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  1. Article ; Online: Surgical Therapies for Parkinson Disease.

    Rawls, Ashley E

    Continuum (Minneapolis, Minn.)

    2022  Volume 28, Issue 5, Page(s) 1301–1313

    Abstract: Purpose of review: Parkinson disease (PD) is a progressive neurodegenerative disorder that is often difficult to manage with medications alone. This article reviews the current therapeutic surgical interventions for PD, patient selection criteria, ... ...

    Abstract Purpose of review: Parkinson disease (PD) is a progressive neurodegenerative disorder that is often difficult to manage with medications alone. This article reviews the current therapeutic surgical interventions for PD, patient selection criteria, timing of patient referral to surgical services, procedure overview, and future directions.
    Recent findings: Adaptive, or closed-loop, deep brain stimulation is a promising therapy that can detect ongoing circuit changes and deliver appropriate stimulation based on the patient's dominant symptom and level of dopaminergic medication.
    Summary: Patients with PD can benefit from surgical interventions that can be added to their medication regimen. These patients should be referred to comprehensive centers that offer complete multidisciplinary screening evaluation to ensure appropriate patient selection and intervention selection. With the appropriate surgical intervention and continued management from their care team, patients with PD can maximize their quality of life.
    MeSH term(s) Deep Brain Stimulation/methods ; Humans ; Parkinson Disease/diagnosis ; Parkinson Disease/surgery ; Quality of Life
    Language English
    Publishing date 2022-10-12
    Publishing country United States
    Document type Journal Article ; Review
    ISSN 1538-6899
    ISSN (online) 1538-6899
    DOI 10.1212/CON.0000000000001160
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Identification and Management of Persistent Stimulation-Induced Dyskinesia Associated with STN DBS: The See-Saw Dilemma.

    Remz, Matthew A / Wong, Joshua K / Hilliard, Justin D / Tholanikunnel, Tracy / Rawls, Ashley E / Okun, Michael S

    Tremor and other hyperkinetic movements (New York, N.Y.)

    2023  Volume 13, Page(s) 28

    Abstract: Clinical vignette: A 73-year-old woman with Parkinson's disease (PD) underwent implantation of bilateral subthalamic nucleus deep brain stimulators (STN-DBS) to address bilateral upper extremity medication-refractory tremor. Post-operatively, she ... ...

    Abstract Clinical vignette: A 73-year-old woman with Parkinson's disease (PD) underwent implantation of bilateral subthalamic nucleus deep brain stimulators (STN-DBS) to address bilateral upper extremity medication-refractory tremor. Post-operatively, she experienced a "see-saw effect" where small increases in stimulation resulted in improvement in one symptom (tremor) with concurrent worsening in another (dyskinesia).
    Clinical dilemma: SID is usually considered a positive predictor of DBS outcome. However, there are cases where SID cannot be optimized. Lead location and pre-operative characteristics may contribute to this adverse effect. If the combination of programming and medication adjustments fails to resolve SID, what can be done to "rescue" the outcome?
    Clinical solution: Management of SID requires a gradual and steadfast programming approach. Post-operative lead localization can guide advanced programming and decision-making. Rescue surgical interventions may be considered.
    Gap in knowledge: In cases where SID is persistent despite deploying persistent optimization strategies, there is limited guidance on next steps.
    MeSH term(s) Aged ; Female ; Humans ; Dyskinesias ; Parkinson Disease/complications ; Parkinson Disease/therapy ; Subthalamic Nucleus ; Tremor
    Language English
    Publishing date 2023-08-28
    Publishing country England
    Document type Case Reports ; Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 2674453-3
    ISSN 2160-8288 ; 2160-8288
    ISSN (online) 2160-8288
    ISSN 2160-8288
    DOI 10.5334/tohm.780
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: The chromatin repressors EZH2 and Suv4‐20h coregulate cell fate specification during hippocampal development

    Chang, Kai‐Chun / Rhodes, Christopher T. / Zhang, Jesse Q. / Moseley, Madeleine C. / Cardona, Sandra M. / Huang, Shu‐Wei Angela / Rawls, Ashley / Lemmon, Vance P. / Berger, Mitchel S. / Abate, Adam R. / Lin, Chin‐Hsing Annie

    FEBS letters. 2022 Feb., v. 596, no. 3

    2022  

    Abstract: The cell fate transition from radial glial‐like (RGL) cells to neurons and astrocytes is crucial for development and pathological conditions. Two chromatin repressors—the enhancer of zeste homolog 2 and suppressor of variegation 4‐20 homolog—are ... ...

