Amyotrophic lateral sclerosis diagnostic study of choice: Difference between revisions

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== Overview ==
== Overview ==
ALS is a clinical diagnosis. Due to the absence of any disease bio-markers, the presence of both UMN and LMN signs and symptoms in the same body region along with the progression of these symptoms. Modified ''El-Escorial'' diagnostic criteria are used for elucidation of electrophysiological changes for the diagnosis of ALS. El- Escorial criteria are also known as ''Airlie House recommendations''. It was revised in a meeting at Awaji Island, Japan in 2006. Current Airlie house recommendations were revised step by step. Awaji criteria have a significant impact on the diagnosis of ALS.
ALS is a clinical diagnosis. Due to the absence of any disease bio-markers, the presence of both UMN and LMN signs and symptoms in the same body region along with the progression of these symptoms. Modified ''El-Escorial'' diagnostic criteria are used for elucidation of electrophysiological changes for the diagnosis of ALS. El- Escorial criteria are also known as ''Airlie House recommendations''. It was revised in a meeting at Awaji Island, Japan in 2006. Current Airlie house recommendations were revised step by step. Awaji criteria have a significant impact on the early diagnosis of ALS. Currently, Gold coast criteria are used for the diagnosis of ALS.
<ref name="pmid18164242">{{cite journal| author=de Carvalho M, Dengler R, Eisen A, England JD, Kaji R, Kimura J | display-authors=etal| title=Electrodiagnostic criteria for diagnosis of ALS. | journal=Clin Neurophysiol | year= 2008 | volume= 119 | issue= 3 | pages= 497-503 | pmid=18164242 | doi=10.1016/j.clinph.2007.09.143 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18164242  }} </ref>


== Diagnostic Study of Choice ==
== Diagnostic Study of Choice ==


=== Study of choice ===
=== Name of Diagnostic Criteria ===
* Equal emphasis on both [[Electromyogram|electromyogram (EMG)]] and clinical abnormalitie are the gold standard method for the diagnosis of amyotrophic lateral sclerosis.<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><ref name="pmid20462913">{{cite journal| author=Mills KR| title=Detecting fasciculations in amyotrophic lateral sclerosis: duration of observation required. | journal=J Neurol Neurosurg Psychiatry | year= 2011 | volume= 82 | issue= 5 | pages= 549-51 | pmid=20462913 | doi=10.1136/jnnp.2009.186833 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20462913 }} </ref>
* Awaji Criteria for the diagnosis of ALS was proposed to facilitate the diagnosis of ALS in the early stage.
Major points include:
* Electromyography is considered equivalent to the clinical examination of the identification for LMN signs and fasciculations.  
<ref name="pmid23261262">{{cite journal| author=Guennoc AM, Camu W, Corcia P| title=[Awaji criteria: new diagnostic criteria for amyotrophic lateral sclerosis]. | journal=Rev Neurol (Paris) | year= 2013 | volume= 169 | issue= 6-7 | pages= 470-5 | pmid=23261262 | doi=10.1016/j.neurol.2012.10.007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23261262 }} </ref>


* [[EMG]] and [[Nerve conduction study|nerve conduction studies]] are most sensitive to detecting the disease and can quantify its trademark characteristic of [[Lower motor neuron|LMN]] degeneration.<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><ref name="pmid22892641">{{cite journal| author=Costa J, Swash M, de Carvalho M| title=Awaji criteria for the diagnosis of amyotrophic lateral sclerosis:a systematic review. | journal=Arch Neurol | year= 2012 | volume= 69 | issue= 11 | pages= 1410-6 | pmid=22892641 | doi=10.1001/archneurol.2012.254 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22892641  }} </ref><ref name="pmid18164242">{{cite journal| author=de Carvalho M, Dengler R, Eisen A, England JD, Kaji R, Kimura J et al.| title=Electrodiagnostic criteria for diagnosis of ALS. | journal=Clin Neurophysiol | year= 2008 | volume= 119 | issue= 3 | pages= 497-503 | pmid=18164242 | doi=10.1016/j.clinph.2007.09.143 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18164242  }} </ref><ref name="pmid21989247">{{cite journal| author=Hardiman O, van den Berg LH, Kiernan MC| title=Clinical diagnosis and management of amyotrophic lateral sclerosis. | journal=Nat Rev Neurol | year= 2011 | volume= 7 | issue= 11 | pages= 639-49 | pmid=21989247 | doi=10.1038/nrneurol.2011.153 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21989247  }} </ref>
*Both Awaji and El-escorial criteria are complicated to apply for the diagnosis of ALS and also prone to errors.


