RESUMEN

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a cause of chronic headaches that are probably driven by raised intracranial pressure (ICP). Cerebral venous sinus pressure is thought to play a role in the underlying pathology, but its relation with intracranial pressure requires further investigation. We aimed to evaluate the concordance between lumbar puncture opening pressure (LPOP) as indicator of the ICP and cerebral venous sinus pressure in patients investigated for IIH. METHODS: In this case-series replication study, all patients with IIH suspicion and who underwent cerebral venous sinus pressure measurement followed immediately by LP opening pressure (LPOP) measurement were retrospectively included. Pearson's correlation and measurement agreement (Bland-Altman plots) between venous pressure and LPOP were analyzed. RESULTS: 52 consecutive patients (46 women; median age, 31 years [IQR = 25-42]) were included. The mean pressure in the superior sagittal sinus (SSS) and in the torcular were 20.9mmHg (SD ± 7.3) and 20.8 mmHg (SD ± 6.8), respectively. The mean LPOP was 22mmHg (SD ± 6.4). Pressure measured in the transverse venous sinus, the torcular, and the SSS correlated with LPOP (p < 0.001). Bland-Altman plots showed that torcular pressure strongly agreed with LPOP (mean difference of 1.7mmHg). The limit of agreement (LOA) (mean difference ± 1.96SD) contained 98.1% of the differences between the two methods, confirming the concordance between the two measures. Torcular pressure and LPOP were consistent in patients with a trans-stenotic pressure gradient ≥ or < to 8 mmHg (mean difference: 1mmHg and 2.4mmHg, respectively), and for those with a LP OP ≥ or < to 18mmHg (mean difference: 1.8mmHg and 1.95mmHg, respectively). CONCLUSIONS: In patients investigated for IIH, the ICP measured at the LP is correlated and concordant with the torcular pressure. These results confirm previous findings and further corroborate the hypothesis that cerebral venous system plays a major role in CSF dynamics and ICP.

Asunto(s)

Senos Craneales , Presión Intracraneal , Seudotumor Cerebral , Punción Espinal , Humanos , Femenino , Adulto , Masculino , Presión Intracraneal/fisiología , Seudotumor Cerebral/fisiopatología , Seudotumor Cerebral/complicaciones , Seudotumor Cerebral/diagnóstico , Senos Craneales/fisiopatología , Senos Craneales/diagnóstico por imagen , Estudios Retrospectivos , Presión Venosa/fisiología

RESUMEN

BACKGROUND: There have been limited data on idiopathic intracranial hypertension (IIH) in Asians and there remain uncertainties whether a cerebrospinal fluid (CSF) pressure of 250 mm CSF is an optimum diagnostic cutoff. The aims of the present study included (1) characterization of IIH patients in Taiwan, (2) comparisons among different diagnostic criteria for IIH, and (3) comparisons between patients with CSF pressures of > 250 and 200-250 mm CSF. METHODS: This retrospective study involved IIH patients based on the modified Dandy criteria from two tertiary medical centers in Taiwan. Clinical manifestations were retrieved from electronic medical records, and findings on ophthalmologic examination and magnetic resonance images (MRIs) were reviewed. RESULTS: A total of 102 patients (71 F/31 M, mean age 33.4 ± 12.2 years, mean CSF pressure 282.5 ± 74.5 mm CSF) were identified, including 46 (45.1%) with obesity (body-mass index ≥ 27.5), and 57 (62.6%) with papilledema. Overall, 80 (78.4%), 55 (53.9%), 51 (50.0%), and 58 (56.9%) patients met the Second and Third Edition of International Classification of Headache Disorders, Friedman, and Korsbæk criteria, respectively. Patients in the 200-250 mm CSF group (n = 40) were less likely to have papilledema (48.5% vs. 70.7%, p = 0.035), transient visual obscuration (12.5% vs. 33.9%, p = 0.005), and horizontal diplopia (10.0% vs. 30.6%, p = 0.006), and had fewer signs on MRIs (2.2 ± 1.3 vs. 2.8 ± 1.0, p = 0.021) when compared with those with CSF pressures > 250 mm CSF (n = 62). However, the percentages of patients with headache (95.0% vs. 87.1%, p = 0.109) at baseline, chronic migraine at six months (31.6% vs. 25.0%, p = 0.578), and visual field defect (86.7% vs. 90.3%, p = 0.709) were similar. CONCLUSIONS: It was found that obesity and papilledema were less common in Asian IIH patients when compared with Caucasian patients. Although patients with CSF pressures of 200-250 mm CSF had a less severe phenotype, the risks of having headache or visual loss were comparable to those in the > 250 mm CSF group. It is possible that a diagnostic cutoff of > 200 mm CSF could be more suitable for Asians, although further studies are still needed.

