1/19. infection with jc virus and possible dysplastic ganglion-like transformation of the cerebral cortical neurons in a case of progressive multifocal leukoencephalopathy.infection of the cerebral cortical neurons with jc virus (JCV) with possible dysplastic ganglion-like alteration of the infected neurons found in a case of progressive multifocal leukoencephalopathy (PML) is described. The patient was a 21-year-old man with common variable immunodeficiency who died of PML after a 9-month clinical course. At autopsy, the white matter of the cerebrum, brainstem, cerebellum, and spinal cord exhibited extensive demyelination and necrosis. Numerous inclusion-bearing oligodendrocytes and bizarre astrocytes were found. In the occipital and temporal cortex, thick band-like aggregates of dysplastic ganglion-like cells (DGLCs) were found. These DGLCs showed immunohistochemical properties of neurons, and nuclei of some DGLCs were immunoreactive for large T antigen of SV40/JCV and p53, but not for capsid protein JCV VP1. in situ hybridization for mRNA of JCV large T antigen revealed positive signals in the nuclei of some DGLCs. These results indicate that JCV infected neurons and it is suggested that binding of the large T antigen with cellular proteins could have resulted in the dysplastic, ganglion cell-like change of the infected neurons, although the possibility that the aggregates of DGLCs represent a pre-existent malformative lesion of the cortex cannot be excluded completely.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/19. Brain involvement in haemolytic-uraemic syndrome: MRI features of coagulative necrosis.We describe radiological demonstration of brain involvement in haemolytic-uraemic syndrome (HUS) in two siblings with a very different clinical course. While the brother presented with a mild, reversible encephalopathy, his sister developed high-signal lesions in the cortex, putamen and caudate nucleus on T1-weighted images, seen as dense areas on CT. biopsy revealed coagulative necrosis due to microthrombosis without haemorrhage, calcification or infection. These findings suggest a possible prognostic role for MRI in cases of encephalopathy due to HUS.- - - - - - - - - - ranking = 0.024281829004192keywords = nucleus (Clic here for more details about this article) |
3/19. Chronic granulomatous neuritis in idiopathic trigeminal sensory neuropathy. Report of two cases.Idiopathic trigeminal sensory neuropathy is a clinically benign disorder in which the main feature is facial numbness limited to the territory of one or more divisions of the trigeminal nerve; the disorder persists for a few weeks to several years. and no underlying disease can be identified. Magnetic resonance (MR) imaging findings are occasionally consistent with a small trigeminal neuroma of the left gasserian ganglion associated with idiopathic trigeminal sensory neuropathy. The authors report on two patients who were treated using a skull base approach in which the gasserian ganglion was exposed and the lesion was removed. The pathological diagnosis was chronic granulomatous neuritis. The authors conclude that, in patients with MR findings suggestive of a small trigeminal neuroma, benign idiopathic trigeminal sensory neuropathy should also be considered in the differential diagnosis. A conservative approach featuring sequential MR imaging studies may avoid an unnecessary surgical exploration.- - - - - - - - - - ranking = 0.28571428571429keywords = ganglion (Clic here for more details about this article) |
4/19. Familial infantile bilateral striatal necrosis: clinical features and response to biotin treatment.BACKGROUND: Infantile bilateral striatal necrosis (IBSN) encompasses several syndromes of bilateral symmetric, spongy degeneration of the caudate nucleus, putamen, and globus pallidus. The familial form of IBSN is rare, and inheritance is either autosomal recessive or maternal. METHOD: The authors describe an Israeli Bedouin kindred in which 15 children born to consanguineous parents were affected with familial IBSN. They evaluated the clinical and radiologic evolution of the disease in 11 patients and the cerebral pathologic findings in one patient. Three of the children were treated with oral biotin 100 mg/day. RESULTS: Inheritance was apparently autosomal recessive. The untreated children had a similar clinical picture including developmental arrest beginning at the age of 7 to 15 months, choreoathetosis, and dysphagia. Pendular nystagmus appeared at a late stage. MRI, performed at various stages of the disease, showed severe basal ganglia atrophy. Postmortem study in one patient showed severe atrophy of the lenticular nuclei with gliosis and loss of neurons. biotin, 100 mg/day, administered to the proband over a period of 15 months, may have slowed progression. In two other children treatment was initiated earlier and appeared to arrest or improve disease. CONCLUSIONS: Familial infantile bilateral striatal necrosis was inherited as an autosomal recessive trait. Clinical features included developmental arrest, dysphagia, and choreoathetosis. Imaging and pathology showed atrophy and degeneration of the basal ganglia. Oral biotin may have benefited three children.- - - - - - - - - - ranking = 0.024281829004192keywords = nucleus (Clic here for more details about this article) |
5/19. Desmoplastic infantile ganglioglioma: a rare tumor with an unusual presentation.Desmoplastic infantile ganglioglioma (world health organization grade I) is a rare neoplasm. Despite their common large size and spectacular radiologic and histologic features, the prognosis after surgical resection is good. We present a new case of this tumor in a 14-month-old boy with a recent history of intracranial hypertension. magnetic resonance imaging revealed a large tumor involving the left collateral trigone with dilatation of the lateral ventricles. Surgery revealed two separate solid tumors: one in the left falco-tentorial region and the other in the left rolandic area. Microscopic examination showed a proliferation of neoplastic astrocytes in reticulin-rich desmoplastic stroma associated with scattered ganglion cells. One year after surgery follow-up magnetic resonance imaging did not show tumor progression.- - - - - - - - - - ranking = 0.14285714285714keywords = ganglion (Clic here for more details about this article) |
