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Neuro-ophthalmology (Aeolus Press) 2024Fulminant idiopathic intracranial hypertension (IIH) is a rapid vision-degrading presentation of IIH with limited published studies. This study composed a narrative... (Review)
Review
Fulminant idiopathic intracranial hypertension (IIH) is a rapid vision-degrading presentation of IIH with limited published studies. This study composed a narrative review of fulminant IIH with the aim of better characterising fulminant IIH presentation and visual outcomes. SCOPUS and PubMed were searched for papers referencing IIH, benign intracranial hypertension, or pseudotumour cerebri. Abstracts were screened for rapid degradation in vision. All studies were required to meet both the modified Dandy and fulminant IIH criteria. Thirty-six studies met the inclusion criteria. Demographics, treatments, and visual outcome data were collected. Case studies made up 69% of the studies and 31% were case series. In total, 72 patients with fulminant IIH were reported, of which 23.6% were paediatric and 96% were female. Surgical intervention occurred in 85% of patients. Anaemia was present in 11% of patients and 85.7% of paediatric patients had a sixth cranial nerve palsy. In conclusion, we propose the following practice guidelines to assist in diagnosing and treating fulminant IIH patients: 1) patients who present with optic disc oedema require urgent visual field testing to evaluate for vision loss; 2) a paediatric patient presenting with a sixth cranial nerve palsy should have a comprehensive eye examination; 3) fulminant IIH can occur in patients with a normal body mass index; and 4) anaemia should be tested for in the setting of fulminant IIH. As little is known about the optimal treatment mechanisms for this presentation, multi-institutional and international collaborations will be a critical step for future research.
PubMed: 38933750
DOI: 10.1080/01658107.2023.2301358 -
Neuro-ophthalmology (Aeolus Press) 2024Nitrous oxide is used as an anaesthetic and analgesic agent in the medical setting and is known to cause raised intracranial pressure. The use of nitrous oxide...
Nitrous oxide is used as an anaesthetic and analgesic agent in the medical setting and is known to cause raised intracranial pressure. The use of nitrous oxide recreationally for the drug's euphoric and relaxant properties has been linked to multiple neurological and psychiatric sequelae including neuropathy, myelopathy, and psychosis. We describe a case of a young person who declared heavy nitrous oxide use resulting in vision-threatening papilloedema secondary to raised intracranial pressure. He underwent emergency lumbar drainage alongside high-dose acetazolamide and parenteral vitamin B injections. To our knowledge, there have yet to be other reports of cases where heavy nitrous oxide use has caused secondary pseudotumor cerebri syndrome.
PubMed: 38933749
DOI: 10.1080/01658107.2023.2301359 -
The Journal of Headache and Pain Jun 2024Currently, there is a relative lack of detailed reports regarding clinical presentation and outcome of idiopathic intracranial hypertension in Asians. This study aims to...
BACKGROUND
Currently, there is a relative lack of detailed reports regarding clinical presentation and outcome of idiopathic intracranial hypertension in Asians. This study aims to describe the clinical features and treatment outcomes of Korean patients with idiopathic intracranial hypertension.
METHODS
We prospectively recruited patients with idiopathic intracranial hypertension from one hospital and retrospectively analyzed the medical records of 11 hospitals in Korea. We collected data regarding preceding medical conditions or suspected medication exposure, headache phenotypes, other associated symptoms, detailed neuroimaging findings, treatments, and outcomes after 1-2 and 3-6 months of treatment.
RESULTS
Fifty-nine (83.1% women) patients were included. The mean body mass index was 29.11 (standard deviation, 5.87) kg/m; only 27 patients (45.8%) had a body mass index of ≥ 30 kg/m. Fifty-one (86.4%) patients experienced headaches, patterns of which included chronic migraine (15/51 [29.4%]), episodic migraine (8/51 [15.7%]), probable migraine (4/51 [7.8%]), chronic tension-type headache (3/51 [5.9%]), episodic tension-type headache (2/51 [3.9%]), probable tension-type headache (2/51 [3.9%]), and unclassified (17/51 [33.3%]). Medication overuse headache was diagnosed in 4/51 (7.8%) patients. After 3-6 months of treatment, the intracranial pressure normalized in 8/32 (25.0%), improved in 17/32 (53.1%), no changed in 7/32 (21.9%), and worsened in none. Over the same period, headaches remitted or significantly improved by more than 50% in 24/39 patients (61.5%), improved less than 50% in 9/39 (23.1%), and persisted or worsened in 6/39 (15.4%) patients.
