Self-management of Fatal Familial Insomnia. Part 2: Case Report

Joyce Schenkein, PhD; Pasquale Montagna, MD

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In This Article

Neuropsychological Insights

DF's resourcefulness in implementing treatment is in itself testimony to the essential preservation of intelligence in patients with FFI. Although formal neuropsychological testing was not performed, a gross assessment can be offered.

Stimulant medication greatly improved DF's attention. Without it, DF didn't know that the phone was ringing. With medication he could safely drive 60 mph for the entire day. Word finding was preserved; humor remained intact. The need to write "to do" lists reflects memory difficulties, whereas the foresight to write such lists and follow their instructions indicates executive efficiency. Subjectively, DF reported his greatest area of confusion to be temporal ordering; he could not keep track of time or days. He tended to speak on the phone for several hours without any notion of duration. However, enormous concentration was required for oral fluency. DF believed that his judgment was impaired. Late in his illness, he provided a needy family with a car by purchasing 2 vehicles and giving them a choice. This left him with a truck that he didn't need.

Unlike the typically mute FFI patient whose subjective serenity is unknowable, DF described his oneiric sleep as extremely gentle and pleasant — like entering a room filled with everyone who he would want to encounter, including deceased friends and relatives who would tell him that everything will be all right. In his words, "to the outside world, I am dead and gone, but to myself, I'm still here, in this wonderful place and it is they who have disappeared."

His "waking REM" was multisensory and included images, voices, and scents. It was experienced as a form of knowing everything about himself, with no more hidden secrets. As might be expected from a sustained "handshake" between the right and left hemispheres, DF's conscious mind experienced himself in a global way. He described his unconscious as filled with "wounded children" who bore "poor witness" to events that had injured them — unable to logically evaluate or rise above these damaging experiences. His FFI put him in the unique position to soothe these children with adult insight, which he often did in the form of written letters when he was "off-line." (Those interested in psychoanalytic theory and/or multiple personality disorder may learn a great deal from FFI patients).

The door that admitted DF into this other world became best defined after long periods of insomnia and was so inviting that he believed that others who have been in this place simply gave into it and allowed themselves to die. In fact, DF's fight against FFI specifically centered on this arena, with the wish to surrender to its serenity as opposed to his real life of handicap and degeneration.

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