    Abstract The cell fate transition from radial glial‐like (RGL) cells to neurons and astrocytes is crucial for development and pathological conditions. Two chromatin repressors—the enhancer of zeste homolog 2 and suppressor of variegation 4‐20 homolog—are expressed in RGL cells in the hippocampus, implicating these epigenetic regulators in hippocampal cell fate commitment. Using a double knockout mouse model, we demonstrated that loss of both chromatin repressors in the RGL population leads to deficits in hippocampal development. Single‐nuclei RNA‐Seq revealed differential gene expression and provided mechanistic insight into how the two chromatin repressors are critical for the maintenance of cycling cells in the dentate gyrus as well as the balance of cell trajectories between neuronal and astroglial lineages.
    Keywords astrocytes ; chromatin ; epigenetics ; gene expression regulation ; hippocampus ; knockout mutants ; neurons ; sequence analysis
    Language English
    Dates of publication 2022-02
    Size p. 294-308.
    Publishing place John Wiley & Sons, Ltd
    Document type Article
    Note JOURNAL ARTICLE
    ZDB-ID 212746-5
    ISSN 1873-3468 ; 0014-5793
    ISSN (online) 1873-3468
    ISSN 0014-5793
    DOI 10.1002/1873-3468.14254
    Database NAL-Catalogue (AGRICOLA)

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  4. Article ; Online: Illusory Responses across the Lewy Body Disease Spectrum.

    Shahid, Marian / Rawls, Ashley / Ramirez, Veronica / Ryman, Sephira / Santini, Veronica E / Yang, Laurice / Sha, Sharon J / Hall, Jacob N / Montine, Thomas J / Lin, Amy / Tian, Lu / Henderson, Victor W / Cholerton, Brenna / Yutsis, Maya / Poston, Kathleen L

    Annals of neurology

    2023  Volume 93, Issue 4, Page(s) 702–714

    Abstract: Objective: This study was undertaken to study pareidolias, or perceived meaningful objects in a meaningless stimulus, in patients across the Lewy body (LB) disease spectrum, where most do not report hallucinations or delusions.: Methods: We studied ... ...

    Abstract Objective: This study was undertaken to study pareidolias, or perceived meaningful objects in a meaningless stimulus, in patients across the Lewy body (LB) disease spectrum, where most do not report hallucinations or delusions.
    Methods: We studied illusory responses on the Noise Pareidolia Task in 300 participants (38 cognitively impaired LB, 65 cognitively unimpaired LB, 51 Alzheimer disease spectrum [AD-s], 146 controls). Pairwise between-group comparisons examined how diagnosis impacts the number of illusory responses. Ordinal regression analysis compared the number of illusory responses across diagnosis groups, adjusting for age, sex, and education. Analyses were repeated after removing participants with reported hallucinations or delusions.
    Results: Cognitively impaired LB participants were 12.3, 4.9, and 4.6 times more likely than control, cognitively unimpaired LB, and AD-s participants, respectively, to endorse illusory responses. After adjusting for age, sex, and education, the probability of endorsing 1 or more illusory responses was 61% in the cognitively impaired LB group, compared to 26% in AD-s, 25% in cognitively unimpaired LB, and 12% in control participants. All results were similar after repeated analysis only in participants without hallucinations or delusions. In LB without hallucinations or delusions, 52% with mild cognitive impairment and 66.7% with dementia endorsed at least 1 illusory response.
    Interpretation: We found illusory responses are common in cognitively impaired LB patients, including those without any reported psychosis. Our data suggest that, prior to the onset of hallucinations and delusions, the Noise Pareidolia Task can easily be used to screen for unobtrusive pareidolias in all LB patients. ANN NEUROL 2023;93:702-714.
    MeSH term(s) Humans ; Lewy Body Disease/psychology ; Alzheimer Disease/psychology ; Illusions ; Hallucinations ; Cognitive Dysfunction
    Language English
    Publishing date 2023-02-02
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 80362-5
    ISSN 1531-8249 ; 0364-5134
    ISSN (online) 1531-8249
    ISSN 0364-5134
    DOI 10.1002/ana.26574
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Advancing Equity at the JAMA Network-Self-Reported Demographics of Editors and Editorial Board Members.