Asunto(s)

Seudotumor Cerebral , Humanos , Femenino , Masculino , Adulto , Estudios Retrospectivos , Seudotumor Cerebral/epidemiología , Seudotumor Cerebral/complicaciones , Seudotumor Cerebral/diagnóstico , Taiwán/epidemiología , Pueblo Asiatico , Adulto Joven , Persona de Mediana Edad , Imagen por Resonancia Magnética , Presión del Líquido Cefalorraquídeo/fisiología , Papiledema/diagnóstico

RESUMEN

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a neurological disorder characterized by increased intracranial pressure. Whilst lumbar puncture (LP) is necessary for the diagnosis of IIH, its therapeutic effect remains unclear. Our aim was to evaluate the therapeutic effect of a single LP in people with IIH (pwIIH). METHODS: In this prospective observational study, we analysed short-term neurological and ophthalmological outcomes in pwIIH before, one (D1) and seven days (D7) after the LP. The primary outcome was the change in papilledema degree from baseline. Secondary outcomes included visual outcomes, morphological changes in optical coherence tomography (peripapillary retinal nerve fibre layer [pRNFL] thickness and ganglion cell layer [GCL] volume) and transbulbar sonography (arachnoid optic nerve sheath diameter [AONSD]), and headache outcomes (peak and median headache severity and burden related to headache). RESULTS: We included 30 pwIIH (mean age 32.8 years [SD 8.4], 93.3% female, median cerebrospinal fluid [CSF] opening pressure 33.0 cmCSF [IQR 26.9-35.3], median body mass index (BMI) 34.8 kg/m2 [IQR 30.9-40.9]). The median papilledema grading at baseline was 2 (Friedman DI (1999) Pseudotumor cerebri. Neurosurg Clin N Am 10(4):609-621 viii); (Mollan SP, Aguiar M, Evison F, Frew E, Sinclair AJ (2019) The expanding burden of idiopathic intracranial hypertension. Eye Lond Engl 33(3):478-485); (Ab D, Gt L, Nj V, Sl G, Ml M, Nj N et al. (2007) Profiles of obesity, weight gain, and quality of life in idiopathic intracranial hypertension (pseudotumor cerebri). Am J Ophthalmol [Internet]. Apr [cited 2024 Jun 2];143(4). https://pubmed.ncbi.nlm.nih.gov/17386271/ ) and was significantly reduced at D7 (2 [1-2], p = 0.011). Median pRNFL thickness had decreased significantly at D7 (-9 µm [-62.5, -1.3], p = 0.035), with pRNFL thickness at baseline being associated with the pRNFL change (F(1,11) = 18.79, p = 0.001). Mean AONSD had decreased significantly at both D1 (-0.74 mm [0.14], p < 0.001) and D7 (-0.65 mm [0.17], p = 0.01), with AONSD at baseline being associated with the change in AONSD at both time points (D1: ß= -0.89, 95% CI -1.37, -0.42, p = 0.002; D7: ß= -0.85, 95% CI -1.42, -0.28, p = 0.007). Peak headache severity was slightly lower at D7 (-1/10 [-3, 0], p = 0.026), whereas median headache severity and headache burden remained unchanged. CONCLUSIONS: This short-term follow-up study in pwIIH undergoing a single LP suggests a moderate effect on ophthalmological but not headache outcomes. The usefulness of LP as a therapeutic measure in IIH remains controversial and should likely be reserved for patients with limited treatment options, e.g., in pregnancy or intolerability to medication.

Asunto(s)

Papiledema , Seudotumor Cerebral , Punción Espinal , Tomografía de Coherencia Óptica , Humanos , Femenino , Adulto , Seudotumor Cerebral/complicaciones , Seudotumor Cerebral/diagnóstico por imagen , Punción Espinal/métodos , Masculino , Estudios Prospectivos , Tomografía de Coherencia Óptica/métodos , Papiledema/diagnóstico por imagen , Papiledema/etiología , Resultado del Tratamiento

