Central Nervous System Systemic Lupus Erythematosus (CNS-SLE) Vasculitis Mimicking Lewy Body Dementia: A Case Report Emphasizing the Role of Imaging With an Analysis of 33 Comparable Cases From the Scientific Literature

Abstract

Introduction: Neuropsychiatric symptoms occur in 30% to 40% of patients living with systemic lupus erythematosus (SLE). Brain imaging may play a pivotal role in determining the etiology as it did for the case presented here. Methods: A new case of central nervous system (CNS) SLE is presented along with an analysis of 33 comparable cases from the scientific literature. Results: A 70-year-old female with subacute cutaneous lupus presented to a university-based geropsychiatry program after 1 year of benign visual hallucinations and several months of shuffling gait, recurrent falls, and forgetfulness. These symptoms were highly suggestive of Lewy body dementia; however, the patient’s history of basal ganglia infarct, cognitive testing demonstrating inattention and executive dysfunction, and follow-up brain imaging, which did not reveal acute findings, aligned with cerebral pathology previously attributed to vasculitis and supported the diagnosis of subcortical dementia due to SLE-CNS vasculitis. Oral prednisone 20 mg daily resolved her symptoms. Over the next 19 months, her prednisone was tapered completely and her symptoms did not return. A systematic literature search identified 33 comparable cases. Conclusion: An analysis of previously published cases suggests that extending the duration of the prednisone taper beyond 1 year may decrease the risk of later occurring neuropsychiatric symptoms in this patient population.

Autoimmune encephalopathies presenting as dementia of subacute onset and rapid progression

“…Neurologic involvement of a rheumatologic disorder can occasionally, initially or during its course, manifest with a subacute dementia syndrome. 61,62 Thus, serum testing for non-neural antibodies such as antinuclear antibody, doublestranded DNA antibodies, antibodies to the extractable nuclear antigen, anti-neutrophilic cytoplasmic antibodies (ANCA), rheumatoid factor, phospholipid antibodies, beta 2 glycoprotein-1 antibodies, lupus anticoagulant, and IgG4 can be considered when clinical features are suggestive to assess for neurologic involvement of rheumatologic disorders. 63 CSF analysis may show evidence of inflammation, including pleocytosis, elevated oligoclonal bands, and elevated IgG index, in up to 50% of patients.…”

Tumor-like Presentation of Cerebral Vasculitis in a Patient With Systemic Lupus Erythematosus: A Biopsy-confirmed Case

“…Early biopsy is imperative to distinguish vasculitis [ 1 ]. Even highly sensitive imaging modalities continue to be developed as imaging in CNS vasculitis is often nonspecific and some patients with NPSLE demonstrate normal MRIs [ 12 ]. The most common finding in SLE vasculitis is the presence of transmural lymphocytic infiltrates with fibrinoid necrosis and either hemorrhage or thrombosis.…”

Theoretical Foundations of Latent Aging and Imitation of Aging

“…Anxiety, depression and neuropsychological effects may occur in the individual when painful symptoms, muscle and joint pain, and skin rashes occur in prominent parts of the body in many organs (Abraham et al, 2020;Conceição et al, 2019;Moustafa et al, 2019 [37-39]). These negative symptoms have a significant cognitive effect on the individual.…”