CBGD
MCID: CRT033
MIFTS: 48

Corticobasal Degeneration (CBGD)

Categories: Mental diseases, Muscle diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Corticobasal Degeneration

MalaCards integrated aliases for Corticobasal Degeneration:

Name: Corticobasal Degeneration 20 53 29 70
Corticobasal Syndrome 20 58 6
Cortical-Basal Ganglionic Degeneration 20
Cortico-Basal Ganglionic Degeneration 20
Cbgd 20

Classifications:

Orphanet: 58  
Rare neurological diseases


Summaries for Corticobasal Degeneration

NINDS : 53 Corticobasal degeneration is a progressive neurological disorder characterized by nerve cell loss and atrophy (shrinkage) of multiple areas of the brain including the cerebral cortex and the basal ganglia. Corticobasal degeneration progresses gradually. Initial symptoms, which typically begin at or around age 60, may first appear on one side of the body (unilateral), but eventually affect both sides as the disease progresses. Symptoms are similar to those found in Parkinson disease, such as poor coordination, akinesia (an absence of movements), rigidity (a resistance to imposed movement),disequilibrium (impaired balance); and limb dystonia (abnormal muscle postures). Other symptoms such as cognitive and visual-spatial impairments, apraxia (loss of the ability to make familiar, purposeful movements), hesitant and halting speech, myoclonus (muscular jerks), and dysphagia (difficulty swallowing) may also occur. An individual with corticobasal degeneration eventually becomes unable to walk.

MalaCards based summary : Corticobasal Degeneration, also known as corticobasal syndrome, is related to frontotemporal lobar degeneration with tdp43 inclusions, grn-related and pick disease of brain. An important gene associated with Corticobasal Degeneration is TBK1 (TANK Binding Kinase 1), and among its related pathways/superpathways are Human cytomegalovirus infection and Brain-Derived Neurotrophic Factor (BDNF) signaling pathway. The drugs Lithium carbonate and Fasudil have been mentioned in the context of this disorder. Affiliated tissues include cortex, brain and eye, and related phenotypes are parkinsonism and progressive extrapyramidal muscular rigidity

GARD : 20 Corticobasal degeneration is characterized by the break down (degeneration) of parts of the brain, including the cerebral cortex and basal ganglia. The cerebral cortex is responsible for most of the brain's processing of information, and the basal ganglia are deep brain structures that help start and control movement. Signs and symptoms of corticobasal degeneration include poor coordination, loss of movement, rigidity, poor balance, unnatural posturing of the muscles, intellectual (cognitive) impairment, speech impairment, muscular jerks, and difficulty swallowing. These symptoms develop and worsen over time. Currently the cause of corticobasal degeneration is not known. Treatment depends on the symptoms in each person. People with corticobasal degeneration usually do not survive beyond an average of 7 years after symptoms begin. Aspiration pneumonia or other complications are usually the cause of death.

Wikipedia : 73 Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and... more...

