Rzekomy guz mózgu (idiopatyczne nadciśnienie śródczaszkowe)
Diagnostyka i diagnoza
Idiopatyczne nadciśnienie śródczaszkowe (IIH), dawniej określane jako rzekomy guz mózgu, charakteryzuje się podwyższonym ciśnieniem wewnątrzczaszkowym bez widocznej przyczyny. Diagnostyka opiera się na kryteriach Friedmana (2013), które wymagają obecności objawów podwyższonego ciśnienia śródczaszkowego, braku zmian neurologicznych poza porażeniem nerwu VI i tarczą zastoinową, prawidłowych wyników badań neuroobrazowych (MRI z kontrastem, MRV), podwyższonego ciśnienia otwarcia płynu mózgowo-rdzeniowego (≥25 cm H₂O u dorosłych, ≥28 cm H₂O u dzieci w sedacji) oraz prawidłowego składu płynu. Kluczowe jest badanie okulistyczne, w tym ocena dna oka, pole widzenia, OCT i USG B, które pozwalają na wykrycie tarczy zastoinowej i monitorowanie zmian. Charakterystyczne zmiany w obrazowaniu to puste siodło tureckie, spłaszczenie tylnej części gałki ocznej, poszerzenie przestrzeni podpajęczynówkowej wokół nerwu wzrokowego oraz zwężenie zatok żylnych poprzecznych.
Diagnostyka rzekomego guza mózgu (idiopatycznego nadciśnienia śródczaszkowego)
Rzekomy guz mózgu (idiopatyczne nadciśnienie śródczaszkowe, IIH) to stan charakteryzujący się podwyższonym ciśnieniem wewnątrzczaszkowym bez widocznej przyczyny. Objawy imitują guza mózgu, stąd nazwa „rzekomy guz mózgu”, choć obecnie preferuje się określenie „idiopatyczne nadciśnienie śródczaszkowe”.12 Diagnostyka tego schorzenia jest procesem wykluczającym – diagnoza zostaje postawiona, gdy inne potencjalne przyczyny nadciśnienia śródczaszkowego zostaną wykluczone.34
Kryteria diagnostyczne
Diagnostyka IIH opiera się głównie na zmodyfikowanych kryteriach Dandy’ego, które ewoluowały na przestrzeni lat. Obecnie stosowane są różne wersje tych kryteriów, w tym kryteria Friedmana z 2013 roku.56 Według tych kryteriów, do rozpoznania IIH konieczne jest stwierdzenie:
- Objawów i oznak podwyższonego ciśnienia śródczaszkowego57
- Braku zmian neurologicznych (z wyjątkiem porażenia nerwu VI i tarczy zastoinowej)8
- Prawidłowych wyników badań neuroobrazowych (z wyjątkiem niektórych zmian charakterystycznych dla IIH)9
- Podwyższonego ciśnienia otwarcia płynu mózgowo-rdzeniowego (≥25 cm H₂O u dorosłych, ≥28 cm H₂O u dzieci w sedacji)810
- Prawidłowego składu płynu mózgowo-rdzeniowego7
- Braku innej przyczyny podwyższonego ciśnienia śródczaszkowego5
Warto zauważyć, że nowsze kryteria Friedmana z 2013 roku uwzględniają również przypadki IIH bez tarczy zastoinowej, gdzie wymagane jest występowanie porażenia nerwu VI lub co najmniej trzech z czterech charakterystycznych zmian neuroobrazowych.1112
Badanie okulistyczne
Pierwszym krokiem w diagnostyce IIH jest szczegółowe badanie okulistyczne, które ma kluczowe znaczenie w procesie diagnostycznym.1314 Obejmuje ono:
- Badanie dna oka w celu wykrycia tarczy zastoinowej (obrzęku tarczy nerwu wzrokowego) – charakterystycznego objawu IIH, obecnego u większości pacjentów1516
- Badanie pola widzenia w celu wykrycia ubytków, szczególnie w obwodowej części pola widzenia117
- Fotografię dna oka do dokumentacji i monitorowania stanu tarczy nerwu wzrokowego1819
- Optyczną koherentną tomografię (OCT) – nieinwazyjne badanie umożliwiające ilościową ocenę obrzęku tarczy nerwu wzrokowego2019
- Ultrasonografię gałki ocznej (USG B) – pomocną w różnicowaniu tarczy zastoinowej od pseudotarczy zastoinowej19
Badania neuroobrazowe
Badania obrazowe mózgu są niezbędne do wykluczenia innych przyczyn podwyższonego ciśnienia śródczaszkowego, takich jak guzy, zakrzepy zatok żylnych czy wodogłowie.1421 Zalecane badania to:
- Rezonans magnetyczny (MRI) mózgu z kontrastem i bez – preferowana metoda ze względu na dokładność i możliwość wykrycia subtelnych zmian2223
- Wenografia rezonansu magnetycznego (MRV) – kluczowa do wykluczenia zakrzepicy zatok żylnych i oceny zwężeń zatok żylnych2425
- Tomografia komputerowa (CT) – alternatywa dla pacjentów, którzy nie mogą mieć wykonanego MRI2627
