Table 1.
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.