Abstract
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.
Publication types
-
Case Reports
-
Research Support, N.I.H., Extramural
MeSH terms
-
Aged
-
Amyloid Neuropathies / pathology*
-
Amyloid Neuropathies / physiopathology
-
Amyloidosis / complications
-
Amyloidosis / pathology*
-
Amyloidosis / physiopathology
-
Antibodies, Monoclonal / therapeutic use
-
Antibodies, Monoclonal, Murine-Derived
-
Arm / innervation
-
Arm / physiopathology
-
Biomarkers / analysis
-
Biomarkers / metabolism
-
Biopsy
-
Diagnosis, Differential
-
Disease Progression
-
Electrodiagnosis
-
Female
-
Humans
-
Immunoglobulin Light Chains / analysis
-
Immunoglobulin Light Chains / metabolism
-
Immunoglobulins, Intravenous / therapeutic use
-
Mononeuropathies / genetics
-
Mononeuropathies / pathology*
-
Mononeuropathies / physiopathology
-
Neural Conduction / physiology
-
Peripheral Nerves / metabolism
-
Peripheral Nerves / pathology*
-
Peripheral Nerves / physiopathology
-
Predictive Value of Tests
-
Rituximab
-
Sensitivity and Specificity
-
Treatment Failure
Substances
-
Antibodies, Monoclonal
-
Antibodies, Monoclonal, Murine-Derived
-
Biomarkers
-
Immunoglobulin Light Chains
-
Immunoglobulins, Intravenous
-
Rituximab