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Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multi-systemic involvement. Nervous system involvement in SLE leads to a series of uncommon and heterogeneous neuropsychiatric (NP) manifestations. Current knowledge on the underlying pathogenic processes and their subsequent pathophysiological changes leading to NP...
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... Research is being conducted to search for reliable laboratory and neuroimaging biomarkers in the diagnosis of NPSLE. It was noted that an increased level of IL-6 in the cerebrospinal fluid showed a positive correlation with acute confusional state [11,101,102]. ...
... Prowadzone są badania w poszukiwaniu wiarygodnych biomarkerów laboratoryjnych i neuroobrazowych w diagnostyce NPSLE. Zauważono, że dodatnią korelację z ostrym stanem splątania wykazał podwyższony poziom IL-6 w płynie mózgowordzeniowym [11,101,102]. ...
Introduction: Systemic lupus erythematosus is an autoimmune disease affecting up to 210 per 100,000 people in Europe, more often among women. The inflammatory process in lupus causes changes in various organs. However, somatic changes are not the only effects of lupus. The neuropsychiatric manifestations of this disease have been given a separate name – neuropsychiatric lupus. Material and methods: A review of the literature available on the PubMed platform in the period of 1987-2023 was performed using the key words: neuropsychiatric systemic lupus erythematosus, mental disorders, mood disorders, sleep disorders, systemic lupus erythematosus. Original studies, review works, meta-analyses and Internet sources were analyzed. Results: Psychotic disorders in lupus occur with a frequency of up to 3%. Risk factors include young age, male gender and glucocorticoids treatment. Mood disorders occur in several to several dozen percent of lupus patients, including depression affecting up to ⅓ of patients. Belimumab, psychotherapy and improving the quality of sleep, the disturbance of which is observed in most patients with lupus, have potential in treatment. Anxiety disorders are seen primarily in the teenage age group, where social phobia predominates – the fear of rejection due to the disease – and they worsen and are exacerbated by rheumatic disease. Cognitive dysfunctions occur in up to 80% of lupus patients. They are probably related to enzymes of metabolic pathways, dyslipidemia and thyroid dysfunction. Conclusions: Mental disorders develop more often in patients with lupus than in the general population and they predispose to autoimmune diseases. Comprehensive diagnosis and psychiatric care of patients with lupus are necessary. Keywords: systemic lupus erythematosus, sleep disorders, mood disorders, neuropsychiatric systemic lupus erythematosus, mental disorders
... However, diagnostic biomarkers in serum and cerebrospinal fluid lack definitive accuracy for clinical use [10,11]. Neuroimaging, even with gold-standard modalities like MRI, may show no significant changes in up to 50% of patients with clinical disease [12]. In our case, clinical, serological, and nerve conduction studies were crucial for diagnosing the neuropsychiatric manifestation, subsequently identified as AIDP. ...
A 17-year-old male diagnosed with systemic lupus erythematosus (SLE), showing poor compliance with medication, presented to our facility with a 20-day history of fever, polyarthritis, and cough. Additionally, he had experienced a seizure episode, followed by a one-day history of altered mentation. Subsequently, he developed pneumonia, respiratory distress, and shock, necessitating ventilator and inotropic support. Neuropsychiatric lupus (NP-lupus) was suspected, and hence high-dose steroids, hydroxychloroquine, and broad-spectrum antibiotics were initiated. Following successful extubation, he manifested ascending flaccid paralysis. The presence of albumin-cytological dissociation and axonal neuropathy confirmed the diagnosis of acute inflammatory demyelinating polyneuropathy (AIDP). He underwent further management with pulse steroids and plasmapheresis. Upon recovery, he was discharged on a regimen of steroids, cyclophosphamide, and hydroxychloroquine. During follow-up, he maintained ambulatory status with no residual neurological sequelae.
... 40 Moreover, imaging methods such as functional magnetic resonance imaging (MRI) and dynamic contrast-enhanced MRI (DCE-MRI) have provided important insights in linking functional aberrancies, BBB permeability, and neuropsychiatric symptoms, primarily in adults with NPSLE. 41 For instance, resting-state functional hypoconnectivity has been associated with memory impairments, whereas altered task-based functional connectivity was linked to cognitive dysfunction in NPSLE. 42,43 Altered cerebral blood flow within gray and white matter distinguished between patients with SLE with or without neuropsychiatric symptoms as well as control subjects. ...
