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{{Amyotrophic lateral sclerosis}}


{{Amyotrophic lateral sclerosis}}
{{CMG}}; {{AE}}{{MMJ}}


==Overview==
==Overview==
The presence of both Upper motor neuron and lower motor neuron signs on Physical examination in the same region with the exclusion of any other Neurological condition is highly suggestive of Amyotrophic lateral sclerosis.
==Physical Examination==
==Physical Examination==
===Pulmonary===
 
* Nocturnal [[hypoventilation]]
*ALS leads to progressive degeneration of the [[motor neurons]] that supply voluntary [[Muscle|muscles]], including [[Lower motor neuron|Lower motor neurons]] in the [[medulla]] and anterior horn of the spinal cord as well as [[Lower motor neuron]]<nowiki/>s in the [[cerebral cortex]].
*[[Pneumonia]] may compromise pulmonary function even further
*Physical examination of patients with amyotrophic lateral sclerosis is usually remarkable for:<ref name="pmid24124634">{{cite journal| author=Gordon PH| title=Amyotrophic Lateral Sclerosis: An update for 2013 Clinical Features, Pathophysiology, Management and Therapeutic Trials. | journal=Aging Dis | year= 2013 | volume= 4 | issue= 5 | pages= 295-310 | pmid=24124634 | doi=10.14336/AD.2013.0400295 | pmc=3794725 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24124634  }} </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><ref name="pmid6696604">{{cite journal| author=Goldfarb BJ, Simon SR| title=Gait patterns in patients with amyotrophic lateral sclerosis. | journal=Arch Phys Med Rehabil | year= 1984 | volume= 65 | issue= 2 | pages= 61-5 | pmid=6696604 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6696604  }} </ref>
*[[Pulmonary failure]]
#[[Lower motor neuron]] signs:
*Pulmonary function test:
#*[[Fasciculation]]
**Decreased forced vital capacity
#*[[Cramps]]
**Inspiratory pressure diminishes
#*[[Muscle atrophy]]
===Gastrointestinal===
#*Marked [[weakness]]
*[[Constipation]]
#[[Upper motor neuron]] signs:
===Neurologic===
#*[[Spasticity]]
*Limb onset:
#*[[Hyperreflexia]]
**Awkwardness when walking or running
#*[[Weakness]]
**Tripping or stumbling
#*Positive [[Babinski sign]]
**Difficulty with simple tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock** Occasionally the symptoms remain confined to one limb; this is known as [[monomelic amyotrophy]].
#*Positive [[Hoffmann sign]]
*Bulbar onset:
#*Emotional lability
**These patients first notice difficulty speaking clearly
#[[Foot drop]]
**Garbled and slurred speech
#Difficulty walking
** Nasality and loss of volume are frequently the first symptoms
#Abnormal gait patterns
**Difficulty swallowing and loss of tongue mobility follow
 
** Eventually total loss of speech and the inability to [[Pulmonary aspiration|protect the airway]] when swallowing are experienced
* ALS begins in the limbs, usually the arms, in about two-thirds of patients. The first symptoms are most often unilateral and focal. As limb function deteriorates, patients become dependent on caregivers.<ref name="pmid24124634">{{cite journal| author=Gordon PH| title=Amyotrophic Lateral Sclerosis: An update for 2013 Clinical Features, Pathophysiology, Management and Therapeutic Trials. | journal=Aging Dis | year= 2013 | volume= 4 | issue= 5 | pages= 295-310 | pmid=24124634 | doi=10.14336/AD.2013.0400295 | pmc=3794725 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24124634  }} </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><ref name="pmid6696604">{{cite journal| author=Goldfarb BJ, Simon SR| title=Gait patterns in patients with amyotrophic lateral sclerosis. | journal=Arch Phys Med Rehabil | year= 1984 | volume= 65 | issue= 2 | pages= 61-5 | pmid=6696604 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6696604  }} </ref>
*[[Muscle weakness]] and [[atrophy]] spread to other parts of the body as the disease progresses
*Sphincter and sensory functions are usually, but not always, spared. Eye movements are preserved until advanced stages.<ref name="pmid24124634">{{cite journal| author=Gordon PH| title=Amyotrophic Lateral Sclerosis: An update for 2013 Clinical Features, Pathophysiology, Management and Therapeutic Trials. | journal=Aging Dis | year= 2013 | volume= 4 | issue= 5 | pages= 295-310 | pmid=24124634 | doi=10.14336/AD.2013.0400295 | pmc=3794725 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24124634  }} </ref>
*Increasing difficulty moving
 
