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International Forum of Allergy &... Jun 2017The conventional treatment for idiopathic intracranial hypertension involves weight loss, steroids, diuretics, and/or serial lumbar punctures; however, if the symptoms... (Review)
Review
BACKGROUND
The conventional treatment for idiopathic intracranial hypertension involves weight loss, steroids, diuretics, and/or serial lumbar punctures; however, if the symptoms persist or worsen, surgical intervention is recommended. Surgical options include cerebrospinal fluid diversion procedures, such as ventriculoperitoneal and lumboperitoneal shunts, and optic nerve decompression with nerve sheath fenestration. The latter can be carried out using an endoscopic approach, but the outcomes of this technique have not been firmly established.
METHODS
This systematic review examined the outcomes of performing endoscopic optic nerve decompression (EOND) in patients with idiopathic intracranial hypertension (IIH). Six studies were included for a total of 34 patients.
RESULTS
The patients presented with visual field disturbances (32 of 32 [100%]), visual acuity disruptions (33 of 34 [97.1%]), papilledema (26 of 34 [76.5%]), and persistent headache (30 of 33 [90.1%]). The mean duration of symptoms ranged from 7 to 32 months. Overall, the patients showed post-EOND improvement in signs and symptoms associated with IIH, specifically visual field deficits (93.8%), visual acuity (85.3%), papilledema (81.4%), and headaches (81.8%). Interestingly, 11 cases showed postoperative improvement in their symptoms with bony decompression of the optic canal alone, without nerve sheath fenestration. There were no major adverse events or complications reported with this approach.
CONCLUSION
EOND appears to be a promising and safe surgical alternative for patients with IIH who fail to respond to medical treatment. Further studies are needed before we can attest to the clinical validity of this procedure.
Topics: Decompression, Surgical; Endoscopy; Humans; Optic Nerve; Pseudotumor Cerebri; Treatment Outcome
PubMed: 28383199
DOI: 10.1002/alr.21927 -
Arquivos Brasileiros de Oftalmologia 2015To investigate the frequency of visual loss (VL), possible predictive factors of VL, and improvement in patients with pseudotumor cerebri (PTC) syndrome.
PURPOSE
To investigate the frequency of visual loss (VL), possible predictive factors of VL, and improvement in patients with pseudotumor cerebri (PTC) syndrome.
METHODS
We reviewed 50 PTC patients (43 females, seven males) who underwent neuro-ophthalmic examination at the time of diagnosis and after treatment. Demographic data, body mass index (BMI), time from symptom onset to diagnosis (TD), maximum intracranial pressure (MIP), occurrence of cerebral venous thrombosis (CVT), and treatment modalities were reviewed. VL was graded as mild, moderate, or severe on the basis of visual acuity and fields. Predictive factors for VL and improvement were assessed by regression analysis.
RESULTS
The mean ± SD age, BMI, and MIP were 35.2 ± 12.7 years, 32.0 ± 7.5 kg/cm2, and 41.9 ± 14.5 cmH2O, respectively. Visual symptoms and CVT were present in 46 and eight patients, respectively. TD (in months) was <1 in 21, 1-6 in 15, and >6 in 14 patients. Patients received medical treatment with (n=20) or without (n=30) surgery. At presentation, VL was mild in 16, moderate in 12, and severe in 22 patients. Twenty-eight patients improved and five worsened. MIP, TD, and hypertension showed a significant correlation with severe VL. The best predictive factor for severe VL was TD >6 months (p=0.04; odds ratio, 5.18). TD between 1 and 6 months was the only factor significantly associated with visual improvement (p=0.042).
CONCLUSIONS
VL is common in PTC, and when severe, it is associated with a delay in diagnosis. It is frequently permanent; however, improvement may occur, particularly when diagnosed within 6 months of symptom onset.
