Spectrum of MRI brain findings in subacute sclerosing panencephalitis

Abstract

Background and objectives: Subacute sclerosing panencephalitis (SSPE) is a progressive catastrophic neurodegenerative disease because of persistent measles viral infection in the brain. This study was designed to determine the spectrum of magnetic resonance imaging (MRI) findings in subacute sclerosing panencephalitis. Design: Case series. Methods: We described the brain MRI findings in 20 pediatric patients with confirmed SSPE with their clinical and electroencephalogram (EEG) correlates. This study was conducted at Aga Khan University Hospital, Karachi, Pakistan between January 2006 and June 2016. Diagnosis of SSPE was on the basis of the clinical signs and symptoms, the characteristic EEG patterns (burst suppression in the early stage and a diffuse, random, slow arrhythmia pattern in the late stage), and high titers of measles antibody in the cerebro-spinal fluid. Results: The mean age at presentation was 7.4 ± 3.3 years. MRI abnormalities included diffuse white matter changes (n = 8), subcortical T2 hyperintesities in both grey and white matter in 1 patient and the brainstem changes in 2 patients. MRI was normal in 8/20 patients. Magnetic resonance spectroscopy (MRS) was performed in 4 patients out of whom 1 patient showed reduced N-acetyl aspartate (NAA) peak with elevated choline peak and inverted doublet lactate peak, 1 showed only reduced NAA, 1 showed isolated choline peak and 1 patient had a normal MRS. Conclusion: MRI brain to date is supportive in understanding the pathology of SSPE. MRI can be normal in patients with SSPE if done early on at the start of the disease.