Occipital emissary vein existence and its impact on the diagnosis of idiopathic intracranial hypertension in pediatric patients.

PURPOSE: This study aimed to investigate the efficacy of occipital emissary vein (OEV) detection in the diagnosis of idiopathic intracranial hypertension (IHH) in the pediatric age group, and to compare the prevalence and luminal diameter of OEV in patients with IHH and in healthy control subjects. METHODS: Conventional magnetic resonance imaging findings were assessed in the patients with IHH and in healthy control subjects who were under the age of 18, by two observers. The presence and luminal dimension of OEV and transverse sinus stenosis were also evaluated and compared between these two groups with magnetic resonance venography techniques. RESULTS: The rate of OEV existence was 7 times higher in the IIH group compared to the control group based on the second observer outcome (p = 0.010, OR = 7.0), with a very good interobserver agreement (Ƙ = 0.85). The dimension of OEV ranged between 0.6 and 2.5 mm. There was no correlation found between the opening pressure and the dimension of OEV (p = 0.834). CONCLUSION: In conclusion, OEV existence could be an additional radiological finding for diagnosing IHH among pediatric patients, alongside other conventional findings.

Risk factors associated with progression from papilloedema to optic atrophy: results from a cohort of 113 patients.

BACKGROUND: The aim of this study was to assess the risk factors for atrophic progression of patients with papilloedema secondary to intracranial hypertension, using optical coherence tomography parameters. METHODS: A retrospective study was conducted at Marseille University Hospitals' Ophthalmology departments between December 2015 and December 2021. All patients with papilloedema resulting from elevated intracranial hypertension at the initial presentation were included. Ophthalmological evaluations included analysing retinal nerve fibre layer (RNFL), ganglion cell layer (GCL) and total peripapillary retinal thickness (RT). RESULTS: The study included 222 eyes from 113 patients. The main aetiologies of intracranial hypertension were idiopathic intracranial hypertension (49/113), intracranial tumours (33/113) and cerebral venous thrombosis (15/113). The initial RNFL and RT showed significant correlations with optic atrophy. The mean RNFL was 199.63 µm in the 'no atrophy' group and 365.28 µm in the 'atrophy' group (p<0.001). Similarly, the mean RT was 483.72 µm in the 'non-atrophy' group and 796.69 µm in the 'atrophy' group (p<0.001). The presence of peripapillary haemorrhages showed a strong correlated with optic atrophy with an OR=19.12 (p<0.001). Impaired initial visual acuity was also associated with final optic atrophy with an OR=7.76 (p=0.020). Furthermore, impaired initial GCL was a major predictor of optic atrophy (OR=18.25 (p=0.021)). CONCLUSION: Our study highlights the risk factors for optic atrophy in papilloedema, aiming to facilitate the early detection of patients at a high risk of vision loss and enable more aggressive medical or surgical management.

How to understand an enlarged Meckel's cave? An anatomical study.

PURPOSE: Dilatation of the trigeminal cavum, or Meckel's cave (MC), is usually considered a radiological sign of idiopathic intracranial hypertension. However, the normal size of the trigeminal cavum is poorly characterized. In this study, we describe the anatomy of this meningeal structure. METHODS: We dissected 18 MCs and measured the length and width of the arachnoid web and its extension along the trigeminal nerve. RESULTS: Arachnoid cysts were clearly attached to the ophthalmic (V1) and maxillary (V2) branches until they entered the cavernous sinus and foramen rotundum, respectively, without extension to the skull base. Arachnoid cysts were close to the mandibular branch toward the foramen ovale, with a median anteromedial extension of 2.5 [2.0-3.0] mm, lateral extension of 4.5 [3.0-6.0] mm, and posterior extension of 4.0 [3.2-6.0] mm. The trigeminal cavum arachnoid had a total width of 20.0 [17.5-25.0] mm and length of 24.5 [22.5-29.0] mm. CONCLUSION: Our anatomical study revealed variable arachnoid extension, which may explain the variability in size of the trigeminal cavum in images and calls into question the value of this structure as a sign of idiopathic intracranial hypertension. The arachnoid web extends beyond the limits described previously, reaching almost double the radiological size of the cavum, particularly at the level of V3 afference of the trigeminal nerve. It is possible that strong adhesion of the arachnoid to the nerve elements prevents the formation of a true subarachnoid space that can be visualized by magnetic resonance imaging.

