MCID: CLS047
MIFTS: 30

Classic Progressive Supranuclear Palsy Syndrome

Categories: Eye diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Classic Progressive Supranuclear Palsy Syndrome

MalaCards integrated aliases for Classic Progressive Supranuclear Palsy Syndrome:

Name: Classic Progressive Supranuclear Palsy Syndrome 59
Steele-Richardson-Olszewski Disease 59
Progressive Supranuclear Palsy 72
Classic Psp Syndrome 59
Richardson Syndrome 59

Characteristics:

Orphanet epidemiological data:

59
classic progressive supranuclear palsy syndrome
Inheritance: Not applicable; Age of onset: Adult; Age of death: elderly;

Classifications:



External Ids:

ICD10 via Orphanet 34 G23.1
UMLS via Orphanet 73 C0038868
Orphanet 59 ORPHA240071
UMLS 72 C0038868

Summaries for Classic Progressive Supranuclear Palsy Syndrome

MalaCards based summary : Classic Progressive Supranuclear Palsy Syndrome, also known as steele-richardson-olszewski disease, is related to feigenbaum bergeron richardson syndrome and atherosclerosis, premature, with deafness, nephropathy, diabetes mellitus, photomyoclonus, and degenerative neurologic disease, and has symptoms including seizures, tremor and photophobia. An important gene associated with Classic Progressive Supranuclear Palsy Syndrome is MAPT (Microtubule Associated Protein Tau). The drugs Promethazine and tannic acid have been mentioned in the context of this disorder. Affiliated tissues include brain, eye and testes, and related phenotypes are falls and postural instability

Related Diseases for Classic Progressive Supranuclear Palsy Syndrome

Graphical network of the top 20 diseases related to Classic Progressive Supranuclear Palsy Syndrome:



Diseases related to Classic Progressive Supranuclear Palsy Syndrome

Symptoms & Phenotypes for Classic Progressive Supranuclear Palsy Syndrome

Human phenotypes related to Classic Progressive Supranuclear Palsy Syndrome:

59 32 (show all 28)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 falls 59 32 hallmark (90%) Very frequent (99-80%) HP:0002527
2 postural instability 59 32 hallmark (90%) Very frequent (99-80%) HP:0002172
3 dysarthria 59 32 frequent (33%) Frequent (79-30%) HP:0001260
4 photophobia 59 32 frequent (33%) Frequent (79-30%) HP:0000613
5 gait imbalance 59 32 frequent (33%) Frequent (79-30%) HP:0002141
6 decreased lacrimation 59 32 frequent (33%) Frequent (79-30%) HP:0000633
7 blepharospasm 59 32 frequent (33%) Frequent (79-30%) HP:0000643
8 blurred vision 59 32 frequent (33%) Frequent (79-30%) HP:0000622
9 bradykinesia 59 32 frequent (33%) Frequent (79-30%) HP:0002067
10 impulsivity 59 32 frequent (33%) Frequent (79-30%) HP:0100710
11 slow saccadic eye movements 59 32 frequent (33%) Frequent (79-30%) HP:0000514
12 vertical supranuclear gaze palsy 59 32 frequent (33%) Frequent (79-30%) HP:0000511
13 conjunctival hyperemia 59 32 frequent (33%) Frequent (79-30%) HP:0030953
14 progressive extrapyramidal muscular rigidity 59 32 frequent (33%) Frequent (79-30%) HP:0007158
15 axial dystonia 59 32 frequent (33%) Frequent (79-30%) HP:0002530
16 slowed slurred speech 59 32 frequent (33%) Frequent (79-30%) HP:0007164
17 tremor 59 32 occasional (7.5%) Occasional (29-5%) HP:0001337
18 abnormal pyramidal sign 59 32 occasional (7.5%) Occasional (29-5%) HP:0007256
19 neuromuscular dysphagia 59 32 occasional (7.5%) Occasional (29-5%) HP:0002068
20 social and occupational deterioration 59 32 occasional (7.5%) Occasional (29-5%) HP:0007086
21 parkinsonism with favorable response to dopaminergic medication 59 32 occasional (7.5%) Occasional (29-5%) HP:0002548
22 akinesia 59 32 occasional (7.5%) Occasional (29-5%) HP:0002304
23 axial muscle stiffness 59 32 occasional (7.5%) Occasional (29-5%) HP:0006921
24 abnormality of saccadic eye movements 59 Frequent (79-30%)
25 dystonia 59 Occasional (29-5%)
26 mental deterioration 59 Very frequent (99-80%)
27 parkinsonism 59 Frequent (79-30%)
28 supranuclear gaze palsy 59 Frequent (79-30%)