W badaniach obrazowych u pacjentów z IIH mogą być widoczne charakterystyczne zmiany wspierające diagnozę:1119
- Puste siodło tureckie (empty sella)
- Spłaszczenie tylnej części gałki ocznej
- Poszerzenie przestrzeni podpajęczynówkowej wokół nerwu wzrokowego
- Zwężenie zatok żylnych poprzecznych
Nakłucie lędźwiowe
Nakłucie lędźwiowe (punkcja lędźwiowa) jest kluczowym badaniem w diagnostyce IIH, które pozwala zmierzyć ciśnienie otwarcia płynu mózgowo-rdzeniowego oraz zbadać jego skład.1428 Procedura ta powinna być wykonywana po wykluczeniu zmian strukturalnych w badaniach neuroobrazowych.297
Podczas nakłucia lędźwiowego:2030
- Pacjent powinien być w pozycji leżącej na boku, zrelaksowany, aby uniknąć fałszywie podwyższonych wyników
- Mierzone jest ciśnienie otwarcia – u pacjentów z IIH wynosi ono ≥25 cm H₂O u dorosłych lub ≥28 cm H₂O u dzieci
- Pobierana jest próbka płynu mózgowo-rdzeniowego do analizy (wygląd, przejrzystość, kolor, liczba komórek, cytologia, posiew, poziom glukozy, białka i elektrolitów)
W przypadku IIH, ciśnienie otwarcia jest podwyższone, natomiast skład płynu mózgowo-rdzeniowego pozostaje prawidłowy.3115
Diagnostyka różnicowa
Diagnostyka różnicowa IIH obejmuje wykluczenie innych stanów mogących powodować podobne objawy:329
- Guzy mózgu
- Zakrzepica zatok żylnych mózgu
- Wodogłowie
- Infekcje (zapalenie opon mózgowo-rdzeniowych)
- Zaburzenia endokrynologiczne
- Zatrucia lub działania niepożądane leków
Szczególną uwagę należy zwrócić na pacjentów niebędących „typowymi” przypadkami IIH (tj. osoby niebędące otyłymi kobietami w wieku rozrodczym), u których konieczne jest bardziej szczegółowe badanie w kierunku wtórnych przyczyn nadciśnienia śródczaszkowego.3334
Trudności diagnostyczne i wyzwania
Diagnostyka IIH może być wyzwaniem z kilku powodów:3335
- Objawy mogą być nieswoiste i wspólne z innymi schorzeniami, np. migreną36
- Ciśnienie płynu mózgowo-rdzeniowego podlega naturalnym wahaniom w ciągu dnia, co może prowadzić do fałszywie ujemnych wyników3738
- Nie wszyscy pacjenci z IIH prezentują klasyczny obraz kliniczny34
- IIH bez tarczy zastoinowej (IIHWOP) jest szczególnie trudna do zdiagnozowania1339
- Występują przypadki wtórnego nadciśnienia śródczaszkowego, które mogą imitować IIH240
Znaczenie wczesnej diagnostyki
Wczesna i prawidłowa diagnostyka IIH ma kluczowe znaczenie, ponieważ:141
- Nieleczone IIH może prowadzić do trwałej utraty wzroku1642
- Wczesne wdrożenie leczenia może zapobiec powikłaniom43
- Szybka diagnoza pozwala na efektywniejsze leczenie44
- Możliwe jest uniknięcie zbędnych interwencji, jeśli diagnoza jest postawiona prawidłowo33
Monitorowanie i obserwacja
Po zdiagnozowaniu IIH konieczne jest regularne monitorowanie stanu pacjenta, obejmujące:1445
- Regularne badania okulistyczne z oceną ostrości wzroku i pola widzenia4647
- Okresowe fotografowanie tarczy nerwu wzrokowego i badania OCT do śledzenia zmian w czasie19
- W niektórych przypadkach powtórne badania obrazowe mózgu32
- Ocenę skuteczności stosowanego leczenia4849
Monitorowanie jest szczególnie istotne, ponieważ przebieg IIH jest zmienny – u niektórych pacjentów objawy ustępują samoistnie w ciągu kilku miesięcy, u innych mogą trwać latami lub nawracać, zwłaszcza przy ponownym przyroście masy ciała.4250
Postępowanie interdyscyplinarne
Diagnostyka i leczenie IIH wymaga współpracy specjalistów z różnych dziedzin:1451
- Neurologa – do ogólnej oceny i koordynacji leczenia52
- Okulisty – do oceny narządu wzroku i monitorowania zmian53
- Neurooftalmologa – specjalisty łączącego te dwie dziedziny54
- Neuroradiologa – do interpretacji badań obrazowych55
- Neurochirurga – w przypadku konieczności interwencji chirurgicznej56
Interdyscyplinarne podejście jest niezbędne ze względu na złożoność schorzenia i jego wpływ na różne układy organizmu, szczególnie układ nerwowy i narząd wzroku.5236
| Metoda diagnostyczna | Cel badania | Typowe wyniki w IIH |
