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Megalencephaly in NF1

Predominantly white matter contribution and mitigation by ADHD

From the aMRI Analysis Laboratory (Drs. Cutting, Mostofsky, Kates, Denckla, and Kaufmann and K.L. Cooper and C.W. Koth), Kennedy Krieger Institute, and the Departments of Neurology (Drs. Cutting, Mostofsky, Denckla, and Kaufmann), Psychiatry (Drs. Kates, Denckla, and Kaufmann), Pediatrics (Drs. Denckla and Kaufmann), Pathology, and Radiology (Dr. Kaufmann), Johns Hopkins University School of Medicine, Baltimore, MD.

Address correspondence and reprint requests to Dr. Walter E. Kaufmann, The Kennedy Krieger Institute, 707 N. Broadway, Room 500, Baltimore, MD 21205; e-mail: wekaufman{at}jhmi.edu

Background: Megalencephaly is a frequent CNS manifestation in neurofibromatosis type 1 (NF1); however, its tissue composition, modification by attention deficit hyperactivity disorder (ADHD), and relationship with unidentified bright objects (UBO) remain controversial.

Methods: Eighteen male patients with NF1, seven of whom had ADHD (NF1+ADHD), were compared with 18 age- and sex-matched controls in terms of MRI-, Talairach-based brain, cerebral, lobar, and sublobar gray and white matter volumes. Twelve subjects with NF1 had UBO in the centrencephalic region, whereas six had no UBO or exclusively infratentorial lesions.

Results: Patients with NF1 without ADHD (NF1-pure) had the largest total cerebral, gray, and white matter volumes with larger parietal/somatosensory white matter volumes than controls, particularly if UBO were present in the basal ganglia. All subjects with NF1 (including NF1+ADHD) had larger total and frontal white matter volumes than controls. Smaller frontal/right prefrontal gray matter volumes were found in NF1+ADHD when compared with NF1-pure patients.

Conclusions: The increase in frontal and parietal white matter volumes in male patients with NF1, including the preferential centrencephalic distribution, supports the hypothesis that NF1’s white matter pathology encompasses but is not limited to visible UBO. Male patients with NF1+ADHD, as compared with NF1-pure patients, showed frontal reductions that are largely consistent with those found in idiopathic ADHD.

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