Tapping-lips aggravated interictal bilateral discharges in EEG in the patients with Rett syndrome: a case report.

Abstract:

BACKGROUND:Rett syndrome (RTT) is a severe neurodevelopmental disorder mainly affecting females. One of the main clinical manifestations is hand stereotypies, which is presumed to based on dysfunction rather than on structural impairment. Reflex interictal discharge precipitated by tapping-lips in electroencephalogram (EEG) is a rare phenomenon among RTT. CASE PRESENTATION:We firstly reported a case of RTT concerning interictal reflex discharge precipitated by tapping- lips. The child, female, 5 years old, presented with a significant regression in motor development and language skills. She almost always tapped the lips with the right hand and stopped only when was interrupted. Her EEG results displayed extensive low amplitude fast wave could be elicited by lightly and slowly- rhythm tapping lips and multifocal bilateral discharges could be precipitated by relatively stronger and quicker rhythm action. It was when the movement stopped that corresponding discharges immediately disappeared. Besides, the reflex discharges were not precipitated by tapping- lips using observer's hand at the certain tempo and intensity. The hand stereotypies did not respond to antiepileptic drugs. CONCLUSIONS:Tapping- lips may be a somatosensory stimulation to precipitate interictal discharges in RTT, which may provide another idea to enrich the insight on hand stereotypies of RTT.

journal_name

BMC Neurol

journal_title

BMC neurology

authors

Luo X,Li C,Wang X,Yu X,Jiang J

doi

10.1186/s12883-019-1296-4

subject

Has Abstract

pub_date

2019-05-01 00:00:00

pages

77

issue

1

issn

1471-2377

pii

10.1186/s12883-019-1296-4

journal_volume

19

pub_type

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