Role of diffusion tensor imaging or magnetic resonance spectroscopy in the diagnosis and disability assessment of amyotrophic lateral sclerosis

Liu, Chanchan; Jiang, Rifeng; Yi, Xiaoou; Zhu, Wenzhen; Bu, Bitao

doi:10.1016/j.jns.2014.12.004

Cited by 15 publications

(14 citation statements)

References 20 publications

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“…The DTI evaluation surprisingly evidenced bilateral impairment of CST diffusion metrics, with clear right-left asymmetry, pointing to a neurodegenerative etiology (15), which clinically appeared less likely at that time. Then, the marked reduction of NAA/Cho + Cr ratio in the MC shown by MRS further supported the hypothesis of UMN degeneration (6)(7)(8).…”

Section: Discussionsupporting

confidence: 60%

“…It may raise the suspect of a MND at the very beginning, especially if presenting in an insidious way, but it is essential to exclude other more likely diagnostic possibilities (i.e., lesional causes) and to consider the progression of the syndrome over time. The role of MR in confirming an extensive UMN involvement of possible degenerative etiology has been highlighted by previous works (6)(7)(8). Moreover, the evaluation of white matter abnormalities by diffusion tensor imaging (DTI) proved useful for distinguishing between ALS and PLS (9,10).…”

Section: Introductionmentioning

confidence: 98%

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Diagnostic contribution of magnetic resonance imaging in an atypical presentation of motor neuron disease

Ugga

Coppola

Cocozza

et al. 2017

Quant. Imaging Med. Surg.

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Motor neuron disease (MND) is a neurodegenerative disease determining progressive and relentless motor deterioration involving both upper and lower motor neurons (UMN and LMN); several variants at onset are described. Here we describe a case of MND presenting as pure spastic monoparesis in which magnetic resonance imaging (MRI) gave a substantial contribution in confirming the diagnosis and assessing the severity of UMN involvement. An isolated pyramidal syndrome, with complete absence of LMN signs, is a rare phenotype in the context of MND (less than 4% of total cases), especially if restricted to only one limb. Several other elements made this case an unusual presentation of MND: the late age of onset (8 th decade), the subacute evolution of symptoms (raising the suspicion of an ischemic or inflammatory, rather than degenerative, etiology), the patient's past medical history (achalasia, erythema nodosum), the increase of inflammatory indices. Conventional MRI showed no focal lesions that could explain the clinical features; therefore, we used advanced MR sequences. Diffusion tensor imaging (DTI) evaluation evidenced bilateral impairment of corticospinal tract (CST) diffusion metrics, with clear right-left asymmetry, pointing to a neurodegenerative etiology, which clinically appeared less likely at that time. Magnetic resonance spectroscopy (MRS) showed a significant reduction of NAA/Cho + Cr ratio in the motor cortex (MC), further supporting the hypothesis of UMN degeneration. In conclusion, in this particular case of MND, whose nosographic framing has not been fully defined, advanced MRI techniques with DTI and MRS proved to be of great usefulness in confirming a diffuse UMN involvement, possibly at a more advanced stage than its clinical expression.

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Section: Discussionsupporting

confidence: 60%

Section: Introductionmentioning

confidence: 98%

Diagnostic contribution of magnetic resonance imaging in an atypical presentation of motor neuron disease

Ugga

Coppola

Cocozza

et al. 2017

Quant. Imaging Med. Surg.

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“…Reduction in neocortex volume, GM and WM volume and brain parenchymal fraction.GM volume reduction is most important in the motor cortex of the contralateral hemisphere of the limb of onset. ALSFRS is positively correlated with GM density in the left postcentral cortex and DPR is negatively correlated with GM density in the right precentral cortexZhu et al, [31, 87]22/22GM reduction in the right and left precentral cortex.Negative with DD, DPR and all other clinical parameters. GM grey matter, WM white matter, BA Brodman area, DD disease duration, DPR Disease progression rate, UMN upper motor neuron…”

Section: Resultsmentioning

confidence: 99%

“…NAA and Cr were correlated in left MCCorrelation between ins in MC and UMN disability, negative correlation between Naa in MC and UMN disability, higher Cho in sever UMN disability groupCervo et al, [90]84/28NAA/(Cho + Cr) was low in the MCNegativeFoerster et al, [95]10/9Low gamma aminobutyric acid in the MC but not in WMNegativeFoerster et al, [55]29/30NAA and gamma aminobutyric acid were low and ins was elevated in the left MCNegative correlation between gamma aminobutyric acid in MC and DD, correlation between Naa peak in MC and ALSFRS-RGovind et al, [92]38/70NAA was low and Cho was elevated in most parts of CST, and Cho/NAA was elevated in all parts of the CSTsNegative correlation between Cho/Naa in the left entire CST and forced vital capacity, negative correlation between Cho/Naa in the left CST and right and left finger tap rate, negative correlation between Cho/Naa in left MC and semiovale centrum and right finger tape or forced vital capacityHan et al, [85]15/15NAA/Cr peak was low in the MC and PLIC, Glu/Cr and Glu + Gln/Cr peaks were low in the MC and PLICNegative correlation between Glu + Gln/Cr with Norris scoreKalra et al, [88, 89]63/18NAA/Cho and NAA/Cr was low in the MC, and Cho/Cr was elevated in the MCRelation between decreased Naa/Cho in the MC and reduced survival.Kalra et al, [88, 89]17/15NAA/Ins, NAA/Cr and NAA/Cho were low in the MC, and Ins/Cr was elevated in the MCNegative (p ≤ 0.05)Liu et al, [87]19/13NAA/Cr was low in the MCNegativeLombardo et al, [79]32/19NAA/Cr was low and ins/Cr and Cho/Cr were elevated in the MC.Abnormalities were correlated with the El Escorial scorePohl et al, [91]70/48NAA, Pcr + Cr, NAA/Cho and NAA/(Pcr + Cr) were low in the MC. At 12 months, NAA/Cho was low and Cho/(Pcr + Cr) was elevatedNot availablePyra et al, [59]14/14NAA/Cho and NAA/Cr were low in the MC and corona radiata, and Cho/Cr was elevated in the MCCorrelation between Naa/Cho peak in MC and DD, negative correlation between Naa/Cho in MC and corona radiata and DPRRooney et al, [86]10/9NAA/(Cho + Cr) was low in the MC and CST but not in other regionsCorrelation between Naa/(Cho + Cr) in the MC and maximum finger tape rateStagg et al, [47]13/14…”

