The summer heat is oppressive. Mr. M, seated beside his pool, looks
at the cold water. What could be better than a refreshing dip? he
thinks. He dives headfi rst into the water and takes a couple of powerful
strokes. Then, suddenly, he stops. He exhales, sinks to the
bottom and simply stares straight ahead. Im drowning, he realizes,
strangely unperturbed. He knows that a few strong kicks would bring him
back to the surface. But he cant quite bring himself to do so.
As luck would have it, his daughter has been watching from inside the house.
She runs out and dives into the pool to save him. The sight of his daughter shakes
Mr. M from his apathy, and just as she reaches him he propels himself upward,
breaking the surface and gasping for air. Later he tells his family, I dont know
what was wrong with me. I just didnt want to swim anymore.
What was happening in Mr. Ms brain as he came within seconds of drowning?
How could he so abruptly lose all desire to act, even to save his own life?
Neurologist Dominique Laplane fi rst described such bizarre behavior in 1981.
A doctor at the Hôpital de la Salpêtrière in Paris at the time, Laplane called the
phenomenon PAP syndrome, from the French perte dauto-activation psychique,
or loss of psychic autoactivation. (Subsequently, other experts have also
labeled the condition loss of mental self-activation or athymhormic syndrome.)
Since then, scientists have come to learn that damage to certain areas of
the brain causes patients to lose their motivation as well as their ability to reach
decisions. It is as if they have become mere spectators to their own lives, no longer
actively participating. By examining the brains of these patients, researchers are
fi nding initial clues to how willfulness arises in all of us.
Yes, Im Starving
Within only a few weeks after the pool incident, Mr. Ms personality underwent a drastic change. The normally active and energetic man became increasingly passive and apathetic. He spent entire days in bed yet felt neither boredom
nor impatience. His family had to remind him constantly to carry out the most basic activities: Come to dinner! Get dressed! Take a shower! Such complete lack of motivation is the most obvious symptom of PAP syndrome. If left to their
own devices, patients will remain in bed or on the couch for hours or even days, doing nothing but lying there awake or asleep. They do not make any plans for the future. Hobbies no longer interest them. Their utter spiritlessness extends even
to fundamental needs; Mr. Ms wife said her husband would have starved to death had she not intervened. Yet he never complained of hunger. Incredibly, PAP patients do experience hunger and pain. They simply lack the will to react. Such inaction injured one 18-year-old woman examined at the Hôpital de la Timone in Marseille,
France. During a visit to the beach, her parents had left her sitting in the shade while they went on an afternoon trek. As the sun moved across the sky, the woman became exposed to the scorching rays and remained there for several hours. She felt
the heat but did not make any effort to take cover and suffered second-degree burns.
PAP patients require external stimuli to spur them on. Once they are encouraged, however, they can carry out complex activities as well as they once had. The patients do not often speak, but when asked direct questions they offer rational
answers about their strange behavior. PAP patients also pass intelligence and memory tests, as long as the examiner keeps urging them to continue. Unfortunately, the effects of external stimuli are only temporary. Soon enough, patients
revert back to silence and apathy. What is going on in these patients heads?
What are they thinking? PAP patients often respond, Nothing. Is that even possibleto be fully awake yet not thinking about anything for hours on end? Evidently so: patients generally describe their mental state as empty. Surprisingly, they do not suffer psychologically from this inertness. They are almost incapable
of experiencing emotions. A once fun-loving, now fully apathetic 70-year-old teacher described her reaction to the death of her nephew this way: Its quite tragic. Before, I would have been totally devastated. But now, its really not such a big deal. Although patients recognize tragic or joyous occasions as being such, they can
no longer sense or express sadness or joy. Their feelings, Laplane notes, are more of an intellectual nature than actual feelings. Some patients develop obsessive behavioral disorderssenseless, repetitive activities such as repeatedly turning a light or the television on and off. While lying in bed, one patient could not stop
himself from continuously counting the ceiling tiles. At times patients irritate people around them with verbal tics, such as constant use of profane words. The cause of these pointless patterns is not known, but perhaps the brain is attempting
to fill the mental emptiness.
Motivation Switched Off
PAP syndrome brings to light an important question facing brain researchers today: How is motivation created to trigger behavior? In PAP patients such as Mr. M, motivational mechanisms seem completely inactive. The patients ignore internal
signals necessary to survival as well as social, moral and civil obligationsthe so-called higher aspects of motivation. In addition, they are unable to see themselves in any kind of future scenario and cannot comprehend the consequences
of their inactions. Using processes such as magnetic resonance
imaging (MRI), researchers have recently begun to unveil the secrets behind this condition. So far in every case of PAP syndrome, an acute illness
has been found that affects some area of the basal ganglia deep inside the brain. The ailments have varied from lack of oxygen caused by clogged blood vessels to carbon monoxide poisoning. Two large tumors were discovered in Mr. Ms brain; the larger of the two, in the left hemisphere, was putting pressure on his basal ganglia. The basal ganglia are long, thin structures that have strong connections to the pathways that bring information from sensory organs to the motor regions (which tell muscles to move). The basal ganglia also connect to the frontal lobe, where problem
solving, planning and decision making are done. MRI studies show that in many PAP patients the frontal lobe is not functioning properly. When working on thought exercises, this area is considerably less active than it is in healthy subjects.
(The Authors)
PATRICK VERSTICHEL and PASCALE LARROUY have studied
several PAP patients together. Verstichel is a neurologist
at the Centre Hospitalier Intercommunal de Créteil in
France. Larrouy wrote her doctoral dissertation on brain
pathways that might cause PAP syndrome.
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