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Neurodegenerative Disorder

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 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

CROGVNeurodegenerative Disorder

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Disorder by Site
      - Disorder of nervous system
        Neurodegenerative Disorder
        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
        Myotonic dystrophy
        Congenital myotonic dystrophy
        Myotonic cataract
        Myotonic dystrophy type 2
        Steinert myotonic dystrophy syndrome
        Neurofibromatosis
        Aggravated Neurofibromatosis
        Neurofibromatosis, type 1
        Neurofibromatosis, type 2
        Neurofibromatosis, type IV, of Riccardi
        Occasional neurofibromas
        Paraspinal neurofibroma
        Plexiform neurofibroma
        Spinal neurofibroma
        Subcutaneous 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
        Idiopathic familial dystonia
        Idiopathic non-familial 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
        Nervous System Paraneoplastic Syndromes
        Anti-NMDA receptor encephalitis
        Limbic encephalitis
        Myasthenia gravis
        Adult-onset myasthenia gravis
        Eaton-Lambert syndrome
        Juvenile myasthenia gravis
        Neonatal myasthenia gravis
        Transient neonatal myasthenia gravis
        Opsoclonus-myoclonus syndrome
        Paraneoplastic cerebellar degeneration
        Paraneoplastic polyneuropathy
        Paraneoplastic Subacute Sensory Neuronopathy
        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
        Rida
        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
        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

New Avenues for the Treatment of Huntington's Disease.

Kim A, Lalonde K, Truesdell A, Gomes Welter P, Brocardo PS, Rosenstock TR, Gil-Mohapel J
Int J Mol Sci 2021 Aug 4;22(16) doi: 10.3390/ijms22168363. PMID: 34445070 Free PMC Article

Treatment Options for Motor and Non-Motor Symptoms of Parkinson's Disease.

Church FC
Biomolecules 2021 Apr 20;11(4) doi: 10.3390/biom11040612. PMID: 33924103 Free PMC Article

Parkinson's disease: Mechanisms, translational models and management strategies.

Raza C, Anjum R, Shakeel NUA
Life Sci 2019 Jun 1;226:77-90. Epub 2019 Apr 10 doi: 10.1016/j.lfs.2019.03.057. PMID: 30980848

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Recent clinical studies

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Apolipoprotein E and Alzheimer's Disease: Findings, Hypotheses, and Potential Mechanisms.

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Oxidative Stress, Synaptic Dysfunction, and Alzheimer's Disease.

Tönnies E, Trushina E
J Alzheimers Dis 2017;57(4):1105-1121. doi: 10.3233/JAD-161088. PMID: 28059794 Free PMC Article

Alzheimer's disease and gut microbiota.

Hu X, Wang T, Jin F
Sci China Life Sci 2016 Oct;59(10):1006-1023. Epub 2016 Aug 26 doi: 10.1007/s11427-016-5083-9. PMID: 27566465

Parkinson's disease: a review.

Beitz JM
Front Biosci (Schol Ed) 2014 Jan 1;6(1):65-74. doi: 10.2741/s415. PMID: 24389262

Epidemiology of Parkinson's disease.

de Lau LM, Breteler MM
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Diagnosis

Treatment Options for Motor and Non-Motor Symptoms of Parkinson's Disease.

Church FC
Biomolecules 2021 Apr 20;11(4) doi: 10.3390/biom11040612. PMID: 33924103 Free PMC Article

Parkinson's disease psychosis: presentation, diagnosis and management.

Schneider RB, Iourinets J, Richard IH
Neurodegener Dis Manag 2017 Dec;7(6):365-376. Epub 2017 Nov 21 doi: 10.2217/nmt-2017-0028. PMID: 29160144

Parkinson's disease: a review.

Beitz JM
Front Biosci (Schol Ed) 2014 Jan 1;6(1):65-74. doi: 10.2741/s415. PMID: 24389262

Huntington's disease.

Walker FO
Lancet 2007 Jan 20;369(9557):218-28. doi: 10.1016/S0140-6736(07)60111-1. PMID: 17240289

Epidemiology of Parkinson's disease.

de Lau LM, Breteler MM
Lancet Neurol 2006 Jun;5(6):525-35. doi: 10.1016/S1474-4422(06)70471-9. PMID: 16713924

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Therapy

New Avenues for the Treatment of Huntington's Disease.

Kim A, Lalonde K, Truesdell A, Gomes Welter P, Brocardo PS, Rosenstock TR, Gil-Mohapel J
Int J Mol Sci 2021 Aug 4;22(16) doi: 10.3390/ijms22168363. PMID: 34445070 Free PMC Article

Treatment Options for Motor and Non-Motor Symptoms of Parkinson's Disease.

Church FC
Biomolecules 2021 Apr 20;11(4) doi: 10.3390/biom11040612. PMID: 33924103 Free PMC Article

Alzheimer's disease and its treatment by different approaches: A review.

Srivastava S, Ahmad R, Khare SK
Eur J Med Chem 2021 Apr 15;216:113320. Epub 2021 Feb 23 doi: 10.1016/j.ejmech.2021.113320. PMID: 33652356

Ferroptosis and its potential role in the physiopathology of Parkinson's Disease.

