1/12. Facial and skeletal malformations, mental retardation, aganglionosis, and neurogenic muscle weakness: a variant of Niikawa-Kuroki syndrome or a new syndrome?We report a 10-year-old boy with multiple congenital anomalies/mental retardation syndrome, who also presented with aganglionosis and neurogenic muscle weakness. Some phenotypic manifestations of our patient overlap with those observed in the Niikawa-Kuroki syndrome; however, the hypothesis of a new distinct entity, with simultaneous involvement of the central and peripheral nervous system, is considered.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/12. Eales' disease with neurological manifestations.Eales' disease is a primary retinal perivasculitis of an undetermined etiology seen predominantly in the Indian subcontinent. However, neurological involvement is rare. We report here a patient of retinal perivasculitis with neurological dysfunction. Our patient is a 39 years male who developed acute diminished vision right eye in March 99, which progressed for four days and remained static. In April 99 he developed acute diminished vision left eye, which progressed to near total blindness in 48 hours. He was undergoing ophthalmic evaluation. Fourty five days later he developed incoordination and weakness left half of body. The examination revealed bilateral retinal perivasculitis with pyramidal signs and left sided cerebellar signs. Investigations revealed an ESR of 40 mm at the end of first hour. His CT head revealed bilateral basal ganglionic infarcts. MRI head revealed enhancing lesions both basal ganglia and right parietal region. cerebrospinal fluid examination showed xanthochromic fluid with markedly elevated protein and lymphocytic pleocytosis. His workup for connective tissue disorders was negative. He was put on ATT with steroids. Eales' disease is presumed allergy to tuberculoprotein. A trial of ATT with steroids has been tried with varying results. Our patient had bilateral retinal perivasculitis and neurological dysfunction. He had lymphocytic pleocytosis with markedly elevated protein in the CSF.- - - - - - - - - - ranking = 0.4348526549183keywords = basal ganglion, ganglion (Clic here for more details about this article) |
3/12. MR imaging findings of neurosarcoidosis of the gasserian ganglion: an unusual presentation.We report the MR imaging findings of an unusual case of neurosarcoidosis of the gasserian ganglion associated with trigeminal neuralgia. No other neurological or extraneurological localization was found. magnetic resonance imaging demonstrated a mass in the Meckel's diverticulum that was isointense on T1-weighted images and hypointense on T2-weighted images. gadolinium-enhanced MR imaging showed heterogeneous enhancement. Although rare, sarcoid infiltration of the gasserian ganglion must be considered in the differential diagnosis of an isolated mass in this localization in patients with trigeminal neuralgia.- - - - - - - - - - ranking = 1.2keywords = ganglion (Clic here for more details about this article) |
4/12. Fine specificities of antibodies in sera and cerebrospinal fluid in herpes virus infections of the central nervous system as detected by the antigen variable immunoblot technique.Applying the immunoblot technique a sensitive and specific method was developed for the detection of intrathecally synthesized antibodies against individual specific proteins that are antigens of various infectious agents causing encephalitis. Paired serum and cerebrospinal fluid (CSF) samples from five patients with herpes virus infections of the central nervous system (CNS) (three herpes simplex virus encephalitis, one varicella zoster virus encephalitis, one zoster ganglionitis) were investigated for the presence of locally produced IgG against the electrophoretically separated antigens of herpes simplex virus (HSV), varicella zoster virus (VZV) and human cytomegalovirus (HCMV), as well as for IgM antibodies in one case of HSV encephalitis. In two cases (HSV encephalitis and VZV encephalitis) four and one antibody, respectively, were found that were synthesized intrathecally only. In the other cases the patterns of sera and CSF antibodies were similar, the CSF antibodies showing an all-over stronger reaction, at identical IgG concentrations. In contrast to the conception of a 'limited heterogeneity' of intrathecal antibody synthesis in encephalitis, we thus found an 'expanded heterogeneity' of the intrathecally synthesized antibodies in comparison to the corresponding serum antibodies.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
5/12. histiocytosis X in adult with skin and uncommon central nervous system involvement.Report of a case of histiocytosis X with adult onset, widespread skin lesions and rare central nervous system involvement resulting in dizziness and ataxia. On autopsy histiocytosis X infiltrates in the central nervous system were demonstrated with granuloma formation in the temporal lobe and nucleus dentatus of the cerebellum.- - - - - - - - - - ranking = 0.0096312191857243keywords = nucleus (Clic here for more details about this article) |