CONCLUSION
Our findings suggest that the features of Asian patients with idiopathic intracranial hypertension may be atypical (i.e., less likely obese, less female predominance). A wide spectrum of headache phenotypes was observed. Medical treatment resulted in overall favorable short-term outcomes; however, the headaches did not improve in a small proportion of patients.
Topics: Humans; Female; Male; Republic of Korea; Adult; Treatment Outcome; Pseudotumor Cerebri; Retrospective Studies; Middle Aged; Young Adult; Prospective Studies
PubMed: 38918698
DOI: 10.1186/s10194-024-01794-3 -
Acta Radiologica (Stockholm, Sweden :... Jun 2024The goals of neuroimaging in idiopathic intracranial hypertension (IIH) are the exclusion of mimickers and effective management of disease. In recent studies, several...
BACKGROUND
The goals of neuroimaging in idiopathic intracranial hypertension (IIH) are the exclusion of mimickers and effective management of disease. In recent studies, several imaging markers have been identified as potential predictors of IIH.
PURPOSE
To investigate the predictive roles of novel radiological markers as the Meckel's cave area, alongside classical radiologic markers in identifying IIH such as the empty sella.
MATERIAL AND METHODS
The patients were classified according to cerebrospinal fluid (CSF) opening pressure as the IIH group and control group. The observational, case-control study included 22 patients with IIH and 22 controls. Groups were compared for presence of empty sella, Meckel's cave area, fat area of posterior neck, fat thickness of scalp, presence of transverse sinus stenosis, and ophthalmic markers, such as increase of optic nerve (ON) sheath diameter.
RESULTS
In the IHH group, higher occurrences of increased ON sheath diameter, ON tortuosity, flattening of the scleral surface, and transverse sinus stenosis were observed ( < 0.001, < 0.001, = 0.046, and = 0.021, respectively). Meckel's cave area and fat area of posterior neck were similar in both groups ( = 0.444 and = 0.794).
CONCLUSION
Ophthalmic markers and transverse sinus stenosis could be utilized as radiologic features supporting early and precise diagnosis of IIH. However, enlargement of Meckel's cave area and measurements of fatty area of posterior neck are not helpful for diagnosis of IIH.
PubMed: 38870347
DOI: 10.1177/02841851241256008 -
The Journal of Headache and Pain Jun 2024Idiopathic intracranial hypertension (IIH) is a debilitating condition characterized by increased intracranial pressure often presenting with chronic migraine-like...
BACKGROUND
Idiopathic intracranial hypertension (IIH) is a debilitating condition characterized by increased intracranial pressure often presenting with chronic migraine-like headache. Calcitonin gene-related peptide (CGRP) plays an important pathophysiological role in primary headaches such as migraine, whilst its role in IIH has not yet been established.
METHODS
This longitudinal exploratory study included patients with IIH, episodic migraine (EM) in a headache-free interval and healthy controls (HC). Blood samples were collected from a cubital vein and plasma CGRP (pCGRP) levels were measured by standardized ELISA.
RESULTS
A total of 26 patients with IIH (mean age 33.2 years [SD 9.2], 88.5% female, median BMI 34.8 kg/m [IQR 30.0-41.4]), 30 patients with EM (mean age 27.6 years [7.5], 66.7% female) and 57 HC (mean age 25.3 years [5.2], 56.1% female) were included. pCGRP levels displayed a wide variation in IIH as well as in EM and HC on a group-level. Within IIH, those with migraine-like headache had significantly higher pCGRP levels than those with non-migraine-like headache (F = 84.79; p < 0.001) and headache absence (F = 84.79; p < 0.001) throughout the observation period, explaining 14.7% of the variance in pCGRP levels. CGRP measurements showed strong intraindividual agreement in IIH (ICC 0.993, 95% CI 0.987-0.996, p < 0.001). No association was found between pCGRP levels and ophthalmological parameters.
