There was nothing in Jane Porter`s early years that foreshadowed the terrible changes that occurred when she was 20.
She was a healthy, happy child and an accomplished student. She could read before starting kindergarten, was valedictorian of her class in high school and earned straight A`s in her freshman year in college.
Outside the classroom, her life seemed full and serene. She had caring parents, good friends and a young man she loved and planned to marry.
Then, during her sophomore year in college, everything began to fall apart; it was as though she had become another person. Her performance in class deteriorated, and she failed her mid-term exams; she neglected her friends and pulled away from her fiance, who talked about calling off the wedding.
What was strange and unsettling was that through all her troubles, Jane Porter smiled. Continually, and for no reason.
She thought she must be going crazy, and the doctors who examined her did not disagree. They decided she suffered from schizophrenia, and one diagnosed hebephrenia, an extremely rare form of this psychiatric disorder. Her parents, however, weren`t convinced. They brought their daughter to Dr. Harold Klawans (pronounced klah-WAHNS), a neurologist who practices at Rush-Presbyterian-St. Luke`s Medical Center in Chicago and teaches at Rush Medical College.
When he saw her, Klawans recognized her smile as a symptom of a neurological, not a psychiatric, sickness.
”It was a peculiar smile-not friendly, not joyous, not happy, but there all the while,” he noted. ”A smile that refused to fade. Closer to a sneer than a smile.”
Neurology, he explained, has a Latin name for this condition-risus sardonicus, which refers to ”a fixed sardonic smile that doesn`t go away”
and points toward several possible diseases. Which one, of course, was the crucial question.
Klawans writes about Jane Porter and how he identified and treated the malady that caused her curious and revealing smile in ”Newton`s Madness”
(Harper & Row, $17.95), his second book about neurological illnesses.
Most of the book`s 22 cases are drawn from the author`s own practice, and several feature an intriguing array of prominent figures from the past, including Russian novelist Fyodor Dostoyevsky, Old Testament prophet Elisha, American composer Scott Joplin and Sir Isaac Newton, the great English mathematician and scientist whose two prolonged episodes of abnormal behavior are the subject of the title chapter.
Klawans is a gifted, humane storyteller who has been praised by critics for making compelling to the ordinary reader the analysis of infirmities as complicated and dispiriting as Parkinson`s disease, Huntington`s chorea, myclonic epilepsy, cluster headaches, Weinicke`s encephalopathy and syphilis. A dominant theme of ”Newton`s Madness” is the doctor as detective, the skilled observer who meticulously gathers evidence and uses judgment, insight and intuition in tracking and apprehending the source of an affliction or behavioral problem.
A review of ”Newton`s Madness” in London`s Sunday Times called Klawans
”the shrewdest medical super-sleuth writing today.”
The gumshoe analogy is particularly fitting, for Klawans, in addition to two nonfiction books, has written three paperback mystery novels-with a doctor as hero.
”One of the aspects that attracted me to neurology was the puzzle solving, the mystery solving, the diagnosis,” he said recently. ”There are mysteries in all areas of medicine, but traditionally it has been especially true of neurology.”
Wearing a starched white coat and leaning back in a swivel chair behind a cluttered desk, Klawans was interviewed in his office, a can of diet root beer at his side.
”When I started in neurology, diagnosis was purely an intellectual exercise,” he said. ”Even today with all the fancy equipment and diagnostic techniques, between 25 and 35 percent of all neurological diseases must still be established clinically, by observation.”
No diagnostic test, Klawans writes, can prove that someone has ”chorea or tics or Parkinson`s disease or dystonia” or many other other conditions:
”The list goes on and on.”
Neurologists must rely on the skill of their eyes, especially in studying a patient`s movements, which can signal the type of disorder. ”Observation begins the moment I see the patient, and I try to do this the first time without the patient`s knowledge,” Klawans said. ”If patients know they`re being observed, they`ll often try to mask their problems or exaggerate them.
”So I always make a point to leave my office and walk into the waiting room to observe my patients. I may not know which patients are mine, so I observe them all while I pretend I`m reading the newspaper or checking records. Frequently I know a lot of what I`m going to know even before I say hello to the patient.”
This approach often bothers patients. ”They feel more secure if you order X-rays and CAT scans and EEGs (electroencephalograms) and the full lineup of blood-chemical tests.”
`You don`t treat EEGs`
He`s concerned that too many doctors are eager to oblige and are relying on tests and technology at the expense of their patients. ”These
sophisticated diagnostic techniques become a crutch. We spend less and less time in examining and communicating with our patients and getting to know them and their disease. Doctors need to be reminded that you don`t treat EEGs and CAT scans. You treat patients.”
Klawans first tried to deliver his message through fiction. ”I wanted to write a book about how you teach people the art and skill of diagnosis,” he said. ”I wrestled with how to do it for four or five months and then it struck me one day-a murder mystery based on a reverse Sherlock Holmes. Sherlock Holmes is based on a physician, so I`ll have a physician based on Sherlock Holmes.”
Arthur Conan Doyle, a Scottish physician, indeed modeled his legendary English detective on Dr. Joseph Bell, Doyle`s mentor in medical school.
