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Progressive neurologic deterioration with unusual findings on magnetic resonance imaging in a 43-year-old man treated for demyelinating disease

Article Abstract:

A 43-year-old paraplegic man with confusion, difficulty communicating, fatigue, weight loss, bed sores, and seizures was found to have granulomatous angiitis of the central nervous system. Granulomatous angiitis is a blood vessel disease in which blood cells accumulate in nodules. The man became paraplegic approximately 4 years earlier and had a brain hemorrhage 15 months earlier. He had been treated with corticosteroids. Intermittent magnetic resonance imaging (MRI) scans and CT scans during the preceding 2 years showed developing nodes in the white matter of the brain and upper spinal cord. Earlier spinal taps showed protein in his cerebrospinal fluid. A brain biopsy showed that the white matter nodes contained various blood cells including monocytes, lymphocytes, and plasmacytes. He was treated with cyclophosphamide while his corticosteroids were gradually withdrawn. Granulomatous angiitis can be definitively diagnosed only with a biopsy.

Author: Vonsattel, Jean-Paul, Ronthal, Michael
Publisher: Massachusetts Medical Society
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1995
Vasculitis, Nervous system diseases, Granuloma

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A 15-year-old girl with hemiparesis, slurred speech, and an intracranial lesion

Article Abstract:

A 15-year-old girl was admitted to a hospital when she developed slurred speech, partial paralysis and difficulty speaking. A CT scan revealed a single abnormality, which her doctors suspected was a tumor. This would be the most likely cause of this kind of abnormality in a young person. However, a brain biopsy showed that she had a demyelinating disease similar to multiple sclerosis. She was treated with corticosteroids and cyclophosphamide.

Author: Vonsattel, Jean-Paul, Zalneraitis, Edwin L.
Publisher: Massachusetts Medical Society
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1998
Demyelinating diseases

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Chorea and progressive dementia in an 88-year-old woman

Article Abstract:

A woman was diagnosed with late-onset Huntington's disease, which began when she was in her mid-60s. Several family members, including those in previous generations, also exhibited this unusual manifestation of Huntington's disease, which usually strikes in middle age.

Author: Vonsattel, Jean-Paul, Sax, Daniel S.
Publisher: Massachusetts Medical Society
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1992
Case studies, Huntington's chorea, Huntington's disease

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Subjects list: Diagnosis
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