Primary Lateral Sclerosis: Difference between revisions

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== Definition  ==
<h2> Definition  </h2>
 
<p>Primary Lateral Sclerosis (PLS) is characterized as being a rare, non-hereditary, idiopathic, slow, and progressive degeneration of the upper motor neurons<span class="fck_mw_ref" _fck_mw_customtag="true" _fck_mw_tagname="ref" name="Statland et al.">Statland, J. M., Barohn, R. J., Dimachkie, M. M., Floeter, M. K., &amp;amp;amp; Mitsumoto, H. (2015). Primary lateral sclerosis. Neurologic Clinics, 33(4), 749-760. doi:10.1016/j.ncl.2015.07.007</span>. 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<sup><span class="fck_mw_ref" _fck_mw_customtag="true" _fck_mw_tagname="ref" name="Gordon et al.">Gordon, P. H., Cheng, B., Katz, I. B., Pinto, M., Hays, A. P., Mitsumoto, H., &amp;amp;amp; Rowland, L. P. (2006). The natural history of primary lateral sclerosis. Neurology, 66(5), 647-653. doi: 10.1212/01.wnl.0000200962.94777.71</span></sup>.  
Primary Lateral Sclerosis (PLS) is characterized as being a rare, non-hereditary, idiopathic, slow, and progressive degeneration of the upper motor neurons<ref>Statland, J. M., Barohn, R. J., Dimachkie, M. M., Floeter, M. K., &amp; Mitsumoto, H. (2015). Primary lateral sclerosis. Neurologic Clinics, 33(4), 749-760. 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<sup><ref>Gordon, P. H., Cheng, B., Katz, I. B., Pinto, M., Hays, A. P., Mitsumoto, H., &amp; Rowland, L. P. (2006). The natural history of primary lateral sclerosis. Neurology, 66(5), 647-653. doi: 10.1212/01.wnl.0000200962.94777.71</ref></sup>.
</p>
 
<h2> Epidemiology  </h2>
== Epidemiology  ==
<h2> Etiology  </h2>
 
<h2> Clinical Presentation  </h2>
== Etiology  ==
<h2> Prognosis  </h2>
 
<h2> Interventions  </h2>
== Clinical Presentation  ==
<h4> Physical Therapy&nbsp;  </h4>
 
<h4> Medical and Surgical  </h4>
== Prognosis  ==
<h2> Differential Diagnoses  </h2>
 
<p>(Statland et al, 2015); (Kuipers-Upmeijer et al, 2001); (Strong &amp; Gordon, 2005)<br /> <br />As PLS is an upper motor neuron issue, there are several potential differential diagnosis. The 2 most likely upper motor neuron diseases that is associated with PLS are …  
== Interventions  ==
</p>
 
<ul><li>Amyotrophic lateral sclerosis (Statland et al, 2015); (Strong &amp; Gordon, 2005)
==== Physical Therapy&nbsp;  ====
</li></ul>
 
<p>&nbsp; &nbsp; &nbsp; &nbsp; - ALS presents with lower and/or upper motor deficits and has fast(er) progression; PLS is slower progression and typically only affects &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp;upper motor neurons.<br />&nbsp; &nbsp; &nbsp; &nbsp; - ALS is more commonly associated with stiffness then PLS (47% vs 4%)<br />&nbsp; &nbsp; &nbsp; &nbsp; - Both ALS and PLS can be assoicated with cognitive issues but is not present in its ‘uncomplicated’ forms (Strong &amp; Gordon, 2005).<br />  
==== Medical and Surgical  ====
</p>
 
<ul><li>Hereditary spastic paraplegias (Strong &amp; Gordon, 2005)
== Differential Diagnoses  ==
</li></ul>
 
<p>&nbsp; &nbsp; &nbsp; &nbsp; &nbsp;- HSP has spasticity of the lower limbs; PLS is associated with spasticity as well and slight weakness but spasticity can affect &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp;speech (spastic dysarthria)<br />&nbsp; &nbsp; &nbsp; &nbsp; &nbsp;- Complicated cases of HSP can result in dementia &amp; mental retardation which may mimic cognitive issues possibly present in &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp;complicated cases of PLS.  
(Statland et al, 2015); (Kuipers-Upmeijer et al, 2001); (Strong &amp; Gordon, 2005)<br> <br>As PLS is an upper motor neuron issue, there are several potential differential diagnosis. The 2 most likely upper motor neuron diseases that is associated with PLS are …  
</p><p>Other potential, but less specific, differential considerations include (Statland et al, 2015)…  
 
</p>
*Amyotrophic lateral sclerosis (Statland et al, 2015); (Strong &amp; Gordon, 2005)
<ul><li>Structural lesions, especially related to the spine (eg. cervical spondylmyelopathy)  
 
