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Fall 2008  
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  The essentials of essential tremor

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Four years ago, an engineer in her late 50s was experiencing difficulty in doing her job, and she came for treatment at Emory’s Movement Disorders Center. Clinician Claudia Testa diagnosed the patient’s condition as essential tremor (ET), a common and complex neurologic movement disorder that may affect the hands, head and neck, face, jaw, tongue, voice, trunk, and sometimes rarely, legs and feet.
     Testa learned that the patient’s large family in North Georgia included others exhibiting the same symptoms.
     Curious, the physician began making weekend trips to visit these family members in their homes and even attend family reunions. On those visits, she took a computer loaded with touch-screen software to measure the degree of essential tremor seen in performing simple tasks such as handwriting. Eventually she gathered data from more than 60 family members, whom she continues to follow. Family data are key to investigating possible genetic causes of tremor, about which little is known.
     Today Testa’s work spans a shrinking divide between practicing medicine and doing the research on which that practice is based. She has become a passionate advocate for not only understanding but also educating others about ET, which is often misunderstood by physicians and patients alike.
     “Many health care providers fail to recognize ET or categorize it as a benign disorder, whereas patients often prefer to think of it as a trait rather than something needing treatment,” says Testa. “Yet ET disrupts social lives and work abilities, interrupting one’s ability to do something as simple as securing a button or writing down a phone message.” In extreme cases, it may preclude participation in the daily activities that most people take for granted, such as walking easily across a room or eating. It is not benign—and it can be treated.
     Part of what complicates diagnosis and confuses patients is that ET is actually a family of disorders covering a wide range of symptoms. While some ET cases may have a clear genetic connection, others may not. Similar tremors can be drug induced, psychogenic, or related to strokes, dystonia, or Parkinson’s disease. Non-ET tremors require different treatments, even when the symptoms appear similar to ET.
     Further complicating the diagnosis, patients also may experience more than one disorder at a time, such as ET plus Parkinson’s. One indicator that separates ET from Parkinson’s is a positive response to ethanol, and physicians find that some ET patients are self-medicating with alcohol. Another dividing line is that psychiatric and behavioral symptoms for ET often relate to anxiety while people with Parkinson’s usually manifest depression. The main distinction is tremor type: ET is generally worst with actions such as handwriting while Parkinson’s disease tremor is usually worst at rest. Another differentiator is that ET and Parkinson’s tremors respond to different medications.
     A clinical diagnosis is challenging. Because risk factors and underlying causes are unknown, health care providers fall back on defining aspects of “classic” tremor in making a diagnosis, using scales of duration (usually three to five years) and family history as supporting evidence.
     Although there is no cure for ET, it can be treated successfully. Treatment options include behavioral therapy, medication, and surgery.
     More research involving larger numbers of subjects is needed for ET. Of approximately 160 clinical trials completed to date, only two have had more than 50 participants, says Testa. Basic research in genetics and neuropathology also is vital to a better understanding of ET.
     Emory is offering a quarterly series of community education events, supported by the International Essential Tremor Foundation and Medtronics. Topics have included specific ET manifestations such as voice tremor, treatment options, clinical and translational research, and the roles that physical therapy and exercise play in ET. —Laura Hauser

For more information about essential tremor community seminars at Emory or Emory’s local ET support group, contact Lynn Ross at 404-728-6300 or slross@emory.edu. For research participation, contact Lisa Miyatake at 404-728-6364 or lmiyata@emory.edu. Emory events also are posted on the International Essential Tremor Foundation website. The IETF offers support groups, referrals, and advocacy for research at 888-387-3667 or www.essentialtremor.org. Committed to “shaking up awareness about ET,” the foundation recently hosted its first national fundraiser, Tulips for Tremors, which raised more than $51,000 through the sale of tulips.

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  Less pain, more gain

    Consumers have long been able to purchase custom-fitted golf clubs, individually tailored designer clothing, and specially molded bras. Now add to that list a custom knee.
     The Emory Orthopaedics and Spine Center is offering patients with moderate arthritis in their knees a new implant that is custom-fitted. Like traditional implants, the iForma replaces worn-out cartilage between the joints of the knee. However, with traditional knee surgery, orthropedists have to cut a large incision in the bone to fit an implant, leading to a long recovery time. Because the new stainless steel implant requires little to no bone cutting, it can be inserted in an outpatient procedure and speeds up recovery.
     The best candidate for the implant is a 40- to 60-year-old with early to moderate osteoarthritis in one knee compartment who has exhausted traditional treatments. Those might include either medications, joint injections, knee cartilage removal, or arthroscopy.
     Eventually all knee implants, even knee replacements, wear out, according to Emory orthopedist Sam Labib. Alhough doctors are unable to predict the iForma implant’s life expectancy, Labib hopes it will last a decade.
     “The implant is not a cure for arthritis,” Labib says. “The implant won’t turn you into a runner. But you can have less pain, and it can push back the date for a knee replacement. As we live longer and longer, we’re seeing patients having knee replacements earlier and earlier. We now have patients in their 80s who are on their fourth knee replacement.”
     Of the seven patients who have received the new implant at Emory, five experienced less knee pain and an improved quality of life. Emory is the first medical center in Georgia to offer the iForma implant. —Kay Torrance

Editor’s note: As consultants to ConforMIS, Inc., the manufacturer of iForma, Labib and other Emory orthopedists advised the company about the implant’s design.

