Nystagmus In Childhood Research Articles

Calming the Visual Storm: Management of Childhood Nystagmus

Childhood nystagmus creates a visual storm both for the affected child and the treating doctor. This problem occurring in the development phase of the child affects not only the vision but also the general development, if not diagnosed and managed in time. Moreover, some forms may even harbor a neurological tumor needing timely management. First, a brief introduction of nystagmus classification, a simplified approach to diagnose the common childhood nystagmus, and the value of electrophysiology will be presented. Next, the approach to treatment, using a thorough clinical examination, illustrated by patient examples of different types of nystagmus will be presented. The different forms of childhood nystagmus are described: idiopathic infantile nystagmus syndrome (IINS), sensory nystagmus (SN), fusion maldevelopment nystagmus (FMDN), spasmus nutans syndrome (SNS), nystagmus blockage syndrome, periodic alternating nystagmus, and others as well as their specific management. The role of electronystagmography and that of neuroimaging in specific conditions is life saving and is described. The role of auditory biofeedback, acupuncture, medical treatment, and surgical procedures like Augmented Anderson procedure, Hertle-Dell'Osso procedure, supra maximal retro-equatorial recession, and posterior fixation have been elucidated. Newer techniques have simplified the management options and improved the functional outcomes in childhood nystagmus. To conclude, children with nystagmus of types IINS, FMDN, SNS, or SN need to be managed differently. It is thus possible to timely manage these children, not only to save their life and improve their vision but also to improve their living quotient.

Evaluation of the diagnostic value of visual electrophysiological examination in childhood nystagmus

Objectives To evaluate the use of electroretinography (ERG) and recording of visual-evoked potentials (VEP) as diagnostic tools in children with early-onset nystagmus to clarify the need to include them as a basic step in the evaluation of cases of early-onset nystagmus, especially with normal ocular examination. Background Nystagmus in children can be caused by many causes, starting from idiopathic motor nystagmus compatible with relatively good vision, ending with ocular abnormalities that impair vision or other neurologic abnormalities that may be life threatening. Knowledge of the exact diagnosis is essential in the rehabilitation of a visually impaired child. Patients and methods In 32 children with early-onset nystagmus, ERG was recorded under light sedation, and VEP were simultaneously recorded to flash stimulus in all patients. Results The study comprised 64 eyes of 32 patients, with their age ranging from 3 months to 7 years. Indications for referral included children with early-onset nystagmus. Children with obvious cause for nystagmus were excluded from the study. Overall, 37.5% of children had been diagnosed as having retinal dysfunction, including cone dystrophy (6.3%) and cone/rod dystrophy (31.2%). Neurological nystagmus was recognized in 34.3% of children. Ocular albinism was diagnosed in 6.3% of cases. One (3.1%) case was diagnosed as optic nerve hypoplasia. Six (18.8%) cases were diagnosed as idiopathic nystagmus. Conclusion This study clarified the need to investigate children with nystagmus by ERG and suggested that the ERG was useful where the diagnosis was uncertain, particularly at an early age with no obvious cause on ocular examination. VEP are complementary to ERG and can be tested simultaneously. Visual electrophysiological examination of children with early-onset nystagmus can establish or exclude retinal and postretinal pathway dysfunction, thus assisting in its classification and subsequent rehabilitation.

Incidence and Types of Pediatric Nystagmus

To report the incidence, prevalent subtypes, and clinical characteristics of pediatric nystagmus diagnosed over a 30-year period. Retrospective, population-based study. Using the Rochester Epidemiology Project, we reviewed the medical records of all children (<19 years) diagnosed as residents in Olmsted County, Minnesota, with any form of nystagmus from January 1, 1976, through December 31, 2005. Seventy-one children were diagnosed during the 30-year period, yielding an annual incidence of 6.72 per 100,000 younger than 19 years (95% confidence interval [CI], 5.15-8.28) Infantile nystagmus, onset by 6months, comprised 62 (87.3%) of the study patients, corresponding to a birth prevalence of 1 in 821. The median age at diagnosis for the cohort was 12.7months (range, 0days to 18.6 years) and 42 subjects (59.2%) were male. The main types of nystagmus, in declining order, were nystagmus associated with retinal/optic nerve disease in 23 (32.4%), idiopathic or congenital motor nystagmus in 22 (31.0%), manifest latent nystagmus or latent nystagmus in 17 (24.0%), and 2 (2.8%) each associated with Chiari malformation, medication use, tumor of the central nervous system, and no diagnosis. Developmental delay was diagnosed in 31 (43.6%), strabismus in 25 (35.2%), and amblyopia in 10 (14.1%). Eighty percent had 20/40 (or equivalent) or better vision at presentation in at least 1 eye. This study provides population-based data on incidence and clinical characteristics of childhood nystagmus in North America. Idiopathic and nystagmus associated with retinal/optic nerve disease were the most common presentations, with most patients having good vision. Developmental delay, strabismus, and amblyopia were common in this cohort.

Portable nonsedated electroretinogram evaluation of children with nystagmus in the pediatric ophthalmology clinic

Childhood nystagmus is a condition frequently evaluated by pediatric ophthalmologists. In young children with nystagmus and anatomically normal eyes, an electroretinogram (ERG) under general anesthesia may be performed to determine if a hereditary retinal disease is present. Sedated ERG in young children is costly, time-consuming, unavailable to many clinicians, and carries the risk of general anesthesia. We evaluated a novel, FDA-approved form of a non-sedated, handheld ERG examination (RetEval) as a screening tool for retinal dysfunction.

Management of childhood nystagmus: Knapp lecturer workshop

To summarize the instructors' experience and provide the scientific basis of recommendations for a simplified medical and surgical treatment plan for childhood forms of nystagmus to the practicing clinician.

Incidence and types of pediatric nystagmus

Purpose To report the incidence, prevalent subtypes, and clinical characteristics of pediatric nystagmus diagnosed over a 30-year period. Design Retrospective, population-based study. Methods Using the Rochester Epidemiology Project, we reviewed the medical records of all children ( Results Seventy-one children were diagnosed during the 30-year period, yielding an annual incidence of 6.72 per 100,000 younger than 19 years (95% confidence interval [CI], 5.15–8.28) Infantile nystagmus, onset by 6 months, comprised 62 (87.3%) of the study patients, corresponding to a birth prevalence of 1 in 821. The median age at diagnosis for the cohort was 12.7 months (range, 0 days to 18.6 years) and 42 subjects (59.2%) were male. The main types of nystagmus, in declining order, were nystagmus associated with retinal/optic nerve disease in 23 (32.4%), idiopathic or congenital motor nystagmus in 22 (31.0%), manifest latent nystagmus or latent nystagmus in 17 (24.0%), and 2 (2.8%) each associated with Chiari malformation, medication use, tumor of the central nervous system, and no diagnosis. Developmental delay was diagnosed in 31 (43.6%), strabismus in 25 (35.2%), and amblyopia in 10 (14.1%). Eighty percent had 20/40 (or equivalent) or better vision at presentation in at least 1 eye. Conclusions This study provides population-based data on incidence and clinical characteristics of childhood nystagmus in North America. Idiopathic and nystagmus associated with retinal/optic nerve disease were the most common presentations, with most patients having good vision. Developmental delay, strabismus, and amblyopia were common in this cohort.

Diagnosis, assessment and management of nystagmus in childhood

Abstract Nystagmus is a rhythmical, repetitive and involuntary movement of the eyes. It is usually from side to side, but sometimes up and down or in a circular motion. It can be distressing to the child and their carers and leads to reduced visual acuity due to the excessive motion of images on the retina. It is helpful to distinguish between infantile nystagmus and acquired nystagmus. Infantile nystagmus usually appears by 3–6 months. Infantile nystagmus can occur with poor vision, or visual deprivation in early life due to congenital cataracts, optic nerve hypoplasia, retinal dystrophies or disease, can be associated to albinism or it can be part of a neurological syndromes or diseases. Neurological disease should be suspected when the nystagmus is asymmetrical or unilateral. Infantile nystagmus can be hereditary, the most common and best analysed form being a mutation of the FRMD7 gene on chromosome Xq26.2. Novel methods to record eye movements and recent advances in the field of genetics have contributed to the improvement in diagnosis and management of nystagmus in children. Glasses, contact lenses and prisms help in improving vision. Pharmacological treatment exists to reduce nystagmus; however, as there are very few randomized controlled trials in this area, most pharmacological treatment options in nystagmus remain empirical. Eye muscle surgery may help in children with abnormal head posture to correct the defect and alleviate neck problems that can arise due to an abnormal head posture.

Benign intermittent upbeat nystagmus in infancy: A new clinical entity

BackgroundUpbeat nystagmus in childhood is a rare phenomenon, which is classically associated with neurological or anterior visual pathway dysfunction. MethodsWe report a series of five infants with an intermittent upbeat nystagmus, without any other ocular or neurological abnormality, having spontaneously resolved before the age of 14 months. ResultsNystagmus beating frequency was between 1.5 and 2.5 Hz for all patients, amplitude was remarkably large due to a baseline position in downgaze. The nystagmus mostly occurred in supine position and could be triggered by head rotations in the supine position. Age of onset was from the first weeks of life to two months; age of resolution was 5–14 months. All infants underwent complete clinical examination with ophthalmological, oto-neurological and neurological assessments and MRI-scan. ConclusionsClinical characteristics of the nystagmus suggest an involvement of the otolithic system, with a secondary recalibration of the vestibulo-ocular pathways; however, no obvious vestibular abnormality was found. The pathophysiology of this nystagmus therefore remains unclear.

