J Korean Neurol Assoc.
1995 Dec;13(4):970-978.
Neurophysiological Studies in a Patient with Hyperekplexia
- Affiliations
-
- 1Department of Neurology, Severance Hoispital Yonsei University, College of Medicine, Korea.
Abstract
- The results of neurophysiological studies in a 20 year-old man who had pathological jumps and falls following unexpected auditory stimuli is presented. He also had action induced hemidystonia and polymyoclonus involving fingers of the both hands. Somatosensory evoked potential study showed no giant cortical wave and jerk-locked back averaging electroencephalogram showed no jerk ,related cortical event. Polymyographic study after auditory stimuli showed electromyographic (EMG) bursts recruitment pattern consistent with hyprekplexia ai)d reticular reflex myocloniis. EMG bursts spread rostrally and caudally from the sternocleidomastoid muscle via a slowly conducting neuronal pathways. These findings are compatible. With hyperekplexia. Pathologically increased gain of the brainstem reticular formation seems to cause hyperekplexia.
- Full Text Links
-
- Actions
-
Cited
- CITED
-
- Close
- Share
-
- Similar articles
-
- A Case Report of Congenital Hyperekplexia in Twin
- Familiar Hyperekplexia, a Potential Cause of Cautious Gait: A New Korean Case and a Systematic Review of Phenotypes
- Transcranial magnetic stimulation parameters as neurophysiological biomarkers in Alzheimer’s disease
- Clinical practice guidelines for intraoperative neurophysiological monitoring: 2020 update
- Hyperekplexia with congenital heart disease: anesthetic concerns and management
