Is Lumbar Puncture Needed? – Noninvasive Assessment of ICP Facilitates Decision Making in Patients with Suspected Idiopathic Intracranial Hypertension

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.

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A Case of Pulsatile Tinnitus Related to Idiopathic Intracranial Hypertension

Korean Journal of Otorhinolaryngology - Head and Neck Surgery ◽

10.3342/kjorl-hns.2020.00052 ◽

2020 ◽

Vol 63 (9) ◽

pp. 422-426

Author(s):

Jong-Won Bae ◽

Kyu-Yup Lee ◽

Myung Hoon Yoo ◽

Da Jung Jung

Keyword(s):

Intracranial Hypertension ◽

Young Women ◽

Idiopathic Intracranial Hypertension ◽

Obese Woman ◽

Pulsatile Tinnitus ◽

Obese Women ◽

Women Of Childbearing Age ◽

Childbearing Age ◽

Early Symptom ◽

Vascular Abnormalities

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.

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Unilateral papilledema in idiopathic intracranial hypertension: A rare entity

European Journal of Ophthalmology ◽

10.1177/1120672120969041 ◽

2020 ◽

pp. 112067212096904

Author(s):

Mousumi Banerjee ◽

Swati Phuljhele Aalok ◽

Deepti Vibha

Keyword(s):

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Pseudotumor Cerebri ◽

Clinical Syndrome ◽

Unknown Etiology ◽

Obese Women ◽

Mechanical Compression ◽

Childbearing Age ◽

Diagnostic Challenge ◽

Rare Presentation

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.

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Idiopathic Intracranial Hypertension

Neuro-Ophthalmology ◽

10.1093/med/9780190603953.003.0009 ◽

2019 ◽

pp. 47-54

Author(s):

Matthew J. Thurtell ◽

Robert L. Tomsak

Keyword(s):

Older Adults ◽

Visual Field ◽

Intracranial Pressure ◽

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Vision Loss ◽

Clinical Signs ◽

Field Testing ◽

Obese Women ◽

Childbearing Age

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.

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Management of Idiopathic Intracranial Hypertension: Experience of a North African Center

Indian Journal of Neurosurgery ◽

10.1055/s-0040-1710107 ◽

2020 ◽

Vol 9 (02) ◽

pp. 085-088

Author(s):

Ibrahim Assoumane ◽

Mahdia Touati ◽

Harissou Adamou ◽

Nadia Lagha ◽

Ibrahim Baaré ◽

...

Keyword(s):

Intracranial Hypertension ◽

Young Women ◽

Idiopathic Intracranial Hypertension ◽

Sinus Thrombosis ◽

Postoperative Outcome ◽

North African ◽

Childbearing Age ◽

Neurosurgical Department ◽

The Mean ◽

Space Occupation

Abstract Background The idiopathic intracranial hypertension (IIH) is characterized by elevation of intracranial pressure (ICP) in the absence of intracerebral space occupation or venous sinus thrombosis. It usually occurs in obese young women but is very rare in childhood. Materials and Methods We conducted a retrospective study in the neurosurgical department of Bab El Oued teaching hospital of Algiers over a period of 8 years from January 2008 to December 2015. We analyzed clinical data of 10 patients with IIH diagnosis, and the surgical technique consisted of an insertion of a lumbo-peritoneal shunt. Results In our study, 80% were women with sex ratio M/F of 0.25; the mean age was 32 years and 60% of our patients were obese. For most of our patients, the postoperative outcome was marked by the disappearance of the headache immediately, disappearance of strabismus and diplopia in the following week, followed by improvement of visual function. Conclusion The IIH is predominantly a disease of women in the childbearing age; surgical treatment is a good option for patients who resisted medication or did not tolerate it as well as for ICH fulminous in emergency cases.

