Amyotrophic lateral sclerosis natural history, complications and prognosis: Difference between revisions
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{{Amyotrophic lateral sclerosis}} | {{Amyotrophic lateral sclerosis}} | ||
==Natural History== | |||
==Complications== | |||
==Prognosis== | |||
*Although the sequence of emerging symptoms and the rate of disease progression vary from person to person, eventually patients will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. | |||
*Difficulty swallowing and chewing impair the patient's ability to eat normally and increase the risk of choking. | |||
*Maintaining weight will then become a problem. | |||
*Because the disease usually does not affect cognitive abilities, patients are aware of their progressive loss of function and may become anxious and depressed. | |||
*A small percentage of patients go on to develop [[frontotemporal dementia]] characterized by profound personality changes; this is more common among those with a family history of dementia. | |||
*A larger proportion of patients experience mild problems with word-generation, attention, or decision-making. Cognitive function may be affected as part of the disease process or could be related to poor breathing at night (nocturnal hypoventilation). Health care professionals need to explain the course of the disease and describe available treatment options so that patients can make informed decisions in advance. | |||
*As the diaphragm and [[intercostal muscle]]s (rib cage) weaken, forced vital capacity and inspiratory pressure diminish. In bulbar onset ALS, this may occur before significant limb weakness is apparent. Bilevel positive pressure ventilation (frequently referred to by the tradename [[BiPAP]]) is frequently used to support breathing, first at night, and later during the daytime as well. | |||
*It is recommended that long before BiPAP becomes insufficient, patients (with the eventual help of their families) must decide whether to have a [[tracheostomy]] and long term mechanical ventilation. Most patients do not elect this route, and instead choose [[Palliative care|palliative hospice care]] at this point. Most people with ALS die of respiratory failure or [[pneumonia]], not the disease itself. | |||
*ALS predominantly affects the motor neurons, and in the majority of cases the disease does not impair a patient's mind, personality, intelligence, or memory. Nor does it affect a person's ability to see, smell, taste, hear, or feel touch. | |||
*Control of eye muscles is the most preserved function, although some patients with an extremely long duration of disease (20+ years) may lose eye control too. Unlike [[multiple sclerosis]], bladder and bowel control are usually preserved in ALS, although as a result of immobility and diet changes, intestinal problems such as constipation can require intensive management. | |||
==References== | ==References== | ||
{{reflist|2}} | |||
{{ | {{WH}} | ||
{{WS}} | |||
[[Category:Needs content]] | [[Category:Needs content]] | ||
Revision as of 15:58, 24 August 2012
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Natural History
Complications
Prognosis
- Although the sequence of emerging symptoms and the rate of disease progression vary from person to person, eventually patients will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms.
- Difficulty swallowing and chewing impair the patient's ability to eat normally and increase the risk of choking.
- Maintaining weight will then become a problem.
- Because the disease usually does not affect cognitive abilities, patients are aware of their progressive loss of function and may become anxious and depressed.
- A small percentage of patients go on to develop frontotemporal dementia characterized by profound personality changes; this is more common among those with a family history of dementia.
- A larger proportion of patients experience mild problems with word-generation, attention, or decision-making. Cognitive function may be affected as part of the disease process or could be related to poor breathing at night (nocturnal hypoventilation). Health care professionals need to explain the course of the disease and describe available treatment options so that patients can make informed decisions in advance.
- As the diaphragm and intercostal muscles (rib cage) weaken, forced vital capacity and inspiratory pressure diminish. In bulbar onset ALS, this may occur before significant limb weakness is apparent. Bilevel positive pressure ventilation (frequently referred to by the tradename BiPAP) is frequently used to support breathing, first at night, and later during the daytime as well.
- It is recommended that long before BiPAP becomes insufficient, patients (with the eventual help of their families) must decide whether to have a tracheostomy and long term mechanical ventilation. Most patients do not elect this route, and instead choose palliative hospice care at this point. Most people with ALS die of respiratory failure or pneumonia, not the disease itself.
- ALS predominantly affects the motor neurons, and in the majority of cases the disease does not impair a patient's mind, personality, intelligence, or memory. Nor does it affect a person's ability to see, smell, taste, hear, or feel touch.
- Control of eye muscles is the most preserved function, although some patients with an extremely long duration of disease (20+ years) may lose eye control too. Unlike multiple sclerosis, bladder and bowel control are usually preserved in ALS, although as a result of immobility and diet changes, intestinal problems such as constipation can require intensive management.