Peripheral Neuropathy Research Laboratory, Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905, USA.
Muscle Nerve. 2010 May;41(5):710-5. doi: 10.1002/mus.21561.
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.
原发性(AL)淀粉样变的周围神经病通常以下肢为主,呈长度依赖性、对称性,影响小(疼痛和自主)纤维,与大纤维一样或更严重。我们报告了一例患者,其进行性多发性上肢单神经病,经神经活检证实为原发性淀粉样变(lambda 轻链),无系统性或自主神经特征。认识到轻链淀粉样变性可能是多发性单神经病的病因,这增加了这种临床表型的鉴别诊断。