| CADASIL mutation and Balo concentric sclerosis: A link between demyelination and ischemia? | |
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MedLine Citation:
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PMID: 22218279 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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A 26-year-old man of Portuguese descent with no significant past medical history presents with subacute onset of right-sided hemiparesis and aphasia, with marked expressive aphasia, word-finding difficulty, and rare paraphrasic errors. Examination demonstrated right central facial weakness, 2-3/5 strength in the right arm and leg, and dysmetria in the right upper and lower extremities. Reflexes were hyperactive on the right with a right-sided Babinski response. MRI of the brain showed multiple bilateral concentric ring-like structures in the centrum semiovale and the corona radiata on T2 imaging (figure, A), consistent with the pattern of Balo's concentric sclerosis. There was associated restricted diffusion in 3 lesions and incomplete ring enhancement in 1 lesion. CSF analysis showed 30 leukocytes (79% lymphocytes, 4% monocytes), erythrocyte count was 850, and oligoclonal bands were absent. He was treated with 1 g of IV methylprednisolone daily for 5 days, with little improvement. He then underwent 5 plasmapheresis exchanges, with significant improvement of motor, sensory, speech deficits, and gait. Forty-five days after discharge, his examination showed 4+/5 strength in the right intrinsic muscles of the hand and a right-sided positional tremor. Gait and tandem gait were normal. He was placed on β-interferon-1a 44 μg subcutaneously 3 times a week, for treatment of presumed clinically isolated syndrome, with good response. One year later, his neurologic examination was essentially normal. |
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Authors:
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Tanuja Chitnis; Travis J Hollmann |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2012-1-4 |
Journal Detail:
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Title: Neurology Volume: - ISSN: 1526-632X ISO Abbreviation: - Publication Date: 2012 Jan |
Date Detail:
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Created Date: 2012-1-5 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0401060 Medline TA: Neurology Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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From the Partners Multiple Sclerosis Center, Department of Neurology (T.C.), and Department of Pathology (T.J.H.), Brigham and Women's Hospital and Harvard Medical School, Boston, MA. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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