s the most common type of adult-onset motor neuron disease.
Adult-onset motor neuron diseases are a group of neurologic disorders
that present in adult life and are characterized primarily by
progressive degeneration and loss of motor neurons. ALS involves upper
and lower motor neurons and presents as an idiopathic, progressive
degeneration of anterior horn cells and their associated neurons,
resulting in progressive muscle weakness, atrophy, and fasciculations.[1, 2]
The disease was first described in 1869 and is commonly called Lou Gehrig disease, after the professional baseball player who died of ALS in 1941.
Other diseases classified as adult-onset motor neuron diseases have more restricted presentations and can evolve into idiopathic ALS if the patient is tracked for a long period. These diseases include the following:
Routinely measure vital capacity in the sitting and recumbent positions. Most often, the recumbent measurement declines prior to the sitting measurement. Gravity assists in lowering the diaphragm as the patient's angle of inclination is increased. As respiratory weakness progresses, patients have increasing difficulty with diaphragmatic movement when supine because of the elimination of this effect from gravity. This results in alveolar hypoventilation and ultimate oxyhemoglobin desaturation.
Difficulty sleeping may be the first symptom of hypoventilation. Patients should be questioned routinely regarding sleep habits, and if a sleep disturbance develops, measure vital capacities sitting and supine. In addition, perform overnight oxygen saturation monitoring to assess for nocturnal hypoxemia and the need for nocturnal noninvasive intermittent positive pressure ventilation (IPPV).
Also see the following:
The disease was first described in 1869 and is commonly called Lou Gehrig disease, after the professional baseball player who died of ALS in 1941.
Other diseases classified as adult-onset motor neuron diseases have more restricted presentations and can evolve into idiopathic ALS if the patient is tracked for a long period. These diseases include the following:
- Progressive bulbar palsy - Pure bulbar involvement
- Progressive muscular atrophy - Pure lower motor neuron degeneration
- - Pure upper motor neuron degeneration
- Adult-onset spinal muscular atrophy - Includes a broad range of primary motor neuron diseases classified by pattern of inheritance, distribution of weakness, or age of onset
Respiratory complications
All deaths directly caused by ALS result from respiratory complications. This occurs primarily from the patient's inability to ventilate as respiratory muscle weakness progresses. In patients with bulbar weakness, aspiration of secretions or food may occur and precipitate pneumonia, resulting in further respiratory compromise; therefore, aggressive respiratory management is necessary in the comprehensive care of patients with ALS.Routinely measure vital capacity in the sitting and recumbent positions. Most often, the recumbent measurement declines prior to the sitting measurement. Gravity assists in lowering the diaphragm as the patient's angle of inclination is increased. As respiratory weakness progresses, patients have increasing difficulty with diaphragmatic movement when supine because of the elimination of this effect from gravity. This results in alveolar hypoventilation and ultimate oxyhemoglobin desaturation.
Difficulty sleeping may be the first symptom of hypoventilation. Patients should be questioned routinely regarding sleep habits, and if a sleep disturbance develops, measure vital capacities sitting and supine. In addition, perform overnight oxygen saturation monitoring to assess for nocturnal hypoxemia and the need for nocturnal noninvasive intermittent positive pressure ventilation (IPPV).
Special concerns
An attorney should be consulted regarding a "living will" and power of attorney should be established prior to the terminal phase of this ultimately fatal disease.Also see the following:
My father was diagnosed with ALS in the summer of 2013; His initial symptoms were quite noticeable. He first experienced weakness in his right arm and his speech and swallowing abilities were profoundly affected. We all did our best to seek help for this disease, no medications they prescribed worked ,we were all scared we might lose him due to his condition, as he had been his brother's caregiver a few years earlier for the same disease before he passed. doctor recommend natural treatment from multivitamincare .org for his ALS we have no choice to give a try on natural organic treatment ,this herbal cure has effectively reverse my father condition ,losing his balance which led to stumbling and falling stop after the completing the herbal supplement which include his weakness in his right arm and his speech, home remedies from multivitamincare .org is the best although their service is a little bit expensive but it worth it, they save lives.
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