Related Experiment Videos
Cortical visual impairment in children with infantile spasms
Insights
Infantile spasms, also known as West syndrome, frequently cause severe visual impairment. Most affected children show little to no improvement in vision, highlighting the need for early recognition.
Area of Science:
- Pediatric Neurology
- Ophthalmology
- Developmental Neuroscience
Background:
- Infantile spasms (West syndrome) are a severe epilepsy syndrome in infants.
- Cortical visual impairment (CVI) is a known complication of infantile spasms.
- This study investigates CVI in West syndrome patients with normal ocular exams.
Observation:
- Ten infants with West syndrome, hypsarrhythmia, and developmental delay were assessed.
- All presented with severe visual inattention despite normal eye examinations.
- Visual behavior was monitored over 14 months to 6 years.
Findings:
- 50% of patients showed no visual behavior improvement.
- The remaining patients had persistent abnormal visual function.
- All participants had moderate to severe intellectual disability.
Implications:
- Cortical visual impairment is a significant, often persistent, issue in West syndrome.
- Pediatric ophthalmologists must be aware of this association.
- Early recognition and management of CVI in infantile spasms are crucial.
Background:
Infantile spasms are often associated with cortical visual impairment.
Method:
We report a series of 10 patients with infantile spasms, hypsarrhythmic electroencephalograms, and developmental delay, (ie, West syndrome), who presented with severe visual inattention despite a normal ocular examination.
Results:
At follow-up (14 months to 6 years), 5 patients (50%) had no improvement in their visual behaviour. Although some degree of improvement was observed in the others, their visual function remained abnormal. All patients had moderate or severe mental retardation.
Conclusions:
The clinical features of West syndrome are reviewed, and the cause of the associated cortical visual impairment is discussed. Cortical visual impairment with infantile spasms is an important association, which pediatric ophthalmologists should recognize.