiliated tissues include spinal cord, bone marrow and brain, and related phenotypes are babinski sign and abnormal upper motor neuron morphology

GARD: 19 Primary lateral sclerosis (PLS) is a rare neuromuscular disease that affects the nerve cells that control the voluntary muscles. Problems in the legs (such as weakness, stiffness, spasticity, and balance problems) are often observed first, but hand clumsiness and changes in speech can be early symptoms, as well. The underlying cause of adult PLS is currently unknown. In most cases, it occurs sporadically in people with no family history of the condition. A subtype of PLS, called juvenile PLS, is caused by changes in the ALS2 gene and is inherited in an autosomal recessive manner.

Orphanet: 58 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.

Disease Ontology: 11 A motor neuron disease characterized by painless but progressive weakness and stiffness of the muscles of the legs.

Wikipedia: 75 Primary lateral sclerosis (PLS) is a very rare neuromuscular disease characterized by progressive muscle... more...

Related Diseases for Lateral Sclerosis

Diseases in the Lateral Sclerosis family:

Primary Lateral Sclerosis, Juvenile Primary Lateral Sclerosis, Adult, 1

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

(show top 50) (show all 1246)
# Related Disease Score Top Affiliating Genes
1 frontotemporal dementia and/or amyotrophic lateral sclerosis 2 33.2 PRPH CHCHD10 C9orf72
2 frontotemporal dementia and/or amyotrophic lateral sclerosis 3 33.2 PRPH CHCHD10 C9orf72
3 frontotemporal dementia and/or amyotrophic lateral sclerosis 4 33.1 SOD1 PRPH CHCHD10 C9orf72
4 frontotemporal dementia and/or amyotrophic lateral sclerosis 7 33.1 VCP FUS
5 amyotrophic lateral sclerosis 19 33.1 SOD1 PRPH C9orf72 ALS2
6 amyotrophic lateral sclerosis-parkinsonism/dementia complex 1 33.1 YWHAQ TRPM7 SOD1 FUS C9orf72
7 amyotrophic lateral sclerosis 6 with or without frontotemporal dementia 33.1 VCP FUS
8 amyotrophic lateral sclerosis 11 33.1 SOD1 SETX FUS C9orf72 ALS2
9 amyotrophic lateral sclerosis type 22 33.0 PRPH CHCHD10 C9orf72
10 amyotrophic lateral sclerosis 20 33.0 SOD1 PRPH HNRNPA1 C9orf72 ALS2
11 amyotrophic lateral sclerosis type 5 32.9 SPG11 ALS2
12 amyotrophic lateral sclerosis 21 32.9 SOD1 SETX PRPH FUS ATXN2 ALS2
13 amyotrophic lateral sclerosis 10 with or without frontotemporal dementia 32.9 SPG11 SOD1 SETX FUS C9orf72 ATXN2
14 amyotrophic lateral sclerosis 8 32.9 SPG11 SOD1 SETX FUS C9orf72 ALS2
15 amyotrophic lateral sclerosis 4, juvenile 32.9 SPG11 SOD1 SETX FUS C9orf72 ATXN2
16 spinal muscular atrophy, distal, autosomal recessive, 4 32.9 SPG11 ALS2
17 amyotrophic lateral sclerosis 18 32.8 SOD1 SIGMAR1 SETX FUS CHCHD10 C9orf72
18 juvenile amyotrophic lateral sclerosis 32.8 SPG11 SIGMAR1 SETX FUS ALS2
19 amyotrophic lateral sclerosis type 12 32.8 SPG11 SOD1 SETX FUS ALS2
20 amyotrophic lateral sclerosis 16, juvenile 32.7 VCP SPG11 SOD1 SIGMAR1 SETX FUS
21 amyotrophic lateral sclerosis type 14 32.6 VCP SOD1 SIGMAR1 SETX FUS CHCHD10
22 amyotrophic lateral sclerosis type 15 32.5 VCP SPG11 SOD1 SIGMAR1 SETX C9orf72
23 inclusion body myopathy with paget disease of bone and frontotemporal dementia 32.5 VCP SOD1 HNRNPA1 FUS CHCHD10 C9orf72
24 amyotrophic lateral sclerosis type 6 32.5 VCP SPG11 SOD1 SIGMAR1 SETX FUS
25 spinocerebellar ataxia 2 32.4 VCP SPG11 SOD1 SETX HNRNPA1 FUS
26 frontotemporal dementia and/or amyotrophic lateral sclerosis 1 32.4 VCP SPG11 SOD1 SIGMAR1 SETX NEFH
27 amyotrophic lateral sclerosis 1 32.2 YWHAQ VCP SPG11 SOD1 SIGMAR1 SETX
28 multisystem proteinopathy 32.2 VCP HNRNPA1 FUS C9orf72 ATXN2
29 progressive bulbar palsy 32.2 SOD1 SETX NEFH FUS C9orf72 ALS2
30 motor neuron disease 32.2 VCP SOD1 SIGMAR1 SETX PRPH NEFH
31 frontotemporal dementia 32.1 YWHAQ VCP SOD1 SETX NEFH HNRNPA1
32 perry syndrome 32.1 VCP FUS C9orf72
33 pick disease of brain 32.0 YWHAQ VCP SOD1 NEFH FUS C9orf72
34 neuromuscular disease 31.8 VCP SPG11 SOD1 SETX NEFH HNRNPA1
35 muscular atrophy 31.7 SIGMAR1 SETX HNRNPA1 FUS CHCHD10 C9orf72
36 nervous system disease 31.5 YWHAQ VCP SOD1 NEFH HNRNPA1 FUS
37 dementia 31.5 VCP TRPM7 SOD1 HNRNPA1 FUS CHCHD10
38 dementia, lewy body 31.4 YWHAQ VCP SOD1 FUS C9orf72
39 aphasia 31.4 VCP FUS C9orf72
40 progressive muscular atrophy 31.2 VCP SOD1 SETX NEFH FUS CHCHD10
41 locked-in syndrome 31.2 SOD1 FUS ALS2
42 spinal muscular atrophy 31.2 SOD1 SIGMAR1 HNRNPA1 FUS CHCHD10 C9orf72
43 alzheimer disease, familial, 1 31.2 YWHAQ VCP SOD1 NEFH FUS C9orf72
44 myopathy 31.2 VCP SOD1 HNRNPA1 FUS CHCHD10 C9orf72
45 spinal and bulbar muscular atrophy, x-linked 1 31.1 VCP SOD1 NEFH FUS C9orf72 ATXN2
46 charcot-marie-tooth disease 31.1 VCP SPG7 SPG11 SOD1 SIGMAR1 SETX
47 pseudobulbar palsy 31.1 SOD1 FUS C9orf72
48 progressive non-fluent aphasia 31.0 VCP C9orf72
49 parkinson disease, late-onset 31.0 YWHAQ VCP SPG11 SOD1 RNF19A FUS
50 nominal aphasia 30.9 VCP FUS C9orf72