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

Cephalalgia ◽

10.1111/j.1468-2982.2007.01442.x ◽

2007 ◽

Vol 27 (11) ◽

pp. 1261-1264 ◽

Cited By ~ 10

Author(s):

F Distelmaier ◽

D Tibussek ◽

DT Schneider ◽

E Mayatepek

Keyword(s):

Seasonal Variation ◽

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Bacterial Infections ◽

Fluid Pressure ◽

Obese Women ◽

Women Of Childbearing Age ◽

Childbearing Age ◽

Age Related ◽

Concurrent Infections

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.

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Etiology, pathogenesis and management of idiopathic intracranial hypertension, and role of optic canal size in asymmetric papilledema: A review

European Journal of Ophthalmology ◽

10.1177/11206721211005709 ◽

2021 ◽

pp. 112067212110057

Author(s):

Chandler Mitchell ◽

Sunu Mathew ◽

Alon Harris ◽

Matthew Lang ◽

Devin Mackay ◽

...

Keyword(s):

Intracranial Pressure ◽

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Disease Process ◽

Unknown Origin ◽

Optic Canal ◽

Definitive Treatment ◽

Pathophysiological Mechanism ◽

Obese Women ◽

Childbearing Age

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.

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Evaluation of cerebrovascular hemodynamics in patients with idiopathic intracranial hypertension using transcranial Doppler

The Egyptian Journal of Neurology Psychiatry and Neurosurgery ◽

10.1186/s41983-020-00250-8 ◽

2020 ◽

Vol 56 (1) ◽

Author(s):

Mahmoud M. Allam ◽

Hatem A. Almasry ◽

Sandra M. Ahmed ◽

Youssuf G. Taha ◽

Mohammed I. Oraby

Keyword(s):

Intracranial Pressure ◽

Intracranial Hypertension ◽

Lumbar Puncture ◽

Transcranial Doppler ◽

Idiopathic Intracranial Hypertension ◽

Pulsatility Index ◽

Research Work ◽

Increased Intracranial Pressure ◽

Significant Relation ◽

Before And After

Abstract Background Idiopathic intracranial hypertension (IIH) is a disorder of increased intracranial pressure without a clear cause and can have serious visual effects. Previous research work suggests that transcranial Doppler measurements of pulsatility index correlate accurately with elevated intracranial pressure. Objective To assess the cerebrovascular hemodynamic changes in patients with IIH using transcranial Doppler before and after lumbar puncture and CSF withdrawal. Methods An interventional study conducted on 40 patients (31 females and 9 males) fulfilling the modified Dandy criteria for diagnosis of idiopathic intracranial hypertension, MRI brain, and MRV was done to the patients. Lumbar puncture was done for all included patients to measure intracranial pressure and CSF withdrawal. Transcranial Doppler was performed for all included before and after lumbar puncture and CSF withdrawal and the following parameters were measured: peak systolic velocity (PSV), end diastolic velocity (EDV), resistivity index (RI), and pulsatility index (PI). Results Significant relation was found between grades of papilledema and PSV, RI, and PI (p value 0.012, 0.025, 0.016) but no significant relation was found between grades of papilledema and EDV (0.102). Significant changes occurred in parameters of TCD pre- and post-CSF withdrawal including PSV, EDV, and PI (p value 0.001, 0.015, 0.019) denoting a significant change in cerebral hemodynamics after CSF withdrawal which denotes a decrease in intracranial pressure. Conclusion Increased intracranial pressure significantly affects cerebral blood flow. A normalization of transcranial Doppler parameters occurs following lowering of intracranial pressure through lumbar puncture and CSF withdrawal.

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11β-Hydroxysteroid dehydrogenase type 1 inhibition in idiopathic intracranial hypertension: a double-blind randomized controlled trial

Brain Communications ◽

10.1093/braincomms/fcz050 ◽

2020 ◽

Vol 2 (1) ◽

Cited By ~ 7

Author(s):

Keira Markey ◽

James Mitchell ◽

Hannah Botfield ◽

Ryan S Ottridge ◽

Tim Matthews ◽

...