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that is increasingly affecting pediatric and adult populations. Neuropsychiatric manifestations (ie, cognitive dysfunction and mood disorders) appear to occur with greater severity and poorer prognosis in childhood‐onset SLE (cSLE) versus adult‐onset SLE, negatively impacting school function, self‐management, and psychosocial health, as well as lifelong health‐related quality of life. In this review, we describe pathogenic mechanisms active in cSLE, such as maladaptive inflammatory processes and ischemia, which are hypothesized to underpin central phenotypes in patients with cSLE, and the role of alterations in protective central nervous system (CNS) barriers (ie, the blood–brain barrier) are also discussed. Recent findings derived from novel neuroimaging approaches are highlighted because the methods employed in these studies hold potential for identifying CNS abnormalities that would otherwise remain undetected with conventional multiple resonance imaging studies (eg, T2‐weighted or fluid‐attenuated inversion recovery sequences). Furthermore, we propose that a more robust presentation of neuropsychiatric symptoms in cSLE is in part due to the harmful impact of a chronic inflammatory insult on a developing CNS. Although the immature status of the CNS may leave patients with cSLE more vulnerable to harboring neuropsychiatric manifestations, the same property may represent a greater urgency to reverse the maladaptive effects associated with a proneuroinflammatory state, provided that effective diagnostic tools and treatment strategies are available. Finally, considering the crosstalk among the CNS and other organ systems affected in cSLE, we postulate that a finer understanding of this interconnectivity and its role in the clinical presentation in cSLE is warranted.
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... Seizures occur in 28% of NPSLE and 80% of NMDARE patients (14,15). Laboratory analyses, especially in NPSLE, include positive anti-ribosomal-P antibodies, anti-neuronal antibodies, lupus anticoagulants, and elevated IL-6 levels (16). A study found that a positive lupus anticoagulant predicts intracranial thrombosis, while anti-ribosomal-P is related to lupus psychosis (16). ...
... Laboratory analyses, especially in NPSLE, include positive anti-ribosomal-P antibodies, anti-neuronal antibodies, lupus anticoagulants, and elevated IL-6 levels (16). A study found that a positive lupus anticoagulant predicts intracranial thrombosis, while anti-ribosomal-P is related to lupus psychosis (16). While the association between NMDARE and SLE is not clearly _________________________________________________________________________________________________________________________________________________________ N. Sanghavi, B. Ayesha ...
Anti-N-methyl-d-aspartate receptor encephalitis (NMDARE) is a B-cell-mediated autoimmune encephalitis with wide non-specific symptoms like acute-onset psychiatric or neurological ones mimicking various other conditions. A careful history and appropriate workup, including cerebrospinal fluid analysis for anti-NMDAR antibodies, imaging, and electroencephalogram, should be conducted, considering all differential diagnoses that can mimic its presentation. Combination therapy with high-dose steroids, plasma exchange, or immunoglobulin therapy has been shown to be more efficacious. In patients who fail first-line therapy, rituximab or cyclophosphamide should be considered. It is essential to rule out ovarian teratoma or other occult malignancies that can cause NMDARE, as removal of the tumor itself resolves this condition. Timely diagnosis and early intervention are necessary to avoid an untoward outcome.
... Distinguishing whether neuropsychiatric symptoms are directly caused by SLE or result from other factors like corticosteroid treatment or psychological burden is crucial for treatment decisions. Various models have been developed to enhance the identification of SLE-related neuropsychiatric symptoms [24,28]. Individualizing the diagnostic approach for each NPSLE patient is necessary because there's no one-size-fitsall method. ...
Neuropsychiatric lupus, also known as neuropsychiatric systemic lupus erythematosus (NPSLE), is a subset of systemic lupus erythematosus (SLE) that affects the central nervous system, leading to a range of neurological and psychiatric symptoms. It occurs in approximately 25-75% of SLE patients, varying prevalence across populations. NPSLE primarily affects women of childbearing age but can occur in individuals of any age or gender, with a higher prevalence among non-Caucasian populations and those with severe SLE symptoms. The exact cause of NPSLE is complex, involving immune dysregulation, autoantibody production, and central nervous system inflammation. Autoantibodies, such as anti-phospholipid and anti-NMDA receptor antibodies, may significantly affect neurological symptoms. Immune-complex deposition and proinflammatory cytokines within the brain contribute to the disease’s pathophysiology. NPSLE encompasses many symptoms, including cognitive impairment, mood disorders, psychosis, seizures, and headaches. Diagnosis relies on clinical evaluation, neuroimaging, cerebrospinal fluid analysis, and autoantibody testing. A multidisciplinary approach involving rheumatologists, neurologists, and psychiatrists is often necessary. Treatment for NPSLE is tailored to the specific symptoms and their severity. It typically involves immunosuppressive therapies like corticosteroids, diseasemodifying antirheumatic drugs (DMARDs), and sometimes biological agents. Adjunctive therapies, including antiepileptic drugs, antipsychotics, and antidepressants, may be necessary for symptom management. Regular monitoring and follow-up are crucial to assess treatment response.