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



Diseases related to Lateral Sclerosis

Symptoms & Phenotypes for Lateral Sclerosis

Human phenotypes related to Lateral Sclerosis:

58 30 (show all 18)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 babinski sign 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0003487
2 abnormal upper motor neuron morphology 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002127
3 generalized hyperreflexia 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0007034
4 dysphagia 58 30 Frequent (33%) Frequent (79-30%)
HP:0002015
5 loss of speech 58 30 Frequent (33%) Frequent (79-30%)
HP:0002371
6 spastic gait 58 30 Frequent (33%) Frequent (79-30%)
HP:0002064
7 emg: chronic denervation signs 58 30 Frequent (33%) Frequent (79-30%)
HP:0003444
8 spastic dysarthria 58 30 Frequent (33%) Frequent (79-30%)
HP:0002464
9 pseudobulbar signs 58 30 Frequent (33%) Frequent (79-30%)
HP:0002200
10 progressive spastic paraparesis 58 30 Frequent (33%) Frequent (79-30%)
HP:0007199
11 motor axonal neuropathy 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0007002
12 cervical spinal cord atrophy 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0010873
13 spasticity 58 Very frequent (99-80%)
14 upper motor neuron dysfunction 58 Very frequent (99-80%)
15 sensory impairment 58 Excluded (0%)
16 atrophy of the spinal cord 58 Occasional (29-5%)
17 abnormal lower motor neuron morphology 58 Excluded (0%)
18 weakness due to upper motor neuron dysfunction 58 Frequent (79-30%)

