Hebrew word of the week: Balagan

Words indicating chaos are quite common — often there’s more than one in every language,* such as tohu va-vohu* in Genesis 1:2. Balagan is used in several Slavic languages, but it originated with the Persian bala-khane “top room, attic” (a place where things are stored in a disorderly fashion).** Such words tend to be somewhat slangy and obscene, as with snafu, which comes from U.S. military slang, an acronym for “situation normal, all f***ed up.” 

Other such words in Hebrew: bet-zonot “brothel”; bardaq “brothel; chaos” (derived from Russian); bordel “bordello” (from French); and the more formal, ’i-seder “disorder.” A derived verb: levalgen “confuse, be confused; disorderly.” A humorous Israeli gardener advertises himself: ba’la-gan “comes to-the-garden.”

*As in English: topsy-turvy, helter-skelter.

**Compare to the Yiddish (Israeli Hebrew) boidem, for attic, a crawlspace, where things not in daily use are stored.

Yona Sabar is a professor of Hebrew and Aramaic in the department of Near Eastern Languages & Cultures at UCLA

Rare Ailment Occurs More in Ashkenazis

After David Rudolph sprained his ankle during a basketball game, his father noticed that the second-grader couldn’t seem to keep his left heel flat on the ground. The problem persisted, sidelining David from his position as catcher on his Little League team, and preventing him from progressing beyond his blue belt in karate.

An orthopedist suspected a contracted Achilles tendon, and sent David for physical therapy. When that failed, David underwent a series of neurological tests, none of which indicated a problem. One physician even suggested a psychological phenomenon called “conversion hysteria,” implying that David had created the problem to get attention.

“We knew that wasn’t the case, because it was keeping him from the activities he loved,” said Mark Rudolph, David’s father.

The Rudolphs continued searching. Finally, a pediatric neurologist diagnosed the problem as dystonia, a condition she had seen only four times in her career. A second specialist confirmed the finding. It had taken almost six months for David to receive a proper diagnosis.

A neurological movement disorder characterized by involuntary muscle contractions and spasms, dystonia forces the body into abnormal positions and twisting movements. It can affect a specific body part, such as the neck, hands or torso, or many parts simultaneously, and can vary in severity from mild to extreme.

Although few people have heard of the disease, dystonia affects approximately 300,000 people in North America — more than better-known conditions such as muscular dystrophy, Huntington’s disease and cystic fibrosis.

There are 16 types of dystonia, and it affects all races and ethnic groups, as well as both genders. However, Ashkenazi Jews have a higher than normal incidence of one type, called early onset (generalized) dystonia. This type of dystonia is associated with a mutation in a gene called DYT1.

Ashkenazi Jews carry the mutation at a rate about 10 times higher than that of the general population. DYT1, identified in 1997, somehow disrupts communication between the brain and muscles. Only one parent needs to have the mutation to pass it on to the next generation, yet most people are unaware that they are carriers.

“Two-thirds of individuals with the mutation are asymtomatic [have no symptoms] or have minor problems,” said Dr. John Menkes, the pediatric neurologist who confirmed David’s diagnosis and a co-founder of the Dystonia Medical Research Foundation. “Many people who have dystonia don’t realize it.”

Early onset dystonia tends to show up between the ages of 5 and 16, usually with symptoms in the foot or hand. The involuntary movements may progress quickly to involve all limbs and the torso, although the rate of progression usually slows noticeably after adolescence.

The disease is not fatal, but there is no cure. Physicians use a variety of therapies to reduce muscle spasms, pain and impaired function and posture.

For David, dytstonia means taking 12 pills daily and wearing a brace to stabilize his right foot and ankle. It also involves physical therapy twice weekly, chiropractor visits and Botox injections for the muscle spasms.

Dystonia does not affect mental functioning. A precocious, bright fourth-grader at Wilshire Boulevard Temple’s Brawerman Elementary School, David speaks in rapid-fire sentences, using such words as “curious” and “concerned” to describe classmates’ reactions to his condition. This summer, he will attend his third local session of the Johns Hopkins University’s Center for Talented Youth.

For David’s parents, dystonia is “the uninvited guest that hasn’t left,” said his mother, Diane. Each day, she and her husband wonder whether the cocktail of medications they administer to their son is really making a difference, and whether it may, in fact, be causing harm.

They try to find a balance between letting David pursue the activities he loves and shielding him from injury. Since being diagnosed two years ago, he has fractured his wrist, elbow and knee because of falls due to balance problems. Should he break a leg, David’s muscle contractions could prevent proper casting of the bone.

