CTICasesDiaphragmatic Crural Hypertrophy in Stiff-Person Syndrome
Imaging findings
Chest CT demonstrates massive, symmetric hypertrophy of the bilateral diaphragmatic crura. A follow-up CT obtained one year after stem cell transplantation shows partial regression and thinning of the hypertrophied crura.
Key takeaways
Stiff-person syndrome (formerly stiff-man syndrome) is a rare autoimmune neurological disorder characterized by progressive muscle rigidity, often involving the abdominal wall. The loss of abdominal wall relaxation during inspiration forces the diaphragm to work against elevated resistance, leading to profound, reversible compensatory hypertrophy of the diaphragmatic crura.
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