Abstract
Subacute necrotizing encephalomyelopathy (SNE) is a genetic disorder of pyruvate metabolism. Until recently the diagnosis of SNE could only be made at autopsy. However, an antemortem diagnosis can now be suggested by the correlation of clinical and laboratory data with computed tomography (CT) and/or magnetic resonance imaging (MRI). Five children with clinical and laboratory data suggesting the diagnosis of Leigh disease were evaluated by CT and MR. MR was found to be more sensitive than CT in the detection of areas of necrosis in the brain of the five children we studied. The absence of focal lesions detected by either modality in one of our patients did not exclude the diagnosis of SNE since focal lesions were present at autopsy one month following CT and MR.
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Leigh D (1951) Subacute necrotizing encephalomyelopathy in an infant. J Neurol Neurosurg 14: 216
Van Erven PM, Cillessen JP, Eekhoff EM, Gabreëls FJ, Disesburg WH, Lemmens WA, Slooff JL, Revier WO, Ruiterbeek W (1987) Leigh syndrome, mitochondrial encephalo(myo)pathy. Clin neurol Neurosurg 89: 217
Chi JG, Yoo HW, Chang KH, CW Kim, HR Moon, KW Ko (1981) Leigh's subacute necrotizing encephalomyelopathy: possible diagnosis by CT scan. Neuroradiology 22: 141
Bianco F, Floris R, Pozzessere G, Rizzo PA (1987) Subacute necrotizing encephalomyelopathy (Leigh's disease). Clinical correlations with computerized tomography in the diagnosis of the juvenile and adult forms. Acta Neurol Scand 76: 214
Gray F, Louarn F, Gherardi R, Etzenbaum JF, Marsault C (1984) Adult form of Leigh's disease: a clinico pathological case with CT scan examination. J Neurol Neurosurg Psychol 47: 1211
Koch TK, Lo WD, Berg BO (1985) Variability of serial CT scans in subacute necrotizing encephalomyelopathy (Leigh disease). Ped Neurol 1: 48
Riikonen R, Donner M (1979) Chronic relapsing course of encephalomyeloradiculopathy in a 6 year old boy. Neuropediatrics 18: 235
Taccone A, DiRocco M, Fondelli P, Cottafava F (1989) Leigh disease: value of CT in presymptomatic patients and variability of the lesions with time. J Comput Assist Tomogr 13: 207
Myers RE (1979) A unitary theory of causation of anoxic and hypoxic brain pathology. Adv Neurol 26: 195
Geyer CA, Sartor KJ, Prensky AJ, Abramson CL, Hodges FJ, Gado MH (1988) Leigh Disease (subacute necrotizing encephalomyelopathy): CT and MR in Five Cases. J Comput Assist Tomogr 12: 40
Davis PC, Hoffman JC, Jr, Braun IF, Ahmann P, Krawiecki N (1987) MR of Leigh's Disease (subacute necrotizing encephalomyelopathy). AJNR 8: 71
Kissel JT, Kolkin S, Chakeres D, Boesel C, Weiss K (1987) Magnetic resonance imaging in a case of autopsy-proved adult subacute necrotizing encephalomyelopathy (Leigh's disease). Arch Neurol 44: 563
Koch TK, Yee MH, Hutchinson HT, Berg BO (1986) Magnetic resonance imaging in subacute necrotizing encephalomyelopathy (Leigh's disease). Annals Neurol 19: 605
Onuma A, Miyabayashi S (1987) Comparative appraisal of CT scan and MRI in the diagnosis of Leigh encephalomyelopathy in two siblings. J Child Neurol 2: 324
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Greenberg, S.B., Faerber, E.N., Riviello, J.J. et al. Subacute necrotizing encephalomyelopathy (Leigh disease): CT and MRI appearances. Pediatr Radiol 21, 5–8 (1990). https://doi.org/10.1007/BF02010803
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DOI: https://doi.org/10.1007/BF02010803