Cause Of Idiopathic Intracranial Hypertension Research Articles

MRI Indices of Glymphatic Function Correlate With Disease Duration in Idiopathic Intracranial Hypertension.

The glymphatic system represents an extravascular network of astrocytic channels responsible for interstitial fluid and solute transit through the brain parenchyma. Its dysfunction has been considered as a possible cause of idiopathic intracranial hypertension (IIH). We enrolled participants with active IIH, treated or cured IIH, and controls. The active IIH group was divided into untreated participants with recently developed (<6 mo) and chronic (6+ mo) disease. Glymphatic function was assessed using diffusion tensor imaging along the paravascular space (DTI-ALPS) to generate an ALPS-index, hypothesized to measure glymphatic function. Participants were imaged before lumbar puncture (LP) if IIH was suspected and following LP when possible. ALPS indices were higher in participants with chronically present, active IIH than in those either with recently developed IIH or control participants. ALPS-indices correlated with papilledema but did not correlate significantly with age, BMI, or intracranial pressure (ICP). Our findings suggest that DTI-ALPS-indices of glymphatic function may be influenced by the chronicity of intracranial hypertension but do not support the hypothesis that glymphatic dysfunction causes IIH. Though these findings are preliminary, glymphatic imaging may be a useful radiographic biomarker in IIH.

Long-term efficacy of venous sinus stenting in the treatment of idiopathic intracranial hypertension.

Previous studies have suggested that cerebral dural sinus stenosis could be a possible underlying cause of idiopathic intracranial hypertension (IIH). Venous sinus stenting (VSS) has emerged as a potential alternative for treating IIH related to dural sinus stenosis. However, most of the documented studies have been conducted in Western countries. In this study, we present the results of 16 Chinese IIH patients who underwent VSS treatment in our single center. We prospectively collected angiographic and manometric data from IIH patients who underwent angioplasty/stenting. All patients had confirmed dural sinus stenosis and had failed maximal medical therapy (MMT). Demographic, clinical, and radiological presentation, as well as long-term follow-up outcomes were collected retrospectively. A total of 16 patients who underwent VSS were enrolled in the present study. Demographic data revealed a mean age of 40 (range 20-55), with 69% (11/16) being female, and a mean body mass index (BMI) of 27.05 (range 19.18-38.04) kg/m2 . All patients presented with papilledema and visual disturbances. During a median follow-up period of 47.5 months, 93.75% (15/16) of patients reported improvement in symptoms, although only 37.5% (6/16) experienced complete resolution. Headaches, blurred vision, and amaurosis related to increased pressure improved in 100% (8/8), 81.25% (13/16), and 75% (3/4) of patients, respectively. However, one patient suffered cerebral infarction and secondary epilepsy soon after VSS, and another patient had recurrence of symptoms due to stent wall thrombosis 2 years later. The significance of venous sinus stenosis in the development of IIH may be undervalued. Our study, based on a Chinese case series, affirms the long-term safety and effectiveness of VSS in treating IIH patients with relatively lower BMI than those from Western countries.

Venous sinus stenting as a treatment approach in patients with idiopathic intracranial hypertension and encephaloceles.

Stenosis of a dural venous sinus is the most common cause of idiopathic intracranial hypertension (IIH) and can be classified as either intrinsic or extrinsic. Intrinsic stenoses are characterized by a focal filling defect within the sinus secondary to an enlarged arachnoid granulation or fibrous septa while extrinsic stenoses tend to be long and smooth-tapered and are most commonly secondary to external compression from the adjacent brain parenchyma. Brain herniations, or encephaloceles, into arachnoid granulations in dural venous sinuses have rarely been reported in the literature in patients with IIH. We propose that dural venous sinus stenting (VSS) may be a safe and effective treatment approach in patients with an encephalocele and IIH. We retrospectively analyze three cases of patients with encephalocele who underwent VSS for treatment of medically refractory IIH at our institution. One patient underwent stenting ipsilateral and two patients underwent stenting contralateral to the side of their encephaloceles. No technical related issues or complications occurred during either of the three stenting procedures. Two out of the three patients had complete resolution in their IIH-related symptoms and normalization of cerebrospinal (CSF) pressures shortly after stenting. We await clinical follow-up in the third patient. Our results suggest that VSS is a technically feasible and effective approach in treating patients with medically refractory IIH and encephaloceles.

