Department of Neurology, Nationwide Children's Hospital, Columbus, OH.
Department of Neurology, Nationwide Children's Hospital, Columbus, OH.
Semin Pediatr Neurol. 2021 Dec;40:100922. doi: 10.1016/j.spen.2021.100922. Epub 2021 Sep 4.
Primary intracranial hypertension (PIH) is characterized by clinical signs of increased intracranial pressure, papilledema, elevated opening pressure, and absence of mass lesion, hydrocephalus, or meningeal enhancement on neuroimaging. Visual changes are a common presenting feature and if untreated there is risk of irreversible vision loss. There have been recent proposed changes to the criteria for PIH along with studies looking at the differences in imaging characteristics between adult and pediatric PIH. The presence of transverse sinus stenosis alone was highly sensitive and specific for pediatric PIH. The Idiopathic Intracranial Hypertension Treatment Trial was an adult, multicenter study that examined the use of acetazolamide and weight loss on the course of PIH. The study confirmed many previously held beliefs including the most common presenting symptom in PIH is headache. Most patients present with bilateral papilledema with 58.2% of patients having symmetric Frisen scale grading and within one grade in 92.8%. Although diplopia is a common reported symptom, very few have evidence of cranial nerve palsy. Male gender, high-grade papilledema, and decreased visual acuity at presentation are risk factors for treatment failure. Acetazolamide use is associated with mild metabolic acidosis. During acetazolamide treatment, monitoring for hypokalemia or aplastic anemia is not recommended. Monitoring transaminases in the titration phase of treatment should be considered due to a case of transaminitis and pancreatitis with elevated lipase. Newer case reports have also seen associations of secondary intracranial hypertension with concurrent COVID-19 infection and MIS-C.
原发性颅内高压症(PIH)的特征是颅内压升高的临床体征、视乳头水肿、颅内压升高、神经影像学检查无肿块病变、脑积水或脑膜强化。视力变化是常见的表现特征,如果不治疗,有视力丧失不可逆转的风险。最近提出了 PIH 标准的修改建议,并对成人和儿童 PIH 的影像学特征差异进行了研究。单纯横窦狭窄对儿童 PIH 具有高度的敏感性和特异性。特发性颅内高压治疗试验是一项成人多中心研究,研究了乙酰唑胺和减肥对 PIH 病程的影响。该研究证实了许多以前的观点,包括 PIH 最常见的表现症状是头痛。大多数患者表现为双侧视乳头水肿,58.2%的患者 Frisen 分级对称,92.8%的患者分级相差一级。尽管复视是一种常见的报告症状,但很少有颅神经麻痹的证据。男性、高级别视乳头水肿和就诊时视力下降是治疗失败的危险因素。乙酰唑胺的使用与轻度代谢性酸中毒有关。在乙酰唑胺治疗期间,不建议监测低钾血症或再生障碍性贫血。由于有一例氨基转移酶升高和胰腺炎伴高淀粉酶血症,应考虑在治疗滴定阶段监测氨基转移酶。最近的病例报告还发现继发性颅内高压与同时发生的 COVID-19 感染和 MIS-C 有关。