By Curtis Copeland, MD; Brian Kay, DO; Mehran Kashefi, DO; Scott Oh, DO; Michael Lewis, MD; Tom Howard, MD; and Jaime Betancourt, MD
Dr. Copeland is a resident PGY-3 in the Department of Anesthesiology, Dr. Kashefi is an assistant professor of medicine, Dr. Oh is an assistant professor of medicine, Dr. Lewis is an assistant professor of surgery, Dr. Betancourt is an assistant professor of medicine, all in the David Geffen School of Medicine at the University of California, Los Angeles. Dr. Kay is a resident PGY-3 and Dr. Howard is an associate professor of pathology, both in the Department of Pathology of the Keck School of Medicine at the University of Southern California, Los Angeles. Dr. Kashefi, Dr. Oh, Dr. Lewis, Dr. Howard, and Dr. Betancourt are staff physicians at the West Los Angeles VA Healthcare Center.
Treatment of the patient in this case was complicated by his cognitive impairment. Dementia is a less common but well-documented consequence of APA syndrome. A case review of 28 patients with the APA syndrome and dementia suggests an early onset of cognitive decline with a mean age of 49 years. There may be no clear preceding history of stroke in > 50% of patients.7 Interestingly, dementia followed initial manifestations of disease by an average of 3.5 years, even in some patients receiving anticoagulation therapy.7
A nuclear medicine study of 22 patients with APA syndrome and mild neuropsychiatric symptoms demonstrated a 73% incidence of cerebral hypoperfusion (55% diffuse and 18% local) based on PET imaging despite unremarkable MRI findings.8 Extended periods of hypoperfusion secondary to arterial thromboses in the temporal and parietal lobes may have been the primary etiology for dementia in this case. As such, the coexistence of neurologic abnormalities and a hypercoagulability state warrants a thorough diagnostic workup for similar disorders, despite the higher prevalence of dementia in advanced age.
Unfortunately, this patient’s cognitive disorder prevented a timely and less invasive bedside biopsy and required a surgical biopsy for which anticoagulation therapy was interrupted. A less invasive biopsy and timelier laboratory findings may have avoided triggers, including trauma from the surgical biopsy and interruptions in anticoagulation therapy, which may have contributed to the onset of CAPS.
Author disclosures The authors report no actual or potential conflicts of interest with regard to this article.
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