MYELINOCLASTIC DIFFUSE SCLEROSIS (SCHILDERS-DISEASE) - REPORT OF A CASE AND REVIEW OF THE LITERATURE

The clinical, neuroimaging, and neuropathologic features of Schilder's disease in a 17-year-old girl are presented and compared to 11 well-d ocumented cases reported since 1912. The evolution of knowledge about Schilder's disease and the confusion in nomenclature are reviewed. Sig ns 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 ede ma, the irregular and incomplete ring enhancement, the discrepancy bet ween size of the lesions and the associated mass effect, and the absen ce of other lesions elsewhere in the brain may help differentiate Schi lder's disease from neoplasm, infection, and other demyelinating lesio ns. Although frozen sections of these lesions are often interpreted as astrocytoma, the inflammatory, primarily histiocytic, nature of Schil der's disease is more easily recognized in paraffin-embedded material. Unique features of this case include multiple unilateral lesions and the cystlike 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 fo r reexamination of the restrictive 1985 criteria of Poser for the diag nosis of Schilder's disease.