Klawans` medical sleuth is a neurologist named Paul Richardson, who, through no coincidence, shares several traits with his creator. Like Klawans, Richardson is now 52, practices at a large teaching hospital in Chicago, worries about his weight and is an avid White Sox fan. (Subtract the last two letters of Richardson, and you have the name of a former Sox player and manager.)
Klawans is a Chicago native, reared on the South Side, a graduate of South Shore High School. He received a bachelor`s degree from the University of Michigan and his medical degree from the University of Illinois.
There was no doubt about a career. ”I always knew I was going to be a doctor. It`s a dominant hereditary disease with 100 percent penetrance,” he said. ”My father and his brother-the two most important men in my life-were doctors.”
He knew the specialty he preferred from his first year in medical school. ”The brain intrigued me. The solution of what happens in the brain when it`s not working right and how that allows us to think about the brain when it is working right were two pieces of an endlessly fascinating puzzle.”
Klawans has a worldwide reputation for his research on Parkinson`s disease and as an authority on Tourette`s syndrome and Huntington`s chorea. He has written 350 medical articles and edited 55 medical and scientific books.
But those achievements were no help eight years ago when he called a publisher to pitch his idea for ”Toscanini`s Fumble,” his first nonfiction book about neurology. ”I was told nobody wanted to read about neurology case histories,” he said.
Klawans then offered his Dr. Paul Richardson manuscript, which had been gathering dust since 1975, when he`d written most of it while attending an international symposium on Parkinson`s disease in Vienna.
”Sins of Commission,” published in 1982 by Chicago`s Contemporary Books, was followed in 1986 and `88 by two other Paul Richardson paperback mysteries, published by Signet Books.
Also in `86, the market for nonfiction books about illnesses of the nervous system suddenly became very hot because of the spectacular and surprising success of ”The Man Who Mistook His Wife for a Hat.”
This collection of actual case histories written by Dr. Oliver Sacks, a New York neurologist, stayed for months on the best-seller lists, prompting Contemporary Books to reconsider and give the green light to the previously rejected ”Toscanini`s Fumble.”
”Oliver Sacks is the Christopher Columbus of this genre of books,”
Klawans said. ”He opened the field for this kind of writing.”
Klawans laughed. ”If there`s a downside, it`s that now I`m expected to be a writer.” This adds pressure to a schedule that has few spare hours, and since he`s not one of those doctors who take Wednesday afternoons off to play golf, he can`t cut back on recreational time.
Remarried in February, Klawans lives in Munster, Ind., with his second wife, Barbara Young Stemer, a professional harpist. ”I`m a morning person, and I try to write for a couple of hours before I head for the hospital,” he said. ”I also try to get in some writing on weekend mornings.”
He writes his first drafts in longhand, then rewrites as he transfers the text to his word processor; at each step, music from American musicals or late 20th Century classical music is playing in the background.
”My interests are music, art and theater,” he said. ”I collect Chicago artists from the 1930s: Tunis Ponsen, William Conger, John Weare. I recently commissioned Weare to paint an oil of old Comiskey Park. It`s beautiful. My fantasy is to have one of my plays produced. I`ve written three or four, and I`ve been working to get them in shape with the Chicago Dramatists Workshop.” Psychiatric vs. neurological
A recurring lesson from ”Toscanini`s Fumble” and ”Newton`s Madness”
is that neurological diseases are sometimes mistaken for psychiatric disorders.
The case of Jane Porter, mentioned at the beginning of this article, and the unbalanced behavior exhibited by Sir Isaac Newton (1642-1727) are two examples.
”Newton`s personality changed drastically when he was 35 and again when he was 51,” Klawans said. ”He was never a terribly social person, but on each occasion he became even more withdrawn and irritable. He accused people of plotting against him, even friends.
”After the second episode, he was never quite the same. All his major scientific work was completed before the second episode.”
Some people attributed his change of personality to the death of his mother, depression or the loss of a laboratory to fire. ”The answer was mercury poisoning,” Klawans said. ”He heated metallic mercury for some of his experiments, and this exposed him to very damaging fumes.”
The book cites evidence of these experiments and the recent analysis of a lock of Newton`s hair by English scientists, who found unusually high concentrations of mercury.
”I think mistaking neurology disease for psychiatric problems happens far less today than it did even 20 years ago,” Klawans said. ”Today people understand that depression, for example, no matter what triggers it, is associated with a biochemical abnormality and is very frequently hereditary.” In making a diagnosis, Klawans disregards a patient`s psychiatric history. ”My neurology professor taught me never to take one. All it can do, he said, is mislead you.
This is because everybody`s crazy. All people have strange relationships with their mothers or fathers or brothers or sisters or wives or husbands or children or lovers.
”Being crazy doesn`t protect you from disease. And whether or not a patient has a neurological problem is a question to be answered on
neurological grounds. You shouldn`t prejudice yourself by knowing whether or not there may be psychiatric grounds.”
Klawans had merely to look into the eyes of Jane Porter and see the greenish ring around the edge of each cornea to know she was not schizophrenic and that, like Newton, she had a form of metal poisoning.
The culprit was an uncommon hereditary ailment called Wilson`s disease that prevented her body from absorbing copper properly.
Because it was detected early enough, the disease could be controlled by medication. Today Jane Porter is a tenured university professor who is married (not to her original fiance) and the mother of two children.
