Primary amyloidosis presenting as upper limb multiple mononeuropathies.
Peripheral neuropathy in primary (AL) amyloidosis is usually lower-limb predominant, length-dependent, symmetrical, and affects small (pain and autonomic) fibers, as much or more than large fibers. We report a patient with stepwise progressive, multiple upper limb mononeuropathies that were due to nerve biopsy-proven primary amyloidosis (lambda light chain), with no systemic or autonomic features. Recognition that light chain amyloidosis may be the cause of a multiple mononeuropathy pattern adds to the differential diagnosis of this clinical phenotype.
Tracy
Jennifer A
JA
Peripheral Neuropathy Research Laboratory, Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905, USA.
Dyck
Peter J
PJ
Dyck
P James B
PJ
D000368:Aged; D017772:Amyloid Neuropathies / Q000473:pathology* / Q000503:physiopathology; D000686:Amyloidosis / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001132:Arm / Q000294:innervation / Q000503:physiopathology; D015415:Biomarkers / Q000032:analysis / Q000378:metabolism; D001706:Biopsy; D003937:Diagnosis, Differential; D018450:Disease Progression; D004568:Electrodiagnosis; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis / Q000378:metabolism; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D020422:Mononeuropathies / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D009431:Neural Conduction / Q000502:physiology; D010525:Peripheral Nerves / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D011237:Predictive Value of Tests; D000069283:Rituximab; D012680:Sensitivity and Specificity; D017211:Treatment Failure
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D015415:Biomarkers; D007147:Immunoglobulin Light Chains; D016756:Immunoglobulins, Intravenous; D000069283:Rituximab
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