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Study Finds Drug May Cut Down Involuntary Movements in Huntington's Disease Patients By 25 Percent
February 16, 2006Many Patients Report Even Greater Improvement
The medication tetrabenazine cut down involuntary movement in patients with Huntington's disease on average by about 25 percent, with many patients experiencing a greater improvement, according to a study in the February 14 issue of the journal Neurology.
Overall, patients who received the medication were six times as likely to be considered by their doctors to have improved considerably, compared to participants who received a placebo.
Dr. Kathleen M. Shannon, neurologist and Huntington's disease specialist at the Huntington's Disease Society of America Center of Excellence at Rush University Medical Center, led the Rush study. Rush was one of 16 sites to participate in the randomized, controlled study which involved 84 patients.
Tetrabenazin is available in Europe and Canada - but not the United States, but is currently being reviewed by the U.S. Food and Drug Administration. If approved, the medication would be the first authorized by the agency expressly for the treatment of Huntington's disease, which affects about 30,000 people in the United States.
"Huntington's disease is an inherited brain disorder that causes patients to experience uncontrollable jerky movements (chorea), as well as changes in personality, behavior, thinking and memory. There are no FDA-approved treatments for the chorea. Anti-psychotic drugs like haloperidol (Haldol) are commonly used to suppress chorea, but they cause many different side effects. This study shows that tetrabenazine can decrease chorea, and the drug is well-tolerated by most research subjects," said Shannon.
Tetrabenazine was originally developed in the 1950s to treat psychosis, but was quickly pushed aside by more effective medications. But doctors in the United Kingdom found it to be effective to treat the excessive involuntary movements of Huntington's, and it is approved for use in several nations. In the United States, tetrabenazine is designated as an "orphan drug" by the FDA since it's targeted to a disease directly affecting fewer than 200,000 people in the nation.
The symptom that tetrabenazine treats - involuntary, writhing movements of the limbs, face, and sometimes the entire body - is the hallmark symptom of Huntington's disease, an inherited neurodegenerative disorder that worsens as brain cells known as medium spiny neurons are killed off by a mutant protein.
The disease brings with it an array of other difficulties as well, including cognitive problems, changes in personality, and psychiatric problems like depression. As many as one-quarter of patients with the disease attempt suicide, and many suffer from progressive cognitive decline.
Unlike Alzheimer's disease, where patients usually lose their memory and insight into their disease at some point, most Huntington's patients understand exactly what is happening to them throughout most of their illness.
The disease usually strikes people in their 30s and 40s, though some patients are affected as early as childhood, while others aren't affected until their older years. Virtually everyone with the disease had a parent with the disease, and children of a person with Huntington's have a 50-percent chance of inheriting the disease.
Thirteen years ago the gene that causes the disease was identified by scientists, and now a simple blood test can tell people whether they will develop the disease or not. But since there is no way known to prevent the disease or slow its progression, and for other reasons as well, many patients decline the test, instead waiting to see if they develop symptoms like the ones they witnessed in a parent.
Patients usually live for 15 to 20 years after the onset of symptoms.
"Tetrabenazine does not help all the features of Huntington's disease, and other medications are commonly needed to improve mood and behavior. For those Huntington's disease patients whose function is impaired by chorea, however, tetrabenazine can help activities of daily living, and improve functional capacity," Shannon said.
"If the drug is approved, physicians will need to work carefully with patients and caregivers to find the appropriate dose. Side effects were seen in about 25% of subjects in the study. The most common side effect was sleepiness, but restlessness, slowed movement and depression were also seen in some subjects. Suicide is common in Huntington's disease, and one subject committed suicide during the study. Patients will also need close supervision so that suicide can be prevented."
The study was conducted by the Huntington Study Group and was funded by Prestwick Pharmaceuticals of Washington, DC, the company that owns the rights to develop and sell the medication in the United States.
The Huntington Study Group is a non-profit, cooperative group of Huntington's disease experts from medical centers throughout North America, Europe and Australia who are dedicated to improving treatment for persons affected by the disease. HSG is supported by the Huntington's Disease Society of America, the Hereditary Disease Foundation, the Huntington Society of Canada, and the High Q Foundation. More information is available at www.Huntington-Study-Group.org.
This release was written by the Parkinson Study Group.
Rush University Medical Center
Dr. Kathleen M. Shannon, neurologist and Huntington's disease specialist at the Huntington's Disease Society of America Center of Excellence at Rush University Medical Center, led the Rush study. Rush was one of 16 sites to participate in the randomized, controlled study which involved 84 patients.
Tetrabenazin is available in Europe and Canada - but not the United States, but is currently being reviewed by the U.S. Food and Drug Administration. If approved, the medication would be the first authorized by the agency expressly for the treatment of Huntington's disease, which affects about 30,000 people in the United States.
