The clinical, neuroimaging, and neuropathologic features of
Schilder's disease in a 17-year-old girl are presented and compared to 11 well-documented cases reported since 1912. The evolution of knowledge about
Schilder's disease and the
confusion in nomenclature are reviewed. Signs and symptoms in this case and others reported in the literature are nonspecific and may mimic mass lesions. Neuroimaging studies also may mimic
brain tumor or
abscess; however, the absence of significant
edema, the irregular and incomplete ring enhancement, the discrepancy between size of the lesions and the associated mass effect, and the absence of other lesions elsewhere in the brain may help differentiate
Schilder's disease from
neoplasm,
infection, and other demyelinating lesions. Although frozen sections of these lesions are often interpreted as
astrocytoma, the inflammatory, primarily histiocytic, nature of
Schilder's disease is more easily recognized in
paraffin-embedded material. Unique features of this case include multiple unilateral lesions and the
cyst-like degeneration present in both lesions. Multiple lesions in
Schilder's disease are characteristically bilateral. The examination of aspirated fluid is the first such report in
Schilder's disease. The limitation of multiple lesions in our case to one hemisphere calls for reexamination of the restrictive 1985 criteria of Poser for the diagnosis of
Schilder's disease.