Abstract
We report a case of Alexander's disease in a black South African boy of 9 years. The child presented with a grossly abnormal stooped posture, generalised weakness, and slurred nasal speech. Computerised tomography revealed diffuse low radio-density confined to the white matter, and enlarged ventricles. Cerebral biopsy confirmed the diagnosis. The histopathological and electron microscopic features are essentially the same as those described in previous reports. We note the presence of dense clumps of material within some of the Rosenthal fibres, and can find no reference in the literature describing this. Sural nerve biopsy was normal.
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- ALEXANDER W. S. Progressive fibrinoid degeneration of fibrillary astrocytes associated with mental retardation in a hydrocephalic infant. Brain. 1949 Sep;72(3):373-81, 3 pl. doi: 10.1093/brain/72.3.373. [DOI] [PubMed] [Google Scholar]
- FRIEDE R. L. ALEXANDER'S DISEASE. Arch Neurol. 1964 Oct;11:414–422. doi: 10.1001/archneur.1964.00460220076010. [DOI] [PubMed] [Google Scholar]
- Herndon R. M., Rubinstein L. J., Freeman J. M., Mathieson G. Light and electron microscopic observations on Rosenthal fibers in Alexander's disease and in multiple sclerosis. J Neuropathol Exp Neurol. 1970 Oct;29(4):524–551. doi: 10.1097/00005072-197010000-00002. [DOI] [PubMed] [Google Scholar]
- Russo L. S., Jr, Aron A., Anderson P. J. Alexander's disease: a report and reappraisal. Neurology. 1976 Jul;26(7):607–614. doi: 10.1212/wnl.26.7.607. [DOI] [PubMed] [Google Scholar]
- Silverman L., Glick D. Studies in histochemistry. LXXXV. Histochemical demonstration of protein with bromsulfalein. J Histochem Cytochem. 1966 May;14(5):425–426. doi: 10.1177/14.5.425. [DOI] [PubMed] [Google Scholar]