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From: "Slate Magazine" <[log in to unmask]>
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Subject: Slate Culture: Can a sore throat lead to Tourette's syndrome?
Date: Wed, 27 Feb 2002 18:39:07 -0800
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medical examiner
Strep Threat
Can a sore throat lead to Tourette's syndrome?
By Emily Yoffe
Posted Wednesday, February 27, 2002, at 12:22 PM PT

******************************************************************

The League of Conservation Voters=92 2001 National Environmental=20
Scorecard reflects Congress' progress on environmental challenges.=20
Detailing vital environmental votes, the scorecard provides=20
individual scores, regional and state averages, most improved=20
members, and members who suffered the biggest score drop.

Click here for our free 2001 Scorecard Action Toolkit, and let them=20
know you're watching.=20

 http://go.msn.com/nl/123901.asp

******************************************************************

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 an=
d drunk her own urine. She listed the people to whom she had shown her priva=
te parts. She asked if this made her a "bad person."

The mother was horrified and baffled. She calmed the girl down and sent her=20=
to school. When her mother picked her up, the girl said she had spread her f=
eces 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=20=
girl said she had blinded her brother with a fork in his eye. She said she w=
anted to step in front of a bus. She said she had swear words stuck in her h=
ead. At first the mother suspected sexual abuse, but the daughter said no on=
e had touched her, and the parents could find no evidence anyone had. The pe=
diatrician said it sounded like a case of obsessive-compulsive disorder, a c=
ondition 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 c=
ases, begin twitching uncontrollably. Doctors at the National Institutes of=20=
Health had a startling suspect: strep throat, one of the most common illness=
es of childhood. Two months before the girl's transformation, she had come d=
own with strep throat four times.=20

Virtually all elementary-school-age children will get a sore throat, often m=
any sore throats, caused by the Group A streptococcus bacterium, and the ove=
rwhelming majority will recover uneventfully. Many will get better without e=
ven seeing a doctor and getting antibiotics, the standard treatment. But the=
re is growing evidence that a range of neurological disorders from temporary=
 tics, such as eye-blinking and head-scratching, to full-blown OCD and Toure=
tte's syndrome are linked to the bacteria. The scientists who connected thes=
e neurological maladies to strep throat named the condition pediatric autoim=
mune 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 i=
ncidence is likely far higher. No one knows the cause, and not even research=
ers in the field know to what degree strep might turn out to play a role in=20=
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=20=
that century the cause for such disorders as schizophrenia, manic-depression=
, Tourette's syndrome, and OCD was believed to be bad parents. One theory wa=
s 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 inheri=
t a bad gene or genes that, in the case of schizophrenia, make people hear v=
oices or, in the case of OCD, have obsessive thoughts. Yet, despite many see=
mingly 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 makin=
g 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 th=
ey have an inflammation in the basal ganglia, a portion of the brain that ac=
ts as a sort of gatekeeper for behavior and movement. It is the same inflamm=
ation 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 Medici=
ne 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 rep=
orted 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=20=
prove the infection connection and that each step forward raises more questi=
ons. For instance, treatments that have been effective in the newly diagnose=
d have been failures in people with chronic cases. Is that because strep is=20=
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 av=
ailable treatments are only effective when the brain is newly under assault?

Paul 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 he=
re). But if bad genes aren't responsible, why do disorders such as OCD/Toure=
tte's/tics run in families? Ewald says there is a place for genetics in the=20=
theory. He posits that genes determine how an individual's immune system rea=
cts=97or overreacts=97to 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 c=
razy, or some of us inherit a gene that makes us crazy because we got a cert=
ain infection. One crucial difference is if the cause is infection, there's=20=
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=20=
illnesses, syphilitic insanity.=20

For germ-theory proponents, the case that strep throat can cause a variety o=
f mental disorders otherwise believed to be either psychological or genetic=20=
in origin is tantalizing. And a model for how that might happen already exis=
ts.

