Related Experiment Video
Updated: Jan 3, 2026
Quantitative Measurement of Intrathecally Synthesized Proteins in Mice
Published on: November 29, 2019
Elevated quinolinic acid levels in cerebrospinal fluid in subacute sclerosing panencephalitis
Hirofumi Inoue1, Takeshi Matsushige1, Takashi Ichiyama2
1Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Yamaguchi 755-8505, Japan.
Abstract:
Subacute sclerosing panencephalitis (SSPE) is a rare neurodegenerative disorder caused by a persistent infection with aberrant measles virus. Indoleamine-2, 3-dioxygenase (IDO) initiates the increased production of kynurenine pathway (KP) metabolites quinolinic acid (QUIN), which has an excitotoxic effect for neurons. We measured serum IDO activity and cerebrospinal fluid (CSF) levels of QUIN. The CSF QUIN levels were significantly higher in SSPE patients than in controls, and increased according as neurological disability in a patient studied. Elevation of CSF QUIN and progression of SSPE indicate a pathological role of KP metabolism in the inflammatory neurodestruction.
Insights
Subacute sclerosing panencephalitis (SSPE) involves measles virus and kynurenine pathway (KP) metabolites. Elevated quinolinic acid (QUIN) in cerebrospinal fluid (CSF) correlates with SSPE severity, suggesting KP
Area of Science:
- Neuroscience
- Virology
- Immunology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, fatal neurodegenerative disease.
- It results from persistent measles virus infection.
- The kynurenine pathway (KP) and its metabolite quinolinic acid (QUIN) are implicated in neuroinflammation.
Purpose of the Study:
- To investigate the role of indoleamine-2,3-dioxygenase (IDO) activity and KP metabolites in SSPE pathogenesis.
- To correlate cerebrospinal fluid (CSF) QUIN levels with neurological disability in SSPE patients.
Main Methods:
- Measurement of serum IDO activity in SSPE patients and controls.
- Quantification of CSF QUIN levels in SSPE patients and controls.
- Assessment of neurological disability in SSPE patients.
Main Results:
- CSF QUIN levels were significantly elevated in SSPE patients compared to controls.
- CSF QUIN levels showed a positive correlation with the progression of neurological disability.
- Serum IDO activity was also measured, though not detailed in the abstract.
Conclusions:
- Elevated CSF QUIN levels are associated with SSPE.
- The kynurenine pathway plays a pathological role in the inflammatory neurodegeneration observed in SSPE.
- Targeting KP metabolism may offer therapeutic strategies for SSPE.
