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Clinical Neurology
Kensuke Ikenaka, Yasuhiro Maeda, Yuji Hotta, Seiichi Nagano, Shinichiro Yamada, Daisuke Ito, Ryota Torii, Keita Kakuda, Harutsugu Tatebe, Naoki Atsuta, Cesar Aguirre, Yasuyoshi Kimura, Kousuke Baba, Takahiko Tokuda, Masahisa Katsuno, Kazunori Kimura, Gen Sobue, Hideki Mochizuki
Summary: This study found that serum ADMA level is associated with the progression and prognosis of amyotrophic lateral sclerosis (ALS) and is an independent biomarker for ALS disease progression and prognosis, reflecting the degree of motor neuron degeneration.
EUROPEAN JOURNAL OF NEUROLOGY
(2022)
Article
Clinical Neurology
Haruhisa Kato, Makiko Naito, Tomoko Saito, Takuto Hideyama, Hiroo Terashi, Shin Kwak, Hitoshi Aizawa
Summary: To clarify the effect of PER on sALS progression, the relationship between ALSFRS-R scores changes and serum PER concentrations was investigated. The study revealed that high concentrations of PER may worsen bulbar symptoms in patients with sALS, implying the potential benefit of measuring serum PER concentrations in these patients.
JOURNAL OF CLINICAL NEUROLOGY
(2023)
Article
Clinical Neurology
Alessandro Introna, Giammarco Milella, Antonella Morea, Maria Ucci, Angela Fraddosio, Stefano Zoccolella, Eustachio D'Errico, Isabella Laura Simone
Summary: The study estimated the predictive and prognostic role of Kings College clinical stage progression rate in survival of ALS patients, finding that higher values of Delta KC were associated with an increased risk of survival event.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Maurizio A. Leone, Jessica Mandrioli, Sergio Russo, Aliona Cucovici, Giulia Gianferrari, Vitalie Lisnic, Dafin Fior Muresanu, Francesco Giuliani, Massimiliano Copetti, Andrea Fontana
Summary: This study suggests that the neutrophil-to-lymphocyte ratio (NLR) could be a potential marker for disease progression and survival in ALS, possibly associated with low-grade inflammation. Further prospective studies are needed to validate these findings.
Article
Clinical Neurology
Huagang Zhang, Lu Chen, Jinzhou Tian, Dongsheng Fan
Summary: By analyzing the association of disease progression and survival in bulbar onset ALS patients, a cutoff duration of 20 months from bulbar onset to first significant limb involvement was found to distinguish IBP from typical bulbar onset ALS effectively. IBP is characterized by a female predominance, relative preservation of limb function, more pure UMN bulbar signs, and a relatively benign prognosis compared to typical bulbar onset ALS.
Article
Neurosciences
Tyler L. Wells, Jacob R. Myles, Turgay Akay
Summary: ALS is a neurodegenerative disease resulting in progressive motor neuron death and typically fatal within 5 years of diagnosis. Research indicates that silencing C-boutons in ALS mouse models can improve muscle innervation and behavioral capabilities, suggesting that manipulating these synapses in combination with targeted training programs could be beneficial for ALS patients, leading to improved mobility and quality of life.
JOURNAL OF NEUROSCIENCE
(2021)
Article
Clinical Neurology
Yuko Kobayakawa, Koji Todaka, Yu Hashimoto, Senri Ko, Wataru Shiraishi, Junji Kishimoto, Jun-ichi Kira, Ryo Yamasaki, Noriko Isobe
Summary: The study developed a new indicator, FVC-DiP, to assess disease progression rate in ALS. This indicator reflected disease progression patterns through %FVC decline, and showed associations with prognosis and TRICALS risk profile.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2022)
Article
Multidisciplinary Sciences
Radhika Khosla, Manjari Rain, Suresh Sharma, Akshay Anand
Summary: ALS is a degenerative disorder of motor neurons, and early diagnosis is crucial for predicting the disease and its progression. The study presents a predictive statistical model for ALS using plasma and CSF biomarkers, showing good validity, sensitivity, and specificity.
