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Journal of Neurology Feb 2022Opsoclonus-myoclonus-ataxia syndrome is a rare neuroimmunologic disorder typically presenting in previously healthy infants and toddlers. It is characterized by a... (Review)
Review
Opsoclonus-myoclonus-ataxia syndrome is a rare neuroimmunologic disorder typically presenting in previously healthy infants and toddlers. It is characterized by a clinical triad of (1) erratic saccadic intrusions; (2) myoclonus and/or ataxia; (3) behavioral features, typified by developmental plateauing, irritability and insomnia. About half of cases are associated with an underlying neuroblastoma and diagnostic imaging is essential once OMAS is suspected. A thorough workup, including serum, urine, and cerebrospinal fluid studies is critical to identify underlying biomarkers of OMAS itself or neuroblastoma. Historically, many children had relatively poor long-term outcomes, with residual neurologic and/or neuropsychiatry sequelae typical. More recent concepts have emphasized combined immunotherapy regimens that offer hope for better outcomes in children with this remarkable, challenging disease.
Topics: Ataxia; Disease Progression; Humans; Infant; Neuroblastoma; Ocular Motility Disorders; Opsoclonus-Myoclonus Syndrome
PubMed: 33779841
DOI: 10.1007/s00415-021-10536-3 -
Journal of Optometry 2022Convergence insufficiency (CI) is a common binocular vision (BV) disorder characterized by difficulty in maintaining motor fusion at near, which affects approximately... (Review)
Review
Convergence insufficiency (CI) is a common binocular vision (BV) disorder characterized by difficulty in maintaining motor fusion at near, which affects approximately 7.5 percent of the population. Diagnostic criteria for the disorder are inconsistent, ranging from one to many clinical signs. Methodology for clinical tests is inconsistent in measurement technique, visual targets, required repetitions, and normative values. This manuscript demonstrates the inconsistencies amongst published studies, and highlights the importance of consistent clinical diagnostic signs, measurement techniques, visual targets, and cut-off criteria. For each clinical sign, the recommended methodology for the procedure is described. Several studies do not take age into account when diagnosing CI in their cohorts. As such, the review emphasizes changes in diagnostic signs with age. This manuscript highlights the need for consistent and clear procedures and diagnostic criteria amongst clinicians and provides the basis for future studies in terms of diagnostic testing required for CI of varying age groups.
Topics: Accommodation, Ocular; Convergence, Ocular; Humans; Ocular Motility Disorders; Vision Disorders; Vision, Binocular
PubMed: 34963569
DOI: 10.1016/j.optom.2021.11.002 -
Klinische Monatsblatter Fur... Nov 2021Nystagmus is defined as rhythmic, most often involuntary eye movements. It normally consists of a slow (pathological) drift of the eyes, followed by a fast central...
Nystagmus is defined as rhythmic, most often involuntary eye movements. It normally consists of a slow (pathological) drift of the eyes, followed by a fast central compensatory movement back to the primary position (refixation saccade). The direction, however, is reported according to the fast phase. The cardinal symptoms are, on the one hand, blurred vision, jumping images (oscillopsia), reduced visual acuity and, sometimes, double vision; many of these symptoms depend on the eye position. On the other hand, depending on the etiology, patients may suffer from the following symptoms: 1. permanent dizziness, postural imbalance, and gait disorder (typical of downbeat and upbeat nystagmus); 2. if the onset of symptoms is acute, the patient may experience spinning vertigo with a tendency to fall to one side (due to ischemia in the area of the brainstem or cerebellum with central fixation nystagmus or as acute unilateral vestibulopathy with spontaneous peripheral vestibular nystagmus); or 3. positional vertigo. There are two major categories: the first is spontaneous nystagmus, i.e., nystagmus which occurs in the primary position as upbeat or downbeat nystagmus; and the second includes various types of nystagmus which are induced or modified by certain factors. Examples are gaze-evoked nystagmus, head-shaking nystagmus, positional nystagmus, and hyperventilation-induced nystagmus. In addition, there are disorders similar to nystagmus, such as ocular flutter or opsoclonus. The most common central types of spontaneous nystagmus are downbeat and upbeat, infantile, pure torsional, pendular fixation, periodic alternating, and seesaw nystagmus. Many types of nystagmus allow a precise neuroanatomical localization: for instance, downbeat nystagmus, which is most often caused by a bilateral floccular lesion or dysfunction, or upbeat nystagmus, which is caused by a lesion in the midbrain or medulla. Examples of drug treatment are the use of 4-aminopyridine for downbeat and upbeat nystagmus, memantine or gabapentin for pendular fixation nystagmus, or baclofen for periodic alternating nystagmus. In this article we are focusing on nystagmus. In a second article we will focus on central ocular motor disorders, such as saccade or gaze palsy, internuclear ophthalmoplegia, and gaze-holding deficits. Therefore, these types of eye movements will not be described here in detail.
