LIVIVO - The Search Portal for Life Sciences

zur deutschen Oberfläche wechseln
Advanced search

Search results

Result 1 - 10 of total 21

Search options

  1. Article: Positron Emission Tomography Molecular Imaging Biomarkers for Amyotrophic Lateral Sclerosis.

    Chew, Sheena / Atassi, Nazem

    Frontiers in neurology

    2019  Volume 10, Page(s) 135

    Abstract: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder with limited treatment options. Despite decades of therapeutic development, only two modestly efficacious disease-modifying drugs-riluzole and edaravone-are available to ALS ... ...

    Abstract Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder with limited treatment options. Despite decades of therapeutic development, only two modestly efficacious disease-modifying drugs-riluzole and edaravone-are available to ALS patients. Biomarkers that can facilitate ALS diagnosis, aid in prognosis, and measure drug pharmacodynamics are needed to accelerate therapeutic development for patients with ALS. Positron emission tomography (PET) imaging has promise as a biomarker for ALS because it permits visualization of central nervous system (CNS) pathology in individuals living with ALS. The availability of PET radioligands that target a variety of potential pathophysiological mechanisms-including cerebral metabolism, neuroinflammation, neuronal dysfunction, and oxidative stress-has enabled dynamic interrogation of molecular changes in ALS, in both natural history studies and human clinical trials. PET imaging has potential as a diagnostic biomarker that can establish upper motor neuron (UMN) pathology in ALS patients without overt UMN symptoms, as a prognostic biomarker that might help stratify patients for clinical trials, and as a pharmacodynamic biomarker that measures the biological effect of investigational drugs in the brain and spinal cord. In this Review, we discuss progress made with 30 years of PET imaging studies in ALS and consider future research needed to establish PET imaging biomarkers for ALS therapeutic development.
    Language English
    Publishing date 2019-03-01
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2564214-5
    ISSN 1664-2295
    ISSN 1664-2295
    DOI 10.3389/fneur.2019.00135
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  2. Article ; Online: Longitudinal comparison of the self-administered ALSFRS-RSE and ALSFRS-R as functional outcome measures in ALS.

    Erb, M Kelley / Calcagno, Narghes / Brown, Roland / Burke, Katherine M / Scheier, Zoe A / Iyer, Amrita S / Clark, Alison / Higgins, Max P / Keegan, Mackenzie / Gupta, Anoopum S / Johnson, Stephen A / Chew, Sheena / Berry, James D

    Amyotrophic lateral sclerosis & frontotemporal degeneration

    2024  , Page(s) 1–11

    Abstract: ... ...

    Abstract Objective
    Language English
    Publishing date 2024-03-19
    Publishing country England
    Document type Journal Article
    ZDB-ID 2705049-X
    ISSN 2167-9223 ; 2167-8421
    ISSN (online) 2167-9223
    ISSN 2167-8421
    DOI 10.1080/21678421.2024.2322549
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  3. Article ; Online: Clinical Reasoning: An 82-year-old man with worsening gait.

    Chew, Sheena / Vodopivec, Ivana / Berkowitz, Aaron L

    Neurology

    2017  Volume 89, Issue 21, Page(s) e246–e252

    MeSH term(s) Aged, 80 and over ; Autoantibodies/blood ; Autoantibodies/cerebrospinal fluid ; Clinical Decision-Making ; Gait Disorders, Neurologic/diagnosis ; Gait Disorders, Neurologic/etiology ; Gait Disorders, Neurologic/physiopathology ; Glutamate Decarboxylase/immunology ; Humans ; Male ; Stiff-Person Syndrome/complications ; Stiff-Person Syndrome/diagnosis ; Stiff-Person Syndrome/immunology
    Chemical Substances Autoantibodies ; Glutamate Decarboxylase (EC 4.1.1.15)
    Language English
    Publishing date 2017-11-21
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 207147-2
    ISSN 1526-632X ; 0028-3878
    ISSN (online) 1526-632X
    ISSN 0028-3878
    DOI 10.1212/WNL.0000000000004672
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  4. Article ; Online: Comparison of Two Clinical Upper Motor Neuron Burden Rating Scales in ALS Using Quantitative Brain Imaging.