*Swallowing ([[dysphagia]])
===HEENT===
*Speaking or forming words ([[dysarthria]])
*Dropped head
*Upper motor neuron involvement:
* Atrophied fasciculating tongu
**[[Spasticity]]
* [[Nystagmus]] in advanced diseases
**[[Hyperreflexia]] or exaggerated reflexes including overactive gag reflex and positive [[Babinski sign]]
* Extra-ocular movements may be abnormal
*Lower Motor Neuron involvement:
* Hearing acuity is normal
** muscle weakness and atrophy,
 
**muscle cramps, and
 
** fleeting twitches of muscles that can be seen under the skin (fasciculations)
 
*45% of patients experience [[pseudobulbar affect]], also known as "emotional lability" attributable to degeneration of bulbar upper motor neurons resulting in exaggeration of motor expressions of emotion
===Lungs===
**uncontrollable laughter
 
** crying or smiling
* Asymmetric chest expansion or decreased chest expansion may be seen
*[[Frontotemporal dementia]]
 
===Heart===
 
*Displaced point of maximal impulse (PMI) suggestive of cardiac muscle atrophy<ref name="pmid25133001">{{cite journal| author=Namazi MH, Khaheshi I, Haybar H, Esmaeeli S| title=Cardiac failure as an unusual presentation in a patient with history of amyotrophic lateral sclerosis. | journal=Case Rep Neurol Med | year= 2014 | volume= 2014 | issue=  | pages= 986139 | pmid=25133001 | doi=10.1155/2014/986139 | pmc=4123615 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25133001  }} </ref>
*Heart murmur<ref name="pmid29021775">{{cite journal| author=Rosenbohm A, Schmid B, Buckert D, Rottbauer W, Kassubek J, Ludolph AC et al.| title=Cardiac Findings in Amyotrophic Lateral Sclerosis: A Magnetic Resonance Imaging Study. | journal=Front Neurol | year= 2017 | volume= 8 | issue=  | pages= 479 | pmid=29021775 | doi=10.3389/fneur.2017.00479 | pmc=5623666 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29021775  }} </ref>
===Abdomen===
* Abdominal examination of patients with ALS is usually normal.
 
===Back===
* Back examination of patients with ALS is usually normal.
 
===Genitourinary===
*Urinary retention is common in patients with ALS. Urological evaluation is indicated in ALS patients with prominent spasticity.<ref name="pmid21506896">{{cite journal| author=Lopes de Carvalho ML, Motta R, Battaglia MA, Brichetto G| title=Urinary disorders in amyotrophic lateral sclerosis subjects. | journal=Amyotroph Lateral Scler | year= 2011 | volume= 12 | issue= 5 | pages= 352-5 | pmid=21506896 | doi=10.3109/17482968.2011.574141 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21506896  }} </ref>
 
===Neuromuscular===
 
* Hyperreflexia or hyporeflexia
* Positive Babinski bilaterally
* Muscle rigidity
* Proximal and distal muscle weakness bilaterally
*Bilateral upper and lower extremity weakness
*Abnormal gait
 
===Extremities===
 
Physical examination of extremities in patients with amyotrophic lateral sclerosis is usually remarkable for:<ref name="pmid24124634">{{cite journal| author=Gordon PH| title=Amyotrophic Lateral Sclerosis: An update for 2013 Clinical Features, Pathophysiology, Management and Therapeutic Trials. | journal=Aging Dis | year= 2013 | volume= 4 | issue= 5 | pages= 295-310 | pmid=24124634 | doi=10.14336/AD.2013.0400295 | pmc=3794725 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24124634  }} </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><ref name="pmid6696604">{{cite journal| author=Goldfarb BJ, Simon SR| title=Gait patterns in patients with amyotrophic lateral sclerosis. | journal=Arch Phys Med Rehabil | year= 1984 | volume= 65 | issue= 2 | pages= 61-5 | pmid=6696604 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6696604  }} </ref>
#[[Lower motor neuron]] signs:
#*[[Fasciculation]]
#*[[Cramps]]
#*[[Muscle atrophy]]
#*Marked [[weakness]]
#[[Upper motor neuron]] signs:
#*[[Spasticity]]
#*[[Hyperreflexia]]
#*[[Weakness]]
#*Positive [[Babinski sign]]
#*Positive [[Hoffmann sign]]
#*Emotional lability
#[[Foot drop]]  
#Difficulty walking
#Abnormal gait patterns