Transorbital sonography and MRI reliability to assess optic nerve sheath diameter in idiopathic intracranial hypertension.

BACKGROUND AND PURPOSE: The purpose of this study was to evaluate the performance of magnetic resonance imaging (MRI) in measuring the optic nerve sheath diameter (ONSD) compared to the established method transorbital sonography (TOS) in patients with idiopathic intracranial hypertension (IIH). METHODS: Twenty-three patients with IIH were prospectively included applying IIH diagnostic criteria. All patients received a lumbar puncture with assessment of the cerebrospinal fluid (CSF) opening pressure to assure the IIH diagnosis. Measurement of ONSD was performed 3 mm posterior to inner sclera surface in B-TOS by an expert examiner, while three independent neuroradiologists took measurements in axial T-weighted MRI examinations. The sella turcica with the pituitary gland (and potential presence of an empty sella) and the trigeminal cavity were also assessed on sagittal and transversal T1-weighted MRI images by one independent neuroradiologist. RESULTS: The means of ONSD between ultrasound and MRI measurements were 6.3 mm (standard deviation [SD] = 0.6 mm) and 6.2 mm (SD = 0.8 mm). The interrater reliability between three neuroradiologists showed a high interclass correlation coefficient (ICC) (confidence interval: .573 < ICC < .8; p < .001). In patients with an empty sella, the ONSD evaluated by MRI was 6.6 mm, while measuring 6.1 mm in patients without empty sella. No correlation between CSF opening pressure and ONSD was found. CONCLUSIONS: MRI can reliably measure ONSD and yields similar results compared to TOS in patients with IIH. Moreover, patients with empty sella showed significantly larger ONSD than patients without empty sella.

Magnetic Resonance Imaging Findings in Idiopathic Intracranial Hypertension With and Without Pulsatile Tinnitus: An Age-Matched Cohort Study.

OBJECTIVE: Many but not all patients with idiopathic intracranial hypertension (IIH) have pulsatile tinnitus (PT). However, little is known about why some patients with IIH develop PT and others do not. The purpose of this study was to determine if any of the classic magnetic resonance imaging (MRI)-detectable markers of IIH differ between patients with and without PT, thereby shedding light on potential pathophysiology. METHODS: A retrospective age-matched cohort study of patients with documented IIH (diagnosed by neuro-ophthalmologist) was performed. All patients had MRI performed around the time of diagnosis. MRIs were assessed for 16 variables known to be associated with IIH (e.g., pituitary displacement/empty sella, optic nerve tortuosity, transverse sinus stenosis, inferior cerebellar tonsils, arachnoid granulations, slit-like ventricles) by two blinded neuroradiologists. All binary variables were analyzed via χ2 test with Yates correction, or Fisher exact when appropriate. Continuous variables were analyzed via Student t test. Inter-rater reliability for binary variables was assessed by Cohen κ . For continuous variables, intraclass correlation coefficient was calculated. RESULTS: Forty age-matched patients with IIH met the inclusion criteria (20 with PT, 20 without PT). For all known binary MRI findings associated with IIH, there were no statistically significant differences between groups. Likewise, there were no statistically significant differences for continuous variables. CONCLUSIONS: The classic MRI findings associated with IIH do not differ between patients with and without PT, suggesting that systemic (rather than localized intrinsic or extrinsic) factors may play a critical role in the pathophysiology.

Accuracy and reliability of magnetic resonance imaging in the diagnosis of idiopathic intracranial hypertension.

