11/18. 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 = nervous system (Clic here for more details about this article) |
12/18. 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 = nervous system (Clic here for more details about this article) |
13/18. A case of neuropsychiatric lupus with myelopathy successfully treated with corticosteroids.This report describes a 16-year old female patient who presented with acute paresis in both lower limbs, acute urinary retention, blurred vision and arthritis. The patient was diagnosed as having systemic lupus erythematosus with myelitis and bilateral abducent nerves palsy. The administration of steroids resulted in marked improvement in her neurological symptoms.- - - - - - - - - - ranking = 0.00011517213810032keywords = neurologic (Clic here for more details about this article) |
14/18. 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 = nervous system (Clic here for more details about this article) |
15/18. Overwhelming leukoencephalopathy as the only sign of neuropsychiatric lupus.We describe a patient with diffuse leukoencephalopathy, a rare central nervous system complication of systemic lupus erythematosus, who died of brain herniation despite aggressive management. brain magnetic resonance imaging revealed diffuse white matter hyperintensities consistent with vasogenic edema. autopsy revealed only widespread cerebral edema. Early recognition and persistent, aggressive treatment will be required to avoid this fatal and rare manifestation of neuropsychiatric lupus.- - - - - - - - - - ranking = 0.2keywords = nervous system (Clic here for more details about this article) |
16/18. Systemic lupus erythematosus with organic brain syndrome: serial electroencephalograms accurately evaluate therapeutic efficacy.A 48-year-old man with systemic lupus erythematosus developed organic brain syndrome. High-dose prednisolone was ineffective, and somnolence without focal signs rapidly developed. Electroencephalogram (EEG) demonstrated a slow basic rhythm (3 Hz), but brain magnetic resonance imaging was normal. Somnolence resolved soon after performing plasma exchange (two sessions). However, memory dysfunction persisted, with EEG demonstrating mild abnormalities (7-8 Hz basic rhythm). Double-filtration plasmapheresis (three sessions) was done, followed by intravenous cyclophosphamide. Immediately after the first plasmapheresis session, memory dysfunction began to improve. After the second dose of cyclophosphamide, intellectual function resolved completely and EEG findings also normalized (basic rhythm of 10 Hz waves). Serial EEG findings precisely reflected the neurological condition and therapeutic efficacy in this patient. In contrast, protein levels in cerebrospinal fluid remained high and did not seem to appropriately reflect the neurological condition in this patient.- - - - - - - - - - ranking = 0.00023034427620063keywords = neurologic (Clic here for more details about this article) |
17/18. magnetic resonance imaging abnormalities in the corpus callosum of a patient with neuropsychiatric lupus.BACKGROUND: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder which can present with a variety of neurologic and psychiatric complications. Currently, there are no consensus neuroimaging findings associated with neuropsychiatric SLE. CASE SUMMARY: We describe the unusual neuroimaging findings in a patient with SLE and a history of epilepsy who presented to a tertiary care emergency department with features of neuropsychiatric lupus, as well as recent seizures. magnetic resonance imaging of the brain revealed an isolated lesion with T2 signal hyperintensity and restricted diffusion in the midline splenium of the corpus callosum. CONCLUSIONS: While establishing the precise etiology underlying the clinical and radiographic observations in this case is difficult, several possibilities are discussed, including acute ischemia and epileptic sequelae. The location of the patient's lesion in a region potentially associated with pure psychotic disorders raises the possibility that such a lesion may be sufficient to produce acute behavior changes and psychotic features in certain patients.- - - - - - - - - - ranking = 0.00011517213810032keywords = neurologic (Clic here for more details about this article) |
18/18. diagnosis and treatment of vasculitis of the central nervous system in a patient with systemic lupus erythematosus.BACKGROUND: A 23-year-old white woman with a 3-year history of systemic lupus erythematosus and a 15-month history of lupus nephritis and retinal vasculitis was successfully treated with antibiotics for pseudomonas aeruginosa pneumonia while on moderate doses of corticosteroids. Even though her pneumonia had improved, she developed acute changes in her mental status that rapidly progressed to encephalopathy with coma. INVESTIGATIONS: physical examination, fundoscopic examination, laboratory tests for metabolic abnormalities, cerebrospinal fluid analysis, microbiology and serologic testing, electroencephalogram, tests for IgM and IgG anticardiolipin antibodies, neuroimaging including CT of the brain and T1-weighted MRI before and after gadolinium contrast, and flow-attenuated inversion recovery MRI. diagnosis: Vasculitis of the central nervous system associated with systemic lupus erythematosus. MANAGEMENT: Intravenous methylprednisolone 1,000 mg/day for 3 days, one dose of intravenous pulse cyclophosphamide 750 mg/m(2), intravenous immunoglobulin 400 mg/kg/day for 4 days, plasmapheresis on alternate days for five cycles, and prednisone 40 mg/day. She continued monthly doses of intravenous pulse cyclophosphamide and intravenous pulse methylprednisolone for 6 months, followed by maintenance infusions every 3 months over 2 years. prednisone was tapered over 18 months. cyclophosphamide was discontinued after 2 years because of poor bone-marrow tolerance, and was replaced with mycophenolate mofetil 3,000 mg/ day and ciclosporin 50 mg twice daily.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
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