1/21. Prolonged cerebral salt wasting syndrome associated with the intraventricular dissemination of brain tumors. Report of two cases and review of the literature.hyponatremia is a frequent event in neurosurgery practice and is usually associated with subarachnoid hemorrhage, head trauma, infections and neoplasms. The two common clinical manifestations are the inappropriate secretion of antidiuretic hormone (SIADH) and the cerebral salt wasting syndrome (CSWS), which were usually attributed to each other due to identical clinical presentation. In contrast to the better-recognized SIADH, there has not been a uniform consensus over the humoral and neural mechanisms of CSWS and functional aspects of renal response. In this article, we report on 2 cases of a primitive neuroectodermal tumor with prolonged CSWS manifested during the intraventricular dissemination of primary disease and the high catabolic stage.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/21. tay-sachs disease: progression of changes on neuroimaging in four cases.The neuroradiological findings in four patients with tay-sachs disease are described in three phases of the clinical course. The basal ganglia and cerebral white matter show low density on computed tomography and high signal intensity on T2-weighted magnetic resonance imaging in the initial phase. The caudate nuclei are characteristically enlarged and protrude into the lateral ventricles in the first and second phases. The cerebral white matter shows low density on the CT which varies in extent from the second to third phases, and the whole brain becomes atrophic in the last phase. Thus, central nervous system involvement in the disease may begin in basal ganglia as well as in cerebral white matter.- - - - - - - - - - ranking = 103.09320696058keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/21. biopsy diagnosis of familial Alexander's disease.A 26-year-old woman presented with headaches, incoordination and a cerebellar mass (1982). The CT scan revealed dilated ventricles and a hypodense space-occupying lesion adjacent to the fourth ventricle. Neuronal loss, gliosis and masses of Rosenthal fibers were seen in biopsy. There was no evidence of neoplasm. A second biopsy 2 years later was similar to the original specimen. A diagnosis of Alexander's disease was suggested. Later that year the patient's 11-year-old brother manifested a clinical picture initially diagnosed as brainstem glioma, but whose biopsy was characteristic of Alexander's disease. There has been a gradual deterioration of these siblings over the past 6 years (1986-1991). No evidence of neoplasm has appeared.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
4/21. MRI of a very rare hereditary ectodermal dysplasia: PIBI(D)S.PIBI(D)S is a acronym for a very rare autosomal recessive syndrome consisting of photosensitivity, mild non-congenital ichthyosis, brittle cystine-deficient hair, impaired intelligence, occasionally decreased fertility and short stature. We report a 12-year-old female patient affected by PIBI(D)S with previously unreported MRI findings of central nervous system dysmyelination.- - - - - - - - - - ranking = 103.09320696058keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/21. Methyl bromide intoxication causes reversible symmetric brainstem and cerebellar MRI lesions.Methyl bromide is toxic to the central and peripheral nervous systems. A patient with occupational exposure to this agent is described. MRI showed strikingly symmetric brainstem and cerebellar lesions. The patient's clinical course and the topography and resolution of his MRI abnormalities suggest that this condition is an energy deprivation syndrome.- - - - - - - - - - ranking = 34.690679248946keywords = nervous system (Clic here for more details about this article) |
6/21. Biochemical heterogeneity of infantile central nervous system spongy degeneration.Aspartoacylase, the enzyme whose activity is deficient in infantile central nervous system spongy degeneration (Canavan-Van Bogaert-Bertrand disease), is detected as an approximately 59-kD protein in the Sephadex G-200 filtration of normal fibroblast extracts. The enzyme activity in homogenates of fibroblasts is protected by leupeptin, a protease inhibitor. In the absence of leupeptin, 90% of aspartoacylase activity is lost. In some patients with infantile spongy degeneration, no activity (less than 2%) can be detected. In some other patients with residual activity in fibroblasts, two separate peaks of enzyme are eluted with molecular weight corresponding to approximately 59 and 19 kD. Aspartoacylase activity in this latter group is protected to the same extent by the presence of leupeptin. However, the elution of two peaks is independent of the presence of leupeptin. This study indicates biochemical heterogeneity in the pathogenesis of infantile spongy degeneration.