Little K H, Lee E L, Frenkel E P
South Med J. 1986 Jun;79(6):677-81. doi: 10.1097/00007611-198606000-00007.
Cranial nerve dysfunction is exceedingly rare in amyloidosis. Cranial nerve deficits have been described in the very rare form of familial amyloidosis in which there appears to be an autosomal dominant pattern of inheritance. By contrast, they are virtually unreported in the two common forms of amyloidosis, that is, secondary amyloidosis (AA), usually associated with a chronic inflammatory lesion, and primary amyloidosis (AL), resulting from light-chain immunoglobulins of plasma cell dyscrasias. Although neurologic involvement in these forms of amyloidosis has been reported in the peripheral nerves, spinal nerve roots, and autonomic ganglia, the absence of reports of cranial nerve lesions is remarkable. We report the case of a woman with multiple bilateral cranial nerve deficits involving the left trigeminal, left facial, right abducens, and hypoglossal nerves. She had nonfamilial amyloidosis associated with a plasma cell dyscrasia of the IgG kappa type with increased production of light-chain immunoglobulins. The rarity of cranial nerve involvement in this clinical setting is highlighted by three previous studies, each of which reported involvement of only a single nerve (either the facial or olfactory nerve). Since aging has been correlated with congophilic changes in the brain, the explanation for the sparing of cranial nerves can be only speculative.
颅神经功能障碍在淀粉样变性中极为罕见。颅神经缺损在极为罕见的家族性淀粉样变性形式中有所描述，其似乎呈现常染色体显性遗传模式。相比之下，在两种常见的淀粉样变性中几乎未见相关报道，即继发性淀粉样变性（AA），通常与慢性炎症性病变相关，以及原发性淀粉样变性（AL），由浆细胞异常增生的轻链免疫球蛋白所致。尽管已有报道称这些类型的淀粉样变性会累及外周神经、脊神经根和自主神经节，但未见颅神经病变的报道仍很显著。我们报告一例患有多种双侧颅神经缺损的女性病例，累及左侧三叉神经、左侧面神经、右侧展神经和舌下神经。她患有非家族性淀粉样变性，与IgG κ型浆细胞异常增生相关，伴有轻链免疫球蛋白产生增加。此前三项研究凸显了在这种临床情况下颅神经受累的罕见性，每项研究均仅报告了单一神经（面神经或嗅神经）受累。由于衰老与大脑中嗜刚果红变化相关，颅神经未受累的原因只能是推测性的。
