Article
Clinical Neurology
Xiang Wang, Jinxiu Chen, Jing Gong, Ying Wu, Xiang-hao Liu
Summary: This case presents a rare occurrence of progressive multifocal leukoencephalopathy (PML) caused by JC virus infection in a previously healthy and immunocompetent patient. The patient showed disease stabilization after receiving a combination treatment, which may contribute to our understanding of virus infections during the coronavirus epidemic.
Article
Immunology
Anni Honkimaa, Joni Suppula, Olli Tynninen, Mika Saarela, Hanna Liimatainen, Pia Laine, Petri Auvinen, Eeva Auvinen
Summary: Progressive multifocal leukoencephalopathy (PML) is a severe neurological condition caused by reactivation of JC polyomavirus (JCPyV) in immunosuppression. Asymptomatic JCPyV persists in peripheral tissues. Upon reactivation, neurotropic rearrangements may emerge, and the virus gains access to the brain. To assess the mechanisms of PML pathogenesis, brain tissue material from PML patients was collected for small RNA sequencing. Upregulation of 8 microRNAs (miRNAs) in PML brain was validated using quantitative microRNA polymerase chain reaction (PCR). Bioinformatics tools were utilized to identify major associations of the upregulated miRNAs: neuroinflammation and blood-brain barrier disruption. The results indicate involvement of human miRNA regulation in PML pathogenesis.
JOURNAL OF INFECTIOUS DISEASES
(2023)
Article
Clinical Neurology
Giulia Berzero, Sabrina Basso, Luca Stoppini, Andrea Palermo, Anna Pichiecchio, Matteo Paoletti, Federica Lucev, Simonetta Gerevini, Andrea Rossi, Elisa Vegezzi, Luca Diamanti, Paola Bini, Matteo Gastaldi, Serena Delbue, Cesare Perotti, Elena Seminari, Maura Faraci, Mario Luppi, Fausto Baldanti, Marco Zecca, Enrico Marchioni, Patrizia Comoli
Summary: The study found that using JCPyV-specific T cell therapy to treat hematological patients with PML is safe and effective, with no treatment-related adverse events observed. Some patients experienced an increase in JCPyV-specific lymphocytes and a decrease in JCPyV viral load in cerebrospinal fluid. Six out of 9 patients achieved PML control, with 5 alive and in good clinical condition at their last follow-up.
ANNALS OF NEUROLOGY
(2021)
Article
Clinical Neurology
Gautier Breville, Igor J. Koralnik, Patrice H. Lalive
Summary: Isolated brainstem lesions at disease onset are extremely rare in PML cases, with most patients experiencing initial clinical worsening. However, some patients may achieve full or partial recovery despite the reserved prognosis due to the vital structures in the brainstem.
EUROPEAN JOURNAL OF NEUROLOGY
(2021)
Article
Virology
Nyater Ngouth, Maria Chiara Monaco, Lorenzo Walker, Sydney Corey, Ijeoma Ikpeama, Gary Fahle, Irene Cortese, Sanchita Das, Steven Jacobson
Summary: This study compares the droplet digital PCR (ddPCR) assay with the gold standard quantitative PCR (qPCR) for the quantification of JC viral loads in clinical samples. The results show a strong correlation between ddPCR and qPCR, with ddPCR demonstrating increased sensitivity. Additionally, a novel triplex ddPCR method for detecting JC viral DNA sequences is described.
Article
Clinical Neurology
Jennifer Meylor, Daniel Crespo Artunduaga, Michael Mendoza, Sam I. Hooshmand, Ahmed Z. Obeidat
Summary: Progressive multifocal leukoencephalopathy (PML) is a central nervous system infection caused by human polyomavirus 2, usually affecting immunocompromised patients. However, this report presents two cases of PML in older individuals with chronic kidney disease (CKD). CKD can lead to immune system dysfunction and potentially increase the risk of PML.
NEUROLOGICAL SCIENCES
(2023)
Article
Clinical Neurology
Marine Joly, Cecile Conte, Charles Cazanave, Vincent Le Moing, Pierre Tattevin, Pierre Delobel, Agnes Sommet, Guillaume Martin-Blondel
Summary: This study examined the epidemiology and prognosis of progressive multifocal leukoencephalopathy using a large cohort of patients in France. The findings revealed a stable incidence rate of the disease in France, with HIV infection being the main predisposing condition. Male patients had a higher risk of mortality compared to females, and patients with solid neoplasms had the worst prognosis.
Article
Clinical Neurology
Eli Hatchwell, Edward B. B. Smith, Shapour Jalilzadeh, Christopher D. D. Bruno, Yassine Taoufik, Houria Hendel-Chavez, Roland Liblau, David Brassat, Guillaume Martin-Blondel, Heinz Wiendl, Nicholas Schwab, Irene Cortese, Maria Chiara Monaco, Luisa Imberti, Ruggero Capra, Jorge R. R. Oksenberg, Jacques Gasnault, Bruno Stankoff, Todd A. A. Richmond, David M. M. Rancour, Igor J. J. Koralnik, Barbara A. A. Hanson, Eugene O. O. Major, Christina R. R. Chow, Peggy S. S. Eis
Summary: PML is a rare and dangerous brain disease caused by JC virus infection, with immunosuppressed patients being more susceptible. Research has identified four genetic variants in immune system genes that are associated with PML risk.
FRONTIERS IN NEUROLOGY
(2022)
Review
Neurosciences
Angela Rocchi, Ilker K. Sariyer, Joseph R. Berger
Summary: Since its definition 65 years ago, progressive multifocal leukoencephalopathy (PML) caused by the JC virus (JCV) continues to affect immunosuppressed patients, leading to demyelination, neurodegeneration, and death. Lack of an animal model and ineffectiveness of current antiviral agents pose major challenges in finding novel treatments for this debilitating disease. This review highlights the established pathogenesis of JCV infection and the need to overcome these challenges to eliminate the virus and prevent PML.
