What Is PML?

When I first tested positive for HIV in 1997, I was throwing a cocktail/dinner party that night; my best friend was moving to Boston the next day.  As I went about preparing hors d'oeuvres he pleaded with me to cancel the party.  I said, “why?”


Knowing the source of one’s own mortality is wildly freeing; it puts things into perspective, and a million tiny grievances, worries, and pointless questions — things that go to make up a life — just fall away, in the flash of a diagnosis.  As an adult, I’ve never known a world without HIV/AIDS.  While certainly not “wanting” to contract the big disease with the little name, I think I always thought of it as my fate, as a foregone conclusion that I could not escape.  I took the necessary precautions, and insisted on the same from my sexual partners (legion though they were not), but the inevitability of hearing “your test came back positive” brought me an odd sense of calm.  One chapter was closing; it would be nearly ten years before the next began.

Looking back on the long winter of 2006-07, I did not foresee the radical life changes awaiting me.  The first “hint” something was very very wrong was 3:00 PM, October 4, 2006; I’d been limping for months, but convinced myself I just needed a new mattress and had been sleeping on my leg funny.  I finally gave in to friends who had been haranguing me for weeks to “get it checked” and saw Dr. Scarsella that day (after canceling on him two days prior to buy my second shiny new Nissan Maxima).  He didn’t seem all that concerned, but wanted me to come back Friday the 6th to see a neurologist.  “Um, I didn’t go to medical school, but I have a limp, not a headache,” I said, hiding a growing sense of panic behind my knowledge of the body’s anatomy and medical specialties.  Dr. Young, the neurologist, tapped my knees with tiny little rubber tools and asked me to play “Pat-a-Cake” with him; it seemed silly, but I was frightened and I had to trust he knew what he was doing — so I said, “you went to school for this?”  Within two months, I resembled a bowl of fresh fruit — juicy, colorful, but not all that exciting.  I could not stand on my own or take two steps unassisted.

One crisp December Saturday morning, my dad got me an Egg McMuffin from McDonald’s (one of my favorite things in the world).  My left arm was useless, so I gripped it in my right hand and proceeded to smash it into my nose, repeatedly (although, obviously, I was aiming for my mouth).  Maybe I should call Dr. Young, something’s not right. “Um, what's that you say?  Brain surgery?  Are you insane?!  That’s like the Cadillac of surgeries!  Besides, I just want my Egg McMuffin — it’s just my right arm…

Surgery was not fun, but the Hydromorphone (better known as Dilaudid or “hospital heroin") was amazing.

Dr. Young came into my room to deliver the news.  “It’s PML Matt, like we suspected, is there anyplace on earth you always wanted to go but haven’t?”  “Well, yes, now that you mention it, London, uh… why do you ask?  And what is PML?  Why do doctors always speak in cutesy sounding acronyms — AIDS almost sounds like it should be helpful, wouldn't you agree?” “This is serious Matthew.”  “Yah, gathered that from the bucket list question; am I going to die, should I be throwing a nutty?”  “Well, normally I’d give you six months, but it took us three months to find this, and the JC virus did a lot of damage during that time, so I’m going to go with two-to-four.”  “Whoa there, slow down, you just said it was PML… now you’re talking about something called JC — what the hell is that?” “It stands for Jesus Christ.”  “Cool your jets there Turbo, not laughing.”  “It causes PML, it is destroying your cerebral cortex, it is inoperable, the best we can hope to do is slow it down with medication, survival rates have improved.”  “That’s just ducky, how many patients you got with this alphabet soup of a disease?”  “That lived?”  “No Einstein, tell me about the dead ones, that would be really helpful.”  “You’re my fourth in twenty years; it’s very rare — people don’t survive this, by the size of the lesion you should be dead already.”  “We really need to work on our bedside manner, don’t we?”

JC virus is an opportunistic infection, and anywhere from 86% to 90% of the general population (i.e., that would be you!) has antibodies for it but it usually remains latent, causing disease only when the immune system has been severely compromised.  PML is a demyelinating disease caused by the JC virus, in which the myelin sheath covering the axons of nerve cells is gradually destroyed, impairing the transmission of nerve impulses.  Think of a frayed electrical cord.

