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Article ; Online: Opsoclonus and ocular flutter: evaluation and management.

Grossman, Scott N / Rucker, Janet C

2023 Volume 34, Issue 6, Page(s) 465–469

Abstract: Purpose of review: Opsoclonus and ocular flutter are saccadic intrusions characterized by spontaneous, back-to-back, fast eye movements (saccades) that oscillate about the midline of central visual fixation without intervening inter-saccadic intervals. ... ...

Abstract	Purpose of review: Opsoclonus and ocular flutter are saccadic intrusions characterized by spontaneous, back-to-back, fast eye movements (saccades) that oscillate about the midline of central visual fixation without intervening inter-saccadic intervals. When this type of movement occurs exclusively in the horizontal plane, it is called ocular flutter. When it occurs in multiple planes (i.e. horizontal, vertical, and torsional) it is called opsoclonus. The most common etiologic categories are parainfectious and paraneoplastic diseases. Less common are toxic-metabolic, traumatic, or idiopathic origins. The mechanism of these movements relates to dysfunction of brainstem and cerebellar machinery involved in the generation of saccades. In this review, we discuss the characteristics of opsoclonus and ocular flutter, describe approaches to clinical evaluation and management of the patient with opsoclonus and ocular flutter, and review approaches to therapeutic intervention. Recent findings: Recent publications demonstrated eye position-dependent opsoclonus present only in left gaze, which may be related to dysfunction of frontal eye fields or structures in the cerebellar vermis. Summary: Opsoclonus and ocular flutter originate from a broad array of neuropathologies and have value from both a neuroanatomic and etiologic perspective.
MeSH term(s)	Humans ; Ocular Motility Disorders/diagnosis ; Ocular Motility Disorders/etiology ; Ocular Motility Disorders/therapy ; Eye Movements ; Saccades
Language	English
Publishing date	2023-08-21
Publishing country	United States
Document type	Review ; Journal Article
ZDB-ID	1049383-9
ISSN	1531-7021 ; 1040-8738
ISSN (online)	1531-7021
ISSN	1040-8738
DOI	10.1097/ICU.0000000000000998
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Article ; Online: Navigating the U.S. regulatory landscape for neurologic digital health technologies.

Busis, Neil A / Marolia, Dilshad / Montgomery, Robert / Balcer, Laura J / Galetta, Steven L / Grossman, Scott N

NPJ digital medicine

2024 Volume 7, Issue 1, Page(s) 94

Language	English
Publishing date	2024-04-12
Publishing country	England
Document type	Journal Article
ISSN	2398-6352
ISSN (online)	2398-6352
DOI	10.1038/s41746-024-01098-5
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Article ; Online: Teaching Video NeuroImage: Alternating Skew Deviation as a Manifestation of Anti-GAD65-Associated Cerebellitis.

Bell, Carter / Drummond, Patrick S / Grossman, Scott N

Neurology

2022 Volume 100, Issue 13, Page(s) 640

MeSH term(s)	Humans ; Ocular Motility Disorders/diagnosis ; Ocular Motility Disorders/etiology ; Strabismus
Chemical Substances	glutamate decarboxylase 2 (EC 4.1.1.15)
Language	English
Publishing date	2022-12-20
Publishing country	United States
Document type	Journal Article
ZDB-ID	207147-2
ISSN	1526-632X ; 0028-3878
ISSN (online)	1526-632X
ISSN	0028-3878
DOI	10.1212/WNL.0000000000206752
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Ui II Zs.153: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Clues From Parinaud: Diagnostic Approaches in Neuro-Ophthalmology.

de Souza, Daniel N / Bell, Carter A / Elkin, Zachary P / Grossman, Scott N

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society

2023

Language	English
Publishing date	2023-08-04
Publishing country	United States
Document type	Journal Article
ZDB-ID	1189901-3
ISSN	1536-5166 ; 1070-8022
ISSN (online)	1536-5166
ISSN	1070-8022
DOI	10.1097/WNO.0000000000001966
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Article ; Online: Vision as a piece of the head trauma puzzle.

Bell, Carter A / Grossman, Scott N / Balcer, Laura J / Galetta, Steven L

Eye (London, England)

2023 Volume 37, Issue 12, Page(s) 2385–2390

Abstract: Approximately half of the brain's circuits are involved in vision and control of eye movements. Therefore, visual dysfunction is a common symptom of concussion, the mildest form of traumatic brain injury (TBI). Photosensitivity, vergence dysfunction, ... ...

Abstract	Approximately half of the brain's circuits are involved in vision and control of eye movements. Therefore, visual dysfunction is a common symptom of concussion, the mildest form of traumatic brain injury (TBI). Photosensitivity, vergence dysfunction, saccadic abnormalities, and distortions in visual perception have been reported as vision-related symptoms following concussion. Impaired visual function has also been reported in populations with a lifetime history of TBI. Consequently, vision-based tools have been developed to detect and diagnose concussion in the acute setting, and characterize visual and cognitive function in those with a lifetime history of TBI. Rapid automatized naming (RAN) tasks have provided widely accessible and quantitative measures of visual-cognitive function. Laboratory-based eye tracking approaches demonstrate promise in measuring visual function and validating results from RAN tasks in patients with concussion. Optical coherence tomography (OCT) has detected neurodegeneration in patients with Alzheimer's disease and multiple sclerosis and may provide critical insight into chronic conditions related to TBI, such as traumatic encephalopathy syndrome. Here, we review the literature and discuss the future directions of vision-based assessments of concussion and conditions related to TBI.
MeSH term(s)	Humans ; Craniocerebral Trauma ; Brain Concussion/diagnosis ; Vision Disorders/diagnosis ; Vision Disorders/etiology ; Eye Movements ; Saccades
Language	English
Publishing date	2023-02-17
Publishing country	England
Document type	Journal Article ; Review ; Comment
ZDB-ID	91001-6
ISSN	1476-5454 ; 0950-222X
ISSN (online)	1476-5454
ISSN	0950-222X
DOI	10.1038/s41433-023-02437-8
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Zs.B 3010: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Myelin Oligodendrocyte Glycoprotein Antibody Disease Optic Neuritis: A Structure-Function Paradox?

Ross, Ruby / Kenney, Rachel / Balcer, Laura J / Galetta, Steven L / Krupp, Lauren / O'Neill, Kimberly A / Grossman, Scott N

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society

2024

Abstract: ... was associated with global thickness, PMB thickness, and nasal/temporal (N/T) ratio, and color vision ... higher N:T ratio in clinically affected eyes compared with unaffected eyes in the acute MOG-ON setting (P ... significant retinal atrophy in MOG-ON. The N:T ratio in acute clinically affected MOG-ON eyes in our study was ...

Abstract	Background: Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is a demyelinating disorder that most commonly presents with optic neuritis (ON) and affects children more often than adults. We report 8 pediatric patients with MOG-associated ON and characterize focal optical coherence tomography (OCT) abnormalities over time that help distinguish this condition from the trajectories of other demyelinating disorders. These OCT findings are examined in the context of longitudinal visual function testing. Methods: This is a retrospective case series of 8 pediatric patients with MOG-associated ON who were referred for neuro-ophthalmic evaluation. Longitudinal data for demographics, clinical history, physical examination, and OCT obtained in the course of clinical evaluations were collected through retrospective medical record review. Results: Patients demonstrated acute peripapillary retinal nerve fiber layer (RNFL) thickening in one or both eyes, consistent with optic disc swelling. This was followed by steady patterns of average RNFL thinning, with 9 of 16 eyes reaching significantly low RNFL thickness using OCT platform reference databases (P < 0.01), accompanied by paradoxical recovery of high-contrast visual acuity (HCVA) in every patient. There was no correlation between HCVA and any OCT measures, although contrast sensitivity (CS) was associated with global thickness, PMB thickness, and nasal/temporal (N/T) ratio, and color vision was associated with PMB thickness. There was a lower global and papillomacular bundle (PMB) thickness (P < 0.01) in clinically affected eyes compared with unaffected eyes. There was also a significantly higher N:T ratio in clinically affected eyes compared with unaffected eyes in the acute MOG-ON setting (P = 0.03), but not in the long-term setting. Conclusions: MOG shows a pattern of prominent retinal atrophy, as demonstrated by global RNFL thinning, with remarkable preservation of HCVA but remaining deficits in CS and color vision. These tests may be better clinical markers of vision changes secondary to MOG-ON. Of the OCT parameters measured, PMB thickness demonstrated the most consistent correlation between structural and functional measures. Thus, it may be a more sensitive marker of clinically significant retinal atrophy in MOG-ON. The N:T ratio in acute clinically affected MOG-ON eyes in our study was higher than the N:T ratio of neuromyelitis optica (NMO)-ON eyes and similar to the N:T ratio in multiple sclerosis (MS)-ON eyes as presented in the prior literature. Therefore, MOG may share a more similar pathophysiology to MS compared with NMO.
Language	English
Publishing date	2024-03-25
Publishing country	United States
Document type	Journal Article
ZDB-ID	1189901-3
ISSN	1536-5166 ; 1070-8022
ISSN (online)	1536-5166
ISSN	1070-8022
DOI	10.1097/WNO.0000000000002124
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Article ; Online: Relapsing White Matter Disease and Subclinical Optic Neuropathy: From the National Multiple Sclerosis Society Case Conference Proceedings

