title: Progressive multifocal leukoencephalopathy complicating untreated chronic lymphatic leukemia: case report and review of the literature.
abstract: A 58-year old female with a four-year history of previously untreated CLL at Binet stage A complained about word finding problems, impaired vision, and gait unsteadiness. Concerning her CLL she was asymptomatic and had never required any specific treatment. Her neurological examination disclosed cognitive alterations, homonyme hemianopia to the right, aphasia, and mild right-sided hemiparesis. Cerebral MRI showed a hyperintense lesion on T2 weighted images without contrast enhancement. CSF examination revealed normal findings, including CSF protein, cell count, cytology and PCR-analysis was negative for the presence of JC virus DNA. On follow-up MRI, performed 2 weeks later, the T2 lesion was further enlarging. Subsequent stereotactic brain biopsy was diagnostic for PML revealing abnormal oligodendrocytes staining positive against antibodies specific for simian vacuolating virus 40. In addition, repeated CSF analyses for JC-Virus DNA in the course of the disease became positive. After confirmation of diagnosis treatment with mirtazapine (30 mg/d) and mefloquine (250 mg/d) was initiated. Rapid clinical progression correlated to further worsening on MRI. Therefore this treatment was terminated after 16 days and the regime was changed to a five-day courses of cytarabine (2 mg/kg/d) combined with intrathecal administration of liposomal cytarabine (50 mg). Due to further clinical progression with global aphasia, blindness and severe right-sided hemiparesia, medication was stopped. The Patient died three and a half months after onset of symptoms.
authors.affiliation: Clinical Department of Neurology, Innsbruck Medical University, Anichstr. 35, 6020 Innsbruck, Austria. Electronic address: franziska.dipauli@i-med.ac.at.
authors.forename: Franziska
authors.initials: F
authors.lastname: Di Pauli
authors.affiliation: Clinical Department of Neurology, Innsbruck Medical University, Anichstr. 35, 6020 Innsbruck, Austria.
authors.forename: Thomas
authors.initials: T
authors.lastname: Berger
authors.affiliation: Division of Haematology & Oncology, Regional Hospital Lienz, Emanuel-von-Hibler-Str 5, 9900 Lienz, Austria.
authors.forename: Alois
authors.initials: A
authors.lastname: Walder
authors.affiliation: Institute of Pathology, Innsbruck Medical University, Anichstr. 35, 6020 Innsbruck, Austria.
authors.forename: Hans
authors.initials: H
authors.lastname: Maier
authors.affiliation: Department of Radiology, Innsbruck Medical University, Anichstr. 35, 6020 Innsbruck, Austria.
authors.forename: Paul
authors.initials: P
authors.lastname: Rhomberg
authors.affiliation: Department of Nuclear Medicine, Innsbruck Medical University, Anichstr. 35, 6020 Innsbruck, Austria.
authors.forename: Christian
authors.initials: C
authors.lastname: Uprimny
authors.affiliation: Division of Haematology & Oncology, Innsbruck Medical University, Anichstr. 35, 6020 Innsbruck, Austria.
authors.forename: Michael
authors.initials: M
authors.lastname: Steurer
authors.affiliation: Clinical Department of Neurology, Innsbruck Medical University, Anichstr. 35, 6020 Innsbruck, Austria.
authors.forename: Guenther
authors.initials: G
authors.lastname: Stockhammer
mesh_terms: D003561:Cytarabine / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D006801:Humans; D007577:JC Virus / Q000472:pathogenicity*; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications*; D007968:Leukoencephalopathy, Progressive Multifocal / Q000150:complications / Q000188:drug therapy* / Q000821:virology; D015767:Mefloquine / Q000627:therapeutic use; D008803:Mianserin / Q000031:analogs & derivatives / Q000627:therapeutic use; D008875:Middle Aged; D000078785:Mirtazapine; D017211:Treatment Failure
chemical_list: D003561:Cytarabine; D008803:Mianserin; D000078785:Mirtazapine; D015767:Mefloquine
keywords: Chronic lymphatic leukemia; JC Virus; Progressive multifocal leukoencephalopathy