Brain fluorodeoxyglucose PET in adrenoleukodystrophy

Ettore Salsano, Giorgio Marotta, Valentina Manfredi, Anna Rita Giovagnoli, Laura Farina, Mario Savoiardo, Davide Pareyson, Riccardo Benti, Graziella Uziel

Research output: Contribution to journalArticlepeer-review

Abstract

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

Original languageEnglish
Pages (from-to)981-989
Number of pages9
JournalNeurology
Volume83
Issue number11
Publication statusPublished - Sep 1 2014

ASJC Scopus subject areas

  • Clinical Neurology
  • Arts and Humanities (miscellaneous)

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