UMLS symptoms related to Lateral Sclerosis:


ataxia; myoclonus; hemiplegia; muscular fasciculation

GenomeRNAi Phenotypes related to Lateral Sclerosis according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 no effect GR00402-S-1 10.18 ALS2 ANG ATXN2 C9orf72 CHCHD10 DPP6
2 no effect GR00402-S-2 10.18 ALS2 ANG ATXN2 C9orf72 DPP6 FGGY

MGI Mouse Phenotypes related to Lateral Sclerosis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 nervous system MP:0003631 10.03 ALS2 ATXN2 C9orf72 CHCHD10 DPP6 FUS
2 cellular MP:0005384 9.73 ANG C9orf72 CHCHD10 FUS NEFH RNF19A
3 behavior/neurological MP:0005386 9.47 ALS2 ATXN2 C9orf72 CHCHD10 FUS NEFH

Drugs & Therapeutics for Lateral Sclerosis

Drugs for Lateral Sclerosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 339)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Mexiletine Approved, Investigational Phase 4 5370-01-4, 31828-71-4 4178
2
Dronabinol Approved, Illicit Phase 4 1972-08-3 16078
3
Cannabidiol Approved, Investigational Phase 4 13956-29-1 521372 644019
4 Coconut Approved Phase 4
5 Anti-Arrhythmia Agents Phase 4
6 Sodium Channel Blockers Phase 4
7 Diuretics, Potassium Sparing Phase 4
8
Olanzapine Approved, Investigational Phase 2, Phase 3 132539-06-1 135398745 4585
9
Valproic acid Approved, Investigational Phase 3 99-66-1 3121
10
Ceftriaxone Approved Phase 3 73384-59-5 5479530
11
Quinidine Approved, Investigational Phase 3 56-54-2, 130-95-0, 804-63-7 1065 441074 3034034 8549
12
Cathine Approved, Experimental, Illicit, Vet_approved, Withdrawn Phase 3 14838-15-4, 492-39-7 131954576 4786 26934
13
Guaifenesin Approved, Investigational, Vet_approved Phase 3 93-14-1 3516
14
Dextromethorphan Approved Phase 3 125-71-3 5362449 5360696
15
Mecobalamin Approved, Investigational Phase 2, Phase 3 13422-55-4
16
Lenograstim Approved, Investigational Phase 2, Phase 3 135968-09-1
17
Hydroxocobalamin Approved Phase 2, Phase 3 13422-51-0 15589840 44475014
18
Acetylcholine Approved, Investigational Phase 2, Phase 3 51-84-3 187
19
Minocycline Approved, Investigational Phase 3 10118-90-8, 13614-98-7 54675783 5281021
20
Mecasermin Approved, Investigational Phase 3 68562-41-4
21
Citalopram Approved Phase 3 59729-32-7, 59729-33-8 2771
22
Edaravone Approved, Investigational Phase 3 89-25-8 4021 70335
23
Memantine Approved, Investigational Phase 2, Phase 3 41100-52-1, 19982-08-2 4054
24
Benzocaine Approved, Investigational Phase 3 1994-09-7, 94-09-7 2337
25
Tannic acid Approved Phase 3 1401-55-4 16129878 16129778
26
Trazodone Approved, Investigational Phase 2, Phase 3 19794-93-5 5533
27
Dolutegravir Approved Phase 3 1051375-16-6 57414794 54726191
28
Lamivudine Approved, Investigational Phase 3 134678-17-4 60825
29
Abacavir Approved, Investigational Phase 3 136470-78-5, 188062-50-2 441300
30
Iron Approved Phase 2, Phase 3 7439-89-6 29936
31
Deferiprone Approved Phase 2, Phase 3 30652-11-0 2972
32
Coal tar Approved Phase 2, Phase 3 8007-45-2
33
Lithium carbonate Approved Phase 2, Phase 3 554-13-2
34
Dopamine Approved Phase 3 62-31-7, 51-61-6 681
35
Pramipexole Approved, Investigational Phase 3 104632-26-0, 104632-25-9, 104632-28-2 4885 119570 59868
36
Sodium citrate Approved, Investigational Phase 3 68-04-2 23431961
37
Ravulizumab Approved, Investigational Phase 3 1803171-55-2
38
Ubidecarenone Approved, Investigational, Nutraceutical Phase 3 303-98-0 5281915
39
Cyanocobalamin Approved, Nutraceutical Phase 2, Phase 3 68-19-9 24892734 16212801 44176380
40
D-Tyrosine Approved, Experimental, Investigational, Nutraceutical Phase 3 133585-56-5, 60-18-4, 556-02-5 1153 6057
41
Citric acid Approved, Nutraceutical, Vet_approved Phase 3 77-92-9 311
42
Cobalamin Experimental Phase 2, Phase 3 13408-78-1 6857388
43
Dexetimide Withdrawn Phase 3 21888-98-2 30843
44
Simendan Investigational Phase 3 131741-08-7
45
Nabiximols Investigational Phase 2, Phase 3 56575-23-6
46
Ibudilast Investigational Phase 2, Phase 3 50847-11-5 3671
47
Trichostatin A Experimental Phase 3 58880-19-6 5562 444732
48 Antipsychotic Agents Phase 2, Phase 3
49 Antiemetics Phase 2, Phase 3
50 Analgesics Phase 2, Phase 3

