WED 094 Diagnostic error rates in diagnosing idiopathic intracranial hypertension

2018 ◽  
Vol 89 (10) ◽  
pp. A10.1-A10
Author(s):  
Scotton Sangeeta ◽  
Liczkowski Anthony ◽  
Mollan Susan P ◽  
Sinclair Alexandra J
Keyword(s):  
Intracranial Hypertension ◽  
Diagnostic Criteria ◽  
Lumbar Puncture ◽  
Error Rate ◽  
Idiopathic Intracranial Hypertension ◽  
Final Diagnosis ◽  
Diagnostic Error ◽  
Error Rates ◽  
Tertiary Centre ◽  
Error Taxonomy

ObjectiveTo quantify the rate of diagnostic error amongst patients with IIH. Additionally to identify factors contributing to diagnostic error.MethodsSequential patients referred with a diagnosis of IIH to the Birmingham tertiary neuro-ophthalmology IIH clinic were prospectively included (October 2013- February 2017) A diagnostic error taxonomy tool was applied to cases referred as ‘definite’ or ‘possible’ IIH. Discrepancy between referred and final diagnosis were recorded. Results212 patients were referred, (96.2% female), 138/212 (65%) with definite IIH and 74/212 (35%) with possible IIH. Of those diagnosed with definite IIH 25% were not IIH and out of those diagnosed with possible IIH 57% were not IIH. Reasons for diagnostic error included incorrectly identifying papilloedema where in fact pseudopapilloedema existed and diagnosing IIH following an isolated lumbar puncture (LP) pressure >25 cmCSF (but in the absence of other diagnostic criteria for IIH). Misdiagnosis lead to 43% receiving unnecessary acetazolamide (or other diuretics) and 14% having multiple LPs.ConclusionsWe noted a high diagnostic error rate amongst IIH patients referred to a tertiary centre for ongoing management. Where there is doubt about the presence of true papilloedema early specialist review may reduce unnecessary treatment and LP’s.

scholarly journals Idiopathic intracranial hypertension in pediatric and adolescent patients

2019 ◽  
Vol 34 (1) ◽  
Author(s):  
Safwat Abouhashem ◽  
Ahmed A. M. Gad ◽  
Mohamed El-Malkey ◽  
Esam A. Daoud
Keyword(s):  
Risk Factors ◽  
Intracranial Hypertension ◽  
Diagnostic Criteria ◽  
Lumbar Puncture ◽  
Idiopathic Intracranial Hypertension ◽  
Pediatric Patients ◽  
Opening Pressure ◽  
Group I ◽  
Group Ii ◽  
Associated Risk Factors

Abstract Objectives Pediatric idiopathic intracranial hypertension is a rare condition, but inappropriate diagnosis and management may lead to devastating outcome with loss of vision and lifelong handicap. Dandy criteria are used for diagnosis of idiopathic intracranial hypertension (IIH) in adult, but these criteria cannot be applied in all pediatric patients. The aim of this study is to evaluate the diagnostic criteria and outcome of management of IIH in pediatric patients. Methods Nineteen patients with IIH and age less than 16 years old have been evaluated for the diagnostic criteria and outcome of management. The patients were classified according to the secondary sexual criteria into adolescent IIH and pediatric IIH. Full neurological and ophthalmological evaluation was completed in all patients. The patients were managed and followed up for a mean period of 12 ± 8.6 months at Zagazig University hospitals or Kingdom Hospital in the time period from 2009 to 2018. Results Nineteen patients (16 females and 3 males) had been diagnosed and treated with idiopathic intracranial hypertension, their age is between 4 and 15 years, and the patients have been divided into two groups. Group I (pediatric IIH) involved 9 patients (6 females and 3 males), and their age is ranging between 4 and 9 years with mean age 5.56 ± 1.9 years while group II (adolescent IIH) involved 10 patients, all of them are females and their age ranges between 12 and 15 years with mean age 13.5 ± 1.3. Diagnostic criteria of the patients are papilledema, symptoms and signs of intracranial hypertension, and elevated CSF opening pressure with normal MRI. Headache, delayed school performance, and sixth nerve palsy are the most common clinical finding in the patients of group I while headache is the most common presenting symptom in group II. Anemia and otitis media are the most common associated risk factors in group I while obesity and female gender were the most common associated risk factors in group II. The minimum value for opening pressure in group I is 180 mmH2O while the minimum value in group II is 250 mm. 16 patients improved after the first lumbar puncture and drainage of CSF; two patients improved after repeated lumbar puncture while lumbo-peritoneal shunt was inserted for two patients, both of them were in group II. Recurrence was diagnosed in four patients (21%), one of them (11.1%) was in group I while three of them (30%) were in group II. Conclusion Pediatric idiopathic intracranial hypertension can be classified into two subtypes: pediatric type and adolescent type according to the secondary sexual criteria. Modified Dandy criteria can be applied for the diagnosis of adolescent type, but pediatric type is not associated with obesity, has no female predominance, and usually responds to the initial lumbar puncture with a low rate of recurrence.