    Makary, Meena M / Weerasekara, Akila / Rodham, Haley / Hightower, Baileigh G / Tseng, Chieh-En J / Chan, James / Chew, Sheena / Paganoni, Sabrina / Ratai, Eva-Maria / Zürcher, Nicole R / Hooker, Jacob M / Atassi, Nazem / Babu, Suma

    ACS chemical neuroscience

    2021  Volume 12, Issue 5, Page(s) 906–916

    Abstract: Several clinical upper motor neuron burden scales (UMNSs) variably measure brain dysfunction in amyotrophic lateral sclerosis (ALS). Here, we compare relationship of two widely used clinical UMNSs in ALS (Penn and MGH UMNSs) with (a) neuroimaging markers ...

    Abstract Several clinical upper motor neuron burden scales (UMNSs) variably measure brain dysfunction in amyotrophic lateral sclerosis (ALS). Here, we compare relationship of two widely used clinical UMNSs in ALS (Penn and MGH UMNSs) with (a) neuroimaging markers of brain dysfunction and (b) neurological impairment status using the gold-standard functional measure, the revised ALS Functional Rating Scale (ALSFRS-R). MGH UMNS measures hyperreflexia alone, and Penn UMNS measures hyperreflexia, spasticity, and pseudobulbar affect. Twenty-eight ALS participants underwent both Penn and MGH UMNSs, at a matching time-point as a simultaneous [
    MeSH term(s) Amyotrophic Lateral Sclerosis/diagnostic imaging ; Brain/diagnostic imaging ; Humans ; Magnetic Resonance Spectroscopy ; Motor Neurons ; Neuroimaging
    Language English
    Publishing date 2021-02-12
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ISSN 1948-7193
    ISSN (online) 1948-7193
    DOI 10.1021/acschemneuro.0c00772
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  5. Article ; Online: Patient reported outcomes in ALS: characteristics of the self-entry ALS Functional Rating Scale-revised and the Activities-specific Balance Confidence Scale.

    Chew, Sheena / Burke, Katherine M / Collins, Ella / Church, Reagan / Paganoni, Sabrina / Nicholson, Katharine / Babu, Suma / Scalia, Jennifer B / De Marchi, Fabiola / Ellrodt, Amy L / Moura, Lidia M V R / Chan, James / Berry, James D

    Amyotrophic lateral sclerosis & frontotemporal degeneration

    2021  Volume 22, Issue 7-8, Page(s) 467–477

    Abstract: Objective: ...

    Abstract Objective:
    MeSH term(s) Ambulatory Care Facilities ; Amyotrophic Lateral Sclerosis/diagnosis ; Disease Progression ; Humans ; Patient Reported Outcome Measures ; Self Report
    Language English
    Publishing date 2021-03-26
    Publishing country England
    Document type Journal Article
    ZDB-ID 2705049-X
    ISSN 2167-9223 ; 2167-8421
    ISSN (online) 2167-9223
    ISSN 2167-8421
    DOI 10.1080/21678421.2021.1900259
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  6. Article ; Online: Brainstem and limbic encephalitis with paraneoplastic neuromyelitis optica.

    Moussawi, Khaled / Lin, David J / Matiello, Marcelo / Chew, Sheena / Morganstern, Daniel / Vaitkevicius, Henrikas

    Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia

    2016  Volume 23, Page(s) 159–161

    Abstract: The spectrum of disorders associated with anti-neuromyelitis optica (NMO) antibody is being extended to include infrequent instances associated with cancer. We describe a patient with brainstem and limbic encephalitis from NMO-immunoglobulin G in serum ... ...