The Rudolphs have watched the condition progress from David’s left foot to his right foot and leg. Having read the literature and seen videos of patients who are bent at the waist or in wheelchairs, they wonder how bad it will get and which of David’s body parts will be affected. Most of all, they grapple with how much to tell their son about his condition.

“We’ve tried to shield him from what the worst could be,” said his father. “But he’s 10 years old. At some point, we won’t be able to protect him.”

Karen Ross understands the emotions experienced by the Rudolphs.

“Dystonia is damaging to individuals and to families,” said Ross, a clinical psychologist. “You never go back to the kind of family you once were.”

Ross became interested in psychology after her son, Michael, was diagnosed with dystonia in 1975. The family had spent three years trying to find out what was wrong. At that time, there were no organizations dedicated to the disease, no support system for families and no awareness of dystonia within the Jewish community.

Ross now serves on the board of the Dystonia Medical Research Foundation — as do the Rudolphs — to try to change that.

“We want to make this the last generation of Jews who have this disease,” Ross said. The Dystonia Medical Research Foundation has provided more than $19 million in grants to scientists since its inception in 1976.

Research is encouraging, although it remains to be seen whether the psychologist’s goal will be met. On the positive side, pediatric neurologist Menkes noted that in dystonia, unlike many other diseases, nothing is missing or defective. Since the gene implicated in dystonia “seems to prevent normal movement of certain cellular components, the idea would be to prevent the action of this bad gene,” he explained.

One disadvantage, however, is that dystonia “doesn’t get any attention in the research community,” said Dr. Marie-Francoise Chesselet, UCLA Medical School’s neurobiology department chair. Chesselet, who has served on the scientific advisory committee of the Dystonia Medical Research Foundation, said that researchers are more interested in working on conditions with greater visibility or prevalence.

Until the DYT1 gene mutation was identified, “dystonia was like a black box,” she said. “People had no idea even how to even approach it…. Now there are some extremely good researchers who, while not primarily interested in dystonia, are interested in those particular biological mechanisms, and are now applying their knowledge and experimental skills to dystonia.”

In fact, researchers have discovered how to silence the mutated DYT1 gene in cultured cells. They are currently trying to do so with worms, flies and mice.

The 1997 discovery of the DYT1 gene also allowed for the development of a genetic test for patients and family members who are possible carriers, as well as prenatal testing to determine if a fetus has the mutated gene.

Two years ago, a man with dystonia and his wife used a technology called premiplantation genetic diagnosis (PGD) to insure that their child would not be born with the disease. PGD involves fertilizing eggs outside the body and screening for genetic disease before returning them to the womb for gestation. The child, born in September 2003, was the first to be born using this technology to check for dystonia.

The condition has also received recent public recognition because of concert pianist Leon Fleisher. Focal dystonia in his right hand forced Fleisher to limit his playing to one-handed pieces. After he received Botox injections, the tension in his hands relaxed sufficiently for him to make his first two-handed recording in 40 years.

Fleischer has donated a portion of the profits from the CD, appropriately titled, “Two Hands,” to the Dystonia Medical Research Foundation and has worked with the foundation’s Musicians With Dystonia group to address the needs of musicians with the condition.

All of these developments are giving hope where there once was none. “Ten years ago, I never thought I’d see effective treatment or a cure. I can now say that’s possible,” said Ross’ son, Michael, now 45 years old and a rabbinical student at the Reconstructionist Rabbinical College in Pennsylvania.

The Rosses and Rudolphs want members of the Jewish community to know about dystonia so that should they encounter it in their own families, they won’t have to wait months or years for a proper diagnosis. They believe that lack of awareness is the only thing preventing the community from taking action.

In terms of awareness and education, “the community has successfully dealt with other Jewish disorders, such as Tay Sachs and Familial Dysautonomia,” said Karen Ross. “The Jewish community cares when it knows what to care about.”

For more information, contact the Dystonia Medical Research Foundation, www.dystonia-foundation.org, (800) 377-3978; Bachmann-Strauss Dystonia & Parkinson Foundation, www.dystonia-parkinsons.org, (212) 241-5614; WE MOVE www.wemove.org, (212) 875-8312; National Institute of Neurological Disorders and Stroke, www.ninds.nih.gov.

‘Finding’ Asperger’s Changed His Life

In “Finding Ben: A Mother’s Journey Through the Maze of
Asperger’s,” (McGraw-Hill/Contemporary Books, 2003) author Barbara LaSalle
writes about her family’s struggle to help her young son overcome a baffling
neurological disorder and have a “regular” existence. Misdiagnosed and
maladjusted, Ben Levinson was labeled as everything from learning disabled to
emotionally disturbed and was even committed to a psychiatric ward before
LaSalle, a marriage and family counselor, was able to correctly diagnose him
with Asperger’s Syndrome (AS).