SAT-262 A Case of Pseudotumor Cerebri After Removal and Recurrence of ACTH-Producing Tumor

Introduction: Pseudotumor cerebri also known as idiopathic intracranial hypertension (IIH) is a condition of elevated cerebrospinal fluid pressure which as a result causes headaches and vision problems. Several case reports and studies have reported a relation of IIH with Cushing’s disease (CD) in adults and children, particularly after surgical or medical therapy. We describe a very uncommon presentation of persistent intracranial hypertension (ICH) in a patient with recurrence of cushing’s disease after her initial surgical resection.Case presentation:38-year-old African-American female with a BMI of 45, type 2 diabetes, HTN and history of Cushing’s syndrome from an ACTH-producing pituitary macroadenoma. She initially presented with increasing weight gain and features of Cushing’s syndrome including hypertension, hyperglycemia, truncal obesity and moon facies. Imaging studies showed a 2 cm intrasellar mass with suprasellar extension without compression of the optic chiasm. Hormonal evaluation confirmed Cushing’s syndrome from an ACTH-producing pituitary macroadenoma. Patient underwent initial trans-sphenoidal hypophysectomy (TSR) and was tapered down to physiologic doses of glucocorticoids.Post-operatively she started complaining of significant headaches and transient vision loss. She followed up with neuro-ophthalmology and was diagnosed with papilledema that was not present in the pre-operative examination. An LP (lumbar puncture) was recommended to assess for ICH, however patient declined the procedure. A year after, a second surgery had to be performed for recurrent pituitary adenoma. Unfortunately, a repeat MRI pituitary shortly after her second surgery revealed recurrent pituitary macroadenoma of 2.2 cm. Patient continued with headaches and underwent an LP with an opening pressure of 28 cm H20 (ICH > 26 cm H2O). She underwent a third TSR with follow up MRI showing gross total resection of the previously seen pituitary mass. For her ICH she was started on acetazolamide but was not able to tolerate due to paresthesias and metallic taste. Her symptoms have improved after her last resection and last MRI brain shows no residual tumor. She is currently on furosemide and focusing on weight loss.Conclusion: Our patient’s presentation is an interesting and unusual case because we believe she had both pseudotumor cerebri (IIH) and real tumor cerebri from the complications of her ACTH-secreting macroadenoma. The cause of IIH after treatment of cushing’s disease is believed to be mostly due to steroid withdrawal after surgical resection or medical treatment comprising hormonal control of cerebrospinal fluid production and absorption. In our patient we suppose that the persistent weight gain caused by the recurrence of her CD could also contribute to her IIH. The treatment in general is the same with physiologic doses of corticosteroids, diuretics and weight loss.

MON-342 Severe Hypocalcemia and Psuedotumor Cerebri: Old, Still Not so Well Known Relationship

