Quantifying disease progression in amyotrophic lateral sclerosis

Simon, Neil; Turner, Martin R; Vucic, Steve; Al‐Chalabi, Ammar; Shefner, Jeremy M.; Lomen‐Hoerth, Catherine; Kiernan, Matthew C.

doi:10.1002/ana.24273

Cited by 140 publications

(92 citation statements)

References 122 publications

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“…It is likely that the microenvironment is already compromised at the time symptoms develop and even more so when ALS is diagnosed; therefore, improving the time to diagnosis as a means to achieve earlier institution of therapy is essential in order to confer the best possible opportunity for MN rescue. The development of more robust biomarkers, including electrodiagnostic testing, transcranial magnetic stimulation, and advanced imaging techniques will complement advances in stem cell therapy by allowing earlier detection of disease, as well as more detailed assessments of therapeutic benefits [125,126].…”

Section: Discussionmentioning

confidence: 99%

“…Assessment of MNs, graft survival, and migration of stem cells in vivo, however, is currently not directly possible, making surrogate endpoints essential. The topic of disease measurements has been the subject of a recent review [125], and is also covered elsewhere in this issue of Neurotherapeutics. Hence, while a full discussion of outcome measures is outside the scope of this review, a few points deserve attention.…”

Section: Discussionmentioning

confidence: 99%

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Recent Advances and the Future of Stem Cell Therapies in Amyotrophic Lateral Sclerosis

2015

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Amyotrophic lateral sclerosis is a progressive neurodegenerative disease of the motor neurons without a known cure. Based on the possibility of cellular neuroprotection and early preclinical results, stem cells have gained widespread enthusiasm as a potential treatment strategy. Preclinical models demonstrate a protective role of engrafted stem cells and provided the basis for human trials carried out using various types of stem cells, as well as a range of cell delivery methods. To date, no trial has demonstrated a clear therapeutic benefit; however, results remain encouraging and are the basis for ongoing studies. In addition, stem cell technology continues to improve, and induced pluripotent stem cells may offer additional therapeutic options in the future. Improved disease models and clinical trials will be essential in order to validate stem cells as a beneficial therapy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Recent Advances and the Future of Stem Cell Therapies in Amyotrophic Lateral Sclerosis

2015

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“…FA changes within the CST at baseline may be even able to predict disability at 6 months according to some studies [31][32][33].…”

Section: Dti Imagesmentioning

confidence: 99%

Finding Markers in Amyotrophic Lateral Sclerosis Diagnosis

Hernández¹

2018

J Neurol Neurosci

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Amyotrophic Lateral Sclerosis (ALS) is an uncommon illness, it is caused by moto neuron degeneration, upper, lower and bulbar muscles are affected. Some research also report degeneration in no motor structures of the brain. We proposed to evaluate Electrophysiological and Image techniques like markers in ALS diagnosis and correlate these results. During January 2015 to January 2017, twenty patients with ALS diagnosis and twenty health subjects were evaluated. Sensory and by motor nerve conduction studies, Electromyography, SomatoSensory Evoked Potentials were done to the patients. 3T MRI image were obtained from the patients and from the health subjects. Post processing MRI techniques like voxel based morphometric, diffusion techniques and corticospinal tract and corpus callosum tractography were applied at different levels of the brain structures. Nerve conduction study was positive in 90% of the patients, SSEP were positive in 60% and EMG abnormalities were observed in 100% of patients. Anatomic MRI was positive in 50% of the patients. Fractional Anisotropy was reduced in ALS group in comparison with health group, more significant at cortex, internal capsule and corpus callosum. Fibers number of cortico-spinal tract and corpus callosum were diminished in ALS group in relation to health group. Also grey and white matter were reduce in ALS group, in areas such as: cingulate gyrus, anterior portion of occipital lobe, left caudate and putamen nucleus, right claustrum nucleus, lower and medium temporal gyrus bilateral, left precentral and post-central gyrus, corpus callosum, corticospinal tract, bilateral internal capsule, bilateral optical radiation, bilateral lower longitudinal fascicle, bilateral hippocampal fimbriae, bilateral radiated corona and pontocerebellar fibers. Electrophysiological studies confirmed ALS diagnosis in 100% of cases. MRI methods show abnormalities in motor and not motor structures of brain in ALS patients. They could be markers in early ALS diagnostic.

