Article
Clinical Neurology
Francesco Gentile, Alessio Maranzano, Federico Verde, Veronica Bettoni, Eleonora Colombo, Alberto Doretti, Marco Olivero, Francesco Scheveger, Claudia Colombrita, Ilaria Bulgarelli, Edoardo Gioele Spinelli, Erminio Torresani, Stefano Messina, Luca Maderna, Federica Agosta, Claudia Morelli, Massimo Filippi, Vincenzo Silani, Nicola Ticozzi
Summary: This study investigates whether routine blood parameters can provide useful biomarkers for amyotrophic lateral sclerosis (ALS). The results show that creatinine is a reliable biomarker, associated with clinical features, disability, and survival in ALS patients. Markers of nutrition/inflammation may offer additional prognostic information, and AST and chloride could assist in predicting disease progression rate and survival.
JOURNAL OF NEUROLOGY
(2023)
Review
Medicine, General & Internal
Wei-Ming Su, Xiao-Jing Gu, Qing-Qing Duan, Zheng Jiang, Xia Gao, Hui-Fang Shang, Yong-Ping Chen
Summary: This study conducted a network meta-analysis and systematic review to summarize the genetic factors associated with the survival of ALS. Variants in ATXN2, C9orf72, FUS, UNC13A, and ZNF521B were found to be associated with shorter survival, while SOD1, TARDBP, TBK1, NEK1, UBQLN2, CCNF, APOE epsilon 4, and KIFAP3 did not show any effect on ALS survival. The findings contribute to our understanding of ALS pathogenesis and can guide clinical trials and drug development.
Article
Clinical Neurology
T. Dharmadasa, J. Howells, J. M. Matamala, N. G. Simon, D. Burke, S. Vucic, M. C. Kiernan
Summary: In amyotrophic lateral sclerosis, patients with inexcitability of all four limbs to transcranial magnetic stimulation have a distinct clinical profile characterized by younger age, lower-limb onset, faster disease progression, greater functional disability, and reduced survival. This measure may serve as an important prognostic marker in the disease.
EUROPEAN JOURNAL OF NEUROLOGY
(2021)
Article
Clinical Neurology
Rosario Vasta, Maria Claudia Torrieri, Fabrizio D'Ovidio, Alberta Circiello, Filippo De Mattei, Umberto Manera, Antonio Canosa, Andrea Calvo, Adriano Chio, Cristina Moglia
Summary: The study found that neck muscle weakness at diagnosis in ALS patients can predict survival and respiratory impairment, providing valuable insights for patient interventions and clinical trial design.
EUROPEAN JOURNAL OF NEUROLOGY
(2021)
Article
Chemistry, Medicinal
Hideki Houzen, Takahiro Kano, Kazuhiro Horiuchi, Masahiro Wakita, Azusa Nagai, Ichiro Yabe
Summary: The long-term survival effect of edaravone in patients with ALS was investigated in a single-center retrospective study. The study showed that edaravone treatment could potentially prolong survival in ALS patients compared to those who did not receive the treatment. No serious adverse effects were reported in the edaravone group during the study period.
Article
Clinical Neurology
Yahui Zhu, Yunyun Huo, Jiongming Bai, Mao Li, Hongfen Wang, Jiao Wang, Xusheng Huang
Summary: This study aimed to investigate the relationship between serum Cystatin C (CysC) and survival in ALS patients. The results showed that ALS patients with high CysC levels had shorter median survival than those with lower CysC levels, and serum CysC level might be an independent predictor of ALS survival.
NEUROLOGICAL SCIENCES
(2023)
Article
Clinical Neurology
Qi-Fu Guo, Wei Hu, Liu-Qing Xu, Hao Luo, Ning Wang, Qi-Jie Zhang
Summary: Serum creatinine is a reliable prognostic marker for ALS, with lower levels predicting poor prognosis and shorter survival in both male and female patients. Serum creatinine levels are positively correlated with ALSFRS-R scores and inversely correlated with the decline rate of ALSFRS-R per month. Additionally, higher decline rates of creatinine per month are associated with significantly shorter survival during the disease progression.
ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY
(2021)
Article
Clinical Neurology
Shotaro Haji, Wataru Sako, Nagahisa Murakami, Yusuke Osaki, Takahiro Furukawa, Yuishin Izumi, Ryuji Kaji
Summary: The meta-analysis showed that serum UA level could be a prognostic biomarker in ALS patients. The studies included were homogeneous, with sensitivity analysis and trim-and-fill method supporting the results' robustness. Publication bias was detected and adjusted for, confirming significant summary effects.
CLINICAL NEUROLOGY AND NEUROSURGERY
(2021)
Article
Clinical Neurology
Liu-Qing Xu, Wei Hu, Qi-Fu Guo, Guo-Rong Xu, Ning Wang, Qi-Jie Zhang
Summary: The study found a survival advantage in male ALS patients with higher baseline serum uric acid levels, compared to female patients. Shorter diagnostic delay, lower BMI, faster disease progression, and lower baseline uric acid levels were associated with shorter survival in males.
FRONTIERS IN NEUROLOGY
(2021)
Article
Biochemistry & Molecular Biology
Stefano Zoccolella, Alessia Giugno, Giammarco Milella, Marco Filardi, Alessandro Introna, Angela Fraddosio, Eustachio D'Errico, Valentina Gnoni, Ludovica Tamburrino, Daniele Urso, Francesca Caputo, Salvatore Misceo, Giancarlo Logroscino
Summary: This study proposed a new scoring scale to assess the bulbar lower motor neurons (LMN) involvement in amyotrophic lateral sclerosis (ALS) and evaluated its prognostic value. The results showed that this scale had good reliability in assessing ALS and could provide unique information in phenotyping and predicting survival.
Article
Nutrition & Dietetics
Juan J. Lopez-Gomez, Maria D. Ballesteros-Pomar, Beatriz Torres-Torres, Begona De la Maza, Maria A. Penacho-Lazaro, Jose M. Palacio-Mures, Cristina Abreu-Padin, Irene Sanz-Gallego, Daniel A. De Luis-Roman
Summary: This study evaluated the influence of PEG implantation on survival and complications in ALS patients, showing that early implantation of PEG can improve nutritional status and prolong survival time.
Article
Cardiac & Cardiovascular Systems
Ana Catarina Pronto-Laborinho, Catarina S. Lopes, Vasco A. Conceicao, Marta Gromicho, Nuno C. Santos, Mamede de Carvalho, Filomena A. Carvalho
Summary: The study found a positive association between gamma' fibrinogen concentration and survival in ALS patients, with those having higher levels surviving longer, independent of other factors. This novel biomarker shows promise for further research in ALS.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2021)
Article
Public, Environmental & Occupational Health
Mirka Hanhisuanto, Eino Solje, Manu Jokela, Jussi O. T. Sipila
Summary: The incidence and prevalence of ALS in Finland are among the highest in the world, with notable differences between the eastern and southwestern parts of the country. The low median life expectancy may be related to the advanced age of patients, high prevalence of C9orf72 repeat expansion, and infrequent use of TIV and riluzole.
Article
Nutrition & Dietetics
Juan Jose Lopez-Gomez, Maria D. Ballesteros-Pomar, Beatriz Torres-Torres, Begona Pintor De la Maza, M. Angeles Penacho-Lazaro, Jose M. Palacio-Mures, Cristina Abreu-Padin, Antonio Lopez-Guzman, Daniel A. De Luis-Roman
Summary: The study evaluated the nutritional status of ALS patients at the beginning of specialized nutritional treatment and its influence on disease progression and survival. The results showed that patients with worse nutritional status had a lower survival median, highlighting the importance of nutritional status in ALS management.
CLINICAL NUTRITION
(2021)
Article
Radiology, Nuclear Medicine & Medical Imaging
Matteo Zanovello, Gianni Soraru, Cristina Campi, Mariagiulia Anglani, Alessandro Spimpolo, Sara Berti, Cinzia Busse, Stefano Mozzetta, Annachiara Cagnin, Diego Cecchin
Summary: This study used a PET/MRI system to investigate the brain metabolism in ALS/FTD patients and found increased glucose metabolism in the brain stem, which may be related to neuroinflammation. Furthermore, hypermetabolism in the medulla oblongata was associated with shortened survival.
JOURNAL OF NUCLEAR MEDICINE
(2022)