Interventional clinical trials:

(show top 50) (show all 716)
# Name Status NCT ID Phase Drugs
1 Role of Non-invasive Ventilation in Amyotrophic Lateral Sclerosis: Volume Versus Pressure Mode Unknown status NCT00560287 Phase 4
2 Care (Canadian ALS Riluzole Evaluation) Multicentre Phase IV Comparative Study of the Effects of Riluzole 50mg Bid on the Survival of ALS Subjects Compared to Historical Controls Completed NCT00542412 Phase 4 Riluzole
3 Feasibility of Telesurveillance and Home Cough Assistance for Amyotrophic Lateral Patients (ALS) Completed NCT00613899 Phase 4
4 Mexiletine for the Treatment of Muscle Cramps in ALS Completed NCT01811355 Phase 4 Mexiletine;Placebo
5 EMERALD TRIAL Open-Label Extension Study (EMERALD OLE) Not yet recruiting NCT04997954 Phase 4 MediCabilis CBD oil
6 A Multicenter, Randomized, Double-Blind, Placebo-Controlled Phase 2 Study of Cu(II)ATSM in Patients With Amyotrophic Lateral Sclerosis/Motor Neuron Disease Unknown status NCT04082832 Phase 2, Phase 3 Cu(II)ATSM;Placebos
7 A Phase III, Multi-Center, Double-Blind, Placebo Controlled, Randomized Study of Creatine Monohydrate in Patients With Amyotrophic Lateral Sclerosis Unknown status NCT00069186 Phase 3 Creatine Monohydrate
8 Japanese Early-stage Clinical Trial of Ultra-high Dose Methylcobalamin for Amyotrophic Lateral Sclerosis: a Pivotal Phase 3 Randomized Controlled Study Unknown status NCT03548311 Phase 3 methylcobalamin;saline solution
9 Randomized, Placebo-controlled Parallel Group Study for the Evaluation of an Oral Dose of 10mg Olanzapine in Combination With Riluzole for the Treatment of Loss of Appetite in Patients With Amyotrophic Lateral Sclerosis (ALS) Unknown status NCT00876772 Phase 2, Phase 3 Olanzapine
10 Treatment Effect of Edaravone in Patients With Amyotrophic Lateral Sclerosis (ALS) in a Representative Iranian Population Unknown status NCT03272802 Phase 2, Phase 3 Edaravone;Riluzole
11 A Study to Evaluate the Efficacy, Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of BIIB067 Administered to Adult Subjects With Amyotrophic Lateral Sclerosis and Confirmed Superoxide Dismutase 1 Mutation Completed NCT02623699 Phase 3 BIIB067
12 Multicenter, Randomised, Double-blind, Placebo-controlled, Parallel Group, Phase 2/3 Study to Compare the Efficacy and Safety of Masitinib Completed NCT02588677 Phase 2, Phase 3 Masitinib (4.5);Riluzole;Placebo;Masitinib (3.0)
13 Phase II/III, Multicenter, Randomized, Parallel Group, Double-blind, Placebo Controlled Study to Assess Safety and Efficacy of TRO19622 in Amyotrophic Lateral Sclerosis (ALS) Patients Treated With Riluzole Completed NCT00868166 Phase 3 Olesoxime;Placebo Comparator;Riluzole
14 An Expanded Controlled Study of MCI-186 for Treatment of Amyotrophic Lateral Sclerosis in Double-Blind, Parallel-Group, Placebo-Controlled Manner (Phase 3) Completed NCT00424463 Phase 3 MCI-186;Placebo of MCI-186
15 An Exploratory Study of MCI-186 for Treatment of Amyotrophic Lateral Sclerosis (Severity Classification III) in Double-Blind, Parallel-Group, Placebo-Controlled Manner Completed NCT00415519 Phase 3 MCI-186;Placebo of MCI-186
16 Noninvasive Ventilation in ALS Patients With Mild Respiratory Involvement Completed NCT00386464 Phase 2, Phase 3
17 Randomized Crossover Design Trial of Vitamin E vs Placebo for Treatment of Cramps in Amyotrophic Lateral Sclerosis. Completed NCT00372879 Phase 3
18 Effect of Intrathecal Administration of Hematopoietic Stem Cells in Patients With Amyotrophic Lateral Sclerosis (ALS) Completed NCT01933321 Phase 2, Phase 3
