scholarly journals Idiopathic Intracranial Hypertension – Pathophysiology Based on Case Series

2016 ◽  
Vol 33 (3) ◽  
pp. 199-209
Author(s):  
Srdjan Ljubisavljević ◽  
Jasna Zidverc Trajković

Summary According to the definition, idiopathic intracranial hypertension (IIH) is a pathological state characterized by an increase in intracranial pressure; however, there are no obvious intracranial pathological processes. The pathophysiology of this disorder is not clear, although there are many reports related to it. We present an overview of possible etiopathogenetic mechanisms, clinical presentations and therapeutic interventions from a series of patients hospitalized with the clinical picture and final diagnosis of idiopathic intracranial hypertension (IIH). All data were collected from the moment of IIH diagnosis as well as three months later. The obtained data showed that IIH is a disease that primarily affects obese women in early and midlife. The positive correlation between values of cerebrospinal fluid pressure and body mass index was observed. The disorders of sexual hormones were identified as a possible etiology for IIH female patients. Headache, papilloedema, decreased visual acuity, vertigo and cranial nerve palsy were identified as the most prevalent IIH clinical presentations. The existence of stenosis and hypoplasia of the sigmoid and transverse sinus were confirmed only in one third of IIH patients. Pharmacotherapy combined with weight loss was efficacious in a large number of patients. In this series, there were no short-term consequences of IIH. The results suggest the importance of early and accurate looking for IIH in obese early and midlife women with any hormonal imbalances having a variety of neurological expression, mostly presented as headaches and visual disturbances. Early detection of IIH might influence the timely treatment and prevent far-reaching and severe clinical consequences.

2014 ◽  
Vol 7 (1) ◽  
pp. 5-9
Author(s):  
Manish Modi ◽  
Karan Gupta

ABSTRACT Objective Pediatric idiopathic intracranial hypertension is an underdiagnosed entity with catastrophic presentations. High index of suspicion with early diagnosis and prompt treatment is the key to successful management. Trans-nasal trans-sphenoid Endoscopic Optic Nerve Fenestration is an effective surgical modality for the reversal of vision loss in pediatric idiopathic intracranial hypertension (IIH). Materials and methods This is a single center observational prospective case series. Five diagnosed pediatric patients of IIH satisfying the modified Dandy criteria and reported to the out-patient services of otolaryngology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India in the year 2012 were included in this study. All children underwent thorough clinical examination, complete Neuro-ophthalmological work-up including Visual acuity (V/A), Visual field charting (V/F), Fundus venogram and Radiological work-up with MRI for special optic nerve sections in sagittal reconstruction. Cerebro-Spinal Fluid pressure (CSF) measured preoperatively for all children. Standard endoscopic optic nerve Sheath Fenestration was performed on all children. visual improvement was assessed by comparing preoperative ophthalmological findings. Results Improvement in vision was taken as a positive outcome. Vision improved in all except one child, who had pre-existing optic nerve atrophy. Conclusion Endoscopic optic nerve fenestration is an effective minimally invasive procedure to revert visual loss in pediatric idiopathic intracranial hypertension. How to cite this article Gupta AK, Gupta K, Modi M, Gupta A. Pediatric Idiopathic Intracranial Hypertension: A not so Rare and Benign Condition. Clin Rhinol An Int J 2014;7(1):5-9.


Cephalalgia ◽  
2007 ◽  
Vol 27 (11) ◽  
pp. 1261-1264 ◽  
Author(s):  
F Distelmaier ◽  
D Tibussek ◽  
DT Schneider ◽  
E Mayatepek

Idiopathic intracranial hypertension is an enigmatic disorder of elevated cerebrospinal fluid pressure. In adulthood, patients are typically obese women of childbearing age; however, in young children the clinical picture is strikingly different, indicating age-related differences in the aetiology of idiopathic intracranial hypertension. To investigate this phenomenon, we analysed the clinical details of 15 pre-pubertal children with the diagnosis of idiopathic intracranial hypertension. Evaluating the date of initial presentation, we discovered a distinct seasonal variation. Ten patients presented between November and March, thus coinciding with the typical season of paediatric viral and bacterial infections in Germany. Therefore, we suggest an association between intracranial hypertension and possibly concurrent infections in these children. Moreover, eight children presented only with ophthalmologic findings without any other obvious symptoms, raising questions regarding the incidence of undetected cases, particularly in this age group.


