Geenen C, Tein I, Ehrlich R M
Division of Neurology, Hospital for Sick Children, Toronto, Ontario, Canada.
Can J Neurol Sci. 1996 May;23(2):141-4. doi: 10.1017/s0317167100038889.
Increased intracranial pressure with encephalopathy has rarely been reported in Addison's disease.
Case Study.
A 16-year-old female who presented with cerebral edema of unknown etiology was eventually diagnosed as having Addison's disease. She had early morning headaches, fatiguability, diarrhea and deterioration in school performance. She was hyponatremic with a serum sodium of 128 mmol/L and hyperkalemic with a serum potassium of 5.9 mmol/L. She had a low serum osmolality (264 mosm), high urine osmolality (533 mosm) and high urine sodium (87 mosm). She had a postural drop in blood pressure and diffuse hyperpigmentation. An ACTH stimulation test revealed a low baseline cortisol and no response to ACTH. Plasma renin activity was increased. Serum ACTH was elevated. She responded well to intravenous fluids and solu-cortef and was discharged on hydrocortisone and florinef. She remains well 18 months after the acute episode with no neurologic complaints or findings.
Addison's Disease should be considered in the differential diagnosis of symptomatic cerebral edema and idiopathic intracranial hypertension.
Addison病伴颅内压升高及脑病的情况鲜有报道。
病例研究。
一名16岁女性因不明原因的脑水肿就诊，最终被诊断为Addison病。她有晨起头痛、易疲劳、腹泻及学业成绩下降的症状。她低血钠，血清钠为128 mmol/L，高血钾，血清钾为5.9 mmol/L。她血清渗透压低（264 mosm），尿渗透压高（533 mosm），尿钠高（87 mosm）。她有体位性血压下降及全身色素沉着。促肾上腺皮质激素（ACTH）刺激试验显示基础皮质醇水平低，对ACTH无反应。血浆肾素活性升高。血清ACTH升高。她对静脉补液和氢化可的松琥珀酸钠反应良好，出院时服用氢化可的松和氟氢可的松。急性发作18个月后她情况良好，无神经方面的主诉或体征。
在有症状的脑水肿和特发性颅内高压的鉴别诊断中应考虑Addison病。
