Neuroimaging findings in hyperargininemia

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Küçük Resim

Tarih

2008

Dergi Başlığı

Dergi ISSN

Cilt Başlığı

Yayıncı

Journal of Neuroimaging

Erişim Hakkı

info:eu-repo/semantics/openAccess

Özet

In hyperarginenemia, there is a defect in argininase enzyme, which is a catalyzer of urea cycle. Though the pathogenesis of neuronal damage in hyperargininemia is not clear, high serum and cerebrospinal fluid arginine levels can be directly related with neuronal damage. In this study, our aim was to assess brain magnetic resonance images and magnetic resonance spectroscopy (MRS) patterns of two siblings with hyperarginenemia. We acquired single voxel MRS from the white matter to show the myelination pattern and to figure out any abnormal peak of metabolite stored due to enzymatic defect. We observed mild cerebral and cerebellar atrophy and infarct at bilateral posterior putamen and insular cortex localization on conventional images and elevated choline/creatine ratios and abnormal peak at 3.8 ppm, most likely representing arginine deposition. To the best of our knowledge, this is the first article revealing the brain MRS pattern of hyperargininemia. We reported the clinical and imaging findings of patients and discuss the correlation.

Açıklama

J Neuroimaging 2008;18:457-462.

Anahtar Kelimeler

Kaynak

Journal of Neuroimaging

WoS Q Değeri

Scopus Q Değeri

Cilt

18

Sayı

4

Künye

Güngör, S., Akıncı, A., Ahmet Kemal, F., Tabel, Y., & Alkan, A. (2008). Neuroimaging Findings İn Hyperargininemia. Journal Of Neuroimaging, 18(4), 457–462.