    Bibbins-Domingo, Kirsten / Flanagin, Annette / Sietmann, Caroline / Bonow, Robert O / Navar, Ann Marie / Shinkai, Kanade / Roberson, Mya L / Ayanian, John Z / Ponce, Ninez / Inouye, Sharon K / Durant, Raegan W / Simon, Melissa A / Rivara, Frederick P / Vela, Monica / Josephson, S Andrew / Rawls, Ashley / Disis, Mary L Nora / Florez, Narjust / Bressler, Neil M /
    Scott, Adrienne W / Piccirillo, Jay F / Osazuwa-Peters, Nosayaba / Christakis, Dimitri A / Duncan, Andrea F / Öngür, Dost / Bagot, Kara S / Kibbe, Melina R / Backhus, Leah Monique / Malani, Preeti N

    JAMA

    2024  Volume 331, Issue 10, Page(s) 837–839

    MeSH term(s) Humans ; Self Report ; Demography
    Language English
    Publishing date 2024-02-09
    Publishing country United States
    Document type Journal Article ; Comment
    ZDB-ID 2958-0
    ISSN 1538-3598 ; 0254-9077 ; 0002-9955 ; 0098-7484
    ISSN (online) 1538-3598
    ISSN 0254-9077 ; 0002-9955 ; 0098-7484
    DOI 10.1001/jama.2024.1709
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: The chromatin repressors EZH2 and Suv4-20h coregulate cell fate specification during hippocampal development.

    Chang, Kai-Chun / Rhodes, Christopher T / Zhang, Jesse Q / Moseley, Madeleine C / Cardona, Sandra M / Huang, Shu-Wei Angela / Rawls, Ashley / Lemmon, Vance P / Berger, Mitchel S / Abate, Adam R / Lin, Chin-Hsing Annie

    FEBS letters

    2021  Volume 596, Issue 3, Page(s) 294–308

    Abstract: The cell fate transition from radial glial-like (RGL) cells to neurons and astrocytes is crucial for development and pathological conditions. Two chromatin repressors-the enhancer of zeste homolog 2 and suppressor of variegation 4-20 homolog-are ... ...

    Abstract The cell fate transition from radial glial-like (RGL) cells to neurons and astrocytes is crucial for development and pathological conditions. Two chromatin repressors-the enhancer of zeste homolog 2 and suppressor of variegation 4-20 homolog-are expressed in RGL cells in the hippocampus, implicating these epigenetic regulators in hippocampal cell fate commitment. Using a double knockout mouse model, we demonstrated that loss of both chromatin repressors in the RGL population leads to deficits in hippocampal development. Single-nuclei RNA-Seq revealed differential gene expression and provided mechanistic insight into how the two chromatin repressors are critical for the maintenance of cycling cells in the dentate gyrus as well as the balance of cell trajectories between neuronal and astroglial lineages.
    MeSH term(s) Enhancer of Zeste Homolog 2 Protein
    Chemical Substances EZH2 protein, human (EC 2.1.1.43) ; Enhancer of Zeste Homolog 2 Protein (EC 2.1.1.43)
    Language English
    Publishing date 2021-12-20
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, Non-P.H.S.
    ZDB-ID 212746-5
    ISSN 1873-3468 ; 0014-5793
    ISSN (online) 1873-3468
    ISSN 0014-5793
    DOI 10.1002/1873-3468.14254
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article: Rare Etiology of Bow Hunter's Syndrome and Systematic Review of Literature.

    Rastogi, Vaibhav / Rawls, Ashley / Moore, Omar / Victorica, Benjamin / Khan, Sheema / Saravanapavan, Pradeepan / Midivelli, Sunitha / Raviraj, Prathap / Khanna, Anna / Bidari, Sharathchandra / Hedna, Vishnumurthy S

    Journal of vascular and interventional neurology

    2015  Volume 8, Issue 3, Page(s) 7–16

    Abstract: Background: Bow Hunter's Syndrome is a mechanical occlusion of the vertebral artery which leads to a reduction in blood flow in posterior cerebral circulation resulting in transient reversible symptomatic vertebrobasilar insufficiency.: Case ... ...

