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Kennedy disease

Kennedy disease (KD) or X-linked spinal-bulbar muscle atrophy is a neuromuscular disease associated with mutations of the androgen receptor (AR). Because of its endocrine manifestations related to the impairment of the AR, it can be viewed as a variation of the disorders of the androgen insensitivity syndrome (AIS). It is named after WR Kennedy, a neurologist who was among the first to describe this disease. more...

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Genetics

As a sex-linked disease, KD affects males, while females are carriers. The gene for the AR is located on the X chromosome (Xq11-q12).

Pathology

The distinctive AR mutation of Kennedy disease, reported in 1991, involves multiplied CAG repeats in the first exon (trinucleotide repeats). Such a CAG repeat encodes a polyglutamine tract in a part of the androgen receptor outside of the binding sites. The more CAG repeats are present, the more severe the disease. The mechanism by which this type of mutation causes neuromuscular disease is not completely understood, specifically as complete AIS does not affect neuromuscular activity. KD may share mechanistic features with other neurodegenerative disorders that are caused by polyglutamine expansion, such as Huntington's disease.

Signs and symptoms

Ages of onset and severity of manifestations in affected males vary from adolescence to old age, but most commonly develop in middle adult life. The latest onset was described in a male of 84 years of age. KD does not usually compromise longevity. The syndrome has neuromuscular and endocrine manifestations:

Neuromuscular

Early signs often include weakness of tongue and mouth muscles, fasciculations, and gradually increasing weakness of proximal limb muscles with muscle wasting. In some cases, premature muscle fatigue begins in adolescence. Neuromuscular management is supportive, and the disease progresses very slowly and often does not lead to extreme disability.

Endocrine

Endocrine manifestations of this disorder are variable and rarely include underdevelopment of internal or external genitalia. In other words, most people affected with Kennedy disease are relatively normal XY men with normal fertility. However, exaggerated and persistent gynecomastia is common and often the only symptom, while in more severe forms testicular atrophy and infertility have been described. Many affected men have the mildly high LH, testosterone, and estradiol levels characteristic of other forms of the androgen insensitivity syndrome.

Homozygous females

Homozygous females, whose both X chromosomes have a mutation leading to CAG expansion of the AR gene, show only mild symptoms of muscle cramps and twitching. No endocrinopathy has been described.

History

This disorder was described by Kennedy in 1968. In 1991 it was recognized that the AR is involved in the disease process. The disease is probably more common than originally thought. A study in Scandinavia suggested a prevalence of 1.3/8,500 making KD the most common form of motor neuron disease in the specific area studied; nobody had been diagnosed before 1995. It has been suggested that some men with KS are may be misdiagnosed to have amyotrophic lateral sclerosis (ALS, also Lou Gehrig's disease).

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Strengthen your heart: the herb arjuna holds promise for those with cardiovascular disease - Herb Brief
From Natural Health, 10/1/02 by Jennifer Kennedy

Health Claims

Arjuna (Terminalia arjuna) is used to treat cardiovascular diseases like angina, cardiomyopathy, and congestive heart failure. It is taken on its own or paired with heart disease drugs.

SUPPLEMENTAL ARJUNA comes from the bark of a tall Indian tree. Ayurvedic doctors have used the bark to treat heart ailments for more than 300 years. The herb is just gaining recognition in America.

How It Works

Research suggests that compounds in arjuna called saponin glycosides enhance your heart's pumping action. The bark contains calcium and magnesium as well, which may play a role in improving muscle function. The antioxidants in the bark strengthen your veins and may prevent heart disease.

Evidence

Several small clinical studies conducted in India support arjuna's use for heart ailments.

In a clinical trial published in the Journal of the Association of Physicians of India in 1999, researchers gave 17 patients with stable angina pectoris either an Ayurvedic formula containing arjuna or the angina drug isosorbide mononitrate. The arjuna formula reduced the frequency of angina attacks from an average of 79 per week to an average of 29 per week. Both groups of patients experienced a reduction in symptoms like elevated heart rate and blood pressure.

A 1997 study in the Indian Heart Journal suggests that arjuna is effective for weakened heart muscles. In it, 24 patients who had suffered heart attacks took either conventional drugs only or conventional drugs along with 500 mg of arjuna every eight hours for three months. Those who received arjuna with the drugs experienced an 18 percent greater improvement in heart pumping activity than those who received only the conventional drugs.

In a 1995 double-blind trial in the International Journal of Cardiology, 12 patients with chronic congestive heart failure were given either 500 mg of arjuna every eight hours or a placebo; both groups also continued their regular drug therapy. Patients who had taken arjuna for two weeks experienced a 19 percent slower heart rate (a desirable effect) than those who took a placebo. Symptoms like fatigue, high blood pressure, and frequent heart contractions also improved. All 12 patients went on taking arjuna for about two years. Ten continued to improve for two to three more months and maintained those improvements during the entire study. The other two participants died from heart disease.

How to Take It

Arjuna is so new in this country that it's not yet found in most stores, but it is sold on websites like www.vitaminlife.com and www.smallflower.com.

For cardiovascular problems, take 1,500 mg of arjuna divided into three doses daily for three to four weeks. Then gradually increase your daily dose to 3,000 mg (also divided into three doses); you can stay at this dose indefinitely, says Virender Sodhi, M.D., N.D., a naturopath and Ayurvedic physician in Bellevue, Wash. This dose is more effective than the smaller doses used in studies, he says.

Caveats

Arjuna appears to be safe. Consult your doctor before taking arjuna if you already take heart medication or if you are pregnant.

The Bottom Line

Centuries of anecdotal evidence support arjuna's ability to treat heart ailments. And the studies that have been conducted on it, while small, are promising.

Jennifer Kennedy is a freelance writer in the Boston area.

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