UMLS symptoms related to Classic Progressive Supranuclear Palsy Syndrome:


seizures, tremor, photophobia, back pain, ophthalmoplegia, pain, headache, bradykinesia, syncope, chronic pain, sciatica, vertigo/dizziness, sleeplessness, forgetful, muscle rigidity, poor mobility

Drugs & Therapeutics for Classic Progressive Supranuclear Palsy Syndrome

Drugs for Classic Progressive Supranuclear Palsy Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 119)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Promethazine Approved, Investigational Phase 4 60-87-7 4927
2
tannic acid Approved Phase 4 1401-55-4
3
Suvorexant Approved, Investigational Phase 4 1030377-33-3
4
Diphenhydramine Approved, Investigational Phase 4 58-73-1, 147-24-0 3100
5
Zolpidem Approved Phase 4 82626-48-0 5732
6
Benzocaine Approved, Investigational Phase 4 94-09-7, 1994-09-7 2337
7
Iodine Approved, Investigational Phase 4 7553-56-2 807
8 Neurotransmitter Agents Phase 4
9 Central Nervous System Depressants Phase 4
10 GABA Agents Phase 4
11 GABA-A Receptor Agonists Phase 4
12 Orexin Receptor Antagonists Phase 4
13 GABA Agonists Phase 4
14 Hypnotics and Sedatives Phase 4
15 Pharmaceutical Solutions Phase 4
16 Calamus Phase 4
17 cadexomer iodine Phase 4
18
Minocycline Approved, Investigational Phase 3 10118-90-8 5281021
19
Rivastigmine Approved, Investigational Phase 3 123441-03-2 77991
20
Pimavanserin Approved, Investigational Phase 3 706779-91-1 16058810
21
Rasagiline Approved Phase 3 136236-51-6 3052776
22
Riluzole Approved, Investigational Phase 3 1744-22-5 5070
23
Coenzyme Q10 Approved, Investigational, Nutraceutical Phase 2, Phase 3 303-98-0 5281915
24 Ubiquinone Phase 2, Phase 3
25 Vitamins Phase 2, Phase 3
26 Nutrients Phase 2, Phase 3
27 Micronutrients Phase 2, Phase 3
28 Trace Elements Phase 2, Phase 3
29 Anti-Bacterial Agents Phase 3
30 Anti-Infective Agents Phase 3
31 Tranquilizing Agents Phase 3
32 Psychotropic Drugs Phase 3
33 Cholinesterase Inhibitors Phase 3
34 Cholinergic Agents Phase 3
35 Serotonin Antagonists Phase 3
36 Serotonin 5-HT2 Receptor Antagonists Phase 3
37 Antiparkinson Agents Phase 3
38 Serotonin Agents Phase 3
39 Antipsychotic Agents Phase 3
40 Anticonvulsants Phase 3
41 Neuroprotective Agents Phase 3
42 Monoamine Oxidase Inhibitors Phase 3
43 Protective Agents Phase 3
44 Excitatory Amino Acid Antagonists Phase 3
45 Excitatory Amino Acids Phase 3
46
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
47
Donepezil Approved Phase 2 120014-06-4 3152
48
Valproic acid Approved, Investigational Phase 2 99-66-1 3121
49
Lithium carbonate Approved Phase 1, Phase 2 554-13-2
50
Dopamine Approved Phase 2 51-61-6, 62-31-7 681