- - - - - - - - - - ranking = 515.46603480292keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/21. The neurological complications of cardiac transplantation.review of the neurological complications encountered in 83 patients who received cardiac homografts over a seven-year period leads to the following conclusions: (1) Neurological disorders are common in transplant recipients, occurring in over 50 per cent of patients. (2) infection was the single most frequent cause of the neurological dysfunction, being responsible for one-third of all CNS complications. (3) The infective organisms were typically those considered to be usually of low pathogenicity: fungi, viruses, protozoa and an uncommon bacterial strain. (4) Other clinical neurological syndromes were related to vascular lesions, often apparently from cerebral ischaemia or infarction occurring during the surgical procedure, metabolic encephalopathies, cerebral microglioma, acute psychotic episodes and back pain from vertebral compression fractures. (5) The infectious complications and probably the development of neoplasms de novo, are related to immunosuppressive therapy which impairs virtually all host defence mechanisms and alters the nature of the host's response to infective agents or other foreign antigens. (6) Because neurological symptoms and signs were usually those of behavioural changes or deterioration in intellectual performance, the neurological examination was often of little value in diagnosing the nature or even the anatomical site of the neuropathological process. (7) The possibility of an infectious origin of the neurological manifestations must be aggressively pursued even in the absence of fever and a significantly abnormal spinal fluid examination. The diagnostic error made most frequently was to ascribe neurological symptoms erroneously to metabolic disturbances or to "intensive care unit psychosis" when they were in fact due to unrecognized CNS infection. (8) maintenance of mean cardiopulmonary bypass pressures above 70 mmHg, particularly in patients with known arteriosclerosis, may reduce operative morbidity. (9) Though increased diagnostic accuracy is possible with routine use of a variety of radiological and laboratory techniques, two further requirements probably must be met before a significant reduction in the frequency of neurological complications will occur: the advent of greater immunospecificity in suppressing rejection of the grafted organ while preserving defences against infection; and a more effective armamentarium of antiviral and antifungal drugs.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/21. Chronic relapsing course of encephalomyeloradiculopathy in a 6-year-old boy.We present a young child with an illness characterized by remissions and exacerbations and signs of disseminated lesions in the central nervous system. Visual evoked response and brainstem auditory evoked potential gave abnormal findings showing lesions also of the optic nerves and brainstem. The CSF IgG index was slightly elevated and myelin basic protein was high. There was also intrathecal antibody production against different viruses, the highest against measles. The HLA type was A3, B7, Dr2. The T4/T8 ratio reflected an immunological active disease. All these signs could have been diagnosed as multiple sclerosis. However, there were also lower motoneuron lesions and metabolic studies showed disturbances in pyruvate metabolism as in Leigh's disease. Cerebral tomography and magnetic resonance imaging showed scattered lesions in the nuclei lentiformis and capsula interna. We would like to stress the importance of careful etiological studies in cases of MS in childhood.- - - - - - - - - - ranking = 103.09320696058keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/21. Central and peripheral nervous system pathology of homocystinuria due to 5,10-methylenetetrahydrofolate reductase deficiency.An autopsy case of homocystinuria due to 5,10-methylenetetrahydrofolate reductase deficiency is presented. A 15-year-old boy had mental retardation, epilepsy, and peripheral neuropathy. A sural nerve biopsy revealed a decreased number of myelinated fibers, abnormally thick myelinated fiber groups, and numerous thinner unmyelinated fibers. The autopsy study revealed unusual findings of peripheral neuropathy and spheroid formation in addition to arterial structural abnormalities and perivascular demyelination which are common in cases of homocystinuria. The peripheral neuropathy and spheroid formation may be related to the low level of serum folic acid. The presence of peripheral neuropathy should be ascertained in cases of 5,10-methylenetetrahydrofolate reductase deficiency.- - - - - - - - - - ranking = 138.76271699578keywords = nervous system (Clic here for more details about this article) |
10/21. Mitochondrial encephalomyopathy. association with an nadh dehydrogenase deficiency.A 17-year-old patient had a progressive hypokinetic-rigid syndrome and several other signs and symptoms that indicated central nervous system involvement. Biochemical studies revealed a reduced form of nicotinamide-adenine dinucleotide dehydrogenase deficiency in skeletal muscle. Clinical signs and symptoms, and their association with an established defect of energy metabolism, led us to classify this disorder as a mitochondrial encephalomyopathy of Leigh's type.- - - - - - - - - - ranking = 103.09320696058keywords = central nervous system, nervous system (Clic here for more details about this article) |
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