Related Diseases for Corticobasal Degeneration

Diseases related to Corticobasal Degeneration via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 180)
# Related Disease Score Top Affiliating Genes
1 frontotemporal lobar degeneration with tdp43 inclusions, grn-related 31.8 RPS27A MAPT
2 pick disease of brain 31.8 RPS27A MAPT
3 semantic dementia 31.7 RPS27A MAPT
4 leukoencephalopathy, hereditary diffuse, with spheroids 31.6 RPS27A MAPT
5 supranuclear palsy, progressive, 1 31.4 RPS27A MAPT GBA
6 frontotemporal dementia 31.4 TBK1 RPS27A MAPT
7 aphasia 31.2 TBK1 MAPT
8 parkinsonism 31.0 MAPT GBA
9 multiple system atrophy 1 30.7 RPS27A MAPT
10 progressive non-fluent aphasia 30.4 TBK1 MAPT
11 creutzfeldt-jakob disease 30.2 RPS27A MAPT
12 movement disease 30.2 MAPT GBA
13 dementia 30.0 TBK1 MAPT GBA
14 olivopontocerebellar atrophy 29.9 RPS27A MAPT
15 motor neuron disease 29.8 TBK1 RPS27A MAPT
16 speech disorder 29.8 MAPT GBA
17 rem sleep behavior disorder 29.6 RPS27A MAPT GBA
18 parkinson disease, late-onset 29.6 RPS27A MAPT GBA
19 essential tremor 29.5 MAPT GBA
20 dementia, lewy body 29.5 RPS27A MAPT GBA
21 progressive supranuclear palsy-corticobasal syndrome 11.2
22 grn-related frontotemporal lobar degeneration 11.0
23 apraxia 11.0
24 myoclonus 10.8
25 dystonia 10.8
26 tremor 10.6
27 alzheimer disease 10.6
28 ideomotor apraxia 10.5
29 cerebral atrophy 10.4
30 argyria 10.4
31 dysphagia 10.4
32 spasticity 10.3
33 kearns-sayre syndrome 10.3
34 agnosia 10.3
35 mutism 10.3
36 amyotrophic lateral sclerosis 1 10.3
37 ataxia and polyneuropathy, adult-onset 10.3
38 focal dystonia 10.3
39 agraphia 10.3
40 lateral sclerosis 10.3
41 dysgraphia 10.3
42 rare neurodegenerative disease 10.3
43 gait apraxia 10.2
44 respiratory failure 10.1
45 akinetic mutism 10.1
46 alien hand syndrome 10.1
47 posterior cortical atrophy 10.1
48 hemiparkinsonism-hemiatrophy syndrome 10.1
49 gerstmann syndrome 10.1
50 hand skill, relative 10.1

Graphical network of the top 20 diseases related to Corticobasal Degeneration:



Diseases related to Corticobasal Degeneration

Symptoms & Phenotypes for Corticobasal Degeneration

Human phenotypes related to Corticobasal Degeneration:

58 31 (show all 23)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 parkinsonism 58 31 hallmark (90%) Very frequent (99-80%) HP:0001300
2 progressive extrapyramidal muscular rigidity 58 31 hallmark (90%) Very frequent (99-80%) HP:0007158
3 gait disturbance 58 31 frequent (33%) Frequent (79-30%) HP:0001288
4 tremor 58 31 frequent (33%) Frequent (79-30%) HP:0001337
5 sensory impairment 58 31 frequent (33%) Frequent (79-30%) HP:0003474
6 dementia 58 31 frequent (33%) Frequent (79-30%) HP:0000726
7 limb dystonia 58 31 frequent (33%) Frequent (79-30%) HP:0002451
8 akinesia 58 31 frequent (33%) Frequent (79-30%) HP:0002304
9 postural instability 58 31 frequent (33%) Frequent (79-30%) HP:0002172
10 bradykinesia 58 31 frequent (33%) Frequent (79-30%) HP:0002067
11 limb apraxia 58 31 frequent (33%) Frequent (79-30%) HP:0030217
12 oromotor apraxia 58 31 frequent (33%) Frequent (79-30%) HP:0007301
13 limb myoclonus 58 31 frequent (33%) Frequent (79-30%) HP:0045084
14 frontal release signs 58 31 frequent (33%) Frequent (79-30%) HP:0000743
15 dysphasia 58 31 occasional (7.5%) Occasional (29-5%) HP:0002357
16 memory impairment 58 31 occasional (7.5%) Occasional (29-5%) HP:0002354
17 aphasia 58 31 occasional (7.5%) Occasional (29-5%) HP:0002381
18 speech apraxia 58 31 occasional (7.5%) Occasional (29-5%) HP:0011098
19 behavioral abnormality 58 31 very rare (1%) Very rare (<4-1%) HP:0000708
20 myoclonus 58 Very frequent (99-80%)
21 abnormality of movement 58 Very rare (<4-1%)
22 involuntary movements 58 Frequent (79-30%)
23 dystonia 58 Occasional (29-5%)