Introduction:Severe headaches with nonspecific characteristics raise concern for idiopathic intracranial hypertension (IIH) in a young adult female patient. Clinical features of severe headache, vision loss, and papilledema; normal neuroimaging and elevated opening pressure on lumbar puncture are diagnostic of IIH or pseudotumor cerebri. Etiology is often attributed to obesity in young female patients. We present a rare case of Pseudotumor cerebri in a non-obese female patient with severe hypocalcemia.Case:A 26 year old Hispanic female patient with past medical history of hypocalcemia, but off her medications, presented to ER for worsening headache for over a month. Without any specific triggers, she began having headaches in her bitemporal region. The pain was continuous, pressure-type, and 9/10 intensity at its worst. It was associated with nausea and blurring of vision and was refractory to ibuprofen. Patient endorsed severe muscle cramps on review of systems. Physical examination demonstrated mild distress due to the headache, positive Trousseau’s sign, and bilateral papilledema. Laboratory studies revealed serum total calcium level of 4.8 mg/dL, albumin 3.5 g/dL, ionized calcium 0.71 mmol/L, serum phosphate 5.3 mg/dL, intact PTH 300.1 pg/mL, 25-OH Vitamin D 14.3 ng/mL and 1, 25-Dihydoxy Vitamin D 14.1 pg/mL, alkaline phosphatase 131 IU/L, 24-hour urinary calcium 48mg/d and 24 hour urinary calcium/creatinine ratio 50 mg/g. Her MRI brain and MR venogram were unremarkable. Lumbar puncture had elevated opening pressure of 46 cm of H2O. The patient was started on Acetazolamide, oral calcium, and calcitriol without resolution of headaches and hypocalcemia. Acetazolamide was discontinued when she developed severe hypokalemia. With diagnosis of pseudohypoparathyroidism, the dose of elemental calcium was doubled and calcitriol dose increased to 1 mcg BID with improvement of serum calcium, symptoms, and papilledema.Discussion:While IIH could be associated with certain medications and systemic conditions, obesity or recent weight gain are the most commonly cited causes of IIH. Severe hypocalcemia is a rare and less known etiology of IIH. Cases of adult patients are reported with a variety of etiologies of severe hypocalcemia. Severe hypocalcemia leading to hypersecretion of cerebrospinal fluid is postulated as a possible mechanism but evidence is lacking in literature. Chronic, rather than acute, severe hypocalcemia is likely to precipitate IIH. Visual symptoms, headache and papilledema are difficult to resolve with conventional medical therapy without correction of underlying severe hypocalcemia. We conclude that before considering surgical interventions for cases which are refractory to medical therapy, rare underlying conditions like severe hypocalcemia must be investigated and treated optimally.

Styloidogenic Jugular Venous Compression Syndrome: Clinical Features and Case Series

Styloidogenic jugular venous compression syndrome (SJVCS) is a rare cause of idiopathic intracranial hypertension (IIH). To elucidate the pathophysiology and the hemodynamics of SJVCS. We conducted a retrospective review of medical records, clinical images, dynamic venography, and manometry for consecutive patients with SJVCS undergoing microsurgical decompression from April 2009 to October 2017. Patients with IIH with normal venography and manometry findings served as controls. Data were analyzed for 10 patients with SJVCS who presented with headaches. Neck flexion exacerbated headaches in 7 patients. Eleven patients with IIH provided control data for normal intracranial venous pressure and styloid process anatomy. Patients with SJVCS had bilateral osseous compression of venous outflow. The styloid processes were significantly longer in patients with SJVCS than in those with IIH (mean [standard deviation (SD)] distance, 31.0 [10.6] vs 19.0 [14.1] mm; P<.01). The styloid process-C1 lateral tubercle distance was shorter in patients with SJVCS than in those with IIH (mean [SD] distance, 2.9 [1.0] vs 9.9 [2.8] mm; P<.01). Patients with SJVCS had significantly higher global venous pressure and a higher pressure gradient across the stenosis site than controls (mean [SD] pressure, 2.86 [2.61] vs 0.13 [1.09] cm H2O; P=.09). All 10 patients with SJVCS experienced venous pressure elevation during contralateral neck turning (mean [SD] pressure, 4.29 [2.50] cm H2O). All 10 patients with SJVCS underwent transcervical microsurgical decompression, and 9 experienced postoperative improvement or resolution of symptoms. One patient had transient postoperative dysphagia and facial drooping, and another patient reported jaw numbness. SJVCS is a novel clinical entity causing IIH. Patients should be evaluated with dynamic venography with manometry. Surgical decompression with removal of osseous overgrowth is an effective treatment in select patients.