==References==
==References==
{{reflist|2}}
{{Reflist|2}}


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Latest revision as of 19:12, 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

The presence of both Upper motor neuron and lower motor neuron signs on Physical examination in the same region with the exclusion of any other Neurological condition is highly suggestive of Amyotrophic lateral sclerosis.

Physical Examination

  1. Lower motor neuron signs:
  2. Upper motor neuron signs:
  3. Foot drop
  4. Difficulty walking
  5. Abnormal gait patterns
  • ALS begins in the limbs, usually the arms, in about two-thirds of patients. The first symptoms are most often unilateral and focal. As limb function deteriorates, patients become dependent on caregivers.[1][2][3]
  • Sphincter and sensory functions are usually, but not always, spared. Eye movements are preserved until advanced stages.[1]

HEENT

  • Dropped head
  • Atrophied fasciculating tongu
  • Nystagmus in advanced diseases
  • Extra-ocular movements may be abnormal
  • Hearing acuity is normal


Lungs

  • Asymmetric chest expansion or decreased chest expansion may be seen

Heart

  • Displaced point of maximal impulse (PMI) suggestive of cardiac muscle atrophy[4]
  • Heart murmur[5]

Abdomen

  • Abdominal examination of patients with ALS is usually normal.

Back

  • Back examination of patients with ALS is usually normal.

Genitourinary

  • Urinary retention is common in patients with ALS. Urological evaluation is indicated in ALS patients with prominent spasticity.[6]

Neuromuscular

  • Hyperreflexia or hyporeflexia
  • Positive Babinski bilaterally
  • Muscle rigidity
  • Proximal and distal muscle weakness bilaterally
  • Bilateral upper and lower extremity weakness
  • Abnormal gait

Extremities

Physical examination of extremities in patients with amyotrophic lateral sclerosis is usually remarkable for:[1][2][3]

  1. Lower motor neuron signs:
  2. Upper motor neuron signs:
  3. Foot drop
  4. Difficulty walking
  5. Abnormal gait patterns

References

  1. ↑ 1.0 1.1 1.2 1.3 Gordon PH (2013). "Amyotrophic Lateral Sclerosis: An update for 2013 Clinical Features, Pathophysiology, Management and Therapeutic Trials". Aging Dis. 4 (5): 295–310. doi:10.14336/AD.2013.0400295. PMC 3794725. PMID 24124634.
  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 3.2 Goldfarb BJ, Simon SR (1984). "Gait patterns in patients with amyotrophic lateral sclerosis". Arch Phys Med Rehabil. 65 (2): 61–5. PMID 6696604.
  4. ↑ Namazi MH, Khaheshi I, Haybar H, Esmaeeli S (2014). "Cardiac failure as an unusual presentation in a patient with history of amyotrophic lateral sclerosis". Case Rep Neurol Med. 2014: 986139. doi:10.1155/2014/986139. PMC 4123615. PMID 25133001.
  5. ↑ Rosenbohm A, Schmid B, Buckert D, Rottbauer W, Kassubek J, Ludolph AC; et al. (2017). "Cardiac Findings in Amyotrophic Lateral Sclerosis: A Magnetic Resonance Imaging Study". Front Neurol. 8: 479. doi:10.3389/fneur.2017.00479. PMC 5623666. PMID 29021775.
  6. ↑ Lopes de Carvalho ML, Motta R, Battaglia MA, Brichetto G (2011). "Urinary disorders in amyotrophic lateral sclerosis subjects". Amyotroph Lateral Scler. 12 (5): 352–5. doi:10.3109/17482968.2011.574141. PMID 21506896.

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