    Abstract The spectrum of disorders associated with anti-neuromyelitis optica (NMO) antibody is being extended to include infrequent instances associated with cancer. We describe a patient with brainstem and limbic encephalitis from NMO-immunoglobulin G in serum and cerebrospinal fluid in the context of newly diagnosed breast cancer. The neurological features markedly improved with excision of her breast cancer and immune suppressive therapy. This case further broadens the NMO spectrum disorders (NMOSD) by an association between NMOSD and cancer and raises the question of coincidental occurrence and the appropriate circumstances to search for a tumor in certain instances of NMO.
    MeSH term(s) Adult ; Autoantibodies/immunology ; Brain Stem/immunology ; Brain Stem/pathology ; Breast Neoplasms/complications ; Breast Neoplasms/diagnosis ; Breast Neoplasms/immunology ; Female ; Humans ; Immunoglobulin G/immunology ; Limbic Encephalitis/complications ; Limbic Encephalitis/diagnosis ; Limbic Encephalitis/immunology ; Neuromyelitis Optica/complications ; Neuromyelitis Optica/diagnosis ; Neuromyelitis Optica/immunology
    Chemical Substances Autoantibodies ; Immunoglobulin G
    Language English
    Publishing date 2016-01
    Publishing country Scotland
    Document type Case Reports ; Journal Article
    ZDB-ID 1193674-5
    ISSN 1532-2653 ; 0967-5868
    ISSN (online) 1532-2653
    ISSN 0967-5868
    DOI 10.1016/j.jocn.2015.08.006
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  7. Article ; Online: Lipocalin-2 is increased in amyotrophic lateral sclerosis.

    Petrozziello, Tiziana / Mills, Alexandra N / Farhan, Sali M K / Mueller, Kaly A / Granucci, Eric J / Glajch, Kelly E / Chan, James / Chew, Sheena / Berry, James D / Sadri-Vakili, Ghazaleh

    Muscle & nerve

    2020  Volume 62, Issue 2, Page(s) 272–283

    Abstract: Background: The exact mechanisms underlying neuroinflammation and how they contribute to amyotrophic lateral sclerosis (ALS) pathogenesis remain unclear. One possibility is the secretion of neurotoxic factors, such as lipocalin-2 (LCN2), that lead to ... ...

    Abstract Background: The exact mechanisms underlying neuroinflammation and how they contribute to amyotrophic lateral sclerosis (ALS) pathogenesis remain unclear. One possibility is the secretion of neurotoxic factors, such as lipocalin-2 (LCN2), that lead to neuronal death.
    Methods: LCN2 levels were measured in human postmortem tissue using Western blot, quantitative real time polymerase chain reaction, and immunofluorescence, and in plasma by enzyme-linked immunosorbent assay. SH-SY5Y cells were used to test the pro-inflammatory effects of LCN2.
    Results: LCN2 is increased in ALS postmortem motor cortex, spinal cord, and plasma. Furthermore, we identified several LCN2 variants in ALS patients that may contribute to disease pathogenesis. Lastly, while LCN2 treatment caused cell death and increased pro-inflammatory markers, treatment with an anti-LCN2 antibody prevented these responses in vitro.
    Conclusions: LCN2 upregulation in ALS postmortem samples and plasma may be an upstream event for triggering neuroinflammation and neuronal death.
    MeSH term(s) Adult ; Aged ; Aged, 80 and over ; Amyotrophic Lateral Sclerosis/genetics ; Amyotrophic Lateral Sclerosis/metabolism ; Amyotrophic Lateral Sclerosis/physiopathology ; Blotting, Western ; Case-Control Studies ; Cell Death ; Cell Line, Tumor ; Cytokines/drug effects ; Cytokines/metabolism ; Enzyme-Linked Immunosorbent Assay ; Female ; Fluorescent Antibody Technique ; Humans ; In Vitro Techniques ; Inflammation/metabolism ; Lipocalin-2/antagonists & inhibitors ; Lipocalin-2/genetics ; Lipocalin-2/metabolism ; Lipocalin-2/pharmacology ; Male ; Middle Aged ; Motor Cortex/metabolism ; Polymorphism, Single Nucleotide ; Real-Time Polymerase Chain Reaction ; Spinal Cord/metabolism
    Chemical Substances Cytokines ; LCN2 protein, human ; Lipocalin-2
    Language English
    Publishing date 2020-05-29
    Publishing country United States
    Document type Journal Article
    ZDB-ID 438353-9
    ISSN 1097-4598 ; 0148-639X
    ISSN (online) 1097-4598
    ISSN 0148-639X
    DOI 10.1002/mus.26911
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  8. Article ; Online: Design of a Randomized, Placebo-Controlled, Phase 3 Trial of Tofersen Initiated in Clinically Presymptomatic SOD1 Variant Carriers: the ATLAS Study.

