Hello darkness my old friend

One of the most troublesome symptoms of PSP is photophobia.  That sounds like a psychiatric condition, but it’s when bright light is uncomfortable or even painful, and it occurs sooner or later in nearly everyone with PSP.  In a few, it’s one of the first symptoms, manifesting in some cases as difficulty watching a brightly spot-lit performer on an otherwise dark stage and progressing to an intolerance even for standard indoor lighting. 

The explanation that I’ve long accepted starts with insufficient blinking, then drying of the surface of the eye, then inflammation, then pain when the pupil attempts to constrict to light.  But I’ve recently learned that it also may be a direct neurological effect of the PSP disease process.  https://pubmed.ncbi.nlm.nih.gov/18328771/  Supporting this theory is the observation that photophobia is a very consistent symptom of benign essential blepharospasm.  That’s where the eyes blink or clench shut involuntarily, with no other neurological issues.  Blepharospasm also occurs as a very frequent component of PSP, suggesting that blepharospasm itself, whether part of PSP or not, includes photophobia without implicating eye surface drying.

Whatever the cause, photophobia can be a very early and important feature of PSP.  But someone with PSP experiencing photophobia should still look for other, more easily treated, causes.  An article by Dr. Thomas Buchanan and colleagues at the University of Utah reviews the diagnosis and treatment of photophobia in the Journal of Neuro-Ophthalmology. (I know you all await each issue eagerly.)

They reviewed the records of every patient with photophobia seen at their center over a 9-year period, finding that 10 patients (9% of the 111 adults) had PSP.  The only disorders accounting for larger percentages were migraine (54%), dry eye syndrome (36%) and eye trauma (8%).  (These total more than 100% because some patients had more than one cause listed by their physicians.)

The article provides a thorough list of disorders causing photophobia.  I’m not going to define these for you, but I suggest you look through the list, hopefully with the advice of your doctor, as some of them have specific treatment.  Of course, in the population of those who already have PSP, the likelihood of any of these other conditions as the cause of their photophobia is very low. 

This list is adapted from: Buchanan TM, Digre KB, Warner JEA, Katz BJ. The unmet challenge of diagnosing and treating photophobia. Journal of Neuro-Ophthalmology: 3/25/2022. 10.1097/WNO.0000000000001556 

Causes of Photophobia

Seeking a treatable primary cause is all well and good, but that takes time, so aggressive treatment at the symptomatic level is the place to start.  The best shaded glasses for photophobia aren’t standard, green sunglasses, but FL-41 tinted glasses.  Those are rose-colored, and you’ll just have to endure jokes about your new outlook on life.  If for some reason you find the green glasses more comfortable, don’t wear them indoors, as your eyes will adapt to the dark and become extra painful when you return to the outdoors. 

Just in case your photophobia is caused by eye drying, lubricant drops, especially those with forms of cellulose, may provide relief.  More aggressive measures include certain medicated eye drops, gel tables inserted in the lower lid, or petrolatum-based lubricants.  Surgical options are available as well, though none of them has been formally tested in people with PSP. 

There’s a specialty, believe it or not, called “neuro-optometry.”  Those folks are usually easier to get an appointment with than a general ophthalmologist or neuro-ophthalmologist and may be more comfortable managing chronic, PSP-related problems like photophobia.  Furthermore, they don’t do surgery themselves, so they are a good source of unbiased advice on that score.

Long PSP

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.