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 34 free, full-text research articles on human participants. First 3 results:

Imaging of glia activation in people with primary lateral sclerosis.
 

Author(s): Sabrina Paganoni, Mohamad J Alshikho, Nicole R Zürcher, Paul Cernasov, Suma Babu, Marco L Loggia, James Chan, Daniel B Chonde, David Izquierdo Garcia, Ciprian Catana, Caterina Mainero, Bruce R Rosen, Merit E Cudkowicz, Jacob M Hooker, Nazem Atassi

Journal:

 

Glia activation is thought to contribute to neuronal damage in several neurodegenerative diseases based on preclinical and human - studies, but its role in primary lateral sclerosis (PLS) is unknown.

Last Updated: 31 Dec 1969

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Magnetic susceptibility in the deep layers of the primary motor cortex in Amyotrophic Lateral Sclerosis.
 

Author(s): M Costagli, G Donatelli, L Biagi, E Caldarazzo Ienco, G Siciliano, M Tosetti, M Cosottini

Journal:

 

Amyotrophic Lateral Sclerosis (ALS) is a progressive neurological disorder that entails degeneration of both upper and lower motor neurons. The primary motor cortex (M1) in patients with upper motor neuron (UMN) impairment is pronouncedly hypointense in Magnetic Resonance (MR) T2* ...

Last Updated: 31 Dec 1969

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Primary Lateral Sclerosis and Early Upper Motor Neuron Disease: Characteristics of a Cross-Sectional Population.
 

Author(s): Christina N Fournier, Alyssa Murphy, Lorena Loci, Hiroshi Mitsumoto, Catherine Lomen-Hoerth, Yasushi Kisanuki, Zachary Simmons, Nicholas J Maragakis, April L McVey, Tawfiq Al-Lahham, Terry D Heiman-Patterson, Jinsy Andrews, Erin McDonnell, Merit Cudkowicz, Nazem Atassi

Journal: J Clin Neuromuscul Dis. 2016 Mar;17(3):99-105.

 

The goals of this study were to characterize clinical and electrophysiologic findings of subjects with upper motor neuron disease and to explore feasibility of clinical trials in this population.

Last Updated: 31 Dec 1969

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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 4 free, full-text review articles on human participants. First 3 results:

Primary Lateral Sclerosis.
 

Author(s): Jeffrey M Statland, Richard J Barohn, Mazen M Dimachkie, Mary Kay Floeter, Hiroshi Mitsumoto

Journal: Neurol Clin. 2015 Nov;33(4):749-60.

 

Primary lateral sclerosis is characterized by insidious onset of progressive upper motor neuron dysfunction in the absence of clinical signs of lower motor neuron involvement. Patients experience stiffness; decreased balance and coordination; mild weakness; and, if the bulbar region ...

Last Updated: 31 Dec 1969

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Brain-Specific Cytoskeletal Damage Markers in Cerebrospinal Fluid: Is There a Common Pattern between Amyotrophic Lateral Sclerosis and Primary Progressive Multiple Sclerosis?
 

Author(s): Ahmed Abdelhak, Andreas Junker, Johannes Brettschneider, Jan Kassubek, Albert C Ludolph, Markus Otto, Hayrettin Tumani

Journal:

 

Many neurodegenerative disorders share a common pathophysiological pathway involving axonal degeneration despite different etiological triggers. Analysis of cytoskeletal markers such as neurofilaments, protein tau and tubulin in cerebrospinal fluid (CSF) may be a useful approach to ...

Last Updated: 31 Dec 1969

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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: 31 Dec 1969

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Use of Dalfampridin in Primary Lateral Sclerosis
 

Status: Recruiting

Condition Summary: Motor Neuron Disease, Upper

 

Last Updated: 7 Jan 2018

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Acoustic and Perceptual Markers of Dysarthria in Amyotrophic Lateral Sclerosis (ALS)
 

Status: Recruiting

Condition Summary: Amyotrophic Lateral Sclerosis; Primary Lateral Sclerosis; Kennedy's Disease

 

Last Updated: 13 Aug 2018

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Clinical Procedures to Support Research
 

Status: Recruiting

Condition Summary: Amyotrophic Lateral Sclerosis; ALS-Frontotemporal Dementia; Primary Lateral Sclerosis; Progressive Muscular Atrophy

 

Last Updated: 4 Apr 2018

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