Deniz Yüksel1, Barış Diren2, Hakan Ulubay2, Sakir Altunbaşak2, Banu Anlar2. 1. From the Department of Pediatric Neurology (D.Y.), Dr. Sami Ulus Children's Hospital, Ankara; Department of Radiology (B.D., H.U.), Ankara Medicana Hospital, Ankara; Department of Pediatric Neurology (S.A.), Çukurova University Faculty of Medicine, Adana; and Department of Pediatric Neurology (B.A.), Hacettepe University Faculty of Medicine, Ankara, Turkey. drdeniz_yuksel@yahoo.com.tr. 2. From the Department of Pediatric Neurology (D.Y.), Dr. Sami Ulus Children's Hospital, Ankara; Department of Radiology (B.D., H.U.), Ankara Medicana Hospital, Ankara; Department of Pediatric Neurology (S.A.), Çukurova University Faculty of Medicine, Adana; and Department of Pediatric Neurology (B.A.), Hacettepe University Faculty of Medicine, Ankara, Turkey.
Abstract
OBJECTIVE: We performed diffusion tensor imaging (DTI) and magnetic resonance spectroscopy (MRS) studies in a group of patients with subacute sclerosing panencephalitis (SSPE) in order to estimate the pathologic process underlying the phenotypic variability. METHODS: Patients with SSPE who had MRI including DTI and MRS examinations were evaluated according to their clinical status as determined by the SSPE Scoring System and their mental age as determined by tests appropriate for age and developmental level. Comparisons of fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values and metabolite ratios of frontal periventricular white matter, parieto-occipital periventricular white matter, and globus pallidus in both hemispheres were made between control and SSPE groups, and between SSPE subgroups. RESULTS: Control (n = 18) and SSPE (n = 39) groups differed in all DTI and MRS parameters except FA, choline (Cho), and Cho/creatine (Cr). SSPE cases had higher ADC and lower N-acetylaspartate (NAA), NAA/Cho, and NAA/Cr in all regions of interest, suggesting cell loss. Disease progression rate and neurologic deficit appeared to be associated with the degree of ADC elevation and NAA reduction: the group with severe global deterioration had the lowest NAA (230.75 ± 197.97 in forceps minor), and rapid progression was associated with acute reduction in NAA. CONCLUSIONS: The combination of MRS and diffusion MRI findings suggests neuronal loss can be a primary target in rapidly or subacutely progressing SSPE, and preservation or regeneration of axonal structure may be beneficial in chronic cases.
OBJECTIVE: We performed diffusion tensor imaging (DTI) and magnetic resonance spectroscopy (MRS) studies in a group of patients with subacute sclerosing panencephalitis (SSPE) in order to estimate the pathologic process underlying the phenotypic variability. METHODS:Patients with SSPE who had MRI including DTI and MRS examinations were evaluated according to their clinical status as determined by the SSPE Scoring System and their mental age as determined by tests appropriate for age and developmental level. Comparisons of fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values and metabolite ratios of frontal periventricular white matter, parieto-occipital periventricular white matter, and globus pallidus in both hemispheres were made between control and SSPE groups, and between SSPE subgroups. RESULTS: Control (n = 18) and SSPE (n = 39) groups differed in all DTI and MRS parameters except FA, choline (Cho), and Cho/creatine (Cr). SSPE cases had higher ADC and lower N-acetylaspartate (NAA), NAA/Cho, and NAA/Cr in all regions of interest, suggesting cell loss. Disease progression rate and neurologic deficit appeared to be associated with the degree of ADC elevation and NAA reduction: the group with severe global deterioration had the lowest NAA (230.75 ± 197.97 in forceps minor), and rapid progression was associated with acute reduction in NAA. CONCLUSIONS: The combination of MRS and diffusion MRI findings suggests neuronal loss can be a primary target in rapidly or subacutely progressing SSPE, and preservation or regeneration of axonal structure may be beneficial in chronic cases.
Authors: Mariarosaria Valente; Ilaria Del Negro; Daniele Bagatto; Riccardo Garbo; Christian Lettieri; Andrea Bernardini; Annacarmen Nilo; Maria Rosaria Peri; Davide Pecori; Gian Luigi Gigli Journal: BMJ Neurol Open Date: 2021-07-23