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  1. Article ; Online: Secondary trigeminal neuralgia diagnosed in orofacial pain setting as epidermoid tumor.

    Heir, Gary M / Johnson, Stephen A / Kuchukulla, Manvitha / Kalladka, Mythili / Mangal, Jaya

    Quintessence international (Berlin, Germany : 1985)

    2022  Volume 53, Issue 7, Page(s) 624–630

    Abstract: This case report describes the rare occurrence of an epidermoid tumor compressing the ipsilateral trigeminal nerve resulting in secondary trigeminal neuralgia. MRI is the imaging modality of choice for the diagnosis of secondary trigeminal neuralgia. The ...

    Abstract This case report describes the rare occurrence of an epidermoid tumor compressing the ipsilateral trigeminal nerve resulting in secondary trigeminal neuralgia. MRI is the imaging modality of choice for the diagnosis of secondary trigeminal neuralgia. The epidermoid tumor was discovered by an orofacial pain specialist after reassessing the MRI study, previously reported as normal. Clinicians encounter a diagnostic dilemma when the clinical picture is not consistent with the MRI reports, clinical presentation, and expected results of treatment. The reassessment of the MRI and discovery of the epidermoid tumor resulted in a prompt referral to a neurosurgeon with a successful treatment outcome.
    MeSH term(s) Facial Pain/etiology ; Humans ; Magnetic Resonance Imaging/adverse effects ; Magnetic Resonance Imaging/methods ; Neoplasms/complications ; Neoplasms/pathology ; Trigeminal Nerve/pathology ; Trigeminal Neuralgia/diagnosis ; Trigeminal Neuralgia/etiology
    Language English
    Publishing date 2022-06-21
    Publishing country Germany
    Document type Case Reports ; Journal Article
    ZDB-ID 7436-6
    ISSN 1936-7163 ; 0033-6572
    ISSN (online) 1936-7163
    ISSN 0033-6572
    DOI 10.3290/j.qi.b3045159
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Upper limb movements as digital biomarkers in people with ALS.

    Straczkiewicz, Marcin / Karas, Marta / Johnson, Stephen A / Burke, Katherine M / Scheier, Zoe / Royse, Tim B / Calcagno, Narghes / Clark, Alison / Iyer, Amrita / Berry, James D / Onnela, Jukka-Pekka

    EBioMedicine

    2024  Volume 101, Page(s) 105036

    Abstract: Background: Objective evaluation of people with amyotrophic lateral sclerosis (PALS) in free-living settings is challenging. The introduction of portable digital devices, such as wearables and smartphones, may improve quantifying disease progression and ...

    Abstract Background: Objective evaluation of people with amyotrophic lateral sclerosis (PALS) in free-living settings is challenging. The introduction of portable digital devices, such as wearables and smartphones, may improve quantifying disease progression and hasten therapeutic development. However, there is a need for tools to characterize upper limb movements in neurologic disease and disability.
    Methods: Twenty PALS wore a wearable accelerometer, ActiGraph Insight Watch, on their wrist for six months. They also used Beiwe, a smartphone application that collected self-entry ALS Functional Rating Scale-Revised (ALSFRS-RSE) survey responses every 1-4 weeks. We developed several measures that quantify count and duration of upper limb movements: flexion, extension, supination, and pronation. New measures were compared against ALSFRS-RSE total score (Q1-12), and individual responses to specific questions related to handwriting (Q4), cutting food (Q5), dressing and performing hygiene (Q6), and turning in bed and adjusting bed clothes (Q7). Additional analysis considered adjusting for total activity counts (TAC).
    Findings: At baseline, PALS with higher Q1-12 performed more upper limb movements, and these movements were faster compared to individuals with more advanced disease. Most upper limb movement metrics had statistically significant change over time, indicating declining function either by decreasing count metrics or by increasing duration metric. All count and duration metrics were significantly associated with Q1-12, flexion and extension counts were significantly associated with Q6 and Q7, supination and pronation counts were also associated with Q4. All duration metrics were associated with Q6 and Q7. All duration metrics retained their statistical significance after adjusting for TAC.
    Interpretation: Wearable accelerometer data can be used to generate digital biomarkers on upper limb movements and facilitate patient monitoring in free-living environments. The presented method offers interpretable monitoring of patients' functioning and versatile tracking of disease progression in the limb of interest.
    Funding: Mitsubishi-Tanabe Pharma Holdings America, Inc.
    MeSH term(s) Humans ; Amyotrophic Lateral Sclerosis/diagnosis ; Upper Extremity ; Wrist ; Disease Progression ; Biomarkers
    Chemical Substances Biomarkers
    Language English
    Publishing date 2024-03-02
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 2851331-9
    ISSN 2352-3964
    ISSN (online) 2352-3964
    DOI 10.1016/j.ebiom.2024.105036
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. 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

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  4. Article ; Online: Guillain-Barré Syndrome in the Placebo and Active Arms of a COVID-19 Vaccine Clinical Trial: Temporal Associations Do Not Imply Causality.

