Idiopathic Intracranial Hypertension

A patient with low pressure idiopathic intracranial hypertension and multiple cranial neuropathies

Authors: Arora A, Sreenivasan S, Naeem Raza M.

Idiopathic intracranial hypertension is a disorder of increased intracranial pressure of unknown cause. Idiopathic intracranial hypertension can present with a wide spectrum of neurological signs, especially cranial nerve palsies, which are mostly thought to be caused by a pressure-dependent stretching mechanism. Treatment is guided by aetiology whenever possible; otherwise drainage of CSF by ventriculostomy or shunt is needed.

Idiopathic intracranial hypertension in pediatric population: Case series from India

Authors: Roy AG, Vinayan KP, Kumar A.

Background: Idiopathic intracranial hypertension (IIH) is a well described entity in adults. In pediatric age group the presentation of disease can vary depending on the age of patients and is less frequently reported. Aim: The aim of this study is to describe the clinical features, investigations, treatment and outcome of IIH in pediatric population (age <18 years). Materials and Methods: This retrospective hospital based study was carried out on 25 children with diagnosis of IIH based on modified Dandys criteria. Their clinical, investigation, treatment, outcome and follow-up for 2 year period were analyzed. Results: Out of the 25 children, the youngest child was 4-month-old infant. The commonest symptom was headache (76%) followed by vomiting and papilledema (72%). The mean cerebrospinal fluid (CSF) pressure was 330 mm of H 2 O. In Infants irritability and bulging anterior fontanelle was seen. A total of 24 patients showed a complete resolution of symptom. None of patient had recurrence over a period of 2 years follow-up. Conclusion: IIH can present at any age group. This is the largest series of IIH reported in pediatric population in India. The clinical features are similar to adult patients except in infants. Absence of papilledema does not exclude the diagnosis of IIH. CSF pressure monitoring is needed in suspected cases of IIH. Early and prompt treatment can prevent deficits.

Idiopathic intracranial hypertension: A possible complication in the natural history of advanced prostate cancer

Authors: Valcamonico F, Arcangeli G, Consoli F, Nonnis D, Grisanti S, Gatti E, Berruti A, Ferrari V.

Idiopathic intracranial hypertension is a variety of intracranial hypertension that is extremely rare in men. Obesity and hypogonadism are the most important predictive factors. Etiological hypotheses include increased central venous pressure, and various hormonal and metabolic changes commonly found in obese patients. We described the case of an obese man with prostate cancer who showed a consistent bodyweight increase during treatment with taxanes and prednisone. He was hospitalized because of a severe loss of vision as a consequence of idiopathic intracranial hypertension. A complete symptom remission was obtained after 3 weeks of anti-edema therapies (steroids, acetazolamide). Castration-resistant prostate cancer is a risk factor for idiopathic intracranial hypertension. Long-term androgen deprivation therapy, bodyweight increase, and fluid retention during chronic steroid administration and taxane chemotherapy might favor the disease onset. This severe complication has a good outcome, and should be suspected in the presence of symptoms and signs of intracranial hypertension.

Idiopathic intracranial hypertension; research progress and emerging themes

Authors: Batra R, Sinclair A.

Idiopathic intracranial hypertension (IIH) is a condition characterised by increased intracranial pressure of unknown cause predominantly seen in obese women of childbearing age and associated with a history of recent weight gain. The aetiology is poorly understood and there are no evidence-based guidelines on the management of the disease. We aim to provide a review of the recent literature outlining the latest advances in this field over the past few years. Areas of emerging interest related to the pathophysiology of IIH will be discussed, such as the role of obesity, adipose tissue and 11β-hydroxysteroid dehydrogenase type 1. We consider the latest research on the role of venous sinus stenosis in IIH and ex vivo advances into cerebrospinal fluid drainage via the arachnoid granulation tissue. The latest techniques for optic nerve head evaluation and the role of optical coherence tomography will be summarised. Finally, we will discuss recent advances in the management of IIH, including weight loss, and medical and surgical treatment strategies.

