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Definition
By Mayo Clinic staffPrimary lateral sclerosis (PLS) causes weakness in your voluntary muscles, such as those you use to control your legs, arms and tongue. Primary lateral sclerosis is a type of motor neuron disease, which causes muscle nerve cells to slowly die, causing weakness.
Primary lateral sclerosis can happen at any age, but it's more common after age 40. A subtype of primary lateral sclerosis, known as juvenile primary lateral sclerosis, begins in early childhood and is caused by an abnormal gene passed from parents to children.
Primary lateral sclerosis is often mistaken for another, more common motor neuron disease called amyotrophic lateral sclerosis (ALS). However, primary lateral sclerosis progresses more slowly than ALS, and in most cases isn't considered fatal.
- NINDS primary lateral sclerosis information page. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/primary_lateral_sclerosis/primary_lateral_sclerosis.htm. Accessed Aug. 17, 2010.
- Juvenile primary lateral sclerosis. National Institutes of Health Genetics Home Reference. http://ghr.nlm.nih.gov/condition=juvenileprimarylateralsclerosis. Accessed Aug. 16, 2010.
- Murray B, et al. Disorders of upper and lower motor neurons. In: Bradley WG, et al. Neurology in Clinical Practice. 5th ed. Philadelphia, Pa.: Butterworth-Heinemann Elsevier; 2008. http://www.mdconsult.com/book/player/book.do?method=display&type=bookPage&decorator=header&eid=4-u1.0-B978-0-7506-7525-3..50116-3&displayedEid=4-u1.0-B978-0-7506-7525-3..50116-3--cesec19&uniq=215117994&isbn=978-0-7506-7525-3&sid=1040472768. Accessed Aug. 16, 2010.
- Brugman F, et al. Adult-onset primary lateral sclerosis is not associated with mutations in the ALS2 gene. Neurology. 2007;69:702.
- Tartaglia MC, et al. Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis: Examination of symptoms and signs at disease onset and during follow-up. Archives of Neurology. 2007;64:232.
- Gordon PH, et al. Clinical features that distinguish PLS, upper motor neuron-dominant ALS, and typical ALS. Neurology. 2009;72:1948.
- Hadano S, et al. Molecular and cellular function of ALS2/alsin: Implication of membrane dynamics in neuronal development and degeneration. Neurochemistry International. 2007;51:74.
- Motor neuron diseases fact sheet. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/motor_neuron_diseases/detail_motor_neuron_diseases.htm. Accessed Aug. 17, 2010.
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