Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 42-year-old female with scleroderma experienced two exacerbations in which behavioral changes were the main clinical features. On both occasions she presented with paranoid delusions, perceptual aberrations, and disorientation. After treatment with corticosteroids, the patient's mental status returned to normal, and her electroencephalogram showed an increase in alpha wave frequency, which is consistent with a resolving delirium. Unlike systemic lupus erythematosus, scleroderma rarely involves the central nervous system. This case illustrates an unusual manifestation of progressive systemic sclerosis, primary cerebral involvement which presented as an acute organic brain syndrome. Connective tissue diseases, notably systemic lupus erythematosus, often present neuropsychiatric symptoms. Despite the fact that there appears to be a clinical and pathological continuum among the connective tissue diseases, an organic psychosis rarely occurs in progressive systemic sclerosis (scleroderma. Described here is a patient with scleroderma in whom behavioral abnormalities were the main features of two exacerbations of the disease.
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PMID:Scleroderma cerebritis, an unusual manifestation of progressive systemic sclerosis. 111 15

Sensory neuroophthalmic abnormalities due to cerebral lupus erythematosus, with involvement of visual pathways posterior to the optic chiasm, occurred in 12 patients with systemic lupus erhthematosus. Five underwent detailed evaluation because of an hallucination, 4 for visual loss, and 3 for both. Hallucinations were either unformed (for example, bright lights, straight lines) or highly formed (for example, faces), in which case they were invariably recognized by the patient as inappropriate. In no instance did they occur in association with delirium, confusion, or use of hallucinogenic drugs. Patients with loss of vision had scotomas, homonymous field defects, and cortical blindness. These features indicate disease in the posterior cerebral artery circulation, a localization often supported by ancillary neurologic findings, for example, vocal cord paralysis, diminished gag reflex. Thus, various visual dysfunctions may occur in systemic lupus erythematosus due to cerebral vasculitis. At times they may be the most prominent and disabling feature of the disorder.
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PMID:Cerebral disorders of vision in systemic lupus erythematosus. 114 49

In extracerebral systemic lupus erythematosus (SLE), the complement system plays a prominent pathogenic role, and decreased serum concentration of the 4th component (C4) is a reliable indicator of systemic disease activity. In diffuse CNS-SLE, however, the pathogenic role of complement is less clear. In 12 patients with active diffuse CNS-SLE presenting with delirium (4), organic personality syndrome (3), or generalized seizures (5), we determined the CSF indexes of the complement components C3, C4, and factor B, and of IgG, IgA, and IgM. There was a significant increase of the C4 index in these patients compared with controls and a significantly higher CSF C4 index in patients with an increased IgM index. We conclude that intrathecal C4 is being produced in diffuse CNS-SLE.
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PMID:Diffuse CNS involvement in systemic lupus erythematosus: intrathecal synthesis of the 4th component of complement. 221 52

A patient with SLE whose initial manifestation was with a rare delirious disorder manifested as pseudocyesis is described. The psychiatric manifestations were considered to be due to SLE and treated with pulse steroid therapy. Special attention is focused on treatment and MRI.
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PMID:Systemic lupus erythematosus presenting as pseudocyesis. 790 46

Forty-three female inpatients with active systemic lupus erythematosus (SLE) were studied by a multidisciplinary team to answer the following research questions: 1) What are the features of the psychopathology in patients with active SLE? and 2) In these patients, what is the relationship between psychiatric disorders and symptoms and signs suggesting activity of SLE in the CNS? Our a priori hypothesis was that, in patients with active SLE, those with psychiatric manifestations would have more symptoms and signs of CNS activity than those without psychiatric manifestations. Psychiatric evaluation consisted of standardized psychiatric instruments and diagnostic criteria. The assessment of SLE systemic and central nervous system (CNS) activity consisted of rheumatologic, neurologic, and ophthalmologic evaluations; serum and cerebral spinal fluid (CSF) analysis; brain computerized tomography (CT); and electroencephalogram (EEG). Twenty-seven patients (63%) presented psychiatric symptoms (Psychiatric Group), and 16 (37%) patients presented no current psychiatric diagnosis (Nonpsychiatric Group). These groups were compared in terms of the above variables. Depressive syndrome was the most frequent diagnosis (44%) followed by delirium (7%) and dementia (5%). Psychiatric symptoms were associated with subjective cognitive impairment (85%) and neurologic abnormality (85%). Widened cortical sulci was the most frequent CT alteration and was equally common in both groups. No statistical difference was found between the 2 groups regarding their general clinical evaluation, serum and CSF exams, or EEG alterations. To determine whether the severity of psychiatric symptoms was related to CNS activity, we divided the 27 patients with psychiatric manifestations into 2 groups: the Major Group--18 patients with major psychopathology, and the Minor Group--9 patients with mild depressive syndromes.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Psychiatric manifestations of systemic lupus erythematosus: clinical features, symptoms, and signs of central nervous system activity in 43 patients. 804 Dec 45