RESUMEN

Neurological long Covid (NLC) is a major post-acute sequela of SARS-CoV-2 infection, affecting up to 10% of infected patients. The clinical presentation of patients with NLC is varied, but general NLC symptoms have been noted to closely mimic symptoms of cerebral venous outflow disorders (CVD). Here we review key literature and discuss evidence supporting this comparison. We also aimed to describe the similarity between CVD symptomatology and neuro-NLC symptoms from two perspectives: a Twitter-distributed survey for long covid sufferers to estimate nature and frequency of neurological symptoms, and through a small cohort of patients with long covid who underwent CVD work up per our standard workflow. Over 700 patients responded, and we argue that there is a close symptom overlap with those of CVD. CVD workup in a series of 6 patients with neurological long COVID symptoms showed jugular vein stenosis by CT venography and varying degrees of increased intracranial pressure. Finally, we discuss the potential pathogenic association between vascular inflammation, associated with COVID-19 infection, venous outflow congestion, and its potential involvement in NLC.

RESUMEN

BACKGROUND: Idiopathic intracranial hypertension (IIH) can affect both hearing and balance due to increased inner ear pressure. AIMS/OBJECTIVES: This study aimed to evaluate the impact of increased inner ear pressure on hearing and balance in patients with IIH using auditory and vestibular tests. MATERIAL AND METHODS: Twenty-four IIH patients and 28 healthy controls underwent oVEMP, pure tone audiometry, tympanometry, and acoustic reflex tests pre-lumbar punctures. IIH patients received acetazolamide. Pre- and post-treatment results, tinnitus, and vertigo scores were compared. Post-treatment oVEMP and audiometry results were compared between groups. RESULTS: Pre-treatment oVEMP showed a significant left N1 latency difference (p = 0.049). Post-treatment, left ear amplitude (p = 0.035) and both ear amplitude ratios (p = 0.044 and p = 0.047) increased significantly. Audiometry had no significant changes (p < 0.05). Tinnitus and vertigo scores decreased significantly (p ≤ 0.001). CONCLUSION: Prolonged oVEMP latency suggests IIH may impact the brain stem and vestibular nerve, while increased amplitude values indicate peripheral vestibular involvement. IIH affects hearing across all frequencies, especially at 4000 Hz, impacting both hearing and balance. SIGNIFICANCE: Understanding the effects of IIH on auditory and vestibular functions can guide effective treatments, improving quality of life for patients by addressing both hearing and balance issues.

RESUMEN

BACKGROUND: Pediatric idiopathic intracranial hypertension (IIH) is a rare and challenging condition. As implied by the nomenclature, the etiologies remain unknown, and multiple etiologies are being investigated. In this study, we explored the potential role of increased systemic or cerebral venous pressure in the pathogenesis. METHOD: An observational cohort study following the STROBE guidelines, including prepubertal children with clinical symptoms and imaging findings consistent with IIH referred to the neurosurgical department, was conducted. The patients underwent a comprehensive diagnostic protocol, including MRI, continuous intracranial pressure (ICP) monitoring, and endovascular venography with venous pressure measurements. RESULTS: The study included 11 consecutive patients (six boys and five girls) with an average age of 2.3 years, and an average BMI of 18.4. Among these, one patient was found to have venous stenosis with a gradient; the other 10 patients presented with normal intracranial anatomy. All patients exhibited elevated venous pressures, with an average superior sagittal sinus pressure of 18.9 mmHg, average internal jugular vein pressure of 17.0 mmHg, and average central venous pressure of 15.9 mmHg. Daytime ICP averaged 12.9 mmHg, whereas nighttime ICP averaged 17.2 mmHg with either A- or B-waves in 10 of the 11 patients. Despite pathological ICP, only three patients had papilledema. CONCLUSIONS: All patients had an increased systemic venous pressure, indicating a possible pathological factor for prepubertal IIH. Additionally, our findings show that young children often only partly meet the Friedman criteria due to a lack of papilledema, emphasizing the need for pediatric-specific diagnostic criteria. Further large-scale studies are needed to confirm these findings and to explore the underlying reasons for this increase in venous pressure and potential new treatment avenues.

RESUMEN

Background: Coexisting intracranial pathologies of distinct etiology which require intervention are rare. Only a handful of cases have been reported in the literature. The effects of each treatment option on both pathologies need to be considered during management. We describe the first report of the management of a patient with concurrent symptomatic tuberculum sellae meningioma (TSM) and idiopathic intracranial hypertension (IIH). Case Description: A 58-year-old male presented with 2 weeks of vision loss and 3 months of headaches. He was found to have an inferior hemi-field deficit in the left eye and bilateral papilledema. Imaging studies revealed bilateral transverse sinus stenosis and a TSM abutting the left optic nerve. The opening pressure was 40 cmH2O. An expanded-endoscopic endonasal approach was performed for mass resection. Intraoperatively, a lumbar drain was placed to aid skull base repair integrity before definitive treatment was obtained. On postoperative day 9, a right transverse-sigmoid sinus stent was placed for IIH treatment. The patient was discharged the following day. Conclusion: Our management of this patient targeted the etiologies of each symptomatic pathology. Stenting provided treatment for the IIH and mass resection for the vision loss. Both the order and approaches to treatment were felt to maximize patient benefit while minimizing harm.