6/12. Idiopathic orthostatic hypotension and the Shy and Drager syndrome; Physiological studies in four cases; pathological report of one case.Five cases of idiopathic orthostatic hypotension are presented. Physiological tests were performed in four cases which demonstrated that the lesion in the sympathetic system was most probably situated in the intermediolateral columns in three cases. In one case the lesion was localized on the efferent pathway either at the preganglionic or the postganglionic level. These four patients had also evidence of parasympathetic dysfunction. In the fifth case the clinical diagnosis of Shy and Drager syndrome was confirmed by pathological examination of the nervous system which revealed intermediolateral cell loss and the pathological findings commonly observed in the striato-nigral and olivo-ponto-cerebellar degenerations.- - - - - - - - - - ranking = 0.4keywords = ganglion (Clic here for more details about this article) |
7/12. Ocular histopathologic and biochemical studies of the cerebrohepatorenal syndrome (Zellweger's syndrome) and its relationship to neonatal adrenoleukodystrophy.The eyes of three infants with cerebrohepatorenal disease (Zellweger's syndrome) who died demonstrated ganglion cell loss, gliosis of the nerve fiber layer and optic nerve, optic atrophy, and changes resembling those of retinitis pigmentosa in the retina and pigment epithelium. Ultrastructural examination showed bileaflet inclusions identical to those seen in neonatal adrenoleukodystrophy in the pigment epithelium and in pigmented macrophages, but these were absent in the cornea. Biochemical analysis of tissues demonstrated an excessive amount of very-long-chain fatty acids in the ocular tissues, an abnormality also found in adrenoleukodystrophy. These histopathologic and biochemical results demonstrated that the cerebrohepatorenal syndrome and neonatal adrenoleukodystrophy are similar in regard to ocular abnormalities and the presence of saturated very-long-chain fatty acids.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
8/12. Pain due to lesions of central nervous system removed by sympathetic block.Eight patients were studied in whom a lesion within the central nervous system caused constant pain and hyperpathia. Blockade of the sympathetic supply to the periphery was carried out in each patient by stellate ganglion block or intravenous infusion of guanethidine 15 mg in 30 ml saline into a limb on the affected side. On almost every occasion the pain and hypersensitivity were reduced, sometimes completely. Thus chronic pain and hyperpathia arising from a lesion in the central nervous system may be abolished by blocking the sympathetic supply to the periphery; this effect may be achieved when not all the peripheral nerves of the affected region have had their sympathetic nerve supply blocked. Such blockade may be worth repeating in the hope of achieving lasting relief of the intractable pain.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
9/12. Clinical and neuropathological aspects of long-term damage to the central nervous system after lithium medication.A female patient, who died at the age of 61 and had suffered from several manic-depressive psychoses for more than 30 years, developed three phases of intoxication under lithium therapy. There was a 15-year history of electro- and Pentetrazol-induced convulsive therapy prior to lithium medication; neuroleptics were still administered during lithium therapy. The last lithium intoxication, 3 years prior to death was during a low-dosage therapy with normal lithium levels followed by severe lasting impairment: akinesia, rigidity, dysarthria, ataxia, and an organic alteration in character. For the first time, neuropathological findings could be established in such a case: extensive damage to granule and purkinje cells in the cerebellum; gliosis in the dentate nucleus, the inferior olives, and the nucleus ruber; cytoplasmic inclusions in various nerve cells of the cranial nerve nuclei; cytoplasmic vacuoles, especially in the cells of the supra-optic nucleus. Surprisingly little damage could be found in the substantia nigra and in the neostriatum. The clinical course as well as the pattern and intensity of the brain damage oppose an interpretation as a consequence of preceding convulsive shock therapy.- - - - - - - - - - ranking = 0.028893657557173keywords = nucleus (Clic here for more details about this article) |
10/12. neuroblastoma presenting as central nervous system disease.neuroblastoma may be extremely difficult to recognize, particularly when the tumor presents as a primary central nervous system disease. central nervous system involvement may be considered as primary intracerebral neuroblastoma, metastases to the cranium from an occult primary, primary intraorbital neuroblastoma originating in the ciliary ganglion, metastatic intraorbital neuroblastoma from an occult primary, primary intraspinal neuroblastoma originating in dorsal root ganglia, intraspinal metastatic disease, and distant effects such as myoclonic encephalopathy. Primary neuroblastoma within the ciliary ganglion and primary intraspinal neuroblastoma are extremely rare entities. Illustrative cases the demonstrate the broad spectrum of neurologic presentations are offered. The second known report of neuroblastoma in association with primary pulmonary hypoventilation (Ondine curse) is included.- - - - - - - - - - ranking = 0.4keywords = ganglion (Clic here for more details about this article) |
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