Idiopathic intracranial hypertension: Key signs and symptoms characterized

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1. Idiopathic intracranial hypertension is predominantly a disease of obese young women with headache being the most common symptom. 

2. In patients with visual loss, the most common perimetric finding was partial arcuate visual field defect with an enlarged blind spot, and patients tended to have mild loss of visual acuity. 

Evidence Rating Level: 2 (Good) 

Study Rundown: Idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, is a syndrome characterized by increased intracranial pressure (ICP) and its associated signs and symptoms with no other secondary cause of increased ICP. This study found IIH to be a disease primarily of young, obese women (98% of study participants) who mostly presented with headache as the initial symptom. Transient visual obscurations, back pain, and tinnitus (pulse synchronized and non-pulsatile) were also commonly reported. In patients with visual loss, the most common perimetric finding was partial arcuate visual field defect with an enlarged blind spot, and patients tended to have mild loss of visual acuity.

The strengths of this study include a prospectively conducted, in-depth, and thorough assessment of patients in multiple domains related to IIH, including detailed diagnostic testing, strict adherence to modified Dandy criteria for inclusion, and functional-behavioral assessments relevant to IIH. The majority of patients enrolled being women suggests that IIH may predominantly be a disease of women, and men carrying diagnoses of IIH may have an underlying secondary cause.  Further, the study elucidated the typical symptoms found in this population with detailed assessments of the severity via validated tools and techniques. One drawback of the study is the exclusion of patients with moderate to severe visual loss, which comprises 2/3rds of the IIH population.

Click to read the study in JAMA Neurology

Relevant Reading: The Pseudotumor Cerebri Syndrome

In-Depth [prospective cohort]: The study population in this report included the 165 patients with mild visual loss (161 women, 4 men) enrolled in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) across 38 sites in the US and Canada over a 3 year period. In order to be included, patients met modified Dandy criteria for IIH and had perimetric mean deviation (PMD) in the -2 to -7dB range on 24-2 Swedish interactive testing algorithm. Each patient had general, ophthalmologic, and neurologic examinations, MRI, lumbar puncture, and genetic studies, among other laboratory investigations. The patients also underwent perimetry, fundus photography, and obesity evaluation, in addition to several questionnaires.

The average (SD) age of patients enrolled in the study was 29.0 (7.4). Sixty percent of these patients were white, 25% were black, and 5% of these identified a family member with IIH.  The mean (SD) BMI was 39.9 (8.3). The most common initial symptom was headache (84%) followed by transient visual obscurations (68%), back pain (53%), and pulse synchronized tinnitus (52%). 23% of patients reported non-pulsatile tinnitus. The average (SD) PMD in the worst eye was -3.5 (1.1) dB and the best eye was -2.3 (1.1). The most common visual field defect was a partial arcuate defect with an enlarged blind spot, and the average (SD) CSF opening pressure was 343.5 (86.9) mm H2O. The average (SD) mental health summary score was 44.6 (12.6), and the mean for US women between ages 25-34 was 48.

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