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Six cases of apparent and four cases of histopathologically confirmed vasculitis of the central nervous system (CNS), including one case of histopathologically documented vasculitis with encephalitis associated with coccidioidal meningitis (CM), are presented. Vasculitic complications included changes in mental status as well as stroke-like findings of aphasia, hemianopsia, and hemiparesis. Seven patients died. Vasculitic complications were unanticipated and often abrupt in onset, and delayed therapeutic intervention was characteristic. The diagnosis of vasculitis/encephalitis due to Coccidioides immitis infection must be based on clinical judgment, since serum antibody titers, cerebrospinal fluid findings, and initial radiological studies are not always helpful. Institution of both intravenous and intracisternal administration of amphotericin B and possibly concomitant intravenous administration of dexamethasone may be warranted in situations in which the association of C. immitis with CNS vasculitis or encephalitis appears likely before serologic or cultural confirmation of C. immitis infection involving the CNS is available.

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At the time of spinal fusion, muscle biopsies were taken from both the convex and concave side at the apex of the curve from 27 patients with idiopathic scoliosis. Histochemical and morphometric studies showed generally small and atrophic type 1 muscle fibers on both sides, with preservation of the normal type 1 predominance. The most striking abnormality was a type 2A predominance over type 2B fibers, the reverse of normal in the paraspinous muscle. These changes showed no correlation with sex, age, or electrical stimulation but showed a significantly greater severity with the duration and severity of the curve. We conclude that these changes are a secondary muscle adaptation to the curve and not its primary etiology.

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The muscle fibers in 19 patients with nonidiopathic scoliosis were generally small, especially on the concave side of the curve. Type 1 fibers were more frequently atrophic than type 2 fibers, but the former usually remained larger than the latter. Type 1 preponderance was greater on the convex side of the curve; type 2A preponderance occurred bilaterally. These changes are similar to those of idiopathic scoliosis. They resemble those of endurance training or stretch and are interpreted as secondary to the curve.

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Muscle biopsies from the apex of both sides of the curve of 31 patients with idiopathic scoliosis showed abnormalities in fiber-type distribution in 68 per cent and in fiber size in 55 per cent. There was no preference for either side. Type 1 fiber predominance was as common as type 1 fiber deficiency. Atrophy occurred in 33 per cent and affected mainly type 1 fibers: atrophy of type 2 fibers was rare. Hypertrophy was limited to type 2 fibers, and occurred in 26 per cent. The strength factor for type 1 fibers exceeded that for type 2. Type 2A fibers were no larger than 2B fibers: there was a large type 2A predominance, more so on the convex side. Most of the muscle changes appear to be secondary and compensatory: none suggests a pathogenesis for the curve.

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Central pontine myelinolysis (CPM) was found at autopsy in 21 of 220 consecutive patients with chronic liver disease. It showed the same incidence in chronic nonalcoholic as in chronic alcoholic liver disease but did not occur in acute liver disease. No patients had clinical symptoms of CPM, although 15 had encephalopathy. The lesion was active in 13 and inactive in 8. Laboratory data was incomplete in 2 patients with active and 8 with inactive CPM. Of 11 closely monitored patients with active CPM, 6 had a rapid rise in serum sodium of at least 8 meq/l per day, sustained for 5 or more days, and preceding death by 8-21 days; the other 5 showed no rise during 15-64 days prior to death. All patients with a large, rapid, sustained rise in serum sodium showed CPM at autopsy, but half of those with active CPM showed no such sodium changes even though closely monitored.

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Histopathologic studies of the cerebral cortex, hippocampus, and three subcortical nuclei were performed in four patients with Parkinson's disease whose mental status had been evaluated by neuropsychologic testing. Clinicopathologic correlations suggest that dementia with marked visuospatial disturbance as well as severe aphasia may be associated with severe neuronal loss in subcortical nuclei, without significant numbers of plaques or tangles in the hippocampus and cerebral cortex. Furthermore, memory loss may not be consistently related to neuronal loss in the nucleus basalis of Meynert, particularly in non-Lewy body parkinsonism.

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There is little information on the muscle fiber changes or the fiber type affected in supraspinal hemiplegia. Muscle biopsy specimens from 20 patients with stroke, obtained during orthopedic reconstruction, were examined by modern histochemistry. Atrophy was present in all of the muscles, affecting Type 1 fibers in 100% and Type 2 fibers in 95% of the patients. Type 2 atrophy was more severe than Type 1 atrophy. Group atrophy and fiber type grouping, present in 40%, seemed related to peripheral nerve or root damage. Hypertrophy of Type 1 fibers was present in 45%, associated with Type 2 hypertrophy in 15%. Although diffuse morphometric atrophic seemed not to correlate with the level of motor activity in this group of 20 patients, hypertrophy appeared related to activity. Hence, efforts to mobilize and rehabilitate stroke patients cannot prevent atrophy of some fibers, they seem to stimulate a hypertrophy not seen in inactive patients.

