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Diaphragm paralysis in primary systemic amyloidosis.
John L Berk1, Janice F Wiesman, Martha Skinner
1The Pulmonary Center, Boston University School of Medicine, MA 02118, USA. jberk@lung.bumc.bu.edu
Summary
Primary systemic amyloidosis can cause nerve damage and diaphragm weakness. This case highlights a novel association between AL amyloidosis-induced neuropathy and respiratory muscle dysfunction, suggesting new therapeutic considerations.
Area of Science:
- Neurology
- Pulmonology
- Hematology
Background:
- Primary (AL) systemic amyloidosis is a plasma cell disorder characterized by amyloid deposition.
- Neuropathy is a known complication, but diaphragmatic involvement is rare.
Observation:
- A patient with AL amyloidosis presented with mononeuropathy multiplex and subsequent diaphragmatic failure.
- Standard treatment with high-dose melphalan and autologous stem cell transplantation was ineffective for neuropathy and diaphragm dysfunction.
Findings:
- This report details the first association of AL amyloid-induced neuropathy with significant diaphragm dysfunction.
- Nocturnal non-invasive ventilation demonstrated efficacy in managing respiratory compromise.
Implications:
- This case expands the understanding of AL amyloidosis complications, emphasizing the potential for respiratory muscle involvement.
- It underscores the importance of considering diaphragm function in AL amyloidosis patients with neuropathy and the potential benefit of ventilatory support.