19 A Long-Term Study in Patients With Amyotrophic Lateral Sclerosis (ALS) Completed NCT00445172 Phase 2, Phase 3 E0302 (mecobalamin)
20 A Phase 3, Randomized Double-Blind, Placebo-Controlled Multicenter Study to Evaluate Efficacy and Safety of Repeated Administration of NurOwn® (Autologous Mesenchymal Stem Cells Secreting Neurotrophic Factors) in Participants With ALS Completed NCT03280056 Phase 3
21 A Phase II/III Study in Patients With Amyotrophic Lateral Sclerosis (ALS) Completed NCT00444613 Phase 2, Phase 3 E0302 (mecobalamin);Placebo
22 Phase II/III Randomized, Placebo-Controlled Trial of Arimoclomol in SOD1 Positive Familial Amyotrophic Lateral Sclerosis (ALS) Completed NCT00706147 Phase 2, Phase 3 Arimoclomol;Placebo
23 Repetitive Transcranial Magnetic Stimulation in Amyotrophic Lateral Sclerosis Completed NCT00833820 Phase 2, Phase 3
24 A Phase 3, Multi-National, Double-Blind, Randomized, Placebo-Controlled, Stratified, Parallel Group, Study to Evaluate the Safety, Tolerability and Efficacy of Tirasemtiv in Patients With Amyotrophic Lateral Sclerosis (ALS) Completed NCT02496767 Phase 3 Tirasemtiv;Placebo tablets
25 A Randomized, Double-Blind, Placebo-Controlled Sequential Clinical Trial of Sodium Valproate in ALS Completed NCT00136110 Phase 3 Sodium Valproate
26 Minocycline to Treat Amyotrophic Lateral Sclerosis Completed NCT00047723 Phase 3 minocycline
27 Insulin-like Growth Factor-1 in Amyotrophic Lateral Sclerosis (ALS) Completed NCT00035815 Phase 3 Insulin like growth factor, type 1;Placebo
28 Phase 2-3 - Memantine for Disability in Amyotrophic Lateral Sclerosis Completed NCT00353665 Phase 2, Phase 3 Memantine (Ebixa);riluzole;Placebo
29 Clinical Trial Ceftriaxone in Subjects With Amyotrophic Lateral Sclerosis (ALS) Completed NCT00349622 Phase 3 ceftriaxone
30 A Confirmatory Study of MCI-186 for Treatment of Amyotrophic Lateral Sclerosis in Double-blind, Parallel-group, Placebo-controlled Manner. Completed NCT00330681 Phase 3 MCI-186;Placebo of MCI-186
31 A Double-Blind Controlled, Multicenter Phase II/III Study to Assess the Safety and Efficacy of AVP-923 (Dextromethorphan/Quinidine) in the Treatment of Pseudobulbar Affect in Patients With Amyotrophic Lateral Sclerosis Completed NCT00021697 Phase 3 AVP-923
32 An Open-label, 8- Week, Flexible Dose Trial of Escitalopram (Lexapro®) in Comorbid Major Depression With Amyotrophic Lateral Sclerosis and Multiple Sclerosis Completed NCT00965497 Phase 3 escitalopram
33 A Randomized, Double-Blind, Placebo-Controlled Study of Safety and Efficacy of Botulinum Toxin Type B (Myobloc) in Sialorrhea in Amyotrophic Lateral Sclerosis Completed NCT00125203 Phase 2, Phase 3 Botulinum toxin type B (Myobloc)
34 Effects of Oral Levosimendan (ODM-109) on Respiratory Function in Patients With ALS: Open-Label Extension for Patients Completing Study 3119002 Completed NCT03948178 Phase 3 Levosimendan
35 An Open-label Safety Extension Study of TRO19622 in Amyotrophic Lateral Sclerosis (ALS) Patients Treated With Riluzole Completed NCT01285583 Phase 2, Phase 3 TRO19622
36 A Phase 3, Open-Label Extension Study of Tirasemtiv for Patients With Amyotrophic Lateral Sclerosis (ALS) Who Completed VITALITY-ALS (CY 4031) Completed NCT02936635 Phase 3 tirasemtiv
37 A Fase II, Randomized, Double-Blind, Placebo-Controlled, Multicentre Study for the Safety and Efficacy on Spasticity Symptoms of a Cannabis Sativa Extract in Motor Neuron Disease Patients Completed NCT01776970 Phase 2, Phase 3 Cannabis Sativa extract Oromucosal spray
