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Degenerative Disease of Nervous System, Unspecified

MedGen UID:: 101195
•Concept ID:: C0524851
•: Disease or Syndrome

Synonyms:

Degenerative Condition, Neurologic; Degenerative Conditions, Neurologic; Degenerative Diseases, Nervous System; Degenerative Diseases, Neurologic; Degenerative Neurologic Disease; Degenerative Neurologic Diseases; Degenerative Neurologic Disorder; Degenerative Neurologic Disorders; Nervous System Degenerative Diseases; Neurodegenerative Disease; Neurodegenerative Diseases; Neurodegenerative Disorder; Neurodegenerative Disorders; Neurologic Degenerative Condition; Neurologic Degenerative Conditions; Neurologic Degenerative Disease; Neurologic Degenerative Diseases; Neurologic Disease, Degenerative; Neurologic Diseases, Degenerative; Neurologic Disorder, Degenerative; Neurologic Disorders, Degenerative

Definition

Hereditary and sporadic conditions which are characterized by progressive nervous system dysfunction. These disorders are often associated with atrophy of the affected central or peripheral nervous system structures. [from MeSH]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVDegenerative Disease of Nervous System, Unspecified

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Disorder by Site
      - Disorder of nervous system
        Degenerative Disease of Nervous System, Unspecified
        Chronic traumatic encephalopathy
        Heredodegenerative Disorders, Nervous System
        Alexander disease
        Alexander disease type I
        Alexander disease type II
        Batten-Turner congenital myopathy
        Congenital myotonia, autosomal dominant form
        Congenital myotonia, autosomal recessive form
        Chorea-acanthocytosis
        Cockayne syndrome
        Cockayne syndrome type 1
        Cockayne syndrome type 2
        Cockayne syndrome type 3
        COFS syndrome
        Familial amyloid polyneuropathy
        Gerstmann-Straussler-Scheinker syndrome
        Hereditary Central Nervous System Demyelinating Diseases
        Adrenoleukodystrophy
        Galactosylceramide beta-galactosidase deficiency
        Metachromatic leukodystrophy
        Pelizaeus-Merzbacher disease
        Spongy degeneration of central nervous system
        Hereditary motor and sensory neuropathy
        Alstrom syndrome
        Charcot-Marie-Tooth disease
        Giant axonal neuropathy
        Hereditary spastic paraplegia
        Phytanic acid storage disease
        Hereditary optic atrophy
        Autosomal dominant optic atrophy classic form
        Dominant hereditary optic atrophy
        Leber optic atrophy
        Wolfram syndrome
        Hereditary sensory and autonomic neuropathy
        Congenital insensitivity to pain with hyperhidrosis
        Familial dysautonomia
        X-linked hereditary sensory and autonomic neuropathy with hearing loss
        Huntington disease
        Juvenile Huntington disease
        Westphal disease
        Kennedy disease
        Lafora disease
        Epilepsy, progressive myoclonic, 2b
        Myoclonic epilepsy of Lafora 1
        Myotonic dystrophy
        Congenital myotonic dystrophy
        Myotonic cataract
        Myotonic dystrophy type 2
        Steinert myotonic dystrophy syndrome
        Neurofibromatosis
        Aggravated Neurofibromatosis
        Neurofibroma by anatomical location
        Neurofibromatosis, type 1
        Neurofibromatosis, type 2
        Neurofibromatosis, type IV, of Riccardi
        Plexiform neurofibroma
        Neuronal ceroid lipofuscinosis
        Adult neuronal ceroid lipofuscinosis
        Ceroid lipofuscinosis, neuronal, 4 (Kufs type)
        Ceroid lipofuscinosis, neuronal, 6A
        Congenital neuronal ceroid lipofuscinosis
        Infantile neuronal ceroid lipofuscinosis
        Juvenile neuronal ceroid lipofuscinosis
        Late-infantile neuronal ceroid lipofuscinosis
        Neuronal ceroid lipofuscinosis 1
        Neuronal ceroid lipofuscinosis 3
        Neuronal ceroid lipofuscinosis 5
        Neuronal ceroid lipofuscinosis 7
        Neuronal ceroid lipofuscinosis 9
        Neuronal ceroid lipofuscinosis 10
        Neuronal ceroid lipofuscinosis 8 northern epilepsy variant
        Neuronal Ceroid Lipofuscinosis Type 4B
        Parkinsonism due to ATP13A2 deficiency
        Pigmentary pallidal degeneration
        Atypical pantothenate kinase-associated neurodegeneration
        Classic pantothenate kinase-associated neurodegeneration
        Spinal Muscular Atrophy of Childhood
        spinocerebellar disease
        Early onset cerebellar ataxia with myoclonus
        Friedreich ataxia
        Olivopontocerebellar atrophy
        Spinocerebellar atrophy
        Torsion dystonia
        Acquired idiopathic torsion dystonia
        Familial idiopathic torsion dystonia
        Tourette syndrome
        Tuberous sclerosis syndrome
        Autosomal dominant polycystic kidney disease type 1 with tuberous sclerosis
        Tuberous sclerosis 1
