Abstract

Leigh's syndrome also known as subacute necrotizing encephalomyopathy is a rare progressive neurological disease with a reserved prognosis affecting the brainstem and the basal nuclei, the symptomatology of which is revealed in the majority of cases during the first two years of life.
In this article we report the case of an 4 years old child referred to our center following a consultation for delayed psychomotor acquisitions with axial hypotonia and elevated lactate levels. Brain MRI showed a characteristic appearance of this syndrome. This clinical case reports the MRI aspect of Leigh's syndrome, a rare and serious pathology responsible for bilateral and symmetrical lesions of the basal ganglia.

Key words: Leigh Disease, Basal ganglia, Magnetic Resonance Imaging