1/19. Autologous stem cell transplantation in a case of treatment resistant central nervous system lupus.This case report describes a young woman with systemic lupus erythematosus starting at 16 years of age and giving rise to severe neurological complications including bilateral opticus neuritis and transverse myelitis. Despite heavy immunosuppression her condition steadily aggravated. At this point it was decided to perform autologous stem cell transplantation. Haematopoietic stem cells were mobilised with cyclophosphamide and granulocyte colony stimulating factor. Enrichment of CD34( )cells was followed by depletion of peripheral T and B cells. The post-transplantation course was uneventful, and all the neurological deficits improved promptly during the 15 months of follow up. This is the first description of successful autologous stem cell transplantation in a case of life threatening central nervous system lupus.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/19. Alterations of cerebral glucose metabolism indicate progress to severe morphological brain lesions in neuropsychiatric systemic lupus erythematosus.Neuropsychiatric systemic lupus erythematosus (SLE) is frequently associated with deficits in brain glucose metabolism, even if morphological imaging by magnetic resonance imaging (MRI) shows no abnormalities. In these patients it is unclear whether or not the changes of brain metabolism measured by F-18-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) may progress to lesions of cerebral structure. We describe a 20-year-old woman with SLE who presented with depression, headache and impairment of memory. Initially, a cranial MRI was negative, but FDG-PET revealed significant hypometabolism in the frontal and parieto-temporo-occipital regions on both sides as well as hypermetabolism in the nuclei caudati. Within two months the patient developed an acute confusional state, seizures, visual disturbances and cranial MRI became positive showing hyperintensities at the basal ganglia and the temporo-occipital regions. Focal cerebral symptoms responded to treatment with high dose corticosteroids and brain lesions in MRI disappeared. However, a second FDG-PET showed persistent hypometabolism at frontal regions in accordance with the persistence of subclinical depression. To our knowledge, this is the first SLE case report showing that functional brain lesions visualized by FDG-PET may be a risk factor for subsequent structural brain damage seen in MRI. Thus, FDG-PET may help to verify cerebral involvement of SLE earlier than MRI.- - - - - - - - - - ranking = 0.050197226866348keywords = brain (Clic here for more details about this article) |
3/19. echolalia as a novel manifestation of neuropsychiatric systemic lupus erythematosus."That tongue of yours, by which I have been tricked, shall have its power curtailed and enjoy the briefest use of speech." With these words, Hera, of Greek mythology, deprived the nymph Echo of spontaneous speech, constraining her instead to merely repeating the words of others. echolalia, which derives from the word "echo," is disordered speech in which an individual persistently repeats what is heard. echolalia has been described in patients with a number of neuropsychiatric illnesses including autism and Tourette's syndrome. Neuropsychiatric systemic lupus erythematosus (NPSLE) is a heterogeneous disease with protean manifestations that may occur in approximately 25% to 50% of patients with systemic lupus erythematosus (SLE). Although the most common manifestations include cognitive dysfunction (50%) and seizures (20%), NPSLE may also present as peripheral neuropathy (15%), psychosis (10%), or other central nervous system abnormalities. We report the case of a 57-year-old woman with SLE and echolalia.- - - - - - - - - - ranking = 0.2keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/19. Selective involvement of the choroid plexus on cerebral magnetic resonance images: a new radiological sign in patients with systemic lupus erythematosus with neurological symptoms.The selective involvement of the choroid plexus on brain magnetic resonance (MR) images is described in 2 patients with systemic lupus erythematosus presenting with neurological symptoms. The decrease in choroid plexus abnormalities on followup MR examination paralleled the clinical recovery with glucocorticoid therapy in both patients. Our cases indicate that selective involvement of the choroid plexus should be included in the spectrum of the radiological signs for neurological lupus.- - - - - - - - - - ranking = 0.0055774696518165keywords = brain (Clic here for more details about this article) |