MRI findings of elevated intracranial pressure in cerebral venous thrombosis versus idiopathic intracranial hypertension with transverse sinus stenosis

Authors: Ridha MA, Saindane AM, Bruce BB, Riggeal BD, Kelly LP, Newman NJ, Biousse V.

PURPOSE: To determine whether MRI signs suggesting elevated intracranial pressure (ICP) are preferentially found in patients with idiopathic intracranial hypertension (IIH) than in those with cerebral venous thrombosis (CVT).
METHODS: Among 240 patients who underwent standardized contrast-enhanced brain MRI/MRV at our institution between 9/2009 and 9/2011, 60 with abnormal imaging findings on MRV were included: 27 patients with definite IIH, 2 patients with presumed IIH, and 31 with definite CVT. Medical records were reviewed, and imaging studies were prospectively evaluated by the same neuroradiologist to assess for presence or absence of transverse sinus stenosis (TSS), site of CVT if present, posterior globe flattening, optic nerve sheath dilation/tortuosity, and the size/appearance of the sella turcica.
RESULTS: 29 IIH patients (28 women, 19 black, median-age 28, median-body mass index, 34) had bilateral TSS. 31 CVT patients (19 women, 13 black, median-age 46, median-BMI 29) had thrombosis of the sagittal (3), sigmoid (3), cavernous (1), unilateral transverse (7), or multiple (16) sinuses or cortical veins (1). Empty/partially-empty sellae were more common in IIH (3/29 and 24/29) than in CVT patients (1/31 and 19/31) (p<0.001). Flattening of the globes and dilation/tortuosity of the optic nerve sheaths were more common in IIH (20/29 and 18/29) than in CVT patients (13/31 and 5/31) (p<0.04).
CONCLUSION: Although abnormal imaging findings suggestive of raised ICP are more common in IIH, they are not specific for IIH and are found in patients with raised ICP from other causes such as CVT.

Imaging signs in idiopathic intracranial hypertension: Are these signs seen in secondary intracranial hypertension too?

Authors: Hingwala DR, Kesavadas C, Thomas B, Kapilamoorthy TR, Sarma PS.

BACKGROUND: The purpose of this study was to evaluate the difference in the occurrence of the various "traditional" imaging signs of intracranial hypertension (IIH) on magnetic resonance imaging (MRI) in patients with idiopathic (IIH) and secondary intracranial hypertension.
MATERIALS AND METHODS: In a retrospective analysis, the MRI findings of 21 patients with IIH and 60 patients with secondary intracranial hypertension (41 with tumors; 19 with intracranial venous hypertension) were evaluated for the presence or absence of various "traditional" imaging signs of IIH (perioptic nerve sheath distention, vertical buckling of optic nerve, globe flattening, optic nerve head protrusion and empty sella) using the Fisher's exact test. Odds ratios were also calculated. Statistical Package for the Social Sciences version 17.0 was used for statistical analysis. Subgroup analysis of the IIH versus tumors and IIH versus venous hypertension were performed.
RESULTS: Optic nerve head protrusion and globe flattening were significantly associated with IIH. There was no statistically significant difference in the occurrence of rest of the findings. On subgroup analysis, globe flattening and optic nerve head protrusion occurred significantly more often in IIH than in tumors. However, there was no statistically significant difference in the occurrence of any of these findings in patients with IIH and venous hypertension.
CONCLUSIONS: IIH is a diagnosis of exclusion. While secondary causes of raised intracranial pressure (ICP) have obvious clinical findings on MRI, some conditions like cerebral venous thrombosis may have subtle signs and differentiating between primary and secondary causes may be difficult. In the absence of any evident cause of raised ICP, presence of optic nerve head protrusion or globe flattening can suggest the diagnosis of IIH.

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