24 DISCOVERMAGAZINE.COM
SOMKKU/SHUTTERSTOCK (BRAIN) AND CHAIKOM/SHUTTERSTOCKsubclinical seizure. This kind is devoid
of even subtle seizure symptoms like
body twitches and jerks. Yet beneath
the surface, the brain is erupting, like
an underwater volcano.
I ordered an electroencephalogram,
or EEG, which measures brain
activity and can detect abnormal
neuronal irings.
Doctors often anthropomorphize
diseases, talking about them as
though they are the enemy. There’s the
irritable bowel or aggressive tumor, for
example. When I saw Antonia’s EEG
results, I thought, “Angry seizure.”
It showed a series of jagged spikes.
Now I understood why Antonia
wasn’t waking up: She was having
continuous epileptic seizures.
We concluded that Antonia had the
rare syndrome NORSE, or new-onset
refractory status epilepticus. NORSE’s
cardinal symptom is continuous
seizures. The syndrome can attack
people like Antonia, who had never
had an epileptic seizure before.
Though its cause is unknown,
doctors think NORSE is related
to autoimmunity, the set of
conditions in which a person’s
immune system attacks the body.
In this case, antibodies attacked
neurons in Antonia’s brain, causing
abnormal irings and relentless
electrical storms.
Antonia remained comatose for
months, despite receiving every anti-
epileptic medication in our arsenal.
Seven medications were in doses
higher than I had ever seen. Though
she looked physically unchanged, we
knew that with each passing day, the
odds of brain recovery got worse.
The enemy was winning.
Twice we tried high-dose steroids
to suppress the immune system, but
to no avail. We also administered
donated antibodies, called pooled
immunoglobulin, hoping to jump-start
a healthier immune response. When
that failed, we exchanged her blood
plasma with that of a healthy donor,
a process known as plasmapheresis.
It did not help.
With only a handful of NORSE cases
reported in the medical literature, we
had little precedent and no protocol to
guide us. We were shooting in the dark.HAIL MARY DRUG
Antonia had been in a coma for six
months. Her brain continued to seize,
and her body had become brittle and
emaciated. She was dying.
In the ICU conference room, our
team passionately debated whether
to administer a medication called
cyclophosphamide. It’s perhaps the
most potent immunosuppressant,
often used as chemotherapy for
aggressive cancers.
If Antonia’s immune system was
causing her seizures, my argument
went, why not shut it down with
the biggest gun we have? But
colleagues worried the drug wouldrender her vulnerable to a parade of
life-threatening infections. Because
Antonia’s health was so grave, we
took a chance and injected the drug.
During the weeks she was on it, her
white blood cell count — a measure
of her immunity — dropped to
nearly zero. She had fevers, developed
pneumonia and suffered several
bloodstream infections. Her heart rate
rose and her blood pressure dropped,
pulling her into septic shock.
Eventually I handed over Antonia’s
care to another doctor. I received
almost no updates for seven weeks,
until one morning a resident said to
me, “Did you hear about Antonia?” I
braced for bad news. “She’s awake.”
I ran to Antonia’s hospital room.
She was sitting up in bed as Jaclyn
spoon-fed her oatmeal. Her daughter,
Tia, was marching around the room
holding a doll. Now age 2, Tia was
taller and more gregarious than she had
been nine months ago when Antonia
had collapsed.
Because of extensive muscle atrophy,
Antonia was mostly immobile. She
couldn’t walk or feed herself. She could
only lift her left arm 3 inches above the
bed and wiggle her toes. Her language
and cognition had also profoundly
regressed. But despite these challenges,
she could potentially reclaim her
independence through cognitive and
physical rehabilitation.
When Antonia saw me, she
studied me for a moment, then said,
“Walking?”
I assumed she was asking when she
would be able to walk. “Soon, I hope,”
I said. “We want you to be walking
when you’re strong enough.”
Jaclyn laughed. “No, no. She’s not
talking about herself.” Jaclyn motioned
toward Tia prancing about.
“Walking,” Antonia said, beaming.^ DEliezer J. Sternberg is a neurologist at
Massachusetts General Hospital. The cases
described in Vital Signs are real, but names
and certain details have been changed.hough she
looked physically
unchanged, we
knew that with each
passing day, the odds
of brain recovery
got worse. he
enemy was winning.
Vital
Signs