Presentation Of Idiopathic Intracranial Hypertension Research Articles

Atypical Presentation of Idiopathic Intracranial Hypertension: A Case Series and Literature Review.

Idiopathic intracranial hypertension (IIH) is a condition in which intracranial pressure (ICP) increases without an apparent cause. Typically, patients present with headaches, dizziness, pulsatile tinnitus, visual disturbances, blurred vision, diplopia, photophobia, visual field defects, and papilledema on fundoscopy. The association between IIH, spontaneous cerebrospinal fluid (CSF) rhinorrhea, and arachnoid cysts has been discussed in the literature; however, there is no clear explanation for this association. We aimed to present a series of four patients with a confirmed diagnosis of IIH with atypical presentations, discuss the management of each case, and provide an explanation for this association to alert clinicians to the atypical presentation of IIH and facilitate early diagnosis and proper treatment of this condition by CSF diversion. This was a retrospective case series of all patients who were diagnosed with IIH and showed improvement after ventriculoperitoneal shunt insertion after failure of at least one operative intervention resulting from primary radiological and clinical findings in 2001 to 2022. Data on demographics, clinical presentation, radiological findings, surgical management, and diagnostic criteria for IIH were recorded. We identified four patients with a confirmed diagnosis of IIH who presented with atypical presentations as follows: intracranial arachnoid cyst, cervical spine arachnoid cyst, giant Virchow perivascular space, and spontaneous CSF (CSF) rhinorrhea. All patients responded to CSF diversion after failure of surgical treatment targeting the primary pathology. IIH should be suspected after the failure of primary surgical treatment in cases of spontaneous CSF rhinorrhea, spinal and cranial arachnoid cysts, and symptomatic ventriculoperitoneal shunt. Treatment in such situations should be directed toward IIH with CSF diversion.

Headache Phenotypes in Idiopathic Intracranial Hypertension and Its Short-Term Outcomes: A Retrospective Case Series Study.

Idiopathic intracranial hypertension (IIH) presents a complex physiopathology, leading into diverse manifestations, notably variable headache phenotypes. Furthermore, its frequent overlap with migraine complicates the evaluation of treatment benefit for IIH-related headache. Our aim was to investigate if there is any relationship between demographic factors, clinical patterns of headache, treatment response, and headache short-term outcome with the headache phenotype of IIH. This study was a retrospective analysis of demographic, clinical, and treatment features of patients with idiopathic intracranial hypertension presenting with headache and evaluation of headache outcomes in the first 12 months following treatment. Thirty-two patients were included (median age of onset 29.0 years (interquartile range 25.0 - 38.5), 90% females, median body mass index 32.5 kg/m2; 87.5% (n = 28) with papilledema; median cerebrospinal fluid opening pressure 36.5 cm H2O). Patients presented with migraine (n = 11, 34.4%), tension-type (n = 9, 28.1%), or a not-classifiable headache (n = 12, 37.5%). Regarding treatment and short-term follow-up (12 months), there was a failure of medical treatment in 43.8% (n = 14) and a reduction of headaches (≥ 50%) in 62.5% (n = 20) of the patients. Among headache phenotypes, there were no significant differences regarding demographics, clinical features, clinical patterns, or treatment response at baseline. Also, there were no differences regarding response to treatment or headache outcomes in 1, 3, 6, and 12 months of follow-up. In our study, migraine and unclassifiable types were the most commonly reported headache phenotypes. Headache phenotype does not appear to be an essential factor in allowing clinical distinction, treatment response, or predicting the short-term headache outcome of this intriguing entity.

Impact of weight reduction on structural and functional parameters of the optic nerve in idiopathic intracranial hypertension.

