After he suffered a stroke in 2005, Tony Nicklinson developed locked-in
syndrome, a rare condition that left the middle-aged Brit fully paralyzed from
the neck down. He lived on, mentally alert but wholly incapable of taking care
of himself.
He could not walk, feed himself
or brush his own teeth. Devastated when a British court refused to allow him to
commit assisted suicide, Nicklinson stopped eating or accepting fluids. He
developed pneumonia, refused antibiotics, and died this past August 22, 2012.
Although news bulletins focused on his legal efforts to be permitted to
commit assisted suicide, Nicklinson’s tragic disability — seven years of what
his wife called a “living nightmare” — also bears upon stroke awareness and the
availability of the emergency treatment that too few people know about or
receive — the clot-busting drug, tissue plasminogen activator (tPA). Locked-in
syndrome has something to say to everyone at risk for stroke. So does tPA.
In 2005, Stephan Mayer MD,
together with colleagues at the Columbia University College of Physicians and
Surgeons, reported on a unique case of heroic treatment to prevent locked-in
syndrome in a stroke victim.
tPA and a Case of Locked-In
Syndrome
Mayer’s patient, the pastor of a
well-known church in Manhattan, suffered from a “stuttering course” of
brainstem ischemia that lasted days. He first went to the emergency room some
10 hours after he began to experience facial numbness and right-side
weakness. A history of neck pain suggested a vertebral dissection, or tear
in the lining of the main artery that supplies blood to the brain. Transferred
to the neurointensive care unit (Neuro-ICU), his symptoms were varied and
ominous. First the left arm would become weak, afterwards the right; then one
side of his face would become paralyzed. To insure he could breathe, he had to
be intubated.
“We realized he was in the early
stages of an evolving basilar artery syndrome,” recalls Mayer, “the final
result of which, in the worst case, you infarct your whole pons and become
locked-in.” Patients who end up in a complete locked-in state remain conscious
but are completely paralyzed save for the vertical gaze. The condition is
widely recognized as a fate worse than death.
Over two days Mayer presided over
the patient’s disrupted “low flow state” in the occluded basilar artery of his
brainstem. He administered heparin, an anticoagulant, and artificially
raised his blood pressure but neither measure had any appreciable effect. An
angiogram showed blood seeping into the basilar artery and small fragments of
clotted blood. Occlusion of both vertebral arteries shut down the possibility
of a mechanical solution such as angioplasty.
Suddenly, on the second day in
the ICU, the patient became totally quadriplegic. Efforts to reverse it failed.
Mayer went to the patient’s wife.
“I said, ‘We’re losing him. He’s
going to develop this locked-in syndrome. We’ve got to try something.” He
added: “The one thing I can think of doing is giving tPA.”
Were circumstances less than
extraordinary, that meant breaking all the rules. “Forget the three hour [time
window for giving tPA]; this ischemic process had been going on for two days.”
Mayer was purposely keeping blood pressure high, at around 220 systolic, another
contraindication. So was the anticoagulant he administered. Finally, a
diagnosis of arterial dissection was not an approved use for tPA, which raised
genuine concern for catastrophic hemorrhage.
“Look,” Mayer told the patient’s
wife. “It’s high risk. But I don’t know what else to do. It’s a total roll of
the dice and probably won’t work. But otherwise you’re going to just stand
around and watch this guy become locked-in.”
With her approval, he
administered tPA.
“I’ll be damned,” Mayer recalls.
“About an hour later, he started to improve. He started to move both sides.”
Sensation and movement fully returned. Within days he would walk out of the
hospital.
“From a biological point of view,
he was thrombosing [developing blood clots],” recalls Mayer. “By giving the
tPA, it was just enough to open everything up.”
Mayer and his colleagues went on
to write up the case, published in Neurocritical Care. They hoped to
illustrate and underscore that, “Sometimes, when you’re facing certain doom,
you can roll the dice, break the rules, as long as you have eyes wide open
about the risks and benefits.”
The contrast in outcomes between
Mayer’s case and that of Tony Nicklinson also points to the importance of stroke awareness and knowing about the use of tPA to treat stroke, now
recommended within 3-4.5 hours of symptom onset.
“I’m already dead – don’t mourn
for me,” were Tony Nicklinson’s last words before he died after seven years of
unmitigated suffering. When Stephan Mayer’s patient, who was about 60 years old
at the time of his stroke, left the hospital after beating incipient locked-in
syndrome, he took up a new email address. Its username: notdeadyet.
References
Janjua N, Wartenberg KE, Meyers
PM, & Mayer SA (2005). Reversal of locked-in syndrome with anticoagulation,
induced hypertension, and intravenous t-PA. Neurocritical care, 2 (3),
296-9 PMID: 16159079
Zivin JA, Simmons J. tPA for
stroke: the story of a controversial drug. New York: Oxford University
Press; 2011.
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