Optical coherence tomography for the diagnosis and monitoring of idiopathic intracranial hypertension

The objectives of the study were to investigate the value of optical coherence tomography in detecting papilledema in patients with idiopathic intracranial hypertension (IIH), a disease which is difficult to monitor and which can lead to permanent visual deficits; to analyze retinal changes over time. In this non-interventional case–control study, spectral-domain optical coherence tomography (SD-OCT) was used to analyze the retinal and optic nerve head (ONH) morphology of 21 patients with IIH and 27 age- and sex-matched healthy controls over time. We analyzed the ONH volume using a custom-made algorithm and employed semi-automated segmentation of macular volume scans to assess the macular retinal nerve fiber layer (RNFL) and ganglion cell layer and inner plexiform layer complex as well as the total macular volume. In IIH patients, the ONH volume was increased and correlated with cerebrospinal fluid (CSF) pressure. The ONH volume decreased after the initiation of treatment with acetazolamide. The macular RNFL volume decreased by 5% in 3.5 months, and a stepwise multivariate regression analysis identified CSF pressure as the main influence on macular RNFL volume at diagnosis. The only factor predicting macular RNFL volume loss over time was ONH volume. SD-OCT can non-invasively monitor changes in retinal and ONH morphology in patients with IIH. Increased ONH volume leads to retinal atrophy in the form of macular RNFL volume loss, presumably due to mechanic jamming of the optic nerve at the disc and subsequent axonal loss.     

A case series of dural venous sinus stenting in idiopathic intracranial hypertension: association of outcomes with optical coherence tomography1

Purpose/Aim: Pseudotumor cerebri or idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure of unknown etiology. A subset of patients has shown benefit from endovascular dural venous sinus stenting (DVSS). We sought to identify a population of IIH patients who underwent DVSS to assess outcomes. Materials and Methods: A retrospective study was performed to identify IIH patients with dural sinus stenosis treated with DVSS. Outcome measures included dural sinus pressure gradients, peripapillary retinal nerve fiber layer (RNFL) thickness using optical coherence tomography and improvement in symptoms. Results: Seventeen patients underwent DVSS. Average pre- and post-intervention pressure gradients were 23.06 and 1.18 mmHg, respectively (p < 0.0001). Sixteen (94%) noted improvement in headache, fourteen (82%) had visual improvement and all (100%) patients had improved main symptom. Of 11 patients with optical coherence tomography, 8 showed decreased RNFL thickness and 3 remained stable; furthermore, these 11 patients had improved vision with improved papilledema in 8, lack of pre-existing papilledema in 2 and stable, mild edema in 1 patient. Conclusions: Our series of patients with dural sinus stenosis demonstrated improvement in vision and reduction in RNFL thickness. DVSS appears to be a useful treatment for IIH patients with dural sinus stenosis.     

Idiopathic intracranial hypertension from the perspective of headache center

Idiopathic intracranial hypertension (IIH) is a pathological state defined as an increase of intracranial pressure in the absence of a causative pathological process. The aim of this study was to evaluate the clinical features of the patients with IIH diagnosed in our Headache Center according to the current knowledge of this disorder. In the retrospective and cross-sectional analysis of 3395 patients we present 12 newly diagnosed IIH patients, ten women and two men, aged from 19 to 51, with obtained values of cerebrospinal fluid pressure between 250 and 680 mm of water. The symptoms of IIH clinical presentation have been headache, reported by 92 % of patients; papilledema, noted in 67 %; and cranial nerve impairment (25 %). The results obtained from presented patients confirmed the presence of headache features that are included in criteria for headache attributed with IIH in majority of them: progressive, daily, diffuse, non-pulsatile headache with aggravation by coughing or straining. Decrease of pain intensity after lumbar puncture was noted in all patients. We notice the relatively small proportion of patients with headache attributed to IIH among the patients treated in our Headache Center. The prevalence of IIH is not low and headache is the most frequent presenting symptom; therefore, we could only conclude that some chronic headache patients refractory for treatment are patients with IIH.     

