Amyotrophic lateral sclerosis natural history, complications and prognosis: Difference between revisions

Jump to navigation Jump to search
No edit summary
 
(19 intermediate revisions by 4 users not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{Amyotrophic lateral sclerosis}}
{{CMG}}{{AE}}{{MMJ}}
==Overview==
The prognosis of Amyotrophic lateral sclerosis is generally poor with the majority of patients dying within 3-5 years of diagnosis.
==Natural History, Complications, and Prognosis==
===Natural History===
*The symptoms of Amyotrophic lateral sclerosis usually develop in the fifth decade of life.<ref name="pmid18079297">{{cite journal| author=Logroscino G, Traynor BJ, Hardiman O, Chio' A, Couratier P, Mitchell JD et al.| title=Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues. | journal=J Neurol Neurosurg Psychiatry | year= 2008 | volume= 79 | issue= 1 | pages= 6-11 | pmid=18079297 | doi=10.1136/jnnp.2006.104828 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18079297  }} </ref><ref name="pmid26629397">{{cite journal| author=Zarei S, Carr K, Reiley L, Diaz K, Guerra O, Altamirano PF et al.| title=A comprehensive review of amyotrophic lateral sclerosis. | journal=Surg Neurol Int | year= 2015 | volume= 6 | issue=  | pages= 171 | pmid=26629397 | doi=10.4103/2152-7806.169561 | pmc=4653353 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26629397  }} </ref>
*Only 5% of the cases have an onset <30 years of age.<ref name="pmid18079297">{{cite journal| author=Logroscino G, Traynor BJ, Hardiman O, Chio' A, Couratier P, Mitchell JD et al.| title=Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues. | journal=J Neurol Neurosurg Psychiatry | year= 2008 | volume= 79 | issue= 1 | pages= 6-11 | pmid=18079297 | doi=10.1136/jnnp.2006.104828 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18079297  }} </ref><ref name="pmid26629397">{{cite journal| author=Zarei S, Carr K, Reiley L, Diaz K, Guerra O, Altamirano PF et al.| title=A comprehensive review of amyotrophic lateral sclerosis. | journal=Surg Neurol Int | year= 2015 | volume= 6 | issue=  | pages= 171 | pmid=26629397 | doi=10.4103/2152-7806.169561 | pmc=4653353 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26629397  }} </ref>
===Complications===
*With the progression of ALS, patients develop the distinctive feature of a combination of UMN and LMN degeneration signs within the same CNS region.


{{Amyotrophic lateral sclerosis}}
*Common complications of ALS include:<ref name="pmid26629397">{{cite journal| author=Zarei S, Carr K, Reiley L, Diaz K, Guerra O, Altamirano PF et al.| title=A comprehensive review of amyotrophic lateral sclerosis. | journal=Surg Neurol Int | year= 2015 | volume= 6 | issue=  | pages= 171 | pmid=26629397 | doi=10.4103/2152-7806.169561 | pmc=4653353 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26629397  }} </ref>
**[[Pulmonary]] complications:
**[[Dyspnea]], [[Orthopnea]], [[Hypoventilation]], [[Pneumonia]]
**[[Cognitive|Cognitive dysfunction]]
**Weight loss, which is an indicative of a poor prognosis
**The main cause of death in ALS is respiratory failure.
 
===Prognosis===
 
*Prognosis is generally poor, and the 30 months survival rate of patients with ALS is approximately 5%.<ref name="pmid19762894">{{cite journal| author=Talbot K| title=Motor neuron disease: the bare essentials. | journal=Pract Neurol | year= 2009 | volume= 9 | issue= 5 | pages= 303-9 | pmid=19762894 | doi=10.1136/jnnp.2009.188151 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19762894  }} </ref>
*Only 20% of the patients survive between 5 and 10 years after symptoms onset.<ref name="pmid19762894">{{cite journal| author=Talbot K| title=Motor neuron disease: the bare essentials. | journal=Pract Neurol | year= 2009 | volume= 9 | issue= 5 | pages= 303-9 | pmid=19762894 | doi=10.1136/jnnp.2009.188151 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19762894  }} </ref>
*Reduced survival to the disease is related to the older age of symptom onset, early [[respiratory]] [[muscle]] dysfunction, and [[bulbar]] onset disease. On the other hand, [[limb]]-onset disease, younger age at presentation of the disease and longer diagnostic delay are independent predictors of prolonged [[survival]].<ref name="pmid17210625">{{cite journal| author=Vucic S, Kiernan MC| title=Abnormalities in cortical and peripheral excitability in flail arm variant amyotrophic lateral sclerosis. | journal=J Neurol Neurosurg Psychiatry | year= 2007 | volume= 78 | issue= 8 | pages= 849-52 | pmid=17210625 | doi=10.1136/jnnp.2006.105056 | pmc=2117729 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17210625  }} </ref>


