The Arnold-Chiari malformation presenting in adult life. A report of thirteen cases and a review of the literature.
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Biomedical subjects
Publications and source records attributed to S Horenstein.
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Whipple's disease presenting as a neurological disease without gastrointestinal symptoms is an unusual occurrence. A 40 year old man suffered hypersomnia, memory loss and progressive ophthalmoplegia for 6 months prior to death. The nature of this disease was not established during life. Extensive granulomatous inflammation affecting the hypothalamus, hippocampus and periaqueductal gray matter of the brain was found to represent Whipple's disease by electron microscopy. Characteristic lesions were also present in spleen, mesenteric lymph nodes, small intestine and myocardium. Bacillary bodies and membranous inclusions similar to those seen in visceral lesions of Whipple's disease were present in macrophages. The findings supported the theory of direct involvement of the central nervous system by bacilli rather than a metabolic origin for the lesions.
Speech was studied in 18 patients who had a positive radioisotope brain scan within the left hemisphere and a history of a single stroke. Seventeen patients had definite language impariment. In most cases of either fluent or nonfluent aphasia, the center of the scan was behind the central sulcus. Patients with perisylvian involvement were much more likely to have severe comprehension deficit than those without such involvement. Those patients with the most severely decreased fluency were significantly more likely than those more fluent to have markedly decreased comprehension. In an occasional patient, involvement of fluency and comprehension was divergent, suggesting that these processes may be differentially affected.
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