Hereditary spastic paraplegia

Common Name(s)

Hereditary spastic paraplegia, Hereditary Spastic Paraparesis, Familial Spastic Paraparesis

Hereditary spastic paraplegia is a group of hereditary, degenerative, neurological disorders that primarily affect the upper motor neurons. Upper motor neurons in the brain and spinal cord deliver signals to the lower motor neurons which in turn, carry messages to the muscles. In hereditary spastic paraplegia, upper motor neurons slowly degenerate so the muscles do not receive the correct messages causing progressive spasticity (increased muscle tone/stiffness) and weakness of the legs leading to difficulty walking.  As degeneration continues, symptoms worsen.  
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Hereditary spastic paraplegia" 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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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Hereditary spastic paraplegia" 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 "Hereditary spastic paraplegia" returned 99 free, full-text research articles on human participants. First 3 results:

[The broad phenotypic spectrum of SCA-3: hereditary spastic paraplegia].
 

Author(s): Sergio A Rodríguez-Quiroga, Dolores González-Morón, Tomoko Arakaki, Nélida Garreto, Marcelo A Kauffman

Journal: Medicina (B Aires). 2013 ;73(6):552-4.

 

Machado-Joseph disease (MJD) is the most frequent dominantly inherited spinocerebellar ataxia. A marked phenotypic variability is a characteristic of this disorder that could involve non-cerebellar presentations. Based on several case reports describing pyramidal dysfunction as the ...

Last Updated: 20 Dec 2013

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Electrophysiological characterisation of motor and sensory tracts in patients with hereditary spastic paraplegia (HSP).
 

Author(s): Kathrin N Karle, Rebecca Schüle, Stephan Klebe, Susanne Otto, Christian Frischholz, Inga Liepelt-Scarfone, Ludger Schöls

Journal:

 

Hereditary spastic paraplegias (HSPs) are characterised by lower limb spasticity due to degeneration of the corticospinal tract. We set out for an electrophysiological characterisation of motor and sensory tracts in patients with HSP.

Last Updated: 15 Nov 2013

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REEPing the benefits of an animal model of hereditary spastic paraplegia.
 

Author(s): Ariel Y Deutch, Peter Hedera, Roger J Colbran

Journal: J. Clin. Invest.. 2013 Oct;123(10):4134-6.

 

The hereditary spastic paraplegias (HSPs) are characterized by spasticity of the leg muscles due to axonal degeneration of corticospinal neurons. Beetz et al. report that the core motor phenotype and axonal pathology of HSPs are recapitulated in mice lacking the HSP-associated gene ...

Last Updated: 2 Oct 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 "Hereditary spastic paraplegia" returned 6 free, full-text review articles on human participants. First 3 results:

Hereditary spastic paraplegia: clinico-pathologic features and emerging molecular mechanisms.
 

Author(s): John K Fink

Journal: Acta Neuropathol.. 2013 Sep;126(3):307-28.

 

Hereditary spastic paraplegia (HSP) is a syndrome designation describing inherited disorders in which lower extremity weakness and spasticity are the predominant symptoms. There are more than 50 genetic types of HSP. HSP affects individuals of diverse ethnic groups with prevalence ...

Last Updated: 27 Aug 2013

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Neuropathology: many paths lead to hereditary spastic paraplegia.
 

Author(s): Robert M Gould, Scott T Brady

Journal: Curr. Biol.. 2004 Oct;14(20):R903-4.

 

Studies with animal models are providing new insights into the pathology of hereditary spastic paraplegia, particularly how mutations in multiple, converging pathways can lead to this family of neuropathies.

Last Updated: 22 Oct 2004

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Is the transportation highway the right road for hereditary spastic paraplegia?
 

Author(s): Andrew H Crosby, Christos Proukakis

Journal: Am. J. Hum. Genet.. 2002 Nov;71(5):1009-16.

 

The term "hereditary spastic paraplegia" (HSP) refers to a genetically and clinically diverse group of disorders whose primary feature is progressive spasticity of the lower extremities. The condition arises because of degeneration of the longest motor and sensory axons on the spinal ...

Last Updated: 25 Oct 2002

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Genetic and Physical Study of Childhood Nerve and Muscle Disorders
 

Status: Recruiting

Condition Summary: Muscular Dystrophies; Muscle Myopathies; Hereditary Spastic Paraplegias; Inherited Neuropathies; Inherited Neuromuscular Conditions

 

Last Updated: 7 Jun 2014

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