The first time I met a group of severely disabled Lyme disease patients I spent hours listening to their stories, some of them heartbreaking, and mourned, with them, their lives of frustration and pain. A month later, when I met the same patients again, several could not recall me. At first I was insulted. Had I been that forgettable, my empathy that banal?
Then I realized something I had never fully grasped despite my research, despite my own Lyme disease. Unless you have personally encountered the shadowland of the most afflicted patients-a world eclipsed by strange lapses of memory, broken speech, and the struggle to follow the simplest train of thought-you cannot begin to fathom the dense, disabling fog that may accompany the disease.
To this day, popular perception holds that Lyme disease is an affliction of knees, characterized by swollen joints and an inability to serve in tennis or descend a flight of stairs. Musculoskeletal symptoms can be a hallmark of Lyme disease, but the early rheumatologists had recognized just one part of the elephant-it would take more time, and a broad array of specialists, for the widening picture to emerge.
Some of those early patients, selected for studies because of their remitting-relapsing arthritis, recalled the appearance of an expanding red rash before the arthritis had begun. Taking the cue, the Yale scientists looked for patients with the rash-early Lyme-and began studying them prospectively, at the true onset of disease. They found that only some patients went on to develop arthritis. In others, the rash gave way to headaches and stiff necks (meningitis); facial drooping, choking, or visual loss like I suffered (attack of the cranial nerves); or terrible shooting pains throughout the torso and limbs. When these devastating signs appeared together, often the diagnosis was Lyme.
At Stony Brook, meanwhile, the neurologist John Halperin studied a far less devastating but more common "peripheral neuropathy," a kind of numbness or "pins and needles" feeling in the extremities. Could the intermittent numbness and tingling in one patient's fingers derive from the same dysfunction as the stabbing pain in another patient's torso and legs? By 1990, using the tool of electromyography (EMG) to study nerve cells, Halperin found that these symptoms, though diverse, were all due to the same thing: damaged nerve cells and, more specifically, abnormalities in the axon, the long, slender part of the cell that propagates nerve impulses along. The neurons were being "picked off" one at a time, in scattered clumps, as if snipers were at work. If the disease took out a big chunk in one place, you might get shooting pain. If it took out tiny, scattered groups of nerves you could get numbness in the toes or a weakness when you walked. Halperin's study, published in the journal Brain, concluded that the underlying pattern of nerve cell abnormality was the same no matter what the complaint. "All of them really had the same disease," he said. "It was just variations on a theme."
Other times Lyme caused psychiatric disease. One of the first to have this insight was Andrew Pachner, a Yale neurologist who moonlighted at psychiatric hospitals. On one such gig he was asked to evaluate a twelve-year-old boy who, prior to admission, had pedaled his stationary
bicycle barely stopping to sleep or eat. Before the start of this behavior, the boy had been an excellent, hardworking student with a talent for soccer. But his soccer days were disrupted when he developed painful, swollen knees and was diagnosed with Lyme arthritis. The
child was treated with doxycycline and seemed to get well. When his obsessive pedaling began some years later, his prior Lyme disease was already a distant memory, and no one saw the relationship between the two.
Except for Pachner: Given what he knew about syphilis, he wondered whether Lyme disease and the obsessive cycling might be linked. In a leap of insight, he moved the boy to Yale and infused him with penicillin for fourteen days. It was like a miracle. Literally within days the child started to improve, interacting with staff and eating food. Two weeks later he returned home and went back to school. When Pachner saw him a few months after that he had even returned to soccer. He seemed cured. In 1989, writing for the Archives of Neurology, Andrew Pachner, by then at the Georgetown University School of Medicine, described six cases of central nervous system Lyme disease, of which his "bicycle boy" was just one. Another patient, a twenty-one-year-old man, had violent outbursts and wild laughing, attributed to a herpes virus thought to infect his brain. But he tested positive for Lyme disease and, treated with antibiotics, was finally cured. A six-year-old girl, so afflicted with vertigo that she staggered, tested positive for Lyme and was treated; she, too, got well.
The German neurologist Rudolph Ackermann found that the sickest of these neuroborreliosis patients suffered an inflammation of the brain and spinal cord called encephalomyelitis, also seen in syphilis. When the condition involved the spine it resembled multiple sclerosis and when it involved the brain, particularly the cerebral cortex, it could produce psychoses or seizures. The condition was progressive and degenerative without treatment, but even after antibiotic therapy, most of the patients retained the symptoms, though to a lesser degree.
From the devastating syndrome described by Ackermann to the odd presentations reported by Pachner, neuroborreliosis appeared almost protean, and, like syphilis, could be mistaken for a host of other ills. Syphilis had long been known as "the great imitator" among doctors.
Now Pachner declared that Lyme disease was "the new great imitator."
His statement seemed to unleash a torrent of bizarre reports flowing into the medical journals. A group from Stanford described a twenty five-year-old woman with hallucinations, hypersexuality, nightmares, and a rash. Scientists from Germany found Lyme could cause Tourette's syndrome, catatonia, and even schizophrenia. Several teams have reported Lyme disease masquerading as-or even triggering-Parkinson's disease. And in what would be a breakthrough of enormous scope-if borne out in other studies-neuropathologist Judit Miklossy of the University of Lausanne in Switzerland reported that she had isolated spirochetes from the blood, cerebrospinal fluid, and brain tissue of fourteen Alzheimer's patients upon autopsy. More than two dozen published papers associate neuroborreliosis with stroke, and others document that Lyme disease may cause seizures.
Perhaps most tantalizing is the work done on ALS. In 1987, for instance, a Wisconsin team found that four of fifty-four patients diagnosed with ALS also tested positive for Lyme disease-and since ALS is fatal, decided that treatment with antibiotics wouldn't hurt. Following the treatment, one of the four patients was stabilized, the progression of her symptoms halted for good. Intrigued by the report, Halperin did a formal follow-up, testing nineteen ALS patients from the hyperendemic area of Suffolk County, New York. Of these, nine had Lyme antibodies and the Stony Brook doctors, like their Wisconsin colleagues, treated them with antibiotics. Three patients, those with abnormalities primarily in the lower part of the body, improved. But three of the sickest patients declined dramatically following treatment, which seemed to hasten their death. Though the Stony Brook scientists couldn't say for sure what was going on, they affirmed the statistical significance between ALS and Lyme disease, and theorized that in those who deteriorated, the cause might be the flood of dying spirochetes themselves.
Click here for Neurological Lyme Disease, part two.
(Follow this link to read my personal story.)
Excerpted from Cure Unknown: Inside the Lyme Epidemic, St. Martin's Press, 2008