Disease activity in idiopathic intracranial hypertension: a 3-month follow-up study

Idiopathic intracranial hypertension (IIH) is a disorder of raised intracranial pressure (ICP) in the absence of identifiable pathology. The purpose of this study was to evaluate the clinical presentation and monitor a 3-month course using frequent optical coherence tomography (OCT) evaluations, visual field testings and lumbar opening pressure measurements. A longitudinal study of 17 patients with newly diagnosed IIH and 20 healthy overweight controls were included in the study. Peripapillary retinal nerve fiber layer thickness (RNFLT) and retinal thickness (RT) measurements (Stratus OCT-3, fast RNFL 3.4 protocol), and Humphrey visual field testing were evaluated at regular intervals. Repeat lumbar puncture was performed at final visit (n = 13). The diagnostic delay was 3 months and initial symptoms were headache (94%), visual blurring (82%) and pulsatile tinnitus (65%). Complete clinical remission was achieved in 65%, partial in 29% and unchanged symptoms in 6%. Total average RNFLT and RT decreased significantly during the follow-up period (p < 0.0001 and p < 0.0001, respectively). Changes in RNFLT and RT correlated with improvements in visual field mean deviation (MD) (RNFLT: p = 0.006; RT: p = 0.03) and pattern standard deviation (PSD) (RNFLT: p = 0.002; RT: p = 0.003). In patients with weight-loss >3.5% of BMI, ICP decreased significantly (p = 0.0003). In patients with weight-loss <3.5% of BMI, changes in ICP were insignificant (p = 0.6). OCT combined with visual field testing may be a valuable objective tool to monitor IIH patients and the short term IIH outcome is positive. Weight-loss is the main predictor of a favorable outcome with respect to CSF pressure.     

Retinal nerve fiber layer axonal loss and visual dysfunction in optic neuritis

Axonal loss is thought to be a likely cause of persistent disability after a multiple sclerosis relapse; therefore, noninvasive in vivo markers specific for axonal loss are needed. We used optic neuritis as a model of multiple sclerosis relapse to quantify axonal loss of the retinal nerve fiber layer (RNFL) and secondary retinal ganglion cell loss in the macula with optical coherence tomography. We studied 25 patients who had a previous single episode of optic neuritis with a recruitment bias to those with incomplete recovery and 15 control subjects. Optical coherence tomography measurement of RNFL thickness and macular volume, quantitative visual testing, and electrophysiological examination were performed. There were highly significant reductions (p < 0.001) of RNFL thickness and macular volume in affected patient eyes compared with control eyes and clinically unaffected fellow eyes. There were significant relationships among RNFL thickness and visual acuity, visual field, color vision, and visual-evoked potential amplitude. This study has demonstrated functionally relevant changes indicative of axonal loss and retinal ganglion cell loss in the RNFL and macula, respectively, after optic neuritis. This noninvasive RNFL imaging technique could be used in trials of experimental treatments that aim to protect optic nerves from axonal loss.     

Complete ophthalmoplegia: A rare presentation of idiopathic intracranial hypertension

Idiopathic intracranial hypertension (IIH) is a disorder defined by clinical criteria that include signs and symptoms isolated to those produced by increased intracranial pressure (ICP; e. g., headache, papilledema, and vision loss), elevated ICP with normal cerebrospinal fluid (CSF) composition, and no other cause of intracranial hypertension evident on neuroimaging or other evaluations. The most common signs in IIH are papilledema, visual field loss, and unilateral or bilateral sixth cranial nerve palsy. Here we report a case of IIH presenting as headache with vision loss, papilledema, complete ophthalmoplegia with proptosis in one eye, and sixth cranial nerve palsy in the other eye. Patient was managed with acetazolamide, topiramate, and diuretics. Symptoms remained static and she was planned for urgent CSF diversion procedure.     