|---|---|---|
| Badanie okulistyczne z oceną dna oka | Wykrycie tarczy zastoinowej i ocena stanu nerwu wzrokowego | Obecna tarcza zastoinowa (obrzęk tarczy nerwu wzrokowego) |
| Badanie pola widzenia | Ocena funkcji wzrokowej i wykrycie ubytków | Możliwe ubytki w polu widzenia, zwłaszcza obwodowym |
| MRI mózgu z kontrastem | Wykluczenie innych przyczyn nadciśnienia śródczaszkowego | Prawidłowy obraz mózgu lub charakterystyczne zmiany (puste siodło, spłaszczenie gałek ocznych) |
| MR wenografia (MRV) | Ocena zatok żylnych mózgu | Wykluczenie zakrzepicy, możliwe zwężenie zatok żylnych poprzecznych |
| Nakłucie lędźwiowe | Pomiar ciśnienia płynu mózgowo-rdzeniowego i analiza jego składu | Ciśnienie otwarcia ≥25 cm H₂O, prawidłowy skład płynu |
| OCT | Ilościowa ocena obrzęku tarczy nerwu wzrokowego | Pogrubienie warstwy włókien nerwowych siatkówki |
Diagnostyka rzekomego guza mózgu (idiopatycznego nadciśnienia śródczaszkowego) wymaga kompleksowego podejścia obejmującego szczegółowe badanie okulistyczne, badania neuroobrazowe i nakłucie lędźwiowe. Ze względu na ryzyko trwałej utraty wzroku, wczesna i prawidłowa diagnoza ma kluczowe znaczenie dla skutecznego leczenia i zapobiegania powikłaniom.14 Konieczne jest również interdyscyplinarne podejście i regularne monitorowanie stanu pacjenta po rozpoznaniu schorzenia.5236
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Materiały źródłowe
- #1 Idiopathic Intracranial Hypertension: Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/21968-idiopathic-intracranial-hypertension
Idiopathic intracranial hypertension (IIH) is increased pressure in your skull. It occurs because of cerebrospinal fluid buildup around your brain. The cause is unknown. […] A former name for IIH is pseudotumor cerebri. Pseudotumor cerebri means false brain tumor. Providers used to call it this because the symptoms can be similar to a brain tumor. […] To diagnose IIH, a healthcare provider will take your medical history and perform a physical exam. Theyll learn more about what symptoms you experience and order several diagnostic tests to rule out conditions with similar symptoms. Tests may include: Eye exam with a visual field test to check for blind spots in your vision, Brain CT scan or MRI, Spinal tap (lumbar puncture). […] The goals of IIH treatment are to decrease pressure on your brain and prevent vision loss. Depending on the severity, your healthcare provider may recommend: Taking medications, Undergoing surgery, Weight management. […] Timely treatment at the first sign of vision changes or symptoms can help reduce your risk of complications like permanent vision loss. Treatment is often successful at relieving symptoms. Your healthcare provider can give you the best information on what you can expect in your situation.
- #2 Pseudotumor cerebri (idiopathic intracranial hypertension) – Symptoms & causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/pseudotumor-cerebri/symptoms-causes/syc-20354031
Pseudotumor cerebri (SOO-doe-too-mur SER-uh-bry) occurs when the pressure inside your skull (intracranial pressure) increases for no obvious reason. It’s also called idiopathic intracranial hypertension. […] Symptoms mimic those of a brain tumor. The increased intracranial pressure can cause swelling of the optic nerve and result in vision loss. […] Pseudotumor cerebri can occur in children and adults, but it’s most common in women of childbearing age who are obese. […] The cause of pseudotumor cerebri is unknown. If a cause is determined, the condition is called secondary intracranial hypertension, rather than idiopathic. […] The increased intracranial pressure of pseudotumor cerebri might result from a problem in this absorption process. […] For some people with pseudotumor cerebri, their vision continues to worsen, leading to blindness. […] Associated Procedures include CT scan, lumbar puncture (spinal tap), and MRI.
