Department of Pathology, University of Oklahoma Health Sciences Center

NeuroTest Question #94 Next question Previous question

Answer: (A) Desmin related myopathy. Level of difficulty: 3 NeuroTest NeuroLearn NeuroHelp

Discussion:

• Desmin related myopathy: These cytoplasmic bodies characterized by radiating intermediate filaments (spheroid bodies). The other ultrastructural finding are granulofilamentous materials, also known as dappled dense structures”. These strutures are formed by dense anatomosing and trabeculated granular material that is about 100 nm in diameter is accumulated in between myofibrils or under the sarcolemma. The Z-disc streaming may appear contiguous to the granular deposits.  [Click here to see a related case]
• Tubular aggregate myopathy: Tubular aggregates appear as masses of bundles of long, straight, parallel tubules in between myofibrils especially beneath subsarcolemmal locations. They are often double barrel and contains an inner tube but they may be up to eight inner tubes. The outer tube is usually about 50 nm in diameter but may be as large as 80 nm. The inner tubules are 20-30 nm in diameter. [Click here to see a related case]
• Mitochondrial myopathy: Abnormal mitochondria containing crystalloid structures and abnormal architecture are common findings. Other features include abnormally high number of mitochondria. [Click here to see abnormal mitochondria]

Comment: KarMing-Fung@ouhsc.edu