Clinical analysis of 36 cases of idiopathic intracranial hypertension complicated with iron deficiency anemia

Objective: To investigate the clinical features, imaging findings and prognosis of idiopathic intracranial hypertension (IIH) patients complicated with iron deficiency anemia (IDA). Methods: A total of 307 cases of IIH patients hospitalized in Beijing Tongren Hospital were retrospectively screened between January 1, 2011 and February 28, 2018. There were 49 anemia cases (15.96%) and 45 IDA cases (14.66%), respectively. Finally, 36 IDA patients were enrolled. The clinical characteristics, imaging findings, treatment and prognosis of these patients were analyzed. Results: IIH combined with IDA was more common in women of childbearing age (34/36). There were 30 obese and overweight cases (83.33%), with multiple subacute or chronic course of disease. The visual symptoms in the early IIH patients were first diagnosed in the Department of Ophthalmology. The first symptom was headache with/without visual symptoms (27 cases (75%)). Head MRI detected empty sella or partial empty sella, and 2 cases of venous sinus thrombosis were found in DSA examination. Of the 34 female patients, 24 had simple menometrorrhagia or menstrual disorder. All patients were given methyl acetate to reduce the intracranial pressure and iron therapy. Five patients received low molecular weight heparin-warfarin sequential treatment, 5 cases underwent gynecologic surgery and 2 male cases received hemorrhoid operation. There were 7 cases underwent lumbar cisterna-peritoneal shunt for visual impairment. During the follow-up, intracranial pressure decreased and visual function of patients improved significantly. Conclusions: IIH is frequently found in obese or overweight women at childbearing age and IDA may be an important cause of IIH. IIH can cause serious irreversible visual impairment. Therefore, early identification and active treatment should be performed. Correction of anemia can significantly improve the clinical symptoms of IIH. Operation should be employed for IIH patients with poor visual function or rapid progress, in order to reduce intracranial pressure and improve prognosis as soon as possible.

Cause or Consequence: The Relationship Between Cerebral Venous Thrombosis and Idiopathic Intracranial Hypertension.

The aims of our study were to refer to the complex relationship between idiopathic intracranial hypertension (IIHT) and cerebral sinovenous thrombosis (CSVT), and to determine the differences and commonalities between the patients with and without CSVT in their etiology, along with documenting the uncertainties in concluding on the diagnosis and treatment of these patients. IIHT was diagnosed according to Dandy criteria, while CSVT was screened for by way of a cranial magnetic resonance imaging for all patients and cranial magnetic resonance venography only if the magnetic resonance imaging was nebulous or there was a family history. We retrospectively evaluated a total of 26 patients (9 of whom had CSVT) diagnosed with IIHT between 2014 and 2018. A total of 9 patients with concurrent CSVT were described as suffering from vascular IIHT, while the remaining 17 were described as suffering from other IIHT. Demographic characteristics were similar in both groups (mean age: 12 vs. 11; male/female ratio: 2/7 vs. 5/12 in vascular IIHT and other IIHT, respectively). Clinical findings, cerebrospinal fluid-opening pressure values, and pathologies of etiology were also similar (vitamin D deficiency: 66% vs. 52%; vitamin B12 deficiency: 11% vs. none; iron deficiency: 22% vs. 11%; obesity: 22% vs. 23%). A mixture of acetazolamide, topiramate, anticoagulant therapy, and acetylsalicylic acid were given according to the diagnoses. CSVT is a common clinical entity among the causes of IIHT, and it should be taken into consideration in this patient group. However, there is a need for a common guideline for laboratory and imaging methods to understand the etiopathogenesis of childhood IIHT and determine the patients at risk.

Clinical features of pediatric idiopathic intracranial hypertension.

