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BC, Canada: Man man be suffering from rare form of multiple sclerosis called 'Marburg'
- Thread starter Emily
- Start date
Giuseppe
Emeritus
Re: BC, Canada: Man man be suffering from rare form of multiple sclerosis called 'Marburg'
Various papers discuss this condition. For example:
[Source: Annals of Neurology, full page: (LINK). Abstract, edited.]
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Various papers discuss this condition. For example:
[Source: Annals of Neurology, full page: (LINK). Abstract, edited.]
Acute multiple sclerosis (marburg type) is associated with developmentally immature myelin basic protein
D. D. Wood MD<SUP>1</SUP>, M. A. Moscarello MD<SUP>1,*</SUP>, J. M. Bilbao MD<SUP>2</SUP>, P. O'Connors MD<SUP>3</SUP>
<SUP></SUP>
Article first published online: 8 OCT 2004
DOI: 10.1002/ana.410400106
Copyright ? 1996 American Neurological Association - Annals of Neurology, Volume 40, Issue 1, pages 18?24, July 1996
Author Information: <SUP>1</SUP>Division of Biochemistry, Research Institute, Hospital for Sick Childre, St Michael's Hospital, Toronto, Ontario, Canada <SUP>2</SUP>Department of Patholog, St Michael's Hospital, Toronto, Ontario, Canada <SUP>3 </SUP>Multiple Sclerosis Clinic, St Michael's Hospital, Toronto, Ontario, Canada <SUP>*</SUP>Division of Biochemistry, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada M5G1X8
Publication History: Issue published online: 8 OCT 2004 - Article first published online: 8 OCT 2004 - Manuscript Revised: 1 FEB 1996 - Manuscript Accepted: 1 FEB 1996 - Manuscript Received: 29 NOV 1995
Abstract
We have studied a case of acute, fulminating multiple sclerosis (MS) (Marburg type) at the pathological and biochemical levels. Postmortem examination of the brain revealed extensive areas of gross rarefaction in the hemispheric white matter. Histologically, well-demarcated areas of demyelination with a large influx of macrophages and a subtle perivascular infiltration of lymphocytes were seen with relative preservation of the axis cylinders. Myelin basic protein (MBP) was isolated and purifed from noninvolved white matter. It was slightly larger in molecular weight than MBP from normal brain or from chronic MS brain. The increase in mass was accounted for, in part, by the deimination of 18 of 19 arginyl residues to citrulline, making the patient's MBP much less cationic than MBP from normal white matter. When expressed as the ratio of least cationic form of MBP to the most cationic (C-8/C-1), the normal ratio was 0.82, chronic MS 2.5, and the patient in this study 6.7. Because the ratio of 6.7 was similar to 7.5 found for a 15-month-old infant, MBP was considered to be of the immature form. The data are consistent with a genetic factor influencing the charge microheterogeneity of MBP. The resulting less cationic MBP cannot carry out its normal function of compacting multilayers.
-D. D. Wood MD<SUP>1</SUP>, M. A. Moscarello MD<SUP>1,*</SUP>, J. M. Bilbao MD<SUP>2</SUP>, P. O'Connors MD<SUP>3</SUP>
<SUP></SUP>
Article first published online: 8 OCT 2004
DOI: 10.1002/ana.410400106
Copyright ? 1996 American Neurological Association - Annals of Neurology, Volume 40, Issue 1, pages 18?24, July 1996
Author Information: <SUP>1</SUP>Division of Biochemistry, Research Institute, Hospital for Sick Childre, St Michael's Hospital, Toronto, Ontario, Canada <SUP>2</SUP>Department of Patholog, St Michael's Hospital, Toronto, Ontario, Canada <SUP>3 </SUP>Multiple Sclerosis Clinic, St Michael's Hospital, Toronto, Ontario, Canada <SUP>*</SUP>Division of Biochemistry, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada M5G1X8
Publication History: Issue published online: 8 OCT 2004 - Article first published online: 8 OCT 2004 - Manuscript Revised: 1 FEB 1996 - Manuscript Accepted: 1 FEB 1996 - Manuscript Received: 29 NOV 1995
Abstract
We have studied a case of acute, fulminating multiple sclerosis (MS) (Marburg type) at the pathological and biochemical levels. Postmortem examination of the brain revealed extensive areas of gross rarefaction in the hemispheric white matter. Histologically, well-demarcated areas of demyelination with a large influx of macrophages and a subtle perivascular infiltration of lymphocytes were seen with relative preservation of the axis cylinders. Myelin basic protein (MBP) was isolated and purifed from noninvolved white matter. It was slightly larger in molecular weight than MBP from normal brain or from chronic MS brain. The increase in mass was accounted for, in part, by the deimination of 18 of 19 arginyl residues to citrulline, making the patient's MBP much less cationic than MBP from normal white matter. When expressed as the ratio of least cationic form of MBP to the most cationic (C-8/C-1), the normal ratio was 0.82, chronic MS 2.5, and the patient in this study 6.7. Because the ratio of 6.7 was similar to 7.5 found for a 15-month-old infant, MBP was considered to be of the immature form. The data are consistent with a genetic factor influencing the charge microheterogeneity of MBP. The resulting less cationic MBP cannot carry out its normal function of compacting multilayers.
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