        Tuberous sclerosis 2
        Unverricht-Lundborg syndrome
        Wilson disease
        X-linked intellectual disability
        Classical Lissencephalies and Subcortical Band Heterotopias
        Coffin-Lowry syndrome
        Danon disease
        Fragile X syndrome
        Lesch-Nyhan syndrome
        Menkes kinky-hair syndrome
        Mucopolysaccharidosis, MPS-II
        Pyruvate dehydrogenase complex deficiency
        Rett syndrome
        Motor neuron disease
        Amyotrophic lateral sclerosis
        Amyotrophic Lateral Sclerosis 14, With or Without Frontotemporal Dementia
        Amyotrophic lateral sclerosis type 1
        Amyotrophic lateral sclerosis type 3
        Amyotrophic lateral sclerosis type 4
        Amyotrophic lateral sclerosis type 6
        Amyotrophic lateral sclerosis type 7
        Amyotrophic lateral sclerosis type 8
        Amyotrophic lateral sclerosis type 9
        Amyotrophic lateral sclerosis type 10
        Amyotrophic lateral sclerosis type 11
        Amyotrophic lateral sclerosis type 21
        Amyotrophic lateral sclerosis type 23
        Juvenile amyotrophic lateral sclerosis
        Progressive muscular atrophy
        Primary lateral sclerosis
        Progressive bulbar palsy
        Brown-Vialetto-van Laere syndrome 1
        Progressive spinal muscular atrophy
        Spinal muscular atrophy
        Acute infantile spinal muscular atrophy
        Kugelberg-Welander disease
        Proximal spinal muscular atrophy
        Spinal muscular atrophy, segmental
        Spinal muscular atrophy, type II
        Spinal muscular atrophy, type IV
        Werdnig-Hoffmann disease
        Paraneoplastic neurologic syndrome
        Anti-NMDA receptor encephalitis
        Cancer-associated retinopathy
        Eaton-Lambert syndrome
        Limbic encephalitis
        Myasthenia gravis
        Adult-onset myasthenia gravis
        Juvenile myasthenia gravis
        Neonatal myasthenia gravis
        Transient neonatal myasthenia gravis
        Opsoclonus-myoclonus syndrome
        Paraneoplastic cerebellar degeneration
        Paraneoplastic isolated brainstem encephalitis
        Paraneoplastic polyneuropathy
        Paraneoplastic Subacute Sensory Neuronopathy
        Paraneoplastic sensory ganglionopathy
        Transverse myelitis
        Neuromyelitis optica
        Post poliomyelitis syndrome
        Prion disease
        Bovine Spongiform Encephalopathy
        Creutzfeldt-Jakob disease
        Acquired Creutzfeldt-Jakob disease
        Inherited Creutzfeldt-Jakob disease
        Fatal familial insomnia
        Kuru
        Scrapie
        Variant Creutzfeldt-Jakob disease
        Wasting Disease, Chronic
        Subacute combined degeneration of spinal cord
        Synucleinopathy
        Lewy body dementia
        Multiple system atrophy
        Multiple system atrophy, cerebellar type
        Multiple system atrophy, parkinsonian type
        Shy Drager Syndrome
        Striatonigral degeneration
        Parkinson disease
        Autosomal dominant Parkinson disease 1
        Autosomal dominant Parkinson disease 4
        Autosomal dominant Parkinson disease 8
        Autosomal recessive early-onset Parkinson disease 6
        Autosomal recessive early-onset Parkinson disease 7
        Autosomal recessive juvenile Parkinson disease 2
        Autosomal recessive Parkinson disease 14
        Juvenile paralysis agitans of Hunt
        Kufor-Rakeb syndrome
        Parkinson disease 6
        Parkinson disease 10
        Parkinson disease 11, autosomal dominant, susceptibility to
        Parkinson disease 12
        Parkinson disease 13, autosomal dominant, susceptibility to
        Parkinson disease 16
        Parkinson disease 17
        Parkinson disease 3, autosomal dominant
        Parkinson disease 5, autosomal dominant, susceptibility to
        Parkinson disease, late-onset
        Parkinson disease, mitochondrial
        Parkinsonian-pyramidal syndrome
        Tauopathy
        Alzheimer disease
        Alzheimer disease 2
        Alzheimer disease 3
        Alzheimer disease 4
        Alzheimer disease 6
        Alzheimer disease 7
        Alzheimer disease 8
        Alzheimer disease 9
        Alzheimer disease 10
        Alzheimer disease 11
        Alzheimer disease 12
        Alzheimer disease 13
        Alzheimer disease 14
        Alzheimer disease 16
        Alzheimer disease 17
        Alzheimer disease without neurofibrillary tangles
        Early-onset autosomal dominant Alzheimer disease
        Corticobasal degeneration disorder
        Diffuse Neurofibrillary Tangles with Calcification
        Progressive supranuclear palsy
        Atypical progressive supranuclear palsy syndrome
        Supranuclear palsy, progressive, 1
        Supranuclear palsy, progressive, 2
        Supranuclear palsy, progressive, 3
        TDP-43 proteinopathy
        Degenerations, Frontotemporal Lobar
        Frontotemporal dementia
        Primary progressive non fluent aphasia