We report here a case of neuropsychiatric lupus erythematosus with organic brain syndrome and transverse myelitis which was successfully managed by plasmapheresis. A 27-year-old female with facial rash, arthralgia and fever was diagnosed as having SLE and treated with oral prednisolone (PSL) in June 1996. After 6 weeks she demonstrated muscle pain and a spiking temperature. The dose of PSL was increased but clinical symptoms did not improve. In August, pulse methyl-PSL was performed and she subsequently-developed delirium, impairment of orientation, memory and perception, which were followed by paraplegia of the lower extremities and loss of sphincter control. Intravenous bolus cyclophosphamide was not effective, but liver dysfunction, bone marrow suppression and respiratory failure due to an infection of pneumocystis carinii were observed. We then performed plasmapheresis or immunoabsorption several times. After this treatment steady improvement was observed. High values of antiribosomal P protein antibodies in the serum and interleukin-6 in the cerebrospinal fluid decreased. Small foci of increased signal intensity detected on cranial magnetic resonance imaging and hypoperfused areas on single-photon emission CT diminished. The patient was maintained on low-dose PSL and no recurrence has been observed 15 months from the onset.
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PMID:[A case of severe neuropsychiatric lupus erythematosus treated by plasmapheresis: diagnostic values of serum antiribosomal P protein antibodies and interleukin-6 in cerebrospinal fluid]. 979 79

The treatment of patients with neuropsychiatric systemic lupus erythematosus (NPSLE) can be difficult and complex owing to the variety of nervous system manifestations that can occur, which include peripheral nerve disease, headaches, seizures, cerebrovascular disease, chorea, transverse myelitis, and psychiatric and cognitive disorders. Many of these manifestations can result from metabolic abnormalities or infection or as side effects of medications. Thus, in any patient with suspected NPSLE, it is crucial to exclude secondary causes of the presenting symptoms before assuming that they are due to NPSLE. It is especially important to exclude infection because this is a common cause of both morbidity and mortality in patients with systemic lupus erythematosus (SLE). Symptoms such as anxiety and depression may or may not be related to disease activity. Treatment decisions are based on accurate diagnosis of the specific NPSLE manifestation, which is usually made using tools such as brain imaging, electroencephalography, cerebrospinal fluid analysis, nerve conduction studies, or special serologic tests (eg, determination of antiphospholipid or antiribosomal P antibody levels). It is also important to assess the degree of other SLE- mediated systemic disease activity in a patient with neurologic manifestations to determine if activation of systemic disease activity is also occurring. This is done by measuring complement levels, anti-double-stranded DNA levels, complete blood count, and urinalysis. For some NPSLE manifestations (eg, infrequent seizures, headaches, depression, anxiety, or peripheral neuropathy) that appear without activation of systemic disease, symptomatic treatment is appropriate. For others (eg, psychosis, delirium, or transverse myelopathy without other obvious cause), treatment with high-dose glucocorticoids with or without cyclophosphamide is appropriate whether there is evidence of other systemic disease activity or not. In general, the activity and severity of the leading organ manifestations dictate pharmacologic treatment.
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PMID:Neuropsychiatric Systemic Lupus Erythematosus. 1109 72

Acute confusional state (delirium) appears as one of the common neuropsychiatric manifestations of systemic lupus erythematosus (SLE). It has been suggested that neuropsychiatric SLE (NP-SLE) could promote the occurrence of drug-induced extrapyramidal symptoms (EPS). Atypical antipsychotics have been thought to be useful in management of delirium because of their low incidence of adverse effects including EPS. However, no reports of the use of atypical antipsychotics in delirium due to NP-SLE have been published. Here we report a case of NP-SLE presenting an acute confusional state (delirium), which was successfully managed by risperidone.
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PMID:Risperidone in the treatment of acute confusional state (delirium) due to neuropsychiatric lupus erythematosus: case report. 1508 10

Systemic lupus erythematosus (SLE) is an autoimmune disease that may involve the central nervous system (CNS) resulting in neuropsychiatric manifestations. The associated psychiatric disorders include depression, psychosis, mood disorders, anxiety, cognitive dysfunction, and delirium/ encephalopathy. Several autoantibodies may play a role in the pathogenesis of psychiatric complications of SLE, particularly antibodies against ribosomal P-proteins (anti-P) and possibly antibodies against endothelial cells (AECA). The reported prevalence of anti-P is highly variable in SLE patients and is dependent on different ethnic backgrounds, sensitivity and specificity of the assays employed for autoantibody detection, and the time at which sera were analysed in relation to the clinical event. Controversial data exist on the association of anti-P with psychiatric manifestations of SLE. These autoantibodies have been suggested to be specific markers of the psychiatric manifestations of SLE, particularly of the psychosis and depression, and the antibody level varied with the clinical activity of the disease. Some studies have confirmed the hypothesis of an association of anti-P antibodies with psychiatric manifestations of neuropsychiatric SLE (NPSLE) while others have disputed this relationship. This review summarizes the recent studies about relationship between anti-P antibodies and psychiatric manifestation of SLE.
Lupus 2005
PMID:Anti-ribosomal P-protein and its role in psychiatric manifestations of systemic lupus erythematosus: myth or reality? 1617 27

Psychiatric abnormalities are common in systemic lupus erythematosus (SLE) with a prevalence of 17% to 75%, reflecting different methods of patient selection and assessment, the different professional orientation of clinicians, and lack of an accepted consensus for diagnosing active neuropsychiatric lupus (NPSLE). The psychiatric syndromes included in the ACR Neuropsychiatric Lupus Nomenclature Committee criteria are cognitive dysfunction, acute confusional state (delirium), anxiety disorder, mood disorder, and psychosis. In SLE patients, identification of psychiatric phenomena and the generation of a differential diagnosis are crucial. Possible mechanisms include vascular injury and pathogenic antibodies. Treatment strategies are based on small case studies. The purpose of this review is to discuss clinical manifestations, pathogenesis and the present therapeutic options in psychiatric lupus.
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PMID:Psychiatric manifestations in systemic lupus erythematosus. 1753 89


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