Taly A B, Gupta S K, Anisya V, Shankar S K, Rao S, Das K B, Nagaraja D, Swamy H S
J Assoc Physicians India. 1995 Apr;43(4):249-52.
Of the 220 patients of acute idiopathic demyelinating polyneuritis (AIDP/GBS) seen over a seven year period, 15 patients (M:F:11:4) had a relapsing course (6.8%). Their ages ranged from 8 yrs to 70 yrs. They had 36 episodes at a variable interval of 3 months to 25 yrs. Relapse rate varied from one to four. Antecedent events were noted during 16 episodes in 9 patients but the triggering factors were varied. Clinical features of individual episodes were similar to the acute monophasic illness, although they differed inseverity from one episode to the other. Autonomic disturbances were rare. Albuminocytological dissociation was observed during 19 of the 24 episodes. Electrophysiological abnormalities were observed during 19 of the 24 episodes. Electrophysiological abnormalities were present in all and were comparable with patients of non-recurrent illness. Sural nerve biopsy in 3 patients showed evidence of demyelination, remyelination, Wallerian degeneration and myelin breakdown but none had features of inflammation. With the exception of one death, functional recovery was complete in the majority of patients, irrespective of the type of therapeutic intervention. Acute onset, frequent facial involvement, brief clinical course, near complete recovery and very long asymptomatic periods may distinguish these patients of acute relapsing demyelinating polyneuropathy (ARDP) from chronic relapsing demyelinating polyneuropathy. Relapses in GBS are however unpredictable and recurrent GBS is indistinguishable clinically, electrophysiologically and morphologically from the more frequently seen non-recurrent form of monophasic GB Syndrome. A biochemical or immunological marker may help in this distinction.
在7年期间诊治的220例急性特发性脱髓鞘性多发性神经炎(AIDP/GBS)患者中,15例(男∶女=11∶4)呈复发病程(6.8%)。他们的年龄在8岁至70岁之间。复发间隔时间从3个月至25年不等,共发生了36次复发。复发率为1至4次。9例患者的16次复发有前驱事件,但触发因素各不相同。各次发作的临床特征与急性单相病程相似,尽管发作之间的严重程度有所不同。自主神经功能障碍罕见。24次发作中有19次观察到蛋白细胞分离现象。24次发作中有19次观察到电生理异常。所有患者均存在电生理异常,且与非复发性疾病患者相当。3例患者的腓肠神经活检显示有脱髓鞘、再髓鞘化、华勒氏变性和髓鞘崩解的证据,但均无炎症特征。除1例死亡外,大多数患者功能完全恢复,无论采取何种治疗干预方式。急性起病、频繁累及面部、病程短暂、近乎完全恢复以及很长的无症状期,可能使这些急性复发性脱髓鞘性多发性神经病(ARDP)患者有别于慢性复发性脱髓鞘性多发性神经病。然而,GBS的复发不可预测,复发性GBS在临床、电生理和形态学上与更常见的单相GB综合征的非复发性形式无法区分。一种生化或免疫标志物可能有助于这种区分。