ECTRIMS eLearning

Progressive multifocal leukoencephalopathy associated with natalizumab in multiple sclerosis. Cidofovir and maraviroc therapeutic experience
Author(s): ,
O Belchí
Affiliations:
Neuroimmunology and Multiple Sclerosis Unit (UNIEM), Department of Neurology, Dr. Josep Trueta University Hospital;Neurodegeneration and Neuroinflammation Research Group, Girona Biomedical Research Institute (IDIBGI)
,
H Perkal
Affiliations:
Neurodegeneration and Neuroinflammation Research Group, Girona Biomedical Research Institute (IDIBGI)
,
B Beltran
Affiliations:
Imaging Research Unit, Diagnostic Imaging Institiute (IDI), Dr. Josep Trueta University Hospital, Girona, Spain
,
G Laguillo
Affiliations:
Imaging Research Unit, Diagnostic Imaging Institiute (IDI), Dr. Josep Trueta University Hospital, Girona, Spain
,
A Quiles
Affiliations:
Imaging Research Unit, Diagnostic Imaging Institiute (IDI), Dr. Josep Trueta University Hospital, Girona, Spain
,
R Robles-Cedeño
Affiliations:
Neuroimmunology and Multiple Sclerosis Unit (UNIEM), Department of Neurology, Dr. Josep Trueta University Hospital;Neurodegeneration and Neuroinflammation Research Group, Girona Biomedical Research Institute (IDIBGI)
L Ramió-Torrentà
Affiliations:
Neuroimmunology and Multiple Sclerosis Unit (UNIEM), Department of Neurology, Dr. Josep Trueta University Hospital;Neurodegeneration and Neuroinflammation Research Group, Girona Biomedical Research Institute (IDIBGI)
ECTRIMS Learn. Muñoz-San Martín M. 09/14/16; 145620; EP1525
Ms. María Muñoz-San Martín
Ms. María Muñoz-San Martín
Contributions
Abstract

Abstract: EP1525

Type: ePoster

Abstract Category: Therapy - disease modifying - Risk management for disease modifying treatments

Introduction: Progressive multifocal leukoencephalopathy (PML) is a rare and serious complication associated with natalizumab treatment in relapsing-remitting multiple sclerosis patients, resulting in a 25% mortality and 70% of survivors with moderate to severe sequelae. 638 cases have been reported worldwide as of March 2016.

Cases reports: We describe 2 PML cases that have been diagnosed and managed in our hospital.

1) A 56-year-old woman treated with interferon for 8 years required a change in management to natalizumab and, 50 months later, with anti-JCV antibodies in serum and an index of 2,566 (PML risk of 1/118), developed a PML. She was initially treated with 3 sessions of plasma exchange (PLEX) that had to be discontinued due to an anaphylactic shock, followed by mirtazapine and mefloquine. A month later, an immune reconstitution inflammatory syndrome (IRIS) was detected and this was managed with steroids. Finally, she received cidofovir and the number of copies of JCV in cerebrospinal fluid (CSF) assessed by polymerase chain reaction (PCR) was undetected. After that, she experienced a small clinical improvement (EDSS 7.5).

2) A 35-year-old woman was treated with interferon for 2 years until natalizumab was required. After 47 months, with seropositivity and an index of 3.88 (PML risk of 1/118), she developed a PML-IRIS complex. The patient was treated with steroids, 5 sessions of PLEX, mirtazapine and mefloquine. After clinical and radiological signs of disease progression, which coincided with the higher peak of copies of JCV in CSF, maraviroc and cidofovir was given. Consequently, a significant decrease of JCV in CSF was noted together with a clinical improvement. Following this, she started to have seizures but these were controlled by valproate and levetiracetam. At her last clinic visit she had an EDSS score of 5.5.

Discussion: There is still little evidence on the best therapeutic management for PML. In our experience, the treatment with cidofovir and maraviroc was associated with a better outcome for our patients.

Disclosure:

O. Belchí: nothing to disclose

R. Robles-Cedeño: nothing to disclose

H. Perkal: nothing to disclose

B. Beltrán: nothing to disclose

G. Laguillo: nothing to disclose

A. Quiles: nothing to disclose

Ll. Ramió-Torrentà has received speaking honoraria and travel expenses for scientific meetings and has participated in advisory boards in the past years with Bayer Schering Pharma, Biogen, EMD Merck Serono, Sanofi Genzyme, Novartis, Sanofi-Aventis, Teva Phramaceuticals, Almirall and Roche.

Abstract: EP1525

Type: ePoster

Abstract Category: Therapy - disease modifying - Risk management for disease modifying treatments

Introduction: Progressive multifocal leukoencephalopathy (PML) is a rare and serious complication associated with natalizumab treatment in relapsing-remitting multiple sclerosis patients, resulting in a 25% mortality and 70% of survivors with moderate to severe sequelae. 638 cases have been reported worldwide as of March 2016.

Cases reports: We describe 2 PML cases that have been diagnosed and managed in our hospital.

1) A 56-year-old woman treated with interferon for 8 years required a change in management to natalizumab and, 50 months later, with anti-JCV antibodies in serum and an index of 2,566 (PML risk of 1/118), developed a PML. She was initially treated with 3 sessions of plasma exchange (PLEX) that had to be discontinued due to an anaphylactic shock, followed by mirtazapine and mefloquine. A month later, an immune reconstitution inflammatory syndrome (IRIS) was detected and this was managed with steroids. Finally, she received cidofovir and the number of copies of JCV in cerebrospinal fluid (CSF) assessed by polymerase chain reaction (PCR) was undetected. After that, she experienced a small clinical improvement (EDSS 7.5).

2) A 35-year-old woman was treated with interferon for 2 years until natalizumab was required. After 47 months, with seropositivity and an index of 3.88 (PML risk of 1/118), she developed a PML-IRIS complex. The patient was treated with steroids, 5 sessions of PLEX, mirtazapine and mefloquine. After clinical and radiological signs of disease progression, which coincided with the higher peak of copies of JCV in CSF, maraviroc and cidofovir was given. Consequently, a significant decrease of JCV in CSF was noted together with a clinical improvement. Following this, she started to have seizures but these were controlled by valproate and levetiracetam. At her last clinic visit she had an EDSS score of 5.5.

Discussion: There is still little evidence on the best therapeutic management for PML. In our experience, the treatment with cidofovir and maraviroc was associated with a better outcome for our patients.

Disclosure:

O. Belchí: nothing to disclose

R. Robles-Cedeño: nothing to disclose

H. Perkal: nothing to disclose

B. Beltrán: nothing to disclose

G. Laguillo: nothing to disclose

A. Quiles: nothing to disclose

Ll. Ramió-Torrentà has received speaking honoraria and travel expenses for scientific meetings and has participated in advisory boards in the past years with Bayer Schering Pharma, Biogen, EMD Merck Serono, Sanofi Genzyme, Novartis, Sanofi-Aventis, Teva Phramaceuticals, Almirall and Roche.

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