- #3 Diagnosing and Treating Pseudotumor Cerebri | Neurological Surgeryhttps://neurosurgery.weillcornell.org/condition/pseudotumor-cerebri/diagnosing-and-treating-pseudotumor-cerebri
Pseudotumor cerebri is largely a diagnosis of exclusion if a patient has high intracranial pressure and testing does not reveal any cause, the diagnosis will be idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri. […] If the spinal tap reveals increased intracranial pressure, and tests have not determined any cause for that pressure, the patient will be diagnosed with idiopathic intracranial hypertension, or pseudotumor cerebri. […] Venous sinus stenting is a minimally invasive procedure treatment appropriate for patients who have venous sinus stenosis on MRI or CT and involves the placement of a stent (mesh in the shape of a tube) in one of the narrowed veins in the brain, via small incisions in the groin area.
- #4 Pseudotumor Cerebri – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK536924/
Pseudotumor cerebri (PTC), also known as idiopathic intracranial hypertension (IIH), is a disorder with increased intracranial pressure (ICP) and associated headaches, papilledema, vision changes, or pulsatile tinnitus in the setting of normal imaging and cerebrospinal fluid (CSF) studies. […] This activity reviews the cause and pathophysiology of pseudotumor cerebri and highlights the role of the interprofessional team in its management. […] Timely diagnosis and treatment is a must. […] Diagnosis involves the utilization of the Modified Dandy Criteria. […] The mainstays of medical treatment include diagnostic lumbar puncture, weight loss, carbonic anhydrase inhibitors (acetazolamide), topiramate, diuretics, and steroids. […] For cases refractory to medical treatment, surgery can be an option. […] PTC/idiopathic intracranial hypertension is a disease that can be missed or misdiagnosed, given its overlapping features with many other disease processes.
- #5 Idiopathic intracranial hypertension | Radiology Reference Article | Radiopaedia.orghttps://radiopaedia.org/articles/idiopathic-intracranial-hypertension-1?embed_domain=external.radpair.com%252527%25255b0%25255dfavicon.icofavicon.icofavicon.ico&lang=us
Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a syndrome with signs and symptoms of increased intracranial pressure but where a causative mass or hydrocephalus is not identified. […] The modified Dandy criteria were revised to establish the diagnosis in the Idiopathic Intracranial Hypertension Treatment Trial: presence of signs and symptoms of increased intracranial pressure, absence of localizing findings on neurologic exam except those known to occur from increased intracranial pressure, absence of deformity, displacement, or obstruction of the ventricular system and otherwise normal neurodiagnostic studies, except for evidence of increased CSF pressure; abnormal neuroimaging except for empty sella turcica, optic nerve sheath with filled out CSF spaces, and smooth-walled non-flow-related venous sinus stenosis or collapse should lead to another diagnosis, awake and alert patient, no other cause of increased intracranial pressure present.
- #6 Idiopathic intracranial hypertension – Wikipediahttps://en.wikipedia.org/wiki/Idiopathic_intracranial_hypertension
Lumbar puncture is performed to measure the opening pressure, as well as to obtain cerebrospinal fluid (CSF) to exclude alternative diagnoses. […] The original criteria for IIH were described by Dandy in 1937. […] They were modified by Smith in 1985 to become the „modified Dandy criteria”. […] In a 2002 review, Friedman and Jacobson propose an alternative set of criteria, derived from Smith’s.
- #7 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/1214410-overview
It is essential to perform urgent neuroimaging studies on any patient presenting with bilateral optic nerve edema in order to rule out an intracranial mass. Once a mass lesion is ruled out, a lumbar puncture (LP) is performed to confirm an elevated opening pressure and to evaluate the cerebrospinal fluid (CSF) contents (description of the fluid, analysis of the protein, glucose, blood cell type/count, culture). An LP should never be performed prior to neuroimaging. […] The Dandy criteria (described by Dandy in 1937 and later modified) were the original criteria used to diagnose IIH and are as follows: Symptoms and signs of increased ICP. No other localizing neurologic signs other than those related to increased ICP (eg, unilateral or bilateral sixth nerve paresis, papilledema, or papilledema-related visual loss). Cerebrospinal fluid (CSF) may show increased pressure, but there are no cytologic or chemical abnormalities. Neuroimaging reveals radiographic signs of increased ICP but no structural cause or hydrocephalus. No other causes of increased ICP are found through workup.
