Non-vestibular nystagmus and saccadic intrusions
Abstract Introduction and objective: Nystagmus is a rhythmic and involuntary oscillation of one or both eyes in which a fixation drift and a refixation movement occur. It affects 14-17 out of 10,000 children. There are multiple underlying causes. The objective is to know the different types of non-vestibular nystagmus and non-nystagmic saccadic intrusions, with their characteristics, differential diagnosis, etiologies, diagnosis, and treatment. Method: A narrative bibliographic review has been carried out through the PubMed, Scopus, Google Scholar, and Clinical Key databases. Articles on vestibular nystagmus and clinical cases were excluded. Results: Seventeen articles, book chapters and clinical guidelines of the American Society of Ophthalmology and the Spanish Society of Ophthalmology have been analyzed. Discussion: There are various types of non-vestibular nystagmus that can debut in the pediatric age: infantile nystagmus syndrome, classically called congenital nystagmus, the most frequent; nystagmus syndrome due to maldevelopment of fusion, classically known as latent nystagmus, the second most frequent and related to amblyopia; Spasmus nutans, a syndrome characterized by nystagmus, nodding, and torticollis, the third most frequent; monocular nystagmus such as the Heimann-Bielchowsky phenomenon or myokymia of the superior oblique muscle; alternating periodic nystagmus, which cyclically changes direction, amplitude, and frequency; downbeat nystagmus, most frequent central nystagmus; acquired pendular nystagmus, which may be related to masticatory or palatal disorders; and see-saw nystagmus, with “up and down” eye movement similar to a see-saw. There are also three main types of non-nystagmic saccadic intrusions, which correspond to the opsoclonus syndrome, consisting of disordered multiplanar saccades that can be associated with myoclonus and ataxia; ocular bobbing, with vertical saccadic movements and ocular flutter, with horizontal saccadic movements. Conclusions: The characteristics of the different types of non-vestibular nystagmus and non-nystagmic saccadic intrusions and their possible etiologies should be known, since in a large percentage of cases the underlying cause is serious, frequently neurological. In the same way, we must know how to carry out the diagnosis and the therapeutic options that we have within our reach to improve the quality of life of patients.
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Dorado López-Rosado AM, Muñoz Gallego A, Tejada Palacios P. Aproximación diagnóstica en nistagmus en edad pediátrica. Acta estrabológica: publicación oficial de la Sociedad Española de Estrabología, Pleóptica, Ortóptica, Visión Binocular, Reeducación y Rehabilitación Visual. 2018;47(2):151-76. ISSN 0210-4695
Papageorgiou E, McLean RJ, Gottlob I. Nystagmus in childhood. Pediatr Neonatol 2014;55(5):341–51. DOI: https://doi.org/10.1016/j.pedneo.2014.02.007
Ehrt O. Infantile and acquired nystagmus in childhood. Eur J Paediatr Neurol. 2012;16(6):567–72. DOI: https://doi.org/10.1016/j.ejpn.2012.02.010
Zahidi AAA, Woodhouse JM, Erichsen JT, Dunn MJ. Infantile nystagmus: an optometrist’s perspective. Clin Optom (Auckl). 2017;9:123–31. DOI: https://doi.org/10.2147/OPTO.S126214
Bertsch M, Floyd M, Kehoe T, Pfeifer W, Drack AV. The clinical evaluation of infantile nystagmus: What to do first and why. Ophthalmic Genet. 2017;38(1):22–33. DOI: https://doi.org/10.1080/13816810.2016.1266667
Tariq-Bhatti M, Biousse V, Bose S, Danesh-Meyer HV, Falardeau J, Levin LA et al. Neuro-Ophthalmology. Basic and clinical science course (BCSC). San Francisco, CA: American Academy of Ophthalmology. 2020-2021, p. 233–52. ISBN: 1681044005
Galán Terraza A, González Manrique M. Nistagmus y otras oscilaciones oculares. In: Rebolleda-Fernández G, Muñoz-Negrete FJ, editores. Protocolos en Neurooftalmología. Sociedad Española de Oftalmología. 2010;263-75. ISBN: 978-84-89085-42-8
Gottlob I, Proudlock FA. Aetiology of infantile nystagmus. Curr Opin Neurol. 2014;27:83–91. DOI: https://doi.org/10.1097/WCO.0000000000000058
Suppiej A, Ceccato C, Lonardi V, Reffo ME. Infantile nystagmus without overt eye abnormality: Early features and neuro-ophthalmological diagnosis. Dev Med Child Neurol. 2022; 64(12):1532–8. DOI: https://doi.org/10.1111/dmcn.15284
Aychoua N, Schiff E, Malka S, Tailor VK, Chan HW, Oluonye N, et al. Prospective study of pediatric patients presenting with idiopathic infantile nystagmus-Management and molecular diagnostics. Front Genet. 2022;13:977806. DOI: https://doi.org/10.3389/fgene.2022.977806
Gold DR. Eye movement disorders. Liu GT, Volpe NJ, Galetta SL, editores. Neuro-Ophthalmology, Elsevier;2019, p. 585–610 DOI: https://doi.org/10.1016/B978-0-323-34044-1.00017-1