Professional guidelines

PubMed

The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy.

Wenning GK, Stankovic I, Vignatelli L, Fanciulli A, Calandra-Buonaura G, Seppi K, Palma JA, Meissner WG, Krismer F, Berg D, Cortelli P, Freeman R, Halliday G, Höglinger G, Lang A, Ling H, Litvan I, Low P, Miki Y, Panicker J, Pellecchia MT, Quinn N, Sakakibara R, Stamelou M, Tolosa E, Tsuji S, Warner T, Poewe W, Kaufmann H
Mov Disord 2022 Jun;37(6):1131-1148. Epub 2022 Apr 21 doi: 10.1002/mds.29005. PMID: 35445419 Free PMC Article

Updated International Tuberous Sclerosis Complex Diagnostic Criteria and Surveillance and Management Recommendations.

Northrup H, Aronow ME, Bebin EM, Bissler J, Darling TN, de Vries PJ, Frost MD, Fuchs Z, Gosnell ES, Gupta N, Jansen AC, Jóźwiak S, Kingswood JC, Knilans TK, McCormack FX, Pounders A, Roberds SL, Rodriguez-Buritica DF, Roth J, Sampson JR, Sparagana S, Thiele EA, Weiner HL, Wheless JW, Towbin AJ, Krueger DA; International Tuberous Sclerosis Complex Consensus Group
Pediatr Neurol 2021 Oct;123:50-66. Epub 2021 Jul 24 doi: 10.1016/j.pediatrneurol.2021.07.011. PMID: 34399110

MDS clinical diagnostic criteria for Parkinson's disease.

Postuma RB, Berg D, Stern M, Poewe W, Olanow CW, Oertel W, Obeso J, Marek K, Litvan I, Lang AE, Halliday G, Goetz CG, Gasser T, Dubois B, Chan P, Bloem BR, Adler CH, Deuschl G
Mov Disord 2015 Oct;30(12):1591-601. doi: 10.1002/mds.26424. PMID: 26474316

See all (14612)

Recent clinical studies

Etiology

Aging and aging-related diseases: from molecular mechanisms to interventions and treatments.

Guo J, Huang X, Dou L, Yan M, Shen T, Tang W, Li J
Signal Transduct Target Ther 2022 Dec 16;7(1):391. doi: 10.1038/s41392-022-01251-0. PMID: 36522308 Free PMC Article

Comprehensive Review on Alzheimer's Disease: Causes and Treatment.

Breijyeh Z, Karaman R
Molecules 2020 Dec 8;25(24) doi: 10.3390/molecules25245789. PMID: 33302541 Free PMC Article

Ageing as a risk factor for neurodegenerative disease.

Hou Y, Dan X, Babbar M, Wei Y, Hasselbalch SG, Croteau DL, Bohr VA
Nat Rev Neurol 2019 Oct;15(10):565-581. Epub 2019 Sep 9 doi: 10.1038/s41582-019-0244-7. PMID: 31501588

Ageing, Cellular Senescence and Neurodegenerative Disease.