    Benatar, Michael / Wuu, Joanne / Andersen, Peter M / Bucelli, Robert C / Andrews, Jinsy A / Otto, Markus / Farahany, Nita A / Harrington, Elizabeth A / Chen, Weiping / Mitchell, Adele A / Ferguson, Toby / Chew, Sheena / Gedney, Liz / Oakley, Sue / Heo, Jeong / Chary, Sowmya / Fanning, Laura / Graham, Danielle / Sun, Peng /
    Liu, Yingying / Wong, Janice / Fradette, Stephanie

    Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics

    2022  Volume 19, Issue 4, Page(s) 1248–1258

    Abstract: Despite extensive research, amyotrophic lateral sclerosis (ALS) remains a progressive and invariably fatal neurodegenerative disease. Limited knowledge of the underlying causes of ALS has made it difficult to target upstream biological mechanisms of ... ...

    Abstract Despite extensive research, amyotrophic lateral sclerosis (ALS) remains a progressive and invariably fatal neurodegenerative disease. Limited knowledge of the underlying causes of ALS has made it difficult to target upstream biological mechanisms of disease, and therapeutic interventions are usually administered relatively late in the course of disease. Genetic forms of ALS offer a unique opportunity for therapeutic development, as genetic associations may reveal potential insights into disease etiology. Genetic ALS may also be amenable to investigating earlier intervention given the possibility of identifying clinically presymptomatic, at-risk individuals with causative genetic variants. There is increasing evidence for a presymptomatic phase of ALS, with biomarker data from the Pre-Symptomatic Familial ALS (Pre-fALS) study showing that an elevation in blood neurofilament light chain (NfL) precedes phenoconversion to clinically manifest disease. Tofersen is an investigational antisense oligonucleotide designed to reduce synthesis of superoxide dismutase 1 (SOD1) protein through degradation of SOD1 mRNA. Informed by Pre-fALS and the tofersen clinical development program, the ATLAS study (NCT04856982) is designed to evaluate the impact of initiating tofersen in presymptomatic carriers of SOD1 variants associated with high or complete penetrance and rapid disease progression who also have biomarker evidence of disease activity (elevated plasma NfL). The ATLAS study will investigate whether tofersen can delay the emergence of clinically manifest ALS. To our knowledge, ATLAS is the first interventional trial in presymptomatic ALS and has the potential to yield important insights into the design and conduct of presymptomatic trials, identification, and monitoring of at-risk individuals, and future treatment paradigms in ALS.
    MeSH term(s) Humans ; Superoxide Dismutase-1/genetics ; Amyotrophic Lateral Sclerosis/drug therapy ; Amyotrophic Lateral Sclerosis/genetics ; Superoxide Dismutase/genetics ; Neurodegenerative Diseases ; Oligonucleotides, Antisense/therapeutic use ; Biomarkers ; RNA, Messenger ; Mutation
    Chemical Substances Superoxide Dismutase-1 (EC 1.15.1.1) ; Superoxide Dismutase (EC 1.15.1.1) ; tofersen (2NU6F9601K) ; Oligonucleotides, Antisense ; Biomarkers ; RNA, Messenger ; SOD1 protein, human
    Language English
    Publishing date 2022-05-18
    Publishing country United States
    Document type Randomized Controlled Trial ; Clinical Trial, Phase III ; Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 2316693-9
    ISSN 1878-7479 ; 1933-7213
    ISSN (online) 1878-7479
    ISSN 1933-7213
    DOI 10.1007/s13311-022-01237-4
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  9. Article ; Online: Correction to: Design of a Randomized, Placebo-Controlled, Phase 3 Trial of Tofersen Initiated in Clinically Presymptomatic SOD1 Variant Carriers: the ATLAS Study.

