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. 2019 May 28;20(11):2616. doi: 10.3390/ijms20112616

Table 1.

Clinical phenotypes, symptoms, and prognosis of amyotrophic lateral sclerosis (ALS).

Clinical Phenotypes Regions Symptoms The Prognosis
Limb-onset UMN Spasticity, weakness, and brisk deep tendon reflexes; Overall survival is 5–8 years
LMN fasciculations, wasting, and patients present with gradually ascending distal weakness.
Bulbar-onset UMN Spastic dysarthria, which is characterized by slow, labored, and distorted speech, often with a nasal quality; 2 to 4 years, and depends on the timing of respiratory and limb involvement.
LMN tongue wasting, weakness, and fasciculations, accompanied by flaccid dysarthria*, and later dysphagia.
Primary lateral sclerosis Pure UMN It is characterized by an ascending spastic tetraparesis with involvement of speech in the majority by 3 years, urinary urgency; A slowly progressive condition, with survival for decades.
Progressive muscular atrophy Pure LMN The least well-defined subtype of ALS. Asymmetrical weakness and wasting, often in the legs, which coalesces into four limb lower motor neuron involvement. About 5 years
ALS-frontal lobe dementia syndrome
(frontotemporal lobar degeneration, FTLD)
UMN, LMN, and brain cortex Presentation with frontotemporal dementia. Later developing signs of MND. An overall survival of less than 3 years.

UMN-upper motor neurons, LMN - Lower motor neuron.