"Huntington's disease is an inherited brain disorder that causes patients to experience uncontrollable jerky movements (chorea), as well as changes in personality, behavior, thinking and memory. There are no FDA-approved treatments for the chorea. Anti-psychotic drugs like haloperidol (Haldol) are commonly used to suppress chorea, but they cause many different side effects. This study shows that tetrabenazine can decrease chorea, and the drug is well-tolerated by most research subjects," said Shannon.
Tetrabenazine was originally developed in the 1950s to treat psychosis, but was quickly pushed aside by more effective medications. But doctors in the United Kingdom found it to be effective to treat the excessive involuntary movements of Huntington's, and it is approved for use in several nations. In the United States, tetrabenazine is designated as an "orphan drug" by the FDA since it's targeted to a disease directly affecting fewer than 200,000 people in the nation.
The symptom that tetrabenazine treats - involuntary, writhing movements of the limbs, face, and sometimes the entire body - is the hallmark symptom of Huntington's disease, an inherited neurodegenerative disorder that worsens as brain cells known as medium spiny neurons are killed off by a mutant protein.
The disease brings with it an array of other difficulties as well, including cognitive problems, changes in personality, and psychiatric problems like depression. As many as one-quarter of patients with the disease attempt suicide, and many suffer from progressive cognitive decline.
Unlike Alzheimer's disease, where patients usually lose their memory and insight into their disease at some point, most Huntington's patients understand exactly what is happening to them throughout most of their illness.
The disease usually strikes people in their 30s and 40s, though some patients are affected as early as childhood, while others aren't affected until their older years. Virtually everyone with the disease had a parent with the disease, and children of a person with Huntington's have a 50-percent chance of inheriting the disease.
Thirteen years ago the gene that causes the disease was identified by scientists, and now a simple blood test can tell people whether they will develop the disease or not. But since there is no way known to prevent the disease or slow its progression, and for other reasons as well, many patients decline the test, instead waiting to see if they develop symptoms like the ones they witnessed in a parent.
Patients usually live for 15 to 20 years after the onset of symptoms.
"Tetrabenazine does not help all the features of Huntington's disease, and other medications are commonly needed to improve mood and behavior. For those Huntington's disease patients whose function is impaired by chorea, however, tetrabenazine can help activities of daily living, and improve functional capacity," Shannon said.
"If the drug is approved, physicians will need to work carefully with patients and caregivers to find the appropriate dose. Side effects were seen in about 25% of subjects in the study. The most common side effect was sleepiness, but restlessness, slowed movement and depression were also seen in some subjects. Suicide is common in Huntington's disease, and one subject committed suicide during the study. Patients will also need close supervision so that suicide can be prevented."
The study was conducted by the Huntington Study Group and was funded by Prestwick Pharmaceuticals of Washington, DC, the company that owns the rights to develop and sell the medication in the United States.
The Huntington Study Group is a non-profit, cooperative group of Huntington's disease experts from medical centers throughout North America, Europe and Australia who are dedicated to improving treatment for persons affected by the disease. HSG is supported by the Huntington's Disease Society of America, the Hereditary Disease Foundation, the Huntington Society of Canada, and the High Q Foundation. More information is available at www.Huntington-Study-Group.org.
This release was written by the Parkinson Study Group.
Rush University Medical Center
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Test reveals effectiveness of potential Huntington's disease drugs
A test using cultured cells provides an effective way to screen drugs against Huntington's disease and shows that two compounds - memantine and riluzole - are most effective at keeping cells alive under conditions that mimic the disorder, UT Southwestern Medical Center researchers report.
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"Booster rocket" malfunction implicated in Huntington's disease
CNRS and Inserm research scientists at the Institut Curie have shed new light on the function of huntingtin, the protein whose mutation underlies Huntington's disease. This neurodegenerative disease, like Alzheimer's or Parkinson's, is characterized by the abnormal death of certain neurons. The scientists have discovered that huntingtin, like a "booster rocket", accelerates the transport of a neuron survival factor. When huntingtin is mutated, the "booster rocket" malfunctions, transport slows, protection wanes, and neurons die. This discovery, published in the 9 July 2004 issue of Cell, could in time lead to novel therapeutic methods of blocking the accelerated death of neurons.
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A new pathway for halting neuronal death in Huntington's disease
The body is an extremely complex puzzle in which every piece plays a critical role. Should pieces disappear harmony is compromised. Such is the case with certain neurodegenerative diseases; when neurons suddenly die, the body's ability to function properly is jeopardized. CNRS (1) and INSERM biologists from the Curie Institute are working to understand how neurons die in one specific neurodegenerative disease: Huntington's disease. They have just announced the discovery of two new factors capable of blocking cell death induced in Huntington's disease. They may eventually provide targets for the therapeutic treatment of this type of disease. These discoveries were published in the 7th of Ju
Patients' views about their illness can help treatment planning
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