2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
V Mahendran ◽  
P Ricart ◽  
M Wadley ◽  
A Perry ◽  
S Robinson

Abstract Introduction Idiopathic Intracranial Hypertension (IIH) is a significant cause of preventable blindness. Patients also suffer from debilitating headaches, pulsatile tinnitus, nausea, vomiting, photophobia, and radicular pain. If IIH continues to rise as predicted, treatment cost will increase to 462.7 million GBP annually by 2030. Weight loss is the only proven disease-modifying therapy for reversal of IIH. Bariatric surgery is an attractive treatment option due to superlative weight loss and reversal of related comorbidities. The case series aims to raise awareness of bariatric surgery as a safe and effective treatment modality for IIH. Method The case series consists of a retrospective analysis of four patients with a pre-operative diagnosis of IIH. They were referred to our department for bariatric surgery between January to December 2018. They were followed up for a total of two years. Results In our case series, all four patients were females with a mean age of 34 years. Mean BMI reduced from 47.3 kg/m2 before surgery to 30 kg/m2 with an EWL of 76.4% at the end of two years after surgery. They all showed significant improvement or resolution in their symptoms related to IIH, and none of them required further CSF pressure reducing procedures afterwards. Conclusions Bariatric surgery is a safe and effective method of treating IIH. It is superior compared to medical management and CSF pressure reducing procedures which have high rates of recurrence.


2013 ◽  
Vol 61 (5) ◽  
pp. 488 ◽  
Author(s):  
ArunGrace Roy ◽  
KollencheriPuthenveettil Vinayan ◽  
Anand Kumar

Author(s):  
Jong-Won Bae ◽  
Kyu-Yup Lee ◽  
Myung Hoon Yoo ◽  
Da Jung Jung

Idiopathic intracranial hypertension (IIH) is a disorder that commonly occurs in obese young women of childbearing age and is characterized by symptoms such as pulsatile tinnitus, dizziness, headache, nausea, vomiting, and visual loss without any structural or vascular abnormalities in the intracranial cavity. We recently experienced a case of a 33-year-old obese woman who presented with right-sided pulsatile tinnitus, which is an early symptom for IIH. The patient was successfully treated with weight reduction and carbonic anhydrase inhibitor (acetazolamide). Pulsatile tinnitus requires thorough diagnosis and examination because it can be cured if the anatomical or functional cause is identified and treated. When obese women of childbearing age present with pulsatile tinnitus at the otorhinolaryngology outpatient department, treatment for IIH should be initiated after appropriate examination and diagnosis as pulsatile tinnitus may be the only symptom for IIH.


2020 ◽  
pp. 6054-6059
Author(s):  
Alexandra Sinclair

Idiopathic intracranial hypertension (IIH) (also called pseudotumour cerebri and, previously, benign intracranial hypertension) is a syndrome of raised intracranial pressure in the absence of an intracranial mass lesion, enlargement of the cerebral ventricles, or venous sinus thrombosis. IIH affects predominantly obese women of childbearing age (>90%). The condition has considerable morbidity from permanent visual loss (up to 25% of cases) and chronic disabling headaches, which result in poor quality of life. Patients presenting acutely with papilloedema must be evaluated urgently for secondary causes of raised intracranial pressure (e.g. space occupying lesion and venous thrombosis). After this, the priority is to assess accurately the threat to vision. In most patients, the condition becomes chronic and the disease burden is mostly from chronic headaches, which need active management, alongside visual monitoring. This chapter does not cover paediatric IIH.


