【双语病例】特发性颅内压增高

History: A 63-year-old woman presents with headaches, nausea, vomiting, and vertigo.

病史：女性，63岁，头痛、恶性、呕吐、眩晕。

Sagittal T1, axial T2, and axial T2 fluid-attenuatedinversion-recovery (FLAIR) MR images of the head are shown below.

如下所示MR图像：矢状T1、轴位T2、轴位FLAIR。

Findings

Sagittal T1-weighted MR image shows an empty and expanded sella turcica.

Axial T2 and axial FLAIR images show the optic nerve sheath is widened and expanded with cerebrospinal fluid (CSF) hyperintensity surrounding the optic nerve.

Axial T2 and axial FLAIR images shows posterior flattening of the globes.

表 现

矢状T1WI：空蝶鞍、蝶鞍扩大；轴位T2WI/FLAIR：视神经鞘增宽，视神经周围可见脑脊液样高信号；眼球后方略扁平。

Differential diagnosis

  • Idiopathic intracranial hypertension

  • Mass lesion

  • Obstructive hydrocephalus

  • Venous sinus thrombosis

  • Dural arteriovenous fistula

Diagnosis: Idiopathic intracranial hypertension (IIH)

鉴别诊断

特发性颅内压增高、占位性病变、梗阻性脑积水、静脉窦血栓、硬脑膜动静脉瘘。

最后诊断：特发性颅内压增高

Key points

Idiopathic intracranial hypertension (IIH)

Pathophysiology

As the name suggests, the exact cause of idiopathic intracranial hypertension is not fully understood. The diagnosis is dependent upon exclusion of other causes of intracranial hypertension, including mass lesions and hydrocephalus.Pseudotumor cerebri refers to patients with raised intracranial pressure without hydrocephalus or mass lesion but which may be secondary to findings such as venous sinus thrombosis or dural arteriovenous fistula. There are several theories regarding IIH’s pathophysiology, which include the following:

  • Impaired CSF homeostasis (reduced CSF reabsorption or excess CSF production)

  • Altered vitamin A metabolism, supported by associations with medications including tetracycline and corticosteroids

  • Venous sinus stenosis, which may cause increased venous pressures, leading to decreased CSF resorption, increased ICP and venous sinus compression

病理生理

顾名思义，特发性颅内压增高病因不明，诊断主要依靠排除其它原因的颅内高压，例如：占位性病变、脑积水。脑假性肿瘤是指不是由于占位或脑积水而是继发于静脉窦血栓、硬脑膜动静脉瘘的颅压增高的患者。关于IIH的病因，包括以下几种理论：脑脊液平衡状态受损（脑脊液再吸收减少或者产生过度）；维生素A代谢改变，与四环素、类固醇类药物相关；静脉窦狭窄，可以导致静脉压增高、脑脊液吸收减少、颅压增高和静脉窦高压。

Clinical presentation Classically, idiopathic intracranial hypertension presents with headache and vision changes in obese women of child bearing age.

Modified Dandy criteria for diagnosis of IIH:

  • Signs/symptoms from increased intracranial pressure (headaches, nausea/vomiting, transient visual change, papilledema)

  • No focalizing neurological signs other than CN VI palsy

  • Documented elevated intracranial pressure by lumbar puncture greater than 20 mm in normal-weight individuals and greater than 25 mm in obese individuals with normal CSF composition

  • No evidence of hydrocephalus, mass, or structural or vascular lesion on MRI/MRA

IIH经典临床表现：育龄期肥胖女性有头痛、视力改变的症状。

IIH的诊断标准

有颅压增高的相关症状、体征（头痛、恶心、呕吐、短暂视力改变、视乳头水肿）；除了VI组颅神经（外展神经）麻痹，没有集中的神经症状；脑脊液穿刺颅压大于20mm（正常体重），大于25mm（肥胖人群），脑脊液成分未见异常；MRI/MRA未发现脑积水、占位、结构异常、血管病变。

Imaging features

  • “Empty sella sign” -- Refers to when the pituitary gland is not visible and is associated with longstanding effects of increased intracranial pressure.

  • Optic nerve sheath enlargement with widened ring of CSF signal intensity around the side of the optic nerve

  • Posterior globe flattening, which is associated with increased ocular pressure by transmission of elevated CSF pressure through the optic nerve sheath to the posterior globe, and is associated with the finding of papilledema

  • Optic nerve tortuosity

  • Intraocular protrusion of the optic nerve head

  • Stenosis of the transverse cerebral venous sinuses

影像表现

  • 空蝶鞍：指垂体未见显示，与长期颅压增高有关；

  • 视神经鞘扩大：视神经周围环状的脑脊液样高信号影增宽；

  • 眼球后部扁平：脑脊液压力增高通过视神经鞘传递至眼球后部，导致眼睛压力增高，可见视神经乳头水肿；

  • 视神经弯曲；

  • 视神经乳头凸向眼球内；

  • 脑静脉窦（横窦）变窄。

    如下图

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