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      Catatonia in Juvenile Systemic Lupus Erythematosus: A Case Series

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          Abstract

          Neuropsychiatric systemic lupus erythematosus (NPSLE) presents a significant diagnostic and therapeutic challenge due to its varied clinical manifestations. The prevalence of NPSLE ranges widely, reported between 37% and 95% in different case series, reflecting this condition’s complex and heterogeneous nature. Here we report three cases of juvenile systemic lupus erythematosus (SLE) presenting with catatonia as a rare neuropsychiatric manifestation.

          Case 1 is a 15-year-old male with fever, and pancytopenia, diagnosed with SLE and subsequent development of catatonia. Case 2 is a 14-year-old female with a history of SLE presenting with altered sensorium, restlessness, and catatonia. Case 3 is a 15-year-old male with SLE exhibiting abnormal behaviour and catatonia.

          Treatment strategies for these cases include high-dose steroids, immunosuppression, and benzodiazepines. This case series emphasises the importance of a multidisciplinary approach, prompt diagnosis, aggressive treatment, and vigilant follow-up to optimise outcomes in these vulnerable paediatric patients.

          In conclusion, this case series contributes to the literature on catatonia in paediatric SLE, emphasising the need for expanded awareness, early recognition, and comprehensive management strategies. Further research is warranted to refine predictive factors and establish optimal maintenance protocols for this complex neuropsychiatric manifestation.

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          Most cited references9

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          Catatonia: Our current understanding of its diagnosis, treatment and pathophysiology

          Catatonia is a psychomotor syndrome that has been reported to occur in more than 10% of patients with acute psychiatric illnesses. Two subtypes of the syndrome have been identified. Catatonia of the retarded type is characterized by immobility, mutism, staring, rigidity, and a host of other clinical signs. Excited catatonia is a less common presentation in which patients develop prolonged periods of psychomotor agitation. Once thought to be a subtype of schizophrenia, catatonia is now recognized to occur with a broad spectrum of medical and psychiatric illnesses, particularly affective disorders. In many cases, the catatonia must be treated before any underlying conditions can be accurately diagnosed. Most patients with the syndrome respond rapidly to low-dose benzodiazepines, but electroconvulsive therapy is occasionally required. Patients with longstanding catatonia or a diagnosis of schizophrenia may be less likely to respond. The pathobiology of catatonia is poorly understood, although abnormalities in gamma-aminobutyric acid and glutamate signaling have been suggested as causative factors. Because catatonia is common, highly treatable, and associated with significant morbidity and mortality if left untreated, physicians should maintain a high level of suspicion for this complex clinical syndrome. Since 1989, we have systematically assessed patients presenting to our psychiatry service with signs of retarded catatonia. In this paper, we present a review of the current literature on catatonia along with findings from the 220 cases we have assessed and treated.
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            Neuropsychiatric Systemic Lupus Erythematosus: A 2021 Update on Diagnosis, Management, and Current Challenges

            Patients with systemic lupus erythematosus (SLE) experience neuropsychiatric symptoms. The term neuropsychiatric SLE (NPSLE) is a generic term that refers to a series of neurological and psychiatric symptoms directly related to SLE. In approximately 30% of patients with neuropsychiatric symptoms, SLE is the primary cause (NPSLE), and symptoms manifest more frequently around SLE onset. Neurovascular and psychotic conditions can also lead to NPSLE. Pathogenesis of NPSLE is implicated in both neuroinflammatory and ischemic mechanisms, and it is associated with high morbidity and mortality. After diagnosing and assigning causality, NPSLE treatment is individualized according to the type of neuropsychiatric manifestations, type of the predominant pathway, activity of SLE, and severity of the clinical manifestations. There are many problems to be addressed with regards to the diagnosis and management of NPSLE. Controlled clinical trials provide limited guidance for management, and observational cohort studies support symptomatic, antithrombotic, and immunosuppressive agents. The purpose of this review was to provide a detailed and critical review of the literature on the pathophysiology, diagnosis, and treatment of NPSLE. This study aimed to identify the shortcoming in diagnostic biomarkers, novel therapies against NPSLE, and additional research needs.
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              Interleukin-6 and chemokines in the neuropsychiatric manifestations of systemic lupus erythematosus.

