Brain
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            -acetylaspartate levels correlate  with motor function in  metachromatic leukodystrophy

Dali, Christine í; Hanson, Lars G.; Barton, Norman W.; Fogh, Jørgen; Nair, Nitin; Lund, Allan M.

doi:10.1212/wnl.0b013e3181feb217

Cited by 38 publications

(39 citation statements)

References 38 publications

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“…In the evaluation of therapeutic effects and cerebral changes on follow‐up, the demyelination load was already demonstrated to be more sensitive than the MRI scoring system 8, 28. Still, the sensitivity and specificity of new (more myelin‐specific) MRI parameters might be important to evaluate further in detecting functionally relevant WM changes in the brain as suggested recently, for example, diffusion‐weighted imaging, MR spectroscopy, magnetization transfer imaging, myelin water imaging, cortical thickness determination, and GM/WM volumetry 12, 29, 30, 31…”

Section: Discussionmentioning

confidence: 99%

Demyelination load as predictor for disease progression in juvenile metachromatic leukodystrophy

Strölin

Krägeloh‐Mann

Kehrer

et al. 2017

Ann Clin Transl Neurol

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ObjectiveThe aim of this study was to investigate whether the extent and topography of cerebral demyelination correlates with and predicts disease progression in patients with juvenile metachromatic leukodystrophy (MLD).MethodsA total of 137 MRIs of 46 patients with juvenile MLD were analyzed. Demyelination load and brain volume were quantified using the previously developed Software “clusterize.” Clinical data were collected within the German Leukodystrophy Network and included full scale intelligence quotient (FSIQ) and gross motor function data. Voxel‐based lesion‐symptom mapping (VLSM) across the whole brain was performed to investigate the spatial relationship of cerebral demyelination with motor or cognitive function. The prognostic value of the demyelination load at disease onset was assessed to determine the severity of disease progression.ResultsThe demyelination load (corrected by the individual brain volume) correlated significantly with gross motor function (r = +0.55) and FSIQ (r = −0.55). Demyelination load at disease onset was associated with the severity of disease progression later on (P < 0.01). VLSM results associated frontal lobe demyelination with loss in FSIQ and more central region demyelination with decline of motor function. Especially progression of demyelination within the motor area was associated with severe disease progression.InterpretationWe were able to show for the first time in a large cohort of patients with juvenile MLD that the demyelination load correlates with motor and cognitive symptoms. Moreover, demyelination load at disease onset, especially the involvement of the central region, predicts severity of disease progression. Thus, demyelination load seems a functionally relevant MRI parameter.

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Section: Discussionmentioning

confidence: 99%

Demyelination load as predictor for disease progression in juvenile metachromatic leukodystrophy

Strölin

Krägeloh‐Mann

Kehrer

et al. 2017

Ann Clin Transl Neurol

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“…In patients who are newly diagnosed with juvenile or adult metachromatic leukodystrophy, a combination of MR imaging and MR spectroscopy can be used to judge the state of brain tissue inflammation (109,110). Although mIns is typically increased even in the early stages of metachromatic leukodystrophy, as long as tNAA is still within the normal range, hematopoietic stem cell transplantation is indicated (111,112).…”

Section: Special Review: Clinical Proton Mr Spectroscopy In Central Nmentioning

confidence: 99%

Clinical Proton MR Spectroscopy in Central Nervous System Disorders

Öz¹,

Alger²,

Barker³

et al. 2014

Radiology

559

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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“…The MR spectroscopic profile of MLD is well-known 17 ; for short TEs (20 -40 ms), there is a reduction in the NAA peak, reflecting neuronal distress, along with a significant increase in the myo-inositol peak, which may correspond to either galactosylceramide accumulation or secondary glial activation. The study by Dali et al 18 suggests that the NAA level may be a sensitive disease marker, correlated to clinical motor and cognitive function performance, which can be used to assess treatment efficacy. The lactate resonance described 19 may correspond to anaerobic metabolism from myelin destruction and the elevation in Cho to cell membrane degradation (due to the accumulation of sulfatides).…”

Section: Mr Spectroscopymentioning

confidence: 99%

Toward a Better Understanding of Brain Lesions during Metachromatic Leukodystrophy Evolution

Martin¹,

Sevin²,

Lazarus

et al. 2012

AJNR Am J Neuroradiol

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Brain N -acetylaspartate levels correlate with motor function in metachromatic leukodystrophy

Abstract: We suggest that NAA levels could serve as a sensitive biomarker in children with MLD. Proton MRS may provide a valuable tool for measuring the effects of treatment interventions in this disorder.

Cited by 38 publications

References 38 publications

Demyelination load as predictor for disease progression in juvenile metachromatic leukodystrophy

Demyelination load as predictor for disease progression in juvenile metachromatic leukodystrophy

Clinical Proton MR Spectroscopy in Central Nervous System Disorders

Toward a Better Understanding of Brain Lesions during Metachromatic Leukodystrophy Evolution

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