Introduction: Adult idiopathic intracranial hypertension (IIH) is often linked to obesity, however, causes of IIH in children are not well understood. This project identifies potential risk factors and features of pediatric IIH.Methods: This study consisted of a retrospective chart review of patients ages 5–17 years who were seen at a tertiary care children’s hospital and diagnosed with IIH. Diagnostic criteria included the presence of papilledema, normal neurological exam, normal neuroimaging, normal cerebrospinal fluid composition, and an opening pressure of a lumbar puncture >28 mmHg.Results: Of the 26 cases of IIH, 19 met all diagnostic criteria for this study, while seven patients were probable IIH, as they lacked papilledema. Intracranial pressure ranged from 28 to 66 mmHg, with a mean of 40.23 mmHg (±10.74). Overall, 50.0% (95% CI: 29.9–70.1%) of IIH patients were obese, with patients 12 years of age and younger exhibiting an overall obesity rate of 30.7% and patients 13 years of age and older having an obesity rate of 69.2%. The overall allergy rate in this IIH patient population was 46.2% (95% CI: 26.6–66.6%).Conclusion: Obesity appears to have no association with IIH in younger cases, but it is a more common feature in older children. An autoimmune component may play a role in pediatric IIH, given the high rate of atopy observed in this pediatric IIH patient cohort. Because a diagnosis of IIH can have an absence of optic nerve edema, taking a detailed history and performing a thorough examination are keys to diagnosing IIH in the pediatric population.

Prevalence of idiopathic intracranial hypertension and associated factors in obese children and adolescents.

Idiopathic intracranial hypertension (IIH) is one of the most important and rare complications of obesity. Prevalence of IIH in childhood obesity is not known. The purpose of this study was to determine the prevalence of IIH in obese children and adolescents and to investigate the relevant factors. In this study, 1058 obese children and adolescents between 2 and 18 years of age were included. Funduscopic examination was conducted for all subjects. In cases with papilledema, increase in intracranial pressure was clarified by measuring CSF pressure with a lumbar puncture. Other causes of IIH were ruled out with clinical, laboratory tests and imaging techniques. Idiopathic intracranial hypertension was diagnosed in 14 subjects (1.32%). Rates of headache and systemic hypertension in subjects with IIH was determined to be 78.6% and 28.6%, respectively. Morning cortisol, fasting insulin and HOMA-IR values were found to be significantly higher in this cases (p<0.05). In childhood obesity, IIH should be kept in mind as a serious complication. Funduscopic examination is an easy method that suggests IIH. In particular, obese children with systemic hypertension and refractory headache should be considered for IIH.

Intracranial hypertension and thyroid papillary cancer treated with radioactive iodine: two case reports

Background: Idiopathic Intracranial Hypertension (IIH) presents with symptoms and signs of increased intracranial pressure without cerebral structural lesion and abnormal CSF findings. There are many causes of intracranial hypertension. Largely an underlying cause can't be found. Objective: We presented two cases who operated for papillary carcinoma and given radioactive iodine therapy and can't be determined any other pathologies in etiology. In these two cases, tumor or administration of radioactive iodine is thought to be the cause of IIH. Case 1: Total thyroidectomy was performed and radioactive iodine treatment was given to a 32-year-old female patient with papillary carcinoma in December 2012. One year ago she began complaining of visual obscuration and blurred vision. In this period papillary stasis was detected and MRI was normal, except that the empty sella. CSF pressure was 550 mm/H2O. Improvement in vision was observed after CSF uptake. The complaints decreased after Diazomid treatment. Case 2: Total thyroidectomy was made and radioiodine therapy was given to a 57-year-old female patient due to papillary carcinoma in June 2014. One month before, she was admitted with the complaint of feeling of swelling in her eyes and blurred vision. Papillary stasis was detected, CSF pressure was measured 260 mm/H2O. Brain MRI was normal. Conclusion: In these patients, it was not determined additional abnormality to cause Intracranial Hypertension except papillary carcinoma and radioactive iodine administration. Additionally, papillary stasis was a foreground symptom in these patients, while headache was an insignificant symptom. We don't know whether papillary carcinoma or radioactive iodine treatment caused to IIH. However, we also recommend that the patients with similar situations should be investigated in terms of IIH or this etiology should be kept in mind in patient with IHH.

