Danish Headache Center, Department of Neurology, Rigshospitalet, Glostrup, Denmark.
University of Copenhagen, Copenhagen, Denmark.
Cephalalgia. 2022 Dec;42(14):1510-1520. doi: 10.1177/03331024221120073. Epub 2022 Aug 19.
To identify the most frequent causes of secondary pseudotumor cerebri syndrome and compare phenotype, clinical presentation, and symptoms of secondary pseudotumor cerebri syndrome to the primary form of pseudotumor cerebri syndrome, idiopathic intracranial hypertension.
The study was a prospective cohort study including patients with new-onset pseudotumor cerebri syndrome. Diagnostic work up was standardized. Patients were diagnosed with secondary pseudotumor cerebri syndrome or idiopathic intracranial hypertension according to the revised Friedman criteria. Secondary pseudotumor cerebri syndrome patients were categorized into five causes: medication, systemic causes, sleep apnea, cerebrovascular causes, and several competing causes. Phenotype, clinical presentation, symptoms and neuroimaging were compared between groups.
Out of 278 cases, 28 secondary pseudotumor cerebri syndrome and 120 idiopathic intracranial hypertension patients were included. The most frequent causes of secondary pseudotumor cerebri syndrome were medication (n = 8, 28.6%) and systemic causes (n = 8, 28.6%), followed by sleep apnea (n = 5, 17.9%), cerebrovascular causes (n = 4, 14.3%) and several competing causes (n = 3, 10.7%). Secondary pseudotumor cerebri syndrome and idiopathic intracranial hypertension patients were phenotypically alike and predominately female, premenopausal, and obese. Symptoms and objective findings at disease onset were similar between groups.
Secondary pseudotumor cerebri syndrome should be considered in all patients with suspected pseudotumor cerebri syndrome as secondary pseudotumor cerebri syndrome and idiopathic intracranial hypertension patients are phenotypically and clinically alike. A thorough diagnostic workup is needed as treatment of idiopathic intracranial hypertension and secondary pseudotumor cerebri syndrome is markedly different.
确定继发性假性脑瘤综合征最常见的病因，并将继发性假性脑瘤综合征的表型、临床表现和症状与原发性假性脑瘤综合征（特发性颅内高压）进行比较。
本研究为前瞻性队列研究，纳入新发假性脑瘤综合征患者。诊断工作采用标准化方法。根据修订后的 Friedman 标准，患者被诊断为继发性假性脑瘤综合征或特发性颅内高压。将继发性假性脑瘤综合征患者分为五类病因：药物、系统性疾病、睡眠呼吸暂停、脑血管疾病和多种病因并存。比较各组之间的表型、临床表现、症状和神经影像学表现。
在 278 例患者中，有 28 例继发性假性脑瘤综合征和 120 例特发性颅内高压患者纳入研究。继发性假性脑瘤综合征最常见的病因是药物（n = 8，28.6%）和系统性疾病（n = 8，28.6%），其次是睡眠呼吸暂停（n = 5，17.9%）、脑血管疾病（n = 4，14.3%）和多种病因并存（n = 3，10.7%）。继发性假性脑瘤综合征和特发性颅内高压患者表型相似，主要为女性、绝经前和肥胖。两组患者在疾病发作时的症状和客观发现相似。
在所有疑似假性脑瘤综合征患者中都应考虑继发性假性脑瘤综合征，因为继发性假性脑瘤综合征和特发性颅内高压患者在表型和临床方面相似。需要进行全面的诊断工作，因为特发性颅内高压和继发性假性脑瘤综合征的治疗方法明显不同。
