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KMID : 1144020200160020059
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Journal of Korean Association for Disability and Oral Health
2020 Volume.16 No. 2 p.59 ~ p.63
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Dental treatment in a patient with Charcot-Marie-Tooth disease and Guillain-Barre syndrome: A case report
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Jo Sang-Mi
Lee Ko-Eun
Nam Ok-Hyung
Lee Hyo-Seol
Choi Sung-Chul
Kim Kwang-Chul
Kim Mi-Sun
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Abstract
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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.
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KEYWORD
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Charcot-Marie-Tooth disease, Guillain-Barre syndrome, Outpatient dental treatment
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