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Numbers of neurons in the nucleus basalis of Meynert were estimated in seventeen non-demented patients who died of chronic hepatic or cardiopulmonary disease. Neurons were counted at the site of maximal neuronal density (SMND). This site was chosen by reviewing serial sections around the decussation of the anterior commissure and appeared to be comparable in different individuals. No correlation between numbers of neurons and age could be found. It appears that no uniform neuronal loss occurs in the nucleus basalis with age. Taken together with biochemical studies of cerebral cortical choline acetyltransferase activity, these findings suggest that there is no overall change in cholinergic input to cerebral cortex with age.

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Lymphomas may occur in the central nervous system either as primary lesions or secondary to lymphomas in other sites. While lymphomas secondary to generalized lymphoproliferation may occur in either the brain or the spinal cord, primary lymphomas are found almost exclusively in the brain. Only three primary lymphomas of the cord have been reported. We recently had the opportunity to study a fourth case. Spinal cord lymphoma was not the diagnosis considered in any of these cases. Since the lesion is radiosensitive, inclusion of primary lymphoma in the differential diagnosis of obscure spinal cord tumors could result in a histological diagnosis and effective therapy.

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We report the first histologic findings of the CNS in cephalothoracopagus janiceps monosymmetros. The findings are compared with the other ten reported cases of this type of conjoined twin. The faces and CNSs showed asymmetry of development manifested chiefly in the prosencephalon, specifically the cortical gyri, corpus callosum, and subependymal mantle layer. It is difficult to explain discordance for congenital malformations in monovular or conjoined twins by either genetic or environmental mechanisms. It appears related to the vascular supply of the brain and a state of chronic ischemia rather than to a discrete insult and developmental arrest at a specific time during embryogenesis.

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Multiple myeloma commonly produces neurologic symptoms when it involves the cranium or vertebrae, but rarely invades the CNS or meninges. Intracranial or intraspinal myeloma without lesions in the adjacent bone is extremely rare. To our knowledge, there are only ten reported cases of isolated myeloma in dura and/or brain, and only two limited to the leptomeninges. We wish to report the third case of isolated leptomeningeal myeloma. Both myeloma cells and abnormal globulins were present in the CSF, but absent from serum and bone marrow. Analysis of these 13 cases in dura, brain, or leptomeninges strongly supports the concept that abnormal proteins do not reach the CSF from the serum but are produced by myeloma cells in situ.

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A 28-year-old woman with a eight-year history of rheumatoid arthritis presented with a three-day history of dyspnea. Physical and electrocardiographic findings were consistent with pulmonary hypertension. Arterial blood gases revealed a ventilation-perfusion mismatch. Chest roentgenogram was normal. After transient improvement, she suddenly deteriorated and died. At autopsy, a necrotizing pulmonary panarteritis was found without parenchymal involvement by rheumatoid disease. The pulmonary arteries were the only vessels affected. Immunofluorescent staining revealed immunoproteins scattered throughout the vessel walls without localization to the basement membrane. The unique features of the case are discussed in relation to pulmonary hypertension and rheumatoid lung disease in which vascular lesions are usually associated with honeycomb lung. The association between the rheumatoid arthritis and pulmonary vasculitis was probably coincidental.

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Diabetic myelopathy occurred in 41% of 75 consecutive, unselected diabetic patients in an autopsy study; clinical peripheral neuropathy occurred in 13%, and histologic radiculopathy in 21%. Infection represented 2.7% of the cord lesions. Posterior column demyelination, seen in 27%, apparently has the same metabolic-toxic origin as diabetic neuropathy and radiculopathy; it is an independent lesion, not a secondary manifestation of peripheral demyelination. It occurs slightly more frequently in those with juvenile-onset diabetes. Spinal cord infarcts, seen in 19%, are related to anteriolar sclerosis of the intrinsic vessels of the spinal cord. They have a higher incidence in diabetics than in a nondiabetic aging population, show a predilection for the white matter, and are usually small. The myelopathy is not related to patient age or duration of diabetes. It is often clinically occult.

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We report the cases of two siblings who died at age 21 and 15 years respectively. Both had optic atrophy, pitressinsensitive diabetes insipidus, and insulin-dependent diabetes mellitus, with onset occurring in early childhood. Although there are now 21 patients from 15 families with this syndrome, this is the first time that necropsy findings have become available. They include the expected atrophy of hypothalamic nuclei and degeneration of the optic nerves, chiasm and tract, as well as a totally unexpected degeneration of the pons and cerebellum.

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A female infant with neonatal hypotonia and lethargy was found to have nonketotic hyperglycinemia. She died at the age of 5 days. Autopsy revealed slightly retarded myelination and severe spongy change in the well-myelinated areas of the brain. Analysis of this and the other 26 reported cases suggests that patients with nonketotic hyperglycinemia develop severe mental retardation, not seen in ketotic hyperglycinemia. Elevated glycine levels in the brain and cerebrospinal fluid appear to differentiate these two forms of hyperglycinemia better than the presence of ketosis or leukopenia, and high glycine levels apparently occur in the same areas as the spongy change. While both forms show defective glycine cleavage in the liver, defective glycine cleavage in the brain has been reported only in nonketotic hyperglycinemia.

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