RESUMEN

INTRODUCTION: Cerebral venous sinus stenting (CVSS) is an effective treatment for idiopathic intracranial hypertension (IIH) secondary to dural venous sinus stenosis. Traditional selection of patients for CVSS has been made by microcatheter manometry, but pressure measurements are often equivocal. Here we present the results of a series of cases in which venous flat-panel CT (FP-CT) was used as an adjunct to microcatheter manometry to improve decision making and precise stent placement during CVSS. METHODS: Ten consecutive patients with IIH underwent angiography with microcatheter manometry and venous FP-CT, with CVSS if indicated by the results. Cross-sectional measurements of the narrowed sinus were obtained on FP-CT before and after stenting. After the procedure, clinical outcomes were tracked. Follow-up with quantitative MRA with sinus flow measurements was also performed, when available. RESULTS: There was an exponential correlation between measured pressure gradient and degree of stenosis calculated using venous FP-CT. All patients with both a high degree of stenosis measured by FP-CT and a high pressure gradient across the stenosis showed a clinical benefit from stenting. CONCLUSIONS: True measurement of the cross-sectional area of the dural sinus, made by venous phase FP-CT, has a high degree of correlation with elevated venous pressure gradient across the point of stenosis. Even in a limited series of cases, we found an exponential decrease in flow with increasing severity of stenosis. Furthermore, patients with both an elevated venous pressure gradient and critical stenosis of the sinus on FP-CT showed symptomatic improvement after stenting.

RESUMEN

OBJECTIVES: To determine whether idiopathic intracranial hypertension (IIH) may affect white matter integrity and optic pathways by using diffusion tensor imaging (DTI) and to correlate the DTI metrics with intracranial pressure (ICP). METHODS: This study is a retrospective case-control study. A total of 42 patients who underwent lumbar puncture and those with elevated ICP, meeting the diagnostic criteria for IIH, were included in the study. All patients had supportive magnetic resonance imaging findings for the diagnosis of IIH. The headache control group comprised 36 patients who presented to the Neurology Department with infrequent episodic tension-type headache, had a normal neurologic examination, and had clinical and radiological findings suggestive of normal ICP. For each patient with IIH, clinical findings and ophthalmological measurements were recorded. The apparent diffusion coefficient (ADC), fractional anisotropy (FA), axial diffusivity (AD), and radial diffusivity (RD) values were calculated using a region of interest-based method in different white matter tracts and optic pathways and compared. RESULTS: A total of 42 patients diagnosed with IIH (three males, 39 females), with a mean (standard deviation [SD] age of 38.1 (8.9) years), and 36 headache controls (10 males, 26 females, mean [SD] age; 38.1 [9.4] years) were included in the study. The mean (SD) body mass index (BMI) of the patients with IIH was 25.2 (1.9) kg/m2, and the mean (SD) BMI of the headache controls was 23.3 (1.5) kg/m2 (p < 0.001). Decreased FA values and increased RD values in the cingulum were detected in patients with IIH compared to the headache controls (p = 0.003, Cohen's d = 0.681; p = 0.002 Cohen's d = -0.710). Decreased AD values in the left and right superior cerebellar peduncle and increased ADC values in the middle cerebellar peduncle were detected in patients with IIH compared to the headache controls (p < 0.001, Cohen's d = 0.961; p = 0.009, Cohen's d = 0.607; p = 0.015, Cohen's d = -0.564). Increased ADC and RD values and decreased FA values in optic nerve were detected in patients with IIH (p = 0.010, Cohen's d = -0.603; p = 0.004, Cohen's d = -0.676; p = 0.015 Cohen's d = 0.568). A positive correlation was found between the cerebrospinal fluid pressure and ADC values of the left and right superior and left inferior longitudinal fasciculus, genu of the corpus callosum, and right optic radiation (r = 0.43, p = 0.005; r = 0.31, p = 0.044; r = 0.39, p = 0.010; r = 0.35, p = 0.024; r = 0,41, p = 0.007). There was a positive correlation between the retinal nerve fiber layer thickness and the ADC values of the optic nerve (r = 0.32, p = 0.039). CONCLUSIONS: Intracranial hypertension can be associated with deteriorated DTI values, which might be interpreted as a sign of impaired white matter microstructural integrity in many brain regions beyond the periventricular white matter. Pressure-induced edema and axonal degeneration may be the potential underlying mechanisms of this microstructural damage.

