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Published in Chung Nen Chua, Li Wern Voon, Siddhartha Goel, Ophthalmology Fact Fixer, 2017
Opsoclonus is characterised by spontaneous chaotic rapid conjugate bursts of ocular saccades in all directions. In children it may be associated with neuroblastoma. Other associations include occult visceral carcinoma in adults and post-viral encephalitis. Ocular bobbing is characterised by fast downward ocular movements followed by a slow return of the eyes to the primary position. It is seen in comatose patients with major intracranial haemorrhage. Ocular flutter involves to-and-fro horizontal oscillations that occur when the patient attempts to fixate on a target in the primary position. It is associated with cerebellar lesion. Ocular myoclonus initially resembles ocular bobbing and then becomes more pendular. It is seen in patients with pontine stroke. Spasmus nutan is made up of a triad of nystagmus, involuntary head movement and an abnormal head posture. It usually resolves spontaneously and has no underlying cause.
Opsoclonus myoclonus and ataxia syndrome with supraventricular tachycardia
Published in Baylor University Medical Center Proceedings, 2023
Sydney Garner, Alec Giakas, Katherine Holder, Bernardo Galvan, Hollie Edwards
Opsoclonus-myoclonus and ataxia syndrome (OMAS) is a rare movement disorder in children. Onset is usually between 1 and 3 years of age.1 It is often associated with an underlying neuroblastoma or considered to be parainfectious. Diagnosis can be difficult and requires three of four criteria to be met: opsoclonus or ocular flutter, myoclonus or ataxia, behavioral or sleep disturbances, and neuroblastoma.2 OMAS is treated with immunomodulatory drugs such as steroids, intravenous immunoglobulin (IVIG), and rituximab. Regardless of etiology, early recognition and treatment of OMAS is important to prevent permanent neurologic sequelae.2 To better characterize the clinical profile of this syndrome and its associated conditions, we present a case report of a 9-month-old male experiencing supraventricular tachycardia (SVT) and OMAS without an associated neuroblastoma.
The challenges faced by clinicians diagnosing and treating infantile nystagmus Part II: treatment
Published in Expert Review of Ophthalmology, 2021
Eleni Papageorgiou, Katerina Lazari, Irene Gottlob
Occasionally, infantile nystagmus can be a manifestation of neurological disease, and in these cases, it is usually associated with other red flags pointing at the underlying etiology [5]. Immediate neuroimaging investigation for chiasmal and optic nerve tumors is required in cases of asymmetrical (dissociated) or unilateral nystagmus, vertical or torsional nystagmus (in the absence of retinal disease), acquired nystagmus, and in the presence of accompanying neurological and ophthalmological findings, such as RAPD, optic atrophy, or papilledema [6,7]. For example, monocular vertical nystagmus can be associated with chiasmal or hypothalamic gliomas [8,9]. In addition, patients with see-saw nystagmus should be investigated for suprasellar lesions that compress the mesodiencephalic region [10]. See-saw nystagmus is a type of pendular nystagmus in which there is simultaneous intorsion and elevation of one eye and extorsion and depression of the fellow eye [10]. Patients with achiasma may also present with see-saw nystagmus [11]. Nystagmus should be also differentiated form saccadic intrusions and oscillations, which are abnormal fast eye movements, disrupt visual fixation, and occur in association with cerebellar, brainstem, or cerebral pathology [12]. They are clearly non-rhythmic and are initiated by saccades rather than by the slow (smooth) eye movements, which initiate nystagmus. These are uncommon in children and include square-wave jerks, opsoclonus, ocular flutter and ocular bobbing. In these cases neuroimaging is indicated due to the possible association with systemic abnormalities, i.e. opsoclonus and neuroblastoma [12].
Neuro-ophthalmology of movement disorders
Published in Expert Review of Ophthalmology, 2018
Patients with psychogenic disorders often have ocular complaints and symptoms such as sudden monocular or binocular blindness, tunnel visual field defects, diplopia, blurry vision, oculogyria, voluntary saccadic oscillations that can mimic opsoclonus or ocular flutter, psychogenic BSP or HFS. Two-thirds of patients with psychogenic movement disorders are found to have convergence spasm, manifested by transient dysconjugate gaze with asymmetric ocular convergence, miosis, and accommodation evoked by examination of horizontal gaze mimicking abducens palsy (Figure 2) [83]. It is important to mention that convergence spasm is not specific for psychogenic disorders and can be seen in healthy individuals, subjects with encephalopathy, multiple sclerosis, brainstem lesions, inherited disorders of monoamine metabolism, and strabismus. Of the spontaneous ophthalmologic movement disorders (not elicited by any maneuver such as oculomotor examination), opsoclonus, oculogyria (transient spasms of extraocular muscles leading to abnormal eye deviation), unilateral or bilateral blepharospasm, and atypical hemifacial spasm are the most common [75,84,85]. Acute onset of the symptoms, inconsistent signs, non-progressive course with marked fluctuations, spontaneous remissions, distractibility of the symptoms, response to suggestion, or psychotherapy are common characteristics of functional (psychogenic) disorders [84]. Many patients with functional eye movements have extraocular functional movement disorders including dystonia, gait abnormality, and tremor. In most cases, clinical observation and history of the symptoms evolution are sufficient for the diagnosis. Ancillary diagnostic tests including neuroimaging, neurophysiologic tests can be considered if there are findings on the examination concerning for organic neurological disorder that sometimes can coexist with psychogenic symptoms.