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

• Published in: Clinical neuroradiology (Munich) Vol. 34; no. 3; pp. 577 - 585
• Main Authors: Keerthiraj, D. B., Pandey, Shweta, Kumar Garg, Ravindra, Singh Malhotra, Hardeep, Verma, Rajesh, Kumar Sharma, Praveen, Kumar, Neeraj, Uniyal, Ravi, Rizvi, Imran, Kumar, Sukriti, Parihar, Anit, Jain, Amita
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.09.2024
• Subjects: Adolescent; Brain - diagnostic imaging; Brain - pathology; Child; Disease Progression; Electroencephalography; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging - methods; Male; Medicine; Medicine & Public Health; Neuroimaging - methods; Neurology; Neuroradiology; Neurosurgery; Original Article; Prospective Studies; Subacute Sclerosing Panencephalitis - complications; Subacute Sclerosing Panencephalitis - diagnostic imaging; Young Adult; Subacute sclerosing panencephalitis; Clinical predictors; Cerebral atrophy; White matter abnormalities; Fulminant subacute sclerosing panencephalitis
• ISSN: 1869-1439; 1869-1447; 1869-1447
• DOI: 10.1007/s00062-024-01396-1

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.