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[Non-hereditary +hyperekplexia. Study of a patient with unusual ictal EEG features and review of the literature].

Authors :
Rodríguez-Barrionuevo AC
Núñez-Castain MJ
Bauzano-Poley E
Rodríguez-Vives MA
Source :
Revista de neurologia [Rev Neurol] 1998 Dec; Vol. 27 (160), pp. 1015-8.
Publication Year :
1998

Abstract

Introduction and Clinical Case: We present a male patient which presented distal muscular stiffness, from the first hours of the life. At this time, he also presented episodes of generalized muscular hypertonia with cyanosis and apnoea, started by somato-sensorial stimuli. Those episodes were terminated by passive flexion of the extremities and the head. Later, dream myoclonic jolts appeared. EEG tracings during hypertonic episodes showed an initial artefact potential followed by bursts of rhythmic and repetitive acute potentials in the bilateral fronto-central regions, at a 20-22 Hz frequency, similar to an epileptiform spike-wave burst. The EMG showed a continuous muscular activity, suppressed by rest and the administration of diazepam. Treatment with oral diazepam has been very effective. Now, the patient is four years old, is asymptomatic and continues treatment with oral diazepam. If the dose is decreased, the child starts unstable march, startle response on somatosensory stimuli with falls, and above all nocturnal myoclonia. The patient does not have familiar antecedents of hyperekplexia, even in its minor form.<br />Conclusion: Clinical picture suggests us a not familiar form of hyperekplexia.

Details

Language :
Spanish; Castilian
ISSN :
0210-0010
Volume :
27
Issue :
160
Database :
MEDLINE
Journal :
Revista de neurologia
Publication Type :
Academic Journal
Accession number :
9951028