6/19. Possible evidence for secondary degeneration of central nervous system in the pathogenesis of anencephaly and brain dysraphia. A study in young human fetuses.In an attempt to help elucidate pathogenetically those human cases exemplifying secondary degeneration of the neural tube causing brain dysraphia, macroscopic and histologic observations of two young human fetuses are described. A nine-week-old anencephalic fetus exhibited an absence of spinal cord (amyelia) with retention of neural crest derivatives (dorsal root ganglion cells and thusir processes, and sympathetic ganglia) implying the presence of a neural tube in early gestation. The second, ten-week-old exencephalic case exhibited restricted brain hemorrhage and necrosis of the telencephalon and brain stem amongst otherwise normal brain and spinal cord tissue. These two young fetal cases may represent examples of a previously normal neural tube which has undergone degeneration at a stage where neural crest has already undergone differentiation, and thus distinguishes them from cases of complete dysraphism which probably results from primary degeneration during neurulation.- - - - - - - - - - ranking = 0.14285714285714keywords = ganglion (Clic here for more details about this article) |
7/19. Gastric hepatoid adenocarcinoma with autophagy-related necrosis-like tumor cell death: report of a case.A case of hepatoid adenocarcinoma of the stomach is presented. The characteristic features of the tumor are summarized on the basis of the authors' experience and the literature. Ultrastructural examination revealed patchy condensations of chromatin throughout the nucleus suggestive of necrosis-like programmed cell death (PCD). These nuclear alterations were associated with the occurrence of vacuoles and lipofuscins, conferring an autophagic phenotype to this PCD. Thus, the case reported here provides an example of autophagic-related necrosis-like PCD. Alternative PCDs are reviewed and their morphologic distinction is discussed.- - - - - - - - - - ranking = 0.024281829004192keywords = nucleus (Clic here for more details about this article) |
8/19. Necrotizing retinopathy with herpes zoster ophthalmicus: a light and electron microscopical study.A necrotizing retinopathy following a vesicular cutaneous eruption in the distribution of the right trigeminal nerve developed in a patient who had been receiving systemic corticosteroid therapy one week prior to the onset of herpes zoster ophthalmicus. Seven weeks after the herpetic symptoms began, the patient died of pneumonia following an intracerebral hematoma. At postmortem examination, unexpected multiple focal and confluent lesions, which corresponded to areas of extensive retinal necrosis, were observed in the fundus of the right eye. Intranuclear inclusions with a perinuclear halo were identified within the affected sensory retina. Electron microscopy of the retinal lesions disclosed round to oval enveloped viral particles that were characteristic of the herpes viruses. A mild lymphocytic infiltrate was evident in a demyelinated right Gasserian ganglion. Demyelination and necrosis of the right trigeminal sensory tract and adjacent areas were evident within the brain stem.- - - - - - - - - - ranking = 0.14285714285714keywords = ganglion (Clic here for more details about this article) |
9/19. Hereditary spastic dystonia: a new mitochondrial encephalopathy? Putaminal necrosis as a diagnostic sign.A large kindred, in which either Leber's hereditary optic atrophy, or a hereditary spastic dystonia, or a combination of both manifested over many generations was restudied after the first report on it in 1964. NMR scans revealed bilateral, and, in two patients with hemidystonia, unilateral necrosis with shrinkage of the putamen, in one case associated with total disappearance of the head of the caudate nucleus. Except for age-appropriate cortical atrophy in one instance, no other changes were observed in the brain, brainstem, and cerebellum. The putaminal necrosis appears as typical "striatal slits" on the NMR scans. It is argued that this rare disease, since the princeps description in 1964 only reported in england (1986) and the U.S.A (1986), is most likely a singular type of mitochondrial encephalopathy: it is associated with Leber's optic atrophy, and the NMR changes observed have been signalled in other mitochondrial encephalomyelopathies, such as Leigh's disease and MELAS.- - - - - - - - - - ranking = 0.024281829004192keywords = nucleus (Clic here for more details about this article) |
10/19. herpes simplex retinitis.Fatal encephalitis with accompanying retinitis developed in a previously healthy 18-month-old infant. Clinically the disease appeared as whitish-yellow punctate lesions, perivascular cuffing, and hemorrhage. The antibody titer to herpes simplex rose from 1:8 on the day of admission to 1:256 on the day of death. Postmortem, intranuclear inclusion bodies that were typical of those found with herpesvirus were seen in the brain and retina. Viral particles consistent with those of herpesvirus were found by electron microscopy in the brain and in the inner-nuclear and ganglion-nerve fiber layers of the retina. This demonstrates the direct infectious nature of herpetic retinitis. Hematogenous spread of the virus to the retina is presumed.- - - - - - - - - - ranking = 0.14285714285714keywords = ganglion (Clic here for more details about this article) |
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