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

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.

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

2020 ◽  
Vol 2 (1) ◽  
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.

scholarly journals Update on the Diagnosis and Treatment of Idiopathic Intracranial Hypertension

2019 ◽  
Vol 39 (06) ◽  
pp. 682-691
Author(s):  
Sarah R. Ahmad ◽  
Heather E. Moss
Keyword(s):  
Clinical Trials ◽  
Intracranial Hypertension ◽  
Diagnostic Criteria ◽  
Surgical Management ◽  
Idiopathic Intracranial Hypertension ◽  
Diagnosis And Treatment ◽  
Evidence Based ◽  
Diagnosis And Management

AbstractAdvances in ophthalmic diagnostics and results of interventional clinical trials are shifting diagnosis and management of idiopathic intracranial hypertension (IIH) to be more technology- and evidence-based. In this article, the evidence supporting current diagnostic criteria, evaluation, and medical and surgical management of IIH are reviewed.

Unusual presentation of idiopathic intracranial hypertension

2020 ◽  
Vol 13 (5) ◽  
pp. e234741
Author(s):  
Ayman Alboudi ◽  
Emily C Johnson
Keyword(s):  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Cranial Nerve ◽  
Idiopathic Intracranial Hypertension ◽  
Cranial Nerves ◽  
Imaging Studies ◽  
Sixth Cranial Nerve ◽  
Orbital Pain ◽  
Atypical Presentations ◽  
Multiple Cranial Nerve

Idiopathic intracranial hypertension typically presents with holocephalic headache associated with nausea, vomiting and bilateral papilledema. Involvement of the sixth cranial nerve is relatively common. The involvement of other cranial nerves, however, is rare in this disorder. We describe a patient with idiopathic intracranial hypertension who presented with episodic unilateral retro-orbital pain and multiple cranial nerve abnormalities without papilledema. Imaging studies excluded alternate diagnoses, and the immediate resolution of symptoms after lumbar puncture confirmed that these symptoms were due to intracranial hypertension. Atypical presentations of such a disabling yet treatable disorder is very important to recognise and address.

Lumbar puncture rapidly improves olfaction in patients with idiopathic intracranial hypertension: A cohort study

Cephalalgia ◽  
2020 ◽  
Vol 40 (5) ◽  
pp. 429-436 ◽  
Author(s):  
Nils J Becker ◽  
Sören Enge ◽  
Katharina Maria Kreutz ◽  
Felix Schmidt ◽  
Lutz Harms ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Idiopathic Intracranial Hypertension ◽  
Fluid Pressure ◽  
Olfactory Function ◽  
Clinical Feature ◽  
Healthy Controls ◽  
Cerebrospinal Fluid Drainage ◽  
Olfactory Impairment

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.

scholarly journals Therapeutic lumbar puncture for headache in idiopathic intracranial hypertension: Minimal gain, is it worth the pain?

