Autonomic nervous symptoms as the initial presentation in systemic light-chain amyloidosis with multiple myeloma: a case report / 中华神经科杂志
Chinese Journal of Neurology
; (12): 743-746, 2022.
Article
in Zh
| WPRIM
| ID: wpr-957963
Responsible library:
WPRO
ABSTRACT
Immunoglobulin light chain amyloidosis (AL) is characterized by the extracellular tissue deposition of insoluble fibrils as a result of protein misfolding. These tissue deposits may be responsible for progressive failure in several organs. Among them, neuropathy may be presented as the first manifestation. The patient reported here presented initially with autonomic nervous system impairment, mainly characterized by severe refractory orthostatic hypotension, which became progressively invalidating, forcing the patient to bed. Moreover, since the systemic involvement of the disease, the patient also presented with diarrhea, peripheral polyneuropathy, and kidney dysfunction. Eventually, the massive myocardial depression and infiltration led to a fatal outcome due to ventricular fibrillation. Examination revealed M protein in serum and urine protein electrophoresis. Rectal mucosa and skin biopsy confirmed amyloidosis, and bone marrow biopsy showed cellular infiltration was over 35% with 23% immature plasma cells. The patient was confirmed as AL with multiple myeloma.
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WPRIM
Language:
Zh
Journal:
Chinese Journal of Neurology
Year:
2022
Type:
Article