Orthostatic dysregulation in progressive autonomic failure--a transcranial Doppler sonography monitoring

Hemodynamic changes associated with orthostatic hypotension in one patient with pure progressive autonomic failure (PAF) were studied by a passive (70 degrees tilt of the upper part of the body) and an active orthostatic tests. Mean blood pressure (MBP), heart rate (HR) and mean blood flow velocity (MFV) during transcranial Doppler sonography monitoring of the right middle cerebral artery (RMCA) were determined after 10 minutes of rest and after 1, 10 and 60 minutes passive 70 degrees tilt or active standing. Simultaneously, plasma norepinephrine (NE) levels during recumbency and after 1 and 10 min of the orthostatic manoeuvres were measured. Stand-up tilting induced slight decrease in MBP and MFV without changes in HR. Changes of systemic hemodynamics occurred during the first minute of passive standing and they increased within the first hour however the cerebral hemodynamics remained relatively stable. Active standing was accompanied by a severe decrease in the MBP and the MFV in RMCA, and an increase in vascular resistance immediately after the upright position. The hemodynamic changes were not followed by a secondary cardiac acceleration; they increased within the first minute of active standing and evoked a syncope. During squatting as a self-selected preventive mechanism in our patient an increase in MBP and MFV in RMCA occurred. Plasma NE levels in recumbency were lower than the reference values; they decreased with 12.1% after 10 min of passive tilting and with 24.8% after the first minute of active standing. These results showed that PAF is accompanied by a severe orthostatic dysregulation during active standing, associated with a progressive peripheral autonomic deficiency and disturbed mechanisms against gravitational pooling of the blood to the lower extremities. The orthostatic autoregulation of the cerebral hemodynamics seems to be preserved.