ISOLATED PROGRESSIVE MUSCLE WEAKNESS WITH TUBULAR AGGREGATES

We report 2 isolated cases of slowly progressive muscle weakness in wh ich tubular aggregates in muscle biopsy were found as the major pathol ogical feature, Tubular aggregates were present in both types of fiber s, Electron microscopy revealed the accumulation of double-walled tubu lar structures in dense subsarcolemmal locations or in smaller amounts within the myofibrils, close to cytoplasmic organelles, Myopathy with tubular aggregates is believed to form a distinct clinico-pathologica l entity with 3 well-distinguished clinical groups. The cases reported herein would fall into the group of sporadic isolated progressive mus cle weakness of which only 3 other cases have been previously describe d.