Sonographic assessment of the optic nerve and the central retinal artery in idiopathic intracranial hypertension

PurposeTransorbital sonography easily detects papilledema and enlarged optic nerve sheath diameters (ONSD) in IIH (idiopathic intracranial hypertension) patients. As the central retinal artery is located within the optic nerve, its hemodynamic properties might be affected by the increased pressure. In this study we assessed the diagnostic usefulness of transorbital sonography in IIH with a special focus on color Doppler imaging of the central retinal artery. IIH patients presented papilledema and enlarged ONSD. ONSD accurately predicted an increased intracranial pressure in IIH (cut-off: 5.8 mm, 81% sensitivity, 80% specificity). 24 h following therapeutic lumbar puncture ONSD diminished significantly, whereas papilledema was not changed. PSV (peak systolic velocity) and Vmean (mean flow velocity) of the central retinal artery were increased in IIH patients compared to controls. PSV accurately predicted an increase of intracranial pressure (cut-off: 11.0 cm/s, 70% sensitivity, 69% specificity). PI (pulsatility index), PSV and Vmean decreased following lumbar puncture. PSV and Vmean decreases were statistically significant for right eyes only in which the values changed to normal. In summary, besides ONSD enlargement and papilledema transbulbar sonography demonstrated an alteration of central retinal artery blood flow in IIH patients. Especially PSV might serve as valuable surrogate marker for intracranial pressure in IIH. Furthermore, the change of intra-individual central retinal arteries PI might be a valuable parameter to demonstrate response to lumbar puncture in IIH patients.     

Clinical characterization of idiopathic intracranial hypertension at the Detroit Medical Center

BACKGROUND: Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri (PTC), is a condition characterized by increased intracranial pressure without clinical, laboratory or radiologic evidence of an intracranial space-occupying lesion, meningeal inflammation or venous outflow obstruction. Previous population-based studies evaluated the presenting clinical features of IIH in North-Eastern Libya, Iowa, Louisiana, Minnesota, Northern Ireland, Israel and Western Turkey. However, we report the demographic patterns of a metropolitan North American city. METHODS: We performed a retrospective study of all patients with an initial IIH diagnosis and follow-up at the Detroit Medical Center (DMC), Michigan from 1985 to 2002. Medical records of 174 patients were reviewed, with 77 patients fitting the diagnostic criteria. RESULTS: Seventy-seven patients, 71 females (92.2%) and 6 males (7.8%) were identified. Age ranged from 10 to 50 years, with a mean of 34 years. Fifty patients (65%) were African-American and 24 patients (31%) were Caucasian. The most common presentation was isolated headache (28.6%). Nineteen patients (24.7%) were asymptomatic. Obesity was described in 65 of 74 patients (87.8%) evaluated for body dimensions. CONCLUSIONS: We report an increased number of asymptomatic patients after routine ophthalmic examination. Obese females in metropolitan Detroit would benefit from routine eye evaluations to avoid potential visual loss related to papilledema.     

Idiopathic intracranial hypertension (Pseudotumor cerebri)

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disorder of elevated intracranial pressure of unknown cause. Patients present with daily headache, pulse-synchronous tinnitus, transient visual obscurations, papilledema with its associated visual loss, and diplopia from sixth nerve paresis. Many disease associations have been alleged, but few besides obesity, hypervitaminosis A and related compounds, steroid withdrawal, and female gender have been proven. Although absorption of cerebrospinal fluid (CSF) occurs through arachnoid granulations and extracranial lymphatics, outflow resistance is increased in IIH; therefore, intracranial pressure must increase for CSF to be absorbed. The mainstays of medical treatment are a reduced-sodium weight-reduction program and acetazolamide. If patients fail medical therapy, surgical procedures, most commonly optic nerve sheath fenestration and CSF shunting, are employed. The main morbidity of IIH is visual loss. This is present in most patients and can usually be reversed if recognized early in the course of the disease and treated. Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disorder of elevated intracranial pressure of unknown cause. Patients present with daily headache, pulse-synchronous tinnitus, transient visual obscurations, papilledema with its associated visual loss, and diplopia from sixth nerve paresis. Many disease associations have been alleged, but few besides obesity, hypervitaminosis A and related compounds, steroid withdrawal, and female gender have been proven. Although absorption of cerebrospinal fluid (CSF) occurs through arachnoid granulations and extracranial lymphatics, outflow resistance is increased in IIH; therefore, intracranial pressure must increase for CSF to be absorbed. The mainstays of medical treatment are a reduced-sodium weight-reduction program and acetazolamide. If patients fail medical therapy, surgical procedures, most commonly optic nerve sheath fenestration and CSF shunting, are employed. The main morbidity of IIH is visual loss. This is present in most patients and can usually be reversed if recognized early in the course of the disease and treated.     

