Idiopathic intracranial hypertension

2020 ◽  
pp. 6054-6059
Author(s):  
Alexandra Sinclair

Idiopathic intracranial hypertension (IIH) (also called pseudotumour cerebri and, previously, benign intracranial hypertension) is a syndrome of raised intracranial pressure in the absence of an intracranial mass lesion, enlargement of the cerebral ventricles, or venous sinus thrombosis. IIH affects predominantly obese women of childbearing age (>90%). The condition has considerable morbidity from permanent visual loss (up to 25% of cases) and chronic disabling headaches, which result in poor quality of life. Patients presenting acutely with papilloedema must be evaluated urgently for secondary causes of raised intracranial pressure (e.g. space occupying lesion and venous thrombosis). After this, the priority is to assess accurately the threat to vision. In most patients, the condition becomes chronic and the disease burden is mostly from chronic headaches, which need active management, alongside visual monitoring. This chapter does not cover paediatric IIH.

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.


2018 ◽  
Vol 89 (10) ◽  
pp. A9.4-A10
Author(s):  
Vijay Vivek ◽  
Grant Laura ◽  
Nagar Anindyt ◽  
Bahra Anish

Recent studies have yielded the first evidence-based treatments for idiopathic intracranial hypertension (IIH) and shed light on many aspects of this condition, providing an opportunity to streamline the assessment and management of patients with IIH.This study highlighted the inefficiency of most patient pathways, especially when via outpatients, with resulting delays in diagnosis and /or management. Of note, 37% of patients did not have venography. Up to a third of patients with cerebral venous sinus thrombosis present with isolated signs of elevated intracranial pressure, thus clinically mimicking IIH and emphasising the importance of venography acutely.Acetazolamide was appropriately commenced but rarely titrated to the maximum dose of 4 g/day as supported by the IIHTT. Over 40% of our cohort had a diagnosable co-morbid primary headache disorder which persisted and required additional headache-specific medication in almost half of these. This is in keeping with studies showing that headache is unrelated to raised intracranial pressure in IIH patients, and headache-specific management is a key aspect to the management of IIH patients.In summary, this study highlights significant delays and missed investigations when not performed acutely, emphasising the importance of implementing a clear and visible pathway for the assessment and management of IIH patients.


2019 ◽  
pp. 47-54
Author(s):  
Matthew J. Thurtell ◽  
Robert L. Tomsak

Idiopathic intracranial hypertension is a syndrome of increased intracranial pressure of unknown cause that most often occurs in obese women of childbearing age, although it can also occur in children, men, and older adults. Papilledema is usually present and can cause irreversible vision loss if left untreated. In this chapter, we begin by reviewing the symptoms of this condition, which include headache, transient visual obscurations, and pulse-synchronous tinnitus. We next review the clinical signs and common findings on formal visual field testing. We then discuss the diagnostic evaluation and review the diagnostic criteria for this condition. Lastly, we review the goals and management of this condition, which must be tailored depending on the severity of symptoms, papilledema, and vision loss.


2011 ◽  
pp. 29-35
Author(s):  
Matthew J. Thurtell ◽  
Robert L. Tomsak ◽  
Robert B. Daroff

Idiopathic intracranial hypertension is a syndrome of raised intracranial pressure of unknown cause that most often occurs in obese young women. Bilateral papilledema is usually present and can cause severe, irreversible vision loss if left untreated. In this chapter, we review the symptoms, signs, evaluation, and management of idiopathic intracranial hypertension.


2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Kenneth R. Hoffman ◽  
Sean W. Chan ◽  
Andrew R. Hughes ◽  
Stephen J. Halcrow

Lumbar puncture is performed routinely for diagnostic and therapeutic purposes in idiopathic intracranial hypertension, despite lumbar puncture being classically contraindicated in the setting of raised intracranial pressure. We report the case of a 30-year-old female with known idiopathic intracranial hypertension who had cerebellar tonsillar herniation following therapeutic lumbar puncture. Management followed guidelines regarding treatment of traumatic intracranial hypertension, including rescue decompressive craniectomy. We hypothesize that the changes in brain compliance that are thought to occur in the setting of idiopathic intracranial hypertension are protective against further neuronal injury due to axonal stretch following decompressive craniectomy.


2017 ◽  
Vol 08 (S 01) ◽  
pp. S106-S110 ◽  
Author(s):  
Girish Baburao Kulkarni ◽  
Ravinder Jeet Singh ◽  
Veeranna Gadad ◽  
Subasree Ramakrishnan ◽  
Veerendrakumar Mustare

ABSTRACTIn the majority of patients with raised intracranial pressure, the papilledema is bilateral. Unilateral papilledema is rare in conditions causing intracranial hypertension, and it has been described in Foster–Kennedy syndrome and in some cases of idiopathic intracranial hypertension. It has never been reported in cerebral venous thrombosis. We report a young lady presenting with features of subacute onset of headache with seizures, on evaluation she had superior sagittal and bilateral lateral sinus thrombosis. The risk factors found on evaluation were Vitamin B12 deficiency and hyperhomocysteinemia. On optic fundus examination, she had swollen optic disc on the right side with normal fundus on the left side, confirmed with the orbital ultrasound B-scan and optic coherence tomography. Her magnetic resonance imaging showed features of raised intracranial pressure with thrombosis of the superior sagittal and bilateral lateral sinus thrombosis. She was treated with anticoagulation (heparin followed by oral anticoagulants), antiedema measures, and vitamin supplementation for hyperhomocysteinemia. She improved over time and was asymptomatic during follow-up. We discuss the possible mechanisms described in the literature for unilateral papilledema. This report highlights the need for carefully performing bilateral fundus examination so as not to miss the vision or life-threatening causes of a headache.