Topics: Adult; Body Mass Index; Carbonic Anhydrase Inhibitors; Contraceptives, Oral; Delayed Diagnosis; Female; Headache; Humans; Intracranial Pressure; Male; Middle Aged; Predictive Value of Tests; Pseudotumor Cerebri; Recovery of Function; Regression Analysis; Sinus Thrombosis, Intracranial; Time Factors; Vision Disorders; Visual Acuity; Visual Field Tests; Young Adult
PubMed: 26222108
DOI: 10.5935/0004-2749.20150045 -
Cleveland Clinic Journal of Medicine 1990The spectrum of chronic urticarial disease ranges from chronic urticarial skin lesions alone to well-characterized systemic lupus erythematosus with urticarial...
The spectrum of chronic urticarial disease ranges from chronic urticarial skin lesions alone to well-characterized systemic lupus erythematosus with urticarial vasculitis as the major skin manifestation. Within this spectrum is the syndrome of urticarial vasculitis associated with systemic disease manifestations. There have been six previously recorded cases of urticarial vasculitis associated with pseudotumor cerebri. At least two of these have included membranoproliferative glomerulonephritis. The authors report a case of chronic urticarial disease associated with pseudotumor cerebri and membranoproliferative glomerulonephritis, but without demonstrable vasculitis. It is possible that this represents a distinct entity within the spectrum of chronic urticarial disease and cca be easily screened for in clinical practice.
Topics: Acetazolamide; Adult; Biopsy; Chronic Disease; Dipyridamole; Female; Glomerulonephritis, Membranoproliferative; Humans; Immunohistochemistry; Kidney Glomerulus; Prednisone; Pseudotumor Cerebri; Urticaria
PubMed: 2323023
DOI: 10.3949/ccjm.57.2.197 -
The British Journal of Radiology Aug 2022Idiopathic intracranial hypertension (IIH) represents a clinical disease entity without a clear etiology, that if left untreated, can result in severe outcomes,... (Review)
Review
Idiopathic intracranial hypertension (IIH) represents a clinical disease entity without a clear etiology, that if left untreated, can result in severe outcomes, including permanent vision loss. For this reason, early diagnosis and treatment is necessary. Historically, the role of cross-sectional imaging has been to rule out secondary or emergent causes of increased intracranial pressure, including tumor, infection, hydrocephalus, or venous thrombosis. MRI and MRV, however, can serve as valuable imaging tools to not only rule out causes for secondary intracranial hypertension but can also detect indirect signs of IIH resultant from increased intracranial pressure, and demonstrate potentially treatable sinus venous stenosis. Digital subtraction venographic imaging also plays a central role in both diagnosis and treatment, providing enhanced anatomic delineation and temporal flow evaluation, quantitative assessment of the pressure gradient across a venous stenosis, treatment guidance, and immediate opportunity for endovascular therapy. In this review, we discuss the multiple modalities for imaging IIH, their limitations, and their contributions to the management of IIH.
Topics: Constriction, Pathologic; Cranial Sinuses; Humans; Phlebography; Pseudotumor Cerebri; Stents; Vascular Diseases
PubMed: 35522777
DOI: 10.1259/bjr.20220136 -
Clinical Imaging 2018This study determines the reproducibility of magnetic resonance elastography (MRE) derived brain stiffness in normal volunteers and compares it against pseudotumor...
This study determines the reproducibility of magnetic resonance elastography (MRE) derived brain stiffness in normal volunteers and compares it against pseudotumor patients before and after lumbar puncture (LP). MRE was performed on 10 normal volunteers for reproducibility and 14 pseudotumor patients before and after LP. During LP, opening and closing cerebrospinal fluid (CSF) pressures were recorded before and after removal of CSF and correlated to brain stiffness. Stiffness reproducibility was observed (r > 0.78; p < 0.008). Whole brain opening LP stiffness was significantly (p = 0.04) higher than normals, but no significant difference (p = 0.11) in closing LP measurements. No significant correlation was observed between opening and closing pressure and brain stiffness.
Topics: Adult; Brain; Female; Humans; Intracranial Pressure; Magnetic Resonance Imaging; Male; Pseudotumor Cerebri; Reproducibility of Results; Severity of Illness Index; Young Adult
PubMed: 29459315
DOI: 10.1016/j.clinimag.2018.02.005 -
Journal of Clinical Neuroscience :... Feb 2022The diagnostic utility of neuroradiologic signs associated with idiopathic intracranial hypertension (IIH) for the evaluation of patients presenting with papilloedema...