Keyword(s):

Intracranial Hypertension ◽

Lumbar Puncture ◽

Idiopathic Intracranial Hypertension ◽

Controlled Trial ◽

Hydroxysteroid Dehydrogenase ◽

Opening Pressure ◽

Double Blind ◽

Randomized Controlled

Abstract Treatment options for idiopathic intracranial hypertension are limited. The enzyme 11β-hydroxysteroid dehydrogenase type 1 has been implicated in regulating cerebrospinal fluid secretion, and its activity is associated with alterations in intracranial pressure in idiopathic intracranial hypertension. We assessed therapeutic efficacy, safety and tolerability and investigated indicators of in vivo efficacy of the 11β-hydroxysteroid dehydrogenase type 1 inhibitor AZD4017 compared with placebo in idiopathic intracranial hypertension. A multicenter, UK, 16-week phase II randomized, double-blind, placebo-controlled trial of 12-week treatment with AZD4017 or placebo was conducted. Women aged 18–55 years with active idiopathic intracranial hypertension (>25 cmH2O lumbar puncture opening pressure and active papilledema) were included. Participants received 400 mg of oral AZD4017 twice daily compared with matching placebo over 12 weeks. The outcome measures were initial efficacy, safety and tolerability. The primary clinical outcome was lumbar puncture opening pressure at 12 weeks analysed by intention-to-treat. Secondary clinical outcomes were symptoms, visual function, papilledema, headache and anthropometric measures. In vivo efficacy was evaluated in the central nervous system and systemically. A total of 31 subjects [mean age 31.2 (SD = 6.9) years and body mass index 39.2 (SD = 12.6) kg/m2] were randomized to AZD4017 (n = 17) or placebo (n = 14). At 12 weeks, lumbar puncture pressure was lower in the AZD4017 group (29.7 cmH2O) compared with placebo (31.3 cmH2O), but the difference between groups was not statistically significant (mean difference: −2.8, 95% confidence interval: −7.1 to 1.5; P = 0.2). An exploratory analysis assessing mean change in lumbar puncture pressure within each group found a significant decrease in the AZD4017 group [mean change: −4.3 cmH2O (SD = 5.7); P = 0.009] but not in the placebo group [mean change: −0.3 cmH2O (SD = 5.9); P = 0.8]. AZD4017 was safe, with no withdrawals related to adverse effects. Nine transient drug-related adverse events were reported. One serious adverse event occurred in the placebo group (deterioration requiring shunt surgery). In vivo biomarkers of 11β-hydroxysteroid dehydrogenase type 1 activity (urinary glucocorticoid metabolites, hepatic prednisolone generation, serum and cerebrospinal fluid cortisol:cortisone ratios) demonstrated significant enzyme inhibition with the reduction in serum cortisol:cortisone ratio correlating significantly with reduction in lumbar puncture pressure (P = 0.005, R = 0.70). This is the first phase II randomized controlled trial in idiopathic intracranial hypertension evaluating a novel therapeutic target. AZD4017 was safe and well tolerated and inhibited 11β-hydroxysteroid dehydrogenase type 1 activity in vivo. Reduction in serum cortisol:cortisone correlated with decreased intracranial pressure. Possible clinical benefits were noted in this small cohort. A longer, larger study would now be of interest.

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Evaluation of a method for noninvasive intracranial pressure assessment during infusion studies in patients with hydrocephalus

Journal of Neurosurgery ◽

10.3171/jns.2000.92.5.0793 ◽

2000 ◽

Vol 92 (5) ◽

pp. 793-800 ◽

Cited By ~ 39

Author(s):

Bernhard Schmidt ◽

Marek Czosnyka ◽

Jens Jürgen Schwarze ◽

Dirk Sander ◽

Werner Gerstner ◽

...

