TY - JOUR
T1 - Progressive multifocal leukoencephalopathy in a patient with Franklin disease and hypogammaglobulinemia.
AU - Tasca, Giorgio
AU - Iorio, Raffaele
AU - Basile, Umberto
AU - Lauriola, Libero
AU - Tartaglione, Raffaele
AU - Mirabella, Massimiliano
AU - Ricci, Enzo
AU - Sabatelli, Mario
PY - 2009
Y1 - 2009
N2 - We report an association between histologically confirmed progressive multifocal leukoencephalopathy (PML) and an extremely rare humoral immunodeficiency disease, Franklin disease. In our patient, clinical presentation has been typical and prompted us, together with radiological findings, to perform a brain biopsy to confirm the diagnosis even if there was no evidence of any other risk factor except hypogammaglobulinemia. We suggest that PML should be suspected in patients in whom immunosuppression is not obvious (i.e. not only in the setting of HIV infection or disseminated end-stage lymphomas) and involves defects in humoral immunity.
AB - We report an association between histologically confirmed progressive multifocal leukoencephalopathy (PML) and an extremely rare humoral immunodeficiency disease, Franklin disease. In our patient, clinical presentation has been typical and prompted us, together with radiological findings, to perform a brain biopsy to confirm the diagnosis even if there was no evidence of any other risk factor except hypogammaglobulinemia. We suggest that PML should be suspected in patients in whom immunosuppression is not obvious (i.e. not only in the setting of HIV infection or disseminated end-stage lymphomas) and involves defects in humoral immunity.
KW - Brain biopsy
KW - PML
KW - Brain biopsy
KW - PML
UR - http://hdl.handle.net/10807/3501
U2 - 10.1016/j.jns.2009.04.035
DO - 10.1016/j.jns.2009.04.035
M3 - Article
SN - 0022-510X
VL - 284
SP - 203
EP - 204
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
ER -