To evaluate the effect of weight reduction on the structural and functional parameters of the optic nerve in established cases of papilledema in Idiopathic Intracranial Hypertension (IIH). A prospective observational study in early and established cases of papilledema in IIH presenting from December 2019 to February 2021. Functional parameters (visual acuity, contrast sensitivity, mean deviation, VER), structural parameters (RNFL, GCL-IPL, and optic disc height), and clinical grading of papilledema were measured at baseline and every 6 weeks for 6 months. All patients underwent medical (oral acetazolamide) and diet therapy. Mean body mass index (BMI) at presentation was 26.32 ± 3.52 kg/m² and the mean change in BMI over 6 months was 1.27 ± 0.50 kg/m². 67% of eyes had papilledema of grades 2-3 at the presentation. At the end of 6 months, 75% of eyes had grade 1 papilledema while 13% showed complete resolution. A reduction of 5.32 ± 3.58 in mean deviation (r = 0.316; p-value 0.01) and gain in VER P100 amplitude of 4.2 ± 2.7 µV (r = 0.40; p-value 0.003) were noted over 6 months with reduction in BMI over 6 months. A statistically significant reduction in optic disc height (ODH) was noticed with BMI reduction over 6 months (p = 0.0007; r = 0.45).A reduction of 1.72 ± 0.90 kg/m² (equivalent to 6.53% weight loss) in BMI was associated with a four-grade change in clinical grading of papilledema. Reduction in BMI had a significant impact on both structural and functional parameters of the optic nerve in IIH. The involvement of a well-certified dietician should be an integral part of treatment in IIH.

Simultaneous Neuro-Ophthalmic Presentation and Progression of Idiopathic Intracranial Hypertension and Multiple Sclerosis.

Simultaneous Neuro-Ophthalmic Presentation and Progression of Idiopathic Intracranial Hypertension and Multiple Sclerosis.

Transverse sinus pathologies, vestibular migraine and intracranial hypertension without papilledema

BackgroundIn the absence of papilledema, the presentation of migraine and idiopathic intracranial hypertension (IIH) is very similar. In this respect, an IIH could be presented as a vestibular migraine. Our main objective in this case report is to demonstrate the similarities between IIH and vestibular migraine. CasesThis is a report of 14 patients who have IIH without papilledema presented as vestibular migraine to the clinic and followed from 2020 to 2022. ResultsThe common presentation of patients was ear-facial pain, dizziness, and frequent pulsatile tinnitus. One-fourth of the patients reported episodes of true episodic vertigo. The average age was 37.8, the average BMI was 37.4, and the average lumbar puncture-opening pressure was 25.6 cm H2O. Transverse sinus venous flow alterations caused neuroimaging findings of sigmoid sinus dehiscence, empty sella, or tonsillar ectopia. Most patients improved with carbonic anhydrase inhibitors, and one patient was treated with a dural sinus stent. ConclusionA transverse sinus stenosis, even in the non-dominant site, may elevate the CSF pressure in obese individuals. This stenosis causes dural sinus-related pulsatile tinnitus with characteristics different from those of an arterial origin. Dizziness is a common complaint in patients with IIH, just like VM. In our opinion, episodic vertigo in these patients is the direct effect of CSF flow alterations into the inner ear's vestibule. Patients with mild elevations will be presented to the clinic, similar to migraines with or without the presence of pulsatile tinnitus. Treatment requires lowering intracranial pressure and managing migraine symptoms.

Idiopathic intracranial hypertension presenting with migraine phenotype is associated with unfavorable headache outcomes.

To assess the prognostic impact of migraine headache in idiopathic intracranial hypertension (IIH). Migraine headache is common in IIH, but it is unclear whether it has prognostic relevance. We investigated patients with IIH from the Vienna-IIH-database and differentiated migraine (IIH-MIG) from non-migraine headache (IIH-nonMIG) and without headache (IIH-noHA). Using multivariable models, we analyzed the impact of IIH-MIG on headache and visual outcomes 12 months after diagnosis. Among 97 patients (89% female, mean [SD] age 32.9[11.1]years, median body mass index 32.0 kg/m2 , median cerebrospinal fluid opening pressure 310 mm), 46% were assigned to IIH-MIG, 37% to IIH-nonMIG (11% tension-type, 26% unclassifiable), and 17% to IIH-noHA. Overall, headache improvement was achieved in 77% and freedom of headache in 28%. The IIH-MIG group showed significantly lower rates for headache improvement (67% vs. 89% in IIH-nonMIG, p=0.019) and freedom of headache (11% vs. 33% in IIH-nonMIG and 63% in IIH-noHA, p=0.015). These differences persisted when only analyzing patients with resolved papilledema at follow-up. In contrast, visual worsening was significantly less common in IIH-MIG (9% vs. 28% in IIH-nonMIG and 31% in IIH-noHA, p=0.045). In multivariable models, IIH-MIG was associated with a significantly lower likelihood of achieving headache improvement (odds ratio [OR] 0.57, 95% confidence interval [CI] 0.40-0.78, p < 0.001) and freedom of headache (OR 0.29, 95% CI 0.12-0.46, p < 0.001), but also a lower risk for visual worsening (OR 0.26, 95% CI 0.04-0.82, p < 0.001). In IIH, migraine headache is associated with unfavorable outcomes for headache, even when papilledema has resolved, and possibly favorable visual outcome.