CONCLUSIONS
Although interindividual heterogeneity of pCGRP levels is generally high, migraine-like headache seems to be associated with higher pCGRP levels. CGRP may play a role in the headache pathophysiology at least in a subgroup of IIH.
Topics: Humans; Female; Male; Adult; Calcitonin Gene-Related Peptide; Pseudotumor Cerebri; Migraine Disorders; Longitudinal Studies; Young Adult; Biomarkers
PubMed: 38834953
DOI: 10.1186/s10194-024-01799-y -
Neurology Jul 2024Idiopathic intracranial hypertension (IIH) is a neurologic disorder characterized by symptoms of elevated intracranial pressure in the absence of a clear cause. There is...
BACKGROUND AND OBJECTIVES
Idiopathic intracranial hypertension (IIH) is a neurologic disorder characterized by symptoms of elevated intracranial pressure in the absence of a clear cause. There is a developing theory that IIH may, in part, be related to abnormal cerebral glymphatic clearance. In addition, transverse sinus stenosis (TSS) is a common finding in IIH of unclear pathophysiologic significance. Similarly, whether or not TSS is associated with glymphatic outflow in IIH is unknown. The aim of this investigation was to explore the possible association between glymphatic outflow and extent of TSS in patients with IIH.
METHODS
The study cohort consisted of patients with IIH and healthy controls who were retrospectively identified from our tertiary care institution located in upstate New York from 2016 to 2023. Patients with IIH were included if they had brain MRIs completed with sufficient sequences for analysis. Brain MRIs were computationally analyzed using diffusion tensor imaging analysis along the perivascular space technique to quantify the glymphatic function in patients with IIH. Glymphatic clearance, the primary outcome, was then correlated with the degree of TSS on MR venography using 2 different scoring systems, the 'Farb score' and 'Carvalho score.'
RESULTS
Overall, 81 patients with IIH (70 [86%] female, mean age 29.8 years [SD: 8.2 years], mean BMI 41 [SD: 8.4]) and 10 normal controls were identified with sufficient imaging. Based on the Carvalho TSS score, IIH patients without TSS had significantly lower glymphatic clearance than healthy controls (mean ALPS index: 1.196 [SD: 0.05] vs 1.238 [SD: 0.04], respectively; = 0.018). Furthermore, IIH patients with TSS had significantly lower glymphatic outflow than healthy controls (1.129 [SD: 0.07] vs 1.238 [SD: 0.04], respectively; < 0.0001) and IIH patients without TSS (1.129 [SD: 0.07] vs 1.196 [SD: 0.05], respectively; < 0.0001). In addition, there was a significant association between increasing extent of TSS and declining glymphatic clearance ( < 0.0001, R = 0.62). Finally, IIH patients with severe TSS had significantly lower glymphatic flow than IIH patients with mild stenosis (1.121 [SD: 0.07] vs 1.178 [SD: 0.05], respectively; < 0.0001). These findings were similarly recapitulated using the Farb TSS scoring system.
DISCUSSION
These preliminary findings suggest that the extent of TSS is associated with the degree of glymphatic clearance in IIH, providing novel insights into IIH pathophysiology. Further research is required to clarify the possible causal relationship between TSS and impaired glymphatic clearance in IIH.
Topics: Humans; Female; Male; Glymphatic System; Adult; Pseudotumor Cerebri; Retrospective Studies; Constriction, Pathologic; Transverse Sinuses; Young Adult; Middle Aged; Magnetic Resonance Imaging; Diffusion Tensor Imaging
PubMed: 38833652
DOI: 10.1212/WNL.0000000000209529 -
Japanese Journal of Radiology May 2024Venous outflow impediment is increasingly recognized in idiopathic intracranial hypertension (IIH). We aim to search for the value of tortuous occipital emissary vein...
PURPOSE
Venous outflow impediment is increasingly recognized in idiopathic intracranial hypertension (IIH). We aim to search for the value of tortuous occipital emissary vein (OEV) in IIH by integrating measurable transverse sinus (TS) stenosis in contrast-enhanced MR venography (CE-MRV).
