Cerebellar cortex ultrastructure in ataxia-telangiectasia
Article first published online: 16 OCT 2004
Copyright © 1983 American Neurological Association
Annals of Neurology
Volume 13, Issue 3, pages 297–302, March 1983
How to Cite
Paula-Barbosa, M. M., Ruela, C., Tavares, M. A., Pontes, C., Saraiva, A. and Cruz, C. (1983), Cerebellar cortex ultrastructure in ataxia-telangiectasia. Ann Neurol., 13: 297–302. doi: 10.1002/ana.410130312
- Issue published online: 16 OCT 2004
- Article first published online: 16 OCT 2004
- Manuscript Accepted: 18 JUL 1982
- Manuscript Revised: 12 JUL 1982
- Manuscript Received: 13 MAY 1982
An ultrastructural study of a biopsy fragment from the cerebellar cortex of a patient with ataxia-telangiectasia was performed. There was a sharp reduction in the number of Purkinje cells and an increase in the number of Golgi epithelial cells. Despite the degeneration of Purkinje cell dendrites, the volumetric density of parallel fiber varicosities and the number of synapses in the molecular layer were only slightly reduced. No qualitative nor quantitative abnormalities were noted in the granular layer.
An analysis is made of the severe changes in cerebellar circuitry of this patient and their probable functional implications.