Retinal nerve fiber layer analysis in idiopathic intracranial hypertension

BACKGROUND: The chronic nature of idiopathic intracranial hypertension (IIH) represents a risk factor for progressive optic nerve damage and structural abnormalities of the retina. AIM: We measured the retinal nerve fiber layer (RNFL) thickness in patients followed with the diagnosis of IIH who had no or mild visual impairment to search for possible structural alterations in the retina for diagnostic and prognostic purposes. SETTINGS AND DESIGN: Case-control prospective study. MATERIALS AND METHODS: The study group consisted of 12 women followed and treated with the diagnosis of IIH in our clinic. The selection criteria were the, normal optic nerve, normal visual fields or mild visual field defects (Grade 1-3) by Humphrey perimeter. Randomly assigned, age-matched 12 healthy women were taken as the control group. Retinal nerve fiber layer thickness was evaluated with scanning laser polarimetry and both eyes were studied for each case in both groups. STATISTICAL ANALYSIS USED: Mann-Whitney U test. RESULTS: The mean ages of the patient and the control groups were 34.58+/-4.2 and 34.42+/-5.7 years respectively (P=0.87). The mean duration of disease was 5.5+/-3 years. Some parameters related to RNFL thickness were found to differ significantly between patients with IIH and control subjects. Namely superior ratio (P=0.007), inferior ratio (P=0.039), superior-nasal ratio (P=0.025), maximum modulation (P=0.01) and symmetry (P=0.006) were lower in the patient group than controls. CONCLUSION: Scanning laser polarimetry might be a good adjunct for determining possible structural affects of IIH on the retina in patients with no or mild visual impairment.     

Papilledema in obstructive hydrocephalus caused by giant cell astrocytoma of tuberous sclerosis.

A 5-year-old girl with progressive hemiparesis and headache was found by brain imaging to have a large tumor centered at the foramen of Monro, blocking cerebrospinal outflow and producing massive lateral ventriculomegaly. Total excision of the mass led to a pathologic diagnosis of giant cell astrocytoma. Dermatologic abnormalities had been detected shortly after birth but were unexplained. Abdominal imaging disclosed renal cysts, and ophthalmologic examination disclosed papilledema and retinal plaques. On this basis, a diagnosis of tuberous sclerosis (TS) was finally made. Two months after surgery, papilledema had resolved, and visual function appeared to be normal. Although the patient apparently escaped visual loss, other reports affirm that giant cell astrocytoma, a common tumor in TS, may go undetected for long enough to produce irreversible optic neuropathy from chronic papilledema. Because patients with TS may not report visual loss, they should undergo periodic ophthalmologic screening.     

Perfusion and diffusion magnetic resonance imaging in idiopathic intracranial hypertension

OBJECTIVES: Idiopathic intracranial hypertension (IIH) is characterized by abnormal elevation of intracranial pressure without any underlying etiologic factor. Papilledema is the major clinical finding whereas neuroradiological imaging findings are almost always normal. The aim of this preliminary study was to determine if diffusion and perfusion magnetic resonance imaging in patients with IIH might be beneficial in the management of the disease. MATERIALS AND METHODS: Prospectively, we evaluated standard magnetic resonance, magnetic resonance angiographies and venographies, diffusion and perfusion magnetic resonance findings of 16 patients with IIH and of 16 age-, sex-, and weight-matched normal individuals as a control group. Patients with IIH underwent a detailed neuroophthalmologic examination and lumbar puncture for evaluation of cerebrospinal fluid pressure. Magnetic resonance imaging was performed with 1.5 T equipment. RESULTS: On physical examination, all patients had characteristic papilledema, varying degrees of headache, blurred vision and tinnitus. Cerebrospinal fluid pressure was higher than 250 mm H2O in all patients. A statistically significant decrease in cerebral blood flow in six patients, whereas insignificant increase in two were detected. Cerebral blood volume values were almost similar to normal control group's values. Significant mean transit time prolongation was found in six patients as well. CONCLUSIONS: Idiopathic intracranial hypertension is a clinical syndrome which requires prompt diagnosis and a thorough evaluation. Treatment is crucial for preventing visual loss and improving associated symptoms. It is also important to detect cerebral perfusion changes, as cerebrovascular complications may be associated. Although our patient group is small for statistical evaluation, it is a preliminary study using perfusion and diffusion magnetic resonance which may contribute to IIH management.     

