Costenaro Fabiola, Rodrigues Ticiana C, Ferreira Nelson P, Costa Tiago G da, Schuch Tiago, Boschi Vitor, Czepielewski Mauro A
Endocrine Division, Hospital de Clínicas de Porto Alegre, RS, Brazil.
Arq Bras Endocrinol Metabol. 2011 Jun;55(4):284-7. doi: 10.1590/s0004-27302011000400008.
Benign intracranial hypertension (Pseudotumor cerebri) has been described as related to the reduction in steroid levels in Cushing's disease (CD), especially after surgical remission. Ketoconazole is a common and effective adjuvant therapy for hypercortisolism, but the major concern is liver enzyme dysfunction. We describe here the case of a 12-year old girl with CD who developed benign intracranial hypertension during treatment with ketoconazole. She presented headache, vomiting, a black spot on her right temporal visual field, and signs of elevated intracranial pressure. Pituitary image was normal on magnetic resonance image (MRI), and all symptoms improved after treatment with acetazolamide. We call attention to the diagnosis of this disorder in CD patients, especially children on ketoconazole treatment, because it could be confounded with adrenal insufficiency and lead to definitive severe visual impairment.
良性颅内高压（假性脑瘤）已被描述为与库欣病（CD）中类固醇水平降低有关，尤其是在手术缓解后。酮康唑是治疗皮质醇增多症的常用且有效的辅助疗法，但主要问题是肝酶功能障碍。我们在此描述一名12岁CD女童在酮康唑治疗期间发生良性颅内高压的病例。她出现头痛、呕吐、右侧颞侧视野黑点以及颅内压升高的体征。磁共振成像（MRI）显示垂体影像正常，使用乙酰唑胺治疗后所有症状均有改善。我们提醒注意CD患者，尤其是接受酮康唑治疗的儿童中这种疾病的诊断，因为它可能与肾上腺功能不全相混淆并导致严重的视力损害。
