Lateral Sclerosis (PLS)

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NINDS: ⁵² Primary lateral sclerosis (PLS) is a rare neuromuscular disease with slowly progressive weakness in voluntary muscle movement. PLS belongs to a group of disorders known as motor neuron diseases. PLS affects the upper motor neurons (also called corticospinal neurons) in the arms, legs, and face.  It occurs when nerve cells in the motor regions of the cerebral cortex (the thin layer of cells covering the brain which is responsible for most higher level mental functions) gradually degenerate, causing movements to be slow and effortful.  The disorder often affects the legs first, followed by the body, trunk, arms and hands, and, finally the bulbar muscles (muscles that control speech, swallowing, and chewing).  Symptoms include weakness, muscle stiffness and spasticity, clumsiness, slowing of movement, and problems with balance and speech. PLS is more common in men than in women, with a varied gradual onset that generally occurs between ages 40 and 60. PLS progresses gradually over a number of years, or even decades. Scientists do not believe PLS has a simple hereditary cause.  The diagnosis of PLS requires extensive testing to exclude other diseases. When symptoms begin, PLS may be mistaken for amyotrophic lateral sclerosis (ALS) or spastic paraplegia.  Most neurologists follow an affected individual's clinical course for at least 3 to 4 years before making a diagnosis of PLS.

MalaCards based summary: Lateral Sclerosis, also known as primary lateral sclerosis, is related to frontotemporal dementia and/or amyotrophic lateral sclerosis 2 and frontotemporal dementia and/or amyotrophic lateral sclerosis 3, and has symptoms including ataxia, myoclonus and hemiplegia. An important gene associated with Lateral Sclerosis is SPG7 (SPG7 Matrix AAA Peptidase Subunit, Paraplegin), and among its related pathways/superpathways is Amyotrophic lateral sclerosis (ALS). The drugs Mexiletine and Dronabinol have been mentioned in the context of this disorder. Affiliated tissues include spinal cord, bone marrow and brain, and related phenotypes are babinski sign and abnormal upper motor neuron morphology

GARD: ¹⁹ Primary lateral sclerosis (PLS) is a rare neuromuscular disease that affects the nerve cells that control the voluntary muscles. Problems in the legs (such as weakness, stiffness, spasticity, and balance problems) are often observed first, but hand clumsiness and changes in speech can be early symptoms, as well. The underlying cause of adult PLS is currently unknown. In most cases, it occurs sporadically in people with no family history of the condition. A subtype of PLS, called juvenile PLS, is caused by changes in the ALS2 gene and is inherited in an autosomal recessive manner.

Orphanet: ⁵⁸ Primary lateral sclerosis (PLS) is an idiopathic non-familial motor neuron disease characterized by slowly progressive upper motor neuron dysfunction leading to spasticity, mild weakness in voluntary muscle movement, hyperreflexia, and loss of motor speech production.

Disease Ontology: ¹¹ A motor neuron disease characterized by painless but progressive weakness and stiffness of the muscles of the legs.

Wikipedia: ⁷⁵ Primary lateral sclerosis (PLS) is a very rare neuromuscular disease characterized by progressive muscle... more...

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