Idiopathic intracranial hypertension in Dubai: nature and prognosis

OBJECTIVE: To evaluate the nature and course of idiopathic intracranial hypertension (IIH) in Dubai, UAE. METHODS: In a retrospective study the medical records of 50 patients with IIH were reviewed. All of them were diagnosed according to the Modified Dandy Criteria. RESULTS: There were 46 (92%) women. Mean age at presentation was 35.7 years. Obesity was the commonest associated factor (32%). Headache was reported in 98% followed by double vision (32%). Papilledema was present in all patients at the time of examination (100%). Perimetric study showed mild peripheral visual field constriction in 56%. Only two patients showed severe field constriction and one of them deteriorated rapidly and she became blind. The mean cerebrospinal fluid pressure was 302.5 mmH(2)O. The visual status improved significantly throughout the follow-up period. CONCLUSIONS: Idiopathic intracranial hypertension has relatively benign course in this part of the world and more aggressive treatment is not recommended.     

Headache diagnoses in patients with treated idiopathic intracranial hypertension

The authors reviewed medical records of 82 patients with idiopathic intracranial hypertension (IIH) to determine the frequency of headaches occurring after initial diagnosis and treatment of IIH, classifiable by the International Headache Society guidelines. Sixty-eight percent of patients had definable headache disorders, including episodic tension type headache (30%) and migraine without aura (20%). Patients with IIH frequently have other types of headaches, not necessarily related to increased intracranial pressure.     

Headache in patients with idiopathic intracranial hypertension: a pilot study to assess applicability of ICHD-2 diagnostic criteria

Headache is one of the most common symptoms of idiopathic intracranial hypertension (IIH). The aim of this study was to investigate the applicability of the diagnostic criteria for "Headache attributed to IIH" included in the current classification of headache disorders, particularly as far as the main headache features. A consecutive clinical series of IIH patients with demonstration of increased intracranial pressure by lumbar puncture in the recumbent position were enrolled. Among a total of 22 patients, headache was reported by 14. The proportion of patients reporting the main headache features required by diagnostic criteria were: 93 % for daily or nearly-daily occurrence; 71.5 % for diffuse/non-pulsating pain; 57 % for aggravation by coughing/straining. Thus, these three headache features, at least one of which is required for diagnosis of headache attributed to IIH, were present in the vast majority of our sample, suggesting that their inclusion should be regarded as appropriate. The analysis of our results may suggest possible changes in the current ICDH-2 criteria for headache attributed to IIH, based on the following considerations: the existence of remarkable differences as far as the relative frequency of each headache feature; the fact that diffuse and non-pulsating pain-included in the current classification as a single requirement-were not always found together; the high frequency of migrainous associated symptoms (nausea or photophobia-phonophobia were present in 71.5 % cases).     

Headache in transient or permanent cerebral ischemia. Dutch TIA Study Group

We studied headache features in 3,126 patients with acute cerebral or retinal ischemia. Headache occurred in 18% of these patients (in 16% of all patients with transient ischemic attacks, in 18% of patients with reversible ischemic neurologic deficits, and in 19% of patients with minor strokes) and was mostly continuous in all types of attacks. Headache was present in 16% of patients with monocular visual symptoms. The occurrence of headache was not related to the mode of onset, mode of disappearance, or duration of the attack. Patients with headache more often were known to have heart disease. Headache was less frequent in patients with small deep infarcts, who were more often hypertensive, and in patients with infarcts in the anterior circulation; headache was more frequent in patients with cortical infarcts and in patients with infarcts in the posterior circulation. Patients with a relevant small deep infarct on computed tomographic scan and accompanying headache relatively often reported symptoms compatible with cortical ischemia, such as language disorders or a visual field defect. We conclude that headache is a frequent accompanying symptom in patients with acute cerebral and retinal ischemia and that the occurrence of headache is partly related to the underlying cause of the ischemic lesion.     

