Progression of exaggerated blood pressure variability in annual 24-h ambulatory blood pressure monitoring associated with autonomic dysfunction: a pathological case report.

This study describes the case of an 83-year-old man with long-standing hypertension, urinary dysfunction, and constipation. Annual 24-h ambulatory blood pressure monitoring revealed increased blood pressure variability and reduced nocturnal dipping. One year earlier, exaggerated blood pressure fluctuations and postprandial hypotension had emerged, which persisted despite the reduced use of antihypertensive medication. The tests confirmed orthostatic and postprandial hypotension and low heart rate variability, indicating autonomic dysfunction. Cognitive function was preserved and the patient's physical function remained intact. Imaging findings suggested prodromal dementia with Lewy bodies, although the typical symptoms of this disease were absent. Blood pressure control became difficult and  stabilized after development of chronic renal failure. The patient died of colon cancer and renal failure. An autopsy revealed widespread α-synuclein in the peripheral nerves and cardiac autonomic degeneration, with mild loss of neurons in the substantia nigra but severe degeneration in the locus coeruleus. Lewy body pathology slowly spreads to the hippocampus and neocortex. These findings demonstrated that exaggerated BP variability in ambulatory blood pressure monitoring could be early marker of the progression of autonomic dysfunction, caused by prodromal body-first-type of α-synucleinopathy, before appearance of neurological symptoms.