Progressive multifocal leukoencephalopathy in a patient with Franklin disease and hypogammaglobulinemia

Giorgio Tasca; Raffaele Iorio; Umberto Basile; Libero Lauriola; Raffaele Tartaglione; Massimiliano Mirabella; Enzo Ricci; Mario Sabatelli

doi:10.1016/j.jns.2009.04.035

Progressive multifocal leukoencephalopathy in a patient with Franklin disease and hypogammaglobulinemia

Giorgio Tasca, Raffaele Iorio, Umberto Basile, Libero Lauriola, Raffaele Tartaglione, Massimiliano Mirabella, Enzo Ricci, Mario Sabatelli

Research output: Contribution to journal › Article › peer-review

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	https://doi.org/10.1016/j.jns.2009.04.035
Publication status	Published - Sep 15 2009

Keywords

Franklin disease
Hypogammaglobulinemia
Progressive multifocal leukoencephalopathy

ASJC Scopus subject areas

Clinical Neurology
Neurology

Access to Document

10.1016/j.jns.2009.04.035

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 › peer-review

@article{cc09e26d19a74de3af84d47d4dea20ff,

title = "Progressive multifocal leukoencephalopathy in a patient with Franklin disease and hypogammaglobulinemia",

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.",

keywords = "Franklin disease, Hypogammaglobulinemia, Progressive multifocal leukoencephalopathy",

author = "Giorgio Tasca and Raffaele Iorio and Umberto Basile and Libero Lauriola and Raffaele Tartaglione and Massimiliano Mirabella and Enzo Ricci and Mario Sabatelli",

year = "2009",

month = sep,

day = "15",

doi = "10.1016/j.jns.2009.04.035",

language = "English",

volume = "284",

pages = "203--204",

journal = "Journal of the Neurological Sciences",

issn = "0022-510X",

publisher = "Elsevier",

number = "1-2",

}

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

UR - http://www.scopus.com/inward/record.url?scp=67849097193&partnerID=8YFLogxK

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

C2 - 19428026

AN - SCOPUS:67849097193

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 -

Progressive multifocal leukoencephalopathy in a patient with Franklin disease and hypogammaglobulinemia

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this