</li><li>Infection (eg. HIV, syphilis)  
&nbsp; &nbsp; &nbsp; &nbsp; - ALS presents with lower and/or upper motor deficits and has fast(er) progression; PLS is slower progression and typically only affects &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp;upper motor neurons.<br>&nbsp; &nbsp; &nbsp; &nbsp; - ALS is more commonly associated with stiffness then PLS (47% vs 4%)<br>&nbsp; &nbsp; &nbsp; &nbsp; - Both ALS and PLS can be assoicated with cognitive issues but is not present in its ‘uncomplicated’ forms (Strong &amp; Gordon, 2005).<br>  
</li><li>Demyelinating disease (eg. mutliple sclerosis)  
 
</li><li>Metabolic / toxic (eg. vitamin E deficiency)  
*Hereditary spastic paraplegias (Strong &amp; Gordon, 2005)
</li><li>Neurodegenerative (eg. Parkinsons and parkinson's-plus syndromes)
 
</li></ul>
&nbsp; &nbsp; &nbsp; &nbsp; &nbsp;- HSP has spasticity of the lower limbs; PLS is associated with spasticity as well and slight weakness but spasticity can affect &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp;speech (spastic dysarthria)<br>&nbsp; &nbsp; &nbsp; &nbsp; &nbsp;- Complicated cases of HSP can result in dementia &amp; mental retardation which may mimic cognitive issues possibly present in &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp;complicated cases of PLS.  
<p><br />Special care must be taken when diagnosing as PLS can be misdiagnosed as the above examples, and many more (Kuipers-Upmeijer et al, 2001).<br />  
 
</p>
Other potential, but less specific, differential considerations include (Statland et al, 2015)…  
<h2> References  </h2>
 
<p>Gordon, P. H., Cheng, B., Katz, I. B., Pinto, M., Hays, A. P., Mitsumoto, H., &amp; Rowland, L. P. (2006). The natural history of primary lateral sclerosis. Neurology, 66(5), 647-653. doi: 10.1212/01.wnl.0000200962.94777.71  
*Structural lesions, especially related to the spine (eg. cervical spondylmyelopathy)  
</p><p><br />
*Infection (eg. HIV, syphilis)  
</p><p>Kuipers-Upmeijer, J., de Jager, A., Hew, J., Snoek, J., &amp; van Weerden, T. (2001). Primary lateral sclerosis: Clinical, neurophysiological, and magnetic resonance findings. Journal of Neurology Neurosurgery and Psychiatry, 71(5), 615-620. doi:10.1136/jnnp.71.5.615  
*Demyelinating disease (eg. mutliple sclerosis)  
</p><p><br />
*Metabolic / toxic (eg. vitamin E deficiency)  
</p><p>Singer, M. A., Statland, J. M., Wolfe, G. I., &amp; Barohn, R. J. (2007). Primary Lateral Sclerosis. Muscle &amp; Nerve, 35(3), 291-302. doi: 10.1002/mus.20728  
*Neurodegenerative (eg. Parkinsons and parkinson's-plus syndromes)
</p><p><br />Statland, J. M., Barohn, R. J., Dimachkie, M. M., Floeter, M. K., &amp; Mitsumoto, H. (2015). Primary lateral sclerosis. Neurologic Clinics, 33(4), 749-760. doi:10.1016/j.ncl.2015.07.007  
 
</p><p><br />
<br>Special care must be taken when diagnosing as PLS can be misdiagnosed as the above examples, and many more (Kuipers-Upmeijer et al, 2001).<br>
</p><p>Strong, M. J., &amp; Gordon, P. H. (2005). Primary lateral sclerosis, hereditary spastic paraplegia and amyotrophic lateral sclerosis: Discrete entities or spectrum? Amyotrophic Lateral Sclerosis, 6(1), 8-16. doi:10.1080/14660820410021267<br />
 
</p>
== References  ==
 
Gordon, P. H., Cheng, B., Katz, I. B., Pinto, M., Hays, A. P., Mitsumoto, H., &amp; Rowland, L. P. (2006). The natural history of primary lateral sclerosis. Neurology, 66(5), 647-653. doi: 10.1212/01.wnl.0000200962.94777.71
 
 
 
Kuipers-Upmeijer, J., de Jager, A., Hew, J., Snoek, J., &amp; van Weerden, T. (2001). Primary lateral sclerosis: Clinical, neurophysiological, and magnetic resonance findings. Journal of Neurology Neurosurgery and Psychiatry, 71(5), 615-620. doi:10.1136/jnnp.71.5.615
 
 
 
Singer, M. A., Statland, J. M., Wolfe, G. I., &amp; Barohn, R. J. (2007). Primary Lateral Sclerosis. Muscle &amp; Nerve, 35(3), 291-302. doi: 10.1002/mus.20728
 