38 A Randomized, Double-Blind, Placebo-Controlled, Multi-Center Study of the Safety and Efficacy of Dexpramipexole in Subjects With Amyotrophic Lateral Sclerosis Completed NCT01281189 Phase 3 Dexpramipexole;Placebo
39 Study of Dopamine and Serotonin Transporters in Patients With Amyotrophic Lateral Sclerosis and Controls. Analysis With 123I-FP-CIT (Datscan) and 123I-ADAM Brain SPECT Completed NCT01160263 Phase 3 SPECT : 123 I-FP-CIT (DATSCAN) and 123I-ADAM
40 Efficacy and Safety Study of MCI-186 for Treatment of the Patients With Amyotrophic Lateral Sclerosis (ALS) 2 Completed NCT01492686 Phase 3 MCI-186;Placebo;MCI-186 in open label phase
41 The Effect of Granulocyte Colony Stimulating Factor (GCSF) in the Treatment of Amyotrophic Lateral Sclerosis (ALS) Patients Referred to Tehran Imam Khomeini and Shariati Hospital Centers in 2013 Completed NCT01825551 Phase 2, Phase 3 Granulocyte Colony Stimulating Factor;Placebo
42 Effects of Oral Levosimendan (ODM-109) on Respiratory Function in Patients With ALS Completed NCT03505021 Phase 3 Levosimendan;Placebo for levosimendan
43 A Phase 3, Randomised, Placebo-Controlled Trial of Arimoclomol in Amyotropic Lateral Sclerosis Completed NCT03491462 Phase 3 Arimoclomol;Placebo oral capsule
44 A Double-Blind, Randomized, Placebo-Controlled, Multicenter Study to Assess the Safety and Efficacy and to Determine the Pharmacokinetics of Two Doses of AVP-923 (Dextromethorphan/Quinidine) in the Treatment of Pseudobulbar Affect (PBA) in Patients With Amyotrophic Lateral Sclerosis (ALS) and Multiple Sclerosis (MS) Completed NCT00573443 Phase 3 dextromethorphan hydrobromide 20 mg and quinidine sulfate 10 mg;dextromethorphan hydrobromide 30 mg and quinidine sulfate 10 mg;Placebo
45 A European, Randomised, Double-blind, Active Comparator Controlled, Cross-over, Efficacy and Safety Study of a New 10% Ready To-use Liquid Human Intravenous Immunoglobulin (I10E) Versus Kiovig® in Patients With Multifocal Motor Neuropathy Completed NCT01951924 Phase 3 Biological : I10E (Human normal Immunoglobulin for intravenous administration 100mg/mL);Biological: Kiovig® (Human normal Immunoglobulin for intravenous administration 100mg/mL)
46 A Phase 1-3 Study to Evaluate the Efficacy, Safety, Pharmacokinetics and Pharmacodynamics of Intrathecally Administered ION363 in Amyotrophic Lateral Sclerosis Patients With Fused in Sarcoma Mutations (FUS-ALS) Recruiting NCT04768972 Phase 3 ION363;Placebo
47 A Randomised, Double-blind, Single-centre Study on the Safety, Tolerability and Efficacy of Cannabis Based Medicine Extract (MediCabilis CBD Oil) in Slowing the Disease Progression in Amyotrophic Lateral Sclerosis or Motor Neurone Disease Patients Recruiting NCT03690791 Phase 3 MediCabilis CBD Oil;Placebo Oil
48 HEALEY ALS Platform Trial Recruiting NCT04297683 Phase 2, Phase 3 Zilucoplan;Verdiperstat;CNM-Au8;Pridopidine;SLS-005 Trehalose
49 Motor Neurone Disease - Systematic Multi-Arm Adaptive Randomised Trial Recruiting NCT04302870 Phase 2, Phase 3 Memantine Hydrochloride Oral Solution;Trazodone Hydrochloride oral solution;Placebo oral solution
50 A Double-blind, Randomized, Multicenter, Placebo-Controlled, Parallel, Phase III Clinical Trial to Evaluate the Efficacy and Safety of Lenzumestrocel(Neuronata-R® Inj.) in Patients With Amyotrophic Lateral Sclerosis Recruiting NCT04745299 Phase 3 Riluzole;Placebo Comparator