Idiopathic Intracranial Hypertension Presenting as Seizures

Idiopathic Intracranial Hypertension is a condition characterised by raised cerebrospinal fluid pressure of unknown etiology seen predominantly in obese, middle aged patients. The typical clinical features include headache, pulsatile tinnitus, diplopia and transient visual obscurations. Seizures are a rare symptom seen in IIH patients. We present a case of a young, obese female patient who presented with headache and seizures. Imaging showed evidence of Idiopathic Intracranial Hypertension with temporal lobe encephalocoeles and brain herniation into arachnoid granulations. The diagnosis was subsequently confirmed by clinical workup and she was started on treatment for the same.

Idiopathic Intracranial Hypertension Presenting With Postural and Exercise-Induced Hemifacial Spasm.

Idiopathic Intracranial Hypertension Presenting With Postural and Exercise-Induced Hemifacial Spasm.

Idiopathic Intracranial Hypertension Presenting With Completely Asymmetric Visual Function.

Idiopathic Intracranial Hypertension Presenting With Completely Asymmetric Visual Function.

Optical coherence tomography features and correlation of functional and structural parameters in patients of idiopathic intracranial hypertension.

Purpose:To determine the correlation between functional parameters and optical coherence tomography (OCT) features in patients of idiopathic intracranial hypertension (IIH).Methods:A prospective observational study in early and established cases of papilledema in IIH presenting from December 2017 to February 2019. Functional parameters (visual acuity, contrast sensitivity, mean deviation, VER, and MfERG) and structural parameters (RNFL, GCL-IPL, and optic disc height) were measured at baseline and every 6 weeks for 6 months.Results:At baseline, average RNFL had a moderate negative correlation with mean deviation (r = −0.45; P = 0.0007) and a positive correlation with logMAR visual acuity (r = 0.18; P = 0.17). On the contrary, baseline GCL and logMAR visual acuity had a negative correlation (r = −0.4, P = 0.02). Optic disc height (ODH) had a negative correlation with visual field mean deviation (r = −0.046; P = 0.0005). At 6 months, ODH and GCL-IPL complex had a statistically significant correlation with functional parameters. However, RNFL values did not show any significant correlation with any of the functional parameters. Baseline GCL-IPL and optic disc height values had a moderate and significant correlation with final functional parameters. However, RNFL did not show any correlation with final functional parameters. Correlation between GCL-IPL thickness at 6 weeks and final functional parameters were stronger than that with baseline GCL valuesConclusion:In the setting of severe papilledema, RNFL can misguide the prognosis. GCL-IPL can be a valuable tool for an objective evaluation of the integrity of the optic nerve in IIH and ODH may be used as an alternative or in combination with GCL-IPL in these cases.