Kritsilis M, V Rizou S, Koutsoudaki PN, Evangelou K, Gorgoulis VG, Papadopoulos D
Int J Mol Sci 2018 Sep 27;19(10) doi: 10.3390/ijms19102937. PMID: 30261683 Free PMC Article

Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases.

Lin MT, Beal MF
Nature 2006 Oct 19;443(7113):787-95. doi: 10.1038/nature05292. PMID: 17051205

See all (103387)

Diagnosis

Epilepsy in neurodegenerative diseases.

Neri S, Mastroianni G, Gardella E, Aguglia U, Rubboli G
Epileptic Disord 2022 Apr 1;24(2):249-273. doi: 10.1684/epd.2021.1406. PMID: 35596580

Propagation of Protein Aggregation in Neurodegenerative Diseases.

Vaquer-Alicea J, Diamond MI
Annu Rev Biochem 2019 Jun 20;88:785-810. Epub 2019 Mar 27 doi: 10.1146/annurev-biochem-061516-045049. PMID: 30917002

Olfactory Dysfunction in Neurodegenerative Diseases.

Marin C, Vilas D, Langdon C, Alobid I, López-Chacón M, Haehner A, Hummel T, Mullol J
Curr Allergy Asthma Rep 2018 Jun 15;18(8):42. doi: 10.1007/s11882-018-0796-4. PMID: 29904888

Clinical Neurology and Epidemiology of the Major Neurodegenerative Diseases.

Erkkinen MG, Kim MO, Geschwind MD
Cold Spring Harb Perspect Biol 2018 Apr 2;10(4) doi: 10.1101/cshperspect.a033118. PMID: 28716886 Free PMC Article

Young-onset dementia.

Kuruppu DK, Matthews BR
Semin Neurol 2013 Sep;33(4):365-85. Epub 2013 Nov 14 doi: 10.1055/s-0033-1359320. PMID: 24234358 Free PMC Article

See all (99602)

Therapy

Role of neuroinflammation in neurodegeneration development.

Zhang W, Xiao D, Mao Q, Xia H
Signal Transduct Target Ther 2023 Jul 12;8(1):267. doi: 10.1038/s41392-023-01486-5. PMID: 37433768 Free PMC Article

Activators and Inhibitors of NRF2: A Review of Their Potential for Clinical Development.

Robledinos-Antón N, Fernández-Ginés R, Manda G, Cuadrado A
Oxid Med Cell Longev 2019;2019:9372182. Epub 2019 Jul 14 doi: 10.1155/2019/9372182. PMID: 31396308 Free PMC Article

Tryptophan metabolism as a common therapeutic target in cancer, neurodegeneration and beyond.

Platten M, Nollen EAA, Röhrig UF, Fallarino F, Opitz CA
Nat Rev Drug Discov 2019 May;18(5):379-401. doi: 10.1038/s41573-019-0016-5. PMID: 30760888

Overview on the Effects of N-Acetylcysteine in Neurodegenerative Diseases.

Tardiolo G, Bramanti P, Mazzon E
Molecules 2018 Dec 13;23(12) doi: 10.3390/molecules23123305. PMID: 30551603 Free PMC Article

Coffee, Caffeine, and Health Outcomes: An Umbrella Review.

Grosso G, Godos J, Galvano F, Giovannucci EL
Annu Rev Nutr 2017 Aug 21;37:131-156. doi: 10.1146/annurev-nutr-071816-064941. PMID: 28826374

See all (66021)

Prognosis

The epidemiology of Parkinson's disease.

Ben-Shlomo Y, Darweesh S, Llibre-Guerra J, Marras C, San Luciano M, Tanner C
Lancet 2024 Jan 20;403(10423):283-292. doi: 10.1016/S0140-6736(23)01419-8. PMID: 38245248

Healthy Eating Patterns and Risk of Total and Cause-Specific Mortality.

Shan Z, Wang F, Li Y, Baden MY, Bhupathiraju SN, Wang DD, Sun Q, Rexrode KM, Rimm EB, Qi L, Tabung FK, Giovannucci EL, Willett WC, Manson JE, Qi Q, Hu FB
JAMA Intern Med 2023 Feb 1;183(2):142-153. doi: 10.1001/jamainternmed.2022.6117. PMID: 36622660 Free PMC Article

Ageing, Cellular Senescence and Neurodegenerative Disease.