Effect of changes in optic nerve elasticity on central retinal artery blood flow in patients with idiopathic intracranial hypertension

PurposeTo assess changes in central retinal artery (CRA) blood flow by orbital color-coded Doppler ultrasonography in patients with idiopathic intracranial hypertension (IIH) and their relation with optic nerve (ON) elasticity assessed by shear wave elastography (SWE).MethodsThis study was carried out on 68 eyes of patients diagnosed with IIH and 32 eyes of healthy controls. The severity of papilledema in IIH patients was sub-classified into mild and moderate/severe groups. Color-coded Doppler was used to measure peak systolic velocity (PSV), end diastolic velocity (EDV), mean velocity (V_mean) and pulsatility index (PI) of the CRA.ResultsPSV, V_mean, and SWE were significantly higher in patients with IIH than in controls (p = 0.001). The optimal cut-off values of PSV and V_mean for differentiating IIH patients from controls were 11.25 and 6.75 cm/s with AUC 0.81 and 0.785 respectively. AUC was 0.92 and accuracy 91% for combined PSV, V_mean and SWE differentiation between IIH patients and controls. PSV, V_mean and SWE were significantly different between mild versus moderate/severe papilledema (p = 0.001). PSV and V_mean were correlated with papilledema (r = 0.790 and 0.722 respectively) and SWE (r = 0.818 and 0.761 respectively).ConclusionIIH is associated with decreased ON elasticity and reduced CRA blood flow. Individual and combined color-coded Doppler of the CRA and SWE help in diagnosis of IIH. CRA hemodynamic changes are correlated with papilledema severity and with the extent of biomechanical changes in the ON represented by SWE.     

Static and pulsatile intracranial pressure in idiopathic intracranial hypertension

Objective

The aim of this observational study was to characterize the static and pulsatile intracranial pressure (ICP) in conservatively (medically) treated idiopathic intracranial hypertension (IIH) patients in need of shunt surgery, and also in patients with chronic daily headache (CDH) without visual disturbances.

Methods

The material includes 14 IIH patients and 7 CDH patients in whom ICP was monitored continuously over-night. Static ICP was characterized by mean ICP, pulsatile ICP was characterized by the wave amplitude, rise time, and rise time coefficient.

Results

In the IIH group all 14 had headache and visual disturbances. Mean ICP was high (>15 mmHg) in only 7 patients (50%), while mean ICP wave amplitude was high (≥4 mmHg) in all 14 (100%). All IIH patients were shunted and improved clinically thereafter (i.e., relief from visual disturbances and/or headache). None in the CDH group had high mean ICP or mean ICP wave amplitude, and none were shunted.

Conclusions

In this cohort of 14 conservatively treated IIH patients with lasting and shunt-responsive headache and visual disturbances, the mean ICP wave amplitude was elevated (≥4 mmHg) in all patients despite normal mean ICP (<15 mmHg) in 7 patients (all but one on medication). Therefore, the pulsatile ICP may be more relevant than the static ICP in the diagnostic setting for patients with IIH. Further prospective standardized approaches are warranted.     

立体定向手术治疗海洛因依赖的远期随访分析

目的评估立体定向毁损术治疗海洛因依赖的手术疗效,分析术后复吸原因。方法回顾性分析接受脑立体定向术的154例海洛因成瘾者的临床资料,采用症状自评量表对病人精神卫生状况进行调查随访。结果随访154例,时间5~6年,其中复吸52例(33.8%,复吸组),未复吸102例(66.2%,未复吸组)。复吸组术后躯体化、强迫症状、敌对、偏执、精神病性等因子明显高于未复吸组和国内常模(均P<0.05)。结论立体定向手术是海洛因依赖的安全有效的治疗方法。强迫症状和人格障碍的改善是消除心理依赖的基础及手术戒毒治疗成功的重要因素;精神病性和躯体化是复吸的主要原因。