    Abstract Background: Bow Hunter's Syndrome is a mechanical occlusion of the vertebral artery which leads to a reduction in blood flow in posterior cerebral circulation resulting in transient reversible symptomatic vertebrobasilar insufficiency.
    Case description: We present a case of Bow Hunter's syndrome in a 53-year-old male that occurred after the patient underwent surgical correction of a proximal left subclavian artery aneurysm. Shortly after the surgery, the patient began to complain of transient visual changes, presyncopal spells, and dizziness upon turning his head to the left. A transcranial doppler ultrasound confirmed the diagnosis of Bow Hunter's syndrome.
    Systemic review: We analyzed the data on 153 patients with Bow Hunter's syndrome from the literature. An osteophyte was the most common cause of vertebral artery occlusion, and left vertebral artery was more commonly involved in patients with Bow Hunter's syndrome. Dynamic angiography was the definitive imaging modality to confirm the diagnosis, and surgery was most successful in alleviating symptoms.
    Conclusion: We believe that this is the first case of iatrogenic Bow Hunter's syndrome after surgical intervention for an aneurysm repair, and the largest review of literature of Bow Hunter's syndrome. Dynamic angiography is the gold standard for the diagnosis of Bow Hunter's syndrome. Surgery should be considered as the primary treatment approach in these patients, especially those who have bony compression as the etiology.
    Language English
    Publishing date 2015-04-24
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2627963-0
    ISSN 1944-141X ; 1941-5893
    ISSN (online) 1944-141X
    ISSN 1941-5893
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Blood-brain barrier impairment in an animal model of MPS III B.

    Garbuzova-Davis, Svitlana / Louis, Michael K / Haller, Edward M / Derasari, Hiranya M / Rawls, Ashley E / Sanberg, Paul R

    PloS one

    2011  Volume 6, Issue 3, Page(s) e16601

    Abstract: Background: Sanfilippo syndrome type B (MPS III B) is caused by a deficiency of α-N-acetylglucosaminidase enzyme, leading to accumulation of heparan sulfate within lysosomes and eventual progressive cerebral and systemic multiple organ abnormalities. ... ...

    Abstract Background: Sanfilippo syndrome type B (MPS III B) is caused by a deficiency of α-N-acetylglucosaminidase enzyme, leading to accumulation of heparan sulfate within lysosomes and eventual progressive cerebral and systemic multiple organ abnormalities. However, little is known about the competence of the blood-brain barrier (BBB) in MPS III B. BBB dysfunction in this devastating disorder could contribute to neuropathological disease manifestations.
    Methodology/principal findings: In the present study, we investigated structural (electron microscope) and functional (vascular leakage) integrity of the BBB in a mouse model of MPS III B at different stages of disease, focusing on brain structures known to experience neuropathological changes. Major findings of our study were: (1) endothelial cell damage in capillary ultrastructure, compromising the BBB and resulting in vascular leakage, (2) formation of numerous large vacuoles in endothelial cells and perivascular cells (pericytes and perivascular macrophages) in the large majority of vessels, (3) edematous space around microvessels, (4) microaneurysm adjacent to a ruptured endothelium, (6) Evans Blue and albumin microvascular leakage in various brain structures, (7) GM3 ganglioside accumulation in endothelium of the brain microvasculature.
    Conclusions/significance: These new findings of BBB structural and function impairment in MPS III B mice even at early disease stage may have implications for disease pathogenesis and should be considered in current and future development of treatments for MPS III B.
    MeSH term(s) Acetylglucosaminidase/metabolism ; Albumins/metabolism ; Animals ; Blood-Brain Barrier/enzymology ; Blood-Brain Barrier/pathology ; Blood-Brain Barrier/ultrastructure ; Disease Models, Animal ; Evans Blue/metabolism ; G(M3) Ganglioside/metabolism ; Immunohistochemistry ; Mice ; Mice, Mutant Strains ; Microvessels/pathology ; Microvessels/ultrastructure ; Mucopolysaccharidosis III/enzymology ; Mucopolysaccharidosis III/pathology
    Chemical Substances Albumins ; G(M3) Ganglioside ; Evans Blue (45PG892GO1) ; alpha-N-acetyl-D-glucosaminidase (EC 3.2.1.50) ; Acetylglucosaminidase (EC 3.2.1.52)
    Language English
    Publishing date 2011-03-07
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ISSN 1932-6203
    ISSN (online) 1932-6203
    DOI 10.1371/journal.pone.0016601
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Repeated administrations of human umbilical cord blood cells improve disease outcomes in a mouse model of Sanfilippo syndrome type III B.

    Willing, Alison E / Garbuzova-Davis, Svitlana N / Zayko, Olga / Derasari, Hiranya M / Rawls, Ashley E / James, Chris R / Mervis, Ron F / Sanberg, Cyndy D / Kuzmin-Nichols, Nicole / Sanberg, Paul R

    Cell transplantation

    2014  Volume 23, Issue 12, Page(s) 1613–1630

    Abstract: Sanfilippo syndrome type III B (MPS III B) is an inherited disorder characterized by a deficiency of α-N-acetylglucosaminidase (Naglu) enzyme leading to accumulation of heparan sulfate in lysosomes and severe neurological deficits. We have previously ... ...

