Neurodegenerative Disease
| Classification according to ICD-10 | |
|---|---|
| G31.9 | Degenerative disease of the nervous system, unspecified |
| ICD-10 online (WHO version 2019) | |
As neurodegenerative diseases ( ancient Greek νέυρο- neuro- , German , nerve [s] - ' and Latin dēgenerāre degenerate' or 'degenerate) refers to a variety of diseases caused by the progressive loss of nerve cells of the central nervous system are indicated.
More common neurodegenerative diseases include Alzheimer's disease , Parkinson's disease, and Huntington 's disease .
causes
While some of these diseases are sporadic, others have a strong hereditary component. Despite great research efforts in the last few decades, the causes of most neurodegenerative diseases have not yet been clarified.
The diseases can occur at different ages, are diffuse or generalized and cause characteristic damage patterns in the nervous tissue.
Similarities, Symptoms and Therapy
At the cellular level, mechanisms can be identified that contribute to cell damage in most neurodegenerative diseases. Which includes:
- Disorders of protein homeostasis : misfolding and aggregation of proteins (e.g. beta-amyloid and tau protein in Alzheimer's disease, alpha-synuclein in Parkinson's disease, huntingtin in Huntington 's disease ), disorders in the breakdown of misfolded / aggregated proteins.
- Mutations and dysfunction of heat shock proteins and chaperones
- Excessive occurrence of oxygen radicals (increased oxidative stress)
- Disorders of mitochondrial function
- Disturbances in intracellular transport between the cell body and synapses
- Fragmentation of the Golgi apparatus
- Neurotrophin dysfunction
- local inflammatory reactions ( neuroinflammation )
These disease mechanisms are mutually reinforcing and mutually dependent. Oxygen radicals can lead to changes in individual proteins, which in turn favors their misfolding and aggregation. The accumulation of these proteins then possibly leads to cell death, which in turn can lead to inflammatory processes.
Often times, neurodegeneration begins in distinct areas of the brain and spreads over the course of the disease. Parkinson's disease begins with the loss of dopamine-producing nerve cells in the midbrain , while the first damage in Alzheimer's disease occurs in the area of the hippocampus (medial temporal lobe).
The progressive loss of nerve cells leads to various neurological and psychiatric symptoms. These include disorders of memory, language and orientation, motor skills, mood and the sleep-wake rhythm . Depending on the type of underlying disease and the main localization of the neurodegeneration, different symptoms can be in the foreground. Patients with frontotemporal dementia show a change in personality and social behavior at an early stage, while the ability to remember and orientate is initially not restricted.
At the moment, there are no therapies available for most neurodegenerative diseases that would eliminate the cause of the neurodegenerative diseases. For some diseases, there are only therapies that alleviate the symptoms (e.g. L-Dopa in Parkinson's disease) or delay the progression of the disease (e.g. cholinesterase inhibitors in sporadic Alzheimer's disease).
Classification
The classification of neurodegenerative diseases according to Mackenzie et al. essentially takes place descriptively according to the types of misfolded / aggregated proteins:
Tauopathies
- Alzheimer's disease (AD)
- Progressive supranuclear palsy (PSP)
- Corticobasal Degeneration (CBD)
- Silver Grain Disease (AGD)
- Frontotemporal dementia and parkonsonism of chromosome 17 (FTDP-17)
- Pick's disease
Synucleinopathies
- Parkinson's disease (PD)
- Lewy body dementia (LBD)
- Multiple System Atrophy (MSA)
TDP-43 proteinopathy
- Frontotemporal Flap Degeneration with TDP-43 (FTLD-TDP)
FUSopathies
- Frontotemporal Flap Degeneration with FUS (FTLD-FUS)
- Neuronal intermediate filament inclusion disease (NIFID)
- Basophilic inclusion body disease (BIBD)
Trinucleotide diseases
- Huntington's Disease (HD)
- Kennedy type spinobulbar muscular atrophy (SBMA)
- Friedreich's ataxia
- Spinocerebellar Ataxia (SCA)
- Dentatorubro-Pallidoluysic Atrophy (DRPLA)
Prion diseases
Motor neuron diseases
Neuroaxonal dystrophies
- Infantile neuraxonal dystrophy ( Seitelberger disease)
- Neurodegeneration with iron deposition in the brain (NBIA)
Unclassified
- CANVAS ( cerebellar ataxia, neuropathy, vestibular areflexia syndrome )
- Familial encephalopathy with neuroserpine inclusions
- Frontotemporal lobe degeneration with the ubiquitin proteasome system (FTLD-UPS)
- Gluten ataxia
- Mohr-Tranebjaerg syndrome
Individual evidence
- ↑ Alphabetical directory for the ICD-10-WHO version 2019, volume 3. German Institute for Medical Documentation and Information (DIMDI), Cologne, 2019, p. 630
- ^ A. Brunn: Lecture in general pathology , Department of Neuropathology, University of Cologne, summer semester 2005.
- ↑ a b Kurt A. Jellinger: Recent advances in our understanding of neurodegeneration . In: Journal of Neural Transmission . tape 116 , no. 9 , September 1, 2009, ISSN 0300-9564 , p. 1111–1162 , doi : 10.1007 / s00702-009-0240-y (English).
- ↑ Braun-Scharm, Hellmuth., Deister, Arno., Laux, Gerd .: Psychiatrie and Psychotherapy . 241 tables; [with patient discussions on video CD-ROM]. 4th, completely revised and exp. Edition with video CD-ROM. Thieme, Stuttgart 2009, ISBN 978-3-13-128544-7 .
- ↑ IR Mackenzie: Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. In: Acta neuropathologica. Volume 119, Number 1, January 2010, pp. 1-4, ISSN 1432-0533 . doi: 10.1007 / s00401-009-0612-2 . PMID 19924424 . PMC 2799633 (free full text).
