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Neuropathology and pathogenesis of extrapyramidal movement disorders: a critical update-I. Hypokinetic-rigid movement disorders.
J Neural Transm (Vienna). 2019 08; 126(8):933-995.JN

Abstract

Extrapyramidal movement disorders include hypokinetic rigid and hyperkinetic or mixed forms, most of them originating from dysfunction of the basal ganglia (BG) and their information circuits. The functional anatomy of the BG, the cortico-BG-thalamocortical, and BG-cerebellar circuit connections are briefly reviewed. Pathophysiologic classification of extrapyramidal movement disorder mechanisms distinguish (1) parkinsonian syndromes, (2) chorea and related syndromes, (3) dystonias, (4) myoclonic syndromes, (5) ballism, (6) tics, and (7) tremor syndromes. Recent genetic and molecular-biologic classifications distinguish (1) synucleinopathies (Parkinson's disease, dementia with Lewy bodies, Parkinson's disease-dementia, and multiple system atrophy); (2) tauopathies (progressive supranuclear palsy, corticobasal degeneration, FTLD-17; Guamian Parkinson-dementia; Pick's disease, and others); (3) polyglutamine disorders (Huntington's disease and related disorders); (4) pantothenate kinase-associated neurodegeneration; (5) Wilson's disease; and (6) other hereditary neurodegenerations without hitherto detected genetic or specific markers. The diversity of phenotypes is related to the deposition of pathologic proteins in distinct cell populations, causing neurodegeneration due to genetic and environmental factors, but there is frequent overlap between various disorders. Their etiopathogenesis is still poorly understood, but is suggested to result from an interaction between genetic and environmental factors. Multiple etiologies and noxious factors (protein mishandling, mitochondrial dysfunction, oxidative stress, excitotoxicity, energy failure, and chronic neuroinflammation) are more likely than a single factor. Current clinical consensus criteria have increased the diagnostic accuracy of most neurodegenerative movement disorders, but for their definite diagnosis, histopathological confirmation is required. We present a timely overview of the neuropathology and pathogenesis of the major extrapyramidal movement disorders in two parts, the first one dedicated to hypokinetic-rigid forms and the second to hyperkinetic disorders.

Authors+Show Affiliations

Institute of Clinical Neurobiology, Alberichgasse 5/13, 1150, Vienna, Austria. kurt.jellinger@univie.ac.at.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

31214855

Citation

Jellinger, Kurt A.. "Neuropathology and Pathogenesis of Extrapyramidal Movement Disorders: a Critical update-I. Hypokinetic-rigid Movement Disorders." Journal of Neural Transmission (Vienna, Austria : 1996), vol. 126, no. 8, 2019, pp. 933-995.
Jellinger KA. Neuropathology and pathogenesis of extrapyramidal movement disorders: a critical update-I. Hypokinetic-rigid movement disorders. J Neural Transm (Vienna). 2019;126(8):933-995.
Jellinger, K. A. (2019). Neuropathology and pathogenesis of extrapyramidal movement disorders: a critical update-I. Hypokinetic-rigid movement disorders. Journal of Neural Transmission (Vienna, Austria : 1996), 126(8), 933-995. https://doi.org/10.1007/s00702-019-02028-6
Jellinger KA. Neuropathology and Pathogenesis of Extrapyramidal Movement Disorders: a Critical update-I. Hypokinetic-rigid Movement Disorders. J Neural Transm (Vienna). 2019;126(8):933-995. PubMed PMID: 31214855.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Neuropathology and pathogenesis of extrapyramidal movement disorders: a critical update-I. Hypokinetic-rigid movement disorders. A1 - Jellinger,Kurt A, Y1 - 2019/06/18/ PY - 2019/05/10/received PY - 2019/06/05/accepted PY - 2019/6/20/pubmed PY - 2020/7/29/medline PY - 2019/6/20/entrez KW - Genetics KW - Lewy body pathology KW - Movement disorders KW - Neuropathology KW - Parkinsonism KW - Pathophysiology KW - Polyglutamine repeat disorder KW - Proteinopathies KW - Tauopathies SP - 933 EP - 995 JF - Journal of neural transmission (Vienna, Austria : 1996) JO - J Neural Transm (Vienna) VL - 126 IS - 8 N2 - Extrapyramidal movement disorders include hypokinetic rigid and hyperkinetic or mixed forms, most of them originating from dysfunction of the basal ganglia (BG) and their information circuits. The functional anatomy of the BG, the cortico-BG-thalamocortical, and BG-cerebellar circuit connections are briefly reviewed. Pathophysiologic classification of extrapyramidal movement disorder mechanisms distinguish (1) parkinsonian syndromes, (2) chorea and related syndromes, (3) dystonias, (4) myoclonic syndromes, (5) ballism, (6) tics, and (7) tremor syndromes. Recent genetic and molecular-biologic classifications distinguish (1) synucleinopathies (Parkinson's disease, dementia with Lewy bodies, Parkinson's disease-dementia, and multiple system atrophy); (2) tauopathies (progressive supranuclear palsy, corticobasal degeneration, FTLD-17; Guamian Parkinson-dementia; Pick's disease, and others); (3) polyglutamine disorders (Huntington's disease and related disorders); (4) pantothenate kinase-associated neurodegeneration; (5) Wilson's disease; and (6) other hereditary neurodegenerations without hitherto detected genetic or specific markers. The diversity of phenotypes is related to the deposition of pathologic proteins in distinct cell populations, causing neurodegeneration due to genetic and environmental factors, but there is frequent overlap between various disorders. Their etiopathogenesis is still poorly understood, but is suggested to result from an interaction between genetic and environmental factors. Multiple etiologies and noxious factors (protein mishandling, mitochondrial dysfunction, oxidative stress, excitotoxicity, energy failure, and chronic neuroinflammation) are more likely than a single factor. Current clinical consensus criteria have increased the diagnostic accuracy of most neurodegenerative movement disorders, but for their definite diagnosis, histopathological confirmation is required. We present a timely overview of the neuropathology and pathogenesis of the major extrapyramidal movement disorders in two parts, the first one dedicated to hypokinetic-rigid forms and the second to hyperkinetic disorders. SN - 1435-1463 UR - https://www.unboundmedicine.com/medline/citation/31214855/Neuropathology_and_pathogenesis_of_extrapyramidal_movement_disorders:_a_critical_update_I__Hypokinetic_rigid_movement_disorders_ L2 - https://doi.org/10.1007/s00702-019-02028-6 DB - PRIME DP - Unbound Medicine ER -
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