CASE REPORT Spinal cord progressive multifocal leukoencephalopathy detected premortem by MRI Roger Murayi 1 & James Schmitt 2 & John H. Woo 2 & Joseph R. Berger 1 Received: 5 December 2014 /Revised: 6 April 2015 /Accepted: 9 April 2015 /Published online: 3 July 2015 # Journal of NeuroVirology, Inc. 2015 Introduction Progressive multifocal leukoencephalopathy (PML) is a rare and often fatal demyelinating disease caused by JC virus, a ubiquitous polyomavirus that seroepidemiological studies re- veal is present in more than 50 % of the adult population (Berger 2014). The rarity of PML indicates that multiple bar- riers almost certainly exist to its development. Virtually, all predisposing illnesses are associated with an impairment of cell mediated immunity. From the time of its description in 1958 to the onset of the AIDS pandemic, underlying lympho- proliferative disorders, typically B cell diseases, were the most common predisposing disorders for PML (Brooks and Walker 1984). Currently, HIV is the most common predisposing dis- order. Prior to the availability of antiretroviral therapies, as many as one in 20 HIV-infected individuals died with PML (Berger et al. 1987) and even in the era of effective antiretro- viral therapy, 1.0 % of AIDS deaths are due to PML (Christensen et al. 2010). The gold standard for diagnosis is brain biopsy in which the characteristic histopathological triad of demyelination, enlarged bizarre astrocytes, and enlarged oligodendroglial nuclei can be found, coupled with the dem- onstration of the presence of JC virus by immunocytochemis- try or electron microscopy. However, the diagnosis is more often established on clinical criteria which include an appropriate clinical picture, typical brain MRI findings, and detection of JC virus (JCV) DNA in the CSF by polymerase chain reaction (Berger et al. 2013). Magnetic resonance imaging in PML typically shows mul- tiple foci of demyelination in the supratentorial white matter, most commonly found in the subcortical parietal lobe. Sub- cortical lesions are the most common though periventricular lesions can occur as well and are often similar in appearance to multiple sclerosis. Less commonly, infratentorial white matter lesions can be found in the posterior fossa typically involving the middle cerebellar peduncle and cerebellar white matter. Both posterior fossa and supratentorial lesions are typically hyperintense on fluid-attenuated inversion recovery (FLAIR) imaging and do not enhance (Berger et al. 1987). Involvement of the spinal cord in PML is exceedingly rare, especially on imaging. To our knowledge, there has never been a report of spinal cord lesions in PML found on premortem imaging (Berger et al. 1987). In only a handful of cases, autopsies have revealed typical PML lesions in the spinal cord on histology (Yousry et al. 2012). We present a patient with progressively worsening hemiparesis, ataxia, and diplopia, due to PML associated with idiopathic CD4 lymphopenia resulting in pan lymphocytopenia. Within 6 weeks of presentation, she exhibited extensive infratentorial lesions and a lesion in the cervical spinal cord. To the best of our knowledge, this is the first documented ex- ample of PML demonstrated in the spinal cord premortem. Case report A 58-year-old female with history of hypothyroidism and hy- perlipidemia presented to an outside hospital with sudden on- set of right-sided weakness. She fell while playing golf and had trouble articulating words. At the outside hospital, a brain * Joseph R. Berger jrbneuro@uky.edu 1 Department of Neurology, Perelman School of Medicine, University of Pennsylvania, 3400 Spruce St, 3 W Gates, Philadelphia, PA 19104, USA 2 Department of Radiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA J. Neurovirol. (2015) 21:688–690 DOI 10.1007/s13365-015-0342-2