Kritsilis M, V Rizou S, Koutsoudaki PN, Evangelou K, Gorgoulis VG, Papadopoulos D
Int J Mol Sci 2018 Sep 27;19(10) doi: 10.3390/ijms19102937. PMID: 30261683 Free PMC Article

Amyotrophic lateral sclerosis.

van Es MA, Hardiman O, Chio A, Al-Chalabi A, Pasterkamp RJ, Veldink JH, van den Berg LH
Lancet 2017 Nov 4;390(10107):2084-2098. Epub 2017 May 25 doi: 10.1016/S0140-6736(17)31287-4. PMID: 28552366

Epidemiology of Parkinson's disease.

Elbaz A, Carcaillon L, Kab S, Moisan F
Rev Neurol (Paris) 2016 Jan;172(1):14-26. Epub 2015 Dec 21 doi: 10.1016/j.neurol.2015.09.012. PMID: 26718594

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Clinical prediction guides

Molecular mechanisms of cell death in neurological diseases.

Moujalled D, Strasser A, Liddell JR
Cell Death Differ 2021 Jul;28(7):2029-2044. Epub 2021 Jun 7 doi: 10.1038/s41418-021-00814-y. PMID: 34099897 Free PMC Article

Microglia-mediated neuroinflammation in neurodegenerative diseases.

Subhramanyam CS, Wang C, Hu Q, Dheen ST
Semin Cell Dev Biol 2019 Oct;94:112-120. Epub 2019 May 11 doi: 10.1016/j.semcdb.2019.05.004. PMID: 31077796

Olfactory Dysfunction in Neurodegenerative Diseases.

Marin C, Vilas D, Langdon C, Alobid I, López-Chacón M, Haehner A, Hummel T, Mullol J
Curr Allergy Asthma Rep 2018 Jun 15;18(8):42. doi: 10.1007/s11882-018-0796-4. PMID: 29904888

Inflammation in CNS neurodegenerative diseases.

Stephenson J, Nutma E, van der Valk P, Amor S
Immunology 2018 Jun;154(2):204-219. Epub 2018 Apr 17 doi: 10.1111/imm.12922. PMID: 29513402 Free PMC Article

Pathology of Neurodegenerative Diseases.

Dugger BN, Dickson DW
Cold Spring Harb Perspect Biol 2017 Jul 5;9(7) doi: 10.1101/cshperspect.a028035. PMID: 28062563 Free PMC Article

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Recent systematic reviews

The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy.

Mediterranean diet adherence, gut microbiota, and Alzheimer's or Parkinson's disease risk: A systematic review.

Solch RJ, Aigbogun JO, Voyiadjis AG, Talkington GM, Darensbourg RM, O'Connell S, Pickett KM, Perez SR, Maraganore DM
J Neurol Sci 2022 Mar 15;434:120166. Epub 2022 Jan 26 doi: 10.1016/j.jns.2022.120166. PMID: 35144237

Consensus on the treatment of dysphagia in Parkinson's disease.

Schindler A, Pizzorni N, Cereda E, Cosentino G, Avenali M, Montomoli C, Abbruzzese G, Antonini A, Barbiera F, Benazzo M, Benarroch E, Bertino G, Clavè P, Cortelli P, Eleopra R, Ferrari C, Hamdy S, Huckabee ML, Lopiano L, Marchese-Ragona R, Masiero S, Michou E, Occhini A, Pacchetti C, Pfeiffer RF, Restivo DA, Rondanelli M, Ruoppolo G, Sandrini G, Schapira A, Stocchi F, Tolosa E, Valentino F, Zamboni M, Zangaglia R, Zappia M, Tassorelli C, Alfonsi E
J Neurol Sci 2021 Nov 15;430:120008. Epub 2021 Sep 27 doi: 10.1016/j.jns.2021.120008. PMID: 34624796

The Amyloid-β Pathway in Alzheimer's Disease.

Hampel H, Hardy J, Blennow K, Chen C, Perry G, Kim SH, Villemagne VL, Aisen P, Vendruscolo M, Iwatsubo T, Masters CL, Cho M, Lannfelt L, Cummings JL, Vergallo A
Mol Psychiatry 2021 Oct;26(10):5481-5503. Epub 2021 Aug 30 doi: 10.1038/s41380-021-01249-0. PMID: 34456336 Free PMC Article

Effect of nutrition on neurodegenerative diseases. A systematic review.

Bianchi VE, Herrera PF, Laura R
Nutr Neurosci 2021 Oct;24(10):810-834. Epub 2019 Nov 4 doi: 10.1080/1028415X.2019.1681088. PMID: 31684843

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