Information on Kennedy's Disease

Kennedy's disease, also called is spinal and bulbar muscular atrophy. It is an advanced degenerative condition affecting worse motor neurons. It is one of nine neurodegenerative disorders result by a polyglutamine repeat expansion. Kennedy illness affects the lower motor neurons, which are responsible for movement of the arms. It also affects the nerves which supply the bulbar muscles, which control breathing, swallowing, and talking. Kennedy disease is caused by a genetic defect on the X (female) chromosome.

Kennedy disease is likely to occur in 1 in 40,000 individuals worldwide. Kennedy's illness means the patient's mother carries the faulty gene on one of her X chromosomes. Daughters of patients with Kennedy's disease are also carriers and have a 1 in 2 chance of having a son affected with the disease. Symptoms of Kennedy's disease comprise weakening and wasting of the muscles, particularly the arms and legs. Other major symptoms embrace severe cramps and problems with speech and swallowing. The muscles of the face, lips, tongue, mouth, throat, vocal chords, trunk and limbs may be affected.

Very large calves may also be found in several patients with this disorder. The symptoms usually first seem when the person is aged between 30 and 50 years. Kennedy's disease is gradually progressive. Individuals tend to remain ambulatory until late in the disease, although some may be wheelchair-bound during later stages. The life span of individuals with Kennedy's disease is usually normal. Kennedy disease may have other effects on the body, including gynecomastia, meaning enlargement of breast tissue in males, disorders of fats (lipids) in the blood and diabetes mellitus (blood sugar problems).

There is no known cure for Kennedy's disease. Treatment is symptomatic and helpful. Physical therapy, occupational therapy, and speech therapy are important for maintaining an individual's abilities and for adapting to the progression of the disease. Adaptive equipment can assist maintain mobility and independence. Genetic counseling is also helpful this condition because Kennedy disease is related to the X chromosome, so if a woman is a carrier of the defective gene her sons have a 50% chance of have the disorder and her daughters have a 50% chance of being a carrier. Fathers cannot pass Kennedy disease on to their sons.