==Natural History==
Some ALS subtypes vary according to prognosis. [[LMN]] form of ALS, which includes flail-limb variant and PMA, shows a slower progression than other forms of ALS. A prognosis of 2–4 years is seen in the pure bulbar palsy phenotype, which usually affects women older than 65 years of age. In this type of ALS, the disease remains localized to the [[oropharyngeal]] musculature and [[UMN]] features predominate.<ref name="pmid15204021">{{cite journal| author=Sanjak M, Konopacki R, Capasso R, Roelke KA, Peper SM, Houdek AM et al.| title=Dissociation between mechanical and myoelectrical manifestation of muscle fatigue in amyotrophic lateral sclerosis. | journal=Amyotroph Lateral Scler Other Motor Neuron Disord | year= 2004 | volume= 5 | issue= 1 | pages= 26-32 | pmid=15204021 | doi=10.1080/14660820310017551 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15204021  }} </ref>
==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}}
{{Reflist|2}}


{{WH}}
{{WH}}
{{WS}}
{{WS}}
 
[[Category: (name of the system)]]
[[Category:Needs content]]

Latest revision as of 17:49, 14 September 2021

Amyotrophic lateral sclerosis Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Amyotrophic lateral sclerosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Interventions

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Amyotrophic lateral sclerosis natural history, complications and prognosis On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Amyotrophic lateral sclerosis natural history, complications and prognosis

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Amyotrophic lateral sclerosis natural history, complications and prognosis

CDC on Amyotrophic lateral sclerosis natural history, complications and prognosis

Amyotrophic lateral sclerosis natural history, complications and prognosis in the news

Blogs on Amyotrophic lateral sclerosis natural history, complications and prognosis

Directions to Hospitals Treating Psoriasis

Risk calculators and risk factors for Amyotrophic lateral sclerosis natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

Overview

The prognosis of Amyotrophic lateral sclerosis is generally poor with the majority of patients dying within 3-5 years of diagnosis.

Natural History, Complications, and Prognosis

Natural History

  • The symptoms of Amyotrophic lateral sclerosis usually develop in the fifth decade of life.[1][2]
  • Only 5% of the cases have an onset <30 years of age.[1][2]

Complications

  • With the progression of ALS, patients develop the distinctive feature of a combination of UMN and LMN degeneration signs within the same CNS region.

Prognosis

  • Prognosis is generally poor, and the 30 months survival rate of patients with ALS is approximately 5%.[3]
  • Only 20% of the patients survive between 5 and 10 years after symptoms onset.[3]
  • Reduced survival to the disease is related to the older age of symptom onset, early respiratory muscle dysfunction, and bulbar onset disease. On the other hand, limb-onset disease, younger age at presentation of the disease and longer diagnostic delay are independent predictors of prolonged survival.[4]

Some ALS subtypes vary according to prognosis. LMN form of ALS, which includes flail-limb variant and PMA, shows a slower progression than other forms of ALS. A prognosis of 2–4 years is seen in the pure bulbar palsy phenotype, which usually affects women older than 65 years of age. In this type of ALS, the disease remains localized to the oropharyngeal musculature and UMN features predominate.[5]

References

  1. 1.0 1.1 Logroscino G, Traynor BJ, Hardiman O, Chio' A, Couratier P, Mitchell JD; et al. (2008). "Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues". J Neurol Neurosurg Psychiatry. 79 (1): 6–11. doi:10.1136/jnnp.2006.104828. PMID 18079297.
  2. 2.0 2.1 2.2 Zarei S, Carr K, Reiley L, Diaz K, Guerra O, Altamirano PF; et al. (2015). "A comprehensive review of amyotrophic lateral sclerosis". Surg Neurol Int. 6: 171. doi:10.4103/2152-7806.169561. PMC 4653353. PMID 26629397.
  3. 3.0 3.1 Talbot K (2009). "Motor neuron disease: the bare essentials". Pract Neurol. 9 (5): 303–9. doi:10.1136/jnnp.2009.188151. PMID 19762894.
  4. Vucic S, Kiernan MC (2007). "Abnormalities in cortical and peripheral excitability in flail arm variant amyotrophic lateral sclerosis". J Neurol Neurosurg Psychiatry. 78 (8): 849–52. doi:10.1136/jnnp.2006.105056. PMC 2117729. PMID 17210625.
  5. Sanjak M, Konopacki R, Capasso R, Roelke KA, Peper SM, Houdek AM; et al. (2004). "Dissociation between mechanical and myoelectrical manifestation of muscle fatigue in amyotrophic lateral sclerosis". Amyotroph Lateral Scler Other Motor Neuron Disord. 5 (1): 26–32. doi:10.1080/14660820310017551. PMID 15204021.

Template:WH Template:WS