Rainov N G, Holzhausen H J, Burkert W
Department of Neurosurgery, Faculty of Medicine, Martin-Luther-University, Halle (Saale), Germany.
Clin Neurol Neurosurg. 1995 May;97(2):175-80. doi: 10.1016/0303-8467(95)00017-e.
The case of a 30-year-old male patient is presented who was admitted with a 3-month history of elevated intracranial pressure and reduced-hearing on the right. CT revealed hydrocephalus due to displacement of the 4th ventricle by a large non-enhancing cerebellar mass with calcifications. MRI sequences showed a space-occupying lesion within the right cerebellar hemisphere with unusual septations. At operation, the poorly demarcated lesion was excised from the apparently normal surrounding cerebellar tissue and histologically proven to be a dysplastic gangliocytoma (Lhermitte-Duclos disease). Clinical and neuroradiological appearance of this rare entity, as well as hypotheses on its pathogenesis are discussed.
本文介绍了一名30岁男性患者的病例，该患者因颅内压升高和右耳听力下降3个月入院。CT显示第四脑室被一个大的无强化的小脑钙化肿块推移导致脑积水。MRI序列显示右小脑半球有一个占位性病变，伴有异常分隔。手术中，从看似正常的周围小脑组织中切除了边界不清的病变，组织学证实为发育异常性神经节细胞瘤（Lhermitte-Duclos病）。讨论了这种罕见疾病的临床和神经放射学表现及其发病机制的假说。
