title: Progressive multifocal leukoencephalopathy in a patient with Good's syndrome.
abstract: Good's syndrome (GS) is an immunodeficiency characterised by thymoma, hypogammaglobulinemia and impaired T-cell function. The clinical symptoms are recurrent or chronic infections from common or opportunistic pathogens and diarrhoea. Encephalitis is rare, mostly associated to cytomegalovirus. We present a 65-year-old woman who developed blindness, motor deficits and cognitive changes over a 4-month period. MRI of the brain showed symmetric subcortical white matter changes in the occipital lobes, first thought to correspond to posterior reversible encephalopathy syndrome. A thymoma was found and operated. The patient had no B cells, low immunoglobulins and an inverted CD4/CD8 ratio. GS was diagnosed. In the cerbrospinal fluid >1 million JC virus copies/mL were found and a repeat MRI now showed a picture compatible with progressive multifocal leucoencephalopathy (PML). Her disease had a fatal outcome. The present case is the second reported association between GS and PML.
authors.affiliation: Karolinska University Hospital, Stockholm, Sweden. olafur.sveinsson@karolinska.se
authors.forename: Olafur
authors.initials: O
authors.lastname: Sveinsson
authors.forename: Henrik
authors.initials: H
authors.lastname: Matell
authors.forename: Lars
authors.initials: L
authors.lastname: Herrman
mesh_terms: D000361:Agammaglobulinemia / Q000150:complications*; D000368:Aged; D001766:Blindness / Q000209:etiology; D003072:Cognition Disorders / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007968:Leukoencephalopathy, Progressive Multifocal / Q000150:complications*; D011596:Psychomotor Disorders / Q000209:etiology; D013945:Thymoma / Q000150:complications*; D013953:Thymus Neoplasms / Q000150:complications*
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