2018 ◽  
Vol 31 (5) ◽  
pp. 490-495 ◽  
Author(s):  
Nirmeen A Kishk ◽  
Asmaa M Ebraheim ◽  
Amal S Ashour ◽  
Nashwa M Badr ◽  
Mohamed A Eshra

Purpose Monitoring of raised intracranial pressure (ICP) in patients with idiopathic intracranial hypertension (IIH) is required to prevent secondary optic nerve damage. Sonographic measurement of the optic nerve sheath diameter (ONSD) is a noninvasive method to evaluate intracranial hypertension. Different ONSD cut-off values have been reported probably due to ethnic variations. Our aim was to determine optic nerve sonographic examination cut-off points to predict raised ICP in IIH patients. Methods This case-control study was conducted on 99 IIH post-pubertal female patients (both probable and definite) and 35 age- and sex-matched healthy volunteers. Sonographic ONSD and optic nerve diameter (OND) were obtained 3 mm behind the posterior edge of the globe in a horizontal plane via a 7–13 MHz linear probe. Lumbar puncture was then carried out on the patients. Results The opening cerebrospinal fluid pressure documented in the patient group was 279.64 ± 65.97 mm H2O. A statistically significant difference was found between IIH patients and controls regarding ONSD. The best ONSD cut-off value indicating raised ICP was 6.05 mm with an area under the curve of 0.850 (95% confidence interval 0.805 to 0.894, 73.2% sensitivity and 91.4% specificity). Regarding OND/ONSD ratio, there was an insignificant difference between both groups. Conclusion Sonographic ONSD but not OND/ONSD ratio could offer a bedside adjunct or alternative indicator of elevated ICP in IIH patients. Ethnic differences, however, should be noted when using this parameter.


Cephalalgia ◽  
2014 ◽  
Vol 35 (3) ◽  
pp. 248-261 ◽  
Author(s):  
BR Wakerley ◽  
MH Tan ◽  
EY Ting

Background Idiopathic intracranial hypertension or pseudotumour cerebri is primarily a disorder of young obese women characterised by symptoms and signs associated with raised intracranial pressure in the absence of a space-occupying lesion or other identifiable cause. Summary The overall incidence of idiopathic intracranial hypertension is approximately two per 100,000, but is considerably higher among obese individuals and, given the global obesity epidemic, is likely to rise further. The pathophysiology of this condition is poorly understood, but most theories focus on the presence of intracranial venous hypertension and/or increased cerebrospinal fluid outflow resistance and how this relates to obesity. A lack of randomised clinical trials has resulted in unsatisfactory treatment guidelines and although weight loss is important, especially when used in conjunction with drugs that reduce cerebrospinal fluid production, resistant cases remain difficult to manage and patients invariably undergo neurosurgical shunting procedures. The use of transverse cerebral sinus stenting remains contentious and long-term benefits are yet to be determined. Conclusion An understanding of the clinical features, diagnostic work-up and therapeutic options available for patients with idiopathic intracranial hypertension is important both for neurologists and ophthalmologists as visual loss maybe permanent if untreated.


2018 ◽  
Vol 29 (4) ◽  
pp. NP1-NP4 ◽  
Author(s):  
Justin Yeak ◽  
Mimiwati Zahari ◽  
Sujaya Singh ◽  
Nor Fadhilah Mohamad

Background: Acute ophthalmoparesis without ataxia was designated as ‘atypical Miller Fisher syndrome’ as it presents with progressive, relatively symmetrical ophthalmoplegia, but without ataxia nor limb weakness, in the presence of anti-GQ1b antibody. Idiopathic intracranial hypertension is characterized by signs of raised intracranial pressure occurring in the absence of cerebral pathology, with normal composition of cerebrospinal fluid and a raised opening pressure of more than 20 cmH2O during lumbar puncture. We aim to report a rare case of acute ophthalmoplegia with co-occurrence of raised intracranial pressure. Case Description: A 28-year-old gentleman with body mass index of 34.3 was referred to us for management of double vision of 2 weeks duration. His symptom started after a brief episode of upper respiratory tract infection. His best corrected visual acuity was 6/6 OU. He had bilateral sixth nerve palsy worse on the left eye and bilateral hypometric saccade. His deep tendon reflexes were found to be hyporeflexic in all four limbs. No sensory or motor power deficit was detected, and his gait was normal. Plantar reflexes were downwards bilaterally and cerebellar examination was normal. Both optic discs developed hyperaemia and swelling. Magnetic resonance imaging of brain was normal and lumbar puncture revealed an opening pressure of 50 cmH2O. Anti-GQ1b IgG and anti-GT1a IgG antibody were tested positive. Conclusion: Acute ophthalmoparesis without ataxia can present with co-occurrence of raised intracranial pressure. It is important to have a full fundoscopic assessment to look for papilloedema in patients presenting with Miller Fisher syndrome or acute ophthalmoparesis without ataxia.


Sign in / Sign up

Export Citation Format

Share Document