K. Hasegawa et al., A CLINICOPATHOLOGICAL STUDY OF ADULT HISTIOCYTOSIS-X INVOLVING THE BRAIN, Journal of Neurology, Neurosurgery and Psychiatry, 56(9), 1993, pp. 1008-1012
Adult histiocytosis X involving the CNS caused progressive spastic par
aparesis. The diagnosis was made by immunoreactive anti-S100 protein a
ntibody staining and from the presence of Birbeck granules in biopsy s
pecimens of skin lesions. Neuropathological examination showed massive
proliferation and infiltration of S-100 containing histiocyte-like ce
lls and reactive astrocytes throughout the CNS.