scholarly journals Evolving Evidence in Idiopathic Intracranial Hypertension

Life ◽  
2021 ◽  
Vol 11 (11) ◽  
pp. 1225
Author(s):  
Susan P. Mollan ◽  
Heather E. Moss ◽  
Steffen Hamann

Idiopathic intracranial hypertension (IIH) is an enigmatic disorder characterized by raised intracranial pressure (ICP) with no known cause and it affects both children and adults [...]

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.


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.


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.


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.


2020 ◽  
Vol 143 ◽  
pp. 109832
Author(s):  
Panayiotis Patrikelis ◽  
Stefanos Korfias ◽  
Lambros Messinis ◽  
Athanasia Alexoudi ◽  
Themistoklis Papasilekas ◽  
...  

2020 ◽  
Vol 267 (12) ◽  
pp. 3776-3784
Author(s):  
Olivia Grech ◽  
Susan P. Mollan ◽  
Benjamin R. Wakerley ◽  
Zerin Alimajstorovic ◽  
Gareth G. Lavery ◽  
...  

Abstract Purpose Idiopathic intracranial hypertension (IIH) is a rare disorder characterised by raised intracranial pressure. The underlying pathophysiology is mostly unknown and effective treatment is an unmet clinical need in this disease. This review evaluates key emerging themes regarding disease characteristics, mechanisms contributing to raised intracranial pressure and advances in potential therapeutic targets. Findings IIH is becoming more common, with the incidence rising in parallel with the global obesity epidemic. Current medical management remains centred around weight management, which is challenging. Metabolic investigations of patients have identified specific androgen profiles in cerebrospinal fluid (CSF), which suggest an endocrine dysfunction impacting CSF secretion in IIH. Glucagon-like peptide-1 (GLP-1) and 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1) have been found to play a role in CSF dynamics in IIH and have formed the basis of the first clinical trials looking at new treatments. Conclusions Identification of novel molecular targets thought to underlie IIH pathology is now being translated to clinical trials.


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