Leigh Syndrome: A Rare Mitrochondrial Disorder

  • Archana Shrestha Yadav Resident Phase-B, Department of Pediatric Gastroenterology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka
  • Mahbub Mutanabbi Associate Professor, Department of Pediatrics Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka
  • Gopen Kumar Kundu Department of Paediatric Neurology and Development, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka
  • CA Kawser Professor, Department of Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka
Keywords: Brainstem, Basal Ganglia, Inherited Neuro-metabolic, Necrotizing Encephalopathy

Abstract

Leigh syndrome is a rare inherited neurometabolic subacute necrotizing encephalopathy mostly involving brainstem and basal ganglia, seen in the early childhood. It is characterized by progressive loss of mental and motor abilities associated with abnormal muscle tone, weakness, visual loss and respiratory failure. There is no effective treatment for this condition, as such the prognosis of this condition is very bad with death occurring within the first few years of life most commonly due to respiratory failure. Here we present a rare case of Leigh syndrome seen in a 3 and half years male Bangladeshi child with clinical and laboratory features of Leigh Syndrome suggested by neuro-imaging.

Bangladesh J Child Health 2017; VOL 41 (3) :189-192

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Abstract
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Published
2018-06-10
How to Cite
Yadav, A., Mutanabbi, M., Kundu, G., & Kawser, C. (2018). Leigh Syndrome: A Rare Mitrochondrial Disorder. Bangladesh Journal of Child Health, 41(3), 189-192. https://doi.org/10.3329/bjch.v41i3.36955
Section
Case Reports