Progressive muscle atrophy. A rarely diagnosed variant of amyotrophic lateral sclerosis

摘要: Progressive muscle atrophy (PMA) is a degenerative disease of the lower motor neuron. The course illness and fatal prognosis correspond to those amyotrophic lateral sclerosis (ALS). Neuropathologic genetic findings support categorizing PMA within spectrum ALS, even though no clinical sign disorder upper neuron demonstrable. diagnosis based on advanced extremity pareses atrophies with high progression rate. Respiratory insufficiency determinative prognosis. Absent or late affection bulbar functions characteristic disease. Intraneuronal bunina bodies ubiquitine-positive inclusions, which are established morphologic characteristics found post mortem. treatment options riluzol medication, respiratory therapy, nutrition analogous for typical ALS.