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Progressive multifocal leukoencephalopathy developed in incomplete Heerfordt syndrome, a rare manifestation of sarcoidosis, without steroid therapy responding to cidofovir.

Abstract
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.
AuthorsTakuya Yagi, Hidenori Hattori, Masayuki Ohira, Kazuo Nakamichi, Mutsuyo Takayama-Ito, Masayuki Saijo, Toshihiko Shimizu, Daisuke Ito, Kazushi Takahashi, Norihiro Suzuki
JournalClinical neurology and neurosurgery (Clin Neurol Neurosurg) Vol. 112 Issue 2 Pg. 153-6 (Feb 2010) ISSN: 1872-6968 [Electronic] Netherlands
PMID19892460 (Publication Type: Case Reports, Journal Article)
Copyright2009 Elsevier B.V. All rights reserved.
Chemical References
  • Antiviral Agents
  • Organophosphonates
  • Cytosine
  • Cidofovir
Topics
  • Adult
  • Antiviral Agents (therapeutic use)
  • Cidofovir
  • Cytosine (analogs & derivatives, therapeutic use)
  • Humans
  • Leukoencephalopathy, Progressive Multifocal (diagnostic imaging, etiology)
  • Male
  • Organophosphonates (therapeutic use)
  • Parotid Gland (diagnostic imaging)
  • Radiography
  • Sarcoidosis (diagnostic imaging, drug therapy)
  • Treatment Outcome
  • Uveoparotid Fever (complications, diagnostic imaging)

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