Intracranial hypertension

Spontaneous Cerebrospinal Fluid Otorrhea and Rhinorrhea in Idiopathic Intracranial Hypertension Patients

Authors: Rosenfeld E, Dotan G, Kimchi TJ, Kesler A.

BACKGROUND:: Spontaneous cerebrospinal fluid (CSF) leakage may occur in patients with normal or increased intracranial pressure (ICP). We describe herein spontaneous CSF leakage as a result of chronic increased ICP in 4 patients with idiopathic intracranial hypertension (IIH). Although rhinorrhea previously has been described in IIH patients, to our knowledge this is the first report of otorrhea in these patients. METHODS:: Four patients with spontaneous CSF leakage were examined between 2001 and 2011; 3 presented with rhinorrhea and 1 with otorrhea. Clinical settings and manifestations were analyzed. RESULTS:: All patients were found to have IIH. Three had been diagnosed with IIH several years earlier and had been noncompliant with their medical treatment, whereas in 1 patient, CSF rhinorrhea was the presenting symptom of IIH. CONCLUSION:: CSF leak is a rare complication in IIH patients. We have shown that rhinorrhea can be the presenting sign in these patients and that rhinorrhea and otorrhea can be a late sign of the disease.

Multiple myeloma and paget disease with abnormal skull lesions and intracranial hypertension

Authors: Caravita T, Siniscalchi A, Montinaro E, Bove R, Zaccagnini M, De Pascalis D, Morocutti A, Brusa L, Arciprete F, Cupini M, Perrotti A, Palma E, Fratoni S, De Simone R, Iani C, de Fabritiis P.

We report a 73 years old man with a diagnosis of Paget Disease (PD) and symptomatic Multiple Myeloma (MM). Coexistence of MM and PD has rarely been described. PD mimics many of the features of bone destructive process in MM, making differential diagnosis more complicated. In addition, the presence of serious muscolo-skeletal and metabolic complications in both diseases makes management of patients difficult, worsening the prognosis.The comparison of these two diseases has led to the characterization of a common molecular mechanism represented by the receptor activator of nuclear factor-kB ligand (RANKL)/Osteoprotegerin signaling pathway. The improved comprehension of these mechanisms led to the development of new pharmacologic agents (bisphosphonates, cytokines inhibitors) effective for the treatment of these bone diseases.

Cranial reconstruction for treatment of intracranial hypertension from sclerosteosis: case based update

Authors: Tholpady S, Dodd ZH, Havlik RJ, Fulkerson DH.

Sclerosteosis (OMIM 269500) is a progressive, autosomal recessive, sclerosing bone disorder with a well-defined phenotype. This phenotype is correlated with a deficiency in the protein product sclerostin, leading to bony overgrowth from the loss of inhibition of osteocyte function. Calvarial overgrowth can lead to cranial nerve palsies, visual impairment, and compression of the medulla at the foramen magnum. There is a presumption that calvarial thickening may lead to elevated intracranial pressure in these patients, although pressure measurements have not been published. In this paper, the authors report the case of a 28 year-old Saudi Arabian male with sclerostosis, progressive headaches, and a cervical spinal cord syrinx. A cranial reconstruction was performed by aggressively thinning the thickened cortical bone, thereby expanding the intracranial space. The measured intracranial pressure was 25-40 mmHG under anesthesia. After surgery, the patient had resolution of the headaches and radiographic near-resolution of the syrinx. The authors review their experience and the relevant literature with this rare case.
Copyright © 2012 Elsevier Inc. All rights reserved.

High-pressure headaches: idiopathic intracranial hypertension and its mimics

Authors: Peng KP, Fuh JL, Wang SJ.

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.

Idiopathic Intracranial Hypertension: A Unifying Neuroendocrine Hypothesis through the Adrenal-Brain Axis

Authors: Salpietro V, Polizzi A, Bertè LF, Chimenz R, Chirico V, Manti S, Ferraù V, Salpietro A, Arrigo T, Ruggieri M.

The clinical syndrome idiopathic intracranial hypertension (IIH), also termed pseudotumor cerebri, consists of symptoms of headache, nausea, vomiting and visual field defects in combination with findings of papilledema. IIH is more commonly seen in overweight women where the rise in intracranial pressure is putatively a consequence of an endocrine-based disturbance of electrolytes. Less frequently, it can also occur in children and in men, who may not be overweight. Associated risk factors include primary and secondary aldosteronism, pregnancy, corticosteroid therapy and Cushing disease. Herein, we review the association between these conditions and IIH working toward its having a unifying neuroendocrine hypothesis.

Diet-induced antisecretory factor prevents intracranial hypertension in a dosage-dependent manner

Authors: Johansson E, Al-Olama M, Hansson HA, Lange S, Jennische E.

Intake of specially processed cereal (SPC) stimulates endogenous antisecretory factor (AF) activity, and SPC intake has proven to be beneficial for a number of clinical conditions. The aim of the present study was to investigate the dosage relationship between SPC intake and plasma AF activity and to further correlate achieved AF levels to a biological effect. SPC was fed to rats in concentrations of 5, 10 or 15 % for 2 weeks. A further group was fed 5 % SPC for 4 weeks. AF activity and the complement factors C3c and factor H were analysed in plasma after the feeding period. Groups of rats fed the various SPC concentrations were subjected to a standardised freezing brain injury, known to induce increases in intracranial pressure (ICP). The AF activity in plasma increased after intake of SPC, in a dosage- and time-dependent manner. The complement factors C3c and factor H increased in a time-dependent manner. Measurements of ICP in animals fed with SPC prior to the brain injury showed that the ICP was significantly lower, compared with that of injured rats fed with a standard feed, and that the change was dose and time dependent. AF activity increases, in a dosage- and time-dependent manner, after intake of SPC. The inverse relationship between ICP after a head injury and the percentage of SPC in the feed indicate that the protective effect is, to a large extent, due to AF.

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