GenomeRNAi Phenotypes related to Corticobasal Degeneration according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased Salmonella enterica Typhimurium effector injection GR00133-A-4 8.62 CFL1 TBK1

MGI Mouse Phenotypes related to Corticobasal Degeneration:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 integument MP:0010771 8.92 CFL1 GBA MAPT TBK1

Drugs & Therapeutics for Corticobasal Degeneration

Drugs for Corticobasal Degeneration (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 16)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Lithium carbonate Approved Phase 1, Phase 2 554-13-2
2
Fasudil Investigational Phase 2 103745-39-7
3 Psychotropic Drugs Phase 1, Phase 2
4 Antidepressive Agents Phase 1, Phase 2
5 Vasodilator Agents Phase 2
6 Hormones Phase 2
7 Protein Kinase Inhibitors Phase 2
8 calcium channel blockers Phase 2
9 Calcium, Dietary Phase 2
10
Calcium Nutraceutical Phase 2 7440-70-2 271
11 Tubulin Modulators Phase 1
12 taxane Phase 1
13 Anesthetics
14 Pharmaceutical Solutions
15 ABBV-8E12
16 Nootropic Agents

Interventional clinical trials:

(show all 12)
# Name Status NCT ID Phase Drugs
1 A Pilot Trial of Lithium in Subjects With Progressive Supranuclear Palsy or Corticobasal Degeneration Completed NCT00703677 Phase 1, Phase 2 Lithium
2 Direct Current Brain Polarization for Apraxia in Corticobasal Syndrome Completed NCT00273897 Phase 2
3 A Phase 2a Open-Label Preliminary Safety, Tolerability, and Biomarker Study of Oral Fasudil in Patients With the 4-Repeat Tauopathies of Progressive Supranuclear Palsy-Richardson Syndrome or Corticobasal Syndrome. Recruiting NCT04734379 Phase 2 Fasudil
4 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 Flortaucipir F18
5 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 Completed NCT02133846 Phase 1 TPI 287 2 mg/m2;TPI-287 20 mg/m2;Placebo;TPI-287 6.3 mg/m2
6 Multimodal Assessment For Predicting Specific Pathological Substrate in Frontotemporal Lobar Degeneration Unknown status NCT02964637
7 Noninvasive Cortical Stimulation (rTMS) for Motor and Non-Motor Features of Progressive Supranuclear Palsy (PSP) and Corticobasal Degeneration (CBD) Completed NCT01174771
8 Assessing Skin Biomarkers for Preclinical Diagnosis of PD and Non-PD Parkinsonism Recruiting NCT04518059
9 Positron Emission Tomography (PET) Imaging of Tau Pathology in Neurodegenerative Disease Recruiting NCT03143374
10 The Four-Repeat Tauopathy Neuroimaging Initiative Recruiting NCT02966145
11 Observational Longitudinal Study of Magnetic Resonance Imaging, Specimen Biomarkers, and Clinical Progression in Progressive Supranuclear Palsy and Corticobasal Degeneration Recruiting NCT01804452
12 Expanded Access to ABBV-8E12 No longer available NCT03744546 ABBV-8E12

Search NIH Clinical Center for Corticobasal Degeneration

Genetic Tests for Corticobasal Degeneration

Genetic tests related to Corticobasal Degeneration:

# Genetic test Affiliating Genes
1 Corticobasal Degeneration 29

Anatomical Context for Corticobasal Degeneration

MalaCards organs/tissues related to Corticobasal Degeneration:

40
Cortex, Brain, Eye, Spinal Cord, Temporal Lobe, Cerebellum, Bone

Publications for Corticobasal Degeneration

Articles related to Corticobasal Degeneration:

(show top 50) (show all 2011)
# Title Authors PMID Year
1
Molecular Imaging of Extrapyramidal Movement Disorders With Dementia: The 4R Tauopathies. 61
33431202 2021
2
Brain [F-18]FDG PET for Clinical Dementia Workup: Differential Diagnosis of Alzheimer's Disease and Other Types of Dementing Disorders. 61
33546814 2021
3
Assessment of the role of non-coding RNAs in the pathophysiology of Parkinson's disease. 61
33508286 2021
4
Dentatorubrothalamic tract reduction using fixel-based analysis in corticobasal syndrome. 61
32989557 2021
5
The Role of Neuropsychiatric Symptoms in Research Diagnostic Criteria for Neurodegenerative Diseases. 61
32819825 2021
6
A novel diagnostic marker for progressive supranuclear palsy targeting atrophy of the subthalamic nucleus. 61
33714084 2021
7
AlphaScreen Identifies MSUT2 Inhibitors for Tauopathy-Targeting Therapeutic Discovery. 61
32981422 2021
8
The AD tau core spontaneously self-assembles and recruits full-length tau to filaments. 61
33730588 2021
9
Spinal Cord Stimulation Therapy for Gait Dysfunction in Two Corticobasal Syndrome Patients. 61
32646537 2021
10
Cerebrospinal Fluid Proteins Altered in Corticobasal Degeneration. 61
33660876 2021
11
FDG-PET Patterns Predict Amyloid Deposition and Clinical Profile in Corticobasal Syndrome. 61
33206389 2021
12
A Clinicopathologic Study of Movement Disorders in Frontotemporal Lobar Degeneration. 61
33155746 2021
13
Crossed cerebellar diaschisis on 18F-FDG PET: Frequency across neurodegenerative syndromes and association with 11C-PIB and 18F-Flortaucipir. 61
33691512 2021
14
Machine learning-based decision tree classifier for the diagnosis of progressive supranuclear palsy and corticobasal degeneration. 61
33763863 2021
15
Language impairment in progressive supranuclear palsy and corticobasal syndrome. 61
31321513 2021
16
Strain-specific clearance of seed-dependent tau aggregation by lithium-induced autophagy. 61
33517128 2021
17
Human tauopathy-derived tau strains determine the substrates recruited for templated amplification. 61
33693528 2021
18
Mechanisms of Neurodegeneration in Various Forms of Parkinsonism-Similarities and Differences. 61
33809527 2021
19
Association of PSP phenotypes with survival: A brain-bank study. 61
33581485 2021
20
First symptom guides diagnosis and prognosis in neurodegenerative diseases-a retrospective study of autopsy proven cases. 61
33662165 2021
21
Deep Learning-Based Image Classification in Differentiating Tufted Astrocytes, Astrocytic Plaques, and Neuritic Plaques. 61
33570124 2021
22
Effective Valproic Acid Treatment in a Patient With Delusional Parasitosis Due to Corticobasal Syndrome and Alzheimer Disease. 61
33734170 2021
23
Diagnostic Accuracy of Affective Social Tasks in the Clinical Classification Between the Behavioral Variant of Frontotemporal Dementia and Other Neurodegenerative Disease. 61
33682708 2021
24
Genetic pleiotropy and the shared pathological features of corticobasal degeneration and progressive supranuclear palsy: a case report and a review of the literature. 61
33754963 2021
25
In vitro amplification of pathogenic tau conserves disease-specific bioactive characteristics. 61
33385254 2021
26
Neuroligin-1 in brain and CSF of neurodegenerative disorders: investigation for synaptic biomarkers. 61
33522967 2021
27
18F-AV-1451 positron emission tomography in neuropathological substrates of corticobasal syndrome. 61
33578418 2021
28
Predicting loss of independence and mortality in frontotemporal lobar degeneration syndromes. 61
33563798 2021
29
A systematic review of atypical Alzheimer's disease including behavioural and psychological symptoms. 61
33594793 2021
30
An autopsy case of corticobasal syndrome due to asymmetric degeneration of the motor cortex and substantia nigra with TDP-43 proteinopathy, associated with Alzheimer's disease pathology. 61
33537992 2021
31
Conventional Magnetic Resonance Imaging in the Diagnosis of Parkinsonian Disorders: A Meta-Analysis. 61
33553491 2021
32
[Alien hand syndrome is a rare symptom of stroke]. 61
33570025 2021
33
Mills' Syndrome (Ascending Spastic Hemiparesis) Progressing to Corticobasal Syndrome. 61
33472710 2021
34
Corticobasal Syndrome with Supplementary Motor Area Aphasia. 61
33431730 2021
35
Unravelling Genetic Factors Underlying Corticobasal Syndrome: A Systematic Review. 61
33467748 2021
36
Cerebrospinal fluid biomarker profiling in corticobasal degeneration: Application of the AT(N) and other classification systems. 61
33246219 2021
37
Chinese nutraceuticals and physical activity; their role in neurodegenerative tauopathies. 61
33407732 2021
38
Tau Protein and Frontotemporal Dementias. 61
33433876 2021
39
Psychosis in neurodegenerative disease: differential patterns of hallucination and delusion symptoms. 61
33501939 2021
40
In vivo coupling of dendritic complexity with presynaptic density in primary tauopathies. 61
33631470 2021
41
Neutrophil-to-lymphocyte ratio (NLR) at boundaries of Progressive Supranuclear Palsy Syndrome (PSPS) and Corticobasal Syndrome (CBS). 61
33315235 2021
42
Progressive Supranuclear Palsy and Corticobasal Degeneration. 61
33433875 2021
43
The Role of Frontal Assessment Battery and Frontal Lobe Single-Photon Emission Computed Tomography in the Differential Diagnosis of Progressive Supranuclear Palsy Variants and Corticobasal Syndrome-A Pilot Study. 61
33613435 2021
44
Autosomal Dominant Frontotemporal Lobar Degeneration in a Filipino Family with Progranulin Mutation. 61
33486486 2021
45
A Review of Neuroimaging in Rare Neurodegenerative Diseases. 61
33508841 2021
46
Shape Profile of Corpus Callosum As a Signature to Phenotype Different Dementia. 61
33531781 2021
47
Atypical parkinsonian syndromes in a North African tertiary referral center. 61
33179436 2021
48
A case report of metastatic renal cell carcinoma causing corticobasal syndrome. 61
33433315 2021
49
Cerebrospinal Fluid α-Synuclein Species in Cognitive and Movements Disorders. 61
33477387 2021
50
High-Contrast In Vivo Imaging of Tau Pathologies in Alzheimer's and Non-Alzheimer's Disease Tauopathies. 61
33125873 2021

