Olivopontocerebellar Atrophy Essay -- Biology Biological Essays

Olivopontocerebellar Atrophy Olivopontocerebellar atrophy (OPCA) was first described in 1900 by Dejerine and Thomas. OPCA is a group of dominant inheritance and sporadic neurological disorders characterized by a chronic, progressive, cerebellar ataxia that begins in middle age. The cerebellum and its connections are the primary sites of the disease in chronic progressive disorders that often occur in familial or hereditary patterns. Postmortem studies indicate an atrophy of the cerebellum, pons, and inferior olives. This neuropathological neuronal cell loss permits classification of OPCA as a non-Alzheimer’s neurodegenerative illness. Gross postmortem inspection of the brains of patients with OPCA shows marked shrinkage of the ventral half of the pons, and disappearance of the olivary eminence on the ventral surface of the medulla. These brains also exhibit an atrophy of the cerebellum with degeneration of the middle cerebellar peduncles, and to a lesser extent, of the inferior peduncles. Thus, the cerebellum suffers mainly through atrophy of its afferent fibers. The neocerebellum and the olive undergo the primary degeneration. The purkinje cells of the cerebellar cortex are affected secondarily. Histological examination shows severe degeneration of Purkinje cells, reduction in the number of cells in the molecular and granular layers of the cerebellar cortex, severe loss of the number of cells in the pontine nuclei and olives, and demyelination of the middle cerebellar peduncle. The cerebellar nuclei are well preserved. The tegmentum of the pons, the corticospinal tracts, and the restiform body are also usually unaffected. In clinical cases involving extrapyramidal symptoms, degenerative changes in the striatum, espec... ...s Elevated in Brain of Patients with Dominantly Inherited Olivopontocerebellar Atrophy. Neuroscience Letters (submitted publication). Kish, Stephen J. et al : Brain Amino Acid Abnormalities in Dominantly Inherited Olivopontocerebellar Atrophy. Revised manuscript in preparation for resubmission to J. Neurochemistry. Kish, Stephen J. et. al. Cognitive Deficits in Olivopontocerebellar Atrophy: Implications for the Cholinergic Hypothesis of Alzheimer’s Dementia. Annals of Neurology: 24 (2), 200-206, 1988. Rowland, Lewis P. (ed.): Merritt's Textbook of Neurology, eighth edition. Lea and Febiger. Philadelphia, 1959, pp. 630--631. Snell, Richard S: Clinical Neuroanatomy for Medical Students. Little, Brown, and Company, Boston, 1957, pp. 220--222. Walton, Sir John. Brain's Diseases of the Nervous System. Oxford University Press. New York, 1955, pp. 365. Olivopontocerebellar Atrophy Essay -- Biology Biological Essays Olivopontocerebellar Atrophy Olivopontocerebellar atrophy (OPCA) was first described in 1900 by Dejerine and Thomas. OPCA is a group of dominant inheritance and sporadic neurological disorders characterized by a chronic, progressive, cerebellar ataxia that begins in middle age. The cerebellum and its connections are the primary sites of the disease in chronic progressive disorders that often occur in familial or hereditary patterns. Postmortem studies indicate an atrophy of the cerebellum, pons, and inferior olives. This neuropathological neuronal cell loss permits classification of OPCA as a non-Alzheimer’s neurodegenerative illness. Gross postmortem inspection of the brains of patients with OPCA shows marked shrinkage of the ventral half of the pons, and disappearance of the olivary eminence on the ventral surface of the medulla. These brains also exhibit an atrophy of the cerebellum with degeneration of the middle cerebellar peduncles, and to a lesser extent, of the inferior peduncles. Thus, the cerebellum suffers mainly through atrophy of its afferent fibers. The neocerebellum and the olive undergo the primary degeneration. The purkinje cells of the cerebellar cortex are affected secondarily. Histological examination shows severe degeneration of Purkinje cells, reduction in the number of cells in the molecular and granular layers of the cerebellar cortex, severe loss of the number of cells in the pontine nuclei and olives, and demyelination of the middle cerebellar peduncle. The cerebellar nuclei are well preserved. The tegmentum of the pons, the corticospinal tracts, and the restiform body are also usually unaffected. In clinical cases involving extrapyramidal symptoms, degenerative changes in the striatum, espec... ...s Elevated in Brain of Patients with Dominantly Inherited Olivopontocerebellar Atrophy. Neuroscience Letters (submitted publication). Kish, Stephen J. et al : Brain Amino Acid Abnormalities in Dominantly Inherited Olivopontocerebellar Atrophy. Revised manuscript in preparation for resubmission to J. Neurochemistry. Kish, Stephen J. et. al. Cognitive Deficits in Olivopontocerebellar Atrophy: Implications for the Cholinergic Hypothesis of Alzheimer’s Dementia. Annals of Neurology: 24 (2), 200-206, 1988. Rowland, Lewis P. (ed.): Merritt's Textbook of Neurology, eighth edition. Lea and Febiger. Philadelphia, 1959, pp. 630--631. Snell, Richard S: Clinical Neuroanatomy for Medical Students. Little, Brown, and Company, Boston, 1957, pp. 220--222. Walton, Sir John. Brain's Diseases of the Nervous System. Oxford University Press. New York, 1955, pp. 365.