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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 |