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Brush with Greatness: Stanley Prusiner

Posted in Online First,Special Feature on 3rd Jun 2019

Rajith de Silva, Consultant Neurologist, BHR University Hospitals NHS Trust, Queen’s Hospital, Romford, Essex, UK
Conflict of interest statement: None declared.
Date first submitted: 22/02/19
Acceptance date: 25/02/19
Published online first: 3/6/19

The year was 1994, and I had just completed my stint in research in Creutzfeldt-Jakob disease (CJD) with Bob Will, at the National Surveillance Unit in Edinburgh, UK.  Some of the work I had done with the team had been accepted for presentation at a joint ABN/ANA conference in San Francisco.  I set off proudly, clutching two posters, accompanied by one of the Neuropathologists from the unit – James Ironside.  San Francisco was of course the base of Stanley Prusiner, who had proposed the “prion” hypothesis, as the explanation for some of the unique aspects of the spongiform encephalopathies.  At the time the theory was still under scrutiny and the subject of much debate (although a few years later Prusiner was partially vindicated by the award of the Nobel prize in Medicine; the same mechanism is now thought to underlie many more (possibly all?) degenerative disorders affecting the brain associated with abnormal proteins, including (perhaps) Parkinson’s disease).  It seemed appropriate that James and I should visit Prusiner in his laboratory, during our visit.  It smacked a little of paying homage, but we were excited by the prospect of meeting this controversial character, and it was simply too good an opportunity to miss.

The conference passed without event, and my posters were accorded the appropriate lack of interest they deserved.  Undeterred, James and I set off for Parnassus Heights one October morning, to the UCSF campus there.  Prusiner breezed in, a few minutes late, but was polite, genial and duly condescending.  We had gone armed with more data from the unit.  Variant CJD had not yet appeared, but James, Bob and others were already engaged in an intensive (and ultimately rewarding) surveillance exercise.  We showed Prusiner the data we had been collecting, and he listened patiently to us, rather like a kind Montessori teacher.  His time was clearly precious, and after half an hour, he indicated that our time was up by looking at his watch very deliberately.

We hastily concluded our chat, and got ready to leave.  Prusiner, perhaps feeling a little sorry for us by then, started asking what we planned to do the rest of the day.  The conference organisers had arranged various excursions after the meeting, one of which was a walking tour of San Francisco’s fabled Chinatown.   Our meeting with this famous scientist took a surreal turn at this point, when I explained that I was hoping to join this tour that afternoon.  Prusiner reposted by asking what time I needed to be at the airport.  I looked at him curiously, but also with some bewilderment.  Did he not know that there was a Chinatown in his own City, and that you didn’t need to fly to get there?  Was he really the great man…and, crucially, should he be a Nobel prize contender?  It dawned on me that he had not understood my British-Sri Lankan accent, and thought that I was going to visit China!  More explanations followed, and we departed, flushing.

Prusiner did receive the Nobel prize in 1997, and many more accolades followed.  Our meeting with him took place at a heady time: the world was fascinated by these disorders and the potential impact of Bovine Spongiform Encephalopathy on humans.  Neuroscientists too were intrigued by “prion” biology, and the extent to which Prusiner’s theory accounted for our clinical and laboratory observations.  It was a privilege meeting him, seemingly at the height of his career, that day, although from his perspective it was a considerably more pedestrian encounter, I suspect.  However puerile, what made the memory of that meeting indelible for me was a rather embarrassing misunderstanding – one which did make me query, however briefly, his brilliance!