... The recognition and diagnosis of NP symptoms in SLE patients is hindered by numerous factors including, the lack of standardized assessment, the heterogeneity of neurological findings, and the unreliability of biomarkers for diagnosing and monitoring the disease [4]. There is no biomarker that demonstrates significant ubiquitous or reproducible attribution for NP-SLE diagnosis [5]. Because of the challenging diagnosis and treatment of NP-SLE, studies have shown higher rates of morbidity and mortality in SLE patients with NP manifestations [4]. ...
Introduction: Among the wide variety of clinical manifestations in Systemic Lupus Erythematosus (SLE), cognitive dysfunction (CD) is a subtle finding, where the reported prevalence ranges from 3-88% due to CD assessment inconsistencies and challenges with SLE correlations. Cognitive dysfunction may also be a cornerstone element for a diagnosis of Neuropsychiatric Systemic Lupus Erythematosus (NP-SLE). We present a case of Lupus Cerebritis displaying seizures and oculomotor dysfunctions referred to as Balint Syndrome. To our knowledge, no publications to date have shown a correlation between Lupus Cerebritis and Balint Syndrome. Case Report: A 35-year-old female with a history of lupus, seizures, and migraines presented complaining of a severe headache associated with vomiting. The patient stated the onset of her symptoms was three days prior and had been worsening. While in the emergency room, the patient had a generalized tonic-clonic seizure lasting around 2 minutes at which point neurology was consulted for seizure management. The patient had been seen about a year prior for similar complaints and was started on Keppra to control her epilepsy. The patient was seizure free for about 6 months so Keppra was discontinued. Discussion: A wide array of symptoms are associated with Lupus Cerebritis, which is a rare manifestation following a diagnosis of SLE. Our patient was having seizures and complaints of migraine with severe Balint Syndrome consisting of oculomotor apraxia, optic ataxia, and simultagnosia. Due to some patients’ rapid decline following a diagnosis of SLE and the complex diversity of symptoms, it is crucial to prevent organ failure by treating them immediately, and furthermore, to equip and educate clinicians in identifying atypical presentations. Conclusion: Due to the complexity of autoimmune diseases, patients may present with a plethora of symptomatology, ranging from mild to severe, making a thorough medical history and physical examination imperative elements of a complete workup. Since NP-SLE is a nuanced diagnosis requiring specific management, it is essential to have close follow-ups with neurology.
... Although chorea/hemibalism is classically associated with subthalamic nucleo lesions, presentations with hemichora/hemibalism are described in international records in patients with stoke in white substance, provided they affect the nucleo-linking pathways [13]. There are neither SLE-specific changes nor correlations between imaging findings and clinical manifestations and the MRI are normal in 40% of all NPSLE [14]. So, hemichora/chorea is a classic manifestation of neurolupus, lesions outside the ganglios of the base -already described, maybe by lesion of the pathways between the ganglios of the base. ...
Systemic lupus erythematosus (SLE) is an autoimmune disease with multisystemic manifestations, including central nervous system involvement. Chorea is a hyperkinetic movement disorder, characterized by involuntary, dance-like and poorly coordinated movements. Acute-onset chorea is a rare neuropsychiatric inaugural manifestation of SLE. This presentation is frequently associated with positive antiphospholipid antibodies, and it usually improves with immunosuppressive treatment.
We report the case of a 20-year-old female, who presented with acute onset left hemichorea and fever. Analysis showed active urine sediment. A detailed anamnesis and evaluation revealed several clinical manifestations suggestive of SLE with multiorgan involvement: neurological, renal, cardiac, hematological, joint and mucocutaneous.
This case emphasizes the importance of keeping a high clinical awareness for rarer presentations of common autoimmune disorders, such as SLE, which can be severe and should be promptly treated. Furthermore, the relevance of SLE in the differential diagnosis of acute-onset movement disorders in young patients is highlighted in this report.
