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Figure 2: Algorithm depicting the approach to diagnosis in our case. OA: Organic acidemias, MSUD: Maple syrup urine disease, PC: Pyruvate carboxylase, E3: Lipoamide oxidoreductase, KGDH: Alpha ketoglutarate dehydrogenase, SCOT: Succinyl CoA 3-oxacid CoA transferase, PDH: Pyruvate dehydrogenase, FAO: Fatty acid oxidation, CPTII: Carnitine palmitoyl transferase II, VLCAD: Very-long-chain acyl-CoA dehydrogenase, HMG CoA lyase: 3-hydroxy-3-methylglutaryl-CoA lyase, N: Normal, ↑: Mild elevation, ↑↑: Marked elevation, GC-MS: Gas chromatography mass spectrometer, 3MGA: 3-methyl glutaconic aciduria, AUH: AU RNA binding protein/Enoyl-CoA hydratase, TAZ: Tafazzin, OPA3: Optic atrophy 3, TMEM70: Transmembrane protein 70, DNAJC19: DNAJ heat shock protein family (Hsp40) member C19

Figure 2: Algorithm depicting the approach to diagnosis in our case. OA: Organic acidemias, MSUD: Maple syrup urine disease, PC: Pyruvate carboxylase, E3: Lipoamide oxidoreductase, KGDH: Alpha ketoglutarate dehydrogenase, SCOT: Succinyl CoA 3-oxacid CoA transferase, PDH: Pyruvate dehydrogenase, FAO: Fatty acid oxidation, CPTII: Carnitine palmitoyl transferase II, VLCAD: Very-long-chain acyl-CoA dehydrogenase, HMG CoA lyase: 3-hydroxy-3-methylglutaryl-CoA lyase, N: Normal, ↑: Mild elevation, ↑↑: Marked elevation, GC-MS: Gas chromatography mass spectrometer, 3MGA: 3-methyl glutaconic aciduria, AUH: AU RNA binding protein/Enoyl-CoA hydratase, TAZ: Tafazzin, OPA3: Optic atrophy 3, TMEM70: Transmembrane protein 70, DNAJC19: DNAJ heat shock protein family (Hsp40) member C19