    Márquez Loza, Alejandra M / Holroyd, Kathryn B / Johnson, Stephen A / Pilgrim, David M / Amato, Anthony A

    Neurology

    2021  Volume 96, Issue 22, Page(s) 1052–1054

    Language English
    Publishing date 2021-05-31
    Publishing country United States
    Document type Journal Article ; Comment
    ZDB-ID 207147-2
    ISSN 1526-632X ; 0028-3878
    ISSN (online) 1526-632X
    ISSN 0028-3878
    DOI 10.1212/WNL.0000000000011881
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Teaching NeuroImages: Optic nerve sheath meningioma presenting as gaze-evoked amaurosis.

    Johnson, Stephen A / Chen, John J / Tobin, W Oliver

    Neurology

    2018  Volume 90, Issue 23, Page(s) e2095–e2096

    MeSH term(s) Blindness/diagnostic imaging ; Blindness/etiology ; Female ; Humans ; Magnetic Resonance Imaging ; Meningeal Neoplasms/complications ; Meningeal Neoplasms/diagnostic imaging ; Meningioma/complications ; Meningioma/diagnostic imaging ; Middle Aged ; Optic Nerve Neoplasms/complications ; Optic Nerve Neoplasms/diagnostic imaging
    Language English
    Publishing date 2018-08-14
    Publishing country United States
    Document type Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 207147-2
    ISSN 1526-632X ; 0028-3878
    ISSN (online) 1526-632X
    ISSN 0028-3878
    DOI 10.1212/WNL.0000000000005642
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Longitudinal comparison of the self-entry Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-RSE) and Rasch-Built Overall Amyotrophic Lateral Sclerosis Disability Scale (ROADS) as outcome measures in people with amyotrophic lateral sclerosis.

    Johnson, Stephen A / Burke, Katherine M / Scheier, Zoe A / Keegan, Mackenzie A / Clark, Alison P / Chan, James / Fournier, Christina N / Berry, James D

    Muscle & nerve

    2022  Volume 66, Issue 4, Page(s) 495–502

    Abstract: Introduction/aims: Improved functional outcome measures in amyotrophic lateral sclerosis (ALS) would aid ALS trial design and help hasten drug discovery. We evaluate the longitudinal performance of the Rasch-Built Overall Amyotrophic Lateral Sclerosis ... ...

    Abstract Introduction/aims: Improved functional outcome measures in amyotrophic lateral sclerosis (ALS) would aid ALS trial design and help hasten drug discovery. We evaluate the longitudinal performance of the Rasch-Built Overall Amyotrophic Lateral Sclerosis Disability Scale (ROADS) compared to the Amyotrophic Lateral Sclerosis Functional Rating Scale Revised for Self-Entry (ALSFRS-RSE) as patient reported outcomes of functional status in people with ALS.
    Methods: Participants completed the ROADS and the ALSFRS-RSE questionnaires at baseline, 3-, 6-, and 12- mo using Research Electronic Data Capture as part of a prospective, longitudinal, remote, online survey study of fatigue in ALS from 9/2020 to 12/2021. The scales were compared cross-sectionally (at baseline) and longitudinally. Correlation coefficients, coefficients of variation, and descriptive statistics were assessed.
    Results: A total of 182 adults with ALS consented to the study. This volunteer sample was comprised of predominantly White, non-Hispanic, non-smoking participants. Consented participant survey completion was approximately 90% at baseline and greater than 40% at 12 mo. The ALSFRS-RSE and the ROADS had high, significant agreement at 3 and 6 mo by Cohen's kappa ≥71% (p < 0.001); the number of functional increases or plateaus on the two scales were not significantly different; and the coefficient of variation of functional decline was similar at the 6-month mark, though higher for the ROADS at 3 mo and lower at 12 mo.
    Discussion: Although the ROADS performed similarly to the ALSFRS-RSE in an observational cohort, it has psychometric advantages, such as Rasch-modeling and unidimensionality. It merits further investigation as a patient reported outcome of overall disability and efficacy outcome measure in ALS trials.
    MeSH term(s) Adult ; Amyotrophic Lateral Sclerosis/diagnosis ; Disabled Persons ; Disease Progression ; Humans ; Outcome Assessment, Health Care ; Prospective Studies ; Surveys and Questionnaires
    Language English
    Publishing date 2022-08-15
    Publishing country United States
    Document type Journal Article ; Research Support, U.S. Gov't, Non-P.H.S.
    ZDB-ID 438353-9
    ISSN 1097-4598 ; 0148-639X
    ISSN (online) 1097-4598
    ISSN 0148-639X
    DOI 10.1002/mus.27691
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Pharmacotherapy for Amyotrophic Lateral Sclerosis: A Review of Approved and Upcoming Agents.

    Johnson, Stephen A / Fang, Ton / De Marchi, Fabiola / Neel, Dylan / Van Weehaeghe, Donatienne / Berry, James D / Paganoni, Sabrina

    Drugs

    2022  Volume 82, Issue 13, Page(s) 1367–1388

    Abstract: Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder involving loss of upper and lower motor neurons, with most cases ending in death within 3-5 years of onset. Several molecular and cellular pathways have been ... ...

    Abstract Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder involving loss of upper and lower motor neurons, with most cases ending in death within 3-5 years of onset. Several molecular and cellular pathways have been identified to cause ALS; however, treatments to stop or reverse disease progression are yet to be found. Riluzole, a neuroprotective agent offering only a modest survival benefit, has long been the sole disease-modifying therapy for ALS. Edaravone, which demonstrated statistically significant slowing of ALS disease progression, is gaining approval in an increasing number of countries since its first approval in 2015. Sodium phenylbutyrate and taurursodiol (PB-TURSO) was conditionally approved in Canada in 2022, having shown significant slowing of disease progression and prolonged survival. Most clinical trials have focused on testing small molecules affecting common cellular pathways in ALS: targeting glutamatergic, apoptotic, inflammatory, and oxidative stress mechanisms among others. More recently, clinical trials utilizing stem cell transplantation and other biologics have emerged. This rich and ever-growing pipeline of investigational products, along with innovative clinical trial designs, collaborative trial networks, and an engaged ALS community', provide renewed hope to finding a cure for ALS. This article reviews existing ALS therapies and the current clinical drug development pipeline.
    MeSH term(s) Amyotrophic Lateral Sclerosis/drug therapy ; Biological Products/therapeutic use ; Clinical Trials as Topic ; Disease Progression ; Edaravone/therapeutic use ; Humans ; Neuroprotective Agents/therapeutic use ; Riluzole/therapeutic use
    Chemical Substances Biological Products ; Neuroprotective Agents ; Riluzole (7LJ087RS6F) ; Edaravone (S798V6YJRP)
    Language English
    Publishing date 2022-09-19
    Publishing country New Zealand
    Document type Journal Article ; Review
    ZDB-ID 120316-2
    ISSN 1179-1950 ; 0012-6667
    ISSN (online) 1179-1950
    ISSN 0012-6667
    DOI 10.1007/s40265-022-01769-1
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Visual Evoked Potentials and Intraoperative Awakening in Ophthalmic Artery Sacrifice During Aneurysm Clipping: 2 Cases and Literature Review.

    Nowicki, Kamil W / Johnson, Stephen A / Goldschmidt, Ezequiel / Balzer, Jeffrey / Gross, Bradley A / Friedlander, Robert M

    World neurosurgery

    2020  Volume 139, Page(s) 395–400

    Abstract: Background: Complete aneurysm obliteration is the goal of aneurysm treatment. In selected cases, a neck remnant may be left to preserve a critical branch. Literature on ophthalmic artery sacrifice in the treatment of cerebral aneurysms and subsequent ... ...

    Abstract Background: Complete aneurysm obliteration is the goal of aneurysm treatment. In selected cases, a neck remnant may be left to preserve a critical branch. Literature on ophthalmic artery sacrifice in the treatment of cerebral aneurysms and subsequent risk of vision loss is limited.
    Case description: Herein, we describe 2 cases where the ophthalmic artery originated from the aneurysm dome, resulting in a situation where we either incompletely obliterate the aneurysm or sacrifice the ophthalmic artery in order to completely clip the lesion, risking visual function.
    Conclusions: We report for the first time the use of visual evoked potential monitoring and intraoperative awakening to test visual function following intentional ophthalmic artery sacrifice to demonstrate gross vision preservation.
    MeSH term(s) Evoked Potentials, Visual/physiology ; Female ; Humans ; Intracranial Aneurysm/surgery ; Intraoperative Neurophysiological Monitoring/methods ; Middle Aged ; Neurosurgical Procedures/methods ; Ophthalmic Artery/surgery ; Surgical Instruments
    Language English
    Publishing date 2020-05-04
    Publishing country United States
    Document type Case Reports ; Journal Article ; Review
    ZDB-ID 2534351-8
    ISSN 1878-8769 ; 1878-8750
    ISSN (online) 1878-8769
    ISSN 1878-8750
    DOI 10.1016/j.wneu.2020.04.196
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Wearable device and smartphone data quantify ALS progression and may provide novel outcome measures.

    Johnson, Stephen A / Karas, Marta / Burke, Katherine M / Straczkiewicz, Marcin / Scheier, Zoe A / Clark, Alison P / Iwasaki, Satoshi / Lahav, Amir / Iyer, Amrita S / Onnela, Jukka-Pekka / Berry, James D

    NPJ digital medicine

    2023  Volume 6, Issue 1, Page(s) 34

    Abstract: Amyotrophic lateral sclerosis (ALS) therapeutic development has largely relied on staff-administered functional rating scales to determine treatment efficacy. We sought to determine if mobile applications (apps) and wearable devices can be used to ... ...

    Abstract Amyotrophic lateral sclerosis (ALS) therapeutic development has largely relied on staff-administered functional rating scales to determine treatment efficacy. We sought to determine if mobile applications (apps) and wearable devices can be used to quantify ALS disease progression through active (surveys) and passive (sensors) data collection. Forty ambulatory adults with ALS were followed for 6-months. The Beiwe app was used to administer the self-entry ALS functional rating scale-revised (ALSFRS-RSE) and the Rasch Overall ALS Disability Scale (ROADS) surveys every 2-4 weeks. Each participant used a wrist-worn activity monitor (ActiGraph Insight Watch) or an ankle-worn activity monitor (Modus StepWatch) continuously. Wearable device wear and app survey compliance were adequate. ALSFRS-R highly correlated with ALSFRS-RSE. Several wearable data daily physical activity measures demonstrated statistically significant change over time and associations with ALSFRS-RSE and ROADS. Active and passive digital data collection hold promise for novel ALS trial outcome measure development.
    Language English
    Publishing date 2023-03-06
    Publishing country England
    Document type Journal Article
    ISSN 2398-6352
    ISSN (online) 2398-6352
    DOI 10.1038/s41746-023-00778-y
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Advances in the treatment of Waldenström's macroglobulinemia.

    Johnson, Stephen A

    Expert review of anticancer therapy

    2006  Volume 6, Issue 3, Page(s) 329–334

    Abstract: Waldenström's macroglobulinemia is a distinct disorder characterized by a monoclonal immunoglobulin M paraprotein and morphological evidence of lymphoplasmacytic lymphoma. It is relatively rare, accounting for approximately 2% of all hematological ... ...

    Abstract Waldenström's macroglobulinemia is a distinct disorder characterized by a monoclonal immunoglobulin M paraprotein and morphological evidence of lymphoplasmacytic lymphoma. It is relatively rare, accounting for approximately 2% of all hematological malignancies. The aim of treatment for patients with Waldenström's macroglobulinemia should be to improve the quality and duration of life with minimal side effects in the most cost-effective manner. It is not yet clear if achievement of a complete remission confers clinical benefit and it is possible that prolonging therapy to maximal response may increase toxicity without extra benefit. Plasma exchange is indicated for the acute management of patients with severe problems due to a circulating paraprotein. There are no comparative data but alkylating agent-based treatments, combination therapy or purine analogs are all suitable choices for the initial therapy of patients requiring treatment. In younger patients, in whom high-dose treatment is contemplated, there is a role for the use of rituximab; however, it should be administered with caution in patients with high levels of immunoglobulin M paraprotein or signs of hyperviscosity because of the risk of 'flare' in the paraprotein level and consequent adverse clinical events.
    MeSH term(s) Age Factors ; Clinical Trials as Topic ; Humans ; Prognosis ; Quality of Life ; Waldenstrom Macroglobulinemia/complications ; Waldenstrom Macroglobulinemia/drug therapy ; Waldenstrom Macroglobulinemia/pathology
    Language English
    Publishing date 2006-03
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 2112544-2
    ISSN 1744-8328 ; 1473-7140
    ISSN (online) 1744-8328
    ISSN 1473-7140
    DOI 10.1586/14737140.6.3.329
    Database MEDical Literature Analysis and Retrieval System OnLINE

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