Atypical idiopathic intracranial hypertension presenting as cyclic vomiting syndrome: a case report

BackgroundIdiopathic intracranial hypertension is a disorder of increased intracranial pressure in the absence of cerebrospinal outflow obstruction, mass lesion, or other underlying cause. It is a rare phenomenon in prepubertal children and is most typically found in women of childbearing age. The classic presentation consists of headaches, nausea, vomiting, and visual changes; however, children present more atypically. We report a case of idiopathic intracranial hypertension in an otherwise healthy, 4-year-old child with atypical symptoms resembling those of cyclic vomiting syndrome.Case presentationA 4-year-old Caucasian, otherwise healthy, male child presented to our emergency department with episodic intermittent early-morning vomiting occurring once every 1–3 weeks without interepisodic symptoms, starting 10 months prior. With outpatient metabolic, autoimmune, endocrine, allergy, and gastroenterology work-up all unremarkable, he was initially diagnosed with cyclic vomiting syndrome. Discovery of mild optic nerve sheath distension on magnetic resonance imaging of the brain 10 months after symptom onset led to inpatient admission and a lumbar puncture notable for an opening pressure of 47 mmHg, with normal cell count and protein levels. He had no changes in visual acuity or optic disc edema on dilated fundoscopic examination. The patient was started on acetazolamide, with resolution of episodic emesis at his last follow-up visit 12 weeks after discharge.ConclusionsIdiopathic intracranial hypertension presents atypically in prepubescent children, with about one-fourth presenting asymptomatically, and only 13–52% presenting with “classic” symptoms. With a prevalence of only 0.6–0.7 per 100,000, much remains unknown regarding the underlying pathophysiology in this demographic. Cyclic vomiting syndrome, however, has a much higher prevalence in this age group, with a prevalence of 0.4–1.9 per 100. It is thought to be an idiopathic, periodic disorder of childhood, often linked to neurological conditions such as abdominal migraines, epilepsy, mitochondrial disorders, and structural lesions such as chiari malformation and posterior fossa tumors. While cyclic vomiting syndrome is thought to have a benign course, untreated idiopathic intracranial hypertension can have long-term detrimental effects, such as visual loss or even blindness. We present a case of idiopathic intracranial hypertension presenting with symptoms resembling cyclic vomiting syndrome in a 4-year-old child, diagnosed 10 months after initial onset of symptoms. We aim to demonstrate the need for a high level of clinical suspicion and the need for further investigation into underlying pathophysiology in this vulnerable population.

Idiopathic intracranial hypertension presenting as acute bilateral loss of vision

cute bilateral vision loss is a very rare presentation of idiopathic intracranial hypertension (IIH). We report the case of a 17-year-old female student who presented with a history of moderate degree headache from 7 days and severe loss of vision of 6 days duration. The vision loss initially affected the right eye and over the next 2 days, her left eye was also involved. Her visual acuity in both eyes was 6/60 and she had a relative afferent pupillary defect on the right side. Fundus examination showed bilateral papilledema and perimetry showed severe bilateral constriction of visual fields with the involvement of central areas as well. Cerebrospinal fluid opening pressure was raised and magnetic resonance imaging showed signs suggestive of IIH. She was managed with acetazolamide, topiramate, and lumbar drainage. She showed a gradual improvement in her vision, and finally, her visual acuity returned to normal.

A Unique Subset: Idiopathic Intracranial Hypertension Presenting as Spontaneous CSF Leak of the Anterior Skull Base.

Introduction Spontaneous cerebrospinal fluid (CSF) leaks represent a unique clinical presentation of idiopathic intracranial hypertension (IIH), lacking classical features of IIH, including severe headaches, papilledema, and markedly elevated opening pressures. Methods Following a single-institution retrospective review of patients undergoing spontaneous CSF leak repair, we performed a literature review of spontaneous CSF leak in patients previously undiagnosed with IIH, querying PubMed. Results Our literature review yielded 26 studies, comprising 716 patients. Average age was 51 years with 80.8% female predominance, and average body mass index was 35.5. Presenting symptoms included headaches (32.5%), visual disturbances (4.2%), and a history of meningitis (15.3%). Papilledema occurred in 14.1%. An empty sella was present in 77.7%. Slit ventricles and venous sinus stenosis comprised 7.7 and 31.8%, respectively. CSF leak most commonly originated from the sphenoid sinus (41.1%), cribriform plate (25.4%), and ethmoid skull base (20.4%). Preoperative opening pressures were normal at 22.4 cm H 2 O and elevated postoperatively to 30.8 cm H 2 O. 19.1% of patients underwent shunt placement. CSF leak recurred after repair in 10.5% of patients, 78.6% involving the initial site. A total of 85.7% of these patients were managed with repeat surgical intervention, and 23.2% underwent a shunting procedure. Conclusion Spontaneous CSF leaks represent a distinct variant of IIH, distinguished by decreased prevalence of headaches, lack of visual deficits, and normal opening pressures. Delayed measurement of opening pressure after leak repair may be helpful to diagnose IIH. Permanent CSF diversion may be indicated in patients exhibiting significantly elevated opening pressures postoperatively, refractory symptoms of IIH, or recurrent CSF leak.

