Neurodegenerative diseases ( Greek νέυρο-, neuro- , “nerves-” and Latin dēgenerāre , “degenerate”) are a group of mostly slowly progressive, hereditary or acquired diseases of the nervous system .
Common to these diseases is progressive death of nerve cells ( neurodegeneration ), leading to various neurological symptoms - primarily, dementia and impaired movement. Diseases can occur at different ages, occur diffusely or generalized, a specific type of change is histologically determined. [one]
List of Neurodegenerative Diseases
Neurodegenerative diseases include (according to Mackenzie et al .: [2] )
Taupathy :
- Alzheimer's disease
- Progressive supranuclear paresis of the gaze
- Corticobasal degeneration
- Silver Grain Disease
- Front-Temporal Dementia and Parkinsonism 17 of the Chromosome (FTDP-17)
- Peak Disease
The pathomorphological basis of this group of NDZ is the excessive phosphorylation of a protein called tauprotein and its fixation in brain cells in the form of peculiar pathological structures. This process disrupts the vital activity of the neuron. [3]
Synucleopathies :
- Parkinson's disease
- Dementia with Levi bodies
- Multisystem atrophy
This group is characterized by the accumulation of α-synuclein in the brain cells, which is a complex of 35 amino acids. Various structures are formed from this protein, leading to cell death. The first symptoms are usually motor disorders, later dementia develops. In some patients, along with the accumulation of α-synuclein, deposition of β-amyloid, typical of taupathias, is noted. [four]
TDP-43 proteinopathy
- Degeneration of the frontotemporal lobes with TDP-43 (FTLD-TDP)
Fusopathies
- Degeneration of the frontotemporal lobes with FUS (FTLD-FUS)
- Neuronal intermediate filament inclusion disease (NIFID)
- Basophilic inclusion body disease (BIBD)
Trinucleotide diseases :
- Huntington Chorea
- Spinal Bulbar Muscular Atrophy, Kennedy Type
- Ataxia Friedreich
- Spinocerebellar ataxia
- Dendatororubo-pallidoluysial atrophy (DRPLA)
Hereditary diseases, a characteristic property of which is an increasing number of repeats of the amino acid chains of trinucleotides. Achieving a certain critical number of trinucleotide repeats determines the probability of getting sick equal to 100%. This is called a dynamic mutation. In subsequent generations, the number of trinucleotide repeats is steadily increasing, which leads to a worsening of the course of the disease. This is called genetic anticipation.
Prion diseases :
- Creutzfeldt's disease - Jacob
- Gerstmann's syndrome - Straussler - Scheinker
- Fatal Family Insomnia
- Kuru
Motor neuron disease
- Amyotrophic lateral sclerosis
- Primary lateral sclerosis
- Spinal muscular atrophy
Neuroaxonal dystrophy
- Infantile neuroaxonal dystrophy
- Neurodegeneration with deposition of iron in the brain
Unclassified diseases
- Degeneration of the frontotemporal lobes with the ubiquitin protease system (FTLD-UPS)
- Family encephalopathy with neuroserpine inclusions
Notes
- ↑ Przedborski S., Vila M., Jackson-Lewis V. Neurodegeneration . Per. from English N. D. Firsova (2018)
- ↑ IR Mackenzie: Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. In: Acta neuropathologica. Band 119, Nummer 1, Januar 2010, S. 1-4, ISSN 1432-0533 . DOI : 10.1007 / s00401-009-0612-2 . PMID 19924424 . PMC 2799633 .
- ↑ V. Borodulin. What is taupathy .
- ↑ Litvinenko I.V., Krasakov I.V., Bisaga G.N., Skulyabin D.I., Poltavsky I.D. The modern concept of the pathogenesis of neurodegenerative diseases and the treatment strategy // "Journal of Neurology and Psychiatry", No. 6, 2017; Vol. 2.