5/19. Reversible parkinsonism in systemic lupus erythematosus.Parkinsonism as a manifestation of central nervous system (CNS) lupus is extremely rare. We report the first patient with systemic lupus erythematosus (SLE) who developed a reversible parkinsonian syndrome associated with enhancing subcortical lesions on magnetic resonance imaging (MRI). Following treatment with prednisolone and cyclophosphamide, her bradyphrenia, bradykinesia, hypophonia, rigidity, and abnormal gait progressively improved. Three months after she commenced treatment, repeat MRI scanning demonstrated resolution of the abnormal subcortical white matter enhancement. Our case illustrates unusual clinico-radiologic correlates of reversible parkinsonism in a SLE patient; these findings suggest that disruption of the subcortical frontal pathways may be a possible pathophysiologic mechanism for parkinsonism in cerebral lupus.- - - - - - - - - - ranking = 0.2keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/19. Sustained normalization of cerebral blood-flow after iloprost therapy in a patient with neuropsychiatric systemic lupus erythematosus.We report the case of a 30-year-old caucasian woman affected by SLE who developed neurological symptoms (prosopagnosia and visual-spatial agnosia) after nine years of disease. brain MRI showed no abnormalities while a brain SPECT scan showed diffuse uptake defects and hypoperfusion areas in the right and left frontal-parietal regions. At that time the patient was on hydroxychloroquine (400 mg/day) and oral prednisolone (0.5 mg/kg/day) as maintenance therapy. One year later the patient showed worsening of Raynaud's phenomenon with digital dystrophic lesions and was therefore treated with an intravenous infusion of iloprost (1.5 ng/kg/min per 6h/day for 10 days consecutively), while baseline treatment remained unchanged. One month later the patient showed a dramatic improvement in her cognitive function and subsequent SPECT scans showed the gradual disappearance of perfusion abnormalities. This first report of iloprost treatment in CNS lupus suggests the potential therapeutic usefulness of this drug in patients with SLE and functional CNS involvement.- - - - - - - - - - ranking = 0.0055774696518165keywords = brain (Clic here for more details about this article) |
7/19. Reversible parkinsonian syndrome in systemic and brain vasculitis.A young female patient with chronic renal failure due to a systemic vasculitis and a parkinsonian syndrome secondary to brain vasculitis, most likely systemic lupus erythematosus, is described. The patient had a dramatic response to a pulse of methylprednisolone, with remission of her parkinsonian symptoms.- - - - - - - - - - ranking = 0.027887348259082keywords = brain (Clic here for more details about this article) |
8/19. Lupus cerebritis: a case study.Nervous system involvement in systemic lupus erythematosus (SLE) occurs in 24%-50% of all patients in the united states at some time during the course of their illness. Lupus cerebritis with associated headache, seizures, stroke, and chorea is just one of a wide array of central nervous system disorders SLE patients can develop. It also is one of the most difficult manifestations of lupus to diagnose. Advances in imaging and laboratory analysis have contributed to an earlier and more specific diagnosis of lupus cerebritis. Despite improvements in the ability to treat SLE, management of nervous system manifestations remains unsatisfactory. Controversy exists as to the best approach for treatment. Newer combination therapies based on anecdotal evidence are suggested.- - - - - - - - - - ranking = 0.24962971048905keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/19. central nervous system lupus and pregnancy: 11-year experience at a single center.OBJECTIVE: To describe the pregnancy outcomes in women with central nervous system (CNS) manifestations of lupus. methods: Between 1991 and 2002, the outcome of five pregnancies in four patients with CNS lupus were retrospectively reviewed. All patients had an established history of systemic lupus erythematosus (SLE), and either a history of CNS lupus or active CNS lupus. pregnancy outcomes assessed included term and preterm birth, intrauterine growth restriction, abnormal antepartum testing, perinatal mortality, pre-eclampsia and other maternal morbidities. RESULTS: Evidence of active CNS lupus symptoms developed in three of the five pregnancies. Two pregnancies were complicated by early onset pre-eclampsia, abnormal antepartum testing and extreme prematurity, with one subsequent neonatal death. The remaining three pregnancies had good neonatal outcomes, but were complicated by severe maternal post-pregnancy exacerbations, and the eventual death of one patient. CONCLUSIONS: CNS lupus in pregnancy represents an especially severe manifestation of SLE, and may involve great maternal and fetal risks.- - - - - - - - - - ranking = 0.39851884195622keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/19. Juvenile parkinsonism as a manifestation of systemic lupus erythematosus: case report and review of the literature.Involvement of the central nervous system in systemic lupus erythematosus (SLE) has been well described. It usually includes psychiatric disturbance, seizures, and cranial nerve disorders. movement disorders are less common, chorea being the one most frequently described. A parkinsonian syndrome may be an extremely rare manifestation of cerebral lupus. We report on a case of juvenile parkinsonism as a manifestation of SLE and review the literature.- - - - - - - - - - ranking = 0.2keywords = central nervous system, nervous system (Clic here for more details about this article) |
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