Mesial Prefrontal Cortex Degeneration in Amyotrophic Lateral Sclerosis: A High-Field Proton MR Spectroscopy Study

Usman, U.; Choi, Changho; Camicioli, Richard; Seres, Peter; Lynch, Michael; Sekhon, Ramnik; Johnston, Wendy; Kalra, Sanjay

doi:10.3174/ajnr.a2590

Cited by 20 publications

(17 citation statements)

References 35 publications

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“…41 Another study found trends for reductions in NAA and elevations of myo-inositol levels in the mesial prefrontal cortex. 110 Overall, the NAA:myo-inositol ratio was significantly decreased in patients with ALS, but did not correlate with verbal fluency or cognitive performance. Another study reported decreases in the NAA:Cr ratio in the frontal lobe, and these changes correlated with achievement on the Wisconsin Card Sorting Test.…”

Section: Advanced Neuroimaging In Alsmentioning

confidence: 83%

25 years of neuroimaging in amyotrophic lateral sclerosis

2013

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Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease for which a precise cause has not yet been identified. Standard CT or MRI evaluation does not demonstrate gross structural nervous system changes in ALS, so conventional neuroimaging techniques have provided little insight into the pathophysiology of this disease. Advanced neuroimaging techniques—such as structural MRI, diffusion tensor imaging and proton magnetic resonance spectroscopy—allow evaluation of alterations of the nervous system in ALS. These alterations include focal loss of grey and white matter and reductions in white matter tract integrity, as well as changes in neural networks and in the chemistry, metabolism and receptor distribution in the brain. Given their potential for investigation of both brain structure and function, advanced neuroimaging methods offer important opportunities to improve diagnosis, guide prognosis, and direct future treatment strategies in ALS. In this article, we review the contributions made by various advanced neuroimaging techniques to our understanding of the impact of ALS on different brain regions, and the potential role of such measures in biomarker development.

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Section: Advanced Neuroimaging In Alsmentioning

confidence: 83%

25 years of neuroimaging in amyotrophic lateral sclerosis

2013

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“…The presence of frontotemporal lobar degeneration (FTLD) is supported by reduced NAA indices in various frontal regions including the dorsolateral (11, 23) and mesial prefrontal (19, 45) cortices. Mesial prefrontal cortex neurochemistry is abnormal in patients who for the most part are not cognitively impaired, suggesting MRS may be more sensitive to detecting FTLD than clinical measures (45). “Extra-motor” degeneration was similarly demonstrated in the mid-cingulate gyrus (34), thalamus (34, 46), and basal ganglia (46).…”

Section: Resultsmentioning

confidence: 99%

Magnetic Resonance Spectroscopy in ALS

Kalra

2019

Front. Neurol.

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Proton magnetic resonance spectroscopy (MRS) provides a means of measuring cerebral metabolites relevant to neurodegeneration in vivo . In amyotrophic lateral sclerosis (ALS), neurochemical changes reflecting neuronal loss or dysfunction (decreased N-actylaspartate [NAA]) is most significant in the motor cortex and corticospinal tracts. Other neurochemical changes observed include increased myo-inositol (mIns), a putative marker of gliosis. MRS confirmation of involvement of non-motor regions such as the frontal lobes, thalamus, basal ganglia, and cingulum are consistent with the multi-system facet of motor neuron disease with ALS being part of a MND-FTD spectrum. MRS-derived markers exhibit an encouraging discriminatory ability to identify patients from healthy controls, however more data is needed to determine its ability to assist with the diagnosis in early stages when upper motor neuron signs are limited, and in distinguishing from disease mimics. Longitudinal change of NAA and mIns do not appear to be reliable in monitoring disease progression. Technological advances in hardware and high field scanning are increasing the number of accessible metabolites available for interrogation.

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“…Furthermore, the visual cortex has been shown through histologic and MR measures to remain unaffected during the disease process and comparable to normal age‐matched control tissue . While the aPFC was also selected as an internal control initially, there is MR spectroscopic and morphometric evidence to support the involvement of the frontal lobe region in the disease process . In addition, as the control tissue samples were obtained in a previously formalin fixed state, MR images were not able to be obtained from the control tissue to compare with the MR metrics from the fresh nonfixed ALS tissue.…”

Section: Discussionmentioning

confidence: 99%

Histological–MRI correlation in the primary motor cortex of patients with amyotrophic lateral sclerosis

Meadowcroft

Mutic

Bigler

et al. 2014

Magnetic Resonance Imaging

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Purpose The goal of this study was to establish the relationship between ALS histopathology and quantitative MRI metrics. Methods ALS patients (N=8) in advanced stages of the disease were enrolled and, immediately after death, the brain of each patient was removed. Freshly excised ALS tissue was imaged at 3.0 T with T1 and T2 mapping protocols and subsequently stained with astrocyte, myelin, and neuronal markers. Measures of ALS histological stains were compared to the internal control (primary visual cortex) and longitudinal parametric maps. Results Post-mortem T1-weighted images demonstrate diminished contrast between gray and white matter and alterations in T1 relaxation within the primary motor cortex. An increase in astrocyte number and reactivity as well as evident neuronal loss, a decrease in axonal density, and unraveling of the myelin sheaths in subcortical white matter were found in the ALS primary motor cortex (PMC) exhibiting significant T1 relaxation and contrast changes. Conclusion This study provides a histopathological basis for differences in MR T1 contrast and relaxation seen in the ALS brain.

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Mesial Prefrontal Cortex Degeneration in Amyotrophic Lateral Sclerosis: A High-Field Proton MR Spectroscopy Study

Abstract: BACKGROUND AND PURPOSE:Frontotemporal lobar degeneration is responsible for the cognitive abnormalities seen in patients with ALS. We sought to evaluate the in vivo neurochemical changes associated with this pathology indicative of neuronal loss and gliosis.

Cited by 20 publications

References 35 publications

25 years of neuroimaging in amyotrophic lateral sclerosis

25 years of neuroimaging in amyotrophic lateral sclerosis

Magnetic Resonance Spectroscopy in ALS

Histological–MRI correlation in the primary motor cortex of patients with amyotrophic lateral sclerosis

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