Treatment of idiopathic intracranial hypertension by endovascular improvement of venous drainage of the brain

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a syndrome of elevated intracranial pressure (ICP) in the absence of any known causative factor. Most patients with IIH respond to weight reduction, repeated lumbar punctures and carbonic anhydrase inhibitors, such as acetazolamide and topiramate to reduce cerebrospinal fluid (CSF) production. Despite a number of pathogenetic theories, the cause of IIH remains unknown. With the availability of magnetic resonance (MR) venography and cerebral angiography, venous disease is increasingly being discussed as the etiology of IIH, with a high proportion of patients presenting with nonthrombotic unilateral or bilateral dural venous sinus stenoses. Based on this observation, endovascular stenting of stenotic dural sinuses in patients with IIH has gained popularity. Whether dural venous sinus stenoses are the cause or the consequence of increased ICP is still under debate. In patients with failure of conservative treatment or non-compliance, a more aggressive treatment, such as CSF shunting or surgical optic nerve fenestration, should be performed. For approximately 13 years endovascular stenting of the stenotic sinuses has been used and discussed as an alternative and effective treatment of IIH. Since the first report in 2002, individual cases and case series have been published demonstrating that stents immediately lower the venous pressure gradient, which is associated with clinical improvement. This effect occurs within days or weeks and is permanent in many cases.

A case of idiopathic intracranial hypertension associated with PCOS

Idiopathic intracranial hypertension (IIH) is a rare neurologic disorder. It is also known as pseudotumor cerebri. The incidence of IIH is one to two per 100,000 population annually. The higher incidence is in obese women from 15 to 44 years. The main symptoms are headache and visual loss. It mostly affects women of childbearing age who are overweight or obese. There are many theories of pathogenesis of IIH, but precise pathogenesis is unknown. One of the causes of IIH is intracranial venous sinus thrombosis. It can cause increased cerebrospinal fluid (CSF) pressure by obstruction of venous outflow and blocking of CSF absorption. In polycystic ovary syndrome (PCOS) patients, thrombogenic tendency is increased due to increased aromatization of testosterone to estradiol which could induce estrogen-mediated thrombophilia. The authors present a 14-year-old girl with PCOS stigma who presented with a severe headache and papilledema. These symptoms were not improved by standard medical therapy of IIH and PCOS, but improved after laparoscopic ovarian drilling. The authors report it with a review of the literature.

Investigation into the Molecular Mechanisms Underlying Idiopathic Intracranial Hypertension (P2.099)

Objective: To investigate cerebrospinal fluid (CSF) secretion/drainage rates as putative underlying causes of idiopathic intracranial hypertension (IIH). Background: Various causative factors have been postulated as to the increasing incidence of IIH, however, none with any convincing evidence. Identifying biomarkers, already found to be elevated in patients with IIH, including pro-inflammatory cytokines; chemokine (C-C motif) ligand 2 (CCL2), interleukin (IL)-17 (IL-17), IL-6, IL-1β, tumour necrosis factor-α (TNF-α), as well as adipocyte derived hormone leptin and the glucocorticoid hydrocortisone has been used to present an insight into the pathogenesis of IIH. Design/Methods: To assess the effects of the elevated mediators on CSF volume/secretion rates, an in vivo ventriculo-cisternal perfusion was performed. In addition, an in vivo variable rate infusion technique assessed mediator effects on CSF drainage. Results: Hydrocortisone (2.65 μl/min) and TNF-α (2.58 μl/min) significantly increased CSF secretion rate, when compared to controls (1.90μl/min). IL-6 (0.91 μl/min) caused a significant decrease in CSF secretion rate. The volumes of the initial CSF within the cranium following intraperitoneal mediator injection, prior to perfusion of the animal with artificial CSF containing the mediator, were significantly higher for CCL2 (323.6 μl) and IL-17 (327.0 μl) when compared with controls (226.7 μl). CCL2 (55.6 mmH2O.min/μl) and IL-17 (52.7 mmH2O.min/μl) significantly increased pressure and resistance to CSF drainage when compared with controls (28.76mmH2O.min/μl). Conclusions: An impairment within the CSF drainage mechanisms could be postulated due to the significant increase in initial CSF volume; decrease in overall CSF secretion and increased resistance to CSF drainage associated with CCL2 and IL-17. The opposing results of CSF secretion/drainage suggest an altered compensatory mechanism associated with hydrocortisone, TNF-α and IL-6. Study supported by: Prof. Basil Sharrack, Dr. Ignacio A. Romero, Dr. A. Jane Loughlin, Dr. Jane E. Preston. Disclosure: Dr. Alimajstorovic has nothing to disclose.

