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[Panencephalitis in children in modern conditions: clinical, etiological and mri-aspects]
N V Skripchenko1, G P Ivanova2, E Yu Skripchenko1
1Children's Research and Clinical Center for Infectious Diseases of the Federal Medical and Biological Agency of Russia, St.-Petersburg, Russia; Saint-Petersburg Pediatric Medical University, St.-Petersburg, Russia.
Insights
Panencephalitis in children is often linked to congenital infections, particularly herpes viruses. Early treatment initiation is crucial for positive outcomes in 75% of pediatric panencephalitis cases.
Area of Science:
- Pediatric Neurology
- Infectious Diseases
- Neuroimaging
Background:
- Panencephalitis (PANE) in children presents a significant diagnostic and therapeutic challenge.
- Understanding the clinical spectrum, infectious origins, and neuroimaging findings is vital for effective management.
- The impact of disease duration on PANE presentation and progression requires detailed characterization.
Purpose of the Study:
- To delineate the clinical manifestations, infectious etiologies, and magnetic resonance imaging (MRI) findings in pediatric panencephalitis.
- To analyze how the duration of the disease influences the clinical picture, diagnostic markers, and MRI characteristics of PANE.
- To evaluate the treatment outcomes based on early intervention and disease course.
Main Methods:
- A cohort of 32 children diagnosed with PANE (age 3 months to 17 years) were evaluated.
- Inclusion criterion: diffuse white matter lesions on brain MRI.
- Diagnostic methods included viral/bacterial infection screening, brain and spinal cord MRI, cerebrospinal fluid (CSF) analysis (pleocytosis, oligoclonal IgG, myelin basic protein - MBP), with a 5-10 year follow-up.
Main Results:
- PANE predominantly affects children under 3 years (84.4%) and is frequently associated with congenital infections (71.9%), notably cytomegalovirus and herpes virus 6.
- Chronic, gradual onset (78.3%) is common, with delayed motor and speech development, and symptoms including pyramidal and cerebellar signs.
- Early PANE (symptoms <3 months) shows inflammatory-demyelinating changes, mass effect, contrast enhancement, CSF pleocytosis, and elevated MBP. Later stages (>3 months) exhibit degenerative-sclerosing changes and CSF oligoclonal IgG in 90%.
Conclusions:
- Congenital infections are implicated in nearly two-thirds of pediatric PANE cases, often presenting with extensive symmetrical demyelination in the central nervous system (CNS).
- Clinical and laboratory parameters, along with treatment outcomes, are significantly influenced by the timing of therapeutic intervention.
- Positive treatment dynamics were observed in 75% of cases, highlighting the importance of prompt and appropriate management.
Aim:
To characterize the clinical picture, infectious etiology and MRI results of panencephalitis (PANE) in children, depending on the duration of the disease.
Material And Methods:
Children (n=32) with PANE at the age from 3 months up to 17 years were examined for a group of actual viral and bacterial infections. The inclusion criterion was a diffuse lesion of white matter hemispheres on MRI. MRI of the brain and spinal cord, CSF study (pleocytosis, oligoclonal IgG, the main myelin protein (MBP) were performed. The follow-up was 5-10 years. The complex therapy included etiotropic (antiviral) and pathogenetic agents, with priority given to cytoflavin as a drug with a multimodal effect.
Results:
In 84.4% of cases, PANE in children are recorded under the age of 3 years and in 71.9% of cases, are associated with congenital infections with the prevalence of herpes viruses, especially cytomegalovirus (37,8%) and herpes type 6 (21.9%). In 78.3% of cases, PANE have chronic gradual development more often with a delay in the formation of motor and speech/prespeech skills, and manifested with pyramidal, cerebellar and other symptoms in the future. PANE is accompanied by a lesion of the white matter of the hemispheres in 1/2 cases with periventricular localization, less often in infratentorial structures (46.9%) and spinal cord (21.9%). With the duration of symptoms up to 3 months (n=22) PANE are characterized by inflammatory-demyelinating changes, signs of mass effect, contrast+, pleocytosis in CSF and the increase in MBP (average 4.2±0.8 ng/ml), and, with the duration of more than 3 months, by degenerative-sclerosing changes (n=10) and CSF oligoclonal IgG in 90% of cases.
Conclusion:
In almost 2/3 of cases, PANE are associated with congenital infections, accompanied by extensive symmetrical foci of demyelination in the CNS, and their clinical and laboratory parameters and outcomes depend on the start of treatment, with the positive dynamics in 75% of cases.
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