We thank Fabbrini et al for their comments on our article.1 We will be interested to read their study when it appears in a format other than the single abstract2 published 2 years ago. However, because their comments demonstrate that they misunderstood the main message of our article, we believe that we may not have presented our case clearly enough. We did not mean to state that 80% of patients with progressive supranuclear palsy (PSP) have hypertension, based on the rigid definition of hypertension. Rather, we intended to stress that very early in the disease, before motor symptoms are present, in 80% of patients with PSP a transient phase of autonomic dysfunction marked by high blood pressure can be found from careful study of their charts and medical histories. This phase is entirely regressive and is discarded in more than two thirds of patients with PSP, and it is validated and treated in 25%. Two thirds of these patients would not be diagnosed as having hypertension in a retrospective registry, and the patients themselves would not report their symptoms in most cases. We were only able to diagnose patients with PSP as having hypertension based on our particular interest in this field and our careful inquiries of the patients' general practitioners and family members or caregivers. Because the declines in the blood pressure levels of our patients were so spontaneous, their family physicians had either been reluctant to talk about their symptoms or had believed that the findings were irrelevant. In our series, 10 (25%) of 42 patients, the same percentage that was reported in the abstract by Fabbrini et al2 and in other studies,3,4 were considered truly hypertensive and presymptomatic and were receiving or had received antihypertensive medication. Our study involved more than simply looking for hypertensive patients in our registry. We were able to identify hypertension in patients with PSP based on the atypical early symptoms of the disorder (eg, falls, unexplained changes in mood or personality, increased reflexes, etc) that prompt the general practitioner to look for cerebrovascular disease rather than other subtypes of parkinsonism in the presymptomatic period. This is our message. Perhaps a better title for our article would have been "Presymptomatic, Generally Transient, Short-Lived Hypertension in Progressive Supranuclear Palsy," which was our first choice, but we felt it was too wordy.
Ghika J. Presymptomatic Hypertension in Progressive Supranuclear Palsy—Reply. Arch Neurol. 1998;55(8):1154-1155. doi: