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Stroke-like episodes in autosomal recessive cytochrome oxidase deficiency

1999; Wiley; Volume: 45; Issue: 3 Linguagem: Inglês

10.1002/1531-8249(199903)45

1531-8249

Charles Morin, J J. Dub, Brian H. Robinson, Jacques Lacroix, Jean Michaud, Marc De Braekeleer, G. Geoffroy, Anne Lortie, Christine Blanchette, Marie Lambert, Grant A. Mitchell,

ATP Synthase and ATPases Research

Annals of NeurologyVolume 45, Issue 3 p. 389-392 Brief Communication Stroke-like episodes in autosomal recessive cytochrome oxidase deficiency Charles Morin MD, Charles Morin MD Department of Pediatrics, Hôpital de Chicoutimi, Université du Québec à Chicoutimi, Chicoutimi, Quebec, CanadaSearch for more papers by this authorJacques Dubé MD, Jacques Dubé MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaSearch for more papers by this authorBrian H. Robinson PhD, Brian H. Robinson PhD Department of Biochemical Genetics, Hospital for Sick Children, Toronto, Ontario, CanadaSearch for more papers by this authorJacques Lacroix MD, Jacques Lacroix MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaSearch for more papers by this authorJean Michaud MD, Jean Michaud MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaSearch for more papers by this authorMarc De Braekeleer MD, PhD, Marc De Braekeleer MD, PhD Department of Humanities, Université du Québec à Chicoutimi, Chicoutimi, Quebec, CanadaSearch for more papers by this authorGuy Geoffroy MD, Guy Geoffroy MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaSearch for more papers by this authorAnne Lortie MD, Anne Lortie MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaSearch for more papers by this authorChristine Blanchette MD, Christine Blanchette MD Department of Pediatrics, Hôpital de Roberval, Roberval, Quebec, CanadaSearch for more papers by this authorMarie A. Lambert MD, Marie A. Lambert MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaSearch for more papers by this authorGrant A. Mitchell MD, Corresponding Author Grant A. Mitchell MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaService de génétique médicale, Hôpital Ste-Justine, 3175 Côte Ste-Catherine Road, Montréal, Québec, Canada H3T 1C5Search for more papers by this author Charles Morin MD, Charles Morin MD Department of Pediatrics, Hôpital de Chicoutimi, Université du Québec à Chicoutimi, Chicoutimi, Quebec, CanadaSearch for more papers by this authorJacques Dubé MD, Jacques Dubé MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaSearch for more papers by this authorBrian H. Robinson PhD, Brian H. Robinson PhD Department of Biochemical Genetics, Hospital for Sick Children, Toronto, Ontario, CanadaSearch for more papers by this authorJacques Lacroix MD, Jacques Lacroix MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaSearch for more papers by this authorJean Michaud MD, Jean Michaud MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaSearch for more papers by this authorMarc De Braekeleer MD, PhD, Marc De Braekeleer MD, PhD Department of Humanities, Université du Québec à Chicoutimi, Chicoutimi, Quebec, CanadaSearch for more papers by this authorGuy Geoffroy MD, Guy Geoffroy MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaSearch for more papers by this authorAnne Lortie MD, Anne Lortie MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaSearch for more papers by this authorChristine Blanchette MD, Christine Blanchette MD Department of Pediatrics, Hôpital de Roberval, Roberval, Quebec, CanadaSearch for more papers by this authorMarie A. Lambert MD, Marie A. Lambert MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaSearch for more papers by this authorGrant A. Mitchell MD, Corresponding Author Grant A. Mitchell MD Departments of Radiology, Pediatrics, Neurology Pathology, Hôpital Ste-Justine, Montreal, Quebec, CanadaService de génétique médicale, Hôpital Ste-Justine, 3175 Côte Ste-Catherine Road, Montréal, Québec, Canada H3T 1C5Search for more papers by this author First published: 31 May 2001 https://doi.org/10.1002/1531-8249(199903)45:3 3.0.CO;2-BCitations: 30AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat Abstract Stroke-like episodes, defined as periods of acute localized neurological dysfunction during which brain imagery suggests cerebral ischemia but vascular anatomy is normal, occurred in 3 patients with autosomal recessive Saguenay-Lac St-Jean (SLSJ) cytochrome oxidase (COX) deficiency. The patients developed focal neurological deterioration and frontal hypodensities on cerebral computerized tomography (CT). Arteriography, performed in 1 patient during an acute episode, showed normal vascular anatomy. Nevertheless, capillary shunting was evident both in regions that appeared abnormal on the initial cerebral CT study and in regions that appeared normal but subsequently developed Leigh disease. Stroke-like episodes did not exacerbate systemic acidosis, and acidotic decompensations occurred independently of stroke-like episodes. 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