Presentation Of Neuropsychiatric Systemic Lupus Erythematosus Research Articles

Recurrent Neuropsychiatric Systemic Lupus Erythematosus Presenting with Primary Central Nervous System Lymphoma

We report the case of a systemic lupus erythematosus (SLE) patient who was diagnosed with lupus cerebritis, vasculitis, Epstein-Barr virus infection, and consequently, lymphoma. The patient presented to the emergency department with dysarthria, dysphagia, left-sided hemiparesis, and hypesthesia. Initial brain magnetic resonance imaging (MRI) revealed multiple high signal intensities in the cortex and subcortex. Intravenous methylprednisolone and immunoglobulin administration did not improve her symptoms. However, intravenous rituximab improved the parenchymal and angiographic abnormalities. One month later, she developed another acute left-sided hypesthesia. Brain MRI revealed multiple rim-enhancing lesions with perilesional edema, different from the previous lesion. Intravenous methylprednisolone and cyclophosphamide did not improve her symptoms, and she was finally diagnosed with primary central nervous system lymphoma after brain biopsy. Neurological symptoms improved after chemotherapy. Since the incidence of primary central nervous lymphoma is relatively high in SLE patients, careful diagnosis and accurate treatment are needed.

Electroconvulsive Therapy Is an Effective Treatment Modality in Children With Lupus Cerebritis With Persistent Catatonia.

A 13-year-old girl with neuropsychiatric systemic lupus erythematosus presenting with psychosis and persistent catatonia unresponsive to immunosuppressive agents and a benzodiazepine was successfully treated with ECT.

Neuropsychiatric Systemic Lupus Erythematosus Presenting as Mania With Psychosis Treated Successfully With Intravenous Immunoglobulin: A Diagnosis of Associations and Exclusions.

Neuropsychiatric Systemic Lupus Erythematosus Presenting as Mania With Psychosis Treated Successfully With Intravenous Immunoglobulin: A Diagnosis of Associations and Exclusions.

Intracranial hypertension, nephritis and serositis complicating unexpected pregnancy in a lupus patient

Background: Systemic lupus erythematosus (SLE) flare in pregnancy is challenging. The simultaneous occurrence of intracranial hypertension (IH) is unusual and raises the possibility of being a presentation of neuropsychiatric SLE. Aim of the work: We here discuss the presentation and management of a complicated case of lupus associated with IH, lupus nephritis (LN) and serositis in unplanned pregnancy. Case presentation: A 26-year old SLE patient presented 10 weeks pregnant with marked pleural effusion, hypertension, grade V papilledema, impaired renal functions, proteinuria (2.2 g/24 h), high anti-double stranded deoxyribonucleic acid and consumed complement (C3). Renal biopsy revealed LN (class IV and V). MRI brain demonstrated features consistent with IH and perimetry revealed early optic nerve affection. Ultrasound-guided pleurocentesis and relieving lumbar puncture were performed. Pulse methylprednisolone and cyclosporine A (CsA) (2 mg/kg/day) were provided. Acetazolamide was added to manage the IH and meticulous control of BP was achieved. In 2 weeks renal functions, proteinuria and papilloedema improved. The patient gave birth via vaginal delivery at full term to a normal healthy boy. On follow-up after delivery, proteinuria ranged from 100 to 300 mg/24 h with normal renal functions and fundus. The patient was maintained on CsA, hydroxychloroquine and tapering doses of steroids with no relapses. Conclusion: Pregnant SLE cases presenting with IH, LN and serositis are challenging concerning the management of SLE activity. IH may be a sign of lupus activity and its resolution coincides with the control of the flare. Lower dose CsA is potentially effective in treating LN in pregnancy with no reported side effects.

Chicken or the Egg: Anorexia Nervosa and Systemic Lupus Erythematosus in Children and Adolescents

Systemic lupus erythematosus (SLE) frequently has neuropsychiatric involvement including affective disorders, psychosis, and cognitive dysfunction. Evidence suggests that anorexia nervosa (AN) in adolescents with SLE may be triggered by steroid-induced changes in weight and body shape. We propose that AN may be another manifestation of neuropsychiatric SLE and should be considered in this patient population. A retrospective chart review identified 7 children/adolescents diagnosed with SLE and the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition diagnostic criteria for AN, restrictive subtype, at the Hospital for Sick Children in Toronto between January 1989 and January 2011. One patient developed AN 15 months after being diagnosed with SLE that was attributed to prednisone-induced weight gain and cushingoid appearance. Of the remaining 6 patients, the median age at onset of AN symptoms was 12.2 years and diagnosis of AN was 13.6 years. The median age at SLE diagnosis was 14.2 years with median time after onset of AN symptoms of 20 months (7.5-32 months). All patients had evidence of joint symptoms and a positive antinuclear antibody, and 50% had lymphopenia at the time of SLE diagnosis. Treatment of SLE resulted in improvement of AN in all patients. The timing of the clinical presentation of AN in relation to the diagnosis of SLE and response to SLE treatment suggests that AN may be a novel presentation of neuropsychiatric SLE. Patients with AN who present with or develop joint symptoms, a positive antinuclear antibody, or lymphopenia should be investigated and followed for possible SLE.

