PMID- 31807988
OWN - NLM
STAT- MEDLINE
DCOM- 20210706
LR  - 20210706
IS  - 1538-2443 (Electronic)
IS  - 1355-0284 (Linking)
VI  - 26
IP  - 3
DP  - 2020 Jun
TI  - Epilepsia partialis continua revealing idelalisib-associated PML-IRIS: clinical 
      and pathological features.
PG  - 437-441
LID - 10.1007/s13365-019-00821-3 [doi]
AB  - Idelalisib, a selective phosphatidylinositol 3-kinase delta (PI3Kdelta) inhibitor, is 
      a newly approved second-line drug for patients with chronic lymphocytic leukemia. 
      Recent clinical trials have suggested a possible association between idelalisib 
      treatment and development of progressive multifocal leukoencephalopathy (PML) due 
      to John Cunningham virus (JCV) reactivation. Nevertheless, clinical course and 
      radiological and pathological features of idelalisib-induced PML still need to be 
      clarified. We provide here the first clinicopathological description of 
      idelalisib-associated PML in a patient who developed epilepsia partialis continua 
      (EPC) as the first manifestation of the disease. Since EPC could present without 
      electroencephalogram alterations, it is crucial to recognize the clinical 
      features of this epileptic condition. EPC is characterized by the presence of 
      repetitive, irregular, clonic jerking, often associated with hemiparesis and 
      involvement of distal rather than proximal muscle groups. Moreover, we highlight 
      the importance of brain biopsy in selected cases when there is a high clinical 
      suspicion of PML, despite negative JCV testing in the cerebrospinal fluid. The 
      pathological finding of prominent inflammatory infiltrate observed here was 
      consistent with a diagnosis of immune reconstitution inflammatory syndrome 
      (IRIS). IRIS is often associated with PML as a paradoxical worsening of clinical 
      symptoms due to an overreacting immune response, in the context of previous 
      immunosuppression. The unprecedented pathologic observation of IRIS in 
      idelalisib-associated PML provides further insights into the pathogenesis of this 
      rare neurological side effect.
FAU - Vogrig, Alberto
AU  - Vogrig A
AUID- ORCID: 0000-0002-3652-7061
AD  - Clinical Neurology Unit, Santa Maria della Misericordia University Hospital, 
      Piazzale Santa Maria della Misericordia, 15, 33010, Udine, Italy. 
      alberto.vogrig@gmail.com.
FAU - Gigli, Gian Luigi
AU  - Gigli GL
AD  - Clinical Neurology Unit, Santa Maria della Misericordia University Hospital, 
      Piazzale Santa Maria della Misericordia, 15, 33010, Udine, Italy.
AD  - Neurology Unit, Department of Medicine, University of Udine Medical School, 
      Udine, Italy.
AD  - Department of Mathematics, Informatics and Physics (DMIF), University of Udine, 
      Udine, Italy.
FAU - Nilo, Annacarmen
AU  - Nilo A
AD  - Neurology Unit, Department of Medicine, University of Udine Medical School, 
      Udine, Italy.
FAU - Pessa, Maria Elena
AU  - Pessa ME
AD  - Neurology Unit, Department of Medicine, University of Udine Medical School, 
      Udine, Italy.
FAU - Volpetti, Stefano
AU  - Volpetti S
AD  - Hematology and Cellular Therapy Unit, University Hospital Santa Maria della 
      Misericordia, Udine, Italy.
FAU - Pegolo, Enrico
AU  - Pegolo E
AD  - Institute of Pathology, Department of Medicine, University of Udine Medical 
      School, Udine, Italy.
FAU - Valente, Mariarosaria
AU  - Valente M
AD  - Clinical Neurology Unit, Santa Maria della Misericordia University Hospital, 
      Piazzale Santa Maria della Misericordia, 15, 33010, Udine, Italy.
AD  - Neurology Unit, Department of Medicine, University of Udine Medical School, 
      Udine, Italy.
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20191205
PL  - United States
TA  - J Neurovirol
JT  - Journal of neurovirology
JID - 9508123
RN  - 0 (Antineoplastic Agents)
RN  - 0 (Purines)
RN  - 0 (Quinazolinones)
RN  - YG57I8T5M0 (idelalisib)
SB  - IM
MH  - Antineoplastic Agents/administration & dosage/*adverse effects
MH  - Epilepsia Partialis Continua/*diagnosis/pathology/virology
MH  - Female
MH  - Humans
MH  - Immune Reconstitution Inflammatory Syndrome/*diagnosis/pathology/virology
MH  - JC Virus/*drug effects/growth & development/pathogenicity
MH  - Leukemia, Lymphocytic, Chronic, B-Cell/drug therapy/pathology
MH  - Leukoencephalopathy, Progressive Multifocal/*diagnosis/pathology/virology
MH  - Middle Aged
MH  - Purines/administration & dosage/*adverse effects
MH  - Quinazolinones/administration & dosage/*adverse effects
MH  - Virus Activation/drug effects
OTO - NOTNLM
OT  - Epilepsia partialis continua
OT  - Epilepsy
OT  - Idelalisib
OT  - Immune reconstitution inflammatory syndrome
OT  - Pathology
OT  - Progressive multifocal leukoencephalopathy
EDAT- 2019/12/07 06:00
MHDA- 2021/07/07 06:00
CRDT- 2019/12/07 06:00
PHST- 2019/01/30 00:00 [received]
PHST- 2019/11/21 00:00 [accepted]
PHST- 2019/11/12 00:00 [revised]
PHST- 2019/12/07 06:00 [pubmed]
PHST- 2021/07/07 06:00 [medline]
PHST- 2019/12/07 06:00 [entrez]
AID - 10.1007/s13365-019-00821-3 [pii]
AID - 10.1007/s13365-019-00821-3 [doi]
PST - ppublish
SO  - J Neurovirol. 2020 Jun;26(3):437-441. doi: 10.1007/s13365-019-00821-3. Epub 2019 
      Dec 5.