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medical examiner

Strep Threat

Can a sore throat lead to Tourette's syndrome?

By Yoffe

Posted Wednesday, February 27, 2002, at 12:22 PM PT

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Last February a happy, confident 8-year-old girl went to bed and woke up

the next morning having turned into someone else. She came to her mother

with a series of shocking confessions. She said she had licked people's

bottoms and drunk her own urine. She listed the people to whom she had

shown her private parts. She asked if this made her a " bad person. "

The mother was horrified and baffled. She calmed the girl down and sent

her to school. When her mother picked her up, the girl said she had

spread her feces around the school. The mother casually checked with a

teacher about the girl's behavior and was told she was fine. This went

on day after day. The girl said she had blinded her brother with a fork

in his eye. She said she wanted to step in front of a bus. She said she

had swear words stuck in her head. At first the mother suspected sexual

abuse, but the daughter said no one had touched her, and the parents

could find no evidence anyone had. The pediatrician said it sounded like

a case of obsessive-compulsive disorder, a condition of unknown origin,

and referred the family to a psychiatrist.

Surfing the Web, the mother discovered other cases just like her

daughter's: normal kids who suddenly become consumed by horrible

thoughts or, in some cases, begin twitching uncontrollably. Doctors at

the National Institutes of Health had a startling suspect: strep throat,

one of the most common illnesses of childhood. Two months before the

girl's transformation, she had come down with strep throat four times.

Virtually all elementary-school-age children will get a sore throat,

often many sore throats, caused by the Group A streptococcus bacterium,

and the overwhelming majority will recover uneventfully. Many will get

better without even seeing a doctor and getting antibiotics, the

standard treatment. But there is growing evidence that a range of

neurological disorders from temporary tics, such as eye-blinking and

head-scratching, to full-blown OCD and Tourette's syndrome are linked to

the bacteria. The scientists who connected these neurological maladies

to strep throat named the condition pediatric autoimmune

neuropsychiatric disorders associated with streptococcal infections, or

PANDAS. Some scientists even believe that strep throat might be a factor

in some cases of anorexia nervosa.

It is estimated that about 2 percent of the population suffers from OCD

and/or tics, which are diagnosed on the basis of behavior, making the

conditions more common than schizophrenia and manic-depression. But many

believe the incidence is likely far higher. No one knows the cause, and

not even researchers in the field know to what degree strep might turn

out to play a role in these cases.

That infections can trigger common mental illnesses is not a new idea.

It is a very old one, discredited for most of the 20th century. In the

middle of that century the cause for such disorders as schizophrenia,

manic-depression, Tourette's syndrome, and OCD was believed to be bad

parents. One theory was that OCD was the result of punitive toilet

training. Toward the end of the century, the blame shifted to bad genes.

That idea, which is still the most widely held in the scientific

community, is that the unfortunate few inherit a bad gene or genes that,

in the case of schizophrenia, make people hear voices or, in the case of

OCD, have obsessive thoughts. Yet, despite many seemingly promising

leads, no one has been able to identify this blighted DNA.

But what if the problem isn't bad genes but bad germs? Researchers are

making the connection between OCD and tics with evidence of an

infectious assault to the brain. For example, brain scans of children

with PANDAS show that they have an inflammation in the basal ganglia, a

portion of the brain that acts as a sort of gatekeeper for behavior and

movement. It is the same inflammation seen in a rare neurological

condition that arises from rheumatic fever, a disease caused by strep.

Scientists at both Brown Medical School and Yale University School of

Medicine have infused rats with the blood serum of patients with

Tourette's and/or OCD. How it affected the rats' thoughts is unknown,

but the infused rodents exhibited the tics and grunts stereotypical of

Tourette's. And, as just reported in the Journal of the American Medical

Association, researchers at the University of Rochester Medical Center

identified a small group of children when they first exhibited signs of

OCD and tics and eliminated the symptoms with early antibiotic

treatment.

The researchers themselves warn that these studies, while intriguing,

don't prove the infection connection and that each step forward raises

more questions. For instance, treatments that have been effective in the

newly diagnosed have been failures in people with chronic cases. Is that

because strep is responsible for only a small portion of these

neurological illnesses? If so, what causes the rest? Or could chronic

cases be linked to strep, but the available treatments are only

effective when the brain is newly under assault?

Ewald, a professor of biology at Amherst College, is a leading

theorist of the germs-not-genes movement (read a full explanation of his

theories here <http://www.theatlantic.com/issues/99feb/germs.htm> ). But

if bad genes aren't responsible, why do disorders such as

OCD/Tourette's/tics run in families? Ewald says there is a place for

genetics in the theory. He posits that genes determine how an

individual's immune system reacts—or overreacts—to any given infection.

So, if that's the case, in the end what's the difference? Either some of

us inherit a gene that makes us crazy, or some of us inherit a gene that

makes us crazy because we got a certain infection. One crucial

difference is if the cause is infection, there's the possibility of

prevention or cure (for now, genes can't be fixed). Ewald says, for

example, that the discovery of penicillin is the " biggest success story

in all of psychiatry " because it ended one of the most common mental

illnesses, syphilitic insanity.

For germ-theory proponents, the case that strep throat can cause a

variety of mental disorders otherwise believed to be either

psychological or genetic in origin is tantalizing. And a model for how

that might happen already exists.

