High-pressure headaches: Idiopathic intracranial hypertension and its mimics

Department of Neurology, Neurological Institute, Taipei Veterans General Hospital, No. 201, Sec. 2, Shipai Road, Taipei, Taiwan 11217.
Nature Reviews Neurology (Impact Factor: 15.36). 11/2012; 8(12). DOI: 10.1038/nrneurol.2012.223
Source: PubMed


Idiopathic intracranial hypertension (IIH) is a rare disorder that typically affects obese women of childbearing age, but can also occur in paediatric populations. Patients usually present with diffuse, daily headache and visual disturbances, but either symptom can occur in isolation. Patients with IIH often have papilloedema; however, IIH without papilloedema is fairly common in patients with chronic daily headache. The pathogenesis of IIH is unknown; the high incidence of comorbid bilateral transverse sinus stenosis (BTSS) in patients with IIH suggests that the two conditions are linked, although no direct causal relationship has been established. Cerebrospinal fluid (CSF) pressure monitoring or lumbar puncture-which provides immediate symptomatic relief-are important in making a diagnosis of IIH. Current treatments for IIH include weight reduction, medical treatment, CSF diversion surgery, optic nerve sheath fenestration and, potentially, endovascular stenting (in patients with BTSS). Prevention of visual loss (which can be substantial) is the main goal of treatment. Residual headache and IIH recurrence are not uncommon after treatment, and regular follow-up is, therefore, warranted even in patients who achieve remission. This Review provides an update of current knowledge of the aetiology, pathophysiology and treatment of IIH.

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    ABSTRACT: Background and purpose: Idiopathic intracranial hypertension is a headache syndrome characterized by increased CSF pressure. Compression of the hypophysis and distension of the optic nerve sheath are reliable imaging signs. The purpose of the study was to validate, in patients with idiopathic intracranial hypertension, MR imaging-based volumetric measurements of the optic nerve sheath and hypophysis as an objective observation method for more accurate diagnosis and posttreatment follow-up. Materials and methods: Twenty-three patients with idiopathic intracranial hypertension as well as age-, sex-, and body mass index-matched controls underwent volumetric measurements of the optic nerve, optic nerve sheath, and hypophysis on high-resolution T2-weighted MR images by using a 7-cm surface coil, followed by correlation with CSF opening pressures and clinical symptom scores of visual disturbances and headache. Results: Mean values of optic nerve sheath (341.86 ± 163.69 mm(3) versus 127.56 ± 53.17 mm(3), P < .001) and hypophysis volumes (554.59 ± 142.82 mm(3) versus 686.60 ± 137.84 mm(3), P < .05) differed significantly between healthy and diseased subjects. No significant differences between mean optic nerve volumes were observed. Receiver operating characteristic analysis showed optic nerve sheath volumes of >201.30 mm(3) (sensitivity, 86.96%; specificity, 91.30%) and hypophysis volumes of <611.21 mm(3) (sensitivity, 78.26%; specificity, 69.57%) to be indicative of idiopathic intracranial hypertension diagnosis. In patients with idiopathic intracranial hypertension, no correlations were found between optic nerve sheath and hypophysis volumes and CSF opening pressures or clinical scores of visual disturbances and headache. Conclusions: Semiautomated volumetric measurement of optic nerve sheath and hypophysis has the potential to more accurately diagnose and follow patients with idiopathic intracranial hypertension.
    American Journal of Neuroradiology 09/2013; 35(3). DOI:10.3174/ajnr.A3694 · 3.59 Impact Factor
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    ABSTRACT: Idiopathic intracranial hypertension (IIH) is a potentially blinding disease and may require surgical management when maximal medical treatment has failed. The purpose of this review is to discuss the current literature on surgical and endovascular treatments for IIH. The most commonly performed surgical treatments for IIH are cerebrospinal fluid diversion procedures (e.g. ventriculo- and lumbo-peritoneal shunts) and optic nerve sheath fenestration. Controversy still exists about which is the preferred initial surgical treatment for IIH. Emerging procedures include venous sinus stenting in cases with venous sinus stenosis, and bariatric surgery for weight loss. Cranial (suboccipital or subtemporal) decompression was a more popular surgical procedure in the past, but can still have a role in selected cases with impaired cerebrospinal flow dynamics (e.g. Chiari malformation) or after multiple failed conventional surgical procedures. This review compares and contrasts the surgical management options for IIH.
    Current opinion in neurology 11/2013; 27(1). DOI:10.1097/WCO.0000000000000049 · 5.31 Impact Factor
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    ABSTRACT: Unsuspected idiopathic intracranial hypertension (IIH) is found in a significant minority of patients attending clinics with named headache syndromes, if it is specifically sought out. Chronic fatigue syndrome is frequently associated with headache. Could the same be true of chronic fatigue? Moreover, there are striking similarities between the two conditions. Could they be related? Attempting to answer these questions, we describe the results of a change in clinical practice aimed at capturing patients with chronic fatigue who might have IIH. Cross-sectional. Hospital outpatient and radiology departments. Patients attending a specialist clinic with chronic fatigue syndrome and headache who had lumbar puncture to exclude raised intracranial pressure. Intracranial pressure measured at lumbar puncture and the effect on headache of cerebrospinal fluid drainage. Mean cerebrospinal fluid pressure was 19 cm H2O (range 12-41 cm H2O). Four patients fulfilled the criteria for IIH. Thirteen others did not have pressures high enough to diagnose IIH but still reported an improvement in headache after drainage of cerebrospinal fluid. Some patients also volunteered an improvement in other symptoms, including fatigue. No patient had any clinical signs of raised intracranial pressure. An unknown, but possibly substantial, minority of patients with chronic fatigue syndrome may actually have IIH. An unknown, but much larger, proportion of patients with chronic fatigue syndrome do not have IIH by current criteria but respond to lumbar puncture in the same way as patients who do. This suggests that the two conditions may be related.
    12/2013; 4(12):2042533313507920. DOI:10.1177/2042533313507920
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