- #8 A High-Pressure Situation: Idiopathic Intracranial Hypertension Diagnosis and Treatments – Ophthalmology Advisorhttps://www.ophthalmologyadvisor.com/features/pseudotumor-cerebri-iih-diagnosis-protocol-and-treatment-options/
Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disorder of increased intracranial pressure (ICP), defined as more than 250 mm H2O opening pressure, in the presence of normal neuroimaging and normal cerebral spinal fluid (CSF) content. […] The Modified Dandy Criteria used to diagnose IIH was reviewed and updated in 2013 to account for neuroimaging technologies, the potential for IIH without papilledema, and further delineate primary IIH from secondary causes. […] The Modified Dandy Criteria identifies 5 diagnostic criteria: 1. Papilledema (Or, if no papilledema is present, there must be a CN6 palsy [unilateral or bilateral], or any combination of 3 from the following 4 neuroimaging criteria: empty sella, flattening of the posterior aspect of the globe, distention of the perioptic subarachnoid space with or without a tortuous optic nerve, transverse venous sinus stenosis). 2. Normal neurologic examination except for cranial nerve abnormalities. 3. Neuroimaging: normal brain parenchyma with no evidence of hydrocephalus, mass, or structural lesion, and no abnormal meningeal enhancement on magnetic resonance imaging (MRI) and magnetic resonance venography (MRV). 4. Normal CSF composition 5. Elevated lumbar puncture opening pressure higher than 25 cm H2O CSF in adults or higher than 28 cm H2O CSF in children.
- #9 Idiopathic Intracranial Hypertension (IIH) Workup: Approach Considerations, Laboratory Studies, MRI and CT Scanninghttps://emedicine.medscape.com/article/1214410-workup
If idiopathic intracranial hypertension (IIH) is suspected based on clinical findings, it is important for clinicians to evaluate the visual fields and optic fundi, even if the patient does not report visual symptoms. […] The diagnosis of IIH is primarily clinical and confirmed through brain imaging, ideally using MRI with magnetic resonance venography, which typically shows normal results except for possible narrowing of the venous transverse sinus. […] If it is not contraindicated, a lumbar puncture should be performed to analyze cerebrospinal fluid (CSF). An elevated opening pressure with normal CSF composition supports the diagnosis of idiopathic intracranial hypertension. […] Additionally, the clinical presentation of IIH can be mimicked by certain medications and disorders, which should be ruled out to confirm the diagnosis.
- #10 Idiopathic Intracranial Hypertensionhttps://practicalneurology.com/articles/2020-may/idiopathic-intracranial-hypertension
Treatment of idiopathic intracranial hypertension should be started as soon as diagnosis is confirmed to prevent vision loss. […] The diagnosis of IIH is made using the Friedman criteria, whereas the International Classification of Headache Disorders (ICHD-3) outlines the diagnostic criteria of the associated headache. […] If papilledema is present, the next diagnostic step is to obtain an MRI with and without contrast and venography to exclude the presence of a structural abnormality or cerebral venous sinus thrombosis (CVST). […] If a secondary cause of papilledema is not identified, a lumbar puncture must be performed. Opening pressure of 25 cm or more cerebrospinal fluid (CSF) and otherwise normal CSF confirms the diagnosis of IIH. […] In IIHWOP, the diagnosis can be made if all the above criteria are met except papilledema and cranial nerve 6 palsy is present.
- #11 Idiopathic intracranial hypertension | Radiology Reference Article | Radiopaedia.orghttps://radiopaedia.org/articles/idiopathic-intracranial-hypertension-1?embed_domain=external.radpair.com%252527%25255b0%25255dfavicon.icofavicon.icofavicon.ico&lang=us
A competing set of diagnostic criteria were proposed in 2013 and are also commonly used. The criteria place patients into one of four diagnostic subgroups: definite idiopathic intracranial hypertension: opening pressure 25 cm CSF (H2O) and papilledema, probable idiopathic intracranial hypertension: opening pressure 25 cm CSF and papilledema, definite idiopathic intracranial hypertension without papilledema: opening pressure 25 cm CSF and abducens nerve palsy, suggested idiopathic intracranial hypertension without papilledema: opening pressure 25 cm CSF and 3 out of 4 neuroimaging signs (empty sella, flattening of the posterior aspect of the globe, distension of the perioptic subarachnoid space, or transverse venous sinus stenosis). […] Imaging of the brain with CT or MRI without and with contrast, and possibly CT or MR venography, is essential in patients with suspected idiopathic intracranial hypertension to exclude elevated CSF pressure due to other causes such as brain tumor, dural sinus thrombosis, hydrocephalus, etc. […] Although bony changes are permanent, the rest may be reversible with treatment.
