Cases reported "Neurologic Manifestations"

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1/10. Scleroderma cerebritis, an unusual manifestation of progressive systemic sclerosis.

    A 42-year-old female with scleroderma experienced two exacerbations in which behavioral changes were the main clinical features. On both occasions she presented with paranoid delusions, perceptual aberrations, and disorientation. After treatment with corticosteroids, the patient's mental status returned to normal, and her electroencephalogram showed an increase in alpha wave frequency, which is consistent with a resolving delirium. Unlike systemic lupus erythematosus, scleroderma rarely involves the central nervous system. This case illustrates an unusual manifestation of progressive systemic sclerosis, primary cerebral involvement which presented as an acute organic brain syndrome. connective tissue diseases, notably systemic lupus erythematosus, often present neuropsychiatric symptoms. Despite the fact that there appears to be a clinical and pathological continuum among the connective tissue diseases, an organic psychosis rarely occurs in progressive systemic sclerosis (scleroderma. Described here is a patient with scleroderma in whom behavioral abnormalities were the main features of two exacerbations of the disease.
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2/10. central nervous system involvement following type I aviator's bends complicated by complacency.

    A false sense of security surrounds the possibility of post-flight complications resulting from "aviator's bends." The accepted clinical clue that a patient is at risk for serious complications is the presence of some form of dysbarism at altitude. This principle has been inappropriately extended to imply that serious post-flight complications of the evolved gas syndrome only follow serious in-flight symptoms. This paper, in addition to reporting the occurrence of post-flight neurologic signs in a patient after Type I pain-only bends during an altitude chamber flight, also identifies a broader subtle complacency in the professional community that routinely deals with hypobarics.
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3/10. Cytoplasmic tubular arrays in cells of American Burkitt's type lymphoma.

    Aggregates of tubular arrays associated with cysternae of endoplasmic reticulum were observed in untreated primary American form Burkitt's lymphoma. The tumor occurred in a white female child who died with severe central nervous system (CNS) involvement. Although the significance of the tubular structures and the mechanism by which they are formed remains unknown, the predilection of these structures for lymphoid and reticuloendothelial cells and their frequent association with either viral or suspected viral conditions cannot be overlooked. However, it is possible that the tubular structures may reflect an increased production of antibody protein in response to a viral antigen.
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4/10. Metastatic cystosarcoma phyllodes. A report of 2 cases presenting with neurological symptoms.

    Two cases of cystosarcoma phyllodes of the breast are presented with central nervous system (CNS) metastases appearing several years after mastectomy for the primary lesion. Unusual features in these cases include the widespread metastases themselves, neurologic symptomatology and CNS involvement, metastases into a uterine leiomyoma and into an area of hepatic adenomatous hyperplasia, and glomus-like structures in one of the primaries with similar structures resembling glomus cells in metastases. The distant metastases were of stromal cells only and frequently surrounded epithelial cells to isolate the indigenous glandular structures. The concept that cytosarcoma is a peculiar stromal neoplasm rather than a tumor of dual neoplastic origin is discussed.
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5/10. neurologic manifestations of cogan syndrome.

    cogan syndrome is a multisystem inflammatory vascular disease, characterized by nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Recent reports have directed attention to involvement of other organ systems. Respiratory, cardiovascular, gastrointestinal, and musculoskeletal problems are common, as are laboratory abnormalities and general symptoms such as fever, chills, and weight loss. Prominent neurologic problems in two patients prompted a review of 79 cases of cogan syndrome. More than half had nervous system involvement, including electroencephalographic or spinal fluid abnormality, headache, psychosis, coma, convulsion, neuropathy, and stroke. cogan syndrome should be considered when neurologic deficits are accompanied by eye, ear, and systemic symptoms.
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6/10. neurologic manifestations of legionnaires' disease.

    The neurologic manifestations of legionnaires' disease were reviewed in this paper. Nine (42.9%) of 21 patients seen at this institution had neurologic abnormalities during acute infection, comparable to 52% of patients found in the literature. Abnormal mentation was most common, occurring in all our patients and in 29.6% of patients in the literature. Encephalopathy disproportionate to metabolic disturbances varied from mild confusion to coma, was not associated with increased mortality, and tended to resolve with acute illness although some patients reported persistent memory defects. Rarely did these changes in mentation precede the development of pulmonary infiltrates. headache occurred in two (22.2%) of our patients and in 28.7% of those in the literature. headache, noted in many febrile illnesses, is common but nonspecific in legionnaires' disease. Other neurologic abnormalities including cerebellar dysfunction and focal deficits were relatively infrequent and tended to persist beyond resolution of clinical infection. In some cases these neurologic derangements may have occurred coincidentally with legionnaires' disease. Diagnostic evaluations and autopsies were frequently normal, nonspecific, or revealing of pathology unrelated to infection. Only two patients had evidence of direct invasion of the central nervous system by legionella, and the cause of encephalopathy and neurologic abnormalities in most cases remains unclear. patients with neurologic abnormalities and legionnaires' disease deserve full evaluation to exclude other entities. Only encephalopathy appears to be a characteristic manifestation of legionnaires' disease.
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keywords = nervous system
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7/10. Whipple's disease of the central nervous system.

    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.
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keywords = nervous system
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8/10. Neurological manifestations of gnathostomiasis.

    Neurological features of 24 patients with nervous system gnathostomiasis were reviewed. The commonest presenting features were radiculomyelitis or radiculomyelitis terminating with encephalitis, and subarachnoid haemorrhage. A primary encephalitic form was noted in 2 patients. The salient feature of the disease was a sudden onset of extremely severe radicular pain and/or headache followed by paralysis of the extremities and/or the cranial nerves. Migration signs were also the hallmark of nervous system gnathostomiasis. No single area of the nervous system was inaccessible to the highly invasive gnathostome lava. Multiplicity and/or rapid progress of lesions beyond the degree of cerebral oedema could only be explained by further migration of the parasite. death occurred in 6 patients from direct extensive involvement of vital centres in the brain stem or from complications such as pneumonia or septicaemia. Multiple cranial nerve palsies were usually bad prognostic signs.
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keywords = nervous system
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9/10. The Riley-Day syndrome. Familial dysautonomy, central autonomic dysfunction.

    The Riley-Day syndrome is characterized by a dysfunction of the autonomous nervous system, sensory disturbances, neurological disorders, psychical anomalies and important ophthalmological symptoms, such as absence of tears, corneal anaesthesia, keratinized conjunctiva and cornea; myosis after instillation of methacholine. The diagnosis is based on the absence of fungiform papillae of the tongue and the absence of reaction after intradermic injection of histamine. The inheritance is autosomal recessive. The disease results probably from an enzymatic insufficiency.
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10/10. Systemic mastocytosis--central nervous system features and lymphographic demonstration of lymph node involvement.

    Systemic mastocytosis is uncommon. A patient is reported with many of the characteristic lesions of this disorder but with two features not before described--the occurrence of mixed pyramidal and neuromyopathic neurological signs and the demonstration of lymph nodes with abnormal architecture by lymphography.
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