RESUMEN

Persistent idiopathic facial pain (PIFP), previously known as atypical facial pain (ATFP), is a chronic pain disorder with the characteristic of persistent, undulating pain in the face or the teeth without a known cause or any structural correlation. Women are more commonly affected than men. We report a case of a 38-year-old married female patient with a history of Crohn's disease who presented to the oral and maxillofacial surgery (OMFS) clinic with chronic dull bilateral facial pain and headache mainly affecting the right side of the face and neck without a known cause. She was initially diagnosed with PIFP due to a badly decayed right wisdom tooth. Wisdom teeth were extracted secondary to vague complaints of discomfort due to wisdom teeth; however, no significant improvement was noticed. Further investigations were carried out with new CT scans and magnetic resonance venography (MRV), which revealed evidence of having idiopathic intracranial hypertension (IIH), described as increased intracranial pressure with facial pain, headache, tinnitus, and papilledema. The patient was referred to neurology and received appropriate treatment. She began her treatment with topiramate, then transitioned to acetazolamide, underwent bilateral botulinum toxin (botox) injections into the temporal region, and underwent regular follow-up. The patient was significantly improved. Idiopathic intracranial hypertension must be ruled out in cases of PIFP that do not respond to ordinary treatment measures.

RESUMEN

In spite of expanding research, idiopathic intracranial hypertension (IIH) and its spectrum conditions remain challenging to treat. The failure to develop effective treatment strategies is largely due to poor agreement on a coherent disease pathogenesis model. Herein we provide a hypothesis of a unifying model centered around the internal jugular veins (IJV) to explain the development of IIH, which contends the following: (1) the IJV are prone to both physiological and pathological compression throughout their course, including compression near C1 and the styloid process, dynamic muscular/carotid compression from C3 to C6, and lymphatic compression; (2) severe dynamic IJV stenosis with developments of large cervical gradients is common in IIH-spectrum patients and significantly impacts intracranial venous and cerebrospinal fluid (CSF) pressures; (3) pre-existing IJV stenosis may be exacerbated by infectious/inflammatory etiologies that induce retromandibular cervical lymphatic hypertrophy; (4) extra-jugular venous collaterals dilate with chronic use but are insufficient resulting in impaired aggregate cerebral venous outflow; (5) poor IJV outflow initiates, or in conjunction with other factors, contributes to intracranial venous hypertension and congestion leading to higher CSF pressures and intracranial pressure (ICP); (6) glymphatic congestion occurs but is insufficient to compensate and this pathway becomes overwhelmed; and (7) elevated intracranial CSF pressures triggers extramural venous sinus stenosis in susceptible individuals that amplifies ICP elevation producing severe clinical manifestations. Future studies must focus on establishing norms for dynamic cerebral venous outflow and IJV physiology in the absence of disease so that we may better understand and define the diseased state.

RESUMEN

Lumbar puncture opening pressure (LPOP) exceeding 250mmH2O is key in diagnosing idiopathic intracranial hypertension (IIH), per revised Friedman's criteria. Some patients do not meet LPOP criteria (with or without papilledema), despite having IIH-related symptoms and neuroimaging findings. This study aimed to investigate the radiological findings and clinical symptoms in patients suspected of having IIH without meeting the LPOP criteria. We retrospectively evaluated cerebral venous sinus stenosis using the conduit Farb score (CFS) and other radiological findings suggestive of IIH by computed tomography venography and magnetic resonance venography in females ≥ 18 years-old with chronic headaches, suspected IIH, and LPOP < 250 mm. Eighty-eight women (56 with LPOP < 200 mm H2O and 32 with LPOP ranging between 200 and 250mmH2O) were included. Among patients with LPOP 200-250mmH2O, 40% (12) exhibited three or more radiological findings supporting IIH, compared to 17% (8) in the LPOP < 200 mmH2O group (p = 0.048). Cerebral venous stenosis (CFS ≤ 5) was observed in 80% (24) of those with LPOP 200-250 mmH2O, contrasting with 40% (19) of those with LPOP < 200 mmH2O (p < 0.001). Cerebral venous stenosis was significantly more common in patients with LPOP 200-250 mmH2O than < 200 mmH2O, suggesting that they may benefit from IIH treatment.