<br>Statland, J. M., Barohn, R. J., Dimachkie, M. M., Floeter, M. K., &amp; Mitsumoto, H. (2015). Primary lateral sclerosis. Neurologic Clinics, 33(4), 749-760. doi:10.1016/j.ncl.2015.07.007
 
 
 
Strong, M. J., &amp; Gordon, P. H. (2005). Primary lateral sclerosis, hereditary spastic paraplegia and amyotrophic lateral sclerosis: Discrete entities or spectrum? Amyotrophic Lateral Sclerosis, 6(1), 8-16. doi:10.1080/14660820410021267<br>

Revision as of 19:03, 2 May 2017

Definition

Primary Lateral Sclerosis (PLS) is characterized as being a rare, non-hereditary, idiopathic, slow, and progressive degeneration of the upper motor neuronsStatland, J. M., Barohn, R. J., Dimachkie, M. M., Floeter, M. K., &amp;amp; Mitsumoto, H. (2015). Primary lateral sclerosis. Neurologic Clinics, 33(4), 749-760. doi:10.1016/j.ncl.2015.07.007. 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 yearsGordon, P. H., Cheng, B., Katz, I. B., Pinto, M., Hays, A. P., Mitsumoto, H., &amp;amp; Rowland, L. P. (2006). The natural history of primary lateral sclerosis. Neurology, 66(5), 647-653. doi: 10.1212/01.wnl.0000200962.94777.71.

Epidemiology

Etiology

Clinical Presentation

Prognosis

Interventions

Physical Therapy 

Medical and Surgical

Differential Diagnoses

(Statland et al, 2015); (Kuipers-Upmeijer et al, 2001); (Strong & Gordon, 2005)

As PLS is an upper motor neuron issue, there are several potential differential diagnosis. The 2 most likely upper motor neuron diseases that is associated with PLS are …

  • Amyotrophic lateral sclerosis (Statland et al, 2015); (Strong & Gordon, 2005)

        - ALS presents with lower and/or upper motor deficits and has fast(er) progression; PLS is slower progression and typically only affects                upper motor neurons.
        - ALS is more commonly associated with stiffness then PLS (47% vs 4%)
        - Both ALS and PLS can be assoicated with cognitive issues but is not present in its ‘uncomplicated’ forms (Strong & Gordon, 2005).

  • Hereditary spastic paraplegias (Strong & Gordon, 2005)

         - HSP has spasticity of the lower limbs; PLS is associated with spasticity as well and slight weakness but spasticity can affect                              speech (spastic dysarthria)
         - Complicated cases of HSP can result in dementia & mental retardation which may mimic cognitive issues possibly present in                              complicated cases of PLS.

Other potential, but less specific, differential considerations include (Statland et al, 2015)…

  • Structural lesions, especially related to the spine (eg. cervical spondylmyelopathy)
  • Infection (eg. HIV, syphilis)
  • Demyelinating disease (eg. mutliple sclerosis)
  • Metabolic / toxic (eg. vitamin E deficiency)
  • Neurodegenerative (eg. Parkinsons and parkinson's-plus syndromes)


Special care must be taken when diagnosing as PLS can be misdiagnosed as the above examples, and many more (Kuipers-Upmeijer et al, 2001).

References

Gordon, P. H., Cheng, B., Katz, I. B., Pinto, M., Hays, A. P., Mitsumoto, H., & Rowland, L. P. (2006). The natural history of primary lateral sclerosis. Neurology, 66(5), 647-653. doi: 10.1212/01.wnl.0000200962.94777.71


Kuipers-Upmeijer, J., de Jager, A., Hew, J., Snoek, J., & van Weerden, T. (2001). Primary lateral sclerosis: Clinical, neurophysiological, and magnetic resonance findings. Journal of Neurology Neurosurgery and Psychiatry, 71(5), 615-620. doi:10.1136/jnnp.71.5.615


Singer, M. A., Statland, J. M., Wolfe, G. I., & Barohn, R. J. (2007). Primary Lateral Sclerosis. Muscle & Nerve, 35(3), 291-302. doi: 10.1002/mus.20728


Statland, J. M., Barohn, R. J., Dimachkie, M. M., Floeter, M. K., & Mitsumoto, H. (2015). Primary lateral sclerosis. Neurologic Clinics, 33(4), 749-760. doi:10.1016/j.ncl.2015.07.007


Strong, M. J., & Gordon, P. H. (2005). Primary lateral sclerosis, hereditary spastic paraplegia and amyotrophic lateral sclerosis: Discrete entities or spectrum? Amyotrophic Lateral Sclerosis, 6(1), 8-16. doi:10.1080/14660820410021267

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