Intracranial Pathology in Young Children With Apparently Isolated Nystagmus

To assess the incidence of intracranial pathology in young children who had brain magnetic resonance imaging (MRI) to investigate apparently isolated nystagmus noted within the first few years of life. Retrospective institutional review of such children (up to 5 years old). Twenty-six patients (17 boys, 9 girls) were identified. Nystagmus was clinically bilateral in 22 (85%) and unilateral in 4 (15%). Seventeen cases (65%) were described as horizontal pendular nystagmus, 5 (19%) as horizontal jerk nystagmus, 2 (8%) as vertical pendular nystagmus, and 1 each as downbeat nystagmus and torsional nystagmus (8%). Three of the 26 patients (12%) had significant MRI findings and all three had temporal optic nerve head pallor noted during clinical examination. Nystagmus was horizontal pendular in two patients and rotary in the third. The optic nerve head should be carefully assessed for signs of pallor in young children with nystagmus and, if present, brain MRI should be performed. Otherwise, isolated early childhood nystagmus is unlikely to be associated with intracranial pathology. Clinical description of nystagmus did not predict intracranial pathology in this cohort.

Apport de l’IRM dans les nystagmus congénitaux

Congenital nystagmus (CN) that is present by the age of 3 months is the most common form of nystagmus in childhood. We present a prospective study (2001-2008) in which we report imaging findings in 48 children with CN. Twenty-six boys and 22 girls with CN underwent a complete ophthalmologic assessment and a cerebral MRI (mean age of examination under general anesthesia: 11 months). Three CN groups were formed: neurologic (n=27), sensory visual disturbance (n=14), and isolated (n=7). Cerebral MRI was interpreted by the same pediatric neuroradiologist (NG). Of the children studied, 98 % were born at term. The MRI abnormalities were classified as morphologic abnormalities (malformative or nonmalformative) and as signal abnormalities. The location of brain abnormalities was within the posterior fossa, (brain stem, cerebellum, dental nuclei, cisterna magna) and the cerebral hemisphere (white matter, perivascular spaces, midline commissures, basal ganglia). Pendular nystagmus was prevalent in sensory and neurologic nystagmus. On fundus examination, optic disc abnormalities were present in 70 % (19) of neurologic CN and associated with white matter abnormalities of the optic radiations in 40 % of cases. On MRI, malformative morphologic abnormalities were present in 27 cases, nonmalformative abnormalities were found in 67, and signal abnormalities in 68. Within the brain stem, signal abnormalities were found as a cockade appearance of the posterior pons in the reticular regions (neurologic n=14, sensory n=6, isolated n=3). Other bright (most frequent) signal abnormalities were found within the dentate nuclei of the posterior fossa (neurologic n=10, sensory n=3, isolated n=3) and the cerebral white matter (neurologic n=17, sensory n=7, isolated n=5) of which 24 (neurologic n=15, sensory n=5, isolated n=4) involved the optic radiations. Most of these abnormalities were related and were seen most frequently in neurologic nystagmus. The most frequent association was signal abnormalities of the white matter, ventricular dilatation, and dilatation of the perivascular spaces (60.4 %) (neurologic n=13, sensory n=6). This study showed the fundamental contribution of the cerebral MRI in CN. Cerebral abnormalities were found at the pathways for ocular motility, particularly at the saccadic pathways.

Head and eye movements in children with low vision.

Early childhood nystagmus may herald blindness, brain tumors, benign idiopathic motor nystagmus or spasmus nutans. Nystagmus unique to low vision was sought. Videotapes and head/ eye movements of 18 congenitally visually impaired subjects were analyzed. Nystagmus of congenitally visually impaired subjects was characterized as small horizontal or vertical movements superimposed on larger oscillations. Small and large nystagmus movements were of pendular or jerk type. Slow-phase jerk nystagmus had increasing, constant and decreasing velocities. Fast-phase nystagmus changed direction periodically. Pendular nystagmus was out of phase and evolved to jerk nystagmus in two subjects. All subjects displayed head nodding, and one stabilized gaze with head movements. Eye movement recordings allow characterization and differentiation of subjects with nystagmus and low vision from other nystagmus forms.