For several years, one of CurePSP’s public-facing taglines has been, “Because Hope Matters.” Last week, the CurePSP staff decided to make that advice part of the organization’s actual logo, replacing “Unlocking the Secrets of Brain Disease” at that position.
In trying to help patients with PSP, CBD and many other still-incurable diseases over four decades, I’ve found that hope really does matter. It’s not just a bit of quackery arising from medical impotence. No matter how thin that thread of hope may be, a person’s stress level, and their motivation to work with their physician to do what can be done, are greatly enhanced by some measure of hope: hope that a cure, or at least a way to halt the disease’s progress, may be found in their lifetime; hope that they will be among the minority spared some of the most disabling symptoms; and hope that their survival will beat the average.
That’s why I was most glad a few days ago to see a report from the Rossy Program for PSP Research at the University of Toronto of four patients with clinical diagnoses of PSP surviving 11, 12, 18 and 20 years after symptom onset. The average figure is typically reported as between 6.7 and 7.5 years.
Three of the four Toronto patients had confirmation of PSP at autopsy. The fourth had a very rare condition called pallido-nigro-Luysian atrophy (PNLA), a tauopathy that often mimics PSP and is equally resistant to treatment, but has a much slower course.
Sidebar: I first learned about PNLA in 2004, when I was invited by the journal Movement Disorders to discuss a “clinicopathological correlation.” That’s a teaching exercise where the organizer selects a patient whose diagnosis was difficult or impossible to make during life but whose autopsy gave the answer. The organizer sends a clinical summary to a recognized outside expert or two with no previous knowledge of the patient. They each submit a written discussion and a diagnostic conjecture to be published alongside the autopsy results. The case I was invited to discuss was someone with a clinical picture that looked exactly like PSP except for a 26-year survival. The other discussant and I each independently concluded that the patient had PSP with a long survival was statistically plausible for that condition. But the autopsy showed “primary pallidal degeneration,” of which this was the eighth case ever reported in the medical literature. The pathologist mentioned PNLA as a similarly rare, closely-related condition that he considered as an alternative, but it did not fit the autopsy results quite as well. Now back to business:
Whenever I discuss expected survival duration with a person with PSP or their family, I tell them the truth – as gently as possible. But I also point out that they could beat the average survival figures, especially if they get treatment to help protect from falls, aspiration, infections, malnutrition, and emotional stress. That measure of hope provides a kind of lifeline to grasp, one now corroborated by the medical literature. So here are four more patients proven to have beaten the odds – four more reasons for hope.