O'Neill, Kimberly A / Dugue, Andrew / Abreu, Nicolas J / Balcer, Laura J / Branche, Marc / Galetta, Steven / Graves, Jennifer / Kister, Ilya / Magro, Cynthia / Miller, Claire / Newsome, Scott D / Pappas, John / Rucker, Janet / Steigerwald, Connolly / William, Christopher M / Zamvil, Scott S / Grossman, Scott N / Krupp, Lauren B

Neurology(R) neuroimmunology & neuroinflammation

2024 Volume 11, Issue 2, Page(s) e200194

Abstract: A 16-year-old adolescent boy presented with recurrent episodes of weakness and numbness. Brain MRI demonstrated subcortical, juxtacortical, and periventricular white matter T2 hyperintensities with gadolinium enhancement. CSF was positive for oligoclonal ...

Abstract	A 16-year-old adolescent boy presented with recurrent episodes of weakness and numbness. Brain MRI demonstrated subcortical, juxtacortical, and periventricular white matter T2 hyperintensities with gadolinium enhancement. CSF was positive for oligoclonal bands that were not present in serum. Despite treatment with steroids, IV immunoglobulins, plasmapheresis, and rituximab, he continued to have episodes of weakness and numbness and new areas of T2 hyperintensity on imaging. Neuro-ophthalmologic examination revealed a subclinical optic neuropathy with predominant involvement of the papillomacular bundle. Genetic evaluation and brain biopsy led to an unexpected diagnosis.
MeSH term(s)	Adolescent ; Male ; Humans ; Contrast Media ; Hypesthesia ; Gadolinium ; Optic Nerve Diseases/diagnosis ; Optic Nerve Diseases/etiology ; Leukoencephalopathies
Chemical Substances	Contrast Media ; Gadolinium (AU0V1LM3JT)
Language	English
Publishing date	2024-01-05
Publishing country	United States
Document type	Journal Article
ZDB-ID	2767740-0
ISSN	2332-7812 ; 2332-7812
ISSN (online)	2332-7812
ISSN	2332-7812
DOI	10.1212/NXI.0000000000200194
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Article ; Online: Spastic Paraplegia Type 7-Associated Optic Neuropathy: A Case Series.

Bell, Carter A / Ko, Melissa W / Mackay, Devin D / Bursztyn, Lulu L C D / Grossman, Scott N

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society

2023

Abstract: Background: Hereditary optic neuropathies comprise a group of clinically and genetically heterogeneous disorders. Optic neuropathy has been previously reported in families with spastic paraplegia type 7 (SPG7) gene mutations. However, the typical time ... ...

