A Restricted Form of Cerebellar Cortical Degeneration Occurring in Alcoholic Patients

Auditory brain stem responses in chronic alcoholic patients

“…Although brain stem lesions in WernickeKorsakoff syndrome, central pontine myelinolysis and cerebellar degeneration are well described in neuropathological studies Victor et al 1959Victor et al , 1971, the prevalence of brain stem abnormalities in alcoholic patients has not yet, with few exceptions, been systematically investigated either by pathological examination or by electrophysiological methods. In a study by Rosenhamer and Silfverskiold (1980) on 13 chronic alcoholics with cerebellar ataxia, 10 had abnormally prolonged I-V intervals in the ABRs.…”

“…Among the neurological complications of alcoholism, brain stem lesions are present in Wernicke-Korsakoff syndrome, cerebellar degeneration, and central pontine myelinolysis (Courville 1954;Neubuerger 1957;Adams et al 1959;Victor et al 1959Victor et al , 1971Lynch 1960). In spite of these known effects of alcohol or alcoholism on the brain stem, study on the prevalence of brain stem abnormalities in the general population of alcoholic patients has been ignored.…”

Cerebral Blood Flow and Metabolism in the Wernicke-Korsakoff Syndrome*

“…A similar relationship exists in pernicious anemia (vitamin B12 deficiency), although the cerebral pathologic lesion in this disorder is not so distinctive as the spinal lesion. If the site of the pathology is a clue to the anatomical localization of the metabolic defect in the Wernicke-Korsakoff syndome, one must conclude that thiamine deficiency has a specific effect on the metabolism of cells in the periaqueductal and periventricular gray matter, the mammillary bodies, the dorsomedial nuclei of the thalamus, the hypothalamus, the cerebellum, the hippocampi, and the fornices (3,7), sparing large areas of the brain, specifically the cerebral cortex, which lacks characteristic lesions. These data further clearly demonstrate that fairly localized metabolic defects may result in striking reduction of total cerebral blood flow and metabolism.…”

“…The syndrome first described in 1881 by Carl Wernicke (1) and presently considered to be characterized by disturbances in ocular motility, dementia, severe dysfunction of retentive memory, ataxia, and peripheral neuritis has been the object of many clinical and neuropathological studies (2)(3)(4)(5)(6)(7). It is most frequently observed in severe chronic alcoholics and is thought to be the consequence of nutritional deficiency.…”

Diphtheria toxin mutant selectively kills cerebellar Purkinje neurons.

“…Several inherited diseases in humans, such as ataxia telangectasia and certain lysosomal storage disorders, result in extensive loss of Purkinje cells (1). Loss of Purkinje cells is a prominent component of the histopathologic changes observed in cerebellar injury secondary to phenytoin and chronic ethanol intoxication (2)(3)(4)(5). Paraneoplastic cerebellar degeneration associated with carcinomas is also characterized by widespread disappearance of Purkinje cells (6).…”