... The majority of SLE studies ignore the strategic location of WMH and used lesion volume and number. However, those metrices varies strongly across studies 5 . Until now, only few studies investigated the WMH location in SLE. ...
... To overcome this problem, we performed a two site study. Second, the absence of biomarkers (radiological or laboratory) reliable enough in the diagnostic process make it difficult to create a link between radiological findings and clinical symptoms (clinic-radiological paradox) 5 . ...
Currently, little is known about the spatial distribution of white matter hyperintensities (WMH) in the brain of patients with Systemic Lupus erythematosus (SLE). Previous lesion markers, such as number and volume, ignore the strategic location of WMH. The goal of this work was to develop a fully-automated method to identify predominant patterns of WMH across WM tracts based on cluster analysis. A total of 221 SLE patients with and without neuropsychiatric symptoms from two different sites were included in this study. WMH segmentations and lesion locations were acquired automatically. Cluster analysis was performed on the WMH distribution in 20 WM tracts. Our pipeline identified five distinct clusters with predominant involvement of the forceps major, forceps minor, as well as right and left anterior thalamic radiations and the right inferior fronto-occipital fasciculus. The patterns of the affected WM tracts were consistent over the SLE subtypes and sites. Our approach revealed distinct and robust tract-based WMH patterns within SLE patients. This method could provide a basis, to link the location of WMH with clinical symptoms. Furthermore, it could be used for other diseases characterized by presence of WMH to investigate both the clinical relevance of WMH and underlying pathomechanism in the brain.
... Since the 1980s, aberrant brain MRI has been described in SLE and NP-SLE [100,101]. On conventional MRI (cMRI), a significant proportion of patients with NP-SLE show no abnormalities, and global markers such as lesion load or brain atrophy do not correlate with symptom severity [102,103]. Innovative MRI techniques and software may be more precise in identifying brain variations in NPSLE patients. Researchers were able to map the microstructure of the brain utilizing mean diffusivity and fractional anisotropy (DTI), sophisticated MRI methods including DOI: http://dx.doi.org ...
An autoimmune condition is characterized by a misdirected immunological system that interacts with host antigens. Excess activation of T-and B-lymphocytes, autoan-tibody generation, immune complex deposition, and multi-organ injury are found in systemic lupus erythematosus (SLE), an early autoimmune condition with a substantial hereditary element. A number of environmental factors and lifestyle changes also play a role in the development of SLE. The imbalanced immunity could take part in the dysfunction and injury of different biological organs, including the central and peripheral nervous systems. Neuropsychiatric SLE (NPSLE) can present with focal and diffuse involvements. Clinical manifestations of NPSLE vary from mild cognitive deficits to changed mental status, psychosis, and seizure disorders. Headaches, mood, and cogni-tive problems are the most common neuropsychiatric presentations associated with SLE. NPSLE could be found in 40% of all people who have SLE. The diagnostic inference of NPSLE can be made solely following these secondary causes have been ruled out. The present chapter provides an updated discussion of the clinical presentation, molecular processes, diagnosis, management, and therapy of SLE with emphasizing on NPSLE.
... CSF findings include pleocytosis, elevated protein and IgG index and decreased glucose levels [37,48]. MRI might reveal multifocal infarcts, basal ganglia lesions, white matter T2/FLAIR hyperintensities, cerebral atrophy, edema, hemorrhages and intracranial calcifications [48,51]. Angiography detects stenoses and aneurysms, while on MRA images vascular wall thickening and intramural accumulation of contrast could also be observed [37]. ...
Inflammatory disorders of the central nervous system (CNS) vessels, also called CNS vasculitides, can cause substantial disability or even be fatal. Inflammation of the CNS vessels can be caused by primary angiitis of the CNS (PACNS), inflammatory cerebral amyloid angiopathy, or systemic inflammatory disorders. Clinical symptoms of these disorders are often non-specific, such as encephalopathy, cognitive and affective abnormalities, headache and focal neurological symptoms. Diagnostic workup includes a thorough neuropsychiatric examination, blood and cerebrospinal fluid analysis and magnetic resonance imaging (MRI) of the brain and its vessels. Biopsy of the brain remains the gold standard diagnostic test. Timely diagnosis and treatment initiation is of high importance, as it might prevent severe complications, such as ischemic and hemorrhagic stroke. In this review, we describe the specific characteristics of primary and secondary non-infectious CNS vasculitides which help to establish the diagnosis, discuss the peculiarities of the diagnostic workup and present current treatment recommendations.