    Benatar, Michael / Wuu, Joanne / Andersen, Peter M / Bucelli, Robert C / Andrews, Jinsy A / Otto, Markus / Farahany, Nita A / Harrington, Elizabeth A / Chen, Weiping / Mitchell, Adele A / Ferguson, Toby / Chew, Sheena / Gedney, Liz / Oakley, Sue / Heo, Jeong / Chary, Sowmya / Fanning, Laura / Graham, Danielle / Sun, Peng /
    Liu, Yingying / Wong, Janice / Fradette, Stephanie

    Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics

    2022  Volume 19, Issue 5, Page(s) 1686

    Language English
    Publishing date 2022-09-26
    Publishing country United States
    Document type Published Erratum
    ZDB-ID 2316693-9
    ISSN 1878-7479 ; 1933-7213
    ISSN (online) 1878-7479
    ISSN 1933-7213
    DOI 10.1007/s13311-022-01286-9
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  10. Article ; Online: Susceptibility Etching on MRI in Patients with Microangiopathy.

    Chang, Jennifer / Arani, Keerthi / Chew, Sheena / Frosch, Matthew P / Gonzalez, R Gilberto / Maza, Noor / Romero, Javier M

    Journal of neuroimaging : official journal of the American Society of Neuroimaging

    2017  Volume 27, Issue 1, Page(s) 43–49

    Abstract: Background: We detected a novel imaging sign, which consists of a specific imaging pattern of diffuse susceptibility effect, delineating the cortical-subcortical junction on high-resolution susceptibility-weighted images (SWIs). We describe magnetic ... ...

    Abstract Background: We detected a novel imaging sign, which consists of a specific imaging pattern of diffuse susceptibility effect, delineating the cortical-subcortical junction on high-resolution susceptibility-weighted images (SWIs). We describe magnetic resonance imaging findings in 10 patients with "susceptibility etching" and possible association with their abnormal coagulation profile.
    Materials/methods: A retrospective case series study with a search for cases that demonstrated susceptibility effect at the cortical-subcortical junction on SWI sequences was performed. The patients' respective coagulation profiles including prothrombin time, partial thromboplastin time, fibrinogen, D-dimer values, and platelet counts were reviewed. In addition, clinical history and neurological deficits were recorded.
    Results: We identified 10 patients with the "susceptibility etching" pattern at the cortical-subcortical junction. All patients were acutely ill and had a significantly elevated D-dimer (4,309 mcg/L to >10,000 mcg/L) with variably reduced platelet count. Two patients had reduced fibrinogen and 5 patients had prolonged international normalized ratio. Of the 10 patients, 4 died during hospitalization, within a few days of imaging. Pathology of 1 patient at autopsy demonstrated findings suggestive of a microvascular thrombotic or embolic event without overt parenchymal microhemorrhage.
    Conclusion: In this preliminary case series, we describe patients with "susceptibility etching" on SWI who were also found to have profound coagulation impairment. While other comorbities may also contribute to this novel sign, we suggest that a possible etiology may be secondary to microvascular in situ formation of fine thrombi and/or emboli lodged into an area of vascular caliber reduction and maybe related to thrombotic microangiopathy.
    MeSH term(s) Adult ; Aged ; Brain/diagnostic imaging ; Brain/pathology ; Cerebral Small Vessel Diseases/diagnostic imaging ; Cerebral Small Vessel Diseases/pathology ; Female ; Humans ; Magnetic Resonance Imaging/methods ; Male ; Middle Aged ; Nervous System Diseases/diagnostic imaging ; Nervous System Diseases/pathology ; Retrospective Studies ; Thrombotic Microangiopathies/diagnostic imaging ; Thrombotic Microangiopathies/pathology ; Young Adult
    Language English
    Publishing date 2017
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1071724-9
    ISSN 1552-6569 ; 1051-2284
    ISSN (online) 1552-6569
    ISSN 1051-2284
    DOI 10.1111/jon.12384
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

To top