A 69 YEAR OLD MAN WITH WEAKNESS Teaching NeuroImages Neurology Resident and Fellow Section © 2013 American Academy of Neurology.

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A 69 YEAR OLD MAN WITH WEAKNESS Teaching NeuroImages Neurology Resident and Fellow Section © 2013 American Academy of Neurology

Macroglossia on examination 69-year-old-man Dyspnea followed by slowly progressive proximal leg weakness over two years Macroglossia on examination Ghosh PS, et al

FIGURES Figure 1: Face photograph showing macroglossia (A) Front view and (B) side view. Figure 2: Frozen sections of the deltoid muscle (A) The trichrome stain shows a necrotic muscle fiber (B) The Congo red stain shows extensive amyloid deposition in a perimysial blood vessel wall which is intensely red when viewed under rhodamine optics(C). Ghosh PS, et al

Dyspnea as a presenting manifestation of amyloid myopathy Amyloid myopathy is an underrecognized entity Predominantly presents with progressive proximal weakness in primary amyloidosis Dyspnea results from cardiomyopathy or respiratory muscle weakness Macroglossia due to amyloid deposition is a helpful clinical clue Chemotherapy with cyclophosphamide, dexamethasone and bortezomib shown to improve prognosis in primary amyloidosis References 1. Chapin JE, Kornfeld M, Harris A. Amyloid myopathy: characteristic features of a still underdiagnosed disease. Muscle Nerve. 2005; 31:266-272. 2. Venner CP, Lane T, Foard D. et al. Cyclophosphamide, bortezomib, and dexamethasone therapy in AL amyloidosis is associated with high clonal response rates and prolonged progression-free survival. Blood 2012; 119: 4387-4390. Ghosh PS, et al