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Involvement of Globus Pallidus and Midbrain Nuclei in Pantothenate Kinase-Associated Neurodegeneration

Measurement of T2 and T2* Time

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Abstract

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.

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Conflict of Interest

The authors declare that there is no actual or potential conflict of interest in relation to this article.

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Authors and Affiliations

  1. Dep. of Radiology, CEDIMAT, Plaza de la Salud, Santo Domingo, Republica Dominicana

    R. Fermin-Delgado MD, H. Speckter BA & P. Stoeter MD

  2. Dep. of Neurology, CEDIMAT, Plaza de la Salud, Santo Domingo, Republica Dominicana

    P. Roa-Sanchez MD

  3. Dep. of Investigations, CEDIMAT, Plaza de la Salud, Santo Domingo, Republica Dominicana

    E. Perez-Then MD

  4. Dep. of Neurosurgery, CEDIMAT, Plaza de la Salud, Santo Domingo, Republica Dominicana

    D. Rivera-Mejia MD

  5. Philips Medical Systems LatAm, Sao Paulo, Brazil

    B. Foerster BA

Authors
  1. R. Fermin-Delgado MD
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  2. P. Roa-Sanchez MD
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  3. H. Speckter BA
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  4. E. Perez-Then MD
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  5. D. Rivera-Mejia MD
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  6. B. Foerster BA
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  7. P. Stoeter MD
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Corresponding author

Correspondence to P. Stoeter MD.

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Fermin-Delgado, R., Roa-Sanchez, P., Speckter, H. et al. Involvement of Globus Pallidus and Midbrain Nuclei in Pantothenate Kinase-Associated Neurodegeneration. Clin Neuroradiol 23, 11–15 (2013). https://doi.org/10.1007/s00062-011-0127-9

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  • DOI: https://doi.org/10.1007/s00062-011-0127-9

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