Primary Lateral Sclerosis: Difference between revisions
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'''Original Editor '''- John-Carlo Caballes, David Castro, Alana Griffith, Joyce Tan, Joanne Van | '''Original Editor '''- [http://www.physio-pedia.com/User:John-Carlo_Caballes John-Carlo Caballes], [http://www.physio-pedia.com/User:David_Castro David Castro], [http://www.physio-pedia.com/User:Alana_Griffith Alana Griffith], [http://www.physio-pedia.com/User:Joyce_Tan Joyce Tan], [http://www.physio-pedia.com/User:Joanne_Van Joanne Van] | ||
'''Lead Editors''' | '''Lead Editors''' | ||
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== Definition / Description<br> == | == Definition / Description<br> == | ||
Primary Lateral Sclerosis (PLS) is characterized as being a rare, non-hereditary, idiopathic, slow, and progressive degeneration of the upper motor neurons<ref name="statland et al.">Statland, JM, Barohn, RJ, Dimachkie, MM, Floeter, MK, Mitsumoto, H. Primary lateral sclerosis. Neurol Clin. 2015 Nov;33(4):749-60. Available from: https://www.ncbi.nlm.nih.gov/pubmed/26515619 doi:10.1016/j.ncl.2015.07.007</ref>. PLS lies on a continuum of sporadic motor neuron disorders. This spectrum includes other disorders such as progressive muscular atrophy, which involves only lower motor neurons, as well as amyotrophic lateral sclerosis (ALS), characterized by both upper and lower motor neuron involvement. Many patients diagnosed with PLS continue to have high levels of independence for many years<ref name="gordon et al.">Gordon, PH, Cheng, B, Katz, IB, Pinto, M, Hays, AP, Mitsumoto, H, Rowland, LP. The natural history of primary lateral sclerosis. Neurology. 2006 Mar;66(5):647-53. doi: 10.1212/01.wnl.0000200962.94777.71</ref>.<br> | Primary Lateral Sclerosis (PLS) is characterized as being a rare, non-hereditary, idiopathic, slow, and progressive degeneration of the upper motor neurons<ref name="statland et al.">Statland, JM, Barohn, RJ, Dimachkie, MM, Floeter, MK, Mitsumoto, H. Primary lateral sclerosis. Neurol Clin. 2015 Nov;33(4):749-60. Available from: https://www.ncbi.nlm.nih.gov/pubmed/26515619 doi:10.1016/j.ncl.2015.07.007</ref>. PLS lies on a continuum of sporadic motor neuron disorders. This spectrum includes other disorders such as progressive muscular atrophy, which involves only lower motor neurons, as well as amyotrophic lateral sclerosis (ALS), characterized by both upper and lower motor neuron involvement. Many patients diagnosed with PLS continue to have high levels of independence for many years<ref name="gordon et al.">Gordon, PH, Cheng, B, Katz, IB, Pinto, M, Hays, AP, Mitsumoto, H, Rowland, LP. The natural history of primary lateral sclerosis. Neurology. 2006 Mar;66(5):647-53. doi: 10.1212/01.wnl.0000200962.94777.71</ref>.<br> | ||
== Clinically Relevant Anatomy == | == Clinically Relevant Anatomy == | ||
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add text here relating to clinically relevant anatomy of the condition | add text here relating to clinically relevant anatomy of the condition | ||
== Epidemiology<ref name="singer et al.">Singer, MA, Statland, JM, Wolfe, GI, Barohn, RJ. Primary lateral sclerosis. Muscle Nerve. 2007 Jan;35(3):291-302. doi: 10.1002/mus.20728</ref> == | == Epidemiology<ref name="singer et al.">Singer, MA, Statland, JM, Wolfe, GI, Barohn, RJ. Primary lateral sclerosis. Muscle Nerve. 2007 Jan;35(3):291-302. doi: 10.1002/mus.20728</ref> == | ||
*Approximately 2-5% of adults in neuromuscular clinics will be diagnosed with PLS. | *Approximately 2-5% of adults in neuromuscular clinics will be diagnosed with PLS. | ||
*The age of onset ranges from a mean age of 45.4-53.7 years, though a juvenile form of PLS has been described as well. | *The age of onset ranges from a mean age of 45.4-53.7 years, though a juvenile form of PLS has been described as well. | ||
*Although the difference is not pronounced, there is a slight male predominance over females in being diagnosed with PLS. | *Although the difference is not pronounced, there is a slight male predominance over females in being diagnosed with PLS. | ||
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== Etiology == | == Etiology == | ||
The exact cause of adult-onset PLS remains unknown<ref name="singer et al." />. PLS is a diagnosis of exclusion, meaning that individuals are diagnosed with the condition when their progressive upper motor neuron dysfunctions cannot be explained by any other possible cause. | The exact cause of adult-onset PLS remains unknown<ref name="singer et al." />. PLS is a diagnosis of exclusion, meaning that individuals are diagnosed with the condition when their progressive upper motor neuron dysfunctions cannot be explained by any other possible cause. | ||
== Mechanism of Injury / Pathological Process<br> == | == Mechanism of Injury / Pathological Process<br> == | ||
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== Prognosis == | == Prognosis == | ||
