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Continuum (Minneapolis, Minn.) Oct 2019This article discusses the varied types of paraneoplastic syndromes that commonly have neuro-ophthalmologic manifestations. Diagnostic considerations and therapeutic... (Review)
Review
PURPOSE OF REVIEW
This article discusses the varied types of paraneoplastic syndromes that commonly have neuro-ophthalmologic manifestations. Diagnostic considerations and therapeutic options for individual diseases are also discussed.
RECENT FINDINGS
Paraneoplastic syndromes can affect the afferent and efferent visual systems. Paraneoplastic syndromes may result in reduced visual acuity from retinal degeneration, alterations in melanocyte proliferation and uveal thickening, or acquired nystagmus. Ocular motor abnormalities related to paraneoplastic syndromes may present with symptoms from opsoclonus or from neuromuscular junction disease. Diagnosis remains challenging, but serologic identification of some specific antibodies may be helpful or confirmatory. Treatment, in addition to directed therapies against the underlying cancer, often requires systemic corticosteroids, plasma exchange, or immunosuppression, but some specific syndromes improve with use of targeted pharmacologic therapy.
SUMMARY
Diagnosis and therapy of paraneoplastic syndromes presenting with neuro-ophthalmic symptoms remain a challenge, but strategies are evolving and new approaches are on the horizon.
Topics: Aged; Female; Humans; Middle Aged; Ocular Motility Disorders; Optic Nerve Diseases; POEMS Syndrome; Paraneoplastic Syndromes, Nervous System; Paraneoplastic Syndromes, Ocular; Retinal Diseases; Vision Disorders
PubMed: 31584543
DOI: 10.1212/CON.0000000000000788 -
Continuum (Minneapolis, Minn.) Oct 2019This article provides an overview of nystagmus and saccadic intrusions with the goal of facilitating recognition and differentiation of abnormal eye movements to assist... (Review)
Review
PURPOSE OF REVIEW
This article provides an overview of nystagmus and saccadic intrusions with the goal of facilitating recognition and differentiation of abnormal eye movements to assist with accurate diagnosis of neurologic disease and evidence-based specific treatment of oscillopsia. Myriad advances have been made in the understanding of several types of nystagmus and saccadic intrusions, even in the past 5 to 10 years, especially regarding underlying pathophysiology, leading to pharmacologic advances rooted in physiologic principles.
RECENT FINDINGS
Specific recent advances in the study of nystagmus and saccadic intrusions include (1) improved understanding of the underlying etiologies and mechanisms of nystagmus enhanced or unmasked by provocative maneuvers such as supine position or head shaking; (2) recognition of the differences in behavior and treatment responsivity of acquired pendular nystagmus in demyelinating disease versus oculopalatal myoclonus; (3) recognition that oculopalatal myoclonus results from a dual mechanism of abnormal inferior olivary gap junction connection formation and maladaptive cerebellar learning; and (4) well-controlled clinical trials to evaluate the efficacy of pharmacologic interventions, such as memantine for acquired pendular nystagmus and 4-aminopyridine for downbeat nystagmus.
SUMMARY
Accurate recognition of nystagmus and saccadic intrusions, including familiarity with the subtleties of examination techniques that allow such eye movements to be unmasked, is critical to proper diagnosis and ultimate alleviation of the visual impairment these patients experience.
Topics: Adult; Female; Fixation, Ocular; Humans; Nystagmus, Pathologic; Ocular Motility Disorders; Saccades
PubMed: 31584542
DOI: 10.1212/CON.0000000000000772 -
BMJ Case Reports Feb 2017
Topics: Adolescent; Gait Ataxia; Humans; Male; Myoclonus; Ocular Motility Disorders
PubMed: 28202488
DOI: 10.1136/bcr-2017-219433 -
Journal of Neuro-ophthalmology : the... Jun 2021This review of disorders of the fourth cranial nerve includes discussion on anatomy, examination techniques, congenital and acquired etiologies, differential diagnosis,... (Review)
Review
This review of disorders of the fourth cranial nerve includes discussion on anatomy, examination techniques, congenital and acquired etiologies, differential diagnosis, and management options. The findings of the superior oblique muscle on orbital MRI in patients with fourth nerve palsy have had a major impact on our understanding of this cranial neuropathy. In addition, briefly reviewed are rare disorders of the fourth nerve: superior oblique myokymia, Brown syndrome, and ocular neuromyotonia. It behooves the clinician to have a clear understanding of the role that the fourth cranial nerve plays in a variety of neuro-ophthalmic conditions.
Topics: Cranial Nerve Diseases; Humans; Magnetic Resonance Imaging; Ocular Motility Disorders; Oculomotor Muscles; Trochlear Nerve
PubMed: 33999889
DOI: 10.1097/WNO.0000000000001261 -
Seminars in Ophthalmology 2018Microperimetry (MP) is a technology that allows the study of retinal sensitivity at different foveal and parafoveal areas as well as eye fixation. It is a technique of... (Review)
Review
Microperimetry (MP) is a technology that allows the study of retinal sensitivity at different foveal and parafoveal areas as well as eye fixation. It is a technique of functional evaluation, providing a direct correlation between anatomical and functional outcomes. There are a great variety of studies which evaluate the repeatability or reliability of measurements obtained with this technology and also describe and explore different clinical applications. MP has been shown to be useful in the characterization of sensory and motor conditions, such as amblyopia or nystagmus. Concerning ocular pathology, several studies have confirmed the usefulness of MP for evaluating and analyzing different retinal pathological conditions, such as age-related macular degeneration or glaucoma, and for analyzing the effect of different medical or surgical treatments for these conditions. MP has also been shown to be useful for visual training or rehabilitation in some specific cases.