For more information, visit www.emorysportsmedicine.org or call 404-778-3350. To schedule an appointment, visit www.emoryhealthcare.org or call 404-778-7777.
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  A better bladder repair

The pin on S. Robert Kovac’s white coat reads, “Because we’ve always done it that way,” just below a red cautionary sign. The Emory pelvic reconstructive surgeon’s pin refers to bladder tacking, a procedure that surgeons have performed for more than a century in an attempt to repair bladder prolapse. He believes there is a better way.
     Bladder prolapse occurs when tissues supporting the bladder fail. Over time, the bladder strains against the vaginal wall and prolapses outside its normal position within the vagina. Bladder prolapse can cause discomfort, pressure, and urinary and sexual dysfunction, and it usually occurs in older women who’ve experienced vaginal childbirth, loss of estrogen, and repetitive pelvic muscle straining.
     While bladder tacking may provide temporary relief from pain and vaginal protrusion, it fails more than half the time, with repairs typically lasting less than five years. More recently, surgeons have inserted plastic mesh to improve on the dismal failure rates of bladder tacking. However, the mesh repair has failed to live up to its promise, frequently eroding through the vaginal wall and causing bleeding and pain with coitus. It often needs to be removed.
     “I thought we needed a new approach to the issue,” says Kovac, the John D. Thompson Distinguished Professor of Gynecological Surgery. He has developed a new procedure that has a long-term, 90% success rate in his patients.
     “I frequently see women who’ve had five to 10 bladder tack operations,” Kovac says. “It’s like multiple attempts at failure.”
Assumptions about ways in which supportive tissue tears in vaginal birth have formed the basis for the bladder tacking procedure. In this new procedure, Kovac sutures the supportive tissue of the bladder to the normal supportive sites, using graft materials that assist in remodeling the torn supportive tissues.
     A procedure he developed effectively addresses a common side effect of bladder prolapse. Urinary incontinence affects approximately half of Kovac’s patients. When the tissues surrounding the urethra fail to prevent urine loss, Kovac uses a pubic bone stabilization sling to address the complication. In this procedure, a graft is placed under the urethra and is secured to the pelvic bone with bone anchors.
     Kovac says his improvements on the older procedures have occurred because of a better understanding of pelvic anatomy. The advent of MRI has allowed doctors to see more clearly how pelvic organs function.
     Kovac has shared these new techniques with more than 1,000 surgeons in the United States. “Because we’ve always done it that way” has given way to “because we’ve got a better way to do it.” —Kay Torrance

For more information about the bladder repair procedure or for an appointment at the Emory Center for Pelvic Reconstructive Surgery & Urogynecology, call 404-778-7777 or visit www.emoryhealthcare.org.
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  Beyond watching, waiting, and hoping

     Hazel’s* grown children want their Mama back. They watch, wait, and hope for a glimpse of the witty, capable woman who once had enough energy to care for her family, practice her faith, manage an office, and enjoy gardening, books, and canasta with friends.
     All Hazel seems to do now is sit in her favorite chair and stare, occasionally shifting her gaze from a couple of yellow chickadees outside the window to the only light in her den, a TV that blares loudly enough to rival a rock concert. When the phone rings, she either can’t hear it or doesn’t trouble herself to answer. Supper, if she eats at all, often consists of half an English muffin and a handful of Hershey kisses. Within the past year, Hazel has undergone hip surgery and experienced the deaths of both her younger sister and a dear friend.
     Hazel’s children know all this, and they’ve seen her weather previous adversities successfully. However, this time is different. They miss the Mama they knew.
     In response to such concerns, the Wesley Woods Transitions Senior Program, which provides education and medication management services and structured day psychiatric counseling for older adults, has launched a related program, Families in Transition. The program’s goal is to help family members understand and reconnect with older loved ones, who are struggling to cope with overwhelming life changes. According to Pat Rich, the group’s facilitator, Families in Transition “embraces the family, caregivers, and the older adult. It is broadly focused on both the needs of the caregiver and the care recipient, covering a range of aging-related issues, such as physical and mental illness, memory problems, intergenerational relationships and communication, role changes, deaths of loved ones, and/or changes in living arrangements.”
     The program involves both education and support. The educational aspect helps families understand the biologic and psychologic problems of the older adult, identify individual needs, and locate available resources.
     The facilitator also leads a group discussion with families, which focuses on caring for caregivers who are faced with the challenges of their loved one’s changing emotional and practical needs. Group members offer support to one another and exchange ideas and experiences to help alleviate feelings of burden and forestall caregiver burnout and depression.
     For those with a Hazel in their lives, there is now a step beyond watching and waiting. —Perky Daniel

*Hazel’s story represents a composite sketch rather than an actual description of one particular person’s or family’s experience.

Families in Transition groups help families find ways to develop healthier relationships with elderly loved ones. They meet on the first and third Tuesdays of every month from 6:30 to 7:30 p.m., 52 Executive Park South, Suite 5200, Atlanta, GA 30329. Referring physicians or interested participants may contact Ed Lawrence at 404-728-6975 or ed.lawrence@emoryhealthcare.org for more information.

      Ladies' Man

Backed by 20 years of study, Emory emergency medicine physician Don Stein and his research team recently proved what they had long suspected—that the sex hormone progesterone may offer protective effects to the brain. In April 2007, Stein’s article in the Annals of Emergency Medicine showed that giving progesterone to patients soon after brain injury helped reduce the risk of death and lowered the degree of disability. Soon after, a front-page Wall Street Journal article heralded Stein’s discovery, and this summer he received recognition from yet another national consumer publication. Ladies Home Journal has named Stein one of four winners of its Health Breakthrough Award for 2008, which recognizes medical professionals who have transformed an area of health that dramatically benefits women and families. For more on Stein’s research, see whsc.emory.edu/_pubs/em/medicine/index.html. To watch a video about using progesterone to reduce brain injury, see whsc.emory.edu/multimedia_4patients.cfm.

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