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1/35. 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. ( info)

2/35. intestinal pseudo-obstruction in systemic lupus erythematosus: an uncommon but important clinical manifestation.

    OBJECTIVES: To document intestinal pseudo-obstruction (IpsO) as a recognised clinical manifestation of systemic lupus erythematosus (SLE) and a possible new clinical entity with its apparent association with ureterohydronephrosis. METHODOLOGY: We report six lupus patients who presented with IpsO and review 12 other cases from an English literature search. IpsO is defined as the presence of clinical features suggestive of intestinal obstruction but without organic obstruction, namely absence of bowel sounds, presence of multiple fluid levels on plain abdominal x-rays and exclusion of organic obstruction by imaging or surgical procedure. Other clinical characteristics related to the underlying lupus, serological and histological findings, treatment modalities and outcomes of these patients were reviewed. RESULTS: All 18 patients fulfilled the ACR revised classification criteria for SLE. None showed any clinical features of scleroderma or overlap syndrome. The mean age of onset of IpsO was 29.0 (15-47) y. The female to male ratio was 16:2. Nine patients had IpsO as the initial presentation of their underlying lupus. Coexisting lupus involvement of other organ systems included glomerulonephritis (n=7), thrombocytopenia (n=5) and cerebral lupus (n=3). The serology data and autoantibody profile of some of the previously reported patients were incomplete. In our series, anti-Ro antibody was positive in 5/6 while anti-RNP was found in 1/6 patients only. All our patients had active lupus serology at presentation. 17/18 patients required the use of high dose systemic corticosteroid therapy while one patient responded to topical adrenocorticotrophin hormone treatment. Response was good and was observed early after commencement. azathioprine was used as maintenance therapy in 6/18 patients with good effects. An apparent association with the presence of bilateral ureterohydronephrosis was found in 12/18 patients. These patients presented with dysuria without positive bacterial culture though features of chronic interstitial cystitis were not invariably found in these patients. CONCLUSION: IpsO is an uncommon but important manifestation of SLE. The underlying pathology is not fully understood but it may be related to immune complex deposition. The finding of coexisting ureterohydronephrosis suggests that there may also be a central smooth muscle motility problem of neuropathic or myogenic pathophysiology which may or may not be secondary to vasculitis. Early recognition and treatment of IpsO in SLE is important. ( info)

3/35. Red ear(s) syndrome associated with child neuropsychiatric systemic lupus erythematosus.

    This case illustrates that a child having severe neuropsychiatric systemic lupus erythematosus (NPSLE) with seizure and cerebral vascular disease showed excellent clinical outcome in response to intravenous methylprednisolone and cyclophosphamide pulse, and presented unexplained red ears phenomenon. ( info)

4/35. 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. ( info)

5/35. 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. ( info)

6/35. 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. ( info)

7/35. 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. ( info)

8/35. Recurrent pseudotumor cerebri in systemic lupus erythematosus: a case report.

    pseudotumor cerebri is an uncommon manifestation of neuropsychiatric systemic lupus erythematosus (SLE), and is characterized by an elevated intracranial pressure, papilledema with occasional abducens nerve paresis, absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. pseudotumor cerebri has been reported in a few sporadic cases in patients with systemic lupus erythematosus. However, the recurrent pseudotumor cerebri in patients with systemic lupus erythematosus which has been rarely reported, has not been reported in korea. We experienced a 30-yr-old female patient with SLE who was presented with second attack of severe intractable headache. She was diagnosed pseudotumor cerebri twice and successfully treated with corticosteroid. headache is the common symptom in patients with neuropsychiatric SLE and attributable to various causes. We suggest that it is important to define the cause of headache in patients with SLE and pseudotumor cerebri should be included in the spectrum of clinical manifestations during the course of SLE as a cause of headache. ( info)

9/35. 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. ( info)

10/35. Neuropsychiatric systemic lupus erythematosus presenting as amyotrophic lateral sclerosis.

    A 61-year-old woman with a history of photosensitive dermatitis and recurrent mouth ulcers presented with progressive weakness typical of amyotrophic lateral sclerosis (ALS), and subsequently underwent extensive neurologic and rheumatologic testing. We investigated whether ALS-like motor neuron disease associated with a positive antinuclear antibody (ANA) is really ALS or rather neuropsychiatric systemic lupus erythematosus (NPSLE). On neurologic evaluation, she had prominent bulbar involvement with dysarthria and dysphagia associated with profound lingual fasciculations and a denervating pattern on electromyogram. MRI showed no evidence of cerebral ischemia. Laboratory studies revealed a positive ANA (1:2560 titer), positive antiphospholipid antibodies (GPL and MPL), circulating lupus anticoagulant, and depressed C3 and C4. Repeat MRI studies at 4 and 11 mo revealed an evolving infarct in the paramedian pons consistent with the presence of NPSLE. Therapy was initiated with corticosteroids and intravenous cyclophosphamide, and the neurologic condition did not improve, but also did not progress inexorably as would be expected with ALS. NPSLE, presumably through the mechanism of ischemic vasculopathy, may present as motor neuron disease clinically indistinguishable from ALS. ( info)
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