progressive paralytic neurodegenerative diseases

Types

 amyotrophic lateral sclerosis (ALS) (MIM.105400)
 primary lateral sclerosis (PLS)

Although primary lateral sclerosis (PLS) is similar to amyotrophic lateral sclerosis (ALS) (MIM.105400), they are considered to be clinically distinct progressive paralytic neurodegenerative disorders.

Following a period of diagnostic confusion, the clinical distinction between ALS and PLS became clear and diagnostic criteria were established.

PLS is characterized by degeneration of the upper motor neurons and the corticospinal and corticobulbar tracts, whereas ALS is a more severe disorder characterized by degeneration of both the upper and lower motor neurons.

See also

 autosomal recessive juvenile-onset PLS (MIM.606353), caused by mutations in the ALS2 gene (MIM.606352).