METHODS
Twenty-one IIH patients were evaluated with CE-MRV. Each patient had high LP opening pressure (> 25 cm.HO), and presented papilledema. Age- and sex-matched 21 control subjects who underwent cranial CE-MRV were selected. The OEV and the following features: intraosseous diameter of more than 3 mm, twisted course, and continuous with prominent sub-occipital extracranial veins were named tortuous OEV. TS stenosis was measured by utilizing the coronal T1-VIBE series to calculate quantitative metrics such as TS . The tortuous OEV and TS were registered to create a bivariate logistic regression model to assess the performance of tortuous OEV for IIH when accompanied by TS stenosis.
RESULTS
Six (29%) tortuous OEVs were observed in the IIH group, while no tortuous OEV was identified in the control group (p = 0.021). The mean TS was 2.48 ± 1.19 in patients with IIH and 1.23 ± 0.33 in the control group (p < 0.001). According to regression analysis, tortuous OEV is not predictive of IIH (p = 0.999), while the higher TS (> 1.69) is predictive of IIH (p = 0.022, OR: 8.9; %95 CI; 1.4-59.0) when accompanied together.
CONCLUSION
Tortuous occipital emissary vein is more frequently seen in patients with IIH. However, the tortuous appearance alone does not predict idiopathic intracranial hypertension unless associated with measurable transverse sinus narrowing in CE-MRV.
PubMed: 38805118
DOI: 10.1007/s11604-024-01598-0 -
Expert Review of Neurotherapeutics Jul 2024Idiopathic intracranial hypertension is a neurological condition characterized by a raised intracranial pressure and papilledema, leading to chronic headaches and visual... (Review)
Review
INTRODUCTION
Idiopathic intracranial hypertension is a neurological condition characterized by a raised intracranial pressure and papilledema, leading to chronic headaches and visual disturbances. By recognizing modifiable risk factors and deploying evidence-based interventions, healthcare providers have the potential to mitigate the burden of the disease and improve patient outcomes.
AREAS COVERED
It is well known that the condition occurs in predominantly pre-menopausal females who live with obesity particularly in the context of recent weight gain. This review discusses what risk factors may contribute to the metabolic underpinnings of cerebrospinal fluid dysregulation. There are a number of disease associations that are important to screen for as they can alter management.
EXPERT OPINION
There is emerging evidence to suggest that idiopathic intracranial hypertension is a systemic metabolic disease and it is unknown what are all the risk factors are that precipitate the condition. Targeting certain hardwired risk factors is unachievable. However, as recent weight gain has been identified as a predominant risk factor for the development of the disease and relapse, modification of body weight should be the primary aim of management. Insightful research into the involvement of the neuroendocrine axis driving cerebrospinal fluid dysregulation now has the potential for the development of therapeutic targets.
Topics: Humans; Risk Factors; Pseudotumor Cerebri; Obesity; Weight Gain; Female
PubMed: 38803205
DOI: 10.1080/14737175.2024.2359420 -
Cureus Apr 2024Idiopathic intracranial hypertension (IIH), formerly known as pseudotumor cerebri, represents a challenging diagnostic entity in neurology, characterized by elevated...
Idiopathic intracranial hypertension (IIH), formerly known as pseudotumor cerebri, represents a challenging diagnostic entity in neurology, characterized by elevated intracranial pressure of unknown origin. The classical clinical triad of headache, visual disturbances, and papilledema provides a well-established framework for diagnosis; however, the heterogeneity of IIH presentations, combined with the absence of an overt causative factor, continues to perplex clinicians. This case report delves into the complexities of a rare IIH presentation in a 32-year-old male, highlighting the need for a nuanced understanding of this condition beyond its traditional confines.
PubMed: 38800215
DOI: 10.7759/cureus.59072 -
Neurosurgery Clinics of North America Jul 2024Idiopathic intracranial hypertension is defined by headaches and a decline in visual acuity due to increased intracranial pressure. Treatment options historically...
Idiopathic intracranial hypertension is defined by headaches and a decline in visual acuity due to increased intracranial pressure. Treatment options historically included weight loss, acetazolamide, and/or cerebrospinal fluid diversion surgery. Recent understanding of the contributions of dural venous sinus hypertension and stenosis has led to venous sinus stenting as a treatment option.
Topics: Humans; Acetazolamide; Cranial Sinuses; Intracranial Hypertension; Pseudotumor Cerebri; Stents
PubMed: 38782521
DOI: 10.1016/j.nec.2024.02.001