Brain fluorodeoxyglucose PET in adrenoleukodystrophy.

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.

• Authors: Ettore Salsano, Giorgio Marotta, Valentina Manfredi, Anna Rita Giovagnoli, Laura Farina, Mario Savoiardo, Davide Pareyson, Riccardo Benti, Graziella Uziel
• Journal: Neurology (Neurology) Vol. 83 Issue 11 Pg. 981-9 (Sep 09 2014) ISSN: 1526-632X [Electronic] United States
• PMID: 25098542 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2014 American Academy of Neurology.

Chemical References

• Radiopharmaceuticals
• Fluorodeoxyglucose F18
• Glucose

Topics

• Adrenoleukodystrophy (diagnostic imaging, metabolism)
• Adult
• Brain (diagnostic imaging, 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