Carter J E, Merren M D, Swann K W
Division of Neurology, University of Texas Health Science Center, San Antonio.
J Neurosurg. 1989 Jan;70(1):135-7. doi: 10.3171/jns.1989.70.1.0135.
Lhermitte-Duclos disease is a benign, presumably hamartomatous lesion of the cerebellum which presents clinically as a mass lesion. Pathologically, it consists of thickening of both the molecular and granular cell layers of the cerebral cortex which enlarges the folia but allows for preservation of the gyral pattern of the cerebellar cortex. Preoperative diagnosis with computerized tomography and other studies has not been possible, and even at surgery the diagnosis may be missed because of the preservation of the gyral pattern. The sensitivity of magnetic resonance imaging allows recognition of the cortical nature of the mass lesion, and especially the gyral pattern within the mass lesion, providing a diagnostic image which is unlikely to be confused with any other pathological process in the cerebellum. Preoperative diagnosis of Lhermitte-Duclos disease allows surgeons to plan an appropriate decompressive procedure.
Lhermitte-Duclos病是一种小脑的良性、推测为错构瘤性病变,临床上表现为肿块病变。病理上,它由大脑皮质分子层和颗粒层增厚组成,这使小脑叶片增大,但小脑皮质的脑回形态得以保留。术前通过计算机断层扫描和其他检查无法做出诊断,甚至在手术时也可能因脑回形态得以保留而漏诊。磁共振成像的敏感性使人们能够识别肿块病变的皮质性质,尤其是肿块病变内的脑回形态,提供一种不太可能与小脑中任何其他病理过程相混淆的诊断图像。Lhermitte-Duclos病的术前诊断使外科医生能够规划适当的减压手术。