![]() ![]() Many doctors use the term motor neuron disease and ALS interchangeably.Įarly symptoms of ALS usually include muscle weakness or stiffness in a limb or muscles of the mouth or throat (so-called bulbar muscles). It causes rapid loss of muscle control and eventual paralysis. Symptoms may include breathlessness, shortness of breath that occurs while lying down, recurrent chest infections, disturbed sleep, poor concentration and/or memory, confusion, morning headaches, and fatigue.Īmyotrophic lateral sclerosis (ALS), also known as classical motor neuron disease, affects both the upper and lower motor neurons. ![]() Respiratory insufficiency, a condition in which the lungs cannot properly take in oxygen or expel carbon dioxide, is a feature of most MNDs. Though there are several types of MNDs, they all cause muscle weakness that gradually worsens over time and leads to disability. Female children have a 50 percent chance of inheriting their female parent's X chromosome and a safe X chromosome from their male parent, which usually makes them asymptomatic carriers of the mutation. Female children inherit an X chromosome from each parent. ![]() Male children have a 50 percent risk of inheriting the abnormal X chromosome and developing the disease. Male children inherit an X chromosome from female parent and a Y chromosome from their male parent. X-linked inheritance occurs when the female parent carries the gene on one X chromosome and passes the disorder along to male children.Autosomal recessive diseases often affect more than one person in the same generation (e.g., siblings). These parents are likely to be asymptomatic (without symptoms of the disease). Autosomal recessive means a person must inherit a copy of the defective gene from each parent.There is a 50 percent chance that a child of an affected person will inherit the abnormal gene and develop the disease. Autosomal dominant means that a person needs to inherit only one copy of the defective gene from one parent with the disorder to be at risk of the disease.These conditions are usually inherited in one of several patterns: In cases where a motor neuron disease is inherited, it is usually caused by mutations in a single gene. MNDs are classified according to whether the loss of function (degeneration) is inherited (passed down through family genetics) sporadic (no family history) and whether they affect the upper motor neurons, lower motor neurons, or both. Over time, individuals with MNDs may lose the ability to walk or control other movements. This can make voluntary movements slow and difficult. When the lower motor neurons cannot receive signals from the upper motor neurons, it can cause muscle stiffness (spasticity) and overactive reflexes. These twitches (fasciculations) can be seen and felt below the surface of the skin. The muscles may also start to spontaneously twitch. When the muscles cannot receive signals from the lower motor neurons, they begin to weaken and shrink in size (muscle atrophy or wasting). Upper motor neurons direct the lower motor neurons to produce muscle movements. ![]() Messages or signals from nerve cells in the brain (upper motor neurons) are typically transmitted to nerve cells in the brain stem and spinal cord (lower motor neurons) and then to muscles in the body. This group includes diseases such as amyotrophic lateral sclerosis, progressive bulbar palsy, primary lateral sclerosis, progressive muscular atrophy, spinal muscular atrophy, Kennedy's disease, and post-polio syndrome. Motor neuron diseases (MNDs) are a group of progressive neurological disorders that destroy motor neurons, the cells that control skeletal muscle activity such as walking, breathing, speaking, and swallowing. ![]()
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