An update on the pathophysiology of idiopathic intracranial hypertension alias pseudotumor cerebri.

Idiopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure of unknown cause, leading to severe headache, papilledema and visual disturbances. Its former name, pseudotumor cerebri, has gained popularity recently. The strongest and most consistent risk factors of IIH are obesity and female gender. Infrequently, IIH may present in the absence of papilledema showing a headache profile similar to chronic daily headache with migrainous features. There have been several proposed mechanisms to explain the etiology of this disorder associated with various clinical conditions. In recent years, some inflammatory factors, natriuretic peptides and aquaporins have been proposed as possible contributors of the pathogenesis. On the other hand, some investigators have reported that bilateral transverse sinus stenosis is seen in the majority of IIH patients; therefore, dural sinus stent placement is used in some patients. No single theory has been able to provide a comprehensive answer, and there is no consensus about the exact cause of IIH. The aim of this review was to discuss the new insights on the mysterious pathogenesis of IIH.

Leptomeningeal Carcinomatosis Mimicking Idiopathic Intracranial Hypertension with Transverse Sinus Stenosis

Background: The most common form of pseudotumor cerebri occurs in obese young women in whom it can be caused by transverse sinus stenosis. Such cases used to be called idiopathic intracranial hypertension (IIH). However, leptomeningeal carcinomatosis can produce intracranial hypertention with papilledema, but without hydrocephalus. Case Report: A 48-year-old woman presented with blurred vision and headache for one month. Brain magnetic resonance imaging with contrast showed no abnormality, and lumbar puncture revealed elevated cerebrospinal fluid pressure with hypoglycorrhachia. A magnetic resonance venogram showed a transverse sinus stenosis. She was treated with stent placement within a transverse sinus stenosis, but her condition became progressively worse. Leptomeningeal carcinomatosis was finally confirmed by repeated analyses of cerebrospinal fluid. Conclusion: The cause of IIH is unclear and it remains a diagnosis of exclusion. When confronted by a patient with intracranial hypertension with hypoglycorrhachia, other cause must be excluded before diagnosing IIH. Key Words: Intracranial hypertension; Hypoglycorrhachia; Leptomeningeal carcinomatosis

Leptomeningeal Carcinomatosis Mimicking Idiopathic Intracranial Hypertension with Transverse Sinus Stenosis

Background: The most common form of pseudotumor cerebri occurs in obese young women in whom it can be caused by transverse sinus stenosis. Such cases used to be called idiopathic intracranial hypertension (IIH). However, leptomeningeal carcinomatosis can produce intracranial hypertention with papilledema, but without hydrocephalus. Case Report: A 48-year-old woman presented with blurred vision and headache for one month. Brain magnetic resonance imaging with contrast showed no abnormality, and lumbar puncture revealed elevated cerebrospinal fluid pressure with hypoglycorrhachia. A magnetic resonance venogram showed a transverse sinus stenosis. She was treated with stent placement within a transverse sinus stenosis, but her condition became progressively worse. Leptomeningeal carcinomatosis was finally confirmed by repeated analyses of cerebrospinal fluid. Conclusion: The cause of IIH is unclear and it remains a diagnosis of exclusion. When confronted by a patient with intracranial hypertension with hypoglycorrhachia, other cause must be excluded before diagnosing IIH. J Neurocrit Care 2014;7(2):129-132

