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Diffuse Rosenthal fiber formation in adults. A case report

N Rizzuto, G Ferrari, A Piscioli

    Acta Neuropathologica
    |January 1, 1980
    PubMed
    Summary

    A rare case of Alexander's disease in a young woman presented with tetraparesis and respiratory failure. Neuropathology confirmed Rosenthal fibers and demyelination, suggesting a link to this neurodegenerative disorder.

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    Area of Science:

    • Neuroscience
    • Neuropathology
    • Genetics

    Background:

    • Alexander's disease is a rare, progressive, often fatal, demyelinating neurological disorder.
    • It is characterized by the presence of Rosenthal fibers in the white matter of the brain.

    Observation:

    • A 20-year-old woman presented with acute tetraparesis and respiratory failure, requiring mechanical ventilation.
    • She had a history of residual left lower limb paresis since age 14.
    • Despite intensive care, she experienced bulbar paralysis and died 3 months later.

    Findings:

    • Neuropathological examination revealed widespread Rosenthal fibers in both gray and white matter, with a perivascular and subpial distribution.
    • Mild demyelination was observed in the periventricular white matter and the hilum of the nucleus dentatus.
    • The medulla oblongata showed the highest density of Rosenthal fibers, extending deep into the brain parenchyma.

    Implications:

    • This case highlights the potential for Alexander's disease to manifest with severe neurological deficits and respiratory compromise in young adults.
    • The neuropathological findings support the diagnosis and provide insights into the distribution and severity of the disease process.
    • Further research into the genetic basis and pathogenesis of Alexander's disease is warranted to explore potential therapeutic strategies.

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