Asunto(s)

Seudotumor Cerebral , Punción Espinal , Humanos , Femenino , Adulto , Seudotumor Cerebral/diagnóstico por imagen , Seudotumor Cerebral/fisiopatología , Seudotumor Cerebral/complicaciones , Estudios Retrospectivos , Persona de Mediana Edad , Constricción Patológica/diagnóstico por imagen , Flebografía/métodos , Tomografía Computarizada por Rayos X/métodos , Masculino , Adulto Joven , Papiledema/diagnóstico por imagen , Papiledema/etiología

RESUMEN

BACKGROUND: Idiopathic intracranial hypertension (IIH) mainly affects obese young women, causing elevated intracranial pressure, headaches, and papilledema, risking vision loss and severe headaches. Despite weight loss as the primary treatment, the underlying mechanisms remain unclear. Recent research explores novel therapeutic targets. AIMS: This review aimed to provide a comprehensive understanding of IIH's pathophysiology and clinical features to inform pathogenesis and improve treatment strategies. METHODS: Recent publications on IIH were searched and summarized using PubMed, Web of Science, and MEDLINE. RESULTS: The review highlights potential pathomechanisms and therapeutic advances in IIH. CONCLUSION: IIH incidence is rising, with growing evidence linking it to metabolic and hormonal disturbances. Early diagnosis and treatment remain challenging.

Asunto(s)

Seudotumor Cerebral , Humanos , Seudotumor Cerebral/diagnóstico , Seudotumor Cerebral/terapia , Seudotumor Cerebral/complicaciones , Seudotumor Cerebral/fisiopatología

RESUMEN

BACKGROUND AND PURPOSE: Management of idiopathic intracranial hypertension (IIH) is complex requiring multiple specialized disciplines. In practice, this creates considerable organizational and communicational challenges for healthcare professionals and patients. Thus, an interdisciplinary integrated outpatient clinic for IIH (comprising neurology, neuroophthalmology, neuroradiology, neurosurgery and endocrinology) was established with central coordination and a one-stop concept. Here, the aim was to evaluate the effects of this one-stop concept on objective clinical outcome. METHODS: In a retrospective cohort study, the one-stop era with integrated care (IC) (1 July 2021 to 31 December 2022) was compared to a reference group receiving standard care (SC) (1 July 2018 to 31 December 2019) regarding visual impairment/worsening and headache improvement/freedom 6 months after diagnosis. Multivariate binary logistic regression models were used to adjust for confounders. RESULTS: Baseline characteristics of the IC group (n = 85) and SC group (n = 81) were comparable (female 90.6% vs. 90.1%; mean age 33.6 vs. 32.8 years; median body mass index 31.8 vs. 33.0; median cerebrospinal fluid opening pressure 32 vs. 34 cmH2O; at diagnosis, visual impairment was present in 71.8% vs. 69.1% and chronic headache in 55.3% vs. 56.8% in IC vs. SC). IC was associated with a higher likelihood of achieving both headache improvement (odds ratio [OR] 2.24, 95% confidence interval [CI] 1.52-4.33, p < 0.001) and headache freedom (OR 1.75, 95% CI 1.11-3.09, p = 0.031). Regarding the risk of visual impairment and visual worsening IC was superior numerically but not statistically significantly (OR 0.87, 95% CI 0.69-1.16, p = 0.231, and OR 0.67, 95% CI 0.41-1.25, p = 0.354). CONCLUSIONS: Interdisciplinary integrated care of IIH is favourably associated with headache outcomes and potentially also visual outcomes.

