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Visual and brain stem auditory evoked responses in Wilson's disease
1Department of Neurology, National Institute of Mental Health & Neuro Sciences (NIMHANS), Bangalore, India.
Acta Neurologica Scandinavica
|February 1, 1989
Summary
Sensory evoked potentials reveal subclinical visual and auditory pathway damage in Wilson's disease patients. These findings highlight potential for early detection and monitoring of neurological involvement in this genetic disorder.
Area of Science:
- Neurology
- Neurophysiology
Background:
- Wilson's disease is a genetic disorder causing copper accumulation.
- Neurological and psychiatric symptoms are common, but subclinical involvement is poorly understood.
Purpose of the Study:
- To investigate subclinical sensory pathway disturbances in Wilson's disease using evoked potentials.
- To assess the utility of visual evoked potentials (VEP) and brainstem auditory evoked responses (BAER) in Wilson's disease.
Main Methods:
- Evaluated 15 Wilson's disease patients using pattern reversal visual stimulation and brainstem auditory stimulation.
- Measured P100 latency for VEP and interpeak latencies (I-V, III-V) for BAER.
Main Results:
- Prolonged P100 latency (VEP) observed in 7/13 patients, indicating optic pathway dysfunction.
- Prolonged I-V and III-V interpeak latencies (BAER) found in 8/12 patients, suggesting brainstem auditory pathway involvement.
- Evoked potential abnormalities were present even in the absence of overt symptoms.
Conclusions:
- Evoked potential studies effectively detect subclinical optic and brainstem auditory pathway disturbances in Wilson's disease.
- These electrophysiological techniques show promise for monitoring disease progression and therapeutic response.