| Involvement of Globus Pallidus and Midbrain Nuclei in Pantothenate Kinase-Associated Neurodegeneration : Measurement of T2 and T2* Time. | |
| | |
MedLine Citation:
|
PMID: 22258188 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
|
PURPOSE: To quantify involvement of globus pallidus and two midbrain nuclei (substantia nigra and red nucleus) in Pantothenate Kinase-Associated Neurodegeneration (PKAN). MATERIAL AND METHODS: We performed T2 and T2* weighted imaging with calculation of the corresponding relaxation times on a subset of 5 patients from a larger group of 20 patients with PKAN from the southwest part of the Dominican Republic. Examinations were carried out on a 3T scanner and included a multi-echo spin-echo as well as a multi-echo gradient echo sequence. Results were compared to a control group of 19 volunteers. RESULTS: T2 and T2* weighted sequences showed abnormal signal reduction in the globus pallidus of all patients. On T2* weighted imaging, abnormal signal in the substantia nigra could reliably be detected in 75% of cases, but differentiation from normal was less reliable in T2 weighted scans. Correspondingly, relaxation times differed from normal with very high significance (p < 0.0001) in the globus pallidus, but with with less significance in the substantia nigra (p ≤ 0.03). The red nucleus was not affected. CONCLUSIONS: Signal reduction in the globus pallidus, which probably is due to abnormal accumulation of iron, is severe in PKAN and can be differentiated from normal with high reliability. The substantia nigra is affected to a lesser degree, and the red nucleus is not involved. The reason for this selective susceptibility of normally iron-rich brain structures for pathological accumulation of iron remains speculative. Our quantitative results might be helpful to assess the value of an iron chelation approach to therapy. |
| | |
Authors:
|
R Fermin-Delgado; P Roa-Sanchez; H Speckter; E Perez-Then; D Rivera-Mejia; B Foerster; P Stoeter |
Related Documents
:
|
22218278 - Postmortem verification of ms cortical lesion detection with 3d dir. 21986148 - Pineal parenchymal tumours: minimum apparent diffusion coefficient in prediction of tum... 10369238 - Direct identification of ventrointermediate nucleus of the thalamus on magnetic resonan... 21695468 - Use of transtibial aimer via the accessory anteromedial portal to identify the center o... 18006618 - Significant benefit of multimodal imaging: pet/ct compared with pet alone in staging an... 6381558 - Ct analysis of lung density changes in patients undergoing total body irradiation prior... |
Publication Detail:
|
Type: JOURNAL ARTICLE Date: 2012-1-19 |
Journal Detail:
|
Title: Clinical neuroradiology Volume: - ISSN: 1869-1447 ISO Abbreviation: - Publication Date: 2012 Jan |
Date Detail:
|
Created Date: 2012-1-19 Completed Date: - Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 101526693 Medline TA: Clin Neuroradiol Country: - |
Other Details:
|
Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
|
Dep. of Radiology, CEDIMAT, Plaza de la Salud, Santo Domingo, Republica Dominicana. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
|
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Towards a synthetic view of potato cold and salt stress response by transcriptomic and proteomic ana...
Next Document: [The progression from hypertension to congestive heart failure].