* [[Magnetic resonance imaging|Magnetic resonance imaging (MRI)]] studies of the [[brain]] and [[spinal cord]] are the most useful neuroimaging technique in ALS mainly to exclude syndromes that mimic ALS.<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><ref name="pmid21989247">{{cite journal| author=Hardiman O, van den Berg LH, Kiernan MC| title=Clinical diagnosis and management of amyotrophic lateral sclerosis. | journal=Nat Rev Neurol | year= 2011 | volume= 7 | issue= 11 | pages= 639-49 | pmid=21989247 | doi=10.1038/nrneurol.2011.153 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21989247 }} </ref>
* For all these reasons '''Gold coast criteria'' were established to modify the existing criteria and to simplify the diagnosis of ALS.
Key points of Gold coast criteria are:
 
1. Progressive motor degeneration documented by history/repeated clinical evaluation, with previously normal motor function, and
 
2. Presence of upper and lower motor neuron dysfunction in at least one body region, (with upper and lower motor neuron dysfunction noted in the same body region) or lower motor neuron dysfunction in at least two body regions, and
 
3. Key Investigations (EMG, imaging, etc) that exclude other disease processes.
 
<ref name="pmid32387049">{{cite journal| author=Shefner JM, Al-Chalabi A, Baker MR, Cui LY, de Carvalho M, Eisen A | display-authors=etal| title=A proposal for new diagnostic criteria for ALS. | journal=Clin Neurophysiol | year= 2020 | volume= 131 | issue= 8 | pages= 1975-1978 | pmid=32387049 | doi=10.1016/j.clinph.2020.04.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=32387049 }} </ref>


==References==
==References==

Latest revision as of 16:46, 15 September 2021

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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

ALS is a clinical diagnosis. Due to the absence of any disease bio-markers, the presence of both UMN and LMN signs and symptoms in the same body region along with the progression of these symptoms. Modified El-Escorial diagnostic criteria are used for elucidation of electrophysiological changes for the diagnosis of ALS. El- Escorial criteria are also known as Airlie House recommendations. It was revised in a meeting at Awaji Island, Japan in 2006. Current Airlie house recommendations were revised step by step. Awaji criteria have a significant impact on the early diagnosis of ALS. Currently, Gold coast criteria are used for the diagnosis of ALS.

Diagnostic Study of Choice

Name of Diagnostic Criteria

  • Awaji Criteria for the diagnosis of ALS was proposed to facilitate the diagnosis of ALS in the early stage.

Major points include:

  • Electromyography is considered equivalent to the clinical examination of the identification for LMN signs and fasciculations.

[1]

  • Both Awaji and El-escorial criteria are complicated to apply for the diagnosis of ALS and also prone to errors.
  • For all these reasons 'Gold coast criteria were established to modify the existing criteria and to simplify the diagnosis of ALS.

Key points of Gold coast criteria are:

1. Progressive motor degeneration documented by history/repeated clinical evaluation, with previously normal motor function, and

2. Presence of upper and lower motor neuron dysfunction in at least one body region, (with upper and lower motor neuron dysfunction noted in the same body region) or lower motor neuron dysfunction in at least two body regions, and

3. Key Investigations (EMG, imaging, etc) that exclude other disease processes.

[2]

References

  1. Guennoc AM, Camu W, Corcia P (2013). "[Awaji criteria: new diagnostic criteria for amyotrophic lateral sclerosis]". Rev Neurol (Paris). 169 (6–7): 470–5. doi:10.1016/j.neurol.2012.10.007. PMID 23261262.
  2. Shefner JM, Al-Chalabi A, Baker MR, Cui LY, de Carvalho M, Eisen A; et al. (2020). "A proposal for new diagnostic criteria for ALS". Clin Neurophysiol. 131 (8): 1975–1978. doi:10.1016/j.clinph.2020.04.005. PMID 32387049 Check |pmid= value (help).

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