PURPOSE: To determine the diagnostic utility of brain magnetic resonance imaging (MRI) findings in patients with idiopathic intracranial hypertension (IIH) and to investigate the significance of evaluating radiological findings together with neurological and ophthalmological data in the diagnosis of IIH. MATERIALS AND METHODS: All consecutive patients diagnosed with IIH in our tertiary neuro-ophthalmology center between January 1, 2018 and March 15, 2020, were included in the study. The clinical, radiological, and ophthalmological findings of IIH patients were compared with the control group with similar demographic characteristics. RESULTS: A total of 98 patients, 49 cases and 49 controls, were included in the study. Lateral ventricular index had the highest area under the curve (AUC) value (0.945) for prediction of disease group followed by sella height category (AUC = 0.915) and optic nerve tortuosity (AUC = 0.855) According to the multivariate model we developed, caudate index (OR = 0.572, 95% CI 0.329-0.996), lateral ventricle index (OR = 3.969, 95% CI 1.851-8.509) and bilateral optic nerve tortuosity (OR = 22,784, 95% CI 2.432-213.450) were significant predictors for disease group. CONCLUSION: Tortuosity in the optic nerve, lateral ventricular index and caudate index can be used as MRI parameters supporting the diagnosis of IIH in clinically suspicious cases. A holistic approach to the clinical and radiological findings of the cases in the diagnosis of IIH can prevent overdiagnosis and enable early correct diagnosis.

Dural Venous Sinus Arachnoid Granulations in Patients with Pulsatile Tinnitus.

OBJECTIVE: To determine the relationship, if any, between dural venous sinus arachnoid granulations (AGs) and pulsatile tinnitus. STUDY DESIGN: Retrospective case-control study. METHODS: Between October 1999 and March 2020, magnetic resonance imaging of patients with tinnitus (pulsatile [PT] and nonpulsatile [NPT]) were assessed for the presence of dural venous sinuses AG. During the same interval, patients with AGs found incidentally on all magnetic resonance imagings ordered without an indication of tinnitus were reviewed. Demographic variables recorded included patient age, sex, race, body mass index, and a history of idiopathic intracranial hypertension (IIH) or obstructive sleep apnea. Location of AGs, when present, were recorded. RESULTS: A total of 651 (PT 250, NPT 401) were found to have AGs. AGs had a higher prevalence in PT patients (10.4% [n = 26]) versus NPT patients (0.3% [n = 1]; odds ratio, 31.0; confidence interval 4.1-234; p < 0.001). Of the 77,607 patients who had an indication for imaging other than tinnitus, 230 patients (0.30%) were found to have incidental AGs, suggesting that the NPT cohort was an adequate control. Patients with PT were more likely to have a higher body mass index, be female, be non-White, and have an existing diagnosis of IIH. For all patients with AGs, AGs were more likely to be found in the lateral sinuses (i.e., sigmoid, transverse) in the PT group (odds ratio, 8.1; confidence interval, 1.1-61.1; p = 0.0218). CONCLUSIONS: This study evaluates the association between AG and PT, finding higher rates of AG in patients with PT than in NPT. However, despite the increased prevalence of AG in patients with IIH, these data combined with existing literature would suggest that AGs are not necessarily the missing link to explain PT pathophysiology in IIH.

Evaluation of Clinical Findings with MRI Venography in Patients with Idiopatic Intracranial Hypertension.

OBJECTIVES: To evaluate clinical data and Magnetic Resonance Venography (MRV) findings together in patients with idiopathic intracranial hypertension (IIH). MATERIALS AND METHODS: In this retrospective study between January 2009 and February 2021, conventional MRI and MRV examinations were performed on 30 patients who were admitted to the neurology service of Erciyes University Medical Faculty with the pre-diagnosis of IIH, cerebrospinal fluid (CSF) pressure was measured in these patients to confirm the diagnosis of IIH. Transverse Sinus Stenosis Ratio (TS SR), Superior Sagittal Sinus (SSS) diameter, Sinus Rectus (SR) diameter, Stenosis Segment Length (SSL) were studied. RESULTS: High CSF pressure was detected in 22 of 30 patients with IIH pre-diagnosis. CSF pressure was normal in 8 cases. TS SR was compared in all groups, right TS SR 0.63 ± 0.16, Left TS SR 0.55 ± 0.16 in the patient group with IIH, right TS SR 0.55 ±0.16 in the CSF pressure normal patient group, Right TS SR 0.28 ± 0.07 Left TS SR 0.31 ± 0.07 in the control group Right TS SR It was 0.28 ± 0.09, Sol TS SR was 0.30 ± 0.07. CONCLUSION: Before LP, the cranial venous system must be monitored. TS SR and CSF pressure are directly proportional. The stenosis rate is important for IIH rather than SSL.

