Progressive multifocal leukoencephalopathy developed in incomplete Heerfordt syndrome, a rare manifestation of sarcoidosis, without steroid therapy responding to cidofovir.
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.
Yagi
Takuya
T
Department of Neurology, Keio University School of Medicine, Tokyo, Japan. takuyagi@z7.keio.jp
Hattori
Hidenori
H
Ohira
Masayuki
M
Nakamichi
Kazuo
K
Takayama-Ito
Mutsuyo
M
Saijo
Masayuki
M
Shimizu
Toshihiko
T
Ito
Daisuke
D
Takahashi
Kazushi
K
Suzuki
Norihiro
N
D000328:Adult; D000998:Antiviral Agents / Q000627:therapeutic use*; D000077404:Cidofovir; D003596:Cytosine / Q000031:analogs & derivatives* / Q000627:therapeutic use; D006801:Humans; D007968:Leukoencephalopathy, Progressive Multifocal / Q000000981:diagnostic imaging / Q000209:etiology*; D008297:Male; D063065:Organophosphonates / Q000627:therapeutic use*; D010306:Parotid Gland / Q000000981:diagnostic imaging; D011859:Radiography; D012507:Sarcoidosis / Q000000981:diagnostic imaging / Q000188:drug therapy*; D016896:Treatment Outcome; D014608:Uveoparotid Fever / Q000150:complications* / Q000000981:diagnostic imaging
D000998:Antiviral Agents; D063065:Organophosphonates; D003596:Cytosine; D000077404:Cidofovir