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Diagnosing children presenting with asymmetric pendular nystagmus
F S Shaw1, A Kriss, I Russel-Eggitt
1Department of Ophthalmology, Great Ormond Street Hospital for Children, London, UK. fatima@gordonreid.net
Developmental Medicine and Child Neurology
|September 26, 2001
Summary
Horizontal asymmetric nystagmus in children can indicate serious intracranial issues or retinal conditions. Early VEP/ERG testing is crucial for accurate diagnosis, differentiating between neurological and visual causes.
Area of Science:
- Ophthalmology
- Neurology
- Pediatrics
Background:
- Horizontal asymmetric nystagmus is uncommon in congenital nystagmus.
- It can be associated with intracranial lesions, monocular vision loss, or spasmus nutans.
- Spasmus nutans is diagnosed retrospectively after ruling out other pathologies.
Purpose of the Study:
- To investigate the causes of horizontal asymmetric nystagmus in children.
- To evaluate the diagnostic utility of neuroimaging and electrophysiological tests.
- To highlight the differential diagnosis of asymmetric nystagmus in pediatric patients.
Main Methods:
- Retrospective review of clinical records of 277 children with congenital nystagmus over 8 years.
- Analysis of cases with asymmetric nystagmus (24/277).
- Correlation of clinical findings with neuroimaging and visual evoked potentials (VEPs) / electroretinography (ERG).
Main Results:
- Asymmetric nystagmus was found in 24 children.
- Seven cases were diagnosed with spasmus nutans.
- Twelve cases had intracranial pathology with abnormal VEPs.
- Five cases had congenital sensory defect nystagmus (albinism, cone dysfunction, cone-rod dystrophy).
Conclusions:
- Neuroimaging is essential for asymmetric nystagmus, but retinal diseases and albinism are also important considerations.
- Non-invasive VEP/ERG testing is critical for diagnosing all forms of nystagmus.
- Asymmetric nystagmus warrants a thorough ophthalmological and neurological evaluation.