Primary intracranial pressure disorders include idiopathic intracranial hypertension and spontaneous intracranial hypotension. Remarkable advances have been made in the diagnosis and treatment of these 2entities in recent years. Therefore, the Spanish Society of Neurology\'s Headache Study Group (GECSEN) deemed it necessary to prepare this consensus statement, including diagnostic and therapeutic algorithms to facilitate and improve the management of these disorders in clinical practice. This document was created by a committee of experts belonging to GECSEN, and is based on a systematic review of the literature, incorporating the experience of the participants, and establishes practical recommendations with levels of evidence and grades of recommendation.

BACKGROUND: Pseudotumor cerebri (PC) in prepubertal patients displays certain characteristics that differentiate it from its presentation at the postpubertal stage. The aim of this study is to describe the characteristics of paediatric patients diagnosed with PC at our centre and to compare them according to their pubertal status.
METHODS: We included patients aged between 1 and 18 years who were diagnosed with PC in a tertiary-level hospital between 2006 and 2019 and who met the updated diagnostic criteria for PC. They were classified according to body weight and pubertal status. Subsequently, we analysed results from lumbar punctures, neuroimaging studies, ophthalmological assessments, and treatments received during follow-up.
RESULTS: We included 28 patients, of whom 22 were of prepubertal age and 6 were of postpubertal age. The mean age (standard deviation) was 9.04 (2.86) years. Among the postpubertal patients, 83.3% were boys, 66.7% of whom presented overweight/obesity. In the group of prepubertal patients, 27% were boys, 31.8% of whom were overweight. The most frequent symptoms were headache (89.9%) and blurred vision (42.9%). All patients presented papilloedema, and 21.4% manifested sixth nerve palsy. Possible triggers were identified in 28.6% of cases. Nineteen percent of patients presented clinical recurrence, all of whom were prepubertal patients. Complete clinical resolution was achieved in 55.6% of patients.
CONCLUSIONS: Prepubertal patients with PC show lower prevalence of obesity, higher prevalence of secondary aetiologies, and higher recurrence rates than postpubertal patients.

Spontaneous cerebrospinal fluid (CSF) fistula, of unknown origin, is a rare condition whose aetiology is increasingly related to idiopathic intracranial hypertension (IIH). This study tries to raise awareness that they should not be considered as two different processes, but that fistulas can be a form of debut, requiring a study and subsequent treatment. Repair techniques are described, as well as the study of HII.
We treated 8 patients, 5 women and three men, aged between 46 and 72 years, with a diagnosis of spontaneous CSF fistula, four nasal and four otics who underwent surgical treatment. After repair, a diagnostic study was performed for IIH by MRI and Angio-MRI, presenting in all cases a transverse venous sinus stenosis. The intracranial pressure values obtained by lumbar puncture showed values of 20mm Hg or higher. All patients were diagnosed with HII. The one-year follow-up did not reveal any recurrence of the fistulas, maintaining a control of the HII.
Despite their low frequency of both cranial CSF fistula and IIH, an association of both conditions should be considered by continuing the study and surveillance of these patients after fistula closure.

Lumboperitoneal shunting makes it possible to regulate the flow of cerebrospinal fluid by establishing a connection between the thecal sac and the peritoneal cavity. The main indication for lumboperitoneal shunting in children is idiopathic intracranial hypertension, but the technique is also useful in the treatment of postinfectious, posthemorrhagic, and normotensive hydrocephalus, as well as in the treatment of postsurgical pseudomeningocele or leakage of cerebrospinal fluid. This article reviews nine cases treated at our centre to show the normal imaging findings for lumboperitoneal shunts in children and to provide a succinct review of the possible neurological and abdominal complications associated with this treatment.

Optic nerve drusen are acellular concretions of calcium located in the parenchyma of the optical nerve head described as the most common cause of pseudo-papilloedema, which makes it difficult to differentiate from a true optical disc oedema. Despite it being rare, the drusen of the optic nerve and the papilloedema secondary to idiopathic intracranial hypertension can coexist in the same patient. The case is presented of a 34 year-old woman referred to the Ophthalmology Department with visual discomfort, headaches, and pulsatile tinnitus of two months onset. In the physical examination there was bilateral blurring of the optic disc margin with absence of spontaneous venous pulsation. The diagnosis of idiopathic intracranial hypertension was made by lumbar puncture with the measurement of the opening pressure, and due to the findings of the magnetic resonance scan of the brain and the eye sockets. The optic nerve drusen was an incidental finding in an optical coherence tomography angiography, which would later be confirmed with an eye ultrasound. To our knowledge, this the first case reported in Latin America of the coexistence of optic nerve drusen and papilloedema secondary to idiopathic intracranial hypertension.

