Primary lateral sclerosis
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| Primary lateral sclerosis Classification and external resources | |
| ICD-10 | G12.2 |
|---|---|
| ICD-9 | 335.24 |
| OMIM | 606353 |
| DiseasesDB | 29150 |
| eMedicine | neuro/324 |
| MeSH | D016472 |
Primary lateral sclerosis (PLS) is a rare neuromuscular disease characterized by progressive muscle weakness in the voluntary muscles. PLS belongs to a group of disorders known as motor neuron diseases. Motor neuron diseases develop when the nerve cells that control voluntary muscle movement degenerate and die, causing weakness in the muscles they control.
Contents |
Symptoms and progression
Onset of PLS usually occurs after age 50. Symptoms may include difficulty with balance, weakness and stiffness in the legs, and clumsiness. Other symptoms may include spasticity (sudden, involuntary muscle spasms) in the hands, feet, or legs; foot dragging, and speech problems due to involvement of the facial muscles. The disorder usually begins in the legs, but it may also start in the tongue or the hands.
The disease -- which scientists believe is not hereditary -- progresses gradually over a number of years, or even decades. In PLS, there is no evidence of the degeneration of spinal motor neurons or muscle wasting (amyotrophy) that occurs in amyotrophic lateral sclerosis, which it resembles. Diagnosis of PLS is often delayed because it is mistaken for ALS.
Treatment
Treatment for individuals with PLS is symptomatic. Baclofen and tizanidine may reduce spasticity. Quinine or phenytoin may decrease cramps. Physical therapy often helps prevent joint immobility. Speech therapy may be useful for those with involvement of the facial muscles.
Prognosis
PLS is not fatal. There is no cure, and the progression of symptoms varies. Some people may retain the ability to walk without assistance, but others eventually require wheelchairs, canes, or other assistive devices.
External links
nl:Primaire Lateraal ScleroseAcknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