Mahoney-Sánchez L, Bouchaoui H, Ayton S, Devos D, Duce JA, Devedjian JC
Prog Neurobiol 2021 Jan;196:101890. Epub 2020 Jul 26 doi: 10.1016/j.pneurobio.2020.101890. PMID: 32726602

Disease-modifying therapies in amyotrophic lateral sclerosis.

Chiò A, Mazzini L, Mora G
Neuropharmacology 2020 May 1;167:107986. Epub 2020 Feb 3 doi: 10.1016/j.neuropharm.2020.107986. PMID: 32062193

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Prognosis

Parkinson's disease: Mechanisms, translational models and management strategies.

Raza C, Anjum R, Shakeel NUA
Life Sci 2019 Jun 1;226:77-90. Epub 2019 Apr 10 doi: 10.1016/j.lfs.2019.03.057. PMID: 30980848

Current trends in etiology, prognosis and therapeutic aspects of Parkinson's disease: a review.

Jagadeesan AJ, Murugesan R, Vimala Devi S, Meera M, Madhumala G, Vishwanathan Padmaja M, Ramesh A, Banerjee A, Sushmitha S, Khokhlov AN, Marotta F, Pathak S
Acta Biomed 2017 Oct 23;88(3):249-262. doi: 10.23750/abm.v88i3.6063. PMID: 29083328 Free PMC Article

Mouse Models of Alzheimer's Disease.

Esquerda-Canals G, Montoliu-Gaya L, Güell-Bosch J, Villegas S
J Alzheimers Dis 2017;57(4):1171-1183. doi: 10.3233/JAD-170045. PMID: 28304309

Parkinson's disease: a review.

Beitz JM
Front Biosci (Schol Ed) 2014 Jan 1;6(1):65-74. doi: 10.2741/s415. PMID: 24389262

Epidemiology of Parkinson's disease.

de Lau LM, Breteler MM
Lancet Neurol 2006 Jun;5(6):525-35. doi: 10.1016/S1474-4422(06)70471-9. PMID: 16713924

See all (1304)

Clinical prediction guides

Safety and Efficacy of Omaveloxolone in Friedreich Ataxia (MOXIe Study).

Lynch DR, Chin MP, Delatycki MB, Subramony SH, Corti M, Hoyle JC, Boesch S, Nachbauer W, Mariotti C, Mathews KD, Giunti P, Wilmot G, Zesiewicz T, Perlman S, Goldsberry A, O'Grady M, Meyer CJ
Ann Neurol 2021 Feb;89(2):212-225. Epub 2020 Nov 5 doi: 10.1002/ana.25934. PMID: 33068037 Free PMC Article

Genetic Markers of Alzheimer's Disease.

Nikolac Perkovic M, Pivac N
Adv Exp Med Biol 2019;1192:27-52. doi: 10.1007/978-981-32-9721-0_3. PMID: 31705489

Mouse Models of Alzheimer's Disease.

Esquerda-Canals G, Montoliu-Gaya L, Güell-Bosch J, Villegas S
J Alzheimers Dis 2017;57(4):1171-1183. doi: 10.3233/JAD-170045. PMID: 28304309

Parkinson disease subtypes.

Thenganatt MA, Jankovic J
JAMA Neurol 2014 Apr;71(4):499-504. doi: 10.1001/jamaneurol.2013.6233. PMID: 24514863

Parkinson's disease: a review.

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Recent systematic reviews

The Prevalence of Dementia: A Systematic Review and Meta-Analysis.

Cao Q, Tan CC, Xu W, Hu H, Cao XP, Dong Q, Tan L, Yu JT
J Alzheimers Dis 2020;73(3):1157-1166. doi: 10.3233/JAD-191092. PMID: 31884487

Virtual reality for rehabilitation in Parkinson's disease.

Dockx K, Bekkers EM, Van den Bergh V, Ginis P, Rochester L, Hausdorff JM, Mirelman A, Nieuwboer A
Cochrane Database Syst Rev 2016 Dec 21;12(12):CD010760. doi: 10.1002/14651858.CD010760.pub2. PMID: 28000926 Free PMC Article

The Incidence of Parkinson's Disease: A Systematic Review and Meta-Analysis.

Hirsch L, Jette N, Frolkis A, Steeves T, Pringsheim T
Neuroepidemiology 2016;46(4):292-300. Epub 2016 Apr 23 doi: 10.1159/000445751. PMID: 27105081

The prevalence of Parkinson's disease: a systematic review and meta-analysis.

Pringsheim T, Jette N, Frolkis A, Steeves TD
Mov Disord 2014 Nov;29(13):1583-90. Epub 2014 Jun 28 doi: 10.1002/mds.25945. PMID: 24976103

Parkinson disease subtypes.

Thenganatt MA, Jankovic J
JAMA Neurol 2014 Apr;71(4):499-504. doi: 10.1001/jamaneurol.2013.6233. PMID: 24514863

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Neurodegenerative Disorder

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Practice guidelines

Related information

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