Variations for Corticobasal Degeneration

ClinVar genetic disease variations for Corticobasal Degeneration:

6
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 TBK1 NM_013254.4(TBK1):c.2107G>T (p.Glu703Ter) SNV Likely pathogenic 619188 rs1565825132 GRCh37: 12:64891788-64891788
GRCh38: 12:64498008-64498008
2 ADAM10 NM_001110.4(ADAM10):c.359T>C (p.Ile120Thr) SNV Uncertain significance 870548 GRCh37: 15:58971448-58971448
GRCh38: 15:58679249-58679249
3 TTC3 NM_001320703.2(TTC3):c.5677G>A (p.Val1893Met) SNV Uncertain significance 870549 GRCh37: 21:38568315-38568315
GRCh38: 21:37196014-37196014

Expression for Corticobasal Degeneration

Search GEO for disease gene expression data for Corticobasal Degeneration.

Pathways for Corticobasal Degeneration

Pathways related to Corticobasal Degeneration according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.93 TBK1 RPS27A CFL1
2 11.31 MAPT CFL1
3 10.46 TBK1 RPS27A

GO Terms for Corticobasal Degeneration

Biological processes related to Corticobasal Degeneration according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 neuron projection development GO:0031175 9.26 MAPT GBA
2 response to virus GO:0009615 9.16 TBK1 CFL1
3 I-kappaB kinase/NF-kappaB signaling GO:0007249 8.96 TBK1 RPS27A
4 TRIF-dependent toll-like receptor signaling pathway GO:0035666 8.62 TBK1 RPS27A

Sources for Corticobasal Degeneration

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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