In the early 1990s, Dr. Susan Swedo, a senior investigator at NIH, was hopin=
g to better understand OCD by studying a rare and ancient malady when a chan=
ce remark by a patient's mother led to the description of PANDAS. Swedo was=20=
looking at Sydenham's chorea, known in the Middle Ages as St. Vitus' dance,=20=
a disorder that causes facial grimacing and flailing limbs. Sydenham's occur=
s as a result of rheumatic fever, an autoimmune reaction to untreated strep=20=
throat that can cause inflammation of the heart. It was once the major kille=
r of young children in the United States. But since the use of penicillin to=
 treat strep throat became widespread in the 1940s, rheumatic fever incidenc=
e has declined dramatically. What intrigued Swedo about Sydenham's is that b=
efore the onset of physical symptoms, the young victims often experience OCD=
.

About 25 years ago, researchers discovered the likely neurological basis of=20=
Sydenham's. When we contract strep, our immune system recognizes the invadin=
g proteins on the outside of the bacteria, the antigens, and creates antibod=
ies that attach themselves to the invader. That sends a signal to our white=20=
blood cells to kill the trespasser. But in an unfortunate quirk of nature kn=
own as "molecular mimicry," proteins in the human heart closely resemble str=
ep antigens. In vulnerable individuals, the immune system, instead of stoppi=
ng when the strep is vanquished, continues on an autoimmune rampage against=20=
its own heart. In the case of Sydenham's, the molecular mimicry, and the dam=
age, 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 wa=
s not surprised to find that his older brother had Tourette's syndrome. As S=
wedo 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 t=
hem in for a throat culture because an increase in tics inevitably meant a s=
trep throat.=20

It clicked. Swedo theorized that Sydenham's could just be one manifestation=20=
of neurological damage due to strep. Perhaps there were children who never g=
ot rheumatic fever or Sydenham's but who got OCD or tics directly as a resul=
t 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=20=
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 u=
p. What was needed was a way to stop the autoimmune damage occurring in the=20=
brain.=20

So Swedo and her colleagues used a procedure called plasma exchange or plasm=
apheresis. It's like a high-tech bloodletting. She performed a series of fiv=
e on each patient=97the patient's blood was removed, and the fluid part, the=
 plasma, where the antibodies are found, was discarded and replaced.=20

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. Bu=
t the results are striking. Last April, two months after the onset of her sy=
mptoms, the 8-year-old girl was admitted to NIH for a two-week course of pla=
sma 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 co=
nvinced 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. Wi=
thin a week of returning home she was completely better. Over the course of=20=
the plasma exchange study, 80 percent of the children receiving it maintaine=
d a remarkable improvement in their symptoms a year later.=20

Will there be other neurological disorders linked to strep infection? Dr. Ma=
e Sokol, a specialist in eating disorders at Children's Hospital in Omaha, N=
eb., believes some of her patients with anorexia nervosa had strep-triggered=
 onset. Like the PANDAS patients, they tend to be preteens, and their parent=
s can usually pinpoint exactly when, even to the day, the obsession with foo=
d began, usually within a few weeks of a strep infection. One 10-year-old pa=
tient, after an inadequately treated strep infection, became consumed with t=
he idea that she couldn't swallow solid food. As she began losing weight, sh=
e 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, a=
nd Sokol treated her with a high dose of antibiotics. The girl began eating=20=
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=20=
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 over=
react, other invaders besides strep can set off the process. For example, sa=
ys Kiessling, children with Sydenham's have had recurrences of writhing afte=
r infection with the chicken pox virus or bacteria called Haemophilus influe=
nzae.

While the connection between strep and neurological disorders is intriguing,=
 it is far from proved. Research to find out to what degree strep is respons=
ible for what percentage of OCD and tic disorders is continuing on everythin=
g 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 p=
atients. 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=20=
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=20=
dangerous antibiotic resistance than prevent PANDAS.)

For now, Swedo doesn't have much better advice than teaching children about=20=
washing their hands and not sharing drinking glasses, and for parents of chi=
ldren who have shown neurological symptoms following strep, even minor ones=20=
such as eye-blinking, to be vigilant about sore throats. As the mother of th=
e 8-year old says, "I can't let her get strep."

************************************************************

Also in today's Slate:
 http://go.msn.com/nl/123902.asp

in other magazines: Is Vermont a breeding ground for blood-
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best of the fray: Readers share their thoughts on Puffy, evolution, and tumo=
rs.
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diary: "I found the entire bathroom shellacked with raw sewage."
 http://go.msn.com/nl/123905.asp=20
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