BACKGROUND: Pseudotumor cerebri (PC) in prepubertal patients displays certain characteristics that differentiate it from its presentation at the postpubertal stage. The aim of this study is to describe the characteristics of paediatric patients diagnosed with PC at our centre and to compare them according to their pubertal status.
METHODS: We included patients aged between 1 and 18 years who were diagnosed with PC in a tertiary-level hospital between 2006 and 2019 and who met the updated diagnostic criteria for PC. They were classified according to body weight and pubertal status. Subsequently, we analysed results from lumbar punctures, neuroimaging studies, ophthalmological assessments, and treatments received during follow-up.
RESULTS: We included 28 patients, of whom 22 were of prepubertal age and 6 were of postpubertal age. The mean age (standard deviation) was 9.04 (2.86) years. Among the postpubertal patients, 83.3% were boys, 66.7% of whom presented overweight/obesity. In the group of prepubertal patients, 27% were boys, 31.8% of whom were overweight. The most frequent symptoms were headache (89.9%) and blurred vision (42.9%). All patients presented papilloedema, and 21.4% manifested sixth nerve palsy. Possible triggers were identified in 28.6% of cases. Nineteen percent of patients presented clinical recurrence, all of whom were prepubertal patients. Complete clinical resolution was achieved in 55.6% of patients.
CONCLUSIONS: Prepubertal patients with PC show lower prevalence of obesity, higher prevalence of secondary aetiologies, and higher recurrence rates than postpubertal patients.

Pseudotumor cerebri is a disorder characterized by increased intracranial pressure that predominantly affects obese young women. This paper aims to define the concepts of primary pseudotumor cerebri, in which the cause cannot be identified (also known as idiopathic intracranial hypertension), and secondary pseudotumor cerebri, in which the cause can be identified. We review the current role of imaging techniques in diagnosing pseudotumor cerebri and describe and illustrate the most characteristic imaging findings of the disorder, some of which are included in the diagnostic criteria proposed in 2013. We also consider the fundamental role of interventional radiology in the treatment of pseudotumor cerebri because placing a stent in stenosed venous sinuses is a novel treatment option in patients who are refractory to classical treatment. Finally, we describe the imaging biomarkers that have been evaluated for diagnosing primary pseudotumor cerebri and predicting the response to treatment.

To evaluate the efficacy of lumbo-peritoneal shunt (LPS) in patients of idiopathic intracranial hypertension presenting with visual symptoms.
Between Apr. 2014 and Mar. 2018, 70 patients of Idiopathic Intracranial Hypertension (IIH) underwent treatment at our institution. Patients were evaluated for neurological and ophthalmological status and were subjected to LPS depending on their symptoms.
Mean opening pressure was 29.97±5.33cm of water and mean Body-Mass Index (BMI) was 26.51±3 and the two were significantly correlated (p-value 0.006). All patients with visual symptoms (23) underwent LPS and others (47) were managed medically. All patients with LPS and 25 of medically managed patients improved, while 22 medically-managed patients required LPS due to deterioration in visual symptomatology. The proportion of patients showing complete resolution of features of IIH was significantly different between the three groups. Of the 7 patients with shunt extrusion/migration, only 2 required revision.
LPS is an equally effective and more technically familiar modality for treatment of IIH for neurosurgeons and should be offered to asymptomatic patients with objective visual signs. Shunt extrusion/migration may not always warrant revision due to \"mini-shunt\" that drains Cerebro-Spinal Fluid (CSF) through shunt tract even after extrusion.

BACKGROUND: We present our experience on idiopathic intracranial hypertension (IIH), before and after the introduction of a specific diagnosis and management protocol.
METHODS: A descriptive retrospective study was conducted on patients with IIH over a 25year period (1990-2015), comparing the last 7years (after introduction of the protocol) with the previous 18years.
RESULTS: Among the 18,865 patients evaluated, there were 54 cases of IIH (29 infants and 25 children). A comparison was made between the two time periods: 32 cases in 1990-2008 -published in An Pediatr (Barc). 2009;71:400-6-, and 23 cases in 2008-2015. In post-protocol period, there were 13 patients aged between 3-10months (62% males) with transient bulging fontanelle, and 10 aged between 2-14years (50% males), with papilloedema. A total of 54% of infants had recently finished corticosteroid treatment for bronchitis. In the older children, there was one case associated with venous thrombosis caused by otomastoiditis, one case on corticosteroid treatment for angioma, and another case treated with growth hormone. Transfontanelle ultrasound was performed on all infants, and CT, MRI and angio-MRI was performed on every child. Lumbar puncture was performed on 2 infants in whom meningitis was suspected, and in all children. All patients progressed favourably, with treatment being started in 3 of them. One patient relapsed.
CONCLUSIONS: Characteristics and outcomes of patients overlap every year. IIH usually has a favourable outcome, although it may be longer in children than in infants. It can cause serious visual disturbances, so close ophthalmological control is necessary. The protocol is useful to ease diagnostic decisions, monitoring, and treatment.

Spontaneous cerebrospinal fluid (CSF) fistulas are rather common in daily practice. The aim of the surgical treatment is closure of the leak, but recurrences are quite frequent. The association between spontaneous CSF fistulas and idiopathic intracranial hypertension (IIH) is not uncommon, and this is probably the cause of the low rate of success of the surgical treatment. Symptoms of IIH associated with spontaneous CSF fistula are atypical, and diagnosis is often missed. Continuous intracranial pressure monitoring is very useful in the diagnosis of chronic IIH and in patients with spontaneous CSF fistula, as it helps in making decisions on the treatment of these patients.