A case report of Rett syndrome with remarkable trismus

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  • HOSOHARA Masatoshi
    Department of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Diseases Control, Asahi University School of Dentistry
  • EHARA Yuichi
    Department of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Diseases Control, Asahi University School of Dentistry
  • TANAKA Shiro
    Department of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Diseases Control, Asahi University School of Dentistry
  • GEN Keika
    Department of Disability and Oral Diseases Control, Asahi University School of Dentistry
  • SUMITOMO Shinichiro
    Department of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Diseases Control, Asahi University School of Dentistry
  • SHIKIMORI Michio
    Department of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Diseases Control, Asahi University School of Dentistry

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Other Title
  • 著しい開口障害を呈したRett syndromeの1例
  • イチジルシイ カイコウ ショウガイ オ テイシタ Rett syndrome ノ 1レイ

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Abstract

Rett syndrome is found exclusively in young females, and is a progressive neurologic disease characterized by mental retardation, repeated hand-rubbing behavior, and related symptoms. We report a case of trismus that was treated in a patient with Rett syndrome. <br>A 14-year-old girl (height, 120 cm; weight 20 kg) presented at Asahi University Hospital in December 2006 because of trismus. Her medical history included epilepsy, patent ductus arteriosus, microcephaly, hypertension, and suspected Rett syndrome since 6 years of age. Trismus had been present for several years. Examination revealed mental retardation, scoliosis, manual stereotyped behavior, developmental disease of the feet, and difficulty in independently walking. Her face was symmetric, with a developed mandible angle. Flaredout maxillary front teeth with attrition and jaw opening of 15 mm were confirmed. Radiologic examination showed hypertrophy of both coronoid processes and slight adhesion in the left temporomandibular joint. Hypertrophy of both coronoid processes was diagnosed clinically, and both were removed. The intraoperative jaw opening was 45 mm. The jaw opening has been maintained at 32 mm as of 5 years 9 months after surgery. <br>The case was considered to involve trimus caused by coronoid process enlargement due to development of the masseter and temporal muscles secondary to bruxism.

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