A distinctive clinical feature of PLS is that it has a very slow progression, leading it to be considered to have a more benign prognosis in comparison to ALS<ref name="singer et al." />. The longevity of individuals with PLS is unclear, but it has been estimated to be 7.9 years or longer. Individuals with PLS and minimal EMG changes appear to have a decreased ability to ambulate independently in comparison to those without EMG changes. Nonetheless, patients may still be able to ambulate without aids after several years of being diagnosed. It is common for patients to be diagnosed with PLS but later diagnosed with ALS instead. Due to their similar course of progression, misdiagnosis can occur. A detailed spectrum of PLS categories have been detailed<ref name="singer et al." /><ref name="gordon et al." />. | A distinctive clinical feature of PLS is that it has a very slow progression, leading it to be considered to have a more benign prognosis in comparison to ALS<ref name="singer et al." />. The longevity of individuals with PLS is unclear, but it has been estimated to be 7.9 years or longer. Individuals with PLS and minimal EMG changes appear to have a decreased ability to ambulate independently in comparison to those without EMG changes. Nonetheless, patients may still be able to ambulate without aids after several years of being diagnosed. It is common for patients to be diagnosed with PLS but later diagnosed with ALS instead. Due to their similar course of progression, misdiagnosis can occur. A detailed spectrum of PLS categories have been detailed<ref name="singer et al." /><ref name="gordon et al." />. | ||
== Diagnostic Procedures == | == Diagnostic Procedures == | ||
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=== Physical Therapy === | === Physical Therapy === | ||
=== Medical / Surgical === | |||
=== Medical / Surgical === | |||
== Differential Diagnosis<br> == | == Differential Diagnosis<br> == | ||
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== References<br> == | == References<br> == | ||
<references /> | <references /> | ||
Revision as of 02:46, 5 May 2017
Original Editor - John-Carlo Caballes, David Castro, Alana Griffith, Joyce Tan, Joanne Van
Lead Editors
Definition / Description
[edit | edit source]
Primary Lateral Sclerosis (PLS) is characterized as being a rare, non-hereditary, idiopathic, slow, and progressive degeneration of the upper motor neurons[1]. PLS lies on a continuum of sporadic motor neuron disorders. This spectrum includes other disorders such as progressive muscular atrophy, which involves only lower motor neurons, as well as amyotrophic lateral sclerosis (ALS), characterized by both upper and lower motor neuron involvement. Many patients diagnosed with PLS continue to have high levels of independence for many years[2].
Clinically Relevant Anatomy[edit | edit source]
add text here relating to clinically relevant anatomy of the condition
Epidemiology[3][edit | edit source]
- Approximately 2-5% of adults in neuromuscular clinics will be diagnosed with PLS.
- The age of onset ranges from a mean age of 45.4-53.7 years, though a juvenile form of PLS has been described as well.
- Although the difference is not pronounced, there is a slight male predominance over females in being diagnosed with PLS.
Etiology[edit | edit source]
The exact cause of adult-onset PLS remains unknown[3]. PLS is a diagnosis of exclusion, meaning that individuals are diagnosed with the condition when their progressive upper motor neuron dysfunctions cannot be explained by any other possible cause.
Mechanism of Injury / Pathological Process
[edit | edit source]
add text here relating to the mechanism of injury and/or pathology of the condition
Clinical Presentation[edit | edit source]
add text here relating to the clinical presentation of the condition
Prognosis[edit | edit source]
A distinctive clinical feature of PLS is that it has a very slow progression, leading it to be considered to have a more benign prognosis in comparison to ALS[3]. The longevity of individuals with PLS is unclear, but it has been estimated to be 7.9 years or longer. Individuals with PLS and minimal EMG changes appear to have a decreased ability to ambulate independently in comparison to those without EMG changes. Nonetheless, patients may still be able to ambulate without aids after several years of being diagnosed. It is common for patients to be diagnosed with PLS but later diagnosed with ALS instead. Due to their similar course of progression, misdiagnosis can occur. A detailed spectrum of PLS categories have been detailed[3][2].
Diagnostic Procedures[edit | edit source]
add text here relating to diagnostic tests for the condition
Outcome Measures[edit | edit source]
add links to outcome measures here (see Outcome Measures Database)
Management / Interventions
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Physical Therapy[edit | edit source]
Medical / Surgical[edit | edit source]
Differential Diagnosis
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add text here relating to the differential diagnosis of this condition
Key Evidence[edit | edit source]
add text here relating to key evidence with regards to any of the above headings
Resources
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add appropriate resources here
Case Studies[edit | edit source]
add links to case studies here (case studies should be added on new pages using the case study template)
Recent Related Research (from Pubmed)[edit | edit source]
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References
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- ↑ Statland, JM, Barohn, RJ, Dimachkie, MM, Floeter, MK, Mitsumoto, H. Primary lateral sclerosis. Neurol Clin. 2015 Nov;33(4):749-60. Available from: https://www.ncbi.nlm.nih.gov/pubmed/26515619 doi:10.1016/j.ncl.2015.07.007
- ↑ 2.0 2.1 Gordon, PH, Cheng, B, Katz, IB, Pinto, M, Hays, AP, Mitsumoto, H, Rowland, LP. The natural history of primary lateral sclerosis. Neurology. 2006 Mar;66(5):647-53. doi: 10.1212/01.wnl.0000200962.94777.71
- ↑ 3.0 3.1 3.2 3.3 Singer, MA, Statland, JM, Wolfe, GI, Barohn, RJ. Primary lateral sclerosis. Muscle Nerve. 2007 Jan;35(3):291-302. doi: 10.1002/mus.20728
