D R O W

he 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 pow-

erful strokes. Then, suddenly, he stops. He exhales, sinks to the

bottom and simply stares straight ahead. “I’m drowning,” he real-

izes, strangely unperturbed. He knows that a few strong kicks would bring him

back to the surface. But he can’t 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 don’t know

what was wrong with me. I just didn’t want to swim anymore.”

What was happening in Mr. M’s 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 d’auto-activation psy-

chique, or “loss of psychic autoactivation.” (Subsequently, other experts have also

labeled the condition “loss of mental self-activation” or “athymhormic syn-

drome.”) 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.

BY PATRICK VERSTICHEL AND PASCALE LARROUY

He knows he is suffocating at the bottom of the pool, but he just doesn’t feel like swimming right now

N I N G M R. M

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40 SCIENTIFIC AMERICAN MIND

Yes, I’m StarvingWithin only a few weeks after the pool inci-

dent, Mr. M’s 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 inter-est them. Their utter spiritlessness extends even to fundamental needs; Mr. M’s wife said her hus-band 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 exam-ined 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 ratio-nal answers about their strange behavior. PAP patients also pass intelligence and memory tests, as long as the examiner keeps urging them to con-tinue. Unfortunately, the effects of external stim-uli 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 re-spond, “Nothing.” Is that even possible—to be fully awake yet not thinking about anything for hours on end? Evidently so: patients generally de-scribe their mental state as “empty.”

Surprisingly, they do not suffer psychologi-

cally from this inertness. They are almost inca-pable of experiencing emotions. A once fun-lov-ing, now fully apathetic 70-year-old teacher de-scribed her reaction to the death of her nephew this way: “It’s quite tragic. Before, I would have been totally devastated. But now, it’s 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 intel-lectual nature than actual feelings.

Some patients develop obsessive behavioral disorders—senseless, 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 pro-fane words. The cause of these pointless patterns is not known, but perhaps the brain is attempting to fi ll the mental emptiness.

Motivation Switched OffPAP 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 in-ternal signals necessary to survival as well as so-cial, moral and civil obligations—the so-called higher aspects of motivation. In addition, they are unable to see themselves in any kind of future scenario and cannot comprehend the consequenc-es 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 bas-al 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. M’s 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 re-gions (which tell muscles to move). The basal gan-glia also connect to the frontal lobe, where prob-lem 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 consid-erably less active than it is in healthy subjects.

(The Authors)PATRICK VERSTICHEL and PASCALE LARROUY have stud-ied several PAP patients together. Verstichel is a neurolo-gist at the Centre Hospitalier Intercommunal de Créteil in France. Larrouy wrote her doctoral dissertation on brain pathways that might cause PAP syndrome. C

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People who have experienced other kinds of damage to just the frontal lobe have symptoms similar to those of PAP patients. They, too, are apathetic and fail to organize activities for the fu-ture. Together the basal ganglia and frontal lobe steer motivation and therefore an individual’s will. The basal ganglia determine whether or not the frontal lobe should be activated. They act as a “switch” that can turn on or off our desire to act.

But if the connection between those structures has been impaired, why do PAP patients still act on external stimuli such as a daughter’s face or a wife’s command? Because other pathways can also affect motivation. For example, the frontal lobe can be directly activated by certain areas of the cerebral cortex, including the language cen-ters. When Mr. M’s family members speak to him, the language stimuli travel not only to the limbic system but also to the language areas in the frontal lobe. Having been activated in this way, the fron-tal lobe can make a determination and prompt Mr. M to eat or take a shower. For a moment, he can reconnect with his normal life, thanks to the intervention of a personal prompter.

Then it is back to the couch. Or the bed.PAP syndrome is relatively rare, so little re-

search has been done on how to aid these hapless people. It is unclear whether certain psychotropic drugs can help. Although to observers a victim’s symptoms may seem to mirror depression, most patients, such as Mr. M, do not seem particularly upset about their apathy, so they may not be de-pressed in the clinical sense or respond to com-mon antidepressants. And it may be hard to help patients whose symptoms have been brought on by a brain-damaging event such as a stroke until medicine fi nds a way to compensate for such dam-age. More research is needed into ways to relieve PAP symptoms. As is sometimes the unfortunate case with people who suffer psychiatric ills, med-icine has little to offer, and families or friends of PAP patients may have little choice but to con-stantly prod their loved ones along.

Aspecial neural network called the limbic loop (left) drives our decisions about whether or not to act on external and internal stimuli. Sensory information

travels to various parts of the brain’s limbic system (pur-ple). Here the data are evaluated on an emotional level, and assessments (orange) move through the basal gan-glia to the cingulate gyrus. From there, assessments land in the frontal lobe, which makes a determination. The bas-al ganglia structures act as an on/off switch—they deter-mine if the frontal lobe is to be activated or not.

In patients with PAP syndrome (right), the limbic loop is damaged: the basal ganglia do not ferry information

through to the frontal lobe. Without input, it cannot make a decision to act. The various stimuli carry no emotional importance, so the patient’s motivation or will is not ac-tivated. Patients can act when spoken to directly; lan-guage information jumps from Wernicke’s area (one of the brain’s language centers) directly to the frontal lobe (red arrow), bypassing the limbic loop.

In people suffering from depression, the caudate nu-cleus does not function properly, which dampens respons-es in the frontal lobe. For some schizophrenics, abnor-malities in receptors that respond to the neurotransmitter dopamine decrease the limbic loop’s effectiveness.

When Motivation Dies

Normal state

Frontal lobe

Cingulate gyrus

Basal ganglia

Caudate nucleus

Thalamus

AmygdalaHippocampus

Visualcortex

Parahippocampal gyrus

(Further Reading)◆ Affective Disorders Due to the Loss of Mental Self-Activation:

Comparison with Athymhormia. D. Laplane and B. Dubois in Review of Neurology, Vol. 154, No. 1, pages 35–39; January 1998.

PAP syndrome

Frontal lobe

Wernicke’s area

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