1/12. 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) |
2/12. 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) |
3/12. 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) |
4/12. 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) |
5/12. 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) |
6/12. 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) |
7/12. central nervous system involvement in neonatal lupus erythematosus.Computerized tomography (CT) of the brain was performed in 10 of 11 consecutive infants with neonatal lupus erythematosus (NLE) (five boys and six girls). Ten of the 11 infants had brain neurosonography. Nine of 10 infants had abnormal CT scans. There was diffuse, markedly reduced attenuation of the cerebral white matter in four infants studied in the first week of life, and also in an infant 5 weeks of age. Patchy reduced subcortical white matter attenuation was observed in another 5-week-old infant. basal ganglia calcifications were present in two infants at 2 months of age, one of whom also had mild ventriculomegaly. A patient with macrocephaly studied at 4 months of age had enlarged ventricles and subarachnoid spaces consistent with benign macrocephaly of infancy. Cerebral ultrasound examination was abnormal in all five infants studied in the first week of life and in one infant at 2 months of age. Findings included subependymal cysts (4), echogenic white matter (3), and echogenic lenticulostriate vessels (3). Apart from one case of macrocephaly, there was no clinical evidence of neurologic disease and the subsequent development of these infants has been normal. Subclinical central nervous system (CNS) disease in NLE is likely to be a transient phenomenon that resolves as maternal antibodies are cleared from the infant's circulation. It is important to be aware of these neuroimaging abnormalities to avoid misdiagnosis of congenital viral infection in a newborn with multisystem NLE. The potential for neurologic sequelae is uncertain.- - - - - - - - - - ranking = 0.39851884195622keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/12. A neuroimaging follow up study of a patient with juvenile central nervous system systemic lupus erythematosus.BACKGROUND: The course of central nervous system systemic lupus erythematosus (CNS-SLE) is largely unknown. New imaging techniques are available to assist in monitoring the disease course. OBJECTIVE: To report a case of juvenile CNS-SLE, in which magnetic resonance imaging (MRI) was used to assess disease activity. CASE REPORT: A 10-year-old female patient with SLE presented with convulsions; MRI and computed tomography (CT) of the cerebrum disclosed abnormalities. Despite adequate treatment, two years later she had a generalised convulsion, and MRI showed new lesions. MR spectroscopy (MRS) indicated neuronal loss, inflammation, and metabolically compromised tissue; magnetisation transfer imaging (MTI) showed an increase in whole brain lesion load. After exclusion of a malignancy, CNS-SLE was the most likely diagnosis, and cyclophosphamide pulses were administered. Initially, multiple sclerosis (MS)-like lesions regressed, but despite maximal immunosuppressive drugs, new lesions formed and disappeared. When immunosuppressive drugs had been stopped for six months MRI showed improved lesions and MTI histograms. DISCUSSION: In this case report, the anatomical substrate, metabolic aspect, neuroimaging, and clinical course of MS-like lesions in a child with CNS-SLE are described. The way in which radiological techniques can support clinical decision making in this young patient with progressive CNS-SLE is illustrated.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/12. Leukoencephalopathy and chronic pancreatitis as concomitant manifestations of systemic lupus erythematosus related to anticardiolipin antibodies.Symptoms of leukoencephalopathy led to hospital admission of a 59-year-old woman. In addition, a tumor of unknown nature in the pancreas was identified by abdominal ultrasound and CT scan. Following explorative laparotomy and pancreas tail resection, histopathologic analysis revealed a pancreatic pseudotumor with chronic fibrotic pancreatitis. Systemic lupus erythematosus (SLE) was diagnosed due to the presence of antinuclear antibodies (ANA) in serum, antiphospholipid antibodies, and involvement of the central nervous system. Leukoencephalopathy related to anticardiolipin antibodies in serum is a known but rare manifestation of SLE. The concomitant occurrence of chronic pancreatitis can be caused by the development of SLE-induced vasculitis in the pancreas. Subsequent complications of pancreatitis are responsible for the critical, life-threatening state of these patients and may be prevented by early identification of anticardiolipin antibodies and therapy for SLE.- - - - - - - - - - ranking = 0.2keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/12. renal artery thrombosis in a pediatric case of systemic lupus erythematosus without antiphospholipid antibodies.A 13-year-old Japanese boy with an 11-month history of systemic lupus erythematosus (SLE) without antiphospholipid antibodies (APAs) suddenly developed severe hypertension, associated with fever and generalized seizures, and mild abdominal pain. Emergency abdominal computed tomography (CT) confirmed left renal artery thrombosis, and a renal scintiscan revealed reduced blood flow to the left kidney. Promptly instituted intravenous anticoagulant therapy was not effective for controlling the infarction-reduced renal arterial blood supply. Moreover, he developed stupor due to central nervous system (CNS) lupus a week after the occurrence of the hypertensive episode. Finally, a percutaneous transluminal angioplasty successfully relieved the occlusion of the left renal artery at its origin. The CNS lupus was also successfully treated with intravenous methylprednisolone pulse therapy combined with intrathecal methotrexate and dexamethasone. Although it is well known that SLE patients with APAs have a high incidence of thrombotic complications, to date, renal artery thrombosis has rarely been reported in young patients. This APA-negative SLE patient unusually manifested renal thrombosis associated with CNS lupus.- - - - - - - - - - ranking = 0.2keywords = central nervous system, nervous system (Clic here for more details about this article) |
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