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特发性颅内压增高

 仁医学生 2018-06-11

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【双语病例】Idiopathic Intracranial Hypertension 特发性颅内压增高

双语学影像;本期病例选自AJNR Case of the Week

Shyam Sabat, MD;Penn State Hershey Medical Center;State College, PA


Description 病史简介

A 32-year-old woman with visual disturbances in the left eye and occasional headaches

32岁女性,左眼视物不清,偶尔伴有头痛。


Legends 图例

Sagittal T1WI (A) shows a partially empty sella with A thin rim of pituitary tissue along the floor of the sella. Axial FLAIR IMAGE (B) shows normal-to-decreased size of the lateral ventricles. Axial T2WI (C) shows distended subarachnoid spaces around the optic nerves, with flattening of the posterior globes and mild convexity of optic nerve heads bilaterally. Noncontrast 2D MIP venogram images (D) show focal severe narrowing of the bilateral transverse sinuses (arrows) in the distal two-thirds (arrrows).

T1WI矢状位显示部分空蝶鞍,垂体菲薄,紧贴鞍底。

横断位FLAIR示侧脑室体积略小。

横断位T2WI示视神经周围蛛网膜下腔增宽,眼球后缘扁平,视神经前端呈凸面状。

2D MIP静脉成像显示双层横窦远端2/3重度狭窄。




Idiopathic Intracranial Hypertension

特发性颅内压增高

Background 背景

Idiopathic intracranial hypertension is a clinical syndrome of raised intracranial pressure without any identifiable cause. It typically affects obese women of child-bearing age.

特发性颅内压增高是指无明显诱因的颅内压增高,好发于肥胖的育龄妇女。

Clinical Feature 临床表现

Headaches, transient visual disturbances, pulse-synchronous tinnitus. Clinical diagnosis is supported by high opening pressures on lumbar puncture. If untreated, may result in visual loss.

常见症状包括头痛、一过性视觉障碍、搏动性耳鸣。腰椎穿刺压力升高可临床确诊。如果不采取治疗,可导致失明。

Key Diagnostic Feature 诊断要点
Normal/decreased size of the ventricular system, partially empty sella, flattening/indentation of the posterior sclera, prominent optic nerve sheaths, tortuosity of the optic nerves. Bilateral transverse sinus stenosis is associated with 90% of cases; however, whether this is a cause or effect relationship is debated.
脑室系统体积正常或略偏小,部分性空蝶鞍,巩膜后缘扁平或可见压迹,视神经鞘膨隆,视神经迂曲。
约90%的病例可见双侧横窦狭窄,但这是特发性颅内压增高的原因或继发表现,尚存在争议。
DDx 鉴别诊断
  • Mild cerebral edema

    轻度脑水肿

  • Empty sella syndrome

    空蝶鞍综合征

  • Hypoplastic dural venous sinuses

    硬脑膜静脉窦发育不全

Treatment

Treatment goals are restoration of visual acuity and resolution of the papilledema. Treatment strategies include:

治疗目标是缓解视神经乳头水肿,恢复视力。主要治疗方法包括:

  • Weight loss

    减肥

  • Acetazolamide (furosemide can be added)

    乙酰唑胺(可添加呋喃苯胺酸)

  • Therapeutic lumbar punctures - lumboperitoneal shunt

    腰椎穿刺治疗-腰椎管腹腔分流

  • Optic nerve sheath fenestration

    视神经鞘开窗术

  • Venous stenting has been also proposed if there is no improvement or if there is rapid worsening of visual acuity, but it is still not widely used.

    如果视觉敏感性无明显恢复或急剧恶化,可采取静脉窦支架植入,但这一方法尚未得到广泛应用。




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