Korean J Ophthalmol.  2017 Jun;31(3):263-267. 10.3341/kjo.2015.0161.

Ophthalmologic Features of Lennox-Gastaut Syndrome

• Kim BH
• Yu YS
• Kim SJ

• KMID: 2379883
• DOI: http://doi.org/10.3341/kjo.2015.0161

Abstract

PURPOSE
To describe the characteristics and frequency of ophthalmologic findings in patients with Lennox-Gastaut syndrome (LGS).
METHODS
The medical records of patients diagnosed with LGS at Seoul National University Children's Hospital from January 2004 to August 2014 were retrospectively reviewed. The records of 34 patients (mean age ± standard deviation, 2.66 ± 3.51 years; male, 58.8%) were reviewed. The primary measure was the incidence of ophthalmologic manifestations.
RESULTS
Of the 34 patients, 88.2% had at least one ocular abnormality. Refractive error (52.9%) was the most frequently observed ophthalmologic manifestation in patients with LGS, followed by strabismus (32.4%), cortical visual impairment (23.5%), and retinopathy of prematurity (8.8%). Among these cases, seven patients had exotropia and three had esotropia.
CONCLUSIONS
LGS is a childhood-onset epileptic encephalopathy with variable ophthalmologic manifestations, the most frequent being refractive errors. Patients with suspected LGS should be examined regularly because ophthalmological features can change during their disease course.

Keyword

Epilepsy; Eye manifestations; Lennox-Gastaut syndrome

MeSH Terms

Brain Diseases
Epilepsy
Esotropia
Exotropia
Eye Manifestations
Humans
Incidence
Male
Medical Records
Refractive Errors
Retinopathy of Prematurity
Retrospective Studies
Seoul
Strabismus
Vision Disorders

Reference

1. Markand ON. Lennox-Gastaut syndrome (childhood epileptic encephalopathy). J Clin Neurophysiol. 2003; 20:426–441.

2. Crumrine PK. Lennox-Gastaut syndrome. J Child Neurol. 2002; 17:Suppl 1. S70–S75.

3. Arzimanoglou A, French J, Blume WT, et al. Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol. 2009; 8:82–93.

4. Wen G, Tarczy-Hornoch K, McKean-Cowdin R, et al. Prevalence of myopia, hyperopia, and astigmatism in non-Hispanic white and Asian children: multi-ethnic pediatric eye disease study. Ophthalmology. 2013; 120:2109–2116.

5. McKean-Cowdin R, Cotter SA, Tarczy-Hornoch K, et al. Prevalence of amblyopia or strabismus in asian and non-Hispanic white preschool children: multi-ethnic pediatric eye disease study. Ophthalmology. 2013; 120:2117–2124.

6. Torp-Pedersen T, Boyd HA, Poulsen G, et al. Perinatal risk factors for strabismus. Int J Epidemiol. 2010; 39:1229–1239.

7. Williams C, Northstone K, Howard M, et al. Prevalence and risk factors for common vision problems in children: data from the ALSPAC study. Br J Ophthalmol. 2008; 92:959–964.

8. Matsumoto H, Zaha K, Nakamura Y, et al. Chromosome 9q33q34 microdeletion with early infantile epileptic encephalopathy, severe dystonia, abnormal eye movements,and nephroureteral malformations. Pediatr Neurol. 2014; 51:170–175.

9. Thierry G, Beneteau C, Pichon O, et al. Molecular characterization of 1q44 microdeletion in 11 patients reveals three candidate genes for intellectual disability and seizures. Am J Med Genet A. 2012; 158:1633–1640.