Ibrahim Shahnaz H, Farooq Hira
Department of Pediatrics and Child Health, Aga Khan University, Karachi, Pakistan.
Department of Nutrition, Aga Khan University, Karachi, Pakistan.
Front Nutr. 2023 Jul 24;10:1203144. doi: 10.3389/fnut.2023.1203144. eCollection 2023.
Sub-acute sclerosing panencephalitis (SSPE) is a chronic, progressive neurodegenerative disorder, commonly seen in measles-endemic countries leading to progressive neuronal loss and death. Currently, there is no proven cure for this devastating disease. We started a low glycemic index therapy (LGIT) in children with SSPE using the same principle as per its role in intractable epilepsy.
Low glycemic index diet was started in children with a confirmed diagnosis of SSPE based on Dyken's criteria. All children were then classified into four stages according to disease progression. The response to diet was evaluated by improvement in their myoclonic jerks, motor activities, and changes in their stage of the disease.
A total of 12 children were enrolled. The mean age was 6.65 years (range 3.3-10 years), with a male-to-female ratio of 2:1. Five children were at stage IV, five were at stage III, and two were at stage II at the start of the diet. Nine (75%) children showed improvement in their stage of illness. Of three children who were at stage IV at the initiation of the diet, one improved to stage II and two to stage III. Four children at stage III reverted to stage II. Two children initiated at stage II went into total remission. Seven (58.3%) children showed a >50% reduction in myoclonic jerks with three (25%) having a 100% reduction. Three (25%) children died due to pneumonia.
LGIT may play an effective role in the management of SSPE and gives hope to families having children with this potentially life-threatening disease.
亚急性硬化性全脑炎（SSPE）是一种慢性进行性神经退行性疾病，常见于麻疹流行国家，会导致神经元逐渐丧失和死亡。目前，尚无已证实的治愈这种毁灭性疾病的方法。我们基于其在难治性癫痫中的作用原理，对患有SSPE的儿童开展了低血糖指数疗法（LGIT）。
根据戴肯标准，对确诊为SSPE的儿童开始采用低血糖指数饮食。然后根据疾病进展将所有儿童分为四个阶段。通过肌阵挛抽搐、运动活动的改善以及疾病阶段的变化来评估饮食反应。
共纳入12名儿童。平均年龄为6.65岁（范围3.3 - 10岁），男女比例为2:1。饮食开始时，5名儿童处于IV期，5名处于III期，2名处于II期。9名（75%）儿童的疾病阶段有所改善。饮食开始时处于IV期的3名儿童中，1名改善至II期，2名改善至III期。4名III期儿童恢复至II期。2名II期开始的儿童完全缓解。7名（58.3%）儿童的肌阵挛抽搐减少>50%，其中3名（25%）完全消失。3名（25%）儿童因肺炎死亡。
LGIT可能在SSPE的治疗中发挥有效作用，并给患有这种潜在危及生命疾病患儿的家庭带来希望。