- #12 Diagnostic Criteria For Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) — Ophthalmology Reviewhttps://www.ophthalmologyreview.org/articles/diagnostic-criteria-ptc
[…] In 2013, Friedman, Liu, and Digre revisited the diagnostic criteria to include new information about neuroimaging, reports of pseudotumor cerebri without papilledema, and many other clinical similar conditions that may be lumped into the new PTC syndrome umbrella. […] […] The authors noted that those with true primary pseudotumor cerebri syndrome (i.e., IIH) tended to be older than 3 years old and younger than 60 years old. So although the patient’s age is not listed as a strict requirement for diagnosis, alternate causes for optic nerve swelling or increased intracranial pressure should be considered in those extreme outliers. […] […] If papilledema is not present, then there must either be a CN6 palsy (unilateral or bilateral) or 3 neuroimaging criteria satisfied: Empty sella, Flattening of the posterior aspect of the globe, Distention of the perioptic subarachnoid space with or without a tortuous optic nerve, Transverse venous sinus stenosis.
- #13 The diagnosis and management of idiopathic intracranial hypertension and the associated headachehttps://pmc.ncbi.nlm.nih.gov/articles/PMC4916517/
The current diagnostic criteria for headache attributed to IIH given by the International Classification of Headache Disorders (ICHD-III beta) […] Recently, these criteria have been field tested and new validated criteria have been proposed. […] Characteristic for the condition is the presence of a pulsatile tinnitus that is believed to arise from intensified vascular pulsation occurring with high ICP. […] Increased ICP without papilledema has been reported in unresponsive chronic migraine patients suggesting a diagnosis of IIH without papilledema. […] However, such a diagnosis is challenging and requires caution with additional clinical or neuroradiological confirmation as suggested in the revised diagnostic criteria for pseudotumor cerebri syndrome. […] The first step in the neurological examination of a suspected IIH patient is fundoscopy.
- #14 Pseudotumor cerebri (idiopathic intracranial hypertension) – Diagnosis & treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/pseudotumor-cerebri/diagnosis-treatment/drc-20354036
To diagnose your condition, your doctor will review your symptoms and medical history, conduct a physical examination, and order tests. […] If pseudotumor cerebri is suspected, a doctor trained in eye conditions (ophthalmologist) will look for a distinctive type of swelling affecting the optic nerve in the back of your eye. […] Your doctor is likely to order an Magnetic resonance imaging (MRI) or computed tomography (CT) scan. These tests can rule out other problems that can cause similar symptoms, such as brain tumors and blood clots. […] Your doctor might order a lumbar puncture to measure the pressure inside your skull and analyze your spinal fluid. […] Once you’ve had pseudotumor cerebri, you’ll need to have your vision checked regularly to monitor changes. […] After discussing your symptoms with your family doctor, he or she might refer you to a doctor trained in brain and nervous system conditions (neurologist) or eye conditions (ophthalmologist) or both (neuro-ophthalmologist) for further evaluation. […] For pseudotumor cerebri, questions to ask your doctor include: What tests do I need? […] Your doctor is likely to ask you questions, including: What, if anything, seems to improve your symptoms?
- #15 Pseudotumor Cerebri and Papilledema – Glaucoma Todayhttps://glaucomatoday.com/articles/2019-jan-feb/pseudotumor-cerebri-and-papilledema
Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), describes the perplexing syndrome of increased intracranial pressure (ICP) in the absence of a space-occupying lesion on neuroimaging or other etiology. […] Papilledema is the hallmark sign of idiopathic intracranial hypertension and occurs due to raised intracranial pressure transmitted to the optic nerve sheath. […] Patients with papilledema should be evaluated for an intracranial cause. […] When elevation of ICP is suspected, MRI of the brain with gadolinium and magnetic resonance venography should be promptly conducted to exclude secondary causes of ICP. If no structural abnormality is identified, a lumbar puncture should be performed, documenting the opening pressure, and a CSF sample should be forwarded for analysis and culture. IIH is diagnosed according to the Idiopathic Intracranial Hypertension Treatment Trial modified Dandy criteria. […] Negative neuroimaging (eg, cranial, contrast MRI with magnetic resonance venography); normal CSF content; and an elevated ICP on opening pressure on lumbar puncture establish the diagnosis of IIH.
