What Is Motor Neuron Disease?
9. Progressive Bulbar Palsy
Progressive bulbar palsy (PBP) is also known as Brown-Vialetto-Van Laere syndrome. This condition has a variable clinical course where less than 40% of reported cases have a survival rate of 10 or more years after the onset of symptoms. However, abrupt deterioration has also been reported in 1/6 of patients.
This disease attacks nerves supplying the bulbar muscles and leads to degeneration of the motor neurons in the pyramidal tracts, brain stem, spinal cord, and cerebral cortex. Patients with PBP may experience difficulty with swallowing and talking. There can also be fasciculations, reduced gag reflex, weak facial muscles, weak tongue, and weak palatal movements. This results in difficulty with speech, drooling saliva, and difficulty eating.
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