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[Alexander's disease (author's transl)]

H Gerlach, H Feist, H J Holzhausen

    Zentralblatt Fur Allgemeine Pathologie U. Pathologische Anatomie
    |January 1, 1982
    PubMed
    Summary
    This summary is machine-generated.

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    This case report details Alexander's disease in a young boy, highlighting progressive neurological decline, hydrocephalus requiring a shunt, and characteristic microscopic findings of demyelination and Rosenthal fibers.

    Area of Science:

    • Neurology
    • Pediatric Neurology
    • Neuroscience

    Background:

    • Alexander's disease is a rare, progressive, demyelinating leukoencephalopathy.
    • It is characterized by the presence of Rosenthal fibers in the white matter.
    • Early infantile forms typically present with severe neurological impairment.

    Observation:

    • A case of Alexander's disease in a 4-year-old boy is presented.
    • The disease onset was at 2 months with progressive developmental retardation.
    • Hydrocephalus necessitated a ventriculo-peritoneal shunt at 13 months.

    Findings:

    • Microscopic examination revealed significant demyelination.
    • Numerous Rosenthal fibers were identified in the brain tissue.
    • Cerebral dysfunction led to the patient's death.

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    Implications:

    • This case contributes to understanding the clinical course and morphological features of Alexander's disease.
    • Comparing findings with literature aids in characterizing early infantile presentations.
    • Highlights the severe impact of Alexander's disease on neurological development.