Efficacy of Plasma Exchange in Pharmaco-Resistant Acute Mania in Systemic Lupus Erythematosus

Back to table of contents Previous article Next article This article has been corrected | View Correction LettersFull AccessEfficacy of Plasma Exchange in Pharmaco-Resistant Acute Mania in Systemic Lupus ErythematosusR. Schwan, M.D., Ph.D., M. Tadri, M.D., S. Zuily, M.D., Ph.D., J. Lerond, M.D., Ph.D., and V. Laprevote, M.D., Ph.D.R. SchwanSearch for more papers by this author, M.D., Ph.D., M. TadriSearch for more papers by this author, M.D., S. ZuilySearch for more papers by this author, M.D., Ph.D., J. LerondSearch for more papers by this author, M.D., Ph.D., and V. LaprevoteSearch for more papers by this author, M.D., Ph.D.Published Online:1 Jan 2013https://doi.org/10.1176/appi.neuropsych.11120373AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail To the Editor: Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect any part of the body, including the central nervous system. Psychiatric manifestations during SLE are not unusual; their characteristic features are early occurrence and clinical polymorphism.1,2 This clinical case shows a picture of SLE in which the first manifestations were psychiatric, with, in particular, an episode of mania3 with delirium that was resistant to drug treatment but responded well to plasmapheresis.A 28-year-old, socially well-integrated woman without psychiatric antecedents, was admitted in restraint to our psychiatric ward, after presenting herself at a police station clad only in an overcoat and unable to give her identity. She presented with spatiotemporal disorientation, muddled thinking, mental automaticity, vivid delirium of polymorphic content, and hetero-aggressivity. Somatic and neurological examination results were unexceptional, and the biological analysis results showed leuconeutropenia and a high sedimentation time. Serological tests for syphilis, HIV, HVB, and HVC were negative, and a brain scan and EEG showed no anomalies. A nonspecified psychotic state was diagnosed, and an antipsychotic treatment combining risperidone and loxapine was given.After 3 weeks in the hospital, with a favorable progression and near-complete regression of the delirium, the patient suddenly relapsed, presenting a manic state with psychotic symptoms and megalomaniac delirium.Treatment with olanzapine was started, and loxapine was maintained at a higher dose of 600 mg/day. A thymoregulator (valproic acid) was added 2 weeks later, as the symptoms did not improve.The symptoms of acute mania were accompanied by an episode of fever, a malar erythema, multiple joint pain of the upper limbs, ulceration of buccal and vulvar mucosae, and Raynaud’s syndrome. Biological analysis showed a slight normocytic anemia and leucopenia with neutropenia and lymphopenia. Strongly positive antinuclear antibodies were found (titer above 1/1,280, speckled type). Native anti-DNA antibodies also tested positive, together with anti-RNP/Sm and anti-SSA/RO antibodies. The total complement was lowered, with a collapse of fraction C4.The diagnosis of a severe flare-up of SLE was therefore made. In association with the psychotropic treatment, a course of cyclophosphamide was given every 3 weeks at 0.6 mg/m3. Progression in 1 month was marked by normalizing of biological analysis, but mania symptoms worsened, with more severe behavioral disorders requiring near-continual isolation of the patient. Brain MRI still showed no sign of vascularity.3Therefore the psychotropic treatments were discontinued, and, for maniac symptoms, indication was given for a series of six plasma exchanges.We noted an improvement in the psychiatric symptoms immediately after the first plasmapheresis. After the third plasma exchange session, a regression of the mania symptoms was observed. There still remained brief and fluctuating auditory hallucinations, which totally disappeared 2 weeks after the last plasmapheresis session, marking the total regression of all psychiatric symptoms.No psychiatric treatment was resumed, and no psychiatric relapse has been reported after 1 year of follow-up.To our knowledge, this clinical case is the first description of indication and utilization of plasma exchange for a manic state secondary to an SLE flare-up.University Hospital of Psychiatrie and Psychothérapie, Laxou, France (RS, JL)Faculty of Medicine, Lorraine-University, Vandoeuvre-lès-Nancy, France (RS, SZ)Inserm 9501, University Hospital of Nancy, Nancy, France (RS, MT, SZ, JL, VL)CSAPA (Health Care Centre of Accompaniment and Prevention in Addictology), University Hospital of Nancy, Nancy, France (RS, VL)CHU de Nancy, service de médecine vasculaire, Vandoeuvre-les-Nancy, F-54500, France (SZ)Correspondence: Professor Raymund Schwan, CHU de Nancy; e-mail: r.[email protected]frReferences1 Waterloo K, Omdal R, Husby G, et al.: Neuropsychological function in systemic lupus erythematosus: a five-year longitudinal study. Rheumatology (Oxford) 2002; 41:411–415Crossref, Medline, Google Scholar2 Fernández Ga de Las Heras V, Gorriti MA, García-Vicuña R, et al.: Psychosis leading to the diagnosis of unrecognized systemic lupus erythematosus: a case report. Rheumatol Int 2007; 27:883–885Crossref, Medline, Google Scholar3 Brey RL: Neuropsychiatric lupus: clinical and imaging aspects. Bull NYU Hosp Jt Dis 2007; 65:194–199Medline, Google Scholar4 Alao AO, Chlebowski S, Chung C. Neuropsychiatric systemic lupus erythematosus presenting as bipolar I disorder with catatonic features. Psychosomatics 2009; 50:543–547Crossref, Medline, Google Scholar FiguresReferencesCited byDetailsCited ByOlanzapine6 March 2021 | Reactions Weekly, Vol. 1845, No. 1 Volume 25Issue 1 Winter 2013Pages E02-E03 Metrics PDF download History Published online 1 January 2013 Published in print 1 January 2013

