AL amyloidosis

A plasma cell disorder characterized by the aggregation and deposition of insoluble amyloid fibrils derived from misfolding of monoclonal immunoglobulin light chains usually produced by a plasma cell tumor. It usually presents as primary systemic amyloidosis (PSA) with multiple organ involvement and less frequently as primary localized amyloidosis (PLA) restricted to a single organ.

Proximal muscle weakness

A lack of strength of the proximal muscles.


Total: 2

                       


(per page)
PMID (PMCID)
15052628
 FEMALE Middle Aged
Amyloid myopathy presenting with distal atrophic weakness.
Smestad C, Monstad P, Lindboe CF, Mygland A.
Muscle Nerve. 2004;29(4):605-9.
Amyloid myopathy is a rare complication of primary amyloidosis usually presenting with proximal muscle weakness.
11508448
 FEMALE Middle Aged
A deceptive case of amyloid myopathy: clinical and magnetic resonance imaging features.
Hull KM, Griffith L, Kuncl RW, Wigley FM.
Arthritis Rheum. 2001;44(8):1954-8.
This case not only illustrates that proximal muscle weakness due to primary amyloid myopathy (as found in light chain amyloidosis and transthyretin amyloidosis) can mimic that of polymyositis, but also shows that unique findings on MRI can alert the clinician to the diagnosis of amyloidosis prior to muscle biopsy.