Noticable Findings in Cranial MRI of the Patients with Idiopathic Intracranial Hypertension.

OBJECTIVES: Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure. We investigated the optic nerve, Meckel's cavity, internal carotid artery (ICA) and pituitary findings of IIH by Cranial Magnetic Resonance Imaging (MRI). METHODS: Cranial MRI images of 35 adult patients with IIH and 35 adult subjects with normal cranial MRI results (control) were evaluated. Optic nerve diameter (OND), optic nerve sheat diameter (ONSD), OND/ONSD ratio, ON tortuosity, ON protrusion, posterior scleral flattening, ICA transverse diameter, CSF distance in Meckel's cavity and ICA transverse diameter/CSF distance in Meckel's cavity ratio, and pituitary gland measurements (height and transverse dimension; and Optic chiasm- pituitary gland distance) were measured. RESULTS: OND and ONSD of the IIH group were significantly higher than those of the control groups at anterior and posterior measurements (p < 0.05). OND/ONSD ratio of the IIH group was lower at anterior measurement; and higher at the posterior measurement than the control group (p < 0.05). Right ICA transverse diameter and bilateral CSF distance in Meckel's cavity of the IIH group were higher than those of the control. Optic chiasm- pituitary gland distance of the IIH group was significantly higher than that of the control group (p < 0.05). CONCLUSION: OND/ONSD ratio is different in anterior and posterior measurements. So we recommend measuring OND and ONSD separately in IIH patients to use in the clinical practice. Similarly, optic chiasm-pituitary gland distance is also another point to note for IIH patients on MRI. Our paper adds new approach to IIH in terms of OND/ONSD ratio.

Brain herniation (encephalocele) into arachnoid granulations: prevalence and association with pulsatile tinnitus and idiopathic intracranial hypertension.

PURPOSE: Brain herniation into arachnoid granulations (BHAG) of the dural venous sinuses is a recently described finding of uncertain etiology. The purpose of this study was to investigate the prevalence of BHAG in a cohort of patients with pulsatile tinnitus (PT) and to clarify the physiologic and clinical implications of these lesions. METHODS: The imaging and charts of consecutive PT patients were retrospectively reviewed. All patients were examined with MRI including pre- and post-contrast T1- and T2-weighted sequences. Images were reviewed separately by three blinded neuroradiologists to identify the presence of BHAG. Their location, signal intensity, size, presence of arachnoid granulation, and associated dural venous sinus stenosis were documented. Clinical records were further reviewed for idiopathic intracranial hypertension, history of prior lumbar puncture, and opening pressure. RESULTS: Two hundred sixty-two consecutive PT patients over a 4-year period met inclusion criteria. PT patients with BHAG were significantly more likely to have idiopathic intracranial hypertension than PT patients without BHAG (OR 4.2, CI 1.5-12, p = 0.006). Sixteen out of 262 (6%) patients were found to have 18 BHAG. Eleven out of 16 (69%) patients had unilateral temporal or occipital lobe herniations located in the transverse sinus or the transverse-sigmoid junction. Three out of 16 (19%) patients had unilateral cerebellar herniations and 2/16 (13%) patients had bilateral BHAG. CONCLUSION: In patients with PT, BHAG is a prevalent MRI finding that is strongly associated with the clinical diagnosis of IIH. The pathogenesis of BHAG remains uncertain, but recognition should prompt comprehensive evaluation for IIH.

Idiopathic Intracranial Hypertension is Associated with a Higher Burden of Visible Cerebral Perivascular Spaces: The Glymphatic Connection.

