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The visually evoked potential in Huntington disease
Neurology
|January 1, 1978
Summary
Visually evoked potentials (VEPs) are abnormal in most Huntington disease patients, showing reduced amplitude. This visual pathway abnormality is specific to Huntington disease and not seen in other neurological conditions.
Area of Science:
- Neuroscience
- Ophthalmology
- Genetics
Background:
- Huntington disease (HD) is a progressive neurodegenerative disorder.
- Visual pathway dysfunction may occur in neurodegenerative diseases.
Purpose of the Study:
- To investigate visual evoked potentials (VEPs) in patients with Huntington disease.
- To determine if VEP abnormalities are specific to Huntington disease.
Main Methods:
- VEPs were recorded in 18 patients with Huntington disease and 50 healthy controls.
- VEP components, amplitude, and latency were analyzed.
- VEPs were also assessed in patients with other cerebral disorders and in children of HD patients.
Main Results:
- 17 out of 18 Huntington disease patients exhibited abnormal VEPs.
- Both early and late VEP wave components were affected, with reduced averaged amplitude.
- VEP abnormalities were not observed in other neurological conditions or in unaffected children of HD patients.
Conclusions:
- Visually evoked potentials are significantly abnormal in Huntington disease patients.
- The VEP abnormalities, characterized by reduced amplitude and disorganization, appear specific to Huntington disease.
- VEP testing may serve as a potential biomarker for Huntington disease.