    Abstract Sanfilippo syndrome type III B (MPS III B) is an inherited disorder characterized by a deficiency of α-N-acetylglucosaminidase (Naglu) enzyme leading to accumulation of heparan sulfate in lysosomes and severe neurological deficits. We have previously shown that a single administration of human umbilical cord mononuclear cells (hUCB MNCs) into Naglu knockout mice decreased behavioral abnormalities and tissue pathology. In this study, we tested whether repeated doses of hUCB MNCs would be more beneficial than a single dose of cells. Naglu mice at 3 months of age were randomly assigned to either a Media-only group or one of three hUCB MNC treatment groups--single low dose (3 × 10(6) cells), single high dose (1.8 × 10(7) cells), or multiple doses (3 × 10(6) cells monthly for 6 months) delivered intravenously; cyclosporine was injected intraperitoneally to immune suppress the mice for the duration of the study. An additional control group of wild-type mice was also used. We measured anxiety in an open field test and cognition in an active avoidance test prior to treatment and then at monthly intervals for 6 months. hUCB MNCs restored normal anxiety-like behavior in these mice (p < 0.001). The repeated cell administrations also restored hippocampal cytoarchitecture, protected the dendritic tree, decreased GM3 ganglioside accumulation, and decreased microglial activation, particularly in the hippocampus and cortex. These data suggest that the neuroprotective effect of hUCB MNCs can be enhanced by repeated cell administrations.
    MeSH term(s) Acetylglucosaminidase/deficiency ; Acetylglucosaminidase/metabolism ; Animals ; Anxiety/complications ; Anxiety/physiopathology ; Avoidance Learning ; Behavior, Animal ; Brain/pathology ; Cell Count ; Cognition ; Cord Blood Stem Cell Transplantation ; Dendrites/pathology ; Disease Models, Animal ; Female ; G(M3) Ganglioside/metabolism ; Humans ; Male ; Mice, Knockout ; Microglia/pathology ; Mucopolysaccharidosis III/complications ; Mucopolysaccharidosis III/physiopathology ; Mucopolysaccharidosis III/therapy ; Phenotype ; Treatment Outcome ; Umbilical Cord/cytology ; Urine
    Chemical Substances G(M3) Ganglioside ; alpha-N-acetyl-D-glucosaminidase (EC 3.2.1.50) ; Acetylglucosaminidase (EC 3.2.1.52)
    Language English
    Publishing date 2014
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1135816-6
    ISSN 1555-3892 ; 0963-6897
    ISSN (online) 1555-3892
    ISSN 0963-6897
    DOI 10.3727/096368914X676916
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Repeated Administrations of Human Umbilical Cord Blood Cells Improve Disease Outcomes in a Mouse Model of Sanfilippo Syndrome Type III B.

    Willing, Alison E / Garbuzova-Davis, Svitlana N / Zayko, Olga / Derasari, Hiranya M / Rawls, Ashley E / James, Chris R / Mervis, Ron F / Sanberg, Cyndy D / Kuzmin-Nichols, Nicole / Sanberg, Paul R

    Cell transplantation

    2013  

    Abstract: Sanfilippo syndrome type III B (MPS III B) is an inherited disorder characterized by a deficiency of ?-N-acetylglucosaminidase (Naglu) enzyme leading to accumulation of heparan sulfate in lysosomes and severe neurological deficits. We have previously ... ...

    Abstract Sanfilippo syndrome type III B (MPS III B) is an inherited disorder characterized by a deficiency of ?-N-acetylglucosaminidase (Naglu) enzyme leading to accumulation of heparan sulfate in lysosomes and severe neurological deficits. We have previously shown that a single administration of human umbilical cord mononuclear cells (hUCB MNC) into Nagluknockout mice decreased behavioral abnormalities and tissue pathology. In this study, we tested whether repeated doses of hUCB MNCs would be more beneficial than a single dose of cells. Naglumice at 3 months of age were randomly assigned to either a Media only group, or one of three hUCB MNC treatment groups - single low dose (3x10
    Language English
    Publishing date 2013-12-30
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1135816-6
    ISSN 1555-3892 ; 0963-6897
    ISSN (online) 1555-3892
    ISSN 0963-6897
    DOI 10.3727/096368913X676916
    Database MEDical Literature Analysis and Retrieval System OnLINE

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