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Different Stages of White Matter Changes in the Original HDLS Family Revealed by Advanced MRI Techniques.

Journal article
Authors Christina Sundal
Lars Jönsson
Maria Ljungberg
Jianhui Zhong
Wei Tian
Tong Zhu
Thomas Lindén
Anne Börjesson-Hanson
Oluf Andersen
Sven Ekholm
Published in Journal of neuroimaging : official journal of the American Society of Neuroimaging
Volume 24
Issue 5
Pages 444–452
ISSN 1552-6569
Publication year 2014
Published at Institute of Clinical Sciences, Department of Radiation Physics
Institute of Neuroscience and Physiology
Institute of Neuroscience and Physiology, Department of Clinical Neuroscience and Rehabilitation
Institute of Neuroscience and Physiology, Department of Psychiatry and Neurochemistry
Institute of Clinical Sciences, Department of Radiology
Pages 444–452
Language en
Links dx.doi.org/10.1111/jon.12037
Keywords MRI, DTI, MRS, hereditary leukoencephalopathy with spheroids (HDLS), white matter lesions, autosomal dominant inheritance
Subject categories Radiology, Nuclear Medicine and Medical Imaging

Abstract

BACKGROUND: The temporal evolution of white matter (WM) changes on MR examinations in hereditary diffuse leukoencephalopathy with spheroids (HDLS) is largely unknown. Our purpose was to investigate the evolution of these WM changes with diffusion weighted/tensor imaging (DWI/DTI) and MR Spectroscopy (MRS). METHODS: A newly diagnosed patient with HDLS from the original Swedish family was followed prospectively with 5 MRI as well as DWI/DTI and MRS examinations during 16 months. RESULTS: The DTI eigenvalues demonstrated changes that suggested early myelin and axonal disturbances in the normal appearing WM (NAWM). DWI/DTI showed a rim of decreased diffusion progressively expanding through the WM from the initial frontal periventricular zones, and indicated complete destruction of axons and myelin in the area behind the front. MRS findings were suggestive of axonal destruction in the NAWM. CONCLUSION: We describe HDLS changes in three temporal stages of development corresponding to lesions outside, in the vicinity of, and behind a characteristic rim centrifugally progressing from the ventricular horns. The axonal disturbances indicated by MRS changes in the NAWM support a primary axonal degeneration, as proposed in the original HDLS report, rather than axonal degeneration secondary to demyelination. These findings could help in differential diagnosis of HDLS.

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