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Current Opinion in Ophthalmology Nov 2023Opsoclonus and ocular flutter are saccadic intrusions characterized by spontaneous, back-to-back, fast eye movements (saccades) that oscillate about the midline of... (Review)
Review
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.
Topics: Humans; Ocular Motility Disorders; Eye Movements; Saccades
PubMed: 37603546
DOI: 10.1097/ICU.0000000000000998 -
Seminars in Neurology Mar 1996Opsoclonus is a rare disorder of the saccadic system, in which fixation is continuously interrupted by multivectorial, back-to-back saccades that at times can be seen... (Review)
Review
Opsoclonus is a rare disorder of the saccadic system, in which fixation is continuously interrupted by multivectorial, back-to-back saccades that at times can be seen only with an ophthalmoscope. To diagnose it reliably, eye movement recording is required. Opsoclonus may be a harbinger of an occult malignancy, though many cases are postinfectious, toxic-metabolic or idiopathic. The underlying malignancy is usually neural crest tumors in children and lung, breast, or gynecologic cancer in adults. Opsoclonus can be accompanied by myoclonus and ataxia. Concurrent appearance of oscillations affecting eyes and limbs suggests a common brainstem generator. Dysfunction of the glycinergic omnipause neurons in the nucleus raphe interpositus has been proposed. Autoantibodies against neural epitopes shared with a tumor are implicated in the pathogenesis of opsoclonus in paraneoplastic cases. Because of the association with malignancies, full oncological work-up is indicated in every case. Coexisting opsoclonus carries a relatively good prognosis for the cancer; however, the neurologic disability may remain even if the tumor has been arrested. New, potentially effective immunoadsorption therapy for opsoclonus is currently under investigation.
Topics: Adult; Child, Preschool; Female; Humans; Male; Middle Aged; Ocular Motility Disorders; Saccades
PubMed: 8879053
DOI: 10.1055/s-2008-1040955 -
Pediatric Neurology Apr 2021
Topics: COVID-19; Child, Preschool; Encephalitis; Humans; Infant; Ocular Motility Disorders; Opsoclonus-Myoclonus Syndrome; Receptors, N-Methyl-D-Aspartate; SARS-CoV-2
PubMed: 33662888
DOI: 10.1016/j.pediatrneurol.2020.12.009 -
Archives de Pediatrie : Organe Officiel... Feb 2019Opsoclonus consists of massive erratic rapid eye jerks. They may occur in isolation or in association with myoclonus and ataxia, i.e., opsoclonus-myoclonus syndrome... (Review)
Review
Opsoclonus consists of massive erratic rapid eye jerks. They may occur in isolation or in association with myoclonus and ataxia, i.e., opsoclonus-myoclonus syndrome (OMS). We report the case of a 9-year-old girl who suffered from headaches for several days and was shown to have opsoclonus and left peripheral facial palsy. Work-up excluded the diagnosis of neuroblastoma, but CSF analysis showed aseptic meningitis, and serology for Borrelia burgdorferi (Lyme) was positive. The outcome was favorable with complete regression of symptoms after treatment with ceftriaxone 2g/day for 3 weeks. Although rare, the diagnosis of Lyme neuroborreliosis must be raised in the presence of isolated opsoclonus, particularly if the clinical picture is incomplete and if other features, such as peripheral facial palsy and pleocytosis in the CSF, are present.
Topics: Borrelia burgdorferi; Child; Female; Humans; Lyme Neuroborreliosis; Ocular Motility Disorders
PubMed: 30655046
DOI: 10.1016/j.arcped.2018.11.013 -
Current Opinion in Neurology Feb 2007The aim of this article is to review opsoclonus, with particular emphasis on its immunopathogenesis and pathophysiology. (Review)
Review
PURPOSE OF REVIEW
The aim of this article is to review opsoclonus, with particular emphasis on its immunopathogenesis and pathophysiology.
