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DISEASES OF THE NERVOUS SYSTEM OF PROBABLE BUT UNPROVEN NUTRITIONAL ORIGIN
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The disorders comprised by this category, as the above title implies, have only an uncertain relationship to nutritional de ciency They are found mainly in alcoholics, but their relationship to alcohol is probably not fundamental, since each has also been observed in nonalcoholic patients The belief that these disorders are nutritional in origin is based on certain indirect evidence: (1) Usually, a prolonged period of undernutrition associated with a signi cant loss of weight precedes the neurologic illness In such cases that are associated with alcoholism, the amount of alcohol consumed need not be large, but the dietary deprivation is always severe (2) Examination at the onset of the illness frequently discloses general physical evidence of undernutrition as well as the presence of neurologic disorders of known nutritional etiology (3) Certain attributes of the neuropathologic changes namely their subacute evolution, symmetry, and constancy of localization are precisely the features that characterize neurologic disorders of known nutritional etiology (4) The same syndromes are observed occasionally in nonalcoholics under conditions of dietary depletion (5) There is at least a partial response to correction of the nutritional impairment
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Disorders Due to De ciencies of FatSoluble Vitamins
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Vitamin E De ciency This is of two types: a defect in intestinal absorption and an inherent hepatic enzyme de ciency that blocks incorporation of the vitamin into lipoprotein A rare neurologic disorder of childhood, consisting essentially of spinocerebellar degeneration in association with polyneuropathy and pigmentary retinopathy, has been attributed to a de ciency of vitamin E consequent to prolonged intestinal fat malabsorption (Muller et al; Satya-Murti et al) The same mechanism has been proposed to explain the neurologic disorders that sometimes complicate abetalipoproteinemia (page 827), brocystic disease (Sokol et al), celiac sprue disease (page 977), and extensive intestinal resections (Harding et al) Vitamin E de ciency has also been observed in young children with chronic cholestatic hepatobiliary disease (Rosenblum et al) Ataxia, loss of tendon re exes, ophthalmoparesis, proximal muscle weakness with elevated serum creatine kinase, and decreased sensation are the usual manifestations These symptoms are referable to parts of the nervous system and musculature that are found to be diseased in animals deprived of vitamin E degeneration of Clark s columns, spinocerebellar tracts, posterior columns, nuclei of Goll and Burdach, and sensory roots (Nelson et al) Local differences in the natural concentration of vitamin E in various parts of the nervous system and musculature are believed
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Alcoholic Cerebellar Degeneration
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This term refers to a common and uniform type of degeneration of the vermian and anterior lobes of the cerebellum in alcoholics Its incidence is about twice that of Wernicke disease, but unlike the latter, it is considerably more frequent in men than in women It is characterized clinically by a wide-based stance and gait, varying degrees of instability of the trunk, and ataxia of the legs, the arms being affected to a lesser extent and often not at all Nystagmus
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MAJOR CATEGORIES OF NEUROLOGIC DISEASE
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the Purkinje cells in the anterior and superior aspects of the vermis The cerebellar atrophy is readily visualized by computed tomography (CT) (Fig 41-2) and MRI Two particular forms of this syndrome have not been emphasized suf ciently In one, the clinical abnormalities are limited to an instability of station and gait, individual movements of the limbs being unaffected The pathologic changes in such cases are restricted to the anterosuperior portions of the vermis A second type is strikingly acute but transient Here, except for their reversibility, the cerebellar symptoms are identical to those that characterize the chronic, xed form of the disease In this transient type, the derangement is only one of function ( biochemical lesion ) and has probably not progressed to the point of xed structural changes These forms of cerebellar disease, and particularly the restricted and reversible varieties, cannot be distinguished from the cerebellar manifestations of Wernicke disease either on pathologic or on clinical grounds It is our opinion that the cerebellar ataxia of Wernicke disease and that referred to as alcoholic cerebellar degeneration are based on the same disease process, the former term being applicable when the cerebellar abnormalities are associated with ocular and mental signs and the latter when the cerebellar syndrome stands alone Alcoholic cerebellar degeneration is in all likelihood due to nutritional de ciency and not to the toxic effects of alcohol or other causes, for reasons already indicated Insofar as the cerebellar ataxia usually improves to some extent under the in uence of thiamine alone (see above, under Wernicke-Korsakoff Syndrome ), it is likely that a de ciency of this vitamin is in whole or part responsible for the cerebellar lesion, but this has not been proven
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Marchiafava-Bignami Disease (Primary Degeneration of the Corpus Callosum)
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Figure 41-2 CT scan from a 60-year-old alcoholic patient showing prominence of midline cerebellar sulci A broad-based gait and ataxia of the legs had been present for many years Death was from myocardial infarction The cerebellum, cut in the midsagittal plane (below), shows folial atrophy of the anterosuperior vermis, characteristic of alcoholic cerebellar degeneration
and dysarthria are infrequent In addition to an ataxic (intention) tremor, there may be a tremor of the ngers or hands resembling one of the two types of parkinsonian tremor but appearing only when the limbs are placed in certain sustained postures Mauritz and coworkers have demonstrated that the instability of the trunk in these cases consists of a speci c 3-Hz rhythmic swaying in the anteroposterior direction; by contrast, patients with lesions of the cerebellar hemispheres show only slight postural instability without directional preponderance In most cases, the cerebellar syndrome evolves over a period of several weeks or months, after which it remains unchanged for many years In others, it develops more rapidly or more slowly, but in these cases also the disease eventually stabilizes Occasionally, the cerebellar disorder progresses in a saltatory manner, the symptoms worsening in relation to a severe infectious illness or an attack of delirium tremens The pathologic changes consist of a degeneration of all the neurocellular elements of the cerebellar cortex but particularly of
In 1903, the Italian pathologists Marchiafava and Bignami described a unique alteration of the corpus callosum in three alcoholic patients In each case, coronal sectioning of the xed brain disclosed a pink-gray discoloration of the central portion of the corpus callosum throughout the longitudinal extent of this structure Microscopically, the lesion proved to be con ned to the middle lamina (which makes up about two-thirds of the thickness of the corpus callosum), in which there was a loss of myelin, and to some degree, of the axis cylinders; macrophages were abundant in the altered zone, and astrocytic proliferation had followed The clinical observations in these patients were few and incomplete In 1907, Bignami described a case in which the corpus callosum lesion was accompanied by a similar lesion in the central portion of the anterior commissure These early reports were followed by a spate of articles that con rmed and ampli ed the original clinical and pathologic ndings By 1922 about 40 cases of this disorder had been described in the Italian literature (Mingazzini) With one exception, all the reported cases were in males, and all of these men were insatiable drinkers They drank red wine for the most part but other forms of liquor as well Beginning in 1936, with the report of King and Meehan, the disease came to be recognized throughout the world, and the notions that it had a predilection for drinkers of red wine and a special racial predisposition or geographic locale were abandoned
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