- #16 Pseudotumor Cerebri Patient Guide – Brigham and Women’s Hospitalhttps://www.brighamandwomens.org/neurology/neuro-ophthalmology/pseudotumor-cerebri
Pseudotumor can cause permanent visual loss. Typically, patients first develop loss of their peripheral vision. If Pseudotumor Cerebri is not treated, it can cause gradually progressing visual loss that affects central (straight-ahead) vision. […] The doctor will diagnose Pseudotumor Cerebri based on symptoms and the results of the examination and diagnostic tests. Patients with Pseudotumor Cerebri have swelling of the optic nerve that is visible to the physician during examination with the ophthalmoscope. […] In order to diagnose a patient with Pseudotumor Cerebri, the doctor will have to order additional tests. The first test is an MRI scan of the brain. An MRI uses a large magnet to take a detailed picture of the brain (it does not use X-ray radiation). The MRI will check if there is an abnormal growth in the brain or its coverings that is the reason for elevated pressure in the skull. It will also check for blood clots in the major veins in the brain.
- #17 Idiopathic intracranial hypertension – WikEMhttps://wikem.org/wiki/Idiopathic_intracranial_hypertension
Also known as pseudotumor cerebri/benign intracranial hypertension (BIH) […] Idiopathic intracranial hypertension (Pseudotumor Cerebri) […] Typically, MRI and MR venogram of the orbit and brain to rule out secondary causes of intracranial pressure such as cerebral venous sinus thrombosis. If normal, the patient may need LP to determine opening pressure and r/o other causes of optic nerve edema. […] CSF lab studies by lumbar puncture are negative […] Outpatient visual field testing is the most important method for following these patients (Humphrey VF’s) […] Treatment may be indicated in the following situations: severe/intractable headache, evidence of progressive decrease in visual acuity or visual field loss. Some ophthalmologists suggest treating all patients with papilledema. […] CSF Shunt (ventriculoperitoneal or lumboperitoneal) is often effective if vision is threatened.
- #18 Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) – EyeWikihttps://eyewiki.org/Pseudotumor_Cerebri_(Idiopathic_Intracranial_Hypertension)
Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), is a disorder characterized by increased intracranial pressure (ICP) of unknown cause that predominantly affects obese women of childbearing age. Papilledema is the primary ocular finding and may progressively lead to optic atrophy and blindness if no treatment is provided. […] IIH is a diagnosis of exclusion. Nonetheless, diagnostic criteria were established by Dandy in 1937. However, over the years some modifications were provided and the new criteria are now known as the modified Dandy criteria: […] When evaluating a patient for IIH, a complete ocular examination including a dilated fundus examination, visual field examination, and optic nerve photographs is required. Subsequently, neuroimaging is needed to exclude secondary causes of intracranial hypertension. Magnetic resonance imaging (MRI) and MR venography (MRV) of the brain are usually the imaging modalities of choice.
- #19 How is a Patient Diagnosed? | Harvard Medical School Department of Ophthalmologyhttps://eye.hms.harvard.edu/book/how-patient-diagnosed-and-treated
The presence of three of four MRI features of intracranial hypertension is highly specific for IIH: Empty sella, Optic nerve sheath distension, Posterior globe flattening, Transverse venous sinus stenosis. […] MRI of the brain with and without contrast and MRV of the head should be obtained urgently to exclude an intracranial mass lesion, hydrocephalus, or cerebral venous sinus thrombosis. […] LP should be performed after neuroimaging in order to confirm normal spinal fluid constituents (cell counts, protein, and glucose) and elevated opening pressure. […] In patients with severe papilledema, frequent visual acuity and visual field testing (usually 24-2 or 30-2 standard automated perimetry protocols) are important to assess for response to medical therapy and need for surgical intervention. […] Ocular imaging modalities, including photography and optical coherence tomography (OCT), are invaluable for documenting the degree of papilledema and monitoring response to therapy. […] Orbital B-scan ultrasound helps distinguish papilledema from pseudo-papilledema.