Article
Biochemistry & Molecular Biology
Ozlem Yildiz, Johannes Schroth, Vittoria Lombardi, Valentina Pucino, Yoana Bobeva, Ping Kei Yip, Klaus Schmierer, Claudio Mauro, Timothy Tree, Sian Mari Henson, Andrea Malaspina
Summary: Monocytes expressing active CD11b have been found to regulate neuroinflammation and affect clinical outcomes in amyotrophic lateral sclerosis (ALS). The frequency of active CD11b+ monocytes increased significantly during ALS progression, and higher frequencies of CD11b+ monocytes at baseline were associated with slower disease progression. Additionally, the levels of active CD11b+ monocytes and non-classical monocytes (NCM) in the blood were predictive of clinical outcomes and survival in ALS patients.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Clinical Neurology
Claudia Santos Silva, Miguel Oliveira Santos, Marta Gromicho, Susana Pinto, Michael Swash, Mamede de Carvalho
Summary: Most PLS patients exhibit minor and stable EMG abnormalities, but those with more EMG abnormalities show a faster disease progression.
CLINICAL NEUROPHYSIOLOGY
(2021)
Article
Clinical Neurology
Axel Freischmidt, Anand Goswami, Katharina Limm, Vitaly L. Zimyanin, Maria Demestre, Hannes Glass, Karlheinz Holzmann, Anika M. Helferich, Sarah J. Brockmann, Priyanka Tripathi, Alfred Yamoah, Ina Poser, Peter J. Oefner, Tobias M. Boeckers, Eleonora Aronica, Albert C. Ludolph, Peter M. Andersen, Andreas Hermann, Joachim Weis, Joerg Reinders, Karin M. Danzer, Jochen H. Weishaupt
Summary: Knowledge about converging disease mechanisms in ALS is limited, but the study revealed that downregulation of specific serum microRNAs in ALS patients may be linked to the interaction of fragile X-related proteins with these microRNAs. The aberrant expression and co-aggregation of these proteins with FUS in ALS patients suggest their involvement in the disease pathology, highlighting their potential role as biomarkers or therapeutic targets for ALS. This study emphasizes the systemic aspect of ALS and the significance of using blood microRNAs for translational research to uncover disease mechanisms in ALS.
Article
Neurosciences
Mengmeng Wang, Dandan Liu, Zhizhong Zhang, Wei Xie, Liping Cao, Linfeng Zhu, Meng Liu, Shiying Sheng, Xuegan Lian
Summary: This study utilizing Mendelian randomization found that genetically predicted serum creatinine (sCr) is inversely associated with the risk of amyotrophic lateral sclerosis (ALS) in the European population but not in the East Asian population.
MOLECULAR NEUROBIOLOGY
(2021)
Article
Neurosciences
Rebecca Stevenson, Evgeniia Samokhina, Armaan Mangat, Ilaria Rossetti, Sushmitha S. Purushotham, Chandra S. Malladi, John W. Morley, Yossi Buskila
Summary: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the progressive loss of motor neurons. Recent studies have shown that astrocytes, a type of glial cells, also contribute to the onset and progression of ALS. This study investigates the role of astrocytes in maintaining K+ homeostasis in the brain and demonstrates region-specific alterations in K+ clearance rate in an ALS mouse model. The findings suggest that impaired astrocytic function may contribute to the vulnerability of motor neurons in ALS.
Article
Clinical Neurology
Marta Gromicho, Tiago Leao, Miguel Oliveira Santos, Susana Pinto, Alexandra M. Carvalho, Sara C. Madeira, Mamede De Carvalho
Summary: This study applies dynamic Bayesian networks (DBNs) to analyze the influence of clinical and demographic variables on disease progression rate in amyotrophic lateral sclerosis (ALS) patients. The results show that disease duration and body mass index are the most influential static variables, while maximum expiratory pressure has the largest influence on all progression groups. Additionally, different ALSFRS-R scores and sub-scores have varying degrees of influence on each progression group.
EUROPEAN JOURNAL OF NEUROLOGY
(2022)
Article
Clinical Neurology
Aliona Cucovici, Andrei Ivashynka, Andrea Fontana, Sergio Russo, Letizia Mazzini, Jessica Mandrioli, Vitalie Lisnic, Dafin Fior Muresanu, Maurizio Angelo Leone
Summary: The study found no clear association between coffee or tea consumption and the progression rate of ALS.
FRONTIERS IN NEUROLOGY
(2021)