AI Article Synopsis
- The paper reviews the historical development of understanding degenerative cerebellar disorders and hereditary spastic paraplegias over the last 150 years.
- It highlights key pathological subtypes like Friedreich's ataxia and striatonigral degeneration, along with Hans Joachim Scherer’s contributions.
- The research emphasizes significant progress in the classification of ataxias with around 200 recognized genetic subtypes due to recent molecular findings.
Article Abstract
The aim of this paper is to carry out a historical overview of the evolution of the knowledge on degenerative cerebellar disorders and hereditary spastic paraplegias, over the last century and a half. Original descriptions of the main pathological subtypes, including Friedreich's ataxia, hereditary spastic paraplegia, olivopontocerebellar atrophy and cortical cerebellar atrophy, are revised. Special attention is given to the first accurate description of striatonigral degeneration by Hans Joachim Scherer, his personal and scientific trajectory being clarified. Pathological classifications of ataxia are critically analysed. The current clinical-genetic classification of ataxia is updated by taking into account recent molecular discoveries. We conclude that there has been an enormous progress in the knowledge of the nosology of hereditary ataxias and paraplegias, currently encompassing around 200 genetic subtypes.
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| http://dx.doi.org/10.1007/s12311-021-01328-6 | DOI Listing |
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