Barkovich A J, Frieden I J, Williams M L
Department of Neuroradiology, University of California, San Francisco 94143-0628.
AJNR Am J Neuroradiol. 1994 May;15(5):859-67.
To describe the MR findings of neurocutaneous melanosis in the brain and correlate these with the known pathology and proposed embryologic basis of this disorder.
The brain (seven patients) and spine (three patients) MR scans of seven patients with neurocutaneous melanosis were retrospectively reviewed. In two patients, findings were confirmed at surgery. The pattern of central nervous system involvement was also correlated with known pathologic studies regarding frequency and location of melanotic deposits.
Five patients had regions of T1 shortening in the cerebellum; three of these also had T2 shortening. Five patients had regions of T1 shortening in the anterior temporal lobes. Other areas of involvement included the pia mater over the cerebellum (two patients), pons (one patient), medulla (one patient), and left parietal lobe (one patient). Only two lesions showed enhancement, edema, or necrosis; both were proved malignant melanomas at biopsy. No pial enhancement was detected.
Neurocutaneous melanosis appears to involve the brain in specific locations that can be detected on MR imaging. Knowledge of these locations can aid in differentiating metastases, secondary to malignant degeneration of the large cutaneous nevi, from melanotic deposits that are a part of the disease. Identification of malignant degeneration of the melanotic deposits is difficult; at present, it depends on the identification of growth, edema, or necrosis of the deposits.
描述神经皮肤黑素沉着症的脑部磁共振成像(MR)表现,并将这些表现与该疾病已知的病理学及推测的胚胎学基础相关联。
回顾性分析7例神经皮肤黑素沉着症患者的脑部(7例)和脊柱(3例)MR扫描图像。其中2例患者的检查结果经手术证实。中枢神经系统受累模式也与关于黑素沉着沉积物的频率和位置的已知病理学研究相关联。
5例患者小脑出现T1加权像信号缩短区域;其中3例同时伴有T2加权像信号缩短。5例患者颞叶前部出现T1加权像信号缩短区域。其他受累部位包括小脑表面软脑膜(2例)、脑桥(1例)、延髓(1例)和左侧顶叶(1例)。仅2个病灶表现为强化、水肿或坏死;活检均证实为恶性黑色素瘤。未检测到软脑膜强化。
神经皮肤黑素沉着症似乎在脑部特定部位受累,这些部位可通过MR成像检测到。了解这些部位有助于鉴别源于大型皮肤痣恶性变的转移瘤与作为该疾病一部分的黑素沉着沉积物。黑素沉着沉积物的恶性变很难识别;目前,这取决于对沉积物生长、水肿或坏死的识别。