Diffusion tensor imaging in Joubert syndrome.
- Poretti A Department of Pediatric Neurology, University Children's Hospital, Zurich, Switzerland.
- Boltshauser E
- Loenneker T
- Valente EM
- Brancati F
- Il'yasov K
- Huisman TA
- 2007-09-28
Published in:
- AJNR. American journal of neuroradiology. - 2007
Abnormalities, Multiple
Adolescent
Adult
Brain
Cerebellum
Child
Diffusion Magnetic Resonance Imaging
Humans
Pyramidal Tracts
Syndrome
English
BACKGROUND AND PURPOSE
Neuropathologic findings and preliminary imaging studies demonstrated the absence of pyramidal tract and superior cerebellar peduncular decussation in individual patients with Joubert syndrome (JS). We hypothesized that functional-structural neuroimaging findings do not differ between the genetic forms of JS.
MATERIALS AND METHODS
MR imaging was performed with a 3T MR imaging-unit. Multiplanar T2- and T1-weighted imaging was followed by diffusion tensor imaging (DTI). Isotropic diffusion-weighted images, apparent diffusion coefficient maps, and color-coded fractional anisotropy maps, including tractography, were subsequently calculated.
RESULTS
In all 6 patients studied, DTI showed that the fibers of the superior cerebellar peduncles did not decussate in the mesencephalon and the corticospinal tract failed to cross in the caudal medulla. The patients represented various genetic forms of JS.
CONCLUSION
In JS, the fibers of the pyramidal tract and the superior cerebellar peduncles do not cross, irrespective of the underlying mutation.
Neuropathologic findings and preliminary imaging studies demonstrated the absence of pyramidal tract and superior cerebellar peduncular decussation in individual patients with Joubert syndrome (JS). We hypothesized that functional-structural neuroimaging findings do not differ between the genetic forms of JS.
MATERIALS AND METHODS
MR imaging was performed with a 3T MR imaging-unit. Multiplanar T2- and T1-weighted imaging was followed by diffusion tensor imaging (DTI). Isotropic diffusion-weighted images, apparent diffusion coefficient maps, and color-coded fractional anisotropy maps, including tractography, were subsequently calculated.
RESULTS
In all 6 patients studied, DTI showed that the fibers of the superior cerebellar peduncles did not decussate in the mesencephalon and the corticospinal tract failed to cross in the caudal medulla. The patients represented various genetic forms of JS.
CONCLUSION
In JS, the fibers of the pyramidal tract and the superior cerebellar peduncles do not cross, irrespective of the underlying mutation.
- Language
-
- English
- Open access status
- hybrid
- Identifiers
-
- DOI 10.3174/ajnr.A0703
- PMID 17898198
- Persistent URL
- https://sonar.ch/global/documents/4290
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