CLINICAL AND NEUROIMAGING CORRELATES OF LEIGH SYNDROME , A CASE SERIES AND REVIEW OF LITERATURE

Author:

Leela Prasanna Dadireddy1,Haripriyanka Vaka2

Affiliation:

1. Pediatric Junior Resident, Narayana Medical College And Hospital, Nellore-524003. Andhra Pradesh

2. Pediatric Junior Resident, Narayana Medical College and Hospital, Nellore- 524003. Andhra Pradesh.

Abstract

Leigh syndrome, also referred to as Sub acute necrotising encephalopathy usually presents with symptoms like developmental delay, regression of neurological skills and sudden death. Approximately 25% of the patients are abnormal at birth with features like hypotonia, lactic acidosis, feeding problems, hypoglycemia, hyperbilirubinemia, cardiac complications, seizures and/or hyperammonemia. Here, we present 6 cases of Leigh's disease , who presented within 6 months of life with neurological regression and hypotonia as the predominent manifestations with MRI ndings showing Bilateral symmetrical hyperintensities in T2 weighted images which are characteristic ndings in Leigh's disease.

Publisher

World Wide Journals

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