Lucidi V, Di Capua M, Rosati P, Papadatou B, Castro M
Department of Gastroenterology, Hospital Bambino Gesù, Rome, Italy.
Pediatr Neurol. 1993 Nov-Dec;9(6):494-5. doi: 10.1016/0887-8994(93)90033-9.
Despite having normal height and weight, a 6-year-old girl had frequent bowel movements and slight recurrent chest infections since the age of 4 years and headache for 1 year. The patient appeared healthy, but examination of the ocular fundus revealed papilledema. Cranial computed tomography appeared normal. Lumbar puncture disclosed an elevated opening cerebrospinal fluid pressure, with normal biochemical, cellular, and bacteriologic findings. Laboratory investigations indicated pathologic steatorrhea, elevated electrolytes in 3 sweat tests, and low serum levels of vitamins A and E. The diagnosis of pseudotumor cerebri in a patient with cystic fibrosis was made. After treatment with prednisone (1 mg/kg/day), pancreatic extracts, and vitamin supplements, headache and papilledema resolved and serum vitamin A and E levels subsequently became normal. Older children with cystic fibrosis rarely have benign intracranial hypertension, but when present it is often due to hypervitaminosis during correction of malnutrition. In this child, pseudotumor cerebri and associated hypovitaminosis improved after combined corticosteroid and vitamin treatment.
一名6岁女童身高体重正常，但自4岁起就频繁排便，反复出现轻微的胸部感染，且头痛已有1年。患儿外表健康，但眼底检查发现视乳头水肿。头颅计算机断层扫描显示正常。腰椎穿刺显示脑脊液初压升高，生化、细胞及细菌学检查结果正常。实验室检查表明存在病理性脂肪泻，3次汗液试验电解质升高，血清维生素A和E水平降低。该患儿被诊断为患有囊性纤维化的假性脑瘤。在用泼尼松（1毫克/千克/天）、胰腺提取物和维生素补充剂治疗后，头痛和视乳头水肿消失，血清维生素A和E水平随后恢复正常。年龄较大的囊性纤维化患儿很少出现良性颅内高压，但一旦出现，通常是由于营养不良纠正过程中的维生素过多症所致。在该患儿中，联合使用皮质类固醇和维生素治疗后，假性脑瘤及相关的维生素缺乏症得到改善。