Green KE, Gold DR. Nystagmus and Superior Oblique Myokymia. Controversies in Neuro-Ophthalmic Management, Cham: Springer International Publishing; 2021, p. 157–68. DOI: https://doi.org/10.1007/978-3-030-74103-7_16
Strupp M, Hüfner K, Sandmann R, Zwergal A, Dieterich M, Jahn K, et al. Central Oculomotor Disturbances and Nystagmus. Dtsch Arztebl Int. 2011;108(12):197-204. DOI: https://doi.org/10.3238/arztebl.2011.0197
Pal R, Dutta P, Chatterjee D, Ahuja C, Singh A. Pendular see-saw nystagmus: A rare presenting manifestation of craniopharyngioma. Neuroophthalmology. 2021;45(2):126–9. DOI: https://doi.org/10.1080/01658107.2019.1693602
Bhatia P, Heim J, Cornejo P, Kane L, Santiago J, Kruer MC. Opsoclonus–myoclonus–ataxia syndrome in children. J Neurol. 2022;269(2):750–7. DOI: https://doi.org/10.1007/s00415-021-10536-3
Rossor T, Yeh EA, Khakoo Y, Angelini P, Hemingway C, Irani SR, et al. Diagnosis and management of opsoclonus-myoclonus-ataxia syndrome in children: An international perspective. Neurol Neuroimmunol Neuroinflamm. 2022;9(3):e1153. DOI: https://doi.org/10.1212/nxi.0000000000001153
Manto M, Triarhou LC. Ocular dysmetria, flutter, and opsoclonus: Refining the oculomotor deficits in cerebellar patients. Cerebellum. 2022;22(4):506–26. DOI: https://doi.org/10.1007/s12311-022-01444-x
Papageorgiou E, McLean RJ, Gottlob I. Nystagmus in childhood. Pediatr Neonatol 2014;55(5):341–51. DOI: https://doi.org/10.1016/j.pedneo.2014.02.007
Ehrt O. Infantile and acquired nystagmus in childhood. Eur J Paediatr Neurol. 2012;16(6):567–72. DOI: https://doi.org/10.1016/j.ejpn.2012.02.010
Zahidi AAA, Woodhouse JM, Erichsen JT, Dunn MJ. Infantile nystagmus: an optometrist’s perspective. Clin Optom (Auckl). 2017;9:123–31. DOI: https://doi.org/10.2147/OPTO.S126214
Bertsch M, Floyd M, Kehoe T, Pfeifer W, Drack AV. The clinical evaluation of infantile nystagmus: What to do first and why. Ophthalmic Genet. 2017;38(1):22–33. DOI: https://doi.org/10.1080/13816810.2016.1266667
Tariq-Bhatti M, Biousse V, Bose S, Danesh-Meyer HV, Falardeau J, Levin LA et al. Neuro-Ophthalmology. Basic and clinical science course (BCSC). San Francisco, CA: American Academy of Ophthalmology. 2020-2021, p. 233–52. ISBN: 1681044005
Galán Terraza A, González Manrique M. Nistagmus y otras oscilaciones oculares. In: Rebolleda-Fernández G, Muñoz-Negrete FJ, editores. Protocolos en Neurooftalmología. Sociedad Española de Oftalmología. 2010;263-75. ISBN: 978-84-89085-42-8
Gottlob I, Proudlock FA. Aetiology of infantile nystagmus. Curr Opin Neurol. 2014;27:83–91. DOI: https://doi.org/10.1097/WCO.0000000000000058
Suppiej A, Ceccato C, Lonardi V, Reffo ME. Infantile nystagmus without overt eye abnormality: Early features and neuro-ophthalmological diagnosis. Dev Med Child Neurol. 2022; 64(12):1532–8. DOI: https://doi.org/10.1111/dmcn.15284
Aychoua N, Schiff E, Malka S, Tailor VK, Chan HW, Oluonye N, et al. Prospective study of pediatric patients presenting with idiopathic infantile nystagmus-Management and molecular diagnostics. Front Genet. 2022;13:977806. DOI: https://doi.org/10.3389/fgene.2022.977806
Gold DR. Eye movement disorders. Liu GT, Volpe NJ, Galetta SL, editores. Neuro-Ophthalmology, Elsevier;2019, p. 585–610 DOI: https://doi.org/10.1016/B978-0-323-34044-1.00017-1
Green KE, Gold DR. Nystagmus and Superior Oblique Myokymia. Controversies in Neuro-Ophthalmic Management, Cham: Springer International Publishing; 2021, p. 157–68. DOI: https://doi.org/10.1007/978-3-030-74103-7_16
Strupp M, Hüfner K, Sandmann R, Zwergal A, Dieterich M, Jahn K, et al. Central Oculomotor Disturbances and Nystagmus. Dtsch Arztebl Int. 2011;108(12):197-204. DOI: https://doi.org/10.3238/arztebl.2011.0197
Pal R, Dutta P, Chatterjee D, Ahuja C, Singh A. Pendular see-saw nystagmus: A rare presenting manifestation of craniopharyngioma. Neuroophthalmology. 2021;45(2):126–9. DOI: https://doi.org/10.1080/01658107.2019.1693602
Bhatia P, Heim J, Cornejo P, Kane L, Santiago J, Kruer MC. Opsoclonus–myoclonus–ataxia syndrome in children. J Neurol. 2022;269(2):750–7. DOI: https://doi.org/10.1007/s00415-021-10536-3
Rossor T, Yeh EA, Khakoo Y, Angelini P, Hemingway C, Irani SR, et al. Diagnosis and management of opsoclonus-myoclonus-ataxia syndrome in children: An international perspective. Neurol Neuroimmunol Neuroinflamm. 2022;9(3):e1153. DOI: https://doi.org/10.1212/nxi.0000000000001153
Manto M, Triarhou LC. Ocular dysmetria, flutter, and opsoclonus: Refining the oculomotor deficits in cerebellar patients. Cerebellum. 2022;22(4):506–26. DOI: https://doi.org/10.1007/s12311-022-01444-x
Duque Holguera, V., Ramírez Salas, J. E., Álvarez Álvarez, M., Losada Campa, J., González Sosto, M., San Millán González, M., & Saboya Romero, D. M. (2023). Non-vestibular nystagmus and saccadic intrusions. Revista ORL, e31541. https://doi.org/10.14201/orl.31541
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