Interventional clinical trials:

(show top 50) (show all 93)
# Name Status NCT ID Phase Drugs
1 Treatment of Disturbed Sleep in Progressive Supranuclear Palsy (PSP) Recruiting NCT04014387 Phase 4 Suvorexant;Zolpidem;Placebo oral capsule
2 Longitudinal Multi-Modality Imaging in Progressive Apraxia of Speech Recruiting NCT01818661 Phase 4 AV-1451
3 DaTSCAN Imaging in Aging and Neurodegenerative Disease Enrolling by invitation NCT01453127 Phase 4 I-123 Ioflupane solution injection prior to SPECT scan (DaTscan)
4 A Phase 2/3, Randomized, Double-Blind, Placebo-Controlled, Study to Evaluate the Safety and Efficacy of Davunetide for the Treatment of Progressive Supranuclear Palsy Completed NCT01110720 Phase 2, Phase 3 Davunetide;Placebo
5 Effects of Coenzyme Q10 in PSP and CBD, A Randomized, Placebo-Controlled, Double Blind Cross Over Pilot Study Completed NCT00532571 Phase 2, Phase 3 CoQ10
6 Double-Blind, Randomised, Two-Armed Study for the Evaluation of Efficacy and Safety of Minocycline for Treatment Completed NCT00146809 Phase 3 Minocyline
7 RIVA-PSP: Efficacy of Rivastigmine on Motor, Cognitive and Behavioural Impairment in Progressive Supranuclear Palsy: A Randomised Double Blind Placebo-controlled Clinical Trial Recruiting NCT02839642 Phase 3 Rivastigmine;Placebo
8 A Double-blind, Placebo-controlled, Relapse Prevention Study of Pimavanserin for the Treatment of Hallucinations and Delusions Associated With Dementia-related Psychosis Recruiting NCT03325556 Phase 3 Placebo;Pimavanserin 34 mg;Pimavanserin 20 mg
9 A Randomized, Double-Blind, Placebo-Controlled, Parallel Group Study to Assess the Efficacy, Safety and Tolerability of Rasagiline in Subjects With Progressive Supranuclear Palsy (Phase III) Terminated NCT01187888 Phase 3 Rasagiline;Sugar pill
10 Phase 3 Study of Riluzole in Multiple System Atrophy (MSA) and Progressive Supranuclear Palsy (PSP) (Parkinson's Plus Syndromes) Terminated NCT00211224 Phase 3 Riluzole
11 Study of the Distractibility Syndrome in Patients With Progressive Supranuclear Palsy Unknown status NCT00139373 Phase 2 donepezil
12 Autologous Mesenchymal Stem Cell Therapy in Progressive Supranuclear Palsy: a Randomized, Double-blind, Controlled Clinical Trial Unknown status NCT01824121 Phase 1, Phase 2
13 Rivastigmine (Exelon®) for Treatment of Dementia in Patient With Progressive Supranuclear Paresis Open Label Phase 2 Study Unknown status NCT00522015 Phase 2 rivastigmine
14 Novel Neuroimage Study in Tauopathies With Parkinsonism Unknown status NCT03386669 Phase 2 F-18
15 Randomized Placebo-controlled Trial of Valproic Acid in Patients With Progressive Supranuclear Palsy Completed NCT00385710 Phase 2 valproic acid;Placebo
16 A Pilot Trial of Lithium in Subjects With Progressive Supranuclear Palsy or Corticobasal Degeneration Completed NCT00703677 Phase 1, Phase 2 Lithium
17 Mono-Center, Prospective, Double-Blind, Placebo-Controlled, Randomized Clinical Phase IIa Trial to Assess the Safety, Tolerability, and Immediate Biological Effects of Coenzyme Q10 - nanoQuinon® in Progressive Supranuclear Palsy Completed NCT00328874 Phase 2 Coenzyme Q10
18 Dose-Escalation Trial of Continuously Infused Recombinant-Methionyl Human Glial Cell Line-Derived Neurotrophic Factor for the Treatment of PSP Completed NCT00005903 Phase 2 GDNF & Synchro Med Infusion System
