Yagi T, Hattori H, Ohira M, Nakamichi K, Takayama-Ito M, Saijo M, Shimizu T, Ito D, Takahashi K, Suzuki N
Progressive multifocal leukoencephalopathy developed in incomplete Heerfordt syndrome, a rare manifestation of sarcoidosis, without steroid therapy responding to cidofovir. [JOURNAL ARTICLE]
Clin Neurol Neurosurg 2009 Nov 3.
Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system caused by the JC virus; the mortality rate is high and it is usually refractory to treatment. In non-HIV patients, PML occurs as a late consequence of hematologic malignancies or during prolonged immunosuppression for transplantation or autoimmune disease. We describe a 34-year-old PML patient with incomplete Heerfordt syndrome, a rare type of sarcoidosis, who had not received any immunosuppressants, including steroids, at the onset and who was clinically and radiologically responsive to the antiviral drug cidofovir.
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