RECENT FINDINGS
Infections (West Nile virus, Lyme disease), neoplasms (non-Hodgkin's lymphoma, renal adenocarcinoma), celiac disease, and allogeneic hematopoietic stem cell transplantation can cause opsoclonus. Newly identified autoantibodies include antineuroleukin, antigliadin, antiendomysial, and anti-CV2. Evidence suggests that the autoantigens of opsoclonus reside in postsynaptic density, or on the cell surface of neurons or neuroblastoma cells (where they exert antiproliferative and proapoptotic effects). Most patients, however, are seronegative for autoantibodies. Cell-mediated immunity may also play a role, with B and T-cell recruitment in the cerebrospinal fluid linked to neurological signs. Rituximab, an anti-CD20 monoclonal antibody, seems efficacious as an adjunctive therapy. Although changes in synaptic weighting of saccadic burst neuron circuits in the brainstem have been implicated, disinhibition of the fastigial nucleus in the cerebellum, or damage to afferent projections to the fastigial nucleus, is a more plausible pathophysiologic mechanism which is supported by functional magnetic resonance imaging findings in patients.
SUMMARY
There is increasing recognition that both humoral and cell mediated immune mechanisms are involved in the pathogenesis of opsoclonus. Further studies are needed to further elucidate its immunopathogenesis and pathophysiology in order to develop novel and efficacious therapy.
Topics: Autoantibodies; Autoantigens; Autoimmune Diseases of the Nervous System; Brain Stem; Cerebellar Nuclei; Humans; Immunosuppressive Agents; Nerve Net; Ocular Motility Disorders; Opsoclonus-Myoclonus Syndrome
PubMed: 17215685
DOI: 10.1097/WCO.0b013e3280126b51 -
BMJ Case Reports Feb 2017
Topics: Adolescent; Gait Ataxia; Humans; Male; Myoclonus; Ocular Motility Disorders
PubMed: 28202488
DOI: 10.1136/bcr-2017-219433 -
BMJ Case Reports May 2024
Topics: Humans; Scrub Typhus; Male; Ocular Motility Disorders; Anti-Bacterial Agents
PubMed: 38729659
DOI: 10.1136/bcr-2024-260304 -
Der Nervenarzt Aug 1997Ocular flutter is an ocular motor disorder consisting of involuntary back-to-back saccades in the horizontal plane without a saccadic interval. In opsoclonus, these... (Review)
Review
Ocular flutter is an ocular motor disorder consisting of involuntary back-to-back saccades in the horizontal plane without a saccadic interval. In opsoclonus, these pathological eye movements occur not only in the horizontal but also in the vertical plane. Originally, opsoclonus was described as irregular, conjugate, chaotic and partially continuous rapid eye movements. In this review, the clinical picture, differential diagnosis, etiology, natural course and therapeutic concepts for these eye movement disorders are described. It is stressed that continuous and intermittent forms of opsoclonus can be distinguished and that these differences usually correlate with the severity of the underlying disease. In contrast, the intermittent forms of opsoclonus and ocular flutter are usually only distinguished by the lack of a vertical eye movement component in ocular flutter.
Topics: Adult; Child; Diagnosis, Differential; Electrooculography; Humans; Ocular Motility Disorders; Paraneoplastic Syndromes; Prognosis
PubMed: 9380208
DOI: 10.1007/s001150050173 -
Journal of Neuro-ophthalmology : the... Mar 2022A 78-year-old man was evaluated for altered mentation in the setting of significant uremia. On examination, he was found to be encephalopathic with generalized myoclonus...
A 78-year-old man was evaluated for altered mentation in the setting of significant uremia. On examination, he was found to be encephalopathic with generalized myoclonus and spontaneous opsoclonus. He had no known risk factors for the development of opsoclonus and upon undergoing hemodialysis, experienced near resolution of his eye movement abnormalities, thus highlighting a possible link between the uremic state and opsoclonus.
Topics: Aged; Female; Humans; Male; Ocular Motility Disorders; Renal Dialysis; Uremia
PubMed: 34270515
DOI: 10.1097/WNO.0000000000001327 -
The New England Journal of Medicine Sep 2020
Topics: Aged; Antigens, Neoplasm; Autoantibodies; Brain Stem; Encephalitis; Humans; Male; Nerve Tissue Proteins; Ocular Motility Disorders
PubMed: 32966725
DOI: 10.1056/NEJMicm1914516