1/44. Acute cerebral phaeohyphomycosis due to Wangiella dermatitidis accompanied by cerebrospinal fluid eosinophilia.We report a case of cerebral phaeohyphomycosis due to Wangiella dermaitidis in an immunocompetent adult man. His cerebrospinal fluid (CSF) showed pleocytosis with a high eosinophil count but without peripheral blood eosinophilia. The present case suggested that this black yeast-like fungus should be included when the causes of CSF eosinophilia are considered, even though it is an extremely rare pathogen.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
2/44. Clinical application of the multifocal VEPs.PURPOSE: To determine whether visual field defects can be detected by the multifocal VEP technique. methods: Multifocal VEPs were elicited by a pseudorandom binary m-sequence stimulus (VERIS II). The stimulus was a dartboard-like pattern of 61 sectors, and the luminance of each sector alternated between white and black. The stimulus area subtended approximately 25 degrees. Each recording was divided into 8 equal segments, and the total recording time was about 4 min. Multifocal VEPs were recorded from 25 normal subjects and six patients with visual field loss. The responses summed within 4 quadrants were used in the analysis and were compared with the visual fields obtained by perimetry. RESULTS: In six perimetrically-documented visual field defects, the responses summed over each quadrant of the field were reduced in the corresponding affected quadrants. In addition, recovery of the visual field loss following treatment was accompanied by a recovery of the responses. CONCLUSIONS: Multifocal VEPs summed within four quadrants can be used for an objective evaluation of the visual fields. The testing can be obtained in 4 min with no pain or discomfort to the patient.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
3/44. Neurocutaneous melanosis with malignant leptomeningeal melanoma. A case with metastases outside the nervous system.A case of neurocutaneous melanosis with malignant leptomeningeal melanoma was seen in a black man. The presence of extracranial metastases was noted, and evidence is presented favoring the central nervous system as the primary source.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
4/44. Neuropathology of Raine syndrome.We present three cases of Raine syndrome occurring in siblings of consanguineous parents. Raine syndrome is characterised by generalised osteosclerosis with craniofacial anomalies and intracranial calcifications. So far, only nine cases have been reported, and no evaluation of the distribution and extent of the cerebral mineralisations, as well as their impact on the surrounding tissue, has been undertaken yet. In our cases, calcifications were unevenly distributed throughout the central nervous system, not associated with neuronal loss or dystrophic events and appeared mostly as single calcospherites within the neuropil with occasional confluent deposits at advanced gestational age. There was intense perifocal microgliosis around single immature calcospherites, as well as mild astrogliosis around and within the confluent lesions, in which occasional macrophages could be found. Rarely, mineralisations occurred in blood-vessel walls, mainly affecting basal ganglia. Preferential sites of calcification were parietal and occipital periventricular white matter and corpus callosum, while frontal lobes were mildly affected. The cortex, temporal lobes as well as internal capsule, brain stem, cerebellum, leptomeninges, pituitary gland and choroid plexus were devoid of mineralisations. The subcortical grey matter was moderately involved in the putamen and pallidum, mildly in the caudate nucleus and subependymal germ cell matrix and not at all in the thalamus, Ammon's horn, amygdala and substantia nigra. The distribution of mineral deposits was thus inversely correlated to regional blood circulation and capillary density, with calcifications being concentrated in more sparsely perfused areas but lacking in highly vascularised tissue. This inverse relationship between mineralisation and regional blood flow was reflected in the varying distribution of calcospherites in grey and white matter as well as in the white matter of different lobes.- - - - - - - - - - ranking = 67.461671970487keywords = nigra (Clic here for more details about this article) |
5/44. Striato-nigral degeneration and shy-drager syndrome (idiopathic orthostatic hypotension).The present paper reports on a case which evolved clinically with a Parkinson syndrome and attacks of orthostatic hypotension. Dystrophic lesions were found in the substrantia nigra, putamen and autonomic bulbo-medullary axis. This case demonstrates that striato-nigral degeneration of the Adams, van Bogaert and van der Eecken type and the shy-drager syndrome (orthostatic hypotension with neurologic syndrome) are part of the same pathiologic entity respesenting nervous multisystem degeneration developing in the presenium.- - - - - - - - - - ranking = 404.77003182292keywords = nigra (Clic here for more details about this article) |