RESUMEN

BACKGROUND: Posterior globe flattening (PGF) is a specific neuroimaging sign in patients with idiopathic intracranial hypertension (IIH), but its detection is based on subjective qualitative neuroradiological assessment. This study sought to evaluate the utility of transorbital ultrasound to detect and quantify PGF in IIH patients using the Posterior Globe Angle (PGA). METHODS: Consecutive IIH patients and healthy controls were enrolled in a prospective case-control study. Transorbital ultrasound was performed to assess the presence of PGF. For quantification of PGF, an angular measurement (PGA) was performed with the vertex centering the optic nerve at a predefined distance from the lamina cribrosa and angle legs tangentially aligned to the borders of the vitreous body. PGA measurements were compared between IIH patients and healthy controls. Additionally, the diagnostic accuracy of PGA measurements in detecting PGF was evaluated using ROC analysis. RESULTS: Thirty-one IIH patients (37.3 ± 12.3 years, 29 female) and 28 controls (33.3 ± 11.8 years, 21 female) were compared. PGF was present in 39% of IIH patients and absent in the control group. PGA3mm measurements significantly differed between IIH and controls (116.5° ± 5.5 vs. 111.7° ± 2.9; p < 0.001). A PGA3mm cutoff of ≥ 118.5° distinguished IIH patients from controls with 100% specificity, while retaining a sensitivity of 37.5%. CONCLUSIONS: Transorbital ultrasound may be applied to detect and quantify PGF in IIH patients. Prospective, multicenter studies with extended cohorts and blinded design are needed to validate these preliminary findings and confirm the diagnostic utility of transorbital ultrasound for the assessment of PGF in IIH.

RESUMEN

BACKGROUND: Management of idiopathic intracranial hypertension (IIH) is complex requiring contributions from multiple specialized disciplines. In practice, this creates considerable organizational and communicational challenges. To meet those challenges, we established an interdisciplinary integrated outpatient clinic for IIH with a central coordination and a one-stop concept. Here, we aimed to evaluate effects of this one-stop concept on subjective patient satisfaction and economic outcome in patients with IIH. METHODS: In a retrospective cohort study, we compared the one-stop era with integrated care (IC, 1-JUL-2021 to 31-DEC-2022) to a reference group receiving standard care (SC, 1-JUL-2018 to 31-DEC-2019) regarding subjective patient satisfaction (assessed by the Vienna Patient Inventory). Multivariable binary linear regression models were used to adjust for confounders. RESULTS: Baseline characteristics of the IC group (n = 85) and SC group (n = 81) were comparable (female: 90.6% vs. 90.1%; mean age: 33.6 vs. 32.8 years, educational level: ≥9 years of education 60.0% vs. 59.3%; located in Vienna 75.3% vs. 76.5%). Compared to SC, management within IC concept was associated with statistically significantly higher subjective patient satisfaction (beta = 0.93; p < 0.001) with the strongest effects observed in satisfaction with treatment accessibility and availability (beta = 2.05; p < 0.001). Subgroup analyses of patients with migration background and language barrier consistently indicated stronger effects of IC in these groups. CONCLUSIONS: Interdisciplinary integrated management of IIH statistically significantly and clinically meaningfully improves patient satisfaction - particularly in socioeconomically underprivileged patient groups. Providing structured central coordination to facilitate and improve access to interdisciplinary management provides means to further improve outcome.

Asunto(s)

Instituciones de Atención Ambulatoria , Satisfacción del Paciente , Seudotumor Cerebral , Humanos , Femenino , Masculino , Adulto , Seudotumor Cerebral/terapia , Estudios Retrospectivos , Instituciones de Atención Ambulatoria/organización & administración , Prestación Integrada de Atención de Salud , Grupo de Atención al Paciente/organización & administración , Austria , Persona de Mediana Edad

RESUMEN

Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is characterized by multiple phenotypic conditions such as acute disseminated encephalomyelitis, optic neuritis, and myelitis. MOGAD's spectrum is expanding, with potential symptoms of increased intracranial pressure that are similar to idiopathic intracranial hypertension (IIH). We report a boy with new-onset continuous headache and a brain MRI at onset suggesting idiopathic intracranial hypertension (IIH). The patient showed resistance to treatment with acetazolamide and, after one month, developed optic neuritis in the left eye. Laboratory tests documented positive MOG antibodies (anti-MOG) in the serum. The final diagnosis was MOGAD, with the initial symptoms resembling IIH.

RESUMEN

BACKGROUND: Topiramate is often considered as a second-line medication for the treatment of pseudotumor cerebri syndrome (PTCS), but limited studies exist that evaluate its efficacy in children. METHODS: Retrospective study of patients aged <21 years with PTCS who were treated with topiramate alone or in combination with acetazolamide was performed. Data regarding clinical courses and visual outcomes were recorded. RESULTS: A total of 46 patients were identified. Three (6.5%) patients were treated with topiramate alone, 31 (67.4%) transitioned to topiramate from acetazolamide, and 12 (26.1%) took both topiramate and acetazolamide concurrently. The median time to resolution of papilledema on topiramate was 0.57 years (interquartile range 0.32 to 0.84). Among eyes with papilledema graded on the Frisen scale at topiramate initiation, 40 of 57 (70.2%) were grade 1, nine of 57 (15.8%) were grade 2, and eight of 57 (14.0%) were grade 3. Twenty-seven of 46 (58.7%) reported headache improvement after starting topiramate. The mean dose of topiramate was 1.3 ± 0.8 mg/kg/day. The most common side effect was patient report of cognitive slowing (10 of 46 [21.7%]). All patients on topiramate monotherapy who were compliant with treatment and follow-up had resolution of papilledema with no evidence of visual function loss. CONCLUSIONS: Topiramate can effectively treat PTCS in children with mild to moderate papilledema or in those unable to tolerate acetazolamide. More research is needed to assess the efficacy of topiramate for higher grade papilledema.

