Brain fluorodeoxyglucose PET in adrenoleukodystrophy

Neurology. 2014 Sep 9;83(11):981-9. doi: 10.1212/WNL.0000000000000770. Epub 2014 Aug 6.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • 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

Substances

  • Radiopharmaceuticals
  • Fluorodeoxyglucose F18
  • Glucose