Primary Psychiatric Disorder Masking the Diagnosis of Lupus Cerebritis

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.

Section: Reviewmentioning

confidence: 75%

Neuropsychiatric Systemic Lupus Erythematosus: A 2021 Update on Diagnosis, Management, and Current Challenges

Sarwar¹,

Mohamed²,

Rogers³

et al. 2021

“…High-dose steroids are first-line treatments for active lupus flares. In other cases of neurological complications secondary to lupus, other case reports have shown that high doses for a brief duration helped alleviate neurological symptoms in patients with lupus encephalopathy [ 9 ]. The presence of very mild elevations in CRP and low titers, further points toward a non-inflammatory picture for this patient’s presentation.…”

Section: Discussionmentioning

confidence: 99%

Acute Encephalopathy Workup for a Patient With a History of Lupus Cerebritis and Unremarkable Inflammatory Markers: A Case Report

Dasbach,

Kim,

Pinti

et al. 2024

Lupus is a relatively rare disease; however, many of the patients diagnosed with lupus experience an acute confusional state. Despite the prevalence, lupus cerebritis remains a diagnosis of exclusion due to the number of differential diagnoses of the cause of acute confusional state and unreliable clinical markers. This case report highlights the significant duration it takes to work up a broad differential before initiating treatment for lupus. Our case involves a 65-year-old woman with a documented history of lupus, who presented with acute encephalopathy. Following the comprehensive diagnostic investigation, the patient was treated with high-dose steroids that resolved the patient’s symptoms. This report considers the option of empiric steroid treatment in the setting of acute encephalopathy in individuals with a history of lupus cerebritis with inconclusive test results.

“…The presence of nihilistic delusions such as the patient herself and family members being deceased along with beliefs that eating was pointless since she was already dead revealed poor insight, thus meeting ACR criterion 1 for psychosis in the presence of SLE [ 5 - 7 ]. This patient’s decline in social functioning met ACR criterion 2.…”

Section: Discussionmentioning

confidence: 99%

“…This patient’s SLE presentation was especially notable for several reasons. Neuropsychiatric manifestations as a result of SLE are rare events that occur only in around 2% of patients [ 5 , 10 ]. Lupus cerebritis is less common for a woman of 44 years of age to develop lupus cerebritis than with the pediatric and young adult populations, who are at greater stochastic risk for lupus cerebritis [ 11 , 12 ].…”

Section: Discussionmentioning

confidence: 99%

Systemic Lupus Erythematosus Presenting as Walking Corpse Syndrome

Luma,

Challakere,

Mandap

et al. 2023

Cotard syndrome, also known as “Walking Corpse Syndrome”, is a clinical entity characterized by fixed delusions that one is dead or dying. This is a neuropsychiatric manifestation of brain pathology affecting the non-dominant frontotemporal and parietal lobes, particularly the fusiform gyrus. Prior literature has indicated that the etiology of Cotard syndrome may include structural changes related to brain injury, tumors, and temporal lobe epilepsy. We now present a case in which Cotard syndrome is related to systemic lupus erythematosus (SLE). Neuropsychiatric symptoms are atypical manifestations of SLE. Delusions, hallucinations, and other psychotic symptoms can result as a consequence of the disease itself or from corticosteroid treatment. A diagnosis of SLE-induced psychosis can be elusive; however, conducting a thorough workup is crucial as untreated psychosis secondary to lupus cerebritis can worsen without intervention. We present a clinical unique case of SLE cerebritis, diagnostic challenge, and management.