Asunto(s)

Acetazolamida , Seudotumor Cerebral , Topiramato , Humanos , Topiramato/administración & dosificación , Topiramato/efectos adversos , Topiramato/farmacología , Seudotumor Cerebral/tratamiento farmacológico , Seudotumor Cerebral/inducido químicamente , Niño , Femenino , Masculino , Estudios Retrospectivos , Acetazolamida/efectos adversos , Acetazolamida/uso terapéutico , Acetazolamida/administración & dosificación , Adolescente , Papiledema/tratamiento farmacológico , Papiledema/inducido químicamente , Anticonvulsivantes/efectos adversos , Anticonvulsivantes/administración & dosificación , Preescolar , Resultado del Tratamiento , Quimioterapia Combinada , Inhibidores de Anhidrasa Carbónica/efectos adversos , Inhibidores de Anhidrasa Carbónica/administración & dosificación , Fructosa/análogos & derivados , Fructosa/efectos adversos , Fructosa/uso terapéutico , Fructosa/administración & dosificación

RESUMEN

PURPOSE: Invasive methods such as lumbar puncture and intraventricular catheters are commonly used to measure intracranial pressure (ICP). This study aims to develop quantitative and non-invasive techniques to measure ICP in patients with Idiopathic Intracranial Hypertension (IIH) using magnetic resonance imaging (MRI) findings. METHODS: MRI data obtained from 50 patients with IIH and 30 age- and sex- matched controls were analyzed and optic nerve sheath diameter (ONSD), eyeball transverse diameter (ETD) and optic nerve diameter (OND) were measured. ONSD, ONSD/ETD and OND/ONSD indexes were calculated according to different ONSD measurement distances. Correlations of MRI findings with ICP were calculated. Sensitivity and specificity of all methods were analyzed. RESULTS: ONSD and ONSD/ETD index at 3 mm and 10 mm behind the eyeball were significantly higher (p < 0.001) and OND/ONSD index at 3 mm behind the eyeball was significantly lower (p < 0.001) in the IIH group. The ONSD/ETD index at 3 mm had the highest area under the curve (AUC) value (0.898) with a cut-off of 0.27 mm (82% sensitivity and 91.67% specificity) for predicting high cerebrospinal fluid (CSF) pressure, followed by ONSD measurements at 3 mm (AUC = 0.886) with a cut-off of 6.17 mm (83% sensitivity and 86.67% specificity). The OND/ONSD index at 3 mm posterior to the eyeball decreased significantly as ICP increased, and the strength of the relationship was moderate (p < 0.001; r = -0.358). CONCLUSIONS: ONSD and ONSD/ETD index measured on MRI sequences are potentially useful in detecting elevated ICP. The OND/ONSD index correlates with CSF pressure and these techniques may be helpful in diagnosing IIH.

RESUMEN

Bilateral edematous optic disc swelling from papilledema is caused by elevated intracranial pressure (ICP). Idiopathic intracranial hypertension (IIH), a clinical syndrome with elevated ICP of unclear etiology, is a frequent cause of this condition. IIH typically affects obese middle-aged females. Papilledema usually has a fairly symmetrical bilateral pattern. Unilateral papilledema is a rare disorder that must be detected early to avoid optic nerve damage. However, the etiology of unilateral papilledema remains unclear. Based on bilateral optic nerve sheath diameter measurements, we aimed to find an explanation for the unilaterality in this rare case.

Asunto(s)

Nervio Óptico , Papiledema , Humanos , Papiledema/diagnóstico , Papiledema/etiología , Femenino , Nervio Óptico/patología , Nervio Óptico/diagnóstico por imagen , Disco Óptico , Adulto , Presión Intracraneal , Agudeza Visual , Tomografía de Coherencia Óptica/métodos , Seudotumor Cerebral/diagnóstico , Seudotumor Cerebral/complicaciones , Imagen por Resonancia Magnética