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Figure 1 Her illness began as a chronic
fatigue-like syndrome and progressed to a more severe
cognitive disorder. Conventional neurological
examination was, nevertheless, essentially normal. Peri-ventricular
white matter changes were detected using MRI. The pink
color of the biopsy is an indication of the absence of
inflammatory cells, (lymphocytes and macrophages) that
stain blue.
Figure 2 The pale staining material in the
vacuoles is lipid. The striking irregularly shaped
dark staining materials (inclusions) do not
correspond to any normal cellular structures. The
long fibers are filaments, typical of glial cells.
The round structure at the bottom of the photo is an
axon surrounded by a myelin sheath. Mitochondria,
seen elsewhere in the biopsy showed degenerative
changes.
Figure 3 His illness began as an
attention deficit, behavioral problem. Even when
gross abnormalities were detected on MRI, there
were no clinical signs of motor, sensory or
autonomic nervous system dysfunction. The
markedly vacuolated appearance, without signs of
inflammation, is reminiscent of the changes seen
in diseases attributed to prions, such as mad
cow disease. The child’s mother had previously
been shown to be infected. She continues to have
repeated bouts of a severe personality disorder.
Figure 4 Viral particles were not
seen.
Figure 5 The myelin sheaths show extensive bleeding. The child showed a clinical response to ganciclovir, but subsequently died.
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