Clinical, Autonomic & Electrophysiological Features in Patients with Guillain Barre Syndrome in a Tertiary Care Hospital of Bangladesh

Maftahul Jannat, M. Hannan, S. M. Alam, Md Rafiqul Islam, A. Chowdhury, Md. Habibur Rahman, Saptadipa Das, AM Shafayet Hossain Patwary, Md. Fakrul Islam, Abu Shams Md Hasan Ali Masum
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Abstract

Background: Guillain-Barre Syndrome (GBS) is the most common cause of acute flaccid paralysis in the adult population. It is an acute post infectious immune mediated peripheral neuropathy with a marked variation in pathology, clinical presentation and prognosis. Objective: The aim of the study were to evaluate clinical profile, to assess autonomic involvement & electrophysiological findings in adult patients with GBS. Methods: An observational, cross sectional study was carried out in the Department of Neurology, BSMMU, Dhaka from March, 2015 to September, 2017. Total 43 patients of GBS fulfilling the inclusion criteria were recruited as the study population. Detailed clinical examination, CSF study & nerve conduction study were done. Disability status was measured by Hughes functional grading scale. For autonomic assessment 35 adult healthy control were also included for comparison. Then following tests of autonomic nervous system were performed in both patient and control group 1) resting heart rate and heart rate on changing posture (30: 15 ratio) 2) supine blood pressure and blood pressure on changing posture 3) heart rate response to valsalva maneuver 4) heart rate response to deep breathing and E: I ratio 5) sphincter disturbance by symptoms questionnaire. Results: The mean age of patients was 35±12 years (range18 to 65 years) with slight male predominance (58.1%). Major clinical presentation was weakness of all 4 limbs followed by sensory complaints (44.2%). 7% of the patient had breathing difficulty and dysphagia. Only 4.7 % had diplopia. Among the symptoms of autonomic dysfunction most common symptoms was constipation (30.2 %) followed by palpitation (14%), urinary retention (7%), lightheadedness and urinary incontinence (4.7%). Cranial nerve palsy was present in 34.9% of cases among them facial palsy was found commonly (27.9%), followed by bulbar palsy (7%) and ophthalmoplegia (4.7%). One patient (2.3%) had both facial palsy and ophthalmoplegia. AIDP, AMAN and AMSAN subtypes comprised 32.6%, 37.2% and 20.9% of cases respectively. Regarding autonomic dysfunction variation of heart rate by different maneuver like posture change, deep breathing and valsalva maneuver was found commonly. 30:15 ratio was abnormal in majority of the patients (82.4%) followed by abnormal max-min HR/min on deep breathing (58.1%) and abnormal valsalva ratio (37.2%). Other abnormalities were postural hypotension (38.2%), sinus tachycardia (25.6%), hypertension (16.3%), hypotension (4.7%), and sinus arrhythmia (4.7%). Bowel bladder dysfunction was another autonomic dysfunction among them constipation 30%, urinary retention 7% and urinary incontinence 4.7% of cases. Conclusion: GBS can be presented with variable presentation including autonomic dysfunction. In this study common clinical presentation was limb weakness & different patterns of autonomic dysfunction was found in patients with GBS. Common electrophysiological subtype was AMAN. So in addition to clinical & electrophysiological analysis autonomic evaluation is essential in every patients with GBS as autonomic dysfunction is one of important cause of mortality. Bangladesh Journal of Neuroscience 2019; Vol. 35 (2): 57-62
孟加拉国一家三级医院格林-巴利综合征患者的临床、自主神经和电生理特征
背景:格林-巴利综合征(GBS)是成人急性弛缓性麻痹最常见的病因。它是一种急性感染后免疫介导的周围神经病变,在病理、临床表现和预后上都有明显的差异。目的:本研究旨在评价成人GBS患者的临床特征、自主神经受累情况和电生理表现。方法:2015年3月至2017年9月在达卡BSMMU神经内科进行观察性横断面研究。共招募43例符合纳入标准的GBS患者作为研究人群。进行了详细的临床检查、脑脊液检查和神经传导检查。残障状态采用Hughes功能分级量表。自主神经功能评估还包括35名成人健康对照进行比较。然后分别对患者和对照组进行自主神经系统测试:(1)静息心率和改变体位时心率(30:15);(2)仰卧位血压和改变体位时血压(3)valsalva手法时心率反应(4)深呼吸和E: I比时心率反应(5)症状问卷法括约肌功能障碍。结果:患者平均年龄35±12岁(18 ~ 65岁),男性占58.1%。主要临床表现为四肢无力,其次为感觉不适(44.2%)。7%的患者出现呼吸困难和吞咽困难。只有4.7%的人有复视。在自主神经功能障碍的症状中,最常见的症状是便秘(30.2%),其次是心悸(14%)、尿潴留(7%)、头晕和尿失禁(4.7%)。脑神经麻痹占34.9%,面瘫占27.9%,其次是球麻痹(7%)和眼麻痹(4.7%)。1例(2.3%)同时患有面瘫和眼麻痹。AIDP、AMAN和AMSAN亚型分别占32.6%、37.2%和20.9%。在自主神经功能障碍方面,常见的有姿势改变、深呼吸、valsalva等不同动作引起的心率变化。以30:15比值异常的患者居多(82.4%),其次为深呼吸max-min HR/min异常(58.1%)和valsalva比值异常(37.2%)。其他异常包括体位性低血压(38.2%)、窦性心动过速(25.6%)、高血压(16.3%)、低血压(4.7%)和窦性心律失常(4.7%)。另一种自主神经功能障碍是膀胱功能障碍,其中便秘占30%,尿潴留占7%,尿失禁占4.7%。结论:GBS表现多样,包括自主神经功能障碍。在本研究中,GBS患者的常见临床表现为肢体无力和不同类型的自主神经功能障碍。常见的电生理亚型为AMAN。因此,除了临床和电生理分析外,自主神经功能评估对每一位GBS患者都是必不可少的,因为自主神经功能障碍是导致死亡的重要原因之一。2019年孟加拉国神经科学杂志;Vol. 35 (2): 57-62
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