Diagnosis of idiopathic intracranial hypertension - the importance of excluding secondary causes: A systematic review

Cephalalgia ◽  
2021 ◽  
pp. 033310242110565
Author(s):  
Kubra Kilic ◽  
Johanne Juhl Korsbæk ◽  
Rigmor H Jensen ◽  
Vlasta Vukovic Cvetkovic

Background Idiopathic intracranial hypertension is characterized by increased intracranial pressure without any pathological findings on neuroimaging, except for signs of high intracranial pressure. Before diagnosing idiopathic intracranial hypertension secondary causes of increased intracranial pressure should be excluded. Objective to characterize the phenotype of patients with secondary intracranial hypertension and to identify possible risk factors for secondary intracranial hypertension. Methods We have systematically searched the PubMed database. The publications were analyzed according to the patient phenotype, age, gender, comorbidities, body mass index/weight status, and additional medication. The results are summarized in four categories: medication, infection, hormonal induced intracranial hypertension and miscellaneous groups of diseases related to sIH. Results We identified 105 eligible papers which included 272 cases. There were 49.6% pediatric cases. Among the adult group,70.9% were women. A total of 40.4% of all cases were obese or overweight, 27% among adults and 13.4% among pediatric cases. Increased BMI and recent weight gain, anemia, renal diseases and hypertension were the most frequent comorbidities related to sIH. Conclusion Among sIH patients, 40.4% were obese or overweight; two thirds were women. We recommend that even patients with a typical IIH phenotype should be screened for secondary causes.

Author(s):  
Mahmoud M. Allam ◽  
Hatem A. Almasry ◽  
Sandra M. Ahmed ◽  
Youssuf G. Taha ◽  
Mohammed I. Oraby

Abstract Background Idiopathic intracranial hypertension (IIH) is a disorder of increased intracranial pressure without a clear cause and can have serious visual effects. Previous research work suggests that transcranial Doppler measurements of pulsatility index correlate accurately with elevated intracranial pressure. Objective To assess the cerebrovascular hemodynamic changes in patients with IIH using transcranial Doppler before and after lumbar puncture and CSF withdrawal. Methods An interventional study conducted on 40 patients (31 females and 9 males) fulfilling the modified Dandy criteria for diagnosis of idiopathic intracranial hypertension, MRI brain, and MRV was done to the patients. Lumbar puncture was done for all included patients to measure intracranial pressure and CSF withdrawal. Transcranial Doppler was performed for all included before and after lumbar puncture and CSF withdrawal and the following parameters were measured: peak systolic velocity (PSV), end diastolic velocity (EDV), resistivity index (RI), and pulsatility index (PI). Results Significant relation was found between grades of papilledema and PSV, RI, and PI (p value 0.012, 0.025, 0.016) but no significant relation was found between grades of papilledema and EDV (0.102). Significant changes occurred in parameters of TCD pre- and post-CSF withdrawal including PSV, EDV, and PI (p value 0.001, 0.015, 0.019) denoting a significant change in cerebral hemodynamics after CSF withdrawal which denotes a decrease in intracranial pressure. Conclusion Increased intracranial pressure significantly affects cerebral blood flow. A normalization of transcranial Doppler parameters occurs following lowering of intracranial pressure through lumbar puncture and CSF withdrawal.


2017 ◽  
Vol 9 (1) ◽  
pp. 49-53
Author(s):  
Anas Mohammad Albarrak ◽  
Suleiman Kojan

