Ettore Salsano1, Giorgio Marotta2, Valentina Manfredi2, Anna Rita Giovagnoli2, Laura Farina2, Mario Savoiardo2, Davide Pareyson2, Riccardo Benti2, Graziella Uziel2. 1. From the Departments of Clinical Neurosciences (E.S., D.P.), Diagnostics and Applied Technology (V.M., A.R.G., L.F., M.S.), and Child Neurology (G.U.), Fondazione IRCCS, Istituto Neurologico "C. Besta," Milano, Italy; and Department of Nuclear Medicine (G.M., R.B.), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy. ettore.salsano@istituto-besta.it. 2. From the Departments of Clinical Neurosciences (E.S., D.P.), Diagnostics and Applied Technology (V.M., A.R.G., L.F., M.S.), and Child Neurology (G.U.), Fondazione IRCCS, Istituto Neurologico "C. Besta," Milano, Italy; and Department of Nuclear Medicine (G.M., R.B.), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
Abstract
OBJECTIVE: To investigate the cerebral glucose metabolism in subjects with X-linked adrenoleukodystrophy (X-ALD) by using brain [(18)F]-fluorodeoxyglucose PET (FDG-PET). METHODS: Cross-sectional study in which 12 adults with various forms of X-ALD underwent clinical evaluation and brain MRI, followed by brain FDG-PET, neuropsychological assessment, and personality and psychopathology evaluation using the Symptom Checkist-90-Revised (SCL-90-R) and the Millon Clinical Multiaxial Inventory-III (MCMI-III). RESULTS: When compared to healthy control subjects (n = 27) by using Statistical Parametric Mapping 8 software, the patients with X-ALD-with or without brain MRI changes-showed a pattern of increased glucose metabolism in frontal lobes and reduced glucose metabolism in cerebellum and temporal lobe areas. On single case analysis by Scenium software, we found a similar pattern, with significant (p < 0.02) correlation between the degree of hypermetabolism in the frontal lobes of each patient and the corresponding X-ALD clinical scores. With respect to personality, we found that patients with X-ALD usually present with an obsessive-compulsive personality disorder on the MCMI-III, with significant (p < 0.05) correlation between glucose uptake in ventral striatum and severity of score on the obsessive-compulsive subscale. CONCLUSIONS: We examined cerebral glucose metabolism using FDG-PET in a cohort of patients with X-ALD and provided definite evidence that in X-ALD the analysis of brain glucose metabolism reveals abnormalities independent from morphologic and signal changes detected by MRI and related to clinical severity. Brain FDG-PET may be a useful neuroimaging technique for the characterization of X-ALD and possibly other leukodystrophies.
OBJECTIVE: To investigate the cerebral glucose metabolism in subjects with X-linked adrenoleukodystrophy (X-ALD) by using brain [(18)F]-fluorodeoxyglucose PET (FDG-PET). METHODS: Cross-sectional study in which 12 adults with various forms of X-ALD underwent clinical evaluation and brain MRI, followed by brain FDG-PET, neuropsychological assessment, and personality and psychopathology evaluation using the Symptom Checkist-90-Revised (SCL-90-R) and the Millon Clinical Multiaxial Inventory-III (MCMI-III). RESULTS: When compared to healthy control subjects (n = 27) by using Statistical Parametric Mapping 8 software, the patients with X-ALD-with or without brain MRI changes-showed a pattern of increased glucose metabolism in frontal lobes and reduced glucose metabolism in cerebellum and temporal lobe areas. On single case analysis by Scenium software, we found a similar pattern, with significant (p < 0.02) correlation between the degree of hypermetabolism in the frontal lobes of each patient and the corresponding X-ALD clinical scores. With respect to personality, we found that patients with X-ALD usually present with an obsessive-compulsive personality disorder on the MCMI-III, with significant (p < 0.05) correlation between glucose uptake in ventral striatum and severity of score on the obsessive-compulsive subscale. CONCLUSIONS: We examined cerebral glucose metabolism using FDG-PET in a cohort of patients with X-ALD and provided definite evidence that in X-ALD the analysis of brain glucose metabolism reveals abnormalities independent from morphologic and signal changes detected by MRI and related to clinical severity. Brain FDG-PET may be a useful neuroimaging technique for the characterization of X-ALD and possibly other leukodystrophies.
Authors: Giada Cipollina; Arash Davari Serej; Gianluca Di Nolfi; Andrea Gazzano; Andrea Marsala; Mauro G Spatafora; Marco Peviani Journal: Int J Mol Sci Date: 2020-10-25 Impact factor: 5.923