Heterotopic Purkinje Cells: a Comparative Postmortem Study of Essential Tremor and Spinocerebellar Ataxias 1, 2, 3, and 6
| Autor: | Geoffrey Kelly, Jean Paul G. Vonsattel, Sheng-Han Kuo, Etty Cortes, Phyllis L. Faust, Jesus Gutierrez, William J. Tate, Elan D. Louis |
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| Rok vydání: | 2017 |
| Předmět: |
Adult
Male 0301 basic medicine Pathology medicine.medical_specialty Postmortem studies Cerebellum Neurology Essential Tremor Population Purkinje cell Choristoma Article Spinocerebellar atrophy Purkinje Cells 03 medical and health sciences 0302 clinical medicine Diagnosis medicine Humans Spinocerebellar Ataxias education Aged Aged 80 and over education.field_of_study Essential tremor Middle Aged medicine.disease 030104 developmental biology medicine.anatomical_structure Spinocerebellar ataxia Female Autopsy Neurology (clinical) Psychology Neuroscience 030217 neurology & neurosurgery |
| Zdroj: | The Cerebellum. 17:104-110 |
| ISSN: | 1473-4230 1473-4222 |
| Popis: | BACKGROUND: Essential tremor (ET) is among the most common neurological diseases. Postmortem studies have noted a series of pathological changes in the ET cerebellum. Heterotopic Purkinje cells (PCs) are those whose cell body is mis-localized in the molecular layer. In neurodegenerative settings, these are viewed as a marker of the progression of neuronal degeneration. OBJECTIVE: We (1) quantify heterotopias in ET cases vs. controls, (2) compare ET cases to other cerebellar degenerative conditions (Spinocerebellar ataxia [SCA] 1, 2, 3, and 6), (3) compare these SCAs to one another, and (4) assess heterotopia within the context of associated PC loss in each disease. METHODS: Heterotopic PCs were quantified using a standard LH&E stained section of neocerebellum. Counts were normalized to PC layer length (n-heterotopia count). It is also valuable to consider PC counts when assessing heterotopia, as loss of PCs extends both to normally located as well as heterotopic PCs. Therefore, we divided n-heterotopias by PC counts. RESULTS: There were 96 brains (43 ET, 31 SCA [12 SCA1, 7 SCA2, 7 SCA3, 5 SCA6] and 22 controls). The median number of n-heterotopias in ET cases was two times higher than that of controls (2.6 vs. 1.2, p |
| Databáze: | OpenAIRE |
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