Progressive Multifocal Leukoencephalopathy

pathy:  progressive damage
leuko:  of the white matter
encephalo:  of the brain at

multifocal:  multiple locations

It targets white brain matter, which is mostly comprised of axons from the outermost part of the brain — the cortex.  In the opening months of 2007, things looked very grim indeed for me.  My CD4 cells (T-Cells) had dropped below the threshold where HIV infection becomes AIDS and their downward trend had my doctors at Cedars-Sinai Medical Center in West Hollywood alarmed.  The lack of pathogen-fighting T-Cells in my bloodstream gave the JC virus permission to start destroying the myelin which insulates the axons carrying electrochemical impulses in my brain, effectively blocking the brain’s ability to send instructions to parts of my body, a condition which persists today.  The most noticeable, to the general public, was the communication between my brain and the left side of my body being cut-off resulting in the inability to walk or pick things up with my left hand; unlike paralysis, my left leg and arm, my left foot and hand, “work” — I just can’t tell them what to do, a condition called Hemiparesis.  This is sometimes called left (or right) -sided “weakness,” but that is misleading, because it suggests the affected side does not have the strength to do something; on the contrary, my left side is still every bit as capable of doing everything it could do before 2007, it’s just when my brain sends out the instruction to do it, the message does not get to its intended recipient and is bottlenecked and/or blocked by the damaged axon.

The concern in early 2007 was that as more myelin was destroyed, more communication between the brain and body would be cut-off, including critical conversations like those telling the lungs to breathe in air!  Lungs not full of air are commonly found in dead people, so there was some urgency in stopping the JC virus’ destructive path in my brain.  The only problem was (and is) that scientists do not yet know how to “cure” JC virus, and so it has a tendency to spread throughout the brain destroying myelin like a neurological version of Pac-Man eating all the dots inside an enclosed maze while avoiding four colored ghosts (which represent those T-Cells I had so few of) which float around the maze and try to eat the Pac-Man before he gobbles up all the dots and wins. The more ghosts, the harder it is for the Pac-Man to eat dots while avoiding being eaten himself.


The median survival of patients with PML as a complication of HIV is from 4 to 6 months.  10% of patients survive 12 months or longer depending on management of the underlying cause, which is HIV in my case.  I am in that 10%.  In some cases, the disease slows or stops if the patient's immune system improves; some HIV patients with PML have been able to survive many years with the advent of Highly Active Antiretroviral Therapy (HAART), also known as "the triple cocktail" or "combination therapy" (a class of drugs known as protease inhibitors taken together with two other drugs called reverse transcriptase inhibitors).  So really, the only “treatment” for the JC virus is to increase the patient’s number of ghosts, er...I mean T-Cells.  The JC virus is still eating-up the myelin in my brain, but the increase in CD4 cells means more “ghosts” are chasing the Pac-Man and slowing his progress.

Immunodeficiency diseases occur when an immune system is less active than normal, resulting in recurring and life-threatening infections. Immunodeficiency can either be the result of genetic diseases, or be caused as a side-effect of pharmaceuticals, or by an infection such as Acquired Immune Deficiency Syndrome, which you know as AIDS, that is caused by the retrovirus HIV.  Helper T cells in the body regulate both the innate and adaptive immune responses and help determine which type of immune response the body will make to a particular threat.  Through regular blood testing, they are counted as a barometer of immune system health in HIV/AIDS patients (this is known as a CD4 count — I get one every three months).  In contrast, autoimmune diseases result from a hyperactive immune system attacking normal tissues as if they were foreign organisms.

Ultimately, an immune system is a collection of biological processes within an organism, such as a human being, that protects against disease by identifying and killing pathogens; mine was not functioning, and thus I developed the JC (which actually stands for John Cunningham, the PML patient in whom it was first identified, in 1965) viral infection, the PML.  It is considered a rare disorder, as the number of people affected by it, and certainly living with it as a chronic condition, as I am, is relatively small.  Recently, though, I have noticed it mentioned more and more at the end of commercials on television.

When they’re rattling-off all the potential side effects for some new wonder drug to treat psoriasis, or arthritis, or hemorrhoids… something along the lines of, “happy days in just two weeks, or you could die of this horrible brain thing, so what have you got to loose — give us a go.”  Which is generally what I call it to avoid giving a master class in neurology — horrible brain thing.  If you work in a hospital, a doctor’s office, or the like, you’re getting all of the above, minus the stuff about playing Pat-a-Cake with a neurologist.

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