Section: Resultsmentioning

confidence: 99%

The value of magnetic resonance imaging as a biomarker for amyotrophic lateral sclerosis: a systematic review

et al. 2016

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BackgroundAmyotrophic lateral sclerosis (ALS) is a fatal, rapidly progressive neurodegenerative disease that mainly affects the motor system. A number of potentially neuroprotective and neurorestorative disease-modifying drugs are currently in clinical development. At present, the evaluation of a drug’s clinical efficacy in ALS is based on the ALS Functional Rating Scale Revised, motor tests and survival. However, these endpoints are general, variable and late-stage measures of the ALS disease process and thus require the long-term assessment of large cohorts. Hence, there is a need for more sensitive radiological biomarkers. Various sequences for magnetic resonance imaging (MRI) of the brain and spinal cord have may have value as surrogate biomarkers for use in future clinical trials. Here, we review the MRI findings in ALS, their clinical correlations, and their limitations and potential role as biomarkers.MethodsThe PubMed database was screened to identify studies using MRI in ALS. We included general MRI studies with a control group and an ALS group and longitudinal studies even if a control group was lacking.ResultsA total of 116 studies were analysed with MRI data and clinical correlations. The most disease-sensitive MRI patterns are in motor regions but the brain is more broadly affected.ConclusionDespite the existing MRI biomarkers, there is a need for large cohorts with long term MRI and clinical follow-up. MRI assessment could be improved by standardized MRI protocols with multicentre studies.

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“…Increased Cho and Cho/Cr levels have been reported in people with ALS [15, 22–24] in some studies but not others [16, 21, 25, 26]. Elevated mI and mI/Cr have been detected in the motor cortex in people with ALS [15, 22, 27, 28] but not in early-stage ALS [29].…”

Section: Introductionmentioning

confidence: 99%

Ultra high-field (7tesla) magnetic resonance spectroscopy in Amyotrophic Lateral Sclerosis

Atassi

Triantafyllou

et al. 2017

PLoS ONE

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The main objective of this study was to utilize high field (7T) in vivo proton magnetic resonance imaging to increase the ability to detect metabolite changes in people with ALS, specifically, to quantify levels of glutamine and glutamine separately. The second objective of this study was to correlate metabolic markers with clinical outcomes of disease progression. 13 ALS participants and 12 age-matched healthy controls (HC) underwent 7 Tesla MRI and MRS. Single voxel MR spectra were acquired from the left precentral gyrus using a very short echo time (TE = 5 ms) STEAM sequence. MRS data was quantified using LCModel and correlated to clinical outcome markers. N-acetylaspartate (NAA) and total NAA (tNA, NAA + NAAG) were decreased by 17% in people with ALS compared to HC (P = 0.004 and P = 0.005, respectively) indicating neuronal injury and/or loss in the precentral gyrus. tNA correlated with disease progression as measured by forced vital capacity (FVC) (P = 0.014; Rρ = 0.66) and tNA/tCr correlated with overall functional decline as measured by worsening of the ALS Functional Rating Scale-Revised (ALSFRS-R) (P = 0.004; Rρ = -0.74). These findings underscore the importance of NAA as a reliable biomarker for neuronal injury and disease progression in ALS. Glutamate (Glu) was 15% decreased in people with ALS compared to HC (P = 0.02) while glutamine (Gln) concentrations were similar between the two groups. Furthermore, the decrease in Glu correlated with the decrease in FVC (P = 0.013; Rρ = 0.66), a clinical marker of disease progression. The decrease in Glu is most likely driven by intracellular Glu loss due to neuronal loss and degeneration. Neither choline containing components (Cho), a marker for cell membrane turnover, nor myo-Inositol (mI), a suspected marker for neuroinflammation, showed significant differences between the two groups. However, mI/tNA was correlated with upper motor neuron burden (P = 0.004, Rρ = 0.74), which may reflect a relative increase of activated microglia around motor neurons. In summary, 7T 1H MRS is a powerful non-invasive imaging technique to study molecular changes related to neuronal injury and/or loss in people with ALS.

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Role of diffusion tensor imaging or magnetic resonance spectroscopy in the diagnosis and disability assessment of amyotrophic lateral sclerosis

Cited by 15 publications

References 20 publications

Diagnostic contribution of magnetic resonance imaging in an atypical presentation of motor neuron disease

Diagnostic contribution of magnetic resonance imaging in an atypical presentation of motor neuron disease

The value of magnetic resonance imaging as a biomarker for amyotrophic lateral sclerosis: a systematic review

Ultra high-field (7tesla) magnetic resonance spectroscopy in Amyotrophic Lateral Sclerosis

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