Primary lateral sclerosis

Common Name(s)

Primary lateral sclerosis

Primary lateral sclerosis is a type of motor neuron disease, where nerve cells that control voluntary muscle movement breakdown and die. In primary lateral sclerosis only the upper motor neurons in the brain are affected. Symptoms often begin with problems in the legs (e.g., weakness, stiffness, spasticity, and balance problems), but may also start with hand clumsiness and changes in speech. The symptoms worsen gradually over time, however people with this condition have a normal life expectancy. Progression of symptoms varies from person to person, some people retain the ability to walk without assistance, others eventually require assistive devices such as canes or wheelchairs. Diagnosis requires extensive testing to exclude other diseases. Treatment may include baclofen and tizanidine to reduce spasticity, quinine or phenytoin to reduce cramps, as well as physical and speech therapy as required.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Primary lateral sclerosis" for support, advocacy or research.

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Spastic Paraplegia Foundation, Inc.

We are committed to discovering the cures for HSP and PLS by promoting research, providing accurate information about these disorders, and creating opportunities for mutual support and sharing.

Last Updated: 29 Oct 2012

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General Support Organizations

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How do you compare to others with this condition?

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Primary lateral sclerosis" for support, advocacy or research.

Logo
Spastic Paraplegia Foundation, Inc.

We are committed to discovering the cures for HSP and PLS by promoting research, providing accurate information about these disorders, and creating opportunities for mutual support and sharing.

http://www.sp-foundation.org

Last Updated: 29 Oct 2012

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Primary lateral sclerosis" returned 24 free, full-text research articles on human participants. First 3 results:

Extramotor damage is associated with cognition in primary lateral sclerosis patients.
 

Author(s): Elisa Canu, Federica Agosta, Sebastiano Galantucci, Adriano Chiò, Nilo Riva, Vincenzo Silani, Andrea Falini, Giancarlo Comi, Massimo Filippi

Journal:

 

This is a cross-sectional study aimed at investigating cognitive performances in patients with primary lateral sclerosis (PLS) and using diffusion tensor (DT) magnetic resonance imaging (MRI) to determine the topographical distribution of microstructural white matter (WM) damage in ...

Last Updated: 18 Dec 2013

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Therapeutic potential of mesenchymal stromal cells and MSC conditioned medium in Amyotrophic Lateral Sclerosis (ALS)--in vitro evidence from primary motor neuron cultures, NSC-34 cells, astrocytes and microglia.
 

Author(s): Hui Sun, Karelle Bénardais, Nancy Stanslowsky, Nadine Thau-Habermann, Niko Hensel, Dongya Huang, Peter Claus, Reinhard Dengler, Martin Stangel, Susanne Petri

Journal:

 

Administration of mesenchymal stromal cells (MSC) improves functional outcome in the SOD1G93A mouse model of the degenerative motor neuron disorder amyotrophic lateral sclerosis (ALS) as well as in models of other neurological disorders. We have now investigated the effect of the ...

Last Updated: 26 Sep 2013

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Myelin imaging in amyotrophic and primary lateral sclerosis.
 

Author(s): Shannon Kolind, Rakesh Sharma, Steven Knight, Heidi Johansen-Berg, Kevin Talbot, Martin R Turner

Journal: Amyotroph Lateral Scler Frontotemporal Degener. 2013 Dec;14(7-8):562-73.

 

Primary lateral sclerosis (PLS) has been regarded as a rare, extreme form of amyotrophic lateral sclerosis (ALS). Like ALS, it is a clinical diagnosis without established biomarkers. We sought to explore loss of cerebral myelin in relation to clinical features, including cognitive ...

Last Updated: 4 Nov 2013

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Primary lateral sclerosis" returned 3 free, full-text review articles on human participants. First 3 results:

Is magnetic resonance imaging a plausible biomarker for upper motor neuron degeneration in amyotrophic lateral sclerosis/primary lateral sclerosis or merely a useful paraclinical tool to exclude mimic syndromes? A critical review of imaging applicability in clinical routine.
 

Author(s): Antonio José da Rocha, Antonio Carlos Martins Maia Júnior

Journal: Arq Neuropsiquiatr. 2012 Jul;70(7):532-9.

 

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that affects motor neurons in the cerebral cortex, brainstem, and spinal cord, brain regions in which conventional magnetic resonance imaging is often uninformative. Although the mean time from symptom onset ...

Last Updated: 27 Jul 2012

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Sporadic amyotrophic lateral sclerosis: new hypothesis regarding its etiology and pathogenesis suggests that astrocytes might be the primary target hosting a still unknown external agent.
 

Author(s): Roberto E P Sica, Alejandro F De Nicola, María C González Deniselle, Gabriel Rodriguez, Gisella M Gargiulo Monachelli, Liliana Martinez Peralta, Mariela Bettini

Journal: Arq Neuropsiquiatr. 2011 Aug;69(4):699-706.

 

This article briefly describes the already known clinical features and pathogenic mechanisms underlying sporadic amyotrophic lateral sclerosis, namely excitoxicity, oxidative stress, protein damage, inflammation, genetic abnormalities and neuronal death. Thereafter, it puts forward ...

Last Updated: 30 Aug 2011

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Does primary lateral sclerosis exist? A study of 20 patients and a review of the literature.
 

Author(s): N Le Forestier, T Maisonobe, A Piquard, S Rivaud, L Crevier-Buchman, F Salachas, P F Pradat, L Lacomblez, V Meininger

Journal: Brain. 2001 Oct;124(Pt 10):1989-99.

 

The question of whether primary lateral sclerosis (PLS) is a nosological entity distinct from amyotrophic lateral sclerosis (ALS) has been the subject of controversy since it was first described in the nineteenth century. PLS has been defined as a rare, non-hereditary disease characterized ...

Last Updated: 25 Sep 2001

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Screening and Natural History: Primary Lateral Sclerosis and Related Disorders
 

Status: Recruiting

Condition Summary: Motor Nueron Disease; Nervous System Diseases; Muscle Spasticity; Diffusion Tensor Imaging; Disease Progression; Evoked Potentials, Motor/Physiology

 

Last Updated: 12 Sep 2014

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Genetics of ALS: Identification of Genes With Roles in Familial and Sporadic Amyotrophic Lateral Sclerosis (ALS) and Amyotrophic Lateral Sclerosis (ALS) With Frontotemporal Dementia
 

Status: Recruiting

Condition Summary: Amyotrophic Lateral Sclerosis; Familial Amyotrophic Lateral Sclerosis; Amyotrophic Lateral Sclerosis With Frontotemporal Dementia; Lou Gehrig's Disease; Motor Neuron Disease; Primary Lateral Sclerosis

 

Last Updated: 1 Apr 2014

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Last Updated: 25 Jun 2014

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