JOURNAL OF NEUROVIROLOGY
(2023)
Article
Clinical Neurology
Markus Kinner, Christian Prehn, Ruth Schneider, Christoph Schroeder, Eva Kolb, Ralf Gold, Robert Hoepner, Andew Chan
Summary: This study evaluated the neuropsychological disease course of NTZ-PML patients and found that working memory and visuospatial abilities are core deficits. NTZ-PML patients performed worse in neuropsychological examinations compared to controls, especially affecting visuospatial ability and working memory during different disease stages.
EUROPEAN JOURNAL OF NEUROLOGY
(2021)
Review
Clinical Neurology
Irene Cortese, Daniel S. Reich, Avindra Nath
Summary: The Review provides an overview of progressive multifocal leukoencephalopathy and diseases caused by JC virus, discussing emerging immunotherapeutic approaches for increased survival. It highlights the need to reassess PML pathophysiology and clinical course, and the challenges of reversing underlying immunosuppression in the absence of antiviral therapies. Emerging treatments show promise but caution is advised to manage potential complications such as immune reconstitution inflammatory syndrome and long-term neurological effects.
NATURE REVIEWS NEUROLOGY
(2021)
Article
Medicine, General & Internal
Varun Jain, Hannah Branstetter, Srikar Savaram, Matthew Vasquez, Gabriel Swords, Sina Aghili-Mehrizi, John Rees, Marie Rivera-Zengotita, Mayra Montalvo, Miguel Chuquilin, Addie Patterson, Torge Rempe
Summary: This study is a retrospective review of all PML cases at the University of Florida. The results suggest that PML without overt immunosuppression may be more common than previously described. Therefore, PML should be considered even in the absence of overt immunosuppression.
Article
Medicine, General & Internal
Rachel D. Truong, Vamshi K. Vadlapatla, Nicholas G. Avgeropoulos
Summary: Progressive multifocal leukoencephalopathy (PML) is a viral infection caused by the John Cunningham virus (JCV). We reported a case of a 74-year-old male who presented with progressive memory loss, episodic confusion, and aphasia. Initially suspected as CNS lymphoma, the diagnosis was confirmed as PML through microscopic examination and molecular testing.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Article
Neurosciences
Chia-Yu Chiu, Eva Amenta, Violeta Chavez, Yuriko Fukuta, Rodrigo Hasbun
Summary: The diagnosis of PML is based on clinical, radiographic, and laboratory findings, but a negative JCPyV PCR in the CSF does not always rule out JCPyV-related PML. This review examines the characteristics of biopsy-proven PML in patients with undetectable JCPyV CSF PCR and suggests alternative approaches in this scenario.
JOURNAL OF NEUROVIROLOGY
(2023)
Review
Clinical Neurology
Nora Moehn, Lea Grote-Levi, Franziska Hopfner, Britta Eiz-Vesper, Britta Maecker-Kolhoff, Clemens Warnke, Kurt-Wolfram Suehs, Mike P. Wattjes, Guenter U. Hoeglinger, Thomas Skripuletz
Summary: Progressive multifocal leukoencephalopathy (PML) is an opportunistic viral disease of the brain caused by human polyomavirus 2. It affects patients with compromised immune systems and currently has no effective treatment. The primary therapeutic strategy focuses on reconstituting the immune system, with recent studies showing promising effects with anti-PD-1 antibodies and allogeneic virus-specific T cells.
JOURNAL OF NEUROLOGY
(2022)
Article
Clinical Neurology
Hyunjung Kim, Jhii-Hyun Ahn, Sung Min Ko, Jin Woo Kim
Summary: The pre-navigation balloon technique significantly reduces the occurrence of distal embolism and increases the first-pass effect (FPE) during stent retriever thrombectomy. This technique is particularly effective for patients with ICA occlusion, improving clinical outcomes.
CLINICAL NEUROLOGY AND NEUROSURGERY
(2024)
Review
Clinical Neurology
Mehdi Khaleghi, Joshua Vignolles-Jeong, Bradley Otto, Ricardo Carrau, Daniel Prevedello
Summary: This study presented a rare case of symptomatic ectopic Rathke's cleft cyst (RCC), which was located posterior to the pituitary gland and caused extensive clival erosion. The surgical technique of wide marsupialization of the cyst using intraoperative ultrasound-assisted endoscopic endonasal transclival approach was described, and a systematic literature review of intracranial ectopic RCCs was conducted.
CLINICAL NEUROLOGY AND NEUROSURGERY
(2024)
Article
Clinical Neurology
Oscar H. Del Brutto, Denisse A. Rumbea, Aldo F. Costa, Maitri Patel, Mark J. Sedler, Robertino M. Mera
Summary: This study found an association between cognitive impairment and mortality risk in adults of Amerindian ancestry living in rural Ecuador.
CLINICAL NEUROLOGY AND NEUROSURGERY
(2024)
Article
Clinical Neurology
Yangjingyi Xia, Li Li, Yunze Li, Manyan Hu, Tianrui Zhang, Qinghua Feng, Wenlei Li, Yuan Zhu, Minghua Wu
Summary: This study shows that fasting blood glucose (FBG) level is significantly associated with unfavorable outcome after acute ischemic stroke (AIS) in patients. The risk of unfavorable outcome increases significantly when FBG is above 5.5 mmol/L.
CLINICAL NEUROLOGY AND NEUROSURGERY
(2024)