Lumbo-peritoneal shunt as treatment modality for visual symptoms in idiopathic intracranial hypertension: A single institution experience

ObjectivesTo evaluate the efficacy of lumbo-peritoneal shunt (LPS) in patients of idiopathic intracranial hypertension presenting with visual symptoms. MethodsBetween Apr. 2014 and Mar. 2018, 70 patients of Idiopathic Intracranial Hypertension (IIH) underwent treatment at our institution. Patients were evaluated for neurological and ophthalmological status and were subjected to LPS depending on their symptoms. ResultsMean opening pressure was 29.97±5.33cm of water and mean Body-Mass Index (BMI) was 26.51±3 and the two were significantly correlated (p-value 0.006). All patients with visual symptoms (23) underwent LPS and others (47) were managed medically. All patients with LPS and 25 of medically managed patients improved, while 22 medically-managed patients required LPS due to deterioration in visual symptomatology. The proportion of patients showing complete resolution of features of IIH was significantly different between the three groups. Of the 7 patients with shunt extrusion/migration, only 2 required revision. ConclusionLPS is an equally effective and more technically familiar modality for treatment of IIH for neurosurgeons and should be offered to asymptomatic patients with objective visual signs. Shunt extrusion/migration may not always warrant revision due to “mini-shunt” that drains Cerebro-Spinal Fluid (CSF) through shunt tract even after extrusion.

Idiopathic intracranial hypertension presenting as iron deficiency anemia: a case report

BackgroundThe presentation of idiopathic intracranial hypertension (IIH) in association with iron deficiency anemia (IDA) is rare.Case presentationThis case report depicts the unusual case of a 31-year-old woman of mixed Jamaican and English heritage with IIH who presented initially as IDA in the context of menorrhagia. Subsequent ophthalmic review, lumbar puncture, cerebrospinal fluid analysis and neuroimaging studies revealed severe bilateral optic disc swelling and raised intracranial pressure in keeping with IIH. Prompt treatment of IDA with blood transfusion and orally administered iron supplements, in addition to medical treatment for IIH, contributed to significant improvement of symptoms and prevented long-term visual deficits.ConclusionThe possibility of IDA, albeit rare, should always be considered and investigated appropriately in all patients with IIH, as the treatment of the anemia alone may be sight-saving.

Primary empty sella syndrome presenting with profound bilateral visual loss

Primary empty sella syndrome (PESS) on background idiopathic intracranial hypertension (IIH) presenting with profound bilateral visual loss is a rare clinical occurrence. We report the case of PESS on background IIH in a 37-year-old obese Nigerian female. At presentation, she had a history of an insidious onset of loss of vision in both eyes with an associated history of headaches and tinnitus. Clinical examination and imaging studies revealed papilloedema and empty sella with a completely flattened pituitary gland. This report highlights that PESS could initially present to an ophthalmologist as a case of profound bilateral visual loss.