Abstract	Background: Hereditary optic neuropathies comprise a group of clinically and genetically heterogeneous disorders. Optic neuropathy has been previously reported in families with spastic paraplegia type 7 (SPG7) gene mutations. However, the typical time course and clinical presentation of SPG7-associated optic neuropathy is poorly understood. We report a series of 5 patients harboring pathogenic SPG7 mutations who originally presented to a neuro-ophthalmology clinic with symptoms of optic neuropathy. Methods: Retrospective case series of 5 patients with pathogenic SPG7 mutations and optic atrophy from 3 neuro-ophthalmology clinics. Demographic, clinical, diagnostic, and treatment data were collected and reported by the clinician authors. Results: Five patients ranging in age from 8 to 48 years were evaluated in the neuro-ophthalmology clinic. Although there were variable clinical presentations for each subject, all noted progressive vision loss, typically bilateral, and several also had previous diagnoses of peripheral neuropathy (e.g., Guillain-Barré Syndrome). Patients underwent neuro-ophthalmic examinations and testing with visual fields and optic coherence tomography of the retinal nerve fiber layer. Genetic testing revealed pathogenic variants in the SPG7 gene. Conclusions: Five patients presented to the neuro-ophthalmology clinic with progressive vision loss and were diagnosed with optic atrophy. Although each patient harbored an SPG7 mutation, this cohort was phenotypically and genotypically heterogeneous. Three patients carried the Ala510Val variant. The patients demonstrated varying degrees of visual acuity and visual field loss, although evaluations were completed during different stages of disease progression. Four patients had a previous diagnosis of peripheral neuropathy. This raises the prospect that a single pathogenic variant of SPG7 may be associated with peripheral neuropathy in addition to optic neuropathy. These results support the consideration of SPG7 testing in patients with high suspicion for genetic optic neuropathy, as manifested by symmetric papillomacular bundle damage without clear etiology on initial workup. Applied judiciously, genetic testing, including for SPG7, may help clarify the cause of unexplained progressive optic neuropathies.
Language	English
Publishing date	2023-11-20
Publishing country	United States
Document type	Journal Article
ZDB-ID	1189901-3
ISSN	1536-5166 ; 1070-8022
ISSN (online)	1536-5166
ISSN	1070-8022
DOI	10.1097/WNO.0000000000002039
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Article ; Online: Treatment of Periodic Alternating Nystagmus as a Consequence of Ataxia-Telangiectasia.

Jauregui, Ruben / Bhagat, Dhristie / Garcia, Mekka R / Miller, Claire / Grossman, Scott N

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society

2022 Volume 44, Issue 1, Page(s) e151–e152

MeSH term(s)	Humans ; Ataxia Telangiectasia/complications ; Ataxia Telangiectasia/diagnosis ; Nystagmus, Pathologic/diagnosis ; Nystagmus, Pathologic/etiology
Language	English
Publishing date	2022-10-21
Publishing country	United States
Document type	Journal Article
ZDB-ID	1189901-3
ISSN	1536-5166 ; 1070-8022
ISSN (online)	1536-5166
ISSN	1070-8022
DOI	10.1097/WNO.0000000000001739
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Article: Precision Concussion Management: Approaches to Quantifying Head Injury Severity and Recovery.

de Souza, Daniel N / Jarmol, Mitchell / Bell, Carter A / Marini, Christina / Balcer, Laura J / Galetta, Steven L / Grossman, Scott N

Brain sciences

2023 Volume 13, Issue 9

Abstract: Mitigating the substantial public health impact of concussion is a particularly difficult challenge. This is partly because concussion is a highly prevalent condition, and diagnosis is predominantly symptom-based. Much of contemporary concussion ... ...

Abstract	Mitigating the substantial public health impact of concussion is a particularly difficult challenge. This is partly because concussion is a highly prevalent condition, and diagnosis is predominantly symptom-based. Much of contemporary concussion management relies on symptom interpretation and accurate reporting by patients. These types of reports may be influenced by a variety of factors for each individual, such as preexisting mental health conditions, headache disorders, and sleep conditions, among other factors. This can all be contributory to non-specific and potentially misleading clinical manifestations in the aftermath of a concussion. This review aimed to conduct an examination of the existing literature on emerging approaches for objectively evaluating potential concussion, as well as to highlight current gaps in understanding where further research is necessary. Objective assessments of visual and ocular motor concussion symptoms, specialized imaging techniques, and tissue-based concentrations of specific biomarkers have all shown promise for specifically characterizing diffuse brain injuries, and will be important to the future of concussion diagnosis and management. The consolidation of these approaches into a comprehensive examination progression will be the next horizon for increased precision in concussion diagnosis and treatment.
Language	English
Publishing date	2023-09-21
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2651993-8
ISSN	2076-3425
ISSN	2076-3425
DOI	10.3390/brainsci13091352
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