Rapid Progressive Dementia Presentation and Leptomeningeal Enhancement in Antiphospholipid Syndrome (APLS)
Objective:
Presenting rare presentation and radiologic findings in APLS
Background:
APLS is an uncommon cause of vascular dementia. Venous thrombosis usually presents as an ischemic stroke. APLS is considered in the setting of idiopathic thrombosis, recurrent pregnancy loss, unexplained coagulopathy, and/or microangiopathy. However, a small portion of APLS patients experience insidious neuropsychiatric manifestations and cognitive decline. This case report discusses a patient with an unusual manifestation of APLS, suffering from subacute cognitive decline, more rapid than typically seen in these patients.
Design/Methods:
Cases and literature review
Results:
A 53-year-old woman with hypertension presented with progressive cognition decline and behavior change over 6 months. Her family noticed progressive cognition and function decline without self-insight. Neurologic examination was notable for, inattention, executive dysfunction, and memory recall impairment, but no aphasia or focal sensory-motor deficits. MOCA scoring was 18 out of 30. CT of the head showed chronic microvascular disease, but an MRI of the brain showed multifocal chronic and subacute infarction with bilateral leptomeningeal enhancement. CT angiogram of the head and neck showed segmental stenosis on bilateral vertebral arteries. Subsequent DSA did not show a cerebral vasculitis pattern or thrombosis but atherosclerosis in the basilar artery, left anterior choroidal artery, and vertebral arteries. Extensive serum and cerebrospinal fluid workup for autoimmune diseases, hypercoagulability, and malignancy was negative, except for postivie anti-β2-glycoprotein antibodies. Repeat MRI of the brain showed persistent leptomeningeal enhancement. The patient was started on warfarin, but she continued to decline cognitively, requiring assistance with all of her activities of daily living.
Conclusions:
This case reports demonstrates that APLS can present as rapid progressive cognition decline without other previous neurologic events. In this case, extensive leptomeningeal enhancement was found, possibly related to the patient’s cognitive decline. This disease should be considered in a differential diagnosis of rapidly progressive dementia patients.