Introduction: Idiopathic intracranial hypertension is a rare condition characterized by increased intracranial pressure without clinical, laboratory, or radiological evidence of intracranial pathology. Early management could prevent irreversible outcomes. Case Presentation: A 17-year-old single Arabian female of Arab origin presented with a 2-day complaint of horizontal diplopia and transient visual obscurations. She denied any history of headache or decreased vision. The patient was diagnosed with polycystic ovary syndrome a year prior to presentation. Examination revealed bilateral moderate papilledema and limited left eye abduction. However, visual acuity and fields were normal. Increased intracranial pressure was confirmed by lumbar puncture opening pressure (550 mm H<Sub>2</Sub>O). The cerebrospinal fluid composition and imaging of brain and cerebral venous system were normal. The diagnosis of idiopathic intracranial hypertension was confirmed and the patient was treated with acetazolamide 500 mg twice per day. The symptoms totally resolved within 3 days and the papilledema disappeared after 2 months. Conclusion: Awareness of such uncommon presentation of idiopathic intracranial hypertension emphasizes the critical importance of detailed ophthalmic examination and shows the good prognosis of early management.


2021 ◽  
Vol 10 (8) ◽  
pp. 555-557
Author(s):  
Praveena Kiran Kher ◽  
Jigna Mukesh Motwani ◽  
Sachin Vishwanath Daigavane

Majority of idiopathic intracranial hypertension (IIH) cases present with bilateral papilloedema. Unilateral papilloedema is a rare condition in IIH. This is a report of one such unusual case. Papilloedema is clinically defined as swelling of the optic nerve head secondary to raised intracranial pressure.1 Disc oedema is a non-specific term that includes papilloedema but also refers to a disc swollen from other causes. Classically, presence of papilloedema is most commonly due to an intracranial space occupying lesion leading to raised intracranial pressure; however, it may be caused due to other aetiologies such as hydrocephalus, shunt failures, idiopathic intracranial hypertension and drugs.2 Idiopathic intracranial hypertension, also known as pseudotumour cerebri, is a disorder characterised by increased intracranial pressure of unclear pathogenesis in the absence of other structural and obstructive lesions. This is a case of acute established unilateral disc oedema secondary to idiopathic intracranial hypertension.


Author(s):  
Ahmed Arafa Helaly ◽  
Ahmed Ezzat ◽  
Mohammed Gamal Abdellatif

Idiopathic intracranial hypertension is characterized by an increase in intracranial pressure with no significant structural cause. The diagnostic criteria include general signs and symptoms of generalized increased intracranial pressure or papilledema, increased Cerebrospinal Fluid (CSF) pressure (>250 mm H2 O), and normal brain imaging. It usually occurs in adults but rarely can be seen in children. We report a 7-year-old child with a manifestation of increased intracranial tension after the measles vaccine, associated with bilateral sixth nerve palsy and bilateral facial palsy with lower motor neuron features. Normal magnetic resonance imaging and CSF results were normal, except for an increase in CSF pressure. The symptoms began to be resolved after lumbar puncture with the complete resolution of symptoms after one and half months. We add also acetazolamide at the proper dose to reduce intracranial pressure from the first day of diagnosis.


2018 ◽  
Vol 5 (2) ◽  
pp. 51-54
Author(s):  
Sepideh Paybast ◽  
Farhad Assarzadegan ◽  
Behnam Safarpoor Lima ◽  
Mohsen Koosha

Background: Idiopathic intracranial hypertension (IIH) is an increased intracranial pressure with normal cerebrospinal fluid (CSF) characteristic in the absence of identifiable causes. The most important complication of this disorder is visual impairment. So far, no comprehensive study has been done on the relationship between the opening CSF pressure and visual field defect in IIH. Methods: In this study, 35 patients with increased intracranial pressure who fulfilled modified Dandy’s criteria underwent ophthalmologic examination and lumbar puncture. The opening CSF pressure was categorized into mild (25-30), moderate (30-40) and severe (>40). The degree of visual field defect was reported both quantitatively and qualitatively. Eventually, the statistical relationship was established among these variables. Results: The mean opening CSF pressure was 33.71 CmH2o. Twelve patients had minor CSF pressure, whereas in 14 and 9 patients the CSF pressure was respectively moderate and severe. There was not statistically significant relationship between the visual field defect and CSF pressure. The most common patterns of visual field involvement were enlarged blind spot and peripheral restriction. Conclusion: The most important morbidity in IIH is visual impairment. According to the findings, the visual field impairment is not pertinent to CSF pressure. In other words, neither high CSF pressure predicts intense visual defect, nor low CSF pressure indicates minimal visual impairment.


