Brain fluorodeoxyglucose PET in adrenoleukodystrophy

• Published in: Neurology Vol. 83; no. 11; p. 981
• Main Authors: Salsano, Ettore, Marotta, Giorgio, Manfredi, Valentina, Giovagnoli, Anna Rita, Farina, Laura, Savoiardo, Mario, Pareyson, Davide, Benti, Riccardo, Uziel, Graziella
• Format: Journal Article
• Language: English
• Published: United States 09.09.2014
• Subjects: Adrenoleukodystrophy - diagnostic imaging; Adrenoleukodystrophy - metabolism; Adult; Brain - diagnostic imaging; Brain - metabolism; Brain Mapping - methods; Cross-Sectional Studies; Fluorodeoxyglucose F18; Glucose - metabolism; Humans; Image Processing, Computer-Assisted - methods; Male; Middle Aged; Neuropsychological Tests; Personality - physiology; Personality Assessment; Positron-Emission Tomography; Psychiatric Status Rating Scales; Radiopharmaceuticals; Software; Young Adult
• ISSN: 1526-632X
• DOI: 10.1212/WNL.0000000000000770

To investigate the cerebral glucose metabolism in subjects with X-linked adrenoleukodystrophy (X-ALD) by using brain [(18)F]-fluorodeoxyglucose PET (FDG-PET). 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). 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. 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.