19 The Differential Diagnosis of Parkinson's Disease and Parkinsonism by Positron-emission Tomography With Vesicular Monoamine Transporter Ligand (18F-DTBZ) Completed NCT01824056 Phase 2 18F-FDG
20 An Open-label Trial of Alpha-lipoic Acid/L-acetyl Carnitine for Progressive Supranuclear Palsy (PSP): Effect Upon Oxidative Damage and Mitochondrial Biomarkers Completed NCT01537549 Phase 1, Phase 2 alpha-lipoic acid and L-acetyl carnitine
21 Double-blind, Parallel Group, Placebo-controlled Trial of the Efficacy and Tolerability of Memantine (20 mg) in Frontotemporal Dementia (FTD) Patients Completed NCT00200538 Phase 2 memantine
22 The Effect of Adrenergic Blocker Therapy on Cardiac and Striatal Transporter Uptake in Pre-Motor and Symptomatic Parkinson's Disease Recruiting NCT03775096 Phase 2 Carvedilol
23 A Randomized, Double-Blind, Placebo-Controlled Multiple Dose Study to Assess Efficacy, Safety, Tolerability, and Pharmacokinetics of ABBV-8E12 in Progressive Supranuclear Palsy Active, not recruiting NCT02985879 Phase 2 placebo;ABBV-8E12
24 An Extension Study of ABBV-8E12 in Progressive Supranuclear Palsy (PSP) Active, not recruiting NCT03391765 Phase 2 ABBV-8E12
25 A Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of Intravenously Administered BIIB092 in Participants With Progressive Supranuclear Palsy Active, not recruiting NCT03068468 Phase 2 BIIB092;Placebo
26 A Multi-center, Randomized, Double-blind, Placebo-controlled, Parallel Group Study to Assess Tolerability, Safety, Pharmacokinetics and Effect of AZP2006 on Cerebrospinal Fluid Biomarkers in 36 Patients With Progressive Supranuclear Palsy Not yet recruiting NCT04008355 Phase 2 AZP2006 oral solution;Placebo oral solution
27 18F-AV-1451 Injection for Brain Imaging of Tau in Subjects With Progressive Supranuclear Palsy (PSP), Subjects With Corticobasal Degeneration (CBD) and Healthy Volunteers" Completed NCT02167594 Phase 1 18F-AV-1451;florbetapir F18
28 A Double-Blind, Placebo Controlled, Single Ascending Dose Study to Assess the Safety, Tolerability, and Pharmacokinetics of C2N-8E12 in Subjects With Progressive Supranuclear Palsy Completed NCT02494024 Phase 1 Single dose C2N-8E12;Single dose placebo
29 A Randomized, Double-Blind, Placebo-Controlled, Multiple Ascending Dose Study of Intravenously Administered BMS-986168 in Patients With Progressive Supranuclear Palsy Completed NCT02460094 Phase 1 BIIB092;Placebo
30 A 12 Week Randomized, Double Blind, Placebo-Controlled Pilot Study of Davunetide (NAP, AL-108) in Predicted Tauopathies Completed NCT01056965 Phase 1 davunetide (AL-108, NAP);Placebo nasal spray
31 Evaluation of [18F]MNI-777 PET as a Marker of Tau Pathology in Subjects With Clinically Diagnosed Tauopathies in Comparison to Healthy Subjects Completed NCT02103894 Phase 1 [18F]T807 ([18F]MNI-777)
32 Pilot Study: Open Label Treatment With tDCS for Parkinson's and Related Disorders for Improvement of Speech, Gait and Mood Completed NCT02104401 Phase 1
33 Phase 1 Test-retest Evaluation of [18F]MNI-958 PET as an Imaging Marker for Tau Protein in the Brain of Patients With Alzheimer's Disease and Probable PSP as Compared to Healthy Volunteers Recruiting NCT03545789 Phase 1 [18F]MNI-958
34 Alzheimer's PET Imaging in Racially/Ethnically Diverse Adults Recruiting NCT03706261 Phase 1 18F-MK-6240;18F-Florbetaben
