Neuroimaging Abnormalities in Patients with Subacute Sclerosing Panencephalitis : Prospective Follow-up Study

Area of Science:

• Neurology
• Neuroimaging
• Pediatric Neurology

Background:

• Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological disorder.
• Early diagnosis and monitoring of disease progression are crucial for managing SSPE.

Purpose of the Study:

• To evaluate neuroimaging abnormalities and their progression in SSPE patients.
• To identify clinical predictors of neuroimaging findings in SSPE.
• To assess the impact of treatment on disease progression.

Main Methods:

• Prospective observational study of SSPE patients.
• Clinical assessment, cerebrospinal fluid examination, EEG, and MRI scans.
• Categorization using Dyken's criteria, Jabbour's staging, and definition of fulminant SSPE.
• Treatment with intrathecal interferon-α and antiepileptic drugs.
• 6-month follow-up including Modified Barthel Index and neuroimaging.

Main Results:

• Parieto-occipital white matter hyperintensity (WMH) is the most prevalent and sensitive neuroimaging finding for SSPE diagnosis.
• Diffuse cerebral atrophy was significant in advanced stages (JS III) and progressed over 6 months.
• Longer disease duration independently predicted cerebral atrophy.
• Basal ganglia involvement correlated with movement disorders.
• Fulminant SSPE occurred in 33% of cases, with higher mortality.

Conclusions:

• Parieto-occipital WMH is a key neuroimaging marker for SSPE diagnosis.
• Cerebral atrophy progresses in SSPE despite interferon treatment, particularly in aggressive and fulminant forms.
• Increasing disease duration is a significant predictor of cerebral atrophy progression.