Neurophysiological Studies in a Patient with Hyperekplexia.
- Author:
Myung Sik LEE
1
;
Sang Bock LEE
;
Yong Duk KIM
;
Won Chan KIM
;
Young Cheol CHOI
Author Information
1. Department of Neurology, Severance Hoispital Yonsei University, College of Medicine, Korea.
- Publication Type:Case Report
- MeSH:
Brain Stem;
Electroencephalography;
Evoked Potentials, Somatosensory;
Fingers;
Hand;
Humans;
Myoclonus;
Neurons;
Reflex;
Reticular Formation;
Stiff-Person Syndrome*;
Young Adult
- From:Journal of the Korean Neurological Association
1995;13(4):970-978
- CountryRepublic of Korea
- Language:Korean
-
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.