Cephalalgia ◽  
2020 ◽  
Vol 40 (5) ◽  
pp. 429-436 ◽  
Author(s):  
Nils J Becker ◽  
Sören Enge ◽  
Katharina Maria Kreutz ◽  
Felix Schmidt ◽  
Lutz Harms ◽  
...  

Background A lumbar puncture constitutes an important diagnostic procedure in the evaluation of idiopathic intracranial hypertension. Chronic overflow of cerebrospinal fluid into the sheaths of the olfactory nerves appears to be related to olfactory impairment in these patients. Here, we asked whether cerebrospinal fluid drainage in idiopathic intracranial hypertension patients improves olfactory function. Methods Fourteen idiopathic intracranial hypertension patients and 14 neurologic control patients were investigated before and after lumbar puncture using the extended Sniffin’ Sticks procedure. We assessed odor threshold, discrimination, and identification. In idiopathic intracranial hypertension patients, cerebrospinal fluid was drained until cerebrospinal fluid pressure had normalized. In addition, a third group of 14 healthy controls participated in the two smell tests at similar intervals. Results Relative to healthy controls, threshold, discrimination, and identification composite scores before lumbar puncture were significantly lower in idiopathic intracranial hypertension patients and also in neurologic controls. Following lumbar puncture, threshold, discrimination, and identification scores for neurologic controls remained unchanged whereas idiopathic intracranial hypertension patients showed robust improvement on the composite score as well as on all three subscores (all changes: p < 0.003), quickly regaining olfactory function in the normal range. Cerebrospinal fluid opening pressure was significantly correlated with improvement in threshold, discrimination, and identification score upon cerebrospinal fluid drainage ( r = 0.609, p = 0.021). Conclusion Olfactory impairment is an important, yet underappreciated, clinical feature of idiopathic intracranial hypertension. Lowering of increased intracranial pressure improves hyposmia. Our findings shed new light on the pathophysiology of cerebrospinal fluid circulation in idiopathic intracranial hypertension.


2019 ◽  
Vol 34 (13) ◽  
pp. 806-814 ◽  
Author(s):  
Sama Boles ◽  
Claudia Martinez-Rios ◽  
Daniel Tibussek ◽  
Daniela Pohl

Idiopathic intracranial hypertension, or pseudotumor cerebri, is an increase in cerebrospinal fluid pressure of unknown etiology. It is mostly seen in adults, less frequently in adolescents, rarely in younger children. Only 5 infants meeting idiopathic intracranial hypertension criteria have been mentioned in the literature. We report a case of a previously healthy 9-month-old boy who presented with irritability, decreased appetite, and a bulging fontanelle. Computed tomography (CT) head imaging and cerebrospinal fluid studies revealed normal results. The patient’s symptoms transiently resolved after the initial lumbar puncture, but 11 days later, his fontanelle bulged again. A second lumbar puncture revealed an elevated opening pressure of 35 cmH2O and led to a diagnosis of idiopathic intracranial hypertension in accordance with the modified Dandy Criteria. Treatment with acetazolamide at a dose of 25 mg/kg/d was initiated and the patient remained symptom-free for 6 weeks, followed by another relapse. His acetazolamide dose was increased to 37 mg/kg/d, with no further relapses to date. A diagnosis of idiopathic intracranial hypertension is challenging in infants, because the patients cannot yet verbalize typical idiopathic intracranial hypertension–related symptoms such as positional headaches, diplopia, or pulsatile tinnitus. Furthermore, it is more difficult to assess papilledema in that age group. If undetected and untreated, idiopathic intracranial hypertension may result in permanent visual deficits. Little is known about idiopathic intracranial hypertension in infants, and age-specific treatment guidelines are lacking. We discuss this rare case of infantile idiopathic intracranial hypertension and provide a review of the literature, including an overview of disease characteristics and outcomes of idiopathic intracranial hypertension in this very young age group.


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