The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
권호 표지

A case of dyke-davidoff-masson syndrome associated with hypopituitarism and diabetes mellitus

Dyke-Davidoff-Masson 증후군 환자에서 발생한 뇌하수체 기능저하증과 당뇨병 1예

  • Park, So-Yeon (Department of Internal Medicine, Yonsei University Wonju College of Medicine) ;
  • Lee, Mi-Young (Department of Internal Medicine, Yonsei University Wonju College of Medicine) ;
  • Kim, Jae-Hyun (Department of Internal Medicine, Yonsei University Wonju College of Medicine) ;
  • Kim, Song-Yi (Department of Internal Medicine, Yonsei University Wonju College of Medicine) ;
  • Shin, Jang-Yel (Department of Internal Medicine, Yonsei University Wonju College of Medicine) ;
  • Shin, Young-Goo (Department of Internal Medicine, Yonsei University Wonju College of Medicine) ;
  • Chung, Choon-Hee (Department of Internal Medicine, Yonsei University Wonju College of Medicine)
  • 박소연 (연세대학교 원주의과대학 내과학교실) ;
  • 이미영 (연세대학교 원주의과대학 내과학교실) ;
  • 김재현 (연세대학교 원주의과대학 내과학교실) ;
  • 김송이 (연세대학교 원주의과대학 내과학교실) ;
  • 신장열 (연세대학교 원주의과대학 내과학교실) ;
  • 신영구 (연세대학교 원주의과대학 내과학교실) ;
  • 정춘희 (연세대학교 원주의과대학 내과학교실)
  • Received : 2009.07.12
  • Accepted : 2009.09.23
  • Published : 2010.09.01
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Abstract

Dyke-Davidoff-Masson syndrome (DDMS) is a rare condition characterized by asymmetric cerebral hemispheric growth with unilateral atrophy, ipsilateral compensatory osseous hypertrophy, hyperpneumatization of the paranasal sinuses and mastoid cells, and contralateral paresis. Varying degrees of hemiparesis, hemiplegia, seizures, mental retardation, and facial asymmetry can be associated with DDMS. We report the case of a 26-year-old man with DDMS associated with hypopituitarism who complained of polydipsia and polyuria. After an oral glucose tolerance test, he was diagnosed with type 2 diabetes. There is no report of DDMS associated with other pituitary dysfunction or hyperglycemia. Clinicians should consider the possibility of coexisting pituitary dysfunction or type 2 diabetes in patients with DDMS, as it is obviously important for the patient's outcome.

저자들은 생후 일찍 경련이 발생하여 부전마비 임상적 소견, 방사선학적 검사 및 유전학적 검사를 통해 Dyke-Davidoff-Masson 증후군이 진단된 환자에서 뇌하수체 기능저하증 및 당뇨병이 동반된 사례를 경험하였기에 보고하는 바이다.

Keywords

References

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