Seasonal Variation and Atypical Presentation of Idiopathic Intracranial Hypertension in Pre-Pubertal Children

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.

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. 112067212096904
Author(s):  
Mousumi Banerjee ◽  
Swati Phuljhele Aalok ◽  
Deepti Vibha

Idiopathic Intracranial Hypertension (IIH), also known as pseudotumor cerebri is a clinical syndrome of unknown etiology, which typically affects young, obese women of childbearing age and is characterized by increased intracranial pressure and bilateral papilledema. Unilateral and highly asymmetrical papilledema is a rare presentation in IIH occurring in less than 4% of patients with definite IIH and can poses a diagnostic challenge for the treating physician as it usually raises the suspicion of localized ocular pathology. As per current consensus, papilledema results in stasis of axoplasmic transport due to mechanical compression, leading to secondary vascular changes of venous dilation and hemorrhage. Given this mechanism, the underlying reasons for unilateral and asymmetrical papilledema remain unclear.


2019 ◽  
pp. 47-54
Author(s):  
Matthew J. Thurtell ◽  
Robert L. Tomsak

Idiopathic intracranial hypertension is a syndrome of increased intracranial pressure of unknown cause that most often occurs in obese women of childbearing age, although it can also occur in children, men, and older adults. Papilledema is usually present and can cause irreversible vision loss if left untreated. In this chapter, we begin by reviewing the symptoms of this condition, which include headache, transient visual obscurations, and pulse-synchronous tinnitus. We next review the clinical signs and common findings on formal visual field testing. We then discuss the diagnostic evaluation and review the diagnostic criteria for this condition. Lastly, we review the goals and management of this condition, which must be tailored depending on the severity of symptoms, papilledema, and vision loss.


Author(s):  
Bernhard Schmidt ◽  
Marek Czosnyka ◽  
Danilo Cardim ◽  
Zofia Czosnyka ◽  
Bernhard Rosengarten

Abstract Purpose Idiopathic intracranial hypertension (IIH) usually occurs in obese women of childbearing age. Typical symptoms are headache and sight impairment. Lumbar puncture (LP) is routinely used for both diagnosis and therapy (via cerebrospinal fluid drainage) of IIH. In this study, noninvasively assessed intracranial pressure (nICP) was compared to LP pressure (LPP) in order to clarify its feasibility for the diagnosis of IIH. Materials and Methods nICP was calculated using continuous signals of arterial blood pressure and cerebral blood flow velocity in the middle cerebral artery, a method which has been introduced recently. In 26 patients (f = 24, m = 2; age: 33 ± 11 years), nICP was assessed one hour prior to LPP. If LPP was > 20 cmH2O, lumbar drainage was performed, LPP was measured again, and also nICP was reassessed. Results In total, LPP and nICP correlated with R = 0.85 (p < 0.001; N = 38). The mean difference of nICP-LPP was 0.45 ± 4.93 cmH2O. The capability of nICP to diagnose increased LPP (LPP > 20 cmH2O) was assessed by ROC analysis. The optimal cutoff for nICP was close to 20 cmH2O with both a sensitivity and specificity of 0.92. Presuming 20 cmH2O as a critical threshold for the indication of lumbar drainage, the clinical implications would coincide in both methods in 35 of 38 cases. Conclusion The TCD-based nICP assessment seems to be suitable for a pre-diagnosis of increased LPP and might eliminated the need for painful lumbar puncture if low nICP is detected.


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.


2021 ◽  
pp. 112067212110057
Author(s):  
Chandler Mitchell ◽  
Sunu Mathew ◽  
Alon Harris ◽  
Matthew Lang ◽  
Devin Mackay ◽  
...  

Idiopathic intracranial hypertension (IIH) is characterized by raised intracranial pressure of unknown origin that primarily afflicts obese women of childbearing age. There are several treatment options, but currently there are none that are effective for the entire affected population. The lack of a universally effective treatment is related to an incomplete understanding of the etiology of the condition and the lack of a well-defined pathophysiological mechanism for the disease process. Classically, IIH has been thought of as a diagnosis of exclusion once radiographical imaging has ruled out all other causes of elevated intracranial pressure. Today, we know that imaging does capture subtle changes, and might provide keys to finally understand the pathogenesis of IIH so that a definitive treatment can be discovered or developed. Recently, advancements in radiography, optical coherence tomography, and electroretinography have shown promise for the future of IIH evaluation. A topic within IIH imaging that has recently sparked interest is the possibility that the severity of papilledema may have an association with the size of the optic canal. In this article, we also discuss the recent studies on the relationship between asymmetric papilledema and optic canal size.


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.


Cephalalgia ◽  
2016 ◽  
Vol 37 (6) ◽  
pp. 525-531 ◽  
Author(s):  
Bedia Samancı ◽  
Yavuz Samancı ◽  
Erdem Tüzün ◽  
Güneş Altıokka-Uzun ◽  
Esme Ekizoğlu ◽  
...  

Background Although specific role players are currently unknown, contribution of inflammatory mediators has been suggested in the pathophysiology of idiopathic intracranial hypertension (IIH), which is a disease more prevalent in obese female individuals of childbearing age. We aimed to investigate the levels of adipokines and cytokines to demonstrate possible markers for inflammation that participate in IIH pathophysiology and their association with clinical features of IIH. Methods IIH patients, diagnosed according to the revised criteria, and age-, gender- and body mass index (BMI)-matched healthy controls were enrolled in this study. Serum samples were evaluated for insulin-like growth factor 1, insulin, nesfatin, adiponectin, interleukin (IL)-1β, IL-6, IL-8, leptin, plasminogen activator inhibitor type-1, resistin, tumour necrosis factor-alpha (TNF-α) and monocyte chemotactic protein 1 via enzyme-linked immunosorbent assay or multiplex immunoassays. Results IL-1β level was significantly higher ( p = 0.012), and IL-8 and TNF-α levels were significantly lower in the IIH group ( p < 0.001 and p = 0.008, respectively) compared to the control group. There were no correlations between the cytokine/adipokine levels and age, BMI, disease duration, and cerebrospinal fluid oligoclonal bands. There were also no significant differences in cytokine and adipokine levels between IIH patients regarding visual impairment. However, statistically significant differences were found between IIH patients with relapse versus healthy controls regarding IL-1β ( p = 0.007), IL-8 ( p = 0.001) and TNF-α ( p = 0.017) levels. Other investigated cytokines and adipokines showed no significant alterations in IIH patients investigated in the remission period. Conclusion Altered serum levels of IL-1β, IL-8 and TNF-α seem to be associated with IIH pathogenesis, and these cytokines may be used as prognostic markers in IIH to predict relapse.


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