Diaphragm paralysis in primary systemic amyloidosis.
A patient with primary (AL) systemic amyloidosis developed mononeuropathy multiplex complicated by diaphragmatic failure. High dose melphalan and autologous stem cell transplantation did not ameliorate neuropathy or diaphragm dysfunction. Nocturnal non-invasive ventilation lowered arterial carbon dioxide levels and improved daytime dyspnea. This is the first case associating AL amyloid-induced neuropathy with diaphragm dysfunction.
Berk
John L
JL
The Pulmonary Center, Boston University School of Medicine, MA 02118, USA. jberk@lung.bumc.bu.edu
Wiesman
Janice F
JF
Skinner
Martha
M
Sanchorawala
Vaishali
V
D000368:Aged; D017772:Amyloid Neuropathies / Q000503:physiopathology*; D000686:Amyloidosis / Q000503:physiopathology*; D003964:Diaphragm / Q000000981:diagnostic imaging / Q000503:physiopathology; D005260:Female; D006801:Humans; D011859:Radiography; D012133:Respiratory Paralysis / Q000503:physiopathology*