Idiopathic intracranial hypertension associated with mild traumatic brain injury in a pediatric patient – Case report

AbstractMild head injury has been described as rare cause of idiopathic intracranial hypertension (IIH). In the presence of IIH, initial treatment is clinical and surgical treatment, such as lumboperitoneal shunt. Most cases have a good prognosis. The patient have 9-year-old male, went to the emergency room with a history of accidental fall, presenting headache, vomiting and blurred vision. Physical examination showed good overall condition. Neurological examination: normal. Fundoscopy: incipient bilateral papilledema. Normal cranial CT. The general picture suggested by exclusion of other causes IIH diagnosis. He underwent symptomatic treatment with acetazolamide, painkillers and rest. Discharged from the hospital on the eighth hospital day with no complaints being referred for outpatient treatment.

Visual Loss without Headache in Children with Pseudotumor Cerebri and Growth Hormone Treatment

We report on two prepubescent girls with visual loss due to idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, both treated with recombinant human growth hormone for growth failure. The interval from starting hormone therapy to diagnosis of IIH was 3 and 18 months, respectively. Both girls did not complain of headache and nausea. They were neither obese nor did they suffer from renal insufficiency. In both patients, we observed bilateral optic disc edema with visual loss and elevated cerebrospinal fluid (CSF) pressures. Other causes of IIH were excluded with neuroimaging and CSF examination. Cessation of drug administration is often sufficient for symptom resolution in cases of hormone therapy-associated IIH. However, visual field defects in one girl remained unchanged during follow-up of 8 months. In children with IIH, the spectrum of neurologic and visual manifestations might be variable and unspecific. Diagnosis and management of IIH can be difficult in the absence of headache. Blurred or double vision due to cranial nerve palsy might be the only symptom rather than complaints about reduced visual acuity. Therefore, regular clinical monitoring of visual function and fundus appearance is essential for early diagnosis, efficient management, and improvement of visual outcome in children receiving recombinant human growth hormone.

Undiagnosed Papilledema in a Morbidly Obese Patient Population

Idiopathic intracranial hypertension (IIH) is a rare condition that can lead to significant morbidity from visual loss. The cause of IIH is unknown, but IIH is known to be associated with obesity. Obese patients may be at particularly high risk for suffering vision loss from IIH. The purpose of the present study is to determine the prevalence of undiagnosed or asymptomatic papilledema in a population of morbidly obese individuals and to determine if these patients should undergo routine screening for papilledema. Patients presenting to the UC Davis Bariatric Surgery Clinic between February 2008 and January 2011 who met the National Institutes of Health criteria for bariatric surgery were invited to participate in the study. Those patients who met the inclusion criteria and consented to the study were included. Participants were screened for IIH by nonmydriatic fundus photographs and by concerning symptoms prompting direct referral for neuro-ophthalmologic evaluation. Images were reviewed by a neuro-ophthalmologist, and patients with suspicious optic discs underwent neuro-ophthalmologic evaluation. Patients with findings consistent with IIH were sent for neurological evaluation. A total of 606 patients with an average body mass index of 47 kg/m2 were included in the study. Seventeen of these patients had photographic optic disc findings or symptoms suspicious for IIH. Seven of these patients did not have disc edema on clinical examination. Six patients were not evaluated in the clinic. Four of the 17 patients had subtle optic disc edema confirmed by clinical evaluation and were referred for full neurological workup. These 4 patients had normal neuroimaging, 3 of whom underwent lumbar punctures with borderline high opening pressures. All 4 patients had unremarkable visual field examinations. Fundus abnormalities other than optic disc edema were discovered in 33 patients. Our study suggests that in a morbidly obese patient population, papilledema with significant visual loss is rare. Routine screening with fundus photography of morbidly obese patients likely is not warranted.