Topics: Fixation, Ocular; Humans; Ocular Motility Disorders; Reproducibility of Results; Retinal Diseases; Visual Field Tests; Visual Fields
PubMed: 28991503
DOI: 10.1080/08820538.2017.1375125 -
International Ophthalmology Dec 2017The European Neuromuscular Centre (ENMC) derived the term Congenital Cranial Dysinnervation Disorders in 2002 at an international workshop for a group of congenital... (Review)
Review
The European Neuromuscular Centre (ENMC) derived the term Congenital Cranial Dysinnervation Disorders in 2002 at an international workshop for a group of congenital neuromuscular diseases. CCDDs are congenital, non-progressive ophthalmoplegia with restriction of globe movement in one or more fields of gaze. This group of sporadic and familial strabismus syndromes was initially referred to as the 'congenital fibrosis syndromes' because it was assumed that the primary pathologic process starts in the muscles of eye motility. Over the last few decades, evidence has accumulated to support that the primary pathologic process of these disorders is neuropathic rather than myopathic. This is believed that for normal development of extra ocular muscles and for preservation of muscle fiber anatomy, normal intra-uterine development of the innervation to these muscles is essential. Congenital dysinnervation to these EOMs can lead to abnormal muscle structure depending upon the stage and the extent of such innervational defects. Over last few years new genes responsible for CCDD have been identified, permitting a better understanding of associated phenotypes, which can further lead to better classification of these disorders. Introduction of high-resolution MRI has led to detailed study of cranial nerves courses and muscles supplied by them. Thus, due to better understanding of pathophysiology and genetics of CCDDs, various treatment modalities can be developed to ensure good ocular alignment and better quality of life for patients suffering from the same.
Topics: Cranial Nerves; Genetic Predisposition to Disease; Humans; Ocular Motility Disorders; Oculomotor Muscles
PubMed: 27837354
DOI: 10.1007/s10792-016-0388-z -
Parkinsonism & Related Disorders Mar 2017Oculogyric crisis (OGC) describes the clinical phenomenon of sustained dystonic, conjugate and typically upward deviation of the eyes lasting from seconds to hours. It... (Review)
Review
Oculogyric crisis (OGC) describes the clinical phenomenon of sustained dystonic, conjugate and typically upward deviation of the eyes lasting from seconds to hours. It was initially observed in patients with postencephalitic parkinsonism, but since then a number of conditions have been associated with OGC. These include drug-induced reactions, hereditary and sporadic movement disorders, and focal brain lesions. Here, we systematically review the literature and discuss the spectrum of disorders associated with OGC in order to aid clinicians place this rare but distinctive clinical sign into the appropriate diagnostic context. We also provide a brief synthesis of putative pathophysiological mechanisms, as well as therapeutic recommendations based on the literature and our own experience.
Topics: Antidepressive Agents; Dopamine Antagonists; Dyskinesia, Drug-Induced; Dystonia; Humans; Ocular Motility Disorders
PubMed: 27964831
DOI: 10.1016/j.parkreldis.2016.11.012 -
International Ophthalmology Clinics 2018
Review
Topics: Child; Eye Movements; Humans; Nystagmus, Pathologic; Ocular Motility Disorders; Retrospective Studies
PubMed: 30239363
DOI: 10.1097/IIO.0000000000000243 -
Ophthalmic Plastic and Reconstructive... 2019
Topics: Adolescent; Humans; Male; Ocular Motility Disorders; Oculomotor Muscles; Synkinesis
PubMed: 30932969
DOI: 10.1097/IOP.0000000000001373 -
Journal of Parkinson's Disease 2015This review describes the oculo-visual problems likely to be encountered in Parkinson's disease (PD) with special reference to three questions: (1) are there visual... (Review)
Review
This review describes the oculo-visual problems likely to be encountered in Parkinson's disease (PD) with special reference to three questions: (1) are there visual symptoms characteristic of the prodromal phase of PD, (2) is PD dementia associated with specific visual changes, and (3) can visual symptoms help in the differential diagnosis of the parkinsonian syndromes, viz. PD, progressive supranuclear palsy (PSP), dementia with Lewy bodies (DLB), multiple system atrophy (MSA), and corticobasal degeneration (CBD)? Oculo-visual dysfunction in PD can involve visual acuity, dynamic contrast sensitivity, colour discrimination, pupil reactivity, eye movement, motion perception, and visual processing speeds. In addition, disturbance of visuo-spatial orientation, facial recognition problems, and chronic visual hallucinations may be present. Prodromal features of PD may include autonomic system dysfunction potentially affecting pupil reactivity, abnormal colour vision, abnormal stereopsis associated with postural instability, defects in smooth pursuit eye movements, and deficits in visuo-motor adaptation, especially when accompanied by idiopathic rapid eye movement (REM) sleep behaviour disorder. PD dementia is associated with the exacerbation of many oculo-visual problems but those involving eye movements, visuo-spatial function, and visual hallucinations are most characteristic. Useful diagnostic features in differentiating the parkinsonian symptoms are the presence of visual hallucinations, visuo-spatial problems, and variation in saccadic eye movement dysfunction.
Topics: Humans; Ocular Motility Disorders; Parkinson Disease; Vision Disorders; Visual Perception
PubMed: 26599301
DOI: 10.3233/JPD-150686