Dental treatment in a patient with Charcot-Marie-Tooth disease and Guillain-Barre syndrome: A case report
조상미, 이고은, 남옥형, 이효설, 최성철, 김광철, 김미선,
소속 상세정보
조상미 ( Jo Sang-Mi ) - Kyung Hee University Dental Hospital at Gangdong Department of Pediatric Dentistry
이고은 ( Lee Ko-Eun ) - Kyung Hee University Dental Hospital Department of Pediatric Dentistry
남옥형 ( Nam Ok-Hyung ) - Kyung Hee University Dental Hospital Department of Pediatric Dentistry
이효설 ( Lee Hyo-Seol ) - Kyung Hee University Dental Hospital Department of Pediatric Dentistry
최성철 ( Choi Sung-Chul ) - Kyung Hee University Dental Hospital Department of Pediatric Dentistry
김광철 ( Kim Kwang-Chul ) - Kyung Hee University Dental Hospital at Gangdong Department of Pediatric Dentistry
김미선 ( Kim Mi-Sun ) - Kyung Hee University Dental Hospital at Gangdong Department of Pediatric Dentistry
Abstract
Charcot-Marie-Tooth (CMT) disease is the most frequent hereditary peripheral neuropathy and the prevalence is estimated between 1:1,213 and 1:2,500. Most of the CMT patients have genes that are inherited in an autosomal dominant manner. A mutation in the peripheral myelin protein 22, mitofusin-2, myelin protein zero, or gap junction protein beta 1 gene occurs in over 90% of patients with CMT. These patients show general weakness in various parts of the body and distal sensory loss. Guillain-Barre syndrome (GBS) is the most common severe acute paralytic neuropathy, and the prevalence is estimated at 1:100,000 in children under 15 years of age. It usually occurs post infections or other immune stimulation that can cause autoimmune response. GBS patients show severe weakness, incomplete recovery, and pain. This case report discusses the treatment of a 13-yearold child with CMT disease and GBS without the use of general anesthesia and conscious sedation. In the dental area, she had problems with trauma risk due to weakness of muscles, dry mouth due to mouth breathing, anterior open bite, low tongue position with tongue thrusting, space deficiency, halitosis, and thick gingiva. For these problems, a regular check-up with fluoride application, caries treatments, and extractions of deciduous teeth with local anesthesia due to thick gingiva w ere done.
키워드
Charcot-Marie-Tooth disease; Guillain-Barre syndrome; Outpatient dental treatment
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