Familial amyloidosis MRI

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

MRI may be helpful in the diagnosis of familial amyloidosis. Findings on liver MRI suggestive of familial amyloidosis include liver enlargement with heterogeneous decreased attenuation, asymmetric and triangular hepatomegaly with the apex at the falciform ligament, and parenchymal calcification. Findings on renal MRI suggestive of familial amyloidosis include hypodense lesions on T2, kidney enlargement with heterogeneous decreased attenuation, and parenchymal calcification. Findings on cardiac MRI suggestive of familial amyloidosis include thickening of ventricular and atrial walls and valvular leaflets due to deposition of amyloid fibrils, enlarged atria caused by diastolic dysfunction and/or valvular dysfunction due to amyloid deposition, heart enlargement with heterogeneous decreased attenuation, cardiac calcification, and pericardial effusion.

MRI

In cardiac amyloidosis the MRI findings may include:

• Thickening of ventricular and atrial walls and valvular leaflets due to deposition of amyloid fibrils
• Enlarged atria caused by diastolic dysfunction and/or valvular dysfunction due to amyloid deposition
• Heart enlargement with heterogeneous decreased attenuation
• Cardiac calcifications
• Pericardial effusion (rare)

In hepatic amyloidosis the MRI scan findings may include:

• Liver enlargement with heterogeneous decreased attenuation
• Asymmetric and triangular hepatomegaly with the apex at the falciform ligament (due to mild atrophic change of the lateral border of both hepatic lobes)
• Parenchyma calcification (rare)

In renal amyloidosis the MRI findings may include:

• Hypodense lesions on T2
• Kidney enlargement with heterogeneous decreased attenuation
• Parenchyma calcification (rare)

In amyloidosis involving the peripheral nerves the MRI findings may include:

• Perineural enhancement

Images

MRI showing cardiac amyloidosis (yellow arrow).

MRI showing hypothalamic amyloidosis (yellow arrows).