Electroconvulsive Therapy for Catatonia in Neuropsychiatric Systemic Lupus Erythematosus

Neuropsychiatric systemic lupus erythematosus encompasses neurological syndromes of the central, peripheral, and autonomic nervous system and a variety of psychiatric syndromes. Neuropsychiatric systemic lupus erythematosus presenting as catatonia is uncommon, and treatment of this condition is not well defined. Here we describe a case of neuropsychiatric systemic lupus erythematosus with catatonia and our treatment approach focusing on electroconvulsive therapy in conjunction with cyclophosphamide. We also discuss the pathophysiological underpinnings of the condition and the basis for treatment.

Neuropsychiatric Systemic Lupus Erythematosus Presenting as Bipolar I Disorder With Catatonic Features

The American College of Rheumatology has defined 19 neuropsychiatric syndromes associated with systemic lupus erythematosus (SLE) involving the central, peripheral, and autonomic nervous systems. Neuropsychiatric manifestations of lupus (NPSLE) have been shown to occur in up to 95% of pediatric patients with SLE. The authors describe a 15-year-old African American young woman with a family history positive for bipolar I disorder and schizophrenia, who presented with symptoms consistent with an affective disorder. The patient was diagnosed with Bipolar I disorder with catatonic features and required multiple hospitalizations for mood disturbance. Two years after her initial presentation, the patient was noted to have a malar rash and subsequently underwent a full rheumatologic work-up, which revealed cerebral vasculitis. NPSLE was diagnosed and, after treatment with steroids, the patient improved substantially and no longer required further psychiatric medication or therapy. Given the especially high prevalence of NPSLE in pediatric patients with lupus, it is important for clinicians to recognize that neuropsychiatric symptoms in an adolescent patient may indeed be the initial manifestations of SLE, as opposed to a primary affective disorder.

Cerebral angioedema associated with enalapril

Cerebral angioedema associated with enalapril

Antineuronal antibodies and electroneurophysiological studies in pediatric patients with neuropsychiatric systemic lupus erythematosus

Neuropsychiatric systemic lupus erythematosus (NPSLE) is one of the most difficult manifestations of lupus to diagnose. Measurement of serum brain autoantibodies and assessment of cognitive function by electroneurophysiological studies (electroencephalogram (EEG) and P300) have contributed to an earlier and a more specific diagnosis of NPSLE. Thus, we were stimulated to assess the value of serum antineuronal antibodies and electroneurophysiological studies in diagnosis and early prediction of NPSLE. To investigate this, assessment of serum antineuronal antibodies and cognitive function (clinically and by electroneurophysiological studies) was done in 30 lupus patients [14 (46.7%) with and 16 (53.3%) without clinical evidence of NPSLE] in comparison with 30 healthy matched subjects. Patients without clinical evidence of NPSLE were followed-up clinically by monthly neuropsychiatric evaluation for 18 months. Seropositivity for antineuronal antibodies and abnormalities of EEG and P300 (prolonged latency and/or low amplitude) were found in 60%, 50% and 70%, respectively of lupus patients. During follow-up, 8 out of the 16 patients without clinical evidence of NPSLE developed such evidence [six (75%) had antineuronal seropositivity, five (62.5%) had abnormal EEG, six (75%) had P300 abnormalities and all had at least one abnormal result of these parameters at the time of initial evaluation before clinical presentation of NPSLE]. In conclusion, serum antineuronal antibodies and electroneurophysiological studies may be reliable parameters for diagnosis and early prediction of NPSLE, especially when combined together, before clinical manifestations ensue. Further studies on a large scale are warranted to evaluate the predictive value of these parameters in NPSLE.