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Kennedy's Disease
A progressive debilitating neurodegenerative disorder resulting in muscle cramps and progressive weakness due to degeneration of motor neurons in the brainstem and spinal cord
Prevalence
Unknown
Age of Onset
ICD-10
G12.2
Inheritance
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
5 Facts you should know
FACT
Kennedy's disease is an inherited motor neuron disease that affects males
FACT
The onset of the disease is usually between the ages of 20 and 40, although it has been diagnosed in men from their teens to their 70s
FACT
The disease affects the lower motor neurons that are responsible for the movement of many muscles in the legs, arms, mouth, and throat
FACT
Kennedy disease is caused by a change (mutation) in the AR gene that encodes for a protein known as the androgen receptor on the X chromosome
FACT
Kennedy disease is named after William R. Kennedy, MD, who described the condition in the 1960s
Interest over time
Common signs & symptoms
Decreased fertility
Dysarthria
Dysphonia
Erectile dysfunction
Gait disturbance
Gynecomastia
Hyporeflexia
Muscular hypotonia
Current treatments
There is currently no cure for Kennedy's disease. Treatment aims to relieve symptoms, maintain maximal function, and improve quality of life. Physical therapy and rehabilitation, including the use of braces and walkers, are the best chance for remaining ambulatory as the disease progresses. Some people with Kennedy disease have breast reduction surgery for gynecomastia. The use of anti-androgens has been shown to improve some aspects of the disease is some people; the androgen-dependent nature of the disease is the rationale for the use of anti-androgens in treating Kennedy's disease.