Search NIH Clinical Center for Lateral Sclerosis

Cochrane evidence based reviews: motor neuron disease

Genetic Tests for Lateral Sclerosis

Anatomical Context for Lateral Sclerosis

Organs/tissues related to Lateral Sclerosis:

MalaCards : Spinal Cord, Bone Marrow, Brain, Cortex, Skeletal Muscle, Bone, Tongue

Publications for Lateral Sclerosis

Articles related to Lateral Sclerosis:

(show top 50) (show all 27917)
# Title Authors PMID Year
1
Astrocytic protection of spinal motor neurons but not cortical neurons against loss of Als2/alsin function. 53 62
19304783 2009
2
A novel ALS2 splice-site mutation in a Cypriot juvenile-onset primary lateral sclerosis family. 53 62
19122027 2009
3
SOD1-associated ALS: a promising system for elucidating the origin of protein-misfolding disease. 53 62
19436494 2008
4
Novel homozygous ALS2 nonsense mutation (p.Gln715X) in sibs with infantile-onset ascending spastic paralysis: the first cases from northwestern Europe. 53 62
18523452 2008
5
A strategy for developing effective amyotropic lateral sclerosis pharmacotherapy: from clinical trials to novel pharmacotherapeutic strategies. 53 62
18627324 2008
6
Spastin mutations in sporadic adult-onset upper motor neuron syndromes. 53 62
16240363 2005
7
Mitochondrial proteomic analysis of a cell line model of familial amyotrophic lateral sclerosis. 53 62
15501831 2004
8
Folding of human superoxide dismutase: disulfide reduction prevents dimerization and produces marginally stable monomers. 53 62
15522970 2004
9
An intersubunit disulfide bond prevents in vitro aggregation of a superoxide dismutase-1 mutant linked to familial amytrophic lateral sclerosis. 53 62
15109247 2004
10
17q-linked frontotemporal dementia-amyotrophic lateral sclerosis without tau mutations with tau and alpha-synuclein inclusions. 53 62
15023818 2004
11
Relevance of oxidative injury in the pathogenesis of motor neuron diseases. 53 62
14753657 2003
12
Unstable mutants in the peripheral endosomal membrane component ALS2 cause early-onset motor neuron disease. 53 62
14668431 2003
13
The first nonsense mutation in alsin results in a homogeneous phenotype of infantile-onset ascending spastic paralysis with bulbar involvement in two siblings. 53 62
12919135 2003
14
ALS2, a novel guanine nucleotide exchange factor for the small GTPase Rab5, is implicated in endosomal dynamics. 53 62
12837691 2003
15
Infantile ascending hereditary spastic paralysis (IAHSP): clinical features in 11 families. 53 62
12601111 2003
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[Tauopathies--a new class of neurodegenerative diseases]. 53 62
11256158 2001
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Motor neuron-astrocyte interactions and levels of Cu,Zn superoxide dismutase in sporadic amyotrophic lateral sclerosis. 53 62
7895821 1995
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Cdk5 and aberrant cell cycle activation at the core of neurodegeneration. 62
36453392 2023
19
Neuro faces of beneficial T cells: essential in brain, impaired in aging and neurological diseases, and activated functionally by neurotransmitters and neuropeptides. 62
36453390 2023
20
Long-noncoding RNAs as epigenetic regulators in neurodegenerative diseases. 62
36453400 2023
21
Novel insights into the mechanism of reactive oxygen species-mediated neurodegeneration. 62
36204830 2023
22
Promising application of a new ulnar nerve compound muscle action potential measurement montage in amyotrophic lateral sclerosis: a prospective cross-sectional study. 62
36204862 2023
23
ALS Regional Variants (Brachial Amyotrophic Diplegia and Amyotrophic Leg Diplegia): Still A Diagnostic Challenge in Neurology. 62
36474455 2023
24
Neuroinflammation-Related Proteins NOD2 and Spp1 Are Abnormally Upregulated in Amyotrophic Lateral Sclerosis. 62
36460480 2023
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Long-term air pollution and risk of amyotrophic lateral sclerosis mortality in the Women's Health Initiative cohort. 62
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Using Intensity Modulated Radiation Therapy for the Treatment of Sialorrhea in Amyotrophic Lateral Sclerosis. 62
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Senescent-like Blood Lymphocytes and Disease Progression in Amyotrophic Lateral Sclerosis. 62
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Neuronal Puncta/Aggregate Formation by WT and Mutant UBQLN2. 62
