Abstract
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.
Abbreviations | ||
ADM | = | abductor digiti minimi |
APB | = | abductor pollicus brevis |
EMG | = | electromyography |
FEV1 | = | forced expiratory volume in 1 second |
FVC | = | forced vital capacity |
MAG | = | myelin-associated glycoprotein |
MGUS | = | monoclonal gammopathy of unknown significance |
NCS | = | nerve conduction studies |
PA | = | pulmonary artery |
Pdimax | = | maximal transdiaphragmatic pressure |
Pimax | = | maximal inspiratory pressure |
Pemax | = | maximal expiratory pressure |
Pg | = | gastric pressure |
Ppl | = | pleural pressure |
RA | = | right atrial |
RV | = | right ventricle |
Abbreviations | ||
ADM | = | abductor digiti minimi |
APB | = | abductor pollicus brevis |
EMG | = | electromyography |
FEV1 | = | forced expiratory volume in 1 second |
FVC | = | forced vital capacity |
MAG | = | myelin-associated glycoprotein |
MGUS | = | monoclonal gammopathy of unknown significance |
NCS | = | nerve conduction studies |
PA | = | pulmonary artery |
Pdimax | = | maximal transdiaphragmatic pressure |
Pimax | = | maximal inspiratory pressure |
Pemax | = | maximal expiratory pressure |
Pg | = | gastric pressure |
Ppl | = | pleural pressure |
RA | = | right atrial |
RV | = | right ventricle |