Progressive bulbar palsy is a type of motor neuron disease. Patients will show progressive dysphagia or weakness of the chewing muscles. This disease can also cause aspiration pneumonia, suffocation, etc., which are life-threatening. 1. Cause It is a group of chronic progressive degenerative diseases with unknown etiology that selectively invade the anterior horn cells of the spinal cord, the posterior motor neurons of the brainstem, the cortical pyramidal cells and the pyramidal tract. 2. Clinical manifestations The clinical features are the coexistence of symptoms and signs of upper and lower motor neuron damage, manifested as different combinations of muscle weakness, muscle atrophy and pyramidal tract signs. Sensation and sphincter function are generally not affected. The specific manifestations are atrophy of the tongue muscles, coughing when eating, choking when drinking water through the nostrils, unclear speech, hoarseness, and often accompanied by scoliosis and weakness of the head; the pharyngeal reflex disappears, the soft palate cannot move, and the tongue muscle bundles tremble. The disease often develops after the age of 40 and affects more men than women. The average survival period after the onset of the disease is 2 to 3 years. Symptoms include coughing when drinking water, difficulty swallowing, weakness in chewing, coughing and breathing, and dysarthria; examination shows a drooping upper palate, absent pharyngeal reflex, accumulation of saliva in the pharynx, and obvious atrophy of the tongue muscles accompanied by fasciculations. Involvement of the corticobulbar tract causes hyperreflexia of the jaw, which is accompanied by strong crying and laughing in the later stage, and the coexistence of true and pseudoparalysis. The disease progresses rapidly and has a poor prognosis, with most patients dying from respiratory muscle paralysis and lung infection within 1 to 3 years. 3. Treatment Because the cause of the disease is not yet clear, there is no effective measure to prevent the progression of the disease. 1. Treatment of the cause If the patient is suspected of having a history of chronic heavy metal poisoning, further investigation should be carried out for early prevention and treatment. 2. Symptomatic treatment For those with dysphagia, a nasogastric tube should be placed to feed them a semi-liquid diet. For those with a lot of secretions, tracheotomy may be performed if necessary. For advanced patients, care should be strengthened and aspiration pneumonia should be prevented. |
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