scholarly journals Current Perspectives on Idiopathic Intracranial Hypertension without Papilloedema

Life ◽  
2021 ◽  
Vol 11 (6) ◽  
pp. 472
Author(s):  
Susan P. Mollan ◽  
Yu Jeat Chong ◽  
Olivia Grech ◽  
Alex J. Sinclair ◽  
Benjamin R. Wakerley

The pseudotumor cerebri syndrome embraces disorders characterised by raised intracranial pressure, where the commonest symptom is headache (90%). Idiopathic intracranial hypertension without papilloedema (IIHWOP) is increasingly recognised as a source of refractory headache symptoms and resultant neurological disability. Although the majority of patients with IIHWOP are phenotypically similar to those with idiopathic intracranial hypertension (IIH), it remains uncertain as to whether IIHWOP is nosologically distinct from IIH. The incidence, prevalence, and the degree of association with the world-wide obesity epidemic is unknown. Establishing a diagnosis of IIHWOP can be challenging, as often lumbar puncture is not routinely part of the work-up for refractory headaches. There are published diagnostic criteria for IIHWOP; however, some report uncertainty regarding a pathologically acceptable cut off for a raised lumbar puncture opening pressure, which is a key criterion. The literature provides little information to help guide clinicians in managing patients with IIHWOP. Further research is therefore needed to better understand the mechanisms that drive the development of chronic daily headaches and a relationship to intracranial pressure; and indeed, whether such patients would benefit from therapies to lower intracranial pressure. The aim of this narrative review was to perform a detailed search of the scientific literature and provide a summary of historic and current opinion regarding IIHWOP.

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.


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.


2021 ◽  
Vol 12 ◽  
pp. 493
Author(s):  
Thomas Markus Dhaese ◽  
Leonardo C. Welling ◽  
Alice Magro Kosciasnki ◽  
Gustavo Frigeri ◽  
Judy Auada ◽  
...  

Background: Intracranial pressure (ICP) monitoring has been variously explored as a diagnostic and therapeutic modality in many pathological conditions leading neurological injury. This monitoring standardly depends on an invasive procedure such as cranial or lumbar catheterization. The gold standard for ICP monitoring is through an intraventricular catheter, but this invasive technique is associated with certain risks such as haemorrhage and infection. (1) Also, it is a high-cost procedure and consequently not available in a variety of underprivileged places and clinical situations in which intracranial hypertension is prevalent (3). An accurate non-invasive and low-priced method to measure elevated ICP would therefore be desirable. Under these circumstances, Brazilian scientists developed a non-invasive method for intracranial pressure monitoring (ICP-NI), which uses an electric resistance extensometer that measures micro deformations of the skull and transforms it into an electrical signal. In this case report, the authors describe a pediatrician patient with the diagnosis of idiopathic intracranial hypertension who was successfully submitted to a lumbar puncture under monitorization with this device. Case description: 7 year old girl with progressive symptoms that lead to the diagnosis of idiopathic intracranial hypertension. The patient was submitted to a lumbar punction with continuous non-invasive ICP monitoring. Conclusion: Estimating ICP (non-invasive) from LP monitoring (invasive) often reflect inaccurate ICP results, and affects negatively on IIH diagnosis and a non-invasive diagnostic method could reduce the requirement for invasive approaches, improving patient health outcomes.


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 ◽  
pp. 088307382097223
Author(s):  
Erin Yamamoto ◽  
Darren Farber ◽  
David Rothner ◽  
Manikum Moodley

Pseudotumor cerebri also known as idiopathic intracranial hypertension is a relatively uncommon disorder of unknown pathophysiology. Although pseudotumor cerebri occurs in both children and adults, the pseudotumor cerebri literature is heavily dominated by adult studies. The aim of this study is to retrospectively describe the clinical presentation, imaging, treatment, and outcomes of a large pediatric pseudotumor cerebri population over a 23-year period. We also discuss secondary pseudotumor cerebri (44%) as well as the increasingly recognized patient subgroups without headache (13.3%) and without papilledema (7.3%). Female sex, obesity, and initial symptoms were consistent with the literature; however radiographic findings were surprisingly low in this cohort. Headache outcomes at 1 week, 1 month, and 3 months following initial lumbar puncture/treatment and visual function outcomes are reported.


2020 ◽  
Vol 31 (11) ◽  
pp. 1117-1119
Author(s):  
Sharon Chi ◽  
Amy Weintrob

Idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, is a diagnosis of exclusion. Elevated intracranial pressure (ICP) can result from a variety of inflammatory and structural causes affecting cerebrospinal fluid production and absorption. First described in 1935, syphilis is a well-established cause of elevated ICP, referred to as syphilitic hydrocephalus. We report a case of a 49-year-old man presenting with vision changes and headache who was treated for IIH without resolution of symptoms, and eventually diagnosed with syphilitic hydrocephalus. Syphilis should be considered as a cause of elevated ICP prior to a diagnosis of IIH.


Author(s):  
Julia Juhász ◽  
Johannes Hensler ◽  
Olav Jansen

Background Pseudotumor cerebri, also known as idiopathic intracranial hypertension, is a disorder of increased intracranial pressure of unknown etiology. Main symptom is headache which has a characteristic similar to other headache diseases, therefore the idiopathic intracranial hypertension often remains undetected. The incidence is 1/100 000 with the number of unreported cases being much higher. This article highlights the essential role of MRI in the differential diagnosis. Methods A literature search was carried out on idiopathic intracranial hypertension and Pseudotumor cerebri in English and German. Original and Review articles as well as case reports were taken into account. Since the main focus of the article is MRI diagnostics, some images were selected to illustrate the characteristic MRI morphological features. Results and Conclusion The diagnosis of idiopathic intracranial hypertension is based on an exclusion procedure. Most common syndromes are headache, transient visual obscurations, pulsatile tinnitus and nausea. In the presence of an underlying cause for the increased intracranial pressure one speaks of a secondary intracranial hypertension. The diagnostics include a detailed medical history, neurological and ophthalmic examination, lumbar puncture, and neuroradiological imaging procedures. MRI, in particular, has become increasingly important in recent years, since signs for changes in cerebrospinal fluid pressure are now detectable and well-defined. The therapeutic approaches are symptom-oriented and aim to lower the pressure. With a precise diagnosis and timely start of therapy, idiopathic intracranial hypertension has a good prognosis, especially with regard to the preservation of eyesight. Key Points:  Citation Format


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