What Is Kennedy's Disease?

Onset between 15 and 60 years of age, with an average of 27 years. Adolescent symptoms are mainly muscle spasm and pain, fatigue of the whole body and masticatory muscles, and feminization of male breasts; middle-aged men (40-50 years old) have a slow progression of disease and often have years of muscle painful spasms before onset. Mainly manifested as muscle atrophy and weakness in the proximal (limb and pelvic girdle) limbs, which spread to the distal end as the disease progresses, and the lower limbs are heavier than the upper limbs. The muscles dominated by the medullary motor neurons are also involved, with atrophy and tremor of the tongue muscle, difficulty swallowing, poor articulation and facial weakness, often accompanied by myalgia and muscle bundle tremor, feeling normal, and in some cases with location Action tremor. The tendon reflexes weakened or disappeared without symptoms and signs of injury. Patients may have abnormalities in sexual organs and sexual characteristics such as female breast development (50% to 70%), testicular atrophy and reduced fertility. One third of patients had a right inguinal hernia. Some patients may have abnormalities in psychological tests.

Kennedy's disease

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Kennedy's disease (SBMA), mainly manifested as general muscle atrophy, paralysis and tremor of the whole body muscle, patients with general muscle weakness, especially the weakness of both lower limbs, tongue muscle atrophy is usually one of the main symptoms. There is one case per 40,000 people worldwide, and the onset age range is between 30-50 years old. Most of the patients are male. It is easily confused with motor neuron disease, and patients' lives are usually not affected. The disease can be confirmed by genetic testing.

Chinese name:

Kennedy's disease

English name:

spinal and bulbar muscular atrophy

Aliases:

Spinal bulbar muscular atrophy

Etiology:

Recessive genetic disease

Clinical manifestations:

Muscle atrophy, weakness in the lower limbs, and weakness of the facial muscles, often accompanied by myalgia and tremor of the muscle bundle, feel normal.

Visiting department:

Internal medicine

Kennedy's disease

Onset between 15 and 60 years of age, with an average of 27 years. Adolescent symptoms are mainly muscle spasm and pain, fatigue of the whole body and masticatory muscles, and feminization of male breasts; middle-aged men (40-50 years old) have a slow progression of disease and often have years of muscle painful spasms before onset. Mainly manifested as muscle atrophy and weakness in the proximal (limb and pelvic girdle) limbs, which spread to the distal end as the disease progresses, and the lower limbs are heavier than the upper limbs. The muscles dominated by the medullary motor neurons are also involved, with atrophy and tremor of the tongue muscle, difficulty swallowing, poor articulation and facial weakness, often accompanied by myalgia and muscle bundle tremor, feeling normal, and in some cases with location Action tremor. The tendon reflexes weakened or disappeared without symptoms and signs of injury. Patients may have abnormalities in sexual organs and sexual characteristics such as female breast development (50% to 70%), testicular atrophy and reduced fertility. One third of patients had a right inguinal hernia. Some patients may have abnormalities in psychological tests.

Some patients with diabetes also have diabetes in their sons or female carriers in their families. Carriers may also have sensory nerve damage and abnormalities in psychological testing. ^[1]

Kennedy's disease

The pathological changes included a marked reduction in spinal anterior horn cells and brainstem motor neurons, with intranuclear inclusions. Such inclusions also exist in non-neurological tissues, such as scrotal skin, dermis, kidney, heart, and testis. The spinal dorsal horn and posterior column were also damaged in some patients.

Kennedy's disease is an X-linked recessive genetic disease, which is a mutation in exon 1 of the androgen receptor gene. The gold standard for diagnosis is genetic testing. CAG repeat number determination of androgen receptor gene exon 1. The number of repeats in normal people is 9 to 37, while in Kennedy's disease it is 38 to
72. There is a correlation between the number of CAG repeats and the age of onset and the severity of the disease. The greater the number of repeats, the earlier the age of onset and the more severe the disease. The female offspring of the patient and their maternal relatives should be checked in time to provide a reference for future fertility.

Kennedy's disease

From the epidemiological data and clinically tracked case reports, the disease has a good prognosis and survival is close to normal life.