A clinical and radiological study in patients with idiopathic intracranial hypertension

Idiopathic intracranial hypertension (IIH) is one of the chronic causes of elevated intracranial pressure with no evident cause in neuroimaging and normal CSF analysis. It primarily affects overweight women of childbearing age.Aim of workTo describe the clinical picture of IIH, neuroimaging and response to treatment after 3 months follow up in a sample of Egyptian patients.Patients and methodsThis was a prospective study carried out on 25 patients presented at Alexandria University Hospital with symptomatology of IIH. All the patients were subjected to complete history taking, neurological examination, fundus examination, lumbar puncture, and MRI brain and MR cerebral venography. The patients were followed-up for 3 months for assessment of treatment response.ResultsPatients’ ages ranged from 1 0to 50 years, with a mean BMI 32.32 ± 5.28 kg m2. Headache was the presenting manifestation in 100% of the patients. About 60% of the sample were either misdiagnosed or had a delayed diagnosis. There was a significant positive correlation between CSF opening pressure (r = 0.504, p = 0.010), severity of headache (r = 0.472, p = 0.017) and grade of papilledema. Optic hydropes and empty sellaturcica were the most common MRI abnormalities occurring in 95.8% and 70.8%, respectively. Only 30% of the cases had normal MRV. Stenosis at genu junction and focal stenosis at transverse sinus occurred in 24% and 20% of the cases, respectively. Combined medical and serial lumbar puncture were the most effective line of therapy in the recruited patients.ConclusionHeadache is the most common presentation of IIH, and its severity is positively correlated with papilledema grade. CSF pressure is also positively correlated with papilledema grade.

Lumbo-peritoneal shunt as treatment modality for visual symptoms in idiopathic intracranial hypertension: A single institution experience.

To evaluate the efficacy of lumbo-peritoneal shunt (LPS) in patients of idiopathic intracranial hypertension presenting with visual symptoms. Between Apr. 2014 and Mar. 2018, 70 patients of Idiopathic Intracranial Hypertension (IIH) underwent treatment at our institution. Patients were evaluated for neurological and ophthalmological status and were subjected to LPS depending on their symptoms. Mean opening pressure was 29.97±5.33cm of water and mean Body-Mass Index (BMI) was 26.51±3 and the two were significantly correlated (p-value 0.006). All patients with visual symptoms (23) underwent LPS and others (47) were managed medically. All patients with LPS and 25 of medically managed patients improved, while 22 medically-managed patients required LPS due to deterioration in visual symptomatology. The proportion of patients showing complete resolution of features of IIH was significantly different between the three groups. Of the 7 patients with shunt extrusion/migration, only 2 required revision. LPS is an equally effective and more technically familiar modality for treatment of IIH for neurosurgeons and should be offered to asymptomatic patients with objective visual signs. Shunt extrusion/migration may not always warrant revision due to "mini-shunt" that drains Cerebro-Spinal Fluid (CSF) through shunt tract even after extrusion.

Pediatric Idiopathic Intracranial Hypertension.

The presentation of idiopathic intracranial hypertension (IIH) in pediatric populations has several important distinctions from that in adults, especially among prepubertal patients, in which there is no apparent association with gender or obesity. Pediatric patients are more likely to be asymptomatic or present with atypical symptoms than their adult counterparts, posing a diagnostic challenge in some cases. It is important to be aware of the ways in which diagnostic criteria for IIH are modified from that of adults. Ideal treatment practices and the natural history of pediatric IIH remain unclear. Acetazolamide is the mainstay of medical treatment, but some patients with significant visual loss may require surgical intervention. Multicenter studies to accrue a large number of cases and future prospective studies will help to better define pediatric IIH and to formulate consensus guidelines for treatment and management of these patients.

Reader response: Idiopathic intracranial hypertension: The veno glymphatic connections.

The Medical Hypothesis article by Lenck et al.1 provided an exciting new angle on recent findings concerning CSF circulation in the brain. The authors succinctly summarized clinical and radiologic evidence supporting their hypothesis that dysfunction of veno glymphatic connections lies at the heart of idiopathic intracranial hypertension (IIH). In particular, the authors speculated that chronic overflow of CSF in the sheaths of the olfactory bulbs may result in CSF rhinorrhea by eroding the cribriform plate.1 Olfactory dysfunction, especially a marked impairment in olfactory threshold levels, is an even more common yet underrecognized presentation of IIH.2,3 Indeed, as early as 2008, Dr. Kapoor4 speculated that dysfunction of the extensive lymphatic network around the olfactory nerves might be causally linked to IIH, making hyposmia a more sensitive predictor of IIH than other clinical features.