2020 ◽  
Vol 37 (02) ◽  
pp. 175-181
Author(s):  
David Case ◽  
Joshua Seinfeld ◽  
Christopher Roark ◽  
David Kumpe

AbstractIdiopathic intracranial hypertension (IIH) is a disease process of abnormally increased intracranial pressure in the absence of a mass lesion. Medical management, optic nerve fenestration, and surgical shunting procedures have failed to produce consistently successful results. In an unknown percentage of cases, IIH is caused by dural venous sinus obstruction which can be cured by endovascular treatment with dural venous sinus stent placement. This helps prevent progressive vision loss and worsening papilledema caused by underlying increased intracranial pressure from venous outflow obstruction. Patients are required to have an established diagnosis of IIH, preferably made by a neuroophthalmologist, with clearly documented papilledema or at minimum visual disturbance along with lumbar puncture opening pressure greater than 25 cm H2O. Transverse to sigmoid sinus focal narrowing (intraluminal filling defect or extrinsic compression) must be seen during the venous phase of neuroangiography (NA) along with a pressure gradient of 10 mm Hg or greater across the focal narrowing during dural venous sinus pressure monitoring. A successful reduction is defined as a pressure gradient of less than 10 mm Hg after stent placement. Neuroophthalmologic follow-up occurs within 1 to 2 months to assess for changes in papilledema. If papilledema is unchanged or worsened, NA and hemodynamic evaluation is repeated for consideration of restenting. Appropriate patient selection criteria are required for IIH venous sinus stenting. The utilization of refined endovascular techniques along with postprocedure follow-up protocols can ultimately cure IIH for a select group of patients.


Author(s):  
Marina Ybarra ◽  
Tiago Jeronimo dos Santos ◽  
Edjane Santos Queiroz ◽  
Ludmilla Rachid ◽  
Ruth Rocha Franco ◽  
...  

ABSTRACT Objective: To describe a case of a male adolescent with symptomatic idiopathic intracranial hypertension (IIH) associated with obesity treated with bariatric surgery. Case description: A 16-year-and-6-month-old severely obese boy [weight: 133.6 kg; height: 1.74 m (Z score: +0.14); BMI: 44.1 kg/m2 (Z score: +4.4)], Tanner pubertal stage 5, presented biparietal, high-intensity, and pulsatile headaches, about five times per week, associated with nocturnal awakenings, and partial improvement with common analgesics, for three months. Ophthalmologic evaluation evidenced bilateral papilledema. Cranial computed tomography revealed no mass or anatomic abnormalities. Lumbar puncture showed increased intracranial pressure of 40 cmH2O (reference value: <28 cmH2O) with a normal content. After being diagnosed with IIH, the patient was started on acetazolamide. However, after three months, he was still symptomatic. He was diagnosed with obesity due to excess energy intake and, as he had failed to lose weight after a conventional clinical treatment, bariatric surgery was indicated. The patient (at 16 years and nine months) underwent an uncomplicated laparoscopic sleeve gastrectomy. Ophthalmologic evaluation, performed five months after surgery, revealed normal visual acuity in both eyes and improvement of bilateral papilledema. Follow-up at 18 months showed a 67.5% loss of excess weight (weight: 94.5 kg and BMI: 31.2 kg/m2) and complete resolution of IIH symptoms. Comments: IIH is characterized by increased intracranial pressure with no evidence of deformity or obstruction of the ventricular system on neuroimaging. It has been associated with obesity. Bariatric surgery may be a valid alternative approach for morbidly obese adolescent patients with refractory symptoms.


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