BACKGROUND AND PURPOSE: Research suggests a connection between idiopathic intracranial hypertension and the cerebral glymphatic system. We hypothesized that visible dilated perivascular spaces, possible glymphatic pathways, would be more prevalent in patients with idiopathic intracranial hypertension. This prevalence could provide a biomarker and add evidence to the glymphatic connection in the pathogenesis of idiopathic intracranial hypertension. MATERIALS AND METHODS: We evaluated 36 adult (older than 21 years of age) patients with idiopathic intracranial hypertension and 19 controls, 21-69 years of age, who underwent a standardized MR imaging protocol that included high-resolution precontrast T2- and T1-weighted images. All patients had complete neuro-ophthalmic examinations for papilledema. The number of visible perivascular spaces was evaluated using a comprehensive 4-point qualitative rating scale, which graded the number of visible perivascular spaces in the centrum semiovale and basal ganglia; a 2-point scale was used for the midbrain. Readers were blinded to patient diagnoses. Continuous variables were compared using a Student t test. RESULTS: The mean number of visible perivascular spaces overall was greater in the idiopathic intracranial hypertension group than in controls (4.5 [SD, 1.9] versus 2.9 [SD, 1.9], respectively; P = .004). This finding was significant for centrum semiovale idiopathic intracranial hypertension (2.3 [SD, 1.4] versus controls, 1.3 [SD, 1.1], P = .003) and basal ganglia idiopathic intracranial hypertension (1.7 [SD, 0.6] versus controls, 1.2 [SD, 0.7], P = .009). There was no significant difference in midbrain idiopathic intracranial hypertension (0.5 [SD, 0.5] versus controls, 0.4 [SD, 0.5], P = .47). CONCLUSIONS: Idiopathic intracranial hypertension is associated with an increased number of visible intracranial perivascular spaces. This finding provides insight into the pathophysiology of idiopathic intracranial hypertension, suggesting a possible relationship between idiopathic intracranial hypertension and glymphatic dysfunction and providing another useful biomarker for the disease.

Pulsatile Tinnitus Due to Stenosis of the Marginal Sinus: Diagnosis and Endovascular Treatment.

BACKGROUND AND PURPOSE: Venous pulsatile tinnitus is a disabling condition mainly caused by a stenosis of the lateral sinus. Here, we aimed to report a novel cause of venous pulsatile tinnitus, stenosis of the marginal sinus. MATERIALS AND METHODS: We retrospectively analyzed patients with isolated venous pulsatile tinnitus for which the suspected cause was a stenosis of the marginal sinus, treated or not, between January 2017 and December 2020. Patient charts and imaging were systematically reviewed. All patients underwent noncontrast temporal bone CT and MR imaging. RESULTS: Eight patients (7 women; median age, 36 years) were included. Six patients (75%) were overweight, and 1 patient had idiopathic intracranial hypertension. All patients presented with a typical venous pulsatile tinnitus. The stenosis of the marginal sinus was detected using oblique reconstructions on postcontrast 3D MR imaging. There was no other pathologic finding except ipsilateral stenosis of the lateral sinus in 3 patients. Four patients underwent endovascular therapy with placement of a stent in the marginal sinus, leading to complete resolution of the pulsatile tinnitus for all of them. No complication occurred. Of note, the symptoms of intracranial hypertension also regressed after stent placement in that patient. CONCLUSIONS: Marginal sinus stenosis is a novel cause of venous pulsatile tinnitus, which can be easily detected on MR imaging. Marginal sinus stent placement is safe and efficient. We hypothesized that the marginal sinus stenosis pathophysiology is similar to that of lateral sinus stenosis, which is a common and well-known cause of venous pulsatile tinnitus, explaining the similar clinical presentation and endovascular management.

Dural Venous Sinus Stenosis: Why Distinguishing Intrinsic-versus-Extrinsic Stenosis Matters.

