Suthar Renu, Sankhyan Naveen, Sahu Jitendra K, Khandelwal Niranjan K, Singhi Sunit, Singhi Pratibha
Unit of Pediatric Neurology and Neurodevelopment, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
Department of Radio Diagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India.
Eur J Paediatr Neurol. 2016 May;20(3):352-60. doi: 10.1016/j.ejpn.2016.01.013. Epub 2016 Feb 13.
Acute transvers myelitis (ATM) is a rare and disabling condition in childhood. There are only few reports of clinical profile, prognosis and predictors of ATM from developing countries.
To study the clinical profile of children with ATM and predictors of its outcome.
Retrospective analysis of children <12 years of age diagnosed with ATM over a period of 6 years from a tertiary care institute.
Thirty six children (21 boys, median age-7.5 years) were diagnosed with ATM. Weakness was symmetrical at onset in 27 (75%) children with progression over a median of 2 days (IQR 1-5 days). Severe weakness at onset with lower limb power ≤ 1/5 on MRC scale was present in 27 (75%), a sensory level in 25(69.4%) and bladder dysfunction in 31(86.1%) children. MRI showed longitudinal extensive myelitis (LETM) in 27 (75%) children and the thoracic cord was most commonly affected [18 (50%)]. On a median follow up of 35 months (range IQR 11-57 months); 15 (41.7%) were non ambulatory or required assistance to walk. Severe weakness at onset with power ≤ 1 on MRC scale, spinal shock, respiratory muscle weakness, mechanical ventilation, greater mean time to diagnosis and treatment was associated with bad outcome. ATM was a monophasic illness in all, except in 3 children; all with neuromyelitis optica spectrum disorder. Progression to multiple sclerosis was not seen in any child in our cohort.
In this series of childhood ATM from North India, the disease was severe, monophasic and involved long segments (≥ 3) of cord in majority. Nearly half the children remain dependent on follow up. Delayed diagnosis and delayed initiation of steroid therapy was associated with poor outcome.
急性横贯性脊髓炎(ATM)在儿童期是一种罕见且致残的疾病。来自发展中国家的关于ATM的临床特征、预后及预测因素的报道很少。
研究患有ATM的儿童的临床特征及其预后的预测因素。
对一家三级医疗机构6年间诊断为ATM的12岁以下儿童进行回顾性分析。
36名儿童(21名男孩,中位年龄7.5岁)被诊断为ATM。27名(75%)儿童起病时肌无力为对称性,在中位时间2天(四分位间距1 - 5天)内进展。27名(75%)儿童起病时肌无力严重,下肢肌力按医学研究委员会(MRC)分级≤1/5,25名(69.4%)有感觉平面,31名(86.1%)有膀胱功能障碍。MRI显示27名(75%)儿童有纵向广泛脊髓炎(LETM),最常受累的是胸段脊髓[18名(50%)]。中位随访35个月(范围四分位间距11 - 57个月);15名(41.7%)不能行走或行走需要帮助。起病时肌无力严重,MRC分级≤1、脊髓休克、呼吸肌无力、机械通气、诊断和治疗的平均时间较长与不良预后相关。除3名儿童外,所有患者的ATM均为单相病程;这3名儿童均患有视神经脊髓炎谱系障碍。在我们的队列中,没有儿童进展为多发性硬化。
在这组来自印度北部的儿童ATM病例中,该疾病严重,为单相病程,多数累及脊髓长节段(≥3个)。近一半儿童在随访时仍有功能依赖。诊断延迟和类固醇治疗开始延迟与不良预后相关。