The diagnostic utility of neuroradiologic signs associated with idiopathic intracranial hypertension (IIH) for the evaluation of patients presenting with papilloedema remains yet to be elucidated. This multicentre retrospective cohort study assessed consecutive patients presenting with suspected papilloedema to Auckland District Health Board (NZ) and Stanford University Medical Centre (US), between 2005 and 2019, undergoing magnetic resonance imaging and venography (MRI/MRV) or computed tomography and venography (CT/CTV) prior to lumbar puncture assessment for diagnostic suspicion of IIH. Data were collected regarding demographic, clinical, radiologic, and lumbar puncture parameters, and the diagnosis of IIH was determined according to the Friedman criteria for primary pseudotumor cerebri syndrome. A total of 204 participants (174 females; mean ± SD age 29.9 ± 12.2 years) were included, and 156 (76.5%) participants fulfilled the diagnostic criteria for IIH. The presence of any IIH-associated radiologic sign on MRI/MRV demonstrated a sensitivity (95% CI) of 74.8% (65.8%-82.0%) and specificity (95% CI) of 94.7% (82.7%-98.5%), while radiologic signs on CT/CTV exhibited a sensitivity (95% CI) of 61.0% (49.9%-71.2%) and specificity (95% CI) of 100.0% (83.2%-100.0%). In summary, the modest sensitivities of radiologic signs of IIH would support the routine use of lumbar puncture assessment following neuroimaging to secure the diagnosis. However, the high specificities might lend limited support for the judicious deferment of lumbar puncture assessment among typical IIH demographic patients who consent to the inherent small risk of missed pathology, which has been proposed by some clinicians.
Topics: Adolescent; Adult; Female; Humans; Intracranial Hypertension; Neuroimaging; Papilledema; Phlebography; Pseudotumor Cerebri; Retrospective Studies; Young Adult
PubMed: 34974249
DOI: 10.1016/j.jocn.2021.12.024 -
Romanian Journal of Ophthalmology 2021Idiopathic intracranial hypertension (IIH) is a neuro-ophthalmological syndrome of unknown cause that can be vision-threatening, so an early diagnosis is crucial. We...
Idiopathic intracranial hypertension (IIH) is a neuro-ophthalmological syndrome of unknown cause that can be vision-threatening, so an early diagnosis is crucial. We reported a case of a 68-year-old asymptomatic male referred with a cataract in his right eye (OD). Best-corrected visual acuity (BCVA) was 70 letters (20/ 40) in the OD and 85 letters (20/ 20) in the left eye (OS). Ophthalmological examination revealed a significant nuclear cataract in the OD that explained the visual acuity. Fundus imaging showed a faint nasal margin elevation of the optic disc of both eyes (OU). Optical coherence tomography (OCT) revealed a sectorial retinal nerve fiber layer (RNFL) atrophy in the inferior quadrant in the OS. Nevertheless, visual field (VF) did not demonstrate defects. Neuroimaging was normal and examination of CSF revealed an opening pressure of 500 mmH2O. A diagnosis of IIH was confirmed and acetazolamide 250 mg twice daily was recommended. After 12 months of follow-up, RNFL thickness remained stable and VF did not confirm defects. A routine eye examination was the onset of IIH in our case. Thus, the ophthalmologist played a crucial role in the early diagnosis of this syndrome. Papilledema is usually a key criterion for IIH, so after its detection, exclusion diagnosis and treatment should be initiated in order to avoid permanent visual loss.
