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Abstract Objective Transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failure amongst older adults. The median age upon diagnosis is 75 with 90% of patients reported as men. The amyloidogenic protein is transthyretin (TTR), which is primarily produced in the liver, but is also produced in the choroid plexus and neurons under distress. There are two types of ATTR: an autosomal dominant and a wild-type (wt) variant. ATTRwt is the amyloid precursor in the cardiac depositions found in older adults. We present the first neuropsychological evaluation of a patient with ATTRwt. Method An 87-year-old right-handed White woman reported a 2 year history of slowly progressive short term memory decline. Her family noted rapid cognitive decline and paranoia approximately 6 months ago. Medical history was notable for heart failure, along with lumbar stenosis, carpal tunnel syndrome, arthritis, scoliosis, and hyperlipidemia. ATTRwt diagnosis was made during cardiac clearance for lumbar surgery. Neuroimaging documented age-related cerebral atrophy and chronic microvascular ischemic disease. Results Inspection of data revealed an amnestic presentation, with impaired confrontational naming, spatial judgment, and processing speed performances. She denied depressogenic and anxiolytic symptoms while family endorsed symptoms of apathy and executive dysfunction. Conclusions The relationship between cardiac and nervous systems has garnished increasing attention in the literature and in clinical planning. Overall, the relationship between cardiac TTR amyloid and brain amyloid beta deposition in humans is unclear. Neuropsychologists should be aware of this heart condition that is increasingly recognized in older adults and the intriguing findings implicated around the cardiac-brain axis.
Ismail et al. (Thu,) studied this question.