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Rosenthal fibers in Alexander disease (infantile type) (electron micrograph)

Demonstration of immunoreactivity of Rosenthal fibers for glial fibrillary acidic protein (GFAP) in biopsied cerebrum. This girl’s first symptom was delayed motor development at 23 months, followed by focal seizures. Brain biopsy at age 2.5 years established diagnosis; she died at age 7. (A) Portions of 2 electron-dense Rosenthal fibers decorated with gold particles lie near 2 myelinated axons. (B) Many gold particles are evident over Rosenthal fiber and astrocytic glial filaments. Glutaraldehyde-fixed but un-osmicated, plastic-embedded tissue labeled by postembedding immunogold procedure. (x18,000 for A, x45,000 for B) (Reprinted in modified form with permission. Johnson AB. Alexander disease. In: Moser HW, editor. Neurodystrophies and neurolipidoses. Handbook of clinical neurology. Vol. 66, revised series vol. 22. Amsterdam: Elsevier, 1996:701-10.)