BACKGROUND AND PURPOSE: Dural venous sinus stenosis has been associated with idiopathic intracranial hypertension and isolated venous pulsatile tinnitus. However, the utility of characterizing stenosis as intrinsic or extrinsic remains indeterminate. The aim of this retrospective study was to review preprocedural imaging of patients with symptomatic idiopathic intracranial hypertension and pulsatile tinnitus, classify the stenosis, and assess a trend between stenosis type and clinical presentation while reviewing the frequencies of other frequently seen imaging findings in these conditions. MATERIALS AND METHODS: MRVs of 115 patients with idiopathic intracranial hypertension and 43 patients with pulsatile tinnitus before venous sinus stent placement were reviewed. Parameters recorded included the following: intrinsic or extrinsic stenosis, prominent emissary veins, optic nerve tortuosity, cephalocele, sella appearance, poststenotic fusiform enlargement versus saccular venous aneurysm, and internal jugular bulb diverticula. χ2 cross-tabulation statistics were calculated and recorded for all data. RESULTS: Most patients with idiopathic intracranial hypertension (75 of 115 sinuses, 65%) had extrinsic stenosis, and most patients with pulsatile tinnitus (37 of 45 sinuses, 82%) had intrinsic stenosis. Marked optic nerve tortuosity was more common in idiopathic intracranial hypertension. Cephaloceles were rare in both cohorts, with an increased trend toward the presence in idiopathic intracranial hypertension. Empty sellas were more common in idiopathic intracranial hypertension. Cerebellar tonsils were similarly located at the foramen magnum level in both cohorts. Saccular venous aneurysms were more common in pulsatile tinnitus. Internal jugular bulb diverticula were similarly common in both cohorts. CONCLUSIONS: In this cohort, most patients with idiopathic intracranial hypertension had extrinsic stenosis, and most patients with pulsatile tinnitus had intrinsic stenosis. Awareness and reporting of these subtypes may reduce the underrecognition of potential contributory stenoses in a given patient's idiopathic intracranial hypertension or pulsatile tinnitus.

MRI findings as markers of idiopathic intracranial hypertension.

PURPOSE OF REVIEW: Negative findings on neuroimaging are part of the diagnostic criteria for idiopathic intracranial hypertension (IIH), a syndrome characterized by increased intracranial pressure (ICP). Some positive neuroimaging findings are associated with increased ICP, but their role in diagnosis of IIH has not been established. We provide an overview of these findings and their relevance for diagnosis of raised intracranial pressure. RECENT FINDINGS: MRI acquisition techniques have significantly improved in the last few decades leading to better characterization of the intracranial changes associated with IIH, including empty sella turcica, optic nerve tortuosity, distension of the optic nerve sheath, posterior globe flattening, slit-like ventricles, and venous sinus stenosis. These may be MRI biomarkers of increased ICP. Prevalence difference between people with and without increased ICP, and reversibility of these MRI findings following treatment of increased ICP inform evaluation of their diagnostic potential. SUMMARY: MRI and magnetic resonance venography findings are important tools in the diagnosis of IIH. Empty sella turcica, optic nerve protrusion, distension of the optic nerve sheath, optic nerve tortuosity, posterior globe flattening, and transverse sinus stenosis have been found to be the most promising diagnostic markers for IIH, although absence of these findings does not rule out the diagnosis.

The effect of intracerebral structure volumes on idiopathic intracranial hypertension.

BACKGROUND: This aim of this study was to assess the effect of idiopathic intracranial hypertension (IIH) in the development of intracranial hypertension by measuring patients' cerebrospinal fluid (CSF), brain, and segment volumes. IIH is a disease of unknown cause characterized by chronic CSF pressure elevation and papillary edema. Under the Monro-Kellie doctrine, efforts have been made to explain the disease through an increase in the volumes of one or two of brain, blood or CSF in the intracranial cavity, although these have not been completely successful. METHODS: Twelve IIH patients diagnosed based on the modified Dandy criteria and 15 age- and sex-compatible healthy controls were included in the study. Cases' 3D T1 sequence images were evaluated using the voxel-based volumetric analysis system (volBrain). Intracranial cavity extraction, macrostructures, and subcortical structure volumes were measured and compared. RESULTS: No statistically significant differences were observed between control cases' and IIH patients' CSF, cerebrum, cerebellum, brainstem, and subcortical structure volumes. CONCLUSION: IIH arises from a chronic cause that does not impair the volumetric balance between structures inside the intracerebral cavity.

Increased occurrence of pathological mitochondria in astrocytic perivascular endfoot processes and neurons of idiopathic intracranial hypertension.

