Leigh syndrome. Differential diagnosis in brainstem lesions

We report the case of a 4 year-old-girl with a late infatile onset encephalopathy and first metabolic crisis at the age of 16 months resulting in pyramidal and extrapyramidal involvement associated with severe axial hypotonia.Later she developed ocular movement and sleep abnormalities and recurrent...

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Detalles Bibliográficos
Autor principal: Romero, C.
Otros Autores: Alvarez, M.G, Storino, O., Taratuto, A.L, Alfonso, J.M, Massaro, M., Meli, F.
Formato: Capítulo de libro
Lenguaje:Español
Publicado: 2004
Acceso en línea:Registro en Scopus
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Registro en la Biblioteca Digital
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100 1 |a Romero, C. 
245 1 0 |a Leigh syndrome. Differential diagnosis in brainstem lesions 
246 3 1 |a Síndrome de Leigh. Diagnóstico diferencial en lesiones de tronco cerebral 
260 |c 2004 
270 1 0 |m Romero, C.Montañeses 2325, 1428 Buenos Aires, Argentina; email: cromero@fleni.org.ar 
506 |2 openaire  |e Política editorial 
504 |a Arii, J., Tanabe, Y., Leigh Syndrome: Serial MRI and Clinical Follow-up (2000) AJNR, 21, pp. 502-1509 
504 |a Arbelaez, A., Castillo, M., Stone, J., MRI in 3-methylglutaconic aciduria type 1 (1999) Neuroradiology, 41, pp. 941-942 
504 |a Barkovich, A.J., Good, W.V., Koch, T.K., Berg, B.O., Mitochondrial Disorders: Analysis of their clinical and imaging characteristics (1993) AJNR, 14, pp. 1119-1137 
504 |a Cacic, C., Wilichowski, E., Mejaski-Bosnjak, V., Cytochrome c oxidase partial deficiency-associated Leigh disease presenting as an extrapyramidal syndrome (2001) J Child Neurol, 16 (8), pp. 616-619 
504 |a Detre, J.A., Wang, Z., Bogdan, A.R., Regional variation in Brain lactate in Leigh syndrome by localized HMRS (1991) Ann Neurol, 29, pp. 218-221 
504 |a DiMauro, S., Schon, E.A., Mitochondrial Respiratory-Chain Diseases (2003) N Engl J Med, 348, pp. 2656-2668 
504 |a DiMauro, S., De Vivo, D.C., Genetic heterogeneity in Leigh Syndrome (1996) Ann Neurol, 40, pp. 5-6 
504 |a DiMauro, S., Moraes, C.T., Mitochondrial Encephalomyopathies (1993) Arch Neurol, 50, pp. 1197-2108 
504 |a DiMauro, S., Servidei, S., Zeviani, M., Cytochrome c-oxidase deficiency in Leigh syndrome (1987) Ann Neurol, 22, pp. 498-506 
504 |a Jackson, M.J., Schaefer, J.A., Johnson, M.A., Presentation and clinical investigation of mitochondrial respiratory chain disease: A Study of 51 patients (1993) Brain, 118, pp. 339-357 
504 |a Jiang, Y.W., Quin, J., Yuan, Y., Neuropathologic and Clinical features in eight chinese patients with Leigh disease (2002) J Child Neurol, 6, pp. 450-452. , Jun 17 
504 |a Martinez Bermejo, A., Arcas, J., Roche, M.C., Bilateral hypodensity of the basal ganglia: Clinico-evolutionary correlation in children (2001) Rev Neurol, 33 (2), pp. 101-111 
504 |a Muñoz, A., Mateos, F., Simón, R., Mitochondrial diseases in children: Neuroradiological and Clinical features in 17 patients (1999) Neuroradiology, 41, pp. 920-928 
504 |a Nagashima, T., Mori, M., Katayama, K., cols, Adult Leigh syndrome with mitochondrial DNA mutation at 8993 (1999) Acta Neuropathol, 97, pp. 416-422 
504 |a Playan A, Solano Palacios A, Gonzalez de la Rosa JB, and cols. Leigh syndrome resulting from a de novo mitochondrial DNA mutation (T8993 6). Rev Neurol 2002; Jun 34 (12): 1124-1126; Savasta, S., Comi, G.P., Perini, M.P., Leigh disease: Clinical, neuroradiologic, and biochemical study of three new cases with cytochrome c oxidase deficiency (2001) J Child Neurol, 16 (8), pp. 608-613 
504 |a Takahashi, S., Oki, J., Miyamoto, A., Okuno, A., Proton MRS to study the metabolic changes in the brain of a patient with Leigh syndrome (1999) Brain Dev, 21 (3), pp. 200-204 
504 |a Topcu, M., Saatci, I., Anil Apak, R., Leigh syndrome in a 3-year-old boy with unusual brain MRI and pathologic findings (2000) AJNR, 21, pp. 224-227 
504 |a Valanne, L., Ketonen, L., Majander, A., Neuroradiologic Findings in Children with Mitochondrial Disorders (1998) AJNR, 19, pp. 369-377 
520 3 |a We report the case of a 4 year-old-girl with a late infatile onset encephalopathy and first metabolic crisis at the age of 16 months resulting in pyramidal and extrapyramidal involvement associated with severe axial hypotonia.Later she developed ocular movement and sleep abnormalities and recurrent intestinal pseudo-occlusive epidoses. Metabolic workup was normal in blood and urine, with high lactate on CSF. Muscle histopathologic and histochemical revealed normal structure with evidences of increased oxidative mitocondrial activity. MRI showed symmetrical and bilateral brain stem lesions with low signal in T1 and high signal in T2 and difusion-weighted images. The clinical evolution and radiological abnormalities are consistent with the infantile form of Leigh Syndrome.  |l eng 
593 |a Servicio de Resonancia Magnética, F.L.E.N.I Instituto de Investigaciones Neurológicas Raúl Carrea 
593 |a Servicio de Neuropediatría, F.L.E.N.I Instituto de Investigaciones Neurológicas Raúl Carrea 
593 |a Servicio de Anestesología, F.L.E.N.I Instituto de Investigaciones Neurológicas Raúl Carrea 
593 |a Servicio de Neuropatología, F.L.E.N.I Instituto de Investigaciones Neurológicas Raúl Carrea 
593 |a Montañeses 2325, 1428 Buenos Aires, Argentina 
690 1 0 |a RESPIRATORY CHAIN DISEASES-MTDNA MUTATION-BRAIN MRI 
700 1 |a Alvarez, M.G. 
700 1 |a Storino, O. 
700 1 |a Taratuto, A.L. 
700 1 |a Alfonso, J.M. 
700 1 |a Massaro, M. 
700 1 |a Meli, F. 
773 0 |d 2004  |g v. 29  |h pp. 166-168  |k n. 3  |p Rev. Neurol. Argent.  |x 03250938  |t Revista Neurologica Argentina 
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