This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.
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.
GLOSSARY
- ALMN=
- adrenoleukomyeloneuropathy;
- AMN=
- adrenomyeloneuropathy;
- BBB=
- blood-brain barrier;
- BR=
- base rate;
- C-ALD=
- cerebral adrenoleukodystrophy;
- DSM-IV=
- Diagnostic and Statistical Manual of Mental Disorders, fourth edition;
- FDG=
- fluorodeoxyglucose;
- FDR=
- false discovery rate;
- MCMI-III=
- Millon Clinical Multiaxial Inventory-III;
- OCPD=
- obsessive-compulsive personality disorder;
- SCL-90-R=
- Symptom Checkist-90-Revised;
- SPM=
- Statistical Parametric Mapping;
- X-ALD=
- X-linked adrenoleukodystrophy
Footnotes
↵† Deceased.
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Supplemental data at Neurology.org
- Received November 18, 2013.
- Accepted in final form June 11, 2014.
- © 2014 American Academy of Neurology
AAN Members
We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.
AAN Non-Member Subscribers
Purchase access
Letters: Rapid online correspondence
REQUIREMENTS
You must ensure that your Disclosures have been updated within the previous six months. Please go to our Submission Site to add or update your Disclosure information.
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.
You May Also be Interested in
Efficacy of Ubrogepant in the Acute Treatment of Migraine With Mild Pain vs Moderate or Severe Pain
Dr. Kathleen Digre and Dr. Kendra Pham
► Watch
Topics Discussed
Alert Me
Recommended articles
Motor correlates of occipital glucose hypometabolism in Parkinson’s disease without dementia
The unpleasantness of tonic pain is encoded by the insular cortex
Functional anatomy of neuropsychological deficits after severe traumatic brain injury
Cerebral glucose metabolism in patients with AD and different APOE genotypes