Keyword(s):

Mathematical Model ◽

Intracranial Pressure ◽

Time Course ◽

Cerebral Blood Flow Velocity ◽

Absolute Error ◽

Content Type ◽

Arterial Blood ◽

Potential Clinical Benefit ◽

The Mean ◽

Fine Print

Object. A mathematical model previously introduced by the authors allowed noninvasive intracranial pressure (nICP) assessment. In the present study the authors investigated this model as an aid in predicting the time course of raised ICP during infusion tests in patients with hydrocephalus and its suitability for estimating the resistance to outflow of cerebrospinal fluid (Rcsf).Methods. Twenty-one patients with hydrocephalus were studied. The nICP was calculated from the arterial blood pressure (ABP) waveform by using a linear signal transformation, which was dynamically modified by the relationship between ABP and cerebral blood flow velocity. This model was verified by comparison of nICP with “real” ICP measured during lumbar infusion tests. In all simulations, parallel increases in real ICP and nICP were evident. The simulated Rcsf was computed using nICP and then compared with Rcsf computed from real ICP. The mean absolute error between real and simulated Rcsf was 4.1 ± 2.2 mm Hg minute/ml. By the construction of simulations specific to different subtypes of hydrocephalus arising from various causes, the mean error decreased to 2.7 ± 1.7 mm Hg minute/ml, whereas the correlation between real and simulated Rcsf increased from R = 0.73 to R = 0.89 (p < 0.001).Conclusions. The validity of the mathematical model was confirmed in this study. The creation of type-specific simulations resulted in substantial improvements in the accuracy of ICP assessment. Improvement strategies could be important because of a potential clinical benefit from this method.

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External lumbar drainage in progressive pediatric idiopathic intracranial hypertension

Journal of Neurosurgery Pediatrics ◽

10.3171/2021.2.peds2143 ◽

2021 ◽

pp. 1-7

Author(s):

Gad Dotan ◽

Natalie Hadar Cohen ◽

Hanya M. Qureshi ◽

Mika Shapira Rootman ◽

Yoram Nevo ◽

...

Keyword(s):

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Vision Loss ◽

Symptom Relief ◽

Visual Outcome ◽

Fiber Layer ◽

Lumbar Drainage ◽

Lumbar Drain ◽

Rnfl Thickness ◽

External Lumbar Drainage

OBJECTIVE Pediatric idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure despite normal cerebrospinal fluid and neuroimaging findings. Initial management is typically medical; however, nearly 10% of children will eventually require surgery for persistent headache and/or vision loss. External lumbar drainage, which is a considerably safer treatment option, has not been adequately analyzed in children with medically refractory IIH. METHODS The authors conducted a single-institution retrospective analysis of children with medically refractory IIH who had undergone external lumbar drain (ELD) placement because of worsening papilledema, reflected as increased retinal nerve fiber layer (RNFL) thickness on optical coherence tomography (OCT) testing. The main outcome measures were effects of external lumbar drainage on papilledema resolution, symptoms, and vision. RESULTS The authors analyzed the medical records of 13 children with IIH (11 girls, mean age 15.0 ± 2.3 years) whose mean CSF opening pressure was 45.5 ± 6.8 cm H2O. In all children, the average global RNFL thickness in both eyes significantly increased at ELD placement (right eye 371.8 ± 150.2 μm, left eye 400.3 ± 96.9 μm) compared with presentation thickness (right eye 301.6 ± 110.40 μm, left eye 350.2 ± 107.7 μm) despite acetazolamide medical therapy (20–30 mg/kg/day), leading to ELD placement after 9.5 ± 6.9 days (range 3–29 days). After ELD insertion, there was headache resolution, gradual and continuous improvement in optic disc thickness, and preservation of good vision. CONCLUSIONS ELD placement in children with medically refractory IIH who demonstrated worsening papilledema with increased RNFL thickening on OCT testing typically results in symptom relief and disc edema resolution with good visual outcome, often preventing the need for additional definitive surgeries that carry greater failure and morbidity risks.

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