              To define the cytokine and chemokine profile in cerebrospinal fluid (CSF) from patients with neuropsychiatric systemic lupus erythematosus (NPSLE). Forty-two SLE patients who had been hospitalized because of NP manifestations were studied. Patients were evaluated at hospitalization and 6 months later; a CSF sample was obtained at each evaluation. As controls, CSF from 6 SLE patients with septic meningitis, 16 SLE patients with no history of NP manifestations (non-NPSLE), and 25 patients with nonautoimmune diseases were also studied. Soluble molecules, including cytokines (interleukin-2 [IL-2], IL-4, IL-6, IL-10, tumor necrosis factor alpha [TNFalpha], and interferon-gamma [IFNgamma]) and chemokines (monocyte chemotactic protein 1 [MCP-1], RANTES, IL-8, monokine induced by IFNgamma [MIG], and interferon-gamma-inducible 10-kd protein [IP-10]), were measured with the use of cytometric bead array kits. CSF levels of the following molecules were significantly increased in NPSLE patients as compared with non-NPSLE and nonautoimmune diseases control patients, respectively: IL-6 (32.7 versus 3.0 and 2.96 pg/ml), IL-8 (102.8 versus 29.97 and 19.7 pg/ml), IP-10 (888.2 versus 329.7 [P not significant] and 133.6 pg/ml), RANTES (3.8 versus 2.5 and 2.2 pg/ml), MCP-1 (401.7 versus 257.9 [P not significant] and 136.9 pg/ml), and MIG (35.4 versus 11.4 and 3.5 pg/ml). Low levels of IL-2, IL-4, IL-10, TNFalpha, and IFNgamma were found in all groups. All cytokines and chemokines, except TNFalpha, were significantly higher among the SLE patients with septic meningitis than among the NPSLE patients. Six months later and in the absence of NP manifestations, all elevated molecule levels, except RANTES, in patients with NPSLE had decreased significantly, and no differences were noted between the NPSLE and non-NPSLE groups. A central nervous system response composed of IL-6 and chemokines, but not Th1/Th2 cytokines, is associated with NP manifestations in SLE patients.
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                Author and article information

                Journal
                Mediterr J Rheumatol
                Mediterr J Rheumatol
                MJR
                Mediterranean Journal of Rheumatology
                The Mediterranean Journal of Rheumatology (MJR)
                2529-198X
                September 2024
                24 July 2024
                : 35
                : 3
                : 474-478
                Affiliations
                [1 ]Dept. of Clinical Immunology and Rheumatology, JSS Medical College and Hospital, JSS Academy of Higher Research, Mysore, Karnataka, India
                [2 ]O.M. Health Care, Tamil Nadu, India
                [3 ]Madras Medical College, Chennai, Tamil Nadu, India
                Author notes
                Corresponding Author: Ponniah Subramanian ArulRajamurugan, Associate Professor of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India, Tel.: +91 9840665286, E-mail: arulrajamurugan@ 123456gmail.com
                Author information
                https://orcid.org/0000-0001-7421-2418
                https://orcid.org/0000-0002-4868-1883
                https://orcid.org/0000-0002-2259-1572
                Article
                MJR-35-3-474
                10.31138/mjr.311223.cij
                11500119
                87465a21-f335-42fa-a7da-1a4b735b12f6
                © 2024 The Mediterranean Journal of Rheumatology (MJR)

                This work is licensed under and Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 31 December 2023
                : 18 February 2024
                : 03 March 2024
                Categories
                Case Series

                juvenile sle,movement disorder,neuropsychiatric lupus

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