Classification of Childhood White Matter Disorders Using Proton MR Spectroscopic Imaging

Bizzi, Alberto; Castelli, Gianmarco; Bugiani, Marianna; Barker, Peter B.; Herskovits, Edward H.; Danesi, U.; Erbetta, Alessandra; Moroni, Isabella; Farina, Laura; Uziel, Graziella

doi:10.3174/ajnr.a1106

Cited by 48 publications

(38 citation statements)

References 16 publications

(22 reference statements)

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“…Generally, demyelinating disorders showed increased Cho values, while hypomyelinating disorders showed a normal or decreased level, and white matter rarefaction diseases showed decreased values. [14][15][16] Thus, the decreased level of Cho in the white matter in our patients points predominantly to hypomyelination and/or white matter rarefaction, rather than active demyelination, in agreement with our MR imaging findings. The metabolic profile of NAA in gray and white matter suggests a decrease in neuronal and axonal attenuation in all types of CS, and as in PelizaeusMerzbacher disease, this may be related to secondary neuroaxonal degeneration as well.…”

Section: Neuroimaging Diagnosis Of Cssupporting

confidence: 80%

Neuroimaging In Cockayne Syndrome

Koob¹,

Laugel²,

Durand³

et al. 2010

AJNR Am J Neuroradiol

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SUMMARY:CS is an autosomal recessive multisystem disorder, which is mainly characterized by neurologic and sensory impairment, cachectic dwarfism, and photosensitivity. We describe the neuroimaging features (MR imaging, 1 H-MR spectroscopy, and CT) in the various clinical subtypes of CS from a cohort of genetically and biochemically proved cases. Hypomyelination, calcifications, and brain atrophy were the main imaging features. Calcifications were typically found in the putamen and less often in the cortex and dentate nuclei. Severe progressive atrophy was seen in the supratentorial white matter, the cerebellum, the corpus callosum, and the brain stem. Patients with early-onset disease displayed more severe hypomyelination and prominent calcifications in the sulcal depth of the cerebral cortex, but atrophy was less severe in late-onset patients. On proton MR spectroscopy, lactate was detected and Cho and NAA values were decreased. These combined neuroradiologic findings can help in the differential diagnosis of CS, distinguishing it from other leukoencephalopathies and/or cerebral calcifications in childhood., ABBREVIATIONS: Cho ϭ choline; Cr ϭ creatine; CS ϭ Cockayne syndrome; COFS ϭ cerebro-oculofacio-skeletal syndrome; FLAIR ϭ fluid-attenuated inversion recovery; NAA ϭ N-acetylaspartate

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Section: Neuroimaging Diagnosis Of Cssupporting

confidence: 80%

Neuroimaging In Cockayne Syndrome

Koob¹,

Laugel²,

Durand³

et al. 2010

AJNR Am J Neuroradiol

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“…MR spectroscopy plays an important role alone (104) or in addition to other semiquantitative MR techniques (105) in the differential diagnosis of hereditary leukoencephalopathies. MR spectroscopy provides valuable information about tissue pathophysiology for at least three different metabolic profiles: (a) hypomyelination, (b) white matter rarefaction, and (c) demyelination, which were differentiated with tCho/ tCr and tNAA/tCr ratios in a study of 70 children (104).…”

Section: Demyelinating Diseasesmentioning

confidence: 99%

“…MR spectroscopy provides valuable information about tissue pathophysiology for at least three different metabolic profiles: (a) hypomyelination, (b) white matter rarefaction, and (c) demyelination, which were differentiated with tCho/ tCr and tNAA/tCr ratios in a study of 70 children (104).…”

