Olivopontocerebellar Atrophy

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Olivopontocerebellar Atrophy


Olivopontocerebellar Atrophy(OPCA), is characterized by neuronal degeneration of the cerebellar cortex, the inferior olive, and the pons. The symptoms associated with it are primarily cerebellar ataxia with disturbances in equilibrium and gait. However, broader symptomology is usually seen with OPCA. Current research is focusing on three primary systems thought to be responsible for the etiology of OPCA. They are excitatory amino acid disturbances, oligodendroglial microtubular tangles, and phospholipid metabolism disorders. The only treatment for OPCA is therapy focusing on improving the dysphagia associated with the disorder.

Olivopontocerebellar Atrophy

Olivopontocerebellar Atrophy (OPCA) is a disease characterized primarily by the degeneration of neurons in the cerebellar cortex, pons, and inferior olive. It is a genetic disease, being either autosomal dominant or autosomal recessive in nature. This disorder, which usually occurs in the middle years of life, presents symptoms of cerebellar ataxia, equilibrium disturbance, nystagmus, dysphasia, dysarthria, and possibly intellectual deficits.

According to Merritt, the pathology of OPCA includes loss of Purkinje cells, reduction of the number of neurons in the molecular and granular layers of the cerebellum, degeneration of the folia and white matter of the cerebellum, atrophy of the inferior olives and of the olivo-cerebellar connections, and atrophy of the pontine nuclei, arcuate nuclei, and brachium pontis (15). In addition to this, degeneration of the spinocerebellar tracts, corticospinal tracts, and frontal and temporal lobes has been reported (15).

Biopsies on living OPCA patients have suggested that there are n...

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...cerebellar atrophy. Annals of Neurology, 26:362-367, 1989.

12. Kish, S., Robitaille, Y., El-Awar, M. et. al. Brain amino acid reductions in one family with chromosome 6p-linked dominantly inherited olivopontocerebellar atrophy. Annals of Neurology, 30:780-784, 1991.

13.Landis, D., Rosenburg, R., Landis, S. et. al. Olivopontocerebellar degeneration. Archives of Neurology, 31:295--307, 1974.

14. Makowiec, R., Albin, R., Cha, J-H. et. al. Two types of quisqualate receptors are decreased in human olivopontocerebellar atrophy cerebellar cortex. Brain Research, 523:309-312, 1990.

15. Merritt, Houston. A Textbook of Neurology. Lea and Febiger, Philadelphia. 1967

16. Nakazato, Y., Yamazaki, H., Hirato, J. et. al. Oligodendroglial microtubular tangles in olivopontocerebellar atrophy. Journal of Neuropathology and Experimental Neurology, 49:521-530, 1990.
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