Mendonca J L F, Natal M R C, Viana S L, Coimbra P P A, Viana M A C B, Matsumine M
Hospital Santa Lucia, Brasilia, DF, Brazil.
Neurol India. 2004 Mar;52(1):118-20.
An unusual disorder of cerebellar development, rhombencephalosynapsis is a unique entity which presents with cerebellar fusion and absence of cerebellar vermis on imaging studies, often associated with supratentorial findings. No specific clinical syndrome has been described in these patients so far, and most cases are found in infancy and childhood. MRI and its multiplanar capabilities and high spatial and contrast resolution increased its recognition. Two cases are reported, with emphasis on imaging findings.
菱脑融合是一种不寻常的小脑发育障碍，是一种独特的病症，影像学检查显示小脑融合且小脑蚓部缺如，常伴有幕上病变。目前这些患者尚未描述有特定的临床综合征，大多数病例在婴儿期和儿童期被发现。MRI及其多平面成像能力以及高空间分辨率和对比度分辨率提高了对该病的认识。本文报告两例病例，并重点阐述影像学表现。
