Differential diagnosis of patients with intracranial sinus venous thrombosis related isolated intracranial hypertension from those with idiopathic intracranial hypertension

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Abstract

In patients presenting with intracranial hypertension without hydrocephalus, mass lesions, and with normal cerebrospinal fluid (CSF) composition (pseudotumor cerebri syndrome), the diagnosis of intracranial sinus venous thrombosis (ISVT) has crucial etiological, therapeutic and prognostic implications. Utilizing two well-defined groups of pseudotumor cerebri patients, one with magnetic resonance imaging (MRI) or angiography confirmed ISVT (17 patients) and the other in whom ISVT has been excluded (idiopathic intracranial hypertension [IIH], 27 patients), we investigated the characteristics that might be helpful in distinguishing them. No clinical or auxiliary findings differed between the ISVT and IIH groups except for female gender and lower CSF protein level, which were significantly associated with the latter. While the syndrome pseudotumor cerebri could be due to multiple causes including ISVT, the term IIH should be restricted for patients with isolated intracranial hypertension attributable to no other neurological or systemic disease. Since CT frequently misses ISVT, patients with pseudotumor cerebri syndrome should undergo MRI and MR venography before being labeled as IIH. We conclude that Modified Dandy's Diagnostic Criteria of pseudotumor cerebri, formulated prior to MRI era, can no longer be applied for the diagnosis of IIH.

Introduction

The syndrome of increased intracranial pressure without hydrocephalus, mass lesions, and with normal cerebrospinal fluid (CSF) composition was referred prior to the present era of advanced neuroimaging techniques as pseudotumor cerebri [1], but is currently known as idiopathic intracranial hypertension (IIH) [2], [3]. Modified Dandy's criteria to diagnose pseudotumor cerebri, formulated before the magnetic resonance imaging (MRI) era, advocated computed tomography (CT) as the neuroimaging modality to exclude hydrocephalus and mass lesions [4] (Table 1).

Over one-third of patients with intracranial sinus venous thrombosis (ISVT) can present with intracranial hypertension without any other neurological symptoms or signs [5], [6], [7], [8]. The patients with ISVT presenting with isolated intracranial hypertension can fulfill Modified Dandy's Diagnostic Criteria of pseudotumor cerebri (Table 1), including normal brain CT and normal CSF composition [5], [6], [7], [8]. Although IIH is a self-limiting disorder without sequel in the majority, severe deficits in visual acuity have been reported in up to 25% of patients [9]. ISVT is associated with 10–30% mortality from cerebral infarctions, uncontrolled seizures, pulmonary embolism, or from the underlying disease, such as infection or cancer, which predisposed the patient to ISVT [10], [11]. There is evidence that patients with ISVT treated early with anticoagulants, and those who progress despite anticoagulants with intrasinus urokinase infusions, have more favorable prognosis [12]. Therefore, in patients presenting with isolated intracranial hypertension, diagnosis of ISVT has crucial etiological, therapeutic and prognostic implications.

Today, the term IIH should be restricted to patients in whom all causes of intracranial hypertension, including ISVT, have been excluded [9], [13]. The best diagnostic tool to document ISVT is intra-arterial digital subtraction angiography (DSA) [12]. The advent of MRI, and more specifically magnetic resonance venography (MRV), allows reliable and noninvasive diagnosis of ISVT [14], [15]. Clinical characteristics that can distinguish patients with IIH from those with ISVT related isolated intracranial hypertension might help in selecting patients for MRI/MRV or DSA in a cost-effective manner.

Utilizing two well-characterized groups of patients, one with IIH and the other with ISVT-related isolated intracranial hypertension, we intended to answer the following questions: Are there any clinical or laboratory characteristics that are helpful in distinguishing between these two groups of patients? Do the Modified Dandy's Diagnostic Criteria for pseudotumor cerebri, formulated in 1985 prior to MRI era [4], need revision for the diagnosis of IIH?

Section snippets

Patients and methods

The subjects for this study were seen between January 1991 and March 2001 in the Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, a tertiary referral center, situated at Trivandrum, Kerala, India. Seventeen out of the forty-seven consecutive patients (36%) with ISVT who presented with isolated intracranial hypertension without any other neurological symptoms or signs formed the ISVT group. All these patients underwent MRI/MRV (Signa, 1.5 T, GE Medical

Clinical characteristics

The demographic and clinical characteristics of ISVT and IIH groups of patients are compared in Table 2. While there were more men in the ISVT group, women predominated the IIH group. The distribution of other attributes such as age, duration of symptoms, and prevalence of obesity, visual sequel and risk factors did not differ between the two groups. Other than the postpartum state in one patient, the etiology of ISVT could not be determined. None fulfilled the new international criteria for

ISVT with isolated intracranial hypertension

ISVT has long been recognized as a cause of intracranial hypertension without localizing neurological signs [17], [18]. The reported frequency of ISVT in patients with isolated intracranial hypertension has varied depending upon the patient population studied and the methods used to study them. The CT was the only investigation performed to exclude ISVT in most of the series of patients reported as IIH [2], [3], [19].

Although CT often reveals indirect signs such as venous infarction and brain

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