Primary systemic amyloidosis presenting with demyelinating features.
OBJECTIVE
Primary systemic amyloidosis is a rare disorder that has multisystemic manifestations. The most common neuropathy in systemic amyloidosis is a small-fiber axonal polyneuropathy. When the neuropathy is the presenting feature, diagnosis is usually delayed. The diagnosis of systemic amyloidosis may be more difficult when patients present with an atypical polyneuropathy.


METHODS
Two cases of primary systemic amyloidosis with a multifocal polyneuropathy with demyelinating features are presented.


RESULTS
The patients reported in this series with autopsy proven amyloidosis had evidence of a polyneuropathy with demyelinating features.


CONCLUSIONS
Amyloidosis should be considered in the differential when a patients presents with a polyneuropathy that has demyelinating features.
Gutierrez
A
A
Louisiana State University Health Sciences Center, Department of Neurology and Neuropathology, New Orleans, LA 70112 , USA. agutie@lsuhsc.edu
Turkewitz
L J
LJ
Correa
H
H
England
J D
JD
D000682:Amyloid / Q000276:immunology / Q000378:metabolism; D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology; D001158:Arteries / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D001344:Autopsy; D003711:Demyelinating Diseases / Q000175:diagnosis* / Q000209:etiology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D017809:Fatal Outcome; D006333:Heart Failure / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D050440:Immunoglobulin Subunits / Q000378:metabolism; D008297:Male; D008833:Microcirculation / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology / Q000503:physiopathology; D010525:Peripheral Nerves / Q000098:blood supply / Q000473:pathology / Q000503:physiopathology; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology; D011115:Polyneuropathies / Q000175:diagnosis* / Q000209:etiology* / Q000503:physiopathology; D012678:Sensation Disorders / Q000209:etiology / Q000503:physiopathology; D014781:Viscera / Q000098:blood supply / Q000473:pathology / Q000503:physiopathology
D000682:Amyloid; D050440:Immunoglobulin Subunits