Objective: Recognizing the clinical presentation of Guillain-Barre syndrome in children. Design: Retrospective observational study. Setting: Pediatric wards and Paediatric Intensive Care Unit. Method: A medical record files of 52 patients of GBS, between the periods of March 2011 to February 2016 were recovered from Medical Record and Statistics Section of institute. Files were analyzed for demographic data, clinical features, co-morbid conditions, investigations, electrophysiological data, mode and result of treatment and data was analyzed. Result: In our series males are outnumber to female and most of the patients in age group 6-12 years, 48% were belongs to lower socioeconomic status. In 53.8% patients antecedents were present at the beginning of clinical picture and upper respiratory tract infection (28.84%) & diarrohea (19.23%) was noted with predominant occurrence in monsoon season (52%). Bilateral lower limb weakness was noted in all patients and in 61.9% patients were common complaint pain in lower limb. Cranial nerve involvement was evident in 28.85% and bulbar involvement was commonest followed by facial nerve. A 23.07% patients were presented with respiratory involvement, 9 of them (15.38%) were needed assisted ventilation. Dysautonomic sign/symptoms were present in 26.92% with 73.33% had demyelinating pattern of neuropathy and 9.6% mortality. Conclusion: The clinical characteristics of GBS in children are not consistent and differ from geographical area and racial diversity.
We present a very rare case of mucopolysaccharidosis with atypical presentation such as severe mental retardation, dolicocephalic head, a coarse facial features, no corneal clouding with inguinal hernia. The purpose of presenting this case is to highlight the distinctive manifestation of Hunter Syndrome. Based on thorough clinical examination and radiological survey it is possible to diagnose a case of mucopolysaccharidosis. Multidisciplinary approach will go a long way to manage the patient holistically.
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