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“…Simon et al и R.M. Van den Berg-Vos et al [36,42]. Поражение шейного и пояснично-крестцового регионов в сочетании с вовлечением бульбо-псевдо-бульбарного региона у 9 больных с БАС было про-анализировано нами отдельно (см.…”

Section: таблица 1 (окончание)unclassified

Signs of MRI affection of brain in сlassical Amyotrophic Lateral Sclerosis patients

et al. 2016

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1 ГБоу ВПо «Первый санкт-Петербургский государственный медицинский университет им. акад. И.П. Павлова» минздрава России; 2 Городская многопрофильная больница № 2; 3 ГБоу ВПо «санкт-Петербургский государственный педиатрический медицинский университет» минздрава России; 4 ФГБу «Российский научный центр радиологии и хирургических технологий», санкт-Петербург Поступила в редакцию: 18.02.2016 Принята к печати: 28.04.2016Резюме. Из всей группы пациентов c классическим боковым амиотрофическим склерозом (БАС) (n = 423), которым было выполнено МРТ головного мозга, очаги МР-гиперинтенсивного сигнала в Т2 ВИ были обнаружены только у 41 (9,7 %) больного. Женщин было 20 (48,8 %), мужчин -21 (51,2 %), колебания возраста -от 31 до 74 лет (сред-ний возраст -56 ± 4). Выявленные очаги МР-сигнала локализовались исключительно в проекции интракраниаль-ного сегмента кортикоспинального тракта (ПИКС КСТ), были всегда двусторонние, симметричные, располагались на одном или двух уровнях, чаще выявлялись в заднем бедре внутренней капсулы и ножке мозга (85 и 60 % соот-ветственно), реже -в лучистом венце, семиовальном центре и продолговатом мозге. Отсутствовала достоверная взаимосвязь между изменениями на МРТ головного мозга и возрастом начала болезни, длительностью симптомов и регионом начала атрофии и слабости мышц. Очаги гиперинтенсивного МР-сигнала не менялись во времени и про-странстве, не накапливали контраст при прогрессировании заболевания, что не позволяет использовать рутинную МРТ в Т1 и Т2 ВИ для мониторирования течения заболевания, прогноза и эффективности лечения. Мы можем реко-мендовать проведение МРТ головного мозга для диагностики БАС на ранней стадии и исключения других заболе-ваний, схожих с БАС. Однако диагностическая значимость метода МРТ с напряженностью магнитного поля 1,5 тесла для диагностики классического БАС весьма ограниченна.Ключевые слова: боковой амиотрофический склероз; МРТ головного мозга у больных БАС; болезнь моторного нейрона. Abstract. T2 hyperintensive focal lesions were found on brain MRI only in 41 patients (9.7 %) from the ALS group (n = 423). Female were 20 (48,8 %) and male -21 (51,2 %), aged 31 to 78 years (middle age is 56 ± 4). Focal MRI lesions were found exclusively in projection of the intracranial section of the corticospinal tract. They were always bilateral and symmetrical and were located at one or two levels, most often in the posterior limb of internal capsule and in cerebral peduncle (85 % and 60 % correspondingly). They were less frequent in the radiate crown, semioval center or medulla. There was no reliable correlation between MRI changes and clinical type of ALS, age of disease onset, duration of symptoms and region of onset of muscle weakness and atrophy. Foci of hyperintensive MRI signal in the ALS did not change in terms of time and space, and did SIGNS OF MRI AFFECTION OF BR A IN IN СL A SSIC AL AMYOTROPHIC L ATER AL SCLEROSIS PATIENTS

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Quantifying disease progression in amyotrophic lateral sclerosis

Cited by 140 publications

References 122 publications

Recent Advances and the Future of Stem Cell Therapies in Amyotrophic Lateral Sclerosis

Recent Advances and the Future of Stem Cell Therapies in Amyotrophic Lateral Sclerosis

Finding Markers in Amyotrophic Lateral Sclerosis Diagnosis

Signs of MRI affection of brain in сlassical Amyotrophic Lateral Sclerosis patients

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