While AS and autism diagnoses are increasing at alarming
rates, “Finding Ben” presents a frightening portrait of one family in the days
before treatment was widely available.

The book begins with Levinson’s birth in 1969 and goes
through the many torturous incidents that marked his differences throughout his
childhood and adolescence. It culminates in his arrest for threatening a
residential caretaker in a halfway house where he had been placed, and his long
road back to a normal life. It is disturbing to read, but compelling — the book
is as much about a family dealing with the guilt, anger and denial surrounding
caring for a disabled child as it is about Levinson’s unusual life. 

One bright spot in the family’s struggle was their
involvement with Temple Beth Hillel in Valley Village. Ben attended preschool
and had his bar mitzvah there, and LaSalle says the havurah in which they
participated was especially supportive. Despite his challenges, Levinson was
able to finish Hebrew school and LaSalle said the family still relies on
Levinson at Passover to read the Hebrew portions of the haggadah.

But, for the most part, life with Ben was a constant
challenge. As he grew, his problems increased to include asthma and Crohn’s
Disease, leading to medication which in turn led him to become morbidly obese.
The family tried motor therapy (an early form of occupational therapy), speech
therapy, even a private school where the teachers followed their students through
each grade level, in the hope that Ben might feel comfortable enough to make
friends. He never did.

All the while, LaSalle never stopped searching for answers.
Finally, when Levinson was 23, Dr. Mark Deantonio of UCLA’s Neuropsychiatric
Institute told her the truth: Ben was autistic. Not autistic in the classic
sense, but his problems put him on the autism spectrum.

Two years later, in 1994, the criteria was established in
the medical community for an even more specific diagnosis, that of AS, a
higher-functioning form of autism in which children have normal or even
superior verbal skills and intelligence.

“Finding Ben” is a modern tragedy — not in an exaggerated,
fictional sense, but a true tragedy in that the people involved are simply
living in the wrong time in history. Even Levinson himself, now 34 and
co-author of the book, acknowledges that, had the diagnosis of AS been
available when he was a child, his life would have been infinitely easier.

LaSalle said she started out writing the book as a way of
making sense of everything that had happened to her, to Levinson’s father (an
attorney, referred to as “Steven” in the book), his stepfather, John LaSalle,
and his brother, David. It is clear from talking to LaSalle and from her
writing that she still carries a great deal of guilt. Her honesty about her
feelings for and against her son are shocking: She opens the book with a
description of Levinson that would seem cruel coming from anyone, especially
from a mother. But LaSalle hopes her honesty will open the doors for readers to
come clean with their families and deal with their feelings, even the ugly ones.

“The most important thing is acceptance — that what is, is,”
she said. “We are required to accept and love our children no matter what. That
is the gift we give our kids.”

It is a lesson she almost learned too late. Only by letting
go of Ben as her “project,” and through volunteer work where she met a stroke
victim with even more profound problems than her son’s, was she able to change
her approach from that of “badgering mother” to one of support and acceptance.

“I saw my son as a job,” she said. “He wasn’t someone to
enjoy. I think we all have that [attitude] at times, when we have children with
special needs. But in treating it like it is a job, we miss out on what’s right
in front of us and our children miss out, as well.”

Levinson and his family seem to have made peace with his
diagnosis. He is currently in a 12-step program for people with weight
problems, which he credits with giving him the structure and social network to
finally not only make friends but learn to be a friend as well. An Orthodox rabbi
and his wife who participate in the program have helped him reconnect with “the
spiritual side of Judaism.” Levinson attends Loyola Marymount University where
he is studying American history with plans to graduate next year, possibly to
become a teacher.

Levinson also runs a Web site (www.aspergerjourney.com)
where he shares his insights on his disability and communicates with others
affected by AS. He feels his experience with AS, while difficult, has given him
a valuable perspective.

“One time I was complaining to my sponsor: Why did God put
this burden on me? And my sponsor said, ‘The reason you have had to go through
this is that one day you are going to meet someone who will require your
personal experience. You will be in a unique position to help another human
being,'” Levinson said. “There are a lot of us out there [affected by AS]. I
tell them, don’t be ashamed of who you are, be proud. Start to talk about it as
much as you can. Find people who understand and talk about it with them.
Asperger’s is a daily struggle, but it’s easier now because I’m not in denial.”

Both LaSalle and Levinson will discuss “Finding Ben” on
Friday, April 18, 7 p.m. at Dutton’s Brentwood Books, 11975 San Vicente Blvd., Los
Angeles. (310) 476-6263.