Author:
Ibrahim Shahnaz H.,Farooq Hira
Abstract
IntroductionSub-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.MethodologyLow 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.ResultsA 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.ConclusionLGIT may play an effective role in the management of SSPE and gives hope to families having children with this potentially life-threatening disease.
Subject
Nutrition and Dietetics,Endocrinology, Diabetes and Metabolism,Food Science
Reference26 articles.
1. Subacute sclerosing panencephalitis: clinical and demographic characteristics;Rafique;J Coll Phys Surg Pak.,2014
2. Subacute sclerosing panencephalitis-current perspectives;Jafri;Pediatr Health Med Ther.,2018
3. SSPE-clinical staging, course, and frequency;Jabbour;Arch Neurol.,1975
4. Neuroprogressive disease of post-infectious origin: a review of a resurging subacute sclerosing panencephalitis (SSPE);Dyken;Ment Retard Dev Disabil Res Rev.,2001
5. Basal ganglia lesions in subacute sclerosing panencephalitis;Almeida;Dement Neuropsychol.,2012