Idiopathic intracranial hypertension (IIH) primarily affects fertile, overweight women, and presents with the symptoms of raised intracranial pressure. The etiology is unknown but has been thought to relate to cerebrospinal fluid disturbance or cerebral venous stenosis. We have previously found evidence that IIH is also a disease of the brain parenchyma, evidenced by alterations at the neurogliovascular interface, including astrogliosis, pathological changes in the basement membrane and pericytes, and alterations of perivascular aquaporin-4. The aim of this present electron microscopic study was to examine whether mitochondria phenotype was changed in IIH, particularly focusing on perivascular astrocytic endfeet and neurons (soma and pre- and postsynaptic terminals). Cortical brain biopsies of nine reference individuals and eight IIH patients were analyzed for subcellular distribution and phenotypical features of mitochondria using transmission electron microscopy. We found significantly increased prevalence of pathological mitochondria and reduced number of normal mitochondria in astrocytic endfeet of IIH patients. The degree of astrogliosis correlated negatively with the number of normal mitochondria in astrocytic endfoot processes. Moreover, we found significantly increased number of pathological mitochondria in pre- and postsynaptic neuronal terminals, as well as significantly shortened distance between mitochondria and endoplasmic reticulum contacts. Finally, the length of postsynaptic density, a marker of synaptic strength, was on average reduced in IIH. The present data provide evidence of pathological mitochondria in perivascular astrocytes endfeet and neurons of IIH patients, highlighting that impaired metabolism at the neurogliovascular interface may be a facet of IIH.

11ßHSD1 Inhibition with AZD4017 Improves Lipid Profiles and Lean Muscle Mass in Idiopathic Intracranial Hypertension.

BACKGROUND: The enzyme 11ß-hydroxysteroid dehydrogenase type 1 (11ß-HSD1) determines prereceptor metabolism and activation of glucocorticoids within peripheral tissues. Its dysregulation has been implicated in a wide array of metabolic diseases, leading to the development of selective 11ß-HSD1 inhibitors. We examined the impact of the reversible competitive 11ß-HSD1 inhibitor, AZD4017, on the metabolic profile in an overweight female cohort with idiopathic intracranial hypertension (IIH). METHODS: We conducted a UK multicenter phase II randomized, double-blind, placebo-controlled trial of 12-week treatment with AZD4017. Serum markers of glucose homeostasis, lipid metabolism, renal and hepatic function, inflammation and androgen profiles were determined and examined in relation to changes in fat and lean mass by dual-energy X-ray absorptiometry. RESULTS: Patients receiving AZD4017 showed significant improvements in lipid profiles (decreased cholesterol, increased high-density lipoprotein [HDL] and cholesterol/HDL ratio), markers of hepatic function (decreased alkaline phosphatase and gamma-glutamyl transferase), and increased lean muscle mass (1.8%, P < .001). No changes in body mass index, fat mass, and markers of glucose metabolism or inflammation were observed. Patients receiving AZD4017 demonstrated increased levels of circulating androgens, positively correlated with changes in total lean muscle mass. CONCLUSIONS: These beneficial metabolic changes represent a reduction in risk factors associated with raised intracranial pressure and represent further beneficial therapeutic outcomes of 11ß-HSD1 inhibition by AZD4017 in this overweight IIH cohort. In particular, beneficial changes in lean muscle mass associated with AZD4017 may reflect new applications for this nature of inhibitor in the management of conditions such as sarcopenia.

Clinical characteristics and surgical treatment of haemophilic pseudotumor: A retrospective analysis of thirty-four patients.

