Abstract
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.
| Original language | English |
|---|---|
| Pages (from-to) | 203-204 |
| Number of pages | 2 |
| Journal | Journal of the Neurological Sciences |
| Volume | 284 |
| Issue number | 1-2 |
| DOIs | |
| Publication status | Published - Sep 15 2009 |
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Keywords
- Franklin disease
- Hypogammaglobulinemia
- Progressive multifocal leukoencephalopathy
ASJC Scopus subject areas
- Clinical Neurology
- Neurology
Cite this
Progressive multifocal leukoencephalopathy in a patient with Franklin disease and hypogammaglobulinemia. / Tasca, Giorgio; Iorio, Raffaele; Basile, Umberto; Lauriola, Libero; Tartaglione, Raffaele; Mirabella, Massimiliano; Ricci, Enzo; Sabatelli, Mario.
In: Journal of the Neurological Sciences, Vol. 284, No. 1-2, 15.09.2009, p. 203-204.Research output: Contribution to journal › Article
}
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 - 2009/9/15
Y1 - 2009/9/15
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 - Franklin disease
KW - Hypogammaglobulinemia
KW - Progressive multifocal leukoencephalopathy
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UR - http://www.scopus.com/inward/citedby.url?scp=67849097193&partnerID=8YFLogxK
U2 - 10.1016/j.jns.2009.04.035
DO - 10.1016/j.jns.2009.04.035
M3 - Article
VL - 284
SP - 203
EP - 204
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
SN - 0022-510X
IS - 1-2
ER -