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Neuroimaging Abnormalities in Patients with Subacute Sclerosing Panencephalitis

Prospective Follow-up Study

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Abstract

Objective

This study aimed to assess the neuroimaging abnormalities and their progression in patients with Subacute sclerosing panencephalitis (SSPE) and identify clinical predictors of these imaging findings.

Methods

This prospective observational study evaluated clinical and neuroimaging features in patients with SSPE. Patients were categorized using Dyken’s criteria, Jabbour’s staging system, and the definition of fulminant SSPE. They underwent comprehensive clinical assessments, cerebrospinal fluid examination, Electroencephalogram (EEG), and Magnetic Resonance Imaging (MRI) scans. Treatment involved intrathecal interferon‑α and antiepileptic medications. Functional disability was assessed using the modified Barthel index. Follow-ups were performed at 6 months, including reassessment of Modified Barthel Index (MBI) and Jabbour’s staging and EEG and MRI scans.

Results

The mean age was 13.9 ± 6.7 years, with males comprising 81.5% (44/54) of the cohort. Fulminant SSPE was noted in 33% (18/54) of cases. Disease duration before presentation varied significantly between fulminant and non-fulminant forms (p = 0.001). Neuroimaging abnormalities were more prevalent in JS III stage patients, with diffuse cerebral atrophy being a significant finding (p = 0.011). Basal ganglia involvement correlated with movement disorders. The 6‑month follow-up showed increased cerebral atrophy (p = 0.004). Increasing disease duration was an independent predictor of cerebral atrophy. An Intercomplex interval (ICI) of more than 10 minutes correlated with normal neuroimaging, 10 patients died within the study period, 8 of whom had fulminant SSPE.

Conclusion

Parieto-occipital White matter hyperintensity (WMH) is the most prevalent and sensitive neuroimaging finding for the diagnosis of SSPE. Despite interferon treatment, cerebral atrophy progressed in both aggressive and fulminant SSPE. Increasing disease duration is an independent predictor of cerebral atrophy.

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Availability of data and material

The datasets generated during and or analyzed during the current study are not publicly available but are available from the corresponding author on reasonable request.

Abbreviations

ICI:

Intercomplex interval

JS:

Jabbour staging

MBI:

Modified Barthel index

PC:

Periodic complexes

SSPE:

Subacute sclerosing panencephalitis

WMH:

White matter hyperintensity

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Authors and Affiliations

  1. Department of Neurology, King George’s Medical University, Lucknow, Uttar Pradesh, India

    D. B. Keerthiraj, Shweta Pandey, Ravindra Kumar Garg, Hardeep Singh Malhotra, Rajesh Verma, Praveen Kumar Sharma, Neeraj Kumar, Ravi Uniyal & Imran Rizvi

  2. Department of Radiodiagnosis, King George’s Medical University, Lucknow, Uttar Pradesh, India

    Sukriti Kumar & Anit Parihar

  3. Department of Microbiology, King George’s Medical University, Lucknow, Uttar Pradesh, India

    Amita Jain

Authors
  1. D. B. Keerthiraj
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  2. Shweta Pandey
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  3. Ravindra Kumar Garg
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  4. Hardeep Singh Malhotra
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  5. Rajesh Verma
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  6. Praveen Kumar Sharma
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  7. Neeraj Kumar
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  8. Ravi Uniyal
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  9. Imran Rizvi
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  10. Sukriti Kumar
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  12. Amita Jain
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Contributions

Author contributions included conception and study design (KDB, SP and RKG), data collection or acquisition (KDB, SP and SK), statistical analysis (HSM, RU, NK, and IR), interpretation of results (SP, RKG, RV and PS), drafting the manuscript work or revising it critically for important intellectual content (KDB, SP, AP, and AJ) and approval of final version to be published and agreement to be accountable for the integrity and accuracy of all aspects of the work (all authors).

Corresponding author

Correspondence to Shweta Pandey.

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Conflict of interest

D.B. Keerthiraj, S. Pandey, R. Kumar Garg, H. Singh Malhotra, R. Verma, P. Kumar Sharma, N. Kumar, R. Uniyal, I. Rizvi, S. Kumar, A. Parihar and A. Jain declare that they have no competing interests.

Ethical standards

The study received approval from our institute’s ethics committee. (IRB number: III PGTSC: IIA/P16). Consent to participate; written informed consent was obtained from all the participants or their legal representatives before inclusion in the study. Consent for publication: informed consent was obtained from the participants before submitting the manuscript for publication.

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Keerthiraj, D.B., Pandey, S., Kumar Garg, R. et al. Neuroimaging Abnormalities in Patients with Subacute Sclerosing Panencephalitis. Clin Neuroradiol (2024). https://doi.org/10.1007/s00062-024-01396-1

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