- 期刊
免疫型神經病變的診斷與治療
Diagnosis and Management of Immune-mediated Neuropathies
摘要
免疫型神經病變(immune-mediated neuropathy)或稱為發炎型神經病變(inflammatory neuropathy)是指因免疫系統異常，導致神經軸突、髓鞘或細胞本體受傷所造成的神經病變：病理學上通常伴隨有發炎細胞如淋巴球、巨噬細胞筆的浸潤。病理生理學上，分成免疫傷害(immune injury)與結構傷害(structural injury)兩期，造成髓鞘及神經軸突的損傷。狹義的免疫型神經病變依病程分為急性神經根炎(Guillain-Barré syndrome，GBS)與慢性脫髓鞘性神經炎(chronic inflammatory demyelinating polyneuropathy，CIDP)。臨床上，GBS及CIDP主要都是以進行性的運動及感覺障礙為主要表現，神經的受犯範圍則有甚大差異。於神經傳導檢查，GBS以遠端潛時延長及F-wave潛時延長為主：CIDP的遠端潛時延長、F-wave潛時延長及神經傳導速度下降。病理機制的研究，發現某些對抗醣脂質抗體(anti-ganglioside antibodies)與免疫型神經病變的亞型有關。於GBS，積極的免疫治療可以縮短病患病程、減少併發症與日後的殘存運動障礙：靜脈注射免疫球蛋白(intravenous immunoglobulin，IVIG)與血漿交換有相同的療效，高劑量類固醇對GBS的治療無益。對於CIDP的治療，血漿交換及IVIG兩者皆可減輕神經學缺失，因為CIDP的免疫障礙是持續性的，必需使用類固醇保持療效。免疫型神經病變的診治是神經科醫師的挑戰，然而病人有極佳回復正常生活的契機：不論GBS或CIDP都是症候群，而非單一疾病，實驗室檢查僅能作為臨床評估的參考，不能以此主導臨床診治，詳細的臨床觀察及判斷是診斷免疫型神經病變的唯一依據。
關鍵字
急性神經根炎 ； 慢性脫髓鞘性神經炎 ； 靜脈注射免疫球蛋白 ； 血漿交換 ； 類固醇 ； 神經軸突退化 ； 髓鞘脫失 ； 神經傳導檢查 ； 抗醣脂質抗體 ； 多病灶型運動神經病變 ； Guillain-Barré syndrome ； Miller Fisher syndrome並列摘要
Immune-mediate neuropathies, or inflammatory neuropathies are neuropathies due to the dysregulation of the immune system. The injury to peripheral nerves can be divided into two phases: an early stage of immune injury, and a later stage of structural damage. The overall effects are axonal degeneration or demyelination depending on the target of immunological attacks. According to time course, there are two major types: Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP). Clinical manifestations of both diseases include progressive motor weakness and sensory disturbance with some variations among different patients. The major findings of nerve conduction studies on GBS patients are prolonged distal motor latencies and minimal F-wave latencies with variable reduction of nerve conduction velocities. In CIDP patients, slowed nerve conduction velocities are the usual findings in addition to prolongation of distal motor latencies and minimal F-wave latencies. Certain subtypes of immune-mediated neuropathies are associated with high titers of anti-gangliosdie antibodies. Patients with GBS and CIDP can benefit from immunotherapy. For GBS, plasma exchange and intravenous immunoglobulin (IVIG) are equally effective in reducing complications and neurological disability. Steroid of high dose is, however, harmful to GBS. Plasma exchange and IVIG can alleviate neurological deficits of CIDP with steroid to maintain the effects of plasma exchange and IVIG. In conclusion, careful clinical observations and judgment are the most important issue to manage patients with immune-mediated neuropathies.