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Published on: February 10, 2023
Characteristics of progressive multifocal leukoencephalopathy clarified through internet-assisted laboratory
Kazuo Nakamichi1, Hidehiro Mizusawa, Masahito Yamada
1Department of Virology 1, National Institute of Infectious Diseases, Toyama, Shinjuku-ku, Tokyo, 162-8640, Japan.
Background:
Progressive multifocal leukoencephalopathy (PML), a rare but fatal demyelinating disease caused by JC virus (JCV), occurs mainly in immunocompromised patients. As PML develops in individuals with various underlying disorders sporadically and infrequently, a nationwide survey of PML is difficult. This study was conducted to elucidate the characteristics of PML in Japan through an internet-assisted laboratory surveillance program.
Methods:
A diagnostic support system for PML was established using a real-time PCR assay of JCV DNA in cerebrospinal fluid (CSF), and requests for testing were received from clinicians via specialized websites. Medical histories of patients were collected through standardized questionnaires, and a database of CSF JCV loads and clinical information was created and analyzed.
Results:
For 4 years from April 2007 to March 2011, CSF specimens from 419 patients were tested. Forty-eight individuals were found positive for JCV DNA in their CSF and were diagnosed with PML. PML primarily occurred not only in HIV-positive patients (33.3%) but also in patients with hematologic disorders after receiving stem cell transplantation, chemotherapy, and/or immunosuppressive treatment (39.6%). The frequencies of PML cases among the subjects in these two categories were 20.3% and 23.5%, respectively. Although no significant features were observed with respect to CSF JCV loads in PML patients with an HIV infection or hematologic disorder, males were predominant in both groups (100% and 89.5%, respectively). The proportion of PML cases with autoimmune disorders (6.3%) or solid-organ transplants (2.1%) was smaller than those with HIV infection or hematologic disorders, probably due to the limited availability of therapeutic monoclonal antibodies and transplantation from brain dead donors.
Conclusions:
The results suggest that the internet-assisted laboratory surveillance program might be a useful strategy for collecting precise real-time information on PML on a national level. The current database provides important background information for the diagnosis and treatment of patients with risk factors for PML.
Insights
This study utilized an internet-assisted surveillance program to characterize progressive multifocal leukoencephalopathy (PML) in Japan. The findings highlight PML
Area of Science:
- Neuroscience
- Virology
- Epidemiology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, fatal demyelinating disease caused by the JC virus (JCV).
- PML predominantly affects immunocompromised individuals with diverse underlying conditions, complicating nationwide surveys.
- Characterizing PML epidemiology is crucial for understanding disease patterns and patient risk factors.
Purpose of the Study:
- To elucidate the epidemiological characteristics of PML in Japan.
- To establish and evaluate an internet-assisted laboratory surveillance program for PML.
- To create a real-time database of PML cases and associated clinical information.
Main Methods:
- An internet-assisted laboratory surveillance system was developed for PML diagnosis.
- Real-time PCR assay of JC virus (JCV) DNA in cerebrospinal fluid (CSF) was employed.
- Clinical data and CSF JCV loads were collected via questionnaires and analyzed.
Main Results:
- Over 4 years, 419 CSF specimens were tested, identifying 48 PML cases.
- PML occurred in HIV-positive patients (33.3%) and those with hematologic disorders (39.6%).
- Males predominated in both HIV-associated (100%) and hematologic (89.5%) PML cases; CSF JCV loads showed no significant differences.
Conclusions:
- Internet-assisted surveillance is a viable strategy for real-time PML data collection in Japan.
- The generated database offers valuable insights for diagnosing and managing PML in at-risk populations.
- This approach aids in understanding PML's national prevalence and characteristics.
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