Cephalalgia ◽  
2018 ◽  
Vol 39 (2) ◽  
pp. 245-253 ◽  
Author(s):  
Andreas Yiangou ◽  
James Mitchell ◽  
Keira Annie Markey ◽  
William Scotton ◽  
Peter Nightingale ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Idiopathic Intracranial Hypertension ◽  
Rating Scale ◽  
Acute Effect ◽  
Numeric Rating Scale ◽  
Severe Exacerbation ◽  
Opening Pressure ◽  
Headache Severity ◽  
Numeric Rating

Background Headache is disabling and prevalent in idiopathic intracranial hypertension. Therapeutic lumbar punctures may be considered to manage headache. This study evaluated the acute effect of lumbar punctures on headache severity. Additionally, the effect of lumbar puncture pressure on post-lumbar puncture headache was evaluated. Methods Active idiopathic intracranial hypertension patients were prospectively recruited to a cohort study, lumbar puncture pressure and papilloedema grade were noted. Headache severity was recorded using a numeric rating scale (NRS) 0–10, pre-lumbar puncture and following lumbar puncture at 1, 4 and 6 hours and daily for 7 days. Results Fifty two patients were recruited (mean lumbar puncture opening pressure 32 (28–37 cmCSF). At any point in the week post-lumbar puncture, headache severity improved in 71% (but a small reduction of −1.1 ± 2.6 numeric rating scale) and exacerbated in 64%, with 30% experiencing a severe exacerbation ≥ 4 numeric rating scale. Therapeutic lumbar punctures are typically considered in idiopathic intracranial hypertension patients with severe headaches (numeric rating scale ≥ 7). In this cohort, the likelihood of improvement was 92% (a modest reduction of headache pain by −3.0 ± 2.8 numeric rating scale, p = 0.012, day 7), while 33% deteriorated. Idiopathic intracranial hypertension patients with mild (numeric rating scale 1–3) or no headache (on the day of lumbar puncture, prior to lumbar puncture) had a high risk of post- lumbar puncture headache exacerbation (81% and 67% respectively). Importantly, there was no relationship between lumbar puncture opening pressure and headache response after lumbar puncture. Conclusion Following lumbar puncture, the majority of idiopathic intracranial hypertension patients experience some improvement, but the benefit is small and post-lumbar puncture headache exacerbation is common, and in some prolonged and severe. Lumbar puncture pressure does not influence the post-lumbar puncture headache.

Errors in prostate core biopsy diagnosis in an era of specialisation and double reporting

2020 ◽  
pp. jclinpath-2020-206726
Author(s):  
Cornelia Margaret Szecsei ◽  
Jon D Oxley
Keyword(s):  
Error Rate ◽  
False Positive ◽  
Core Biopsy ◽  
False Negative ◽  
Diagnostic Error ◽  
Error Rates ◽  
False Positive Error ◽  
Biopsy Diagnosis ◽  
False Negative Error ◽  
False Positive Error Rate

AimTo examine the effects of specialist reporting on error rates in prostate core biopsy diagnosis.MethodBiopsies were reported by eight specialist uropathologists over 3 years. New cancer diagnoses were double-reported and all biopsies were reviewed for the multidisciplinary team (MDT) meeting. Diagnostic alterations were recorded in supplementary reports and error rates were compared with a decade previously.Results2600 biopsies were reported. 64.1% contained adenocarcinoma, a 19.7% increase. The false-positive error rate had reduced from 0.4% to 0.06%. The false-negative error rate had increased from 1.5% to 1.8%, but represented fewer absolute errors due to increased cancer incidence.ConclusionsSpecialisation and double-reporting have reduced false-positive errors. MDT review of negative cores continues to identify a very low number of false-negative errors. Our data represents a ‘gold standard’ for prostate biopsy diagnostic error rates. Increased use of MRI-targeted biopsies may alter error rates and their future clinical significance.

Infantile Idiopathic Intracranial Hypertension: A Case Study and Review of the Literature

2019 ◽  
Vol 34 (13) ◽  
pp. 806-814 ◽  
Author(s):  
Sama Boles ◽  
Claudia Martinez-Rios ◽  
Daniel Tibussek ◽  
Daniela Pohl
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Idiopathic Intracranial Hypertension ◽  
Treatment Guidelines ◽  
Fluid Pressure ◽  
Specific Treatment ◽  
Unknown Etiology ◽  
Age Group ◽  
Review Of The Literature

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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