- #20 Idiopathic Intracranial Hypertension (IIH) Workup: Approach Considerations, Laboratory Studies, MRI and CT Scanninghttps://emedicine.medscape.com/article/1214410-workup
Once an intracranial mass lesion has been excluded, a lumbar puncture is recommended. […] It is crucial to measure the opening pressure with the patient in a relaxed decubitus position to avoid inaccurately high readings. […] An opening pressure above 25 cm H2O is considered elevated, yet patients presenting with typical IIH symptoms but normal pressures may still be classified as having „probable” IIH. […] The appearance, clarity, and color of the CSF should be recorded, and samples should be analyzed for cell count, cytology, culture, and levels of glucose, protein, and electrolytes. Typically, these parameters are normal in IIH patients. […] If clinical indicators point to idiopathic intracranial hypertension, it is essential to assess the visual fields and optic fundi, regardless of whether patients present with visual symptoms or not. […] Optical coherence tomography (OCT) also can be a helpful tool to evaluate and monitor optic nerve head edema, as it can characterize and quantify changes in the retinal nerve fiber layer that may be associated with acute and chronic changes in intracranial pressure.
- #21 Idiopathic intracranial hypertension – Wikipediahttps://en.wikipedia.org/wiki/Idiopathic_intracranial_hypertension
For the diagnosis, brain scans (such as MRI) should be done to rule out other potential causes. […] The diagnosis is based on symptoms and a high opening pressure found during a lumbar puncture with no specific cause found on a brain scan. […] To confirm the diagnosis, as well as excluding alternative causes, several investigations are required; more investigations may be performed if the history is not typical or the person is more likely to have an alternative problem: children, men, the elderly, or women who are not overweight. […] Neuroimaging, usually with computed tomography (CT/CAT) or magnetic resonance imaging (MRI), is used to exclude any mass lesions. […] An MR venogram is also performed in most cases to exclude the possibility of venous sinus stenosis/obstruction or cerebral venous sinus thrombosis.
- #22 The diagnosis and management of idiopathic intracranial hypertension and the associated headachehttps://pmc.ncbi.nlm.nih.gov/articles/PMC4916517/
The next step is neuroimaging, preferably a magnetic resonance imaging (MRI) scan of the brain. […] In the clinical setting IIH is documented by a lumbar puncture with manometry. […] Based on the existing evidence and clinical experience the following diagnostic plan has now been developed in our academic center and can be recommended for systematic testing in other settings. […] The target of IIH management is to reduce ICP with the main goals of preservation of vision and relief of headache. […] The first drug of choice in IIH management is acetazolamide, an old diuretic with a significant carbonic anhydrase inhibitor effect. […] The effect of topiramate and acetazolamide was compared in an open-label study of 40 IIH patients, with a main focus on visual fields. […] Although headache is the most frequent symptom in patients with IIH, headache management is a field yet to be explored, since the existing treatment trials have been focused on ICP changes or visual outcome rather than a headache relief.
- #23 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/1214410-overview
Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology characterized by chronically elevated intracranial pressure (ICP), and the most important neurologic manifestation is papilledema. The presentation of acute/subacute symptoms of increased ICP and papilledema should be considered a clinical emergency until a neuroimaging study confirms the absence of an intracranial mass. If left untreated, chronic papilledema may lead to secondary progressive optic atrophy, visual field loss, and ultimately blindness. […] The preferred neuroimaging procedure is a combination of a magnetic resonance image (MRI) of the brain and magnetic resonance venography (MRV) to rule out both an intracranial mass lesion and a dural sinus thrombosis or stenosis. If MRI is not available on the initial presentation, at the very least a computed axial tomography (CAT scan) of the brain can be performed.
- #24 A practical approach to, diagnosis, assessment and management of idiopathic intracranial hypertension | Practical Neurologyhttps://pn.bmj.com/content/14/6/380
Adult patients who present with papilloedema and symptoms of raised intracranial pressure need urgent multidisciplinary assessment including neuroimaging, to exclude life-threatening causes. […] Where there is no apparent underlying cause for the raised intracranial pressure, patients are considered to have idiopathic intracranial hypertension (IIH). […] The diagnostic criteria of IIH are well known and have evolved since Dandy’s initial description in 1937; they include a CSF opening pressure of 25cmH2O. […] However, these criteria recommend imaging only to exclude a venous sinus thrombosis in patients without the typical IIH phenotype (obesity and female sex). […] We feel, however, that it is essential to exclude venous sinus thrombosis (using MRI or CT with venography) in all patients presenting with pseudotumour cerebri, since being female and obese does not preclude the diagnosis of venous sinus thrombosis.
- #25 IIH (PSEUDOTUMOR CEREBRI) SYMPTOMS, CAUSES, AND TREATMENTS.https://virtualheadachespecialist.com/idiopathic-intracranial-hypertension-iih-pseudotumor-cerebri-causes-symptoms-and-treatments/
IIH, idiopathic intracranial hypertension, pseudotumor cerebri […] IIH is a completely different problem than the elevated CSF pressure from a brain tumor though, and treated much differently. […] According to criteria, IