Amyotrophic lateral sclerosis history and symptoms: Difference between revisions
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<ref name="pmid19192301">{{cite journal| author=Wijesekera LC, Leigh PN| title=Amyotrophic lateral sclerosis. | journal=Orphanet J Rare Dis | year= 2009 | volume= 4 | issue= | pages= 3 | pmid=19192301 | doi=10.1186/1750-1172-4-3 | pmc=2656493 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19192301 }} </ref> | <ref name="pmid19192301">{{cite journal| author=Wijesekera LC, Leigh PN| title=Amyotrophic lateral sclerosis. | journal=Orphanet J Rare Dis | year= 2009 | volume= 4 | issue= | pages= 3 | pmid=19192301 | doi=10.1186/1750-1172-4-3 | pmc=2656493 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19192301 }} </ref> | ||
==Emerging Symptoms== | ==Emerging Symptoms== | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]
Overview
The onset of ALS may be so subtle that the symptoms are frequently overlooked. The earliest LMN symptoms are weakness due to muscle atrophy. This is followed by twitching, cramping, or stiffness of affected muscles; muscle weakness affecting an arm or a leg; and/or slurred and nasal speech. The twitching, cramping, etc. associated with ALS is a result of the dying muscle, due to denervation and sometimes probable re-innervation.
History and Symptoms
History
ALS is a neurodegenerative disease that ultimately leads to paralysis. Patients may have a positive history of:
- Weakness start proximally/ distally in upper or lower limbs.
- Twitching
- Cramping
- Gradually Stiffness also develop in affected muscles
- Difficulty swallowing/ speech.
- Drooling
Symptoms
Common symptoms of ALS include:
- Muscle weakness
- Muscle stiffness
- Gait disturbances
- Dysarthria/ dysphagia with bulbar-onset ALS.
- Respiratory difficulties
Emerging Symptoms
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.
References
- ↑ Wijesekera LC, Leigh PN (2009). "Amyotrophic lateral sclerosis". Orphanet J Rare Dis. 4: 3. doi:10.1186/1750-1172-4-3. PMC 2656493. PMID 19192301.