35 Extension Study of ABBV-8E12 in Patients With Progressive Supranuclear Palsy (PSP) Who Completed Study C2N-8E12-WW-104 Active, not recruiting NCT03413319 Phase 1 ABBV-8E12
36 A Multicenter, Open-Label, Long-Term Treatment Study of Intravenously Administered BIIB092 in Patients With Progressive Supranuclear Palsy Who Participated in Study CN002003 Active, not recruiting NCT02658916 Phase 1 BIIB092
37 A 6 Month, Open-Label, Pilot Futility Clinical Trial of Monthly Young Healthy Male Donor Plasma Transfusions for Progressive Supranuclear Palsy Active, not recruiting NCT02460731 Phase 1
38 A 6 Month, Open-Label, Pilot Futility Clinical Trial of Oral Salsalate for Progressive Supranuclear Palsy Active, not recruiting NCT02422485 Phase 1 Salsalate
39 A Phase 1, Randomized, Double-Blind, Placebo-Controlled, Sequential Cohort, Dose-Ranging Study of the Safety, Tolerability, Pharmacokinetics, Pharmacodynamics, and Preliminary Efficacy of TPI 287 in Patients With Primary Four Repeat Tauopathies: Corticobasal Syndrome or Progressive Supranuclear Palsy Active, not recruiting NCT02133846 Phase 1 TPI 287 2 mg/m2;TPI-287 20 mg/m2;Placebo;TPI-287 6.3 mg/m2
40 Foot Mechanical Stimulation for Treatment of Gait and Gait Related Disorders in Parkinson's Disease Unknown status NCT01815281
41 Postural Instability in Progressive Supranuclear Palsy: Why do Patients With PSP Fall? Unknown status NCT01563276
42 Validation of DaTscan for Detection of Parkinsonian Disease and Related Disorders Using Neuropathologically-confirmed Parkinson Disease From Human Brain Tissue Unknown status NCT02138682 l-123 Ioflupane
43 Observational Study Assessing the Diagnostic Contribution of 3-Tesla MRI, CSF Analysis and a Second Opinion in a Specialized Movement Disorder Centre, in Differentiating Between Parkinson's Disease and Atypical Parkinsonism Unknown status NCT01249768
44 Blood Alpha-Synuclein, Gene Expression, and Smell Testing as Diagnostic and Prognostic Biomarkers in Parkinson's Disease Unknown status NCT00653783
45 Innovative Biomarkers in Alzheimer's Disease and Frontotemporal Dementia (FTD): Preventative and Personalized Unknown status NCT01403519
46 High Field MR Imaging (7T and 3T) of the Brainstem, the Deep Nuclei and Their Connections in the Parkinsonian Syndromes. Applications to Prognosis, Pathophysiology and Improvement of Therapeutic Strategies Unknown status NCT01085253
47 Effects of Coenzyme Q10 in Progressive Supranuclear Palsy (PSP): A Multicenter, Randomized, Placebo-controlled, Double Blind Study Completed NCT00382824
48 A Double-Blind, Placebo-Controlled, Randomized, Parallel-Group Study Evaluating the Safety, Tolerability, and Efficacy of Two Different Oral Doses of NP031112, a GSK-3 Inhibitor, Versus Placebo in the Treatment of Patients With Mild-to-Moderate Progressive Supranuclear Palsy Completed NCT01049399 tideglusib;tideglusib;placebo
49 The Role of the Neuropsychological Evaluation for Early Diagnosis of Progressive Supranuclear Palsy Completed NCT03478124
50 Phase 0 Evaluation of [18F]MNI-958 as a Potential PET Radioligand for Imaging Tau Protein in the Brain of Patients With Probable Alzheimer's Disease or Progressive Supranuclear Palsy Compared With Healthy Volunteers Completed NCT03058965 Early Phase 1 [18F]MNI-958;[18F]Florbetapir;DaTscan