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A Spectrophotometric Turbidity Assay to Study Liquid-Liquid Phase Separation of UBQLN2 In Vitro. 62
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IRF5 knockdown reverses TDP-related phenotypes partially by increasing TBK1 expression. 62
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Biophysical Studies of LLPS and Aggregation of TDP-43 LCD. 62
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Subcortical signal alteration of corticospinal tracts. A radiologic manifestation of ARIA: A case report. 62
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An Optimized Stress Granule Detection Method: Investigation of UBQLN2 Effect on Stress Granule Formation. 62
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Masitinib analogues with the N-methylpiperazine group replaced - A new hope for the development of anti-COVID-19 drugs. 62
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Interplay between activation of endogenous retroviruses and inflammation as common pathogenic mechanism in neurological and psychiatric disorders. 62
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Dysregulated expression and distribution of Kif5α in neurites of wobbler motor neurons. 62
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Neuroaxonal and cellular damage/protection by prostanoid receptor ligands, fatty acid derivatives and associated enzyme inhibitors. 62
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Role of Nrf2 in aging, Alzheimer's and other neurodegenerative diseases. 62
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Proteome-wide systems genetics identifies UFMylation as a regulator of skeletal muscle function. 62
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In FUS[1-359]-tg mice O,S-dibenzoyl thiamine reduces muscle atrophy, decreases glycogen synthase kinase 3 beta, and normalizes the metabolome. 62
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A systematic review of digital technology to evaluate motor function and disease progression in motor neuron disease. 62
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A systematic review on the risk of neurodegenerative diseases and neurocognitive disorders in professional and varsity athletes. 62
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The function of FUS in neurodevelopment revealed by the brain and spinal cord organoids. 62
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Targeting epigenetics as a promising therapeutic strategy for treatment of neurodegenerative diseases. 62
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Comprehensive evaluation of human-derived anti-poly-GA antibodies in cellular and animal models of C9orf72 disease. 62
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Unbiased metabolome screen links serum urate to risk of Alzheimer's disease. 62
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The efficacy of acoustic-based articulatory phenotyping for characterizing and classifying four divergent neurodegenerative diseases using sequential motion rates. 62
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In vivo genome editing using novel AAV-PHP variants rescues motor function deficits and extends survival in a SOD1-ALS mouse model. 62
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FUS Mutation Causes Disordered Lipid Metabolism in Skeletal Muscle Associated with ALS. 62
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Feasibility, Acceptability, and Efficacy of Mindfulness Training in People With Upper Motor Neuron Disorders: A Systematic Review. 62
35760105 2022

Variations for Lateral Sclerosis

Expression for Lateral Sclerosis

Search GEO for disease gene expression data for Lateral Sclerosis.

Pathways for Lateral Sclerosis

Pathways related to Lateral Sclerosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.48 SOD1 PRPH NEFH ALS2

GO Terms for Lateral Sclerosis

Cellular components related to Lateral Sclerosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 axon GO:0030424 9.65 SPG11 SETX PRPH NEFH C9orf72 ALS2
2 growth cone GO:0030426 9.28 SIGMAR1 SETX C9orf72 ANG ALS2

Biological processes related to Lateral Sclerosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 neurofilament cytoskeleton organization GO:0060052 8.92 SOD1 NEFH

Sources for Lateral Sclerosis

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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