AIMS: Haemophilic pseudotumor (HPT) is a rare but challenging complication of haemophilia. This study was intended to provide our experience about clinical characteristics and surgical treatment of HPT. METHODS: Clinical medical records were retrieved from the Hemophilia Center, Nanfang Hospital, to identify the patients who had been surgically treated from 1 January 2006 to 31 December 2017 with a definite diagnosis of HPT. Their clinical features, surgical management, outcomes and complications after surgery were analysed. RESULTS: We identified 34 patients with HPT who had surgical treatment over a 12-year period and five of them had multiple HPTs. The incidence of HPT at this centre was 2.3% over the dozen years. A previous trauma leading to the development of HPT was reported in 18 cases (52.9%). The HPT affected only soft tissue in 7 patients, bone and soft tissue in 25 ones and joint in 2 ones. Preoperative infection and fistula formation happened in ten patients, two of whom were related to abdominal HPTs. Enterococcus faecalis was cultured in five cases with fistula formation. HPT associated with pathological fracture was observed in five cases, two of whom were treated by external fixation and 3 by HPT resection and metallic internal fixation. Amputation was performed for nine patients, 6 of whom had preoperative infection and fistula formation. Their follow-up duration averaged 4.2 ± 2.9 years (range, from 1 to 13.5 years) after surgery. Of all our cases, three suffered from postoperative infection, five from recurrence of HPT and two with external fixation from fracture non-union. CONCLUSIONS: HPT patients with preoperative infection had worse prognosis than those without. Surgical treatment plus intensive replacement therapy was effective for HPT but with a high rate of complications. HPT resection and metallic internal fixation rather than external fixation should be recommended for HPT patients with pathological fracture.

Migraine or idiopathic intracranial hypertension: Magnetic resonance venography and magnetic resonance imaging findings.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a disease characterised by increased cerebral pressure without a mass or hydrocephalus. We aimed to differentiate migraine and IIH patients based on imaging findings. RESULTS: Patients with IIH (n = 32), migraine patients (n = 34) and control subjects (n = 33) were evaluated. Routine magnetic resonance imaging, contrast-enhanced 3D magnetic resonance venography and/or T1-weighted 3D gradient-recalled echo were taken with a 1.5 T magnetic resonance scanner. Optic-nerve sheath distention, flattened posterior globe and the height of the pituitary gland were evaluated in the three groups. Transverse sinuses (TS) were evaluated with respect to score of attenuation/stenosis and distribution. Pearson chi-square, Fisher's exact test and chi-square trend statistical analyses were used for comparisons between the groups. A p-value of <0.05 was considered statistically significant. Decreased pituitary gland height, optic-nerve sheath distention and flattened posterior globe were found to be statistically significant (p < 0.001) in IIH patients. Bilateral TS stenosis was also more common in IIH patients than in the control group and migraine group (p = 0.02). CONCLUSION: Decreased pituitary gland height, optic-nerve sheath distention, flattened posterior globe, bilateral stenosis and discontinuity in TS are significant findings in differentiating IIH cases from healthy individuals and migraine patients. Bilateral TS stenosis may be the cause rather than the result of increased intracranial pressure. The increase in intracranial pressure, which is considered to be responsible for the pathophysiology of IIH, is not involved in the pathophysiology of migraine.

Morphological assessment of lamina cribrosa in idiopathic intracranial hypertension.

Purpose: Technological development of optic coherence tomography has enabled a detailed assessment of the optic nerve and deeper structures and in vivo measurements. The aim of this study was to compare the lamina cribrosa morphology of the optic nerve in idiopathic intracranial hypertension (IIH) and healthy individuals. Methods: The lamina cribrosa morphology of optic nerve in 15 eyes with IIH and 17 eyes of healthy individuals were compared. Four parameters such as Bruch membrane opening (BMO), lamina cribrosa thickness (LCT), prelaminar tissue thickness (PTT), and anterior lamina cribrosa surface depth (ALCSD) were retrospectively evaluated. Results: By enhanced depth imaging-optic coherence tomography (EDI-OCT), PTT and BMO were found to be significantly greater (574,35 ± 169,20 µm and 1787,40 ± 140,87 µm, respectively) in IIH patients than healthy individuals (187,18 ± 132,15 µm and 1632,65 ± 162,58 µm, respectively), whereas ALSCD was found to be significantly less in IIH patients (234,49 ± 49,31 µm) than healthy individuals (425,65 ± 65,23 µm). There was not a statistically significant difference regarding LCT between the IIH patients (238,59 ± 17,31 µm) and healthy individuals (244,96 ± 15,32 µm). Conclusion: Increased intracranial pressure causes morphological changes in lamina cribrosa. Assessment of lamina cribrosa with EDI-OCT is important for diagnosis and follow-up of patients with IIH. EDI-OCT is objective, reproducible, and cost-effective assistive imaging tool in IIH patients.