The Man Who Mistook His Wife for a Hat

Gilles de la Tourette published the first documentation of Tourette’s Syndrome in the article “Study on a Nervous Affliction” (1885). Tourette’s is characterized by an excess of nervous energy that causes uncontrolled tics and impulsive, involuntary movements. It is considered a missing link between the schools of neurology and psychology. However, by the 1900s, scientists stopped accepting, talking about, or researching Tourette’s, believing the tics to be psychological or concocted out of researchers’ imagination. In the 1970s, Tourette’s became more widely understood thanks to the Tourette’s Syndrome Association (TSA), which registered members in the thousands.

The opposite of Tourette’s is encephalitis lethargica, or “sleeping sickness,” which is the condition of becoming almost comatose. Sacks recounts how, in 1969, he began treating encephalitic patients with L-Dopa (or levodopa, a dopamine replacement agent), which helped them regain energy but could also induce Tourette’s.

In this chapter, Sacks introduces the reader to a patient he calls Ray, who has been experiencing Tourette’s from the time he was four years old. Ray self-diagnosed his condition and didn’t know anything about the TSA or Haldol, a drug that can be used to treat Tourette’s by counteracting excess dopamine in the brain. Without treatment, Ray couldn’t hold his weekend job as a jazz drum player because of his Tourette’s. Sacks prescribes Ray Haldol, but a few days later, Ray returns with a broken nose and black eye. The Haldol reduced his tics but made him slower, so he lost his timing and ran into a door.

Sacks believes that Ray, who has never known a life without Tourette’s, needs time to prepare for life without it. Sacks halts the medication and proposes that Ray meet regularly with him. His aim is to help Ray conceive of a new life. After three months, Sacks prescribes the same dose of Haldol as before. This time, Ray reacts well and loses his tics altogether.

However, there is still a problem. While Ray leads a more functional life, he misses some of his old energy, quick-wittedness, and “killer instinct,” especially in his music. He is still able to play the drums, but he plays without as much energy, joy, or creativity. He decides to suspend his use of Haldol on weekends. Ultimately, Ray reports that neither state, being on or off Haldol, allows him to be truly free: One makes him subdued, the other out of control. Sacks reflects that Ray has learned resilience through his illness.

Natasha is 89 years old and has taken an interest in a young man. She is concerned about her behavior and goes to Sacks. She explains that she feels well, even “frisky,” but that her friends are worried. Whereas she was always shy, she has now become a flirt. Natasha says she has a sudden “euphoria” and assumes she must be ill.

Natasha has diagnosed herself with “Cupid’s disease,” or syphilis. She worked in a brothel years ago, and her husband brought her out and had her treated before penicillin was available. That would mean her illness has been dormant for 70 years. Tests indicate that she has neurosyphilis, the infection of the brain and spinal cord by syphilis bacteria. This condition presents as disinhibitions, which are causing her to feel youthful again.

In her enjoyment of her current state, Natasha doesn’t want her new disinhibition “cured,” but she also doesn’t want the syphilis to worsen. She asks if it’s possible to stabilize the deterioration at its current level. Her doctors administer penicillin, which kills the spirochetes but doesn’t counteract her personality changes.

Postscript

Sacks has since seen a similar dilemma in Miguel O., a farmhand working in Puerto Rico. Miguel has neurosyphilis, in addition to speech and hearing disabilities, but he draws with great imagination. When Sacks sees Miguel, the patient demonstrates “excess” energy and excitement. Sacks asks the man to draw, which he does with fervor. After Haldol treatments, Miguel can still draw, but he does so without joy. He asks Sacks if he will always feel this “dead,” and Sacks responds that his reaction is typical. Patients can be treated with L-Dopa and gain energy, imagination, and a sense of aliveness, but they may then lose those elevated feelings. Sacks relates Miguel’s case to the introductory paradox, noting the “strange waters” where illness may now be wellness and vice versa: “What a paradox, what a cruelty, what an irony, there is here—that inner life and imagination may lie dull and dormant unless released, awakened, by an intoxication or disease!” (51). Sacks specifically observes how elevated states can be confining or cathartic.

Sacks meets with William Thompson, who recognizes Sacks as an old friend named “Tom.” Asked more about this, Mr. Thompson changes his mind and says that Sacks is not Tom, but another friend named “Chaim.” Mr. Thompson changes his mind again and declares that Sacks is a mechanic. He then sees the stethoscope and proclaims that Sacks is a doctor. Mr. Thompson is disoriented and unsure of his location. Sacks surmises that Mr. Thompson has extreme Korsakoff’s syndrome. Like Jimmie G. from Chapter 2, Mr. Thompson cannot remember his past. Unlike Jimmie, Mr. Thompson makes up stories to compensate for his lack of memory. Sacks calls this “mythomania” and compares Mr. Thompson to a man in a race chasing something he can never grasp. In addition to the memory loss, Sacks notes that Mr. Thompson has also lost his emotional affect. When Mr. Thompson connects with someone he knows, like his younger brother Bob, he has no emotional response.