In the early 1990s, Dr. Swedo, a senior investigator at NIH, was

hoping to better understand OCD by studying a rare and ancient malady

when a chance remark by a patient's mother led to the description of

PANDAS. Swedo was looking at Sydenham's chorea, known in the Middle Ages

as St. Vitus' dance, a disorder that causes facial grimacing and

flailing limbs. Sydenham's occurs as a result of rheumatic fever, an

autoimmune reaction to untreated strep throat that can cause

inflammation of the heart. It was once the major killer of young

children in the United States. But since the use of penicillin to treat

strep throat became widespread in the 1940s, rheumatic fever incidence

has declined dramatically. What intrigued Swedo about Sydenham's is that

before the onset of physical symptoms, the young victims often

experience OCD.

About 25 years ago, researchers discovered the likely neurological basis

of Sydenham's. When we contract strep, our immune system recognizes the

invading proteins on the outside of the bacteria, the antigens, and

creates antibodies that attach themselves to the invader. That sends a

signal to our white blood cells to kill the trespasser. But in an

unfortunate quirk of nature known as " molecular mimicry, " proteins in

the human heart closely resemble strep antigens. In vulnerable

individuals, the immune system, instead of stopping when the strep is

vanquished, continues on an autoimmune rampage against its own heart. In

the case of Sydenham's, the molecular mimicry, and the damage, is found

in the neurons of the basal ganglia of the brain.

Swedo was evaluating a boy thought to have Sydenham's. He didn't, but he

did have OCD and tics, and because these things often run in families,

Swedo was not surprised to find that his older brother had Tourette's

syndrome. As Swedo was talking to the boys' mother, the woman mentioned

that it had become a family joke that whenever her kids' tics got worse,

it was time to take them in for a throat culture because an increase in

tics inevitably meant a strep throat.

It clicked. Swedo theorized that Sydenham's could just be one

manifestation of neurological damage due to strep. Perhaps there were

children who never got rheumatic fever or Sydenham's but who got OCD or

tics directly as a result of an unremarkable sore throat. If that was

the case, it meant there might be something they could do to cure it.

Antibiotics were not the answer for the patients Swedo saw. Because it

was so long between the onset of symptoms and her patients' arrival at

NIH, the initial strep infection had cleared up. What was needed was a

way to stop the autoimmune damage occurring in the brain.

So Swedo and her colleagues used a procedure called plasma exchange or

plasmapheresis. It's like a high-tech bloodletting. She performed a

series of five on each patient—the patient's blood was removed, and the

fluid part, the plasma, where the antibodies are found, was discarded

and replaced.

Swedo's initial study was much too small to be considered definitive. In

all, she has treated only about 30 children with the most devastating

cases. But the results are striking. Last April, two months after the

onset of her symptoms, the 8-year-old girl was admitted to NIH for a

two-week course of plasma exchange. During her first three days in the

hospital, she was unable to eat because of the extreme distress of

seeing other sick people; she was convinced she had made all of them

ill. By the third plasma exchange, the girl was less fraught with worry.

By the fifth, she was almost herself again. Within a week of returning

home she was completely better. Over the course of the plasma exchange

study, 80 percent of the children receiving it maintained a remarkable

improvement in their symptoms a year later.

Will there be other neurological disorders linked to strep infection?

Dr. Mae Sokol, a specialist in eating disorders at Children's Hospital

in Omaha, Neb., believes some of her patients with anorexia nervosa had

strep-triggered onset. Like the PANDAS patients, they tend to be

preteens, and their parents can usually pinpoint exactly when, even to

the day, the obsession with food began, usually within a few weeks of a

strep infection. One 10-year-old patient, after an inadequately treated

strep infection, became consumed with the idea that she couldn't swallow

solid food. As she began losing weight, she liked the result. Six months

and 30 pounds after the onset of her symptoms, she was referred to

Sokol. At that time, the girl had a sinus infection, and Sokol treated

her with a high dose of antibiotics. The girl began eating two days

later. Sokol says there is a possible physiological explanation for such

cases: The part of the brain thought to be responsible for body image is

close to the basal ganglia, which is inflamed in children with PANDAS.

Could other infections trigger PANDAS-like symptoms? Dr. Louise

Kiessling, a professor at Brown Medical School, says there is some

evidence Lyme disease can provoke similar behaviors. And once the immune

system is primed to overreact, other invaders besides strep can set off

the process. For example, says Kiessling, children with Sydenham's have

had recurrences of writhing after infection with the chicken pox virus

or bacteria called Haemophilus influenzae.

While the connection between strep and neurological disorders is

intriguing, it is far from proved. Research to find out to what degree

strep is responsible for what percentage of OCD and tic disorders is

continuing on everything from the chemical level to the epidemiological

one. Researchers are trying to find out if there is a molecule produced

in the brain unique to PANDAS patients. They are also following large

groups of children to see if they can better correlate strep throat and

subsequent behavior disorders. And if the work on a strep vaccine is

successful, widespread inoculation could result in a dramatic decline of

OCD and tics. (The doctors involved in the research all warn against

rampant use of antibiotics, which is more likely to cause dangerous

antibiotic resistance than prevent PANDAS.)

For now, Swedo doesn't have much better advice than teaching children

about washing their hands and not sharing drinking glasses, and for

parents of children who have shown neurological symptoms following

strep, even minor ones such as eye-blinking, to be vigilant about sore

throats. As the mother of the 8-year-old says, " I can't let her get

strep. "

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