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. 2022 Mar 7;93(6):668–678. doi: 10.1136/jnnp-2021-327909

Figure 3.

Figure 3

Diagnostic algorithm for ATTR polyneuropathy. Tissue biopsy may include a target organ such as nerve, heart, bone marrow or skin, or an ‘off-target’ biopsy such as salivary gland or abdominal fat pad aspirate. $Depending on availability; IHC and LD-MS are important for amyloid fibril typing. Bone scintigraphy may be semiquantitatively graded relative to rib uptake with the following grades: grade 0, no uptake and normal bone scan; grade 1, uptake less than rib uptake; grade 2, uptake equal to rib uptake; and grade 3, uptake greater than rib uptake with mild or absent rib uptake abnormalities. Echo and CMR are important investigations for detecting cardiac amyloid disease. ATTR, transthyretin amyloidosis; ATTR-PN, transthyretin amyloidosis–polyneuropathy; CMR, cardiac MRI; CTS, carpal tunnel syndrome; Echo, echocardiogram; IHC, immunohistochemistry; LD-MS, laser dissection–mass spectrometry; MGUS, monoclonal gammopathy of uncertain significance; TTR, transthyretin.