Neurodegenerative Diseases
| 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. |
| Notes: |
GEN or unspecified; prefer specifics; /genet: consider also HEREDODEGENERATIVE DISORDERS, NERVOUS SYSTEM |
| Also Called: |
Degenerative Diseases, Central Nervous System,Degenerative Diseases, Spinal Cord |
Neurodegenerative Diseases Categories.
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Heredodegenerative Disorders, Nervous System - Inherited disorders characterized by progressive atrophy and dysfunction of anatomically or physiologically related neurologic systems. |
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Motor Neuron Disease - Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvment of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089) |
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Tauopathies - Neurodegenerative disorders involving deposition of abnormal tau protein isoforms (TAU PROTEINS) in neurons and glial cells in the brain. Pathological aggregations of tau proteins are associated with mutation of the tau gene on chromosome 17 in patients with ALZHEIMER DISEASE, DEMENTIA, PARKINSONIAN DISORDERS, progressive supranuclear palsy (SUPRANUCLEAR PALSY, PROGRESSIVE), and corticobasal degeneration. |
Neurodegenerative Diseases Definitions and Terms
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