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TWO NOVELS ADDRESS CHALLENGES OF LIVING WITH TOURETTE SYNDROME

Jankovic, Joseph MD; Kadmon, Sheryl RN; Jankovic, Cathy

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Dr. Jankovic is Professor of Neurology and Director of the Parkinson Disease Center and Movement Disorders Clinic at Baylor College of Medicine in Houston. Ms. Kadmon is Executive Director of the Tourette Syndrome Association, and Ms. Jankovic is a media specialist in the Department of Neurology at Baylor College of Medicine.

The hero of Motherless Brooklyn, Lionel Essrog, clearly suffers from severe, undiagnosed Tourette Syndrome (TS). Written by Jonathan Lethem in first person, the book begins with an accurate and comprehensive description of TS, a neurobehavioral disorder manifested by childhood onset of motor and phonic tics, often accompanied by co-morbid behavioral disorders, such as attention deficit with or without hyperactivity, obsessive-compulsive disorder, impulse control problem, and a variety of other abnormal and seemingly bizarre behaviors.

“My mouth won't quit, though mostly I whisper and subvocalize like I'm reading aloud, my Adam's apple bobbing, jaw muscle beating like a miniature heart under my cheek. Patting old ladies gently on the behind, eliciting a giggle. …That's when it comes, the urge to shout in the church, the nursery, the crowded movie house.”

The book provides a realistic account of what it is like to be inside the brain of a TS individual. It describes not only the outward manifestations of the inner torture, the tics, but also the intensity of the premonitory feelings and sensations that precede each movement or sound as if the tics “escape” from the desperate effort by the individual to contain and control them. The straining dam eventually gives. It is exhausting, scary, edgy, and exhilarating.

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BIZARRE BEHAVIOR

Lionel spends his childhood and youth in St. Vincent's Home for Boys, an orphanage in Brooklyn. Although his friends at the orphanage are amused with his sometimes bizarre behavior, they think he is crazy. Referring to himself as “a carnival barker,” he vibrates inside, touches everyone and everything, makes up nonsense words, rhymes, yells, chirps, kisses, imitates, and cannot control his compulsions.

A small time gangster, Frank Minna, befriends young Lionel and several of his friends at the orphanage and has them perform jobs for him. Frank is amused by Lionel's fresh uninhibited behavior, and eventually diagnoses Lionel's TS, thus cementing Lionel's loyalty forever. Eventually Lionel and several of his friends drop out of high school and work full time for Frank. When Frank is murdered, Lionel vows to find his mentor's killers. But his TS gets in his way, muddling his brain, fogging up his mind with gibberish, getting him into trouble. He tries to control his behavior and to focus on solving the murder. Determined, he follows his leads, and in the end solves the mystery.

Lionel's character offers a unique opportunity for anyone interested in understanding the challenge of living with TS. Motherless Brooklyn, while enjoyable to read, accomplishes another, and perhaps more important purpose: it helps the reader gain an inside perspective on how a TS individual behaves as well as the inner forces that drive him to behave, survive, and ultimately prevail. The author should be congratulated for writing a fast moving, captivating story, and for not exploiting Lionel's TS as a literary freak show. Instead, he paints a realistic portrait of a character, who, as a result of his personality partly modified by TS, is alternately irritating and sympathetic, but always interesting and intriguing.

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ICY SPARKS

Figure. Vintage Book...
Figure. Vintage Book...
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The novel Icy Sparks also offers uncanny insight into the inner turmoil and its external manifestations of individuals with TS. This lyrically poetic tale is woven through the eyes of Icy, an orphan growing up in rural Kentucky in the 1950s. Raised by her grandparents and undiagnosed until college, she must contend with and make sense of a world ill-equipped to deal with her mysterious malady. Author Gwyn Hyman Rubio has addressed issues with boisterous humor and jagged poignancy, which are as relevant today as they were 50 years ago.

Icy's symptoms begin in earnest at the age of 10 with unsettling itching, pressure in her head, and the “little invisible rubber bands” attached to her eyelids. Although she does not recognize her initial symptoms as motor and phonic tics, she is tormented by the involuntary movements and sounds, including repeating what others are saying (echolalia) or doing (echopraxia), and shouting obscenities (coprolalia). Desperately trying to suppress these urges, she retreats to the root cellar, where her tics are released in furious bouts. As she becomes increasingly symptomatic her technique becomes decreasingly efficient. The children and adults around her begin to observe her bizarre behavior and her painful journey through adolescence to adulthood begins.

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OBSESSIVE-COMPULSIVE BEHAVIOR

Although a stellar student, Icy is afraid to start school, knowing with certainty that her body will betray her and she will make movements and noises she cannot control. Her worst fears are realized when she begins to exhibit symptoms of obsessive-compulsive disorder, another frequent co-morbidity. Imitation of her teacher's voice and movements, and erasing her papers until they tear, are summarily dealt with by a ping-pong paddle wielded by a teacher as ignorant and cruel as Icy's peers.

The school year soon culminates with an explosive classroom episode instigated by the teacher. Overwhelmed by humiliation and self-doubt, Icy is placed in a small supply room next to the nurse's office.

This pattern of decreased self-esteem, increased anxiety, and social isolation is very common in many children diagnosed today. Further, educational problems are rampant, based primarily on the misunderstood behavioral manifestations of the disorder and, secondarily, due to the increased association of learning differences in this population of children.

Figure. Penguin Book...
Figure. Penguin Book...
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Attempting to discover what ails her, Icy is placed in a state institution outside of Lexington. As the doctors and staff try to unravel her baffling symptoms, Icy must endure placement with severely developmentally disabled children, a cruel aide, and a trial with medication that eradicates not only her symptoms but her spirit as well.

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ROLE OF NEUROLEPTICS

Icy's experiences mirror those of adult TS patients hospitalized in the early 1960s through the 1990s, who describe the debasement of being overdosed with neuroleptics to quell symptoms while incidentally, but effectively, extinguishing the self. Fortunately, Icy's indomitable personality prevails as her intuitive physician stops the medication, rebuilds her self-esteem, and teaches coping mechanisms without ever making a diagnosis.

Icy is sent home to a still largely unreceptive community. However, the loving homespun philosophy and faith of her grandparents and the remarkable Miss Emily, a college educated woman from town and a social outcast herself, sustain and nurture Icy throughout her high school years. Miss Emily, disdained for her morbid obesity, not only tutors Icy for her GED and college entrance exams, but also forces her to believe in a viable future. This, along with the freedom to explore in rural Appalachia, enhances Icy's relentless quest to find her place in the world.

Icy's “place” presents itself unexpectedly after her grandfather dies and her grandmother insists on her coming out of isolation to church. She literally finds her voice, discovering that she not only has an awesome talent for singing, but while doing so experiences an internal calm with a subsequent decrease in symptoms. This phenomenon of decreased distress while engaged in meaningful activity is prevalent in many TS patients, who have a high incidence of artistic, musical, and academic gifts.

Icy Sparks provides realistic insight into the psychosocial difficulties experienced by many affected with TS. It should be read by every physician diagnosing and treating TS patients. The book reminds every health care practitioner that ignorance is the greatest hindrance to healing and that the most important medicine is to nurture the human spirit.

©2001 American Academy of Neurology

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