• Clinical and Experimental Pediatrics
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• 제49권2호
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• pp.208-211
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• 2006

Dyke-Davidoff-Masson 증후군은 일측성 대뇌 반구의 위축과 동측의 대상성 골비대, 반대측의 편마비 등을 특징으로 하는 드문 신경질환이다. 본 증례는 17개월 남아가 좌측 국소적 경련외에 좌측 편마비 및 기타 발달지연을 동반한 경우이다. 당시 시행한 뇌 자기공명영상에서 우측 대뇌 반구의 진행성 위축과 뇌실 확장, 두꺼워진 두개관과 동측 전두부 부비동의 확장, 추체 접형골의 거상을 보였고 뇌 SPECT 촬영상에 우측 대뇌 반구의 위축 및 혈류감소를 보였다. 이 같은 소견들은 Dyke-Davidoff-Masson 증후군에 합당하여 이에 저자들은 본 증례를 문헌 고찰과 함께 보고한다.

• 대한두개안면성형외과학회지
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• 제13권1호
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• pp.50-53
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• 2012

Purpose: The Dyke-Davidoff-Masson syndrome is a rare disease entity that was first reported in 1993, and it is characterized by not only the cerebral hemiatrophy that is accompanied by the ipsilateral ventriculomegaly and ipsilateral compensatory osseous hypertrophy, but also the overgrowth of the paranasal sinuses. No studies have attempted to examine it from perspectives of the skull deformity and plastic surgery. Here, we report our case with a review of the literatures. Methods: A 45-year-old man with Dyke-Davidoff-Masson visited our medical institution with nasal bone fracture. Based on the previously taken brain MRI scans, we measured the degree of craniofacial deformity, and the horizontal distance, which is based on the margin of the skull, as well as the falx cerebri. Results: We made a comparison of the degree of craniofacial deformity. This showed that the mean horizontal distance on the axial view was shorter by approximately 28.46%, as compared with that of the left unaffected side. Conclusion: The Dyke-Davidoff-Masson is characterized by a concurrent presence of the atrophy of the cerebral hemisphere, with the cranial deformity. For the reconstruction of the bone and soft-tissue deformity with Dyke-Davidoff-Masson syndrome, it is needed to perform objective assessments.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제44권4호
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• pp.198-203
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• 2018

Dyke-Davidoff-Masson syndrome is a non-inherited rare condition that presents during childhood and is characterized by seizures, hemiplegia, mental retardation, cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. The present article highlights a case of a 12-year-old male child with additional clinical findings of $caf{\acute{e}}$-au-late pigmentation and ocular lipodermoid. This is the first case report of DykeDavidoff-Masson syndrome to describe oral manifestations, such as unilateral delayed eruption of teeth, hypoplasia, and taurodontism, which could be unique and characteristic of this condition. Oral health care providers and physicians should be aware of these oral observations as dental referrals could warrant early dental prophylactic care and can be useful in diagnosing the possible time of injury and type of Dyke-Davidoff-Masson syndrome.