摘要
目的探讨治疗相关波动性吉兰－巴雷综合征（GBS－TRF）临床和病理特点。方法回顾性分析我院1999—2014年间住院确诊的吉兰－巴雷综合征（GBS）患者，对其中符合GBS－TRF诊断的患者的临床表现、电生理和腓肠神经病理特点进行总结。结果15年间于我院住院确诊的868例GBS患者中，有8例符合GBS－TRF诊断，平均起病年龄34岁，男女比例为7∶1。其中3例患者发病前有感染史，3例合并乙型病毒性肝炎。5例双下肢无力起病，3例双上肢无力起病，6例伴末梢型感觉障碍，5例在发作中曾有呼吸困难。所有患者均无脑神经损害或自主神经受累。脑脊液生化检查均呈蛋白－分离现象。有5例患者发作2次，1例发作3次，2例发作6次。2次发作之间最长间隔46 d，最短间隔14 d，平均23 d。2例患者行腓肠神经活体组织检查，主要病理改变为局灶性神经纤维束受损，受损神经束内有髓纤维数量中－重度减少，可见髓鞘消解腔、薄髓纤维及再生丛。结论与经典GBS相比较，GBS－TRF患者的发病年龄、前驱感染症状、脑脊液改变及神经电生理检测并无明显差别，但以男性多见。腓肠神经活体组织检查提示有类似缺血性周围神经病的病理改变。
ObjectiveTo investigate the clinical and pathological features of Guillain-Barré syndrome with treatment-related fluctuations （GBS-TRF）.MethodsClinical data were obtained from medical records of patients with GBS-TRF during the period 1999 to 2014 in our Hospital. Sural nerve specimens were collected and summarized retrospectively （two cases）.ResultsEight of 868 cases with GBS had at least one TRF including three chronic hepatitis B patients. The onset of disease was ranged in age from six to 63 years, averaging 34 years. It is more common in men than in women in a ratio of seven to one. Triggering infections occurred in three patients. The initial symptom included weakness of the lower limbs （five cases） and upper extremities （three cases）. Sensory symptom was presented in six patients. Five patients had associated respiratory paralysis. None of them had cranial nerve palsy or autonomic dysfunction. The examination of cerebrospinal fluid showed protein and cell separation. Five patients had two attacks, one had three attacks and two had six attacks. The interval between attacks ranged between 14 days and 46 days （mean 23 days）. The striking pathologic finding was the presence of sectional selective nerve fiber degeneration （SNFD） with evidence of demyelination.ConclusionsPatients with GBS-TRF show similar onset age, preceding infection, cerebrospinal fluid findings, and electrophysiologic characteristics comparing to patients with GBS, while there are more male patients than female patients. SNFD found in sural nerve biopsy reveals ischemic neuropathy, which predicts that injury of arterioles might play an important role in the pathogenesis of GBS-TRF.
出处
《中华神经科杂志》
CAS
CSCD
北大核心
2017年第4期283-287,共5页
Chinese Journal of Neurology
