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Primary lateral sclerosis (PLS) usually presents with gradual-onset, progressive, lower-extremity stiffness and pain due to muscle spasticity. Onset is often asymmetrical. [ 2 ] Although the muscles do not appear to atrophy as in ALS (at least initially), the disabling aspect of PLS is muscle spasticity and cramping, and intense pain when those ...
In 1869, the connection between the symptoms and the underlying neurological problems was first described by Jean-Martin Charcot, who initially introduced the term amyotrophic lateral sclerosis in his 1874 paper. [20] Flail arm syndrome, a regional variant of ALS, was first described by Alfred Vulpian in 1886.
[1] [2] They include amyotrophic lateral sclerosis (ALS), [3] [4] progressive bulbar palsy (PBP), pseudobulbar palsy, progressive muscular atrophy (PMA), primary lateral sclerosis (PLS), spinal muscular atrophy (SMA) and monomelic amyotrophy (MMA), as well as some rarer variants resembling ALS. Motor neuron diseases affect both children and ...
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Juvenile primary lateral sclerosis has an autosomal recessive pattern of inheritance, meaning both copies of the gene in each cell are altered. Juvenile primary lateral sclerosis ( JPLS ) also known as primary lateral sclerosis, juvenile ( PLSJ ), is a very rare genetic disorder , with a small number of reported cases, characterized by ...
In contrast to amyotrophic lateral sclerosis or primary lateral sclerosis, PMA is distinguished by the absence of: [citation needed] brisk reflexes; spasticity; Babinski's sign; emotional lability; The importance of correctly recognizing progressive muscular atrophy as opposed to ALS is important for several reasons. The prognosis is a little ...