Search NIH Clinical Center for Classic Progressive Supranuclear Palsy Syndrome

Genetic Tests for Classic Progressive Supranuclear Palsy Syndrome

Anatomical Context for Classic Progressive Supranuclear Palsy Syndrome

MalaCards organs/tissues related to Classic Progressive Supranuclear Palsy Syndrome:

41
Brain, Eye, Testes, Colon

Publications for Classic Progressive Supranuclear Palsy Syndrome

Articles related to Classic Progressive Supranuclear Palsy Syndrome:

(show all 24)
# Title Authors PMID Year
1
Familial aggregation of parkinsonism in progressive supranuclear palsy. 71
19458322 2009
2
A new mutation of the tau gene, G303V, in early-onset familial progressive supranuclear palsy. 71
16157753 2005
3
An English kindred with a novel recessive tauopathy and respiratory failure. 71
14595660 2003
4
Missense and splice site mutations in tau associated with FTDP-17: multiple pathogenic mechanisms. 71
11402146 2001
5
Frequency of tau gene mutations in familial and sporadic cases of non-Alzheimer dementia. 71
11255441 2001
6
MAPT-Related Disorders 71
20301678 2000
7
Untangling tau-related dementia. 71
10767321 2000
8
Pathogenic implications of mutations in the tau gene in pallido-ponto-nigral degeneration and related neurodegenerative disorders linked to chromosome 17. 71
9789048 1998
9
Tau is a candidate gene for chromosome 17 frontotemporal dementia. 71
9629852 1998
10
Frontotemporal lobar degeneration with ubiquitin-only-immunoreactive neuronal changes: broadening the clinical picture to include progressive supranuclear palsy. 38
15459024 2004
11
Accuracy of clinical diagnosis of progressive supranuclear palsy. 38
14978673 2004
12
Progressive supranuclear palsy: where are we now? 38
12849397 2002
13
Progressive supranuclear palsy (Steele-Richardson-Olszewski disease). 38
10621897 1999
14
[Cortical lesions in progressive supranuclear palsy (Steele-Richardson-Olszewski disease)]. 38
10093844 1999
15
A panencephalopathic type of Creutzfeldt-Jakob disease with selective lesions of the thalamic nuclei in 2 Swiss patients. 38
8793245 1996
16
The clinical and pathological spectrum of Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy): a reappraisal. 38
7600092 1995
17
Some specific clinical features differentiate multiple system atrophy (striatonigral variety) from Parkinson's disease. 38
7872884 1995
18
Neuropathology of Parkinson's disease and related syndromes. 38
1584179 1992
19
[Progressive supranuclear paralysis (Steele Richardson-Olszewski disease). After twenty-one years]. 38
2148381 1990
20
[A case of Steel-Richardson-Olszewski syndrome]. 38
1720223 1990
21
[Atypical onset of Steele-Richardson-Olszewski disease]. 38
3368689 1988
22
[The neuropathology of Parkinson syndrome]. 38
2843979 1988
23
[2 cases of isolated dystonia of the upper limb as an early sign of Steele-Richardson-Olszewski disease]. 38
3602792 1987
24
[Steele-Richardson-Olszewski disease without ophthalmoplegia. 6 clinico-anatomic cases]. 38
6612148 1983

Variations for Classic Progressive Supranuclear Palsy Syndrome

Expression for Classic Progressive Supranuclear Palsy Syndrome

Search GEO for disease gene expression data for Classic Progressive Supranuclear Palsy Syndrome.

Pathways for Classic Progressive Supranuclear Palsy Syndrome

GO Terms for Classic Progressive Supranuclear Palsy Syndrome

Sources for Classic Progressive Supranuclear Palsy Syndrome

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