A case study found that epilepsy and rapid cognitive decline in an 70-year-old man initially masked — and complicated — diagnosing his central nervous system (CNS) lupus, but once the disease was identified and treated with immunotherapy, his neurological symptoms considerably improved.
The study, “Neuropsychiatric Systemic Lupus Erythematosus: A Diagnostic Conundrum,” published in the Journal of Clinical Medicine Research, suggests that neuropsychiatric manifestations like seizures be considered “early events” in the course of systemic lupus erythematosus (SLE), and that early immunosuppressive therapy be considered as a way of controlling its progression.
Systemic lupus erythematosus (SLE) is characterized by autoimmunity and variable clinical manifestations. Importantly, SLE patients are at a higher risk of epilepsy than the general population — believed to be a three times greater risk — and people with both SLE and epilepsy are more prone to neuropsychiatric disorders.
The patient had a long history of neuropsychiatric disorders that were never associated to SLE. The patient was presented to the hospital due to worsening cognitive decline, and, according to the authors, upon lab work they could diagnose the patient with SLE according with the American College of Rheumatology (ACR) diagnostic criteria.
The patient, who had a 17-year history of epilepsy, poorly controlled, that was never associated to SLE, arrived at a hospital with worsening cognitive decline and leukopenia (low white blood cell count). According to the authors, lab work results led to an SLE diagnosis that followed American College of Rheumatology (ACR) diagnostic criteria.
He was then given immunosuppressive therapy — oral prednisone (a corticosteroid) once daily to start and, as an outpatient, hydroxychloroquine (an antimalarial) and mycophenolate mofetil twice daily — and considerably improved with, interestingly, his seizures and other neuropsychiatric manifestations significantly reduced. Overall, the patient became able to again independently perform daily living activities that he previously wasn’t able to do.
“This case highlights the fact that seizures and other neuropsychiatric manifestations of SLE can often pre-date other symptoms, making the diagnosis of CNS lupus a challenging one due to the lack of specific laboratory tests, non-specific neuroimaging findings, and inaccessible brain parenchyma for histological confirmation and [the] overall non-specific nature of the disease,” the researchers concluded. “We also believe that early diagnosis and initiation of immunosuppressive therapy will not only lead to better control of CNS symptoms but early immunosuppressive therapy could defer the progression of the underlying autoimmune disease.”