Topics: Cerebellum; Eye Movements; Humans; Nystagmus, Pathologic; Ocular Motility Disorders; Saccades
PubMed: 34784642
DOI: 10.1055/a-1525-0030 -
Journal Francais D'ophtalmologie Feb 2019Brown's syndrome is related to an abnormality of the superior oblique muscle: it is manifested by an oculomotor disorder with active and passive limitation of elevation... (Review)
Review
Brown's syndrome is related to an abnormality of the superior oblique muscle: it is manifested by an oculomotor disorder with active and passive limitation of elevation in adduction, the field of action of the inferior oblique muscle. The origin is congenital or acquired secondary to multiple causes - inflammatory-infectious, traumatic or iatrogenic. The clinical and paraclinical signs are suggestive. Cerebral and orbital imaging including CT and magnetic resonance imaging (MRI) is essential for the diagnosis and management of congenital or acquired Brown's syndrome. The course may yield improvement (spontaneous or after etiological treatment) or a steady state. Treatment is rarely surgical. The indication for surgery requires specific functional and clinical signs: torticollis, hypotropia in primary position, associated strabismus and impaired binocular vision; the long-term results of the various surgical techniques are variable.
Topics: Humans; Magnetic Resonance Imaging; Ocular Motility Disorders; Oculomotor Muscles; Strabismus; Syndrome
PubMed: 30711379
DOI: 10.1016/j.jfo.2018.06.012 -
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 -
American Journal of Ophthalmology Apr 2016
Topics: Female; Humans; Male; Ocular Motility Disorders; Oculomotor Muscles; Strabismus
PubMed: 26944754
DOI: 10.1016/j.ajo.2016.02.006 -
Practical Neurology Apr 2017A brainstem lesion of any type that involves the medial longitudinal fasciculus (MLF) can cause internuclear ophthalmoplegia (INO). This primarily affects conjugate...
A brainstem lesion of any type that involves the medial longitudinal fasciculus (MLF) can cause internuclear ophthalmoplegia (INO). This primarily affects conjugate horizontal gaze and classically manifests as impaired adduction ipsilateral to the lesion and abduction nystagmus contralateral to the lesion. Here, we describe the anatomy of the MLF and review the clinical features of INO. We also describe conjugate horizontal gaze palsy and some of the 'INO-plus' syndromes.
Topics: Afferent Pathways; Brain Stem Infarctions; Eye Movements; Female; Functional Laterality; Humans; Magnetic Resonance Imaging; Male; Nystagmus, Pathologic; Ocular Motility Disorders
PubMed: 27927777
DOI: 10.1136/practneurol-2016-001428 -
Current Opinion in Ophthalmology Nov 2022We set out to describe efferent neuro-ophthalmological complications that have been reported in association with coronavirus disease 2019 (COVID-19) infection. We... (Review)
Review
PURPOSE OF REVIEW
We set out to describe efferent neuro-ophthalmological complications that have been reported in association with coronavirus disease 2019 (COVID-19) infection. We describe syndromes affecting ocular motility and elaborate on mechanisms of disease, including para-infectious inflammation, hypercoagulability, endothelial damage, and direct neurotropic viral invasion. Despite global vaccination programs, COVID-19 continues to pose an international threat that may rarely result in diplopia or nystagmus.
RECENT FINDINGS
Efferent complications include cranial nerve palsies leading to diplopia, either isolated or in association with Miller Fisher syndrome. Nystagmus has been observed in the setting of hemorrhagic acute necrotizing encephalopathy and brainstem infarcts, and opsoclonus syndrome has been described.
SUMMARY
Observed neuro-ophthalmic associations need to be confirmed through larger comparative studies. Meanwhile, the range of possible complications should be recognized by neurologists and ophthalmologists alike, to facilitate faster diagnosis and treatment of both COVID-19 and its neuro-ophthalmic manifestations.
Topics: COVID-19; Cranial Nerve Diseases; Diplopia; Humans; Nystagmus, Pathologic; Ocular Motility Disorders
PubMed: 36165417
DOI: 10.1097/ICU.0000000000000904 -
Journal of Optometry 2020
Topics: Evidence-Based Practice; Humans; Ocular Motility Disorders; Optometry
PubMed: 32553271
DOI: 10.1016/j.optom.2020.06.001 -
Journal of Neuro-ophthalmology : the... Dec 2021Voluntary nystagmus is a common phenomenon in the general population and is believed to be under voluntary control. Some patients have an eye movement disorder...
BACKGROUND
Voluntary nystagmus is a common phenomenon in the general population and is believed to be under voluntary control. Some patients have an eye movement disorder indistinguishable from voluntary nystagmus and yet have no control over these movements.
METHODS
Four patients with involuntary eye movements consisting of spontaneous or induced bursts of saccadic eye movements with no intersaccadic interval are described. All eye movements were typical of "voluntary nystagmus," yet they denied any control over the movements. In none of the patients was there any evidence of psychopathology or secondary gain.
RESULTS
Two types of patients with involuntary eye movements that were indistinguishable from voluntary nystagmus are presented. One group of 2 patients had clear neurological disease, whereas another 2 had no evidence of any neurological pathology.
CONCLUSIONS
The patterns seen in these patients suggest that there is a spectrum of oculomotor saccadic instabilities that includes voluntary nystagmus on one extreme and ocular flutter on the other. In between these 2 are both normal and abnormal patients with eye movements indistinguishable from ocular flutter/voluntary nystagmus that may or may not be induced by convergence. It is suggested that involuntary "voluntary nystagmus" is a real condition and that psychopathology should be ruled in rather than simply assumed.
Topics: Eye Movements; Humans; Nystagmus, Pathologic; Ocular Motility Disorders; Saccades
PubMed: 34001741
DOI: 10.1097/WNO.0000000000001196