Mr. Thomas doesn’t understand there is something missing in his life. Sacks compares Mr. Thompson to Jimmie G. and to Luria’s patient, Zazetsky, who also had Korsakoff’s syndrome. Unlike Jimmie and Zazetsky, Mr. Thompson seems to have lost his “soul” and his capacity for deeper feeling. Where he might have once had emotions, he now has a compulsion to tell stories and make up information to compensate for his lack of memory.

The sisters who work with Sacks try the same spiritual and creative interventions that they tried with Jimmie G on Mr. Thompson, but to no avail. One day, Sacks observes that Mr. Thompson has wandered into the garden by himself. For the first time, Sacks observes Mr. Thompson, who is serene and quiet. He surmises that Mr. Thompson can only find a sense of peace in the absence of people and in the presence of plants. The natural reprieve has temporarily restored Mr. Thompson’s “sense of being” (59).

Mrs. B.’s friends have noticed a drastic change in her personality; she has become superficial, witty, full of humor, and unable to take anything seriously. Mrs. B. has a cerebral tumor. When Sacks meets her, she calls him “father,” then “sister,” then “doctor.” She says that his beard reminds her of a priest, his white uniform of the sisters, and his stethoscope of a doctor. He asks, “You don’t look at all of me?” (60), and she responds “no.” Sacks tries to test whether she understands the differences between pairs, such as the left and the right. Mrs. B. maintains that she comprehends Mrs. B.’s friends have noticed a drastic change in her personality; she has become superficial, witty, full of humor, and unable to take anything seriously. Mrs. B. has a cerebral tumor. When Sacks meets her, she calls him “father,” then “sister,” then “doctor.” She says that his beard reminds her of a priest, his white uniform of the sisters, and his stethoscope of a doctor. He asks, “You don’t look at all of me?” (60), and she responds “no.” Sacks tries to test whether she understands the differences between pairs, such as the left and the right. Mrs. B. maintains that she comprehends the distinction, but that “differences” are “meaningless.”

Sacks is skeptical. He wonders if she really means what she says or if she is hiding a pain. He calls it a “funny, dreadful, nonchalance” (61) and remarks that she is “de-souled.” In his work, Luria describes this nonchalant effect as the result of “equalization” (61).

Postscript

The Germans call this condition Witzelsucht, or “joking disease” (61). English neurologist John Hughlings Jackson calls it a symptom of a nervous “dissolution.” Sacks notes that he has seen similar “silly-happy” behaviors in multiple patients spanning a variety of conditions. Nobody returns from these conditions to tell us what they are like. Sacks feels a horror about this, comparing it to Jorge Luis Borges’s short story “Funes the Memorious” (1942), in which Ireneo Funes remembers everything after sustaining a brain injury. Funes describes his memory as being like a “garbage-heap” (61). Sacks also alludes to Alexander Pope’s The Duncaid (1728), imparting to readers that such an extreme state of “unfathomable silliness” will result in the death of the self.e distinction, but that “differences” are “meaningless.”

In Chapter 10, Sacks describes how Ray learns to cope with his relatively mild Tourette’s syndrome. However, there are more severe forms of Tourette’s. Gilles de la Tourette noted that some Tourette’s disintegrate a personality, forcing the person to succumb to their “super-Tourette’s.”

Sacks relates seeing “Touretters” on the streets of New York. They experienced not only convulsive movements, but also convulsions of the imagination. Sacks sees the streets of New York as a better place to observe Tourette’s than in the clinic. The open location provides him insight into how a “Touretter” might interact with unpredictable stimulations in their natural environment. He calls this “street-neurology."

While on the streets of New York, he sees a gray-haired woman in her sixties convulsing. She is caricaturing everyone who passed her, instantaneously mirroring and exaggerating the movements of 50 passersby in two minutes. The people walking past her experience a shock as they see her mirroring them. Sacks says she is becoming everybody and thus becomes nobody. Eventually, the woman turns into an alleyway where she frantically releases all of the pantomimed gestures in a frenzied burst. Sacks surmises that the “super-Touretter” is driven to delirium, unable to do much about their condition. He posits that it’s likely they are unable to even understand their condition, let alone control it. They cannot find a center or sense of identity. As a result, they become subsumed by their impulses, “faced with extraordinary barriers to individuation” (64).

Sacks compares the spectacle of the woman to an imagined case of “super-Korsakov’s” and considers the constant struggle of the “super-Touretter.” Unlike the former, who will not carry the weight of recognition, “Touretters” are fully aware of their condition: “The Korsakovian, perhaps mercifully, never knows it, but the Touretter perceives his plight with excruciating, and perhaps finally ironic, acuity, though he may be unable, or unwilling, to do much about it” (63). However, Sacks imparts that despite their barriers, those with Tourette’s will fight lifelong to maintain their sense of self. He admires their “will to survive,” which he believes is stronger than both impulse and disease.