Das A, Kalita J, Misra U K
Department of Neurology, Sanjay Gandhi PGIMS, Lucknow.
Electromyogr Clin Neurophysiol. 2004 Mar;44(2):95-102.
Recurrent Guillain Barre' syndrome (RGBS) is a rare condition and there is a paucity of clinical and neurophysiological studies.
This study was undertaken to evaluate the clinical and neurophysiological changes in patients with recurrent GB syndrome and their outcome.
We report 11 patients with RGBS out of 200 patients with GB syndrome seen over last 10 years. These patients had 2 or more attacks of acute inflammatory demyelinating neuropathy with an onset to peak time of 4 weeks or less having complete or near complete recovery. All the patients underwent complete neurological evaluation. The disability was graded on a 0-10 clinical grading scale. Antinuclear antibody, rheumatoid factor C3, C4, urine test for porphyria, serum test for HIV and CSF examination were carried out in all the patients. Motor nerve conduction of median, ulnar, peroneal, F response and concentric needle electromyography were carried out in all. Sensory conduction of ulnar, median and sural, central motor conduction to abductor digiti minimi and tibialis anterior were also carried out.
Patients' age ranged between 5 and 40 years and 6 were children. Eight patients had 2 attacks, 2 had 3 attacks and 1 had 4 attacks. The interval between attacks ranged between 4 months to 10 year (mean 39.5 month). The triggering events were noted in 7 patients and were identical to the previous event in 4 patients. Six patients had pure motor weakness, 4 had associated sensory symptoms and 1 had both sensory symptoms and signs. The severity of different attacks was not related to number of recurrences. Nerve conduction studies were consistent with demyelinating neuropathy. Central motor conduction was abnormal in 5 out of 7 episodes studied Eight patients recovered completely after initial attack and 3 had persistent foot drop at 6 month follow up. Following the second attack 4 patients recoveredfully and the remaining 7 had foot drop and in 2 there was mild proximal weakness as well. After the third attack 1 patient recoveredfully and the other 2 had residual deficit in the form of handgrip weakness and foot drop. There seems to be a tendency to accumulate neurological deficits with increasing frequency of attacks of GB syndrome.
复发性吉兰-巴雷综合征（RGBS）是一种罕见疾病，临床和神经生理学研究较少。
本研究旨在评估复发性吉兰-巴雷综合征患者的临床和神经生理学变化及其预后。
我们报告了过去10年中200例吉兰-巴雷综合征患者中的11例RGBS患者。这些患者有2次或更多次急性炎症性脱髓鞘性神经病发作，起病至高峰时间为4周或更短，且完全或几乎完全恢复。所有患者均接受了全面的神经学评估。残疾程度根据0至10级临床分级量表进行分级。对所有患者进行了抗核抗体、类风湿因子、C3、C4、卟啉尿试验、HIV血清学检测和脑脊液检查。对所有患者进行了正中神经、尺神经、腓总神经运动神经传导、F波检查和同心针肌电图检查。还进行了尺神经、正中神经和腓肠神经感觉传导、小指展肌和胫前肌的中枢运动传导检查。
患者年龄在5岁至40岁之间，其中6例为儿童。8例患者发作2次，2例发作3次，1例发作4次。发作间隔在4个月至10年之间（平均39.5个月）。7例患者记录到了触发事件，其中4例与之前的事件相同。6例患者仅有运动无力，4例伴有感觉症状，1例既有感觉症状又有体征。不同发作的严重程度与复发次数无关。神经传导研究结果与脱髓鞘性神经病一致。在研究的7次发作中，有5次中枢运动传导异常。8例患者在首次发作后完全恢复，3例在6个月随访时仍有持续性足下垂。第二次发作后，4例患者完全恢复，其余7例有足下垂，其中2例还有轻度近端肌无力。第三次发作后，1例患者完全恢复，另外2例有残留缺陷，表现为握力减弱和足下垂。随着吉兰-巴雷综合征发作频率增加，似乎有神经功能缺损累积的趋势。