Section: Demyelinating Diseasesmentioning

confidence: 99%

Clinical Proton MR Spectroscopy in Central Nervous System Disorders

Öz¹,

Alger²,

Barker³

et al. 2014

Radiology

532

356

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A large body of published work shows that proton (hydrogen 1 [ 1 H]) magnetic resonance (MR) spectroscopy has evolved from a research tool into a clinical neuroimaging modality. Herein, the authors present a summary of brain disorders in which MR spectroscopy has an impact on patient management, together with a critical consideration of common data acquisition and processing procedures. The article documents the impact of 1 H MR spectroscopy in the clinical evaluation of disorders of the central nervous system. The clinical usefulness of 1 H MR spectroscopy has been established for brain neoplasms, neonatal and pediatric disorders (hypoxia-ischemia, inherited metabolic diseases, and traumatic brain injury), demyelinating disorders, and infectious brain lesions. The growing list of disorders for which 1 H MR spectroscopy may contribute to patient management extends to neurodegenerative diseases, epilepsy, and stroke. To facilitate expanded clinical acceptance and standardization of MR spectroscopy methodology, guidelines are provided for data acquisition and analysis, quality assessment, and interpretation. Finally, the authors offer recommendations to expedite the use of robust MR spectroscopy methodology in the clinical setting, including incorporation of technical advances on clinical units.q RSNA, 2014 Online supplemental material is available for this article. G.O. (e-mail: gulin@cmrr.umn.edu). 2 The complete list of authors and affiliations is at the end of this article.q RSNA, 2014 Note: This copy is for your personal non-commercial use only. To order presentation-ready copies for distribution to your colleagues or clients, contact us at www.rsna.org/rsnarights. Radiology H MR Spectrum of the Brain: Metabolites and Their Biomarker PotentialMR spectroscopy provides a very different basic "readout" than MR imaging, namely a spectrum rather than an techniques were developed. These early localization techniques included pointresolved spectroscopy (PRESS) (1,2) and stimulated echo acquisition mode (STEAM) (3), methods that are now widely used in clinical MR spectroscopy applications.Preliminary studies revealed large differences in metabolite levels in acute stroke (4), chronic multiple sclerosis (5), and brain tumors compared with healthy brain (6). Although this work stimulated a surge of interest in 1 H MR spectroscopy for diagnosing and assessing CNS disorders during the early days of the "Decade of the Brain" (1990)(1991)(1992)(1993)(1994)(1995)(1996)(1997)(1998)(1999), many suboptimal patient studies (7) and the lack of consistent guidelines have led to a situation where, 20 years later, MR spectroscopy is still considered an "investigational technique" by some medical professionals and health care organizations. However, the ability to make an early, noninvasive diagnosis or to increase confidence in a suspected diagnosis is highly valued by patients and clinicians alike. As a result, an increasing number of imaging centers are incorporating MR spectroscopy into their clinical protocols for brain...

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“…1 A multivoxel MRS study demonstrated that metabolite ratios were similar for leukoencephalopathies, with a similar pathophysiology, and that these ratios may help in the classification of white matter abnormalities. 29 Apart from ADC, FA, and MTR being general measures for tissue damage, we searched for more specific correlations between them and histopathologic parameters (myelin density, axonal density, degree of gliosis, and cell density). We studied all parameters in the same brain sections.…”

Section: Prediction Of Histopathology By Mr Imaging Parametersmentioning

confidence: 99%

Correlating Quantitative MR Imaging with Histopathology in X-Linked Adrenoleukodystrophy

Voorn¹,

Pouwels²,

Powers³

et al. 2011

AJNR Am J Neuroradiol

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BACKGROUND AND PURPOSE:Quantitative MR imaging techniques may improve the pathologic specificity of MR imaging regarding white matter abnormalities. Our purposes were to determine whether ADC, FA, MTR, and MRS metabolites correlate with the degree of white matter damage in patients with X-ALD; whether differences in ADC, FA, and MTR observed in vivo are retained in fresh and formalin-fixed postmortem brain tissue; and whether the differences predict histopathology.

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Classification of Childhood White Matter Disorders Using Proton MR Spectroscopic Imaging

Cited by 48 publications

References 16 publications

Neuroimaging In Cockayne Syndrome

Neuroimaging In Cockayne Syndrome

Clinical Proton MR Spectroscopy in Central Nervous System Disorders

Correlating Quantitative MR Imaging with Histopathology in X-Linked Adrenoleukodystrophy

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