6/44. Pathogenesis of pigment and spheroid formation in Hallervorden-Spatz syndrome and related disorders.In a case of Hallervorden-Spatz syndrome, neuromelanin was found in neurons and, extracellularly, in the globus pallidus and pars reticulata of the substantia nigra. Some cells of pars compacta contained Lewey bodies. We propose that neuromelanin is formed by a metal-catalyzed pseudoperoxidation of lipofuscin, involving increased amounts of iron and copper in the affected regions. A similar mechanism of spheroid formation, often associated with neuromelanin, may result from pathologic accumulations of lipid peroxides during fatty acid oxidation of myelin. We suggest that neuromelanin is a late stage in the metabolism on intraneuronal and extraneuronal lipopigments. Discrepancies among the histochemical features of the pigment in different cases may be explained by differences in amounts of lipofuscin, neuromelanin, and their precursors. We propose relation of peroxidation to the pathogenesis of some related degenerative diseases.- - - - - - - - - - ranking = 67.461671970487keywords = nigra (Clic here for more details about this article) |
7/44. Neuropsychological deficits accompanying striatonigral degeneration.This study presents a structural and functional description of a case of striatonigral degeneration (SND) and emphasizes neuropsychological findings. The patient, a 55-year-old woman with progressive and relatively intractable rigidity and bradykinesia, particularly of the right side, was studied with brain MR scans and with a wide variety of sensory, motor, and cognitive tests known to be subserved by specific brain regions. T2-weighted MR images revealed curvilinear areas of high signal in the lateral putamen at low magnetic field strength (0.3T) and adjacent regions of marked low signal in the posterior-lateral putamen at high magnetic field (1.5T). High signal changes in the insular cortex were also noted on the high field images. letter fluency and short-term memory as well as motor speed, strength, and sequencing were selectively impaired. Taken together, the data of this case suggest that structural involvement of the putamen resulted in dysfunctions usually associated with the primary motor cortex and orbitofrontal cortex, while sparing functions of other frontal regions as well as temporal and parietal cortices.- - - - - - - - - - ranking = 337.30835985243keywords = nigra (Clic here for more details about this article) |
8/44. cladosporium trichoides cerebral phaeohyphomycosis in a liver transplant recipient. Report of a case.cerebral phaeohyphomycosis (also referred to as cerebral chromomycosis), one of the diseases caused by the dematiaceous (black) fungi, is most commonly caused by cladosporium trichoides (referred to by some as Xylohypha bantiana) and is a rare disease, with 31 culture-proven cases reported to date. Although most cases have occurred in immunocompetent hosts, recent experimental evidence suggests that host immunosuppression may predispose patients to the disease. The authors report a case of fatal cerebral phaeohyphomycosis in a liver transplant patient, the first to occur in a transplant patient of any type, to the best of the authors' knowledge. This case provides support for the hypothesis that immunosuppressed patients may be at increased risk for development of this disease.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
9/44. Wilson's disease: computed tomography and magnetic resonance imaging findings.The condition of a patient with Wilson's disease was evaluated by magnetic resonance imaging (MRI) on initial presentation of the illness. The examination revealed mild atrophy of the superior vermis. Symmetric areas of increased signal intensity on T2-weighted images were detected in the anterior thalami, mesencephalic tectum and tegmentum. Marked symmetric hypointensities appeared in the head of caudate, pallida, substantia nigra and red nuclei. The histopathology of Wilson's disease suggests that these hypointensities may be secondary to the presence of protein-bound copper.- - - - - - - - - - ranking = 67.461671970487keywords = nigra (Clic here for more details about this article) |
10/44. Corticonigral degeneration with neuronal achromasia and basal neurofibrillary tangles.A man, aged 58 years, suffered from progressive dementia, parkinsonism, and gaze paralysis for 30 months. autopsy revealed severe degeneration of the substantia nigra, numerous swollen chromatolytic neurons within the cerebral cortex, scattered basal neurofibrillary tangles, and gliosis of the cerebral white matter and basal ganglia. Unusual globular inclusions positive for tau protein were detected within neurons of the upper cortical layers. Although the pathological findings were comparable with corticonigral degeneration with neuronal achromasia, several clinical and pathological features characteristic for progressive supranuclear palsy, progressive subcortical gliosis, and Pick's disease in this and the nine previously reported cases hampered the unequivocal nosological placement.- - - - - - - - - - ranking = 404.77003182292keywords = nigra (Clic here for more details about this article) |
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