Topics: Aged; Humans; Male; Optic Disk; Papilledema; Pseudotumor Cerebri; Retinal Ganglion Cells; Tomography, Optical Coherence
PubMed: 34179586
DOI: 10.22336/rjo.2021.37 -
Journal of Neurology, Neurosurgery, and... Jun 2004Intracranial pressure (ICP) is derived from cerebral blood and cerebrospinal fluid (CSF) circulatory dynamics and can be affected in the course of many diseases of the... (Review)
Review
Intracranial pressure (ICP) is derived from cerebral blood and cerebrospinal fluid (CSF) circulatory dynamics and can be affected in the course of many diseases of the central nervous system. Monitoring of ICP requires an invasive transducer, although some attempts have been made to measure it non-invasively. Because of its dynamic nature, instant CSF pressure measurement using the height of a fluid column via lumbar puncture may be misleading. An averaging over 30 minutes should be the minimum, with a period of overnight monitoring in conscious patients providing the optimal standard. Computer-aided recording with online waveform analysis of ICP is very helpful. Although there is no "Class I" evidence, ICP monitoring is useful, if not essential, in head injury, poor grade subarachnoid haemorrhage, stroke, intracerebral haematoma, meningitis, acute liver failure, hydrocephalus, benign intracranial hypertension, craniosynostosis etc. Information which can be derived from ICP and its waveforms includes cerebral perfusion pressure (CPP), regulation of cerebral blood flow and volume, CSF absorption capacity, brain compensatory reserve, and content of vasogenic events. Some of these parameters allow prediction of prognosis of survival following head injury and optimisation of "CPP-guided therapy". In hydrocephalus CSF dynamic tests aid diagnosis and subsequent monitoring of shunt function.
Topics: Blood Pressure; Cerebrovascular Circulation; Craniocerebral Trauma; Humans; Hydrocephalus; Intracranial Pressure; Monitoring, Physiologic; Prognosis; Pseudotumor Cerebri
PubMed: 15145991
DOI: 10.1136/jnnp.2003.033126 -
Pediatric Neurology Oct 2019Revised diagnostic criteria for pseudotumor cerebri syndrome require three of four neuroimaging findings in the absence of papilledema. We examined the sensitivity and...
BACKGROUND
Revised diagnostic criteria for pseudotumor cerebri syndrome require three of four neuroimaging findings in the absence of papilledema. We examined the sensitivity and specificity of three or more of four of these magnetic resonance imaging (MRI) findings for pseudotumor cerebri syndrome in children.
METHODS
As part of clinical care, patients in whom there was suspicion for pseudotumor cerebri syndrome underwent neurological and fundoscopic examinations, lumbar puncture, MRI, or magnetic resonance venogram. For this retrospective study, we used this information to classify 119 subjects into definite (n = 66) or probable pseudotumor cerebri syndrome (n = 12), elevated opening pressure without papilledema (n = 23), or controls who had normal opening pressure without papilledema (n = 24). A neuroradiologist, unaware of the clinical findings or original MRI report, reviewed MRIs for pituitary gland flattening, flattening of the posterior sclera, optic nerve sheath distention, and transverse venous sinus stenosis.
RESULTS
The presence of three or more MRI findings has a sensitivity of 62% (95% confidence interval: 47% to 75%) and a specificity of 95% (95% confidence interval: 77% to 100%), compared with controls. Two of three (transverse venous sinus stenosis, pituitary gland flattening, flattening of the posterior sclera) had a similar sensitivity and specificity. Transverse venous sinus stenosis alone had a slightly higher sensitivity (74%, 95% confidence interval: 60% to 85%) and specificity (100%, 95% confidence interval: 80% to 100%).
CONCLUSIONS
In children, three of four of the proposed neuroimaging criteria and transverse venous sinus stenosis alone have a moderate sensitivity and robust specificity for pseudotumor cerebri syndrome. MRIs should be reviewed for these criteria, and their presence should raise suspicion for pseudotumor cerebri syndrome in children, particularly if the presence of papilledema is uncertain.
Topics: Adolescent; Child; Confidence Intervals; Constriction, Pathologic; Cranial Sinuses; Female; Humans; Magnetic Resonance Imaging; Male; Neuroimaging; Optic Nerve; Papilledema; Pituitary Gland; Pseudotumor Cerebri; Retrospective Studies; Sclera; Sensitivity and Specificity; Transverse Sinuses
PubMed: 31303369
DOI: 10.1016/j.pediatrneurol.2019.04.010 -
Neurology May 2014
Topics: Brain; Female; Humans; Male; Neuroimaging; Pseudotumor Cerebri
PubMed: 24936621
DOI: 10.1212/wnl.0000000000000510