Kumar Maneesh, Aroor Shrikiran, Mundkur Suneel, Kumar Sandeep
Senior Resident, Department of Paediatrics, KMC Manipal , India .
Professor, Department of Paediatrics, KMC Manipal , India .
J Clin Diagn Res. 2015 Jan;9(1):SC09-12. doi: 10.7860/JCDR/2015/8344.5491. Epub 2015 Jan 1.
Guillain-Barre Syndrome (GBS) is an acute monophasic demyelinating neuropathy characterized by progressive motor weakness of limbs with areflexia.
To study the clinical pattern and outcome of children with Guillain-Barre syndrome.
It was a cross-sectional study conducted in a pediatric unit of tertiary care hospital over a period of 18 months. We assessed the clinical manifestations, results of electro-diagnostic tests, functional status, treatment instituted and outcome of 20 children diagnosed with GBS.
Of the 20 (male to female ratio = 2.3:1) children studied, all had motor weakness, 5 (25%) had sensory loss, 4 (20 %) had cranial nerve palsies and 4 (20%) had autonomic disturbances. Respiratory paralysis was found in 7 (35%) children requiring assisted ventilation. Antecedent illness preceding GBS was recorded in 50% children. The GBS subtype distribution as per electrodiagnostic studies was as follows: acute motor axonal neuropathy (AMAN) in 7 (38.9%), acute motor sensory axonal neuropathy (AMSAN) in 4 (22.2%), acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 4 (22.2%) and both axonal and demyelinating neuropathy in 3 (16.7%). Intravenous immunoglobulins (IVIG) constituted the treatment given in majority of the patients. Plasmapharesis was performed in one child in view of poor response to IVIG. Complete recovery was observed in 14 children and the remaining 3 children experienced only incomplete recovery.
Male preponderance and presence of antecedent illness in a majority of subjects was observed in our study. Regardless of the severity of illness at admission and electrophysiological subtypes, a majority achieved full recovery. Intravenous Immunoglobulin and supportive care form the cornerstone of management in childhood GBS.
吉兰 - 巴雷综合征（GBS）是一种急性单相性脱髓鞘性神经病，其特征为肢体进行性肌无力伴腱反射消失。
研究儿童吉兰 - 巴雷综合征的临床模式及预后。
这是一项在三级护理医院儿科病房进行的为期18个月的横断面研究。我们评估了20例诊断为GBS的儿童的临床表现、电诊断检查结果、功能状态、所采取的治疗措施及预后。
在研究的20例儿童中（男女比例为2.3:1），均有肌无力，5例（25%）有感觉丧失，4例（20%）有脑神经麻痹，4例（20%）有自主神经功能紊乱。7例（35%）儿童出现呼吸麻痹，需要辅助通气。50%的儿童记录到GBS发病前有前驱疾病。根据电诊断研究，GBS亚型分布如下：急性运动轴索性神经病（AMAN）7例（38.9%），急性运动感觉轴索性神经病（AMSAN）4例（22.2%），急性炎症性脱髓鞘性多发性神经根神经病（AIDP）4例（22.2%），轴索性和脱髓鞘性神经病并存3例（16.7%）。大多数患者接受了静脉注射免疫球蛋白（IVIG）治疗。鉴于对IVIG反应不佳，1例儿童进行了血浆置换。14例儿童完全康复，其余3例儿童仅不完全恢复。
我们的研究观察到男性居多且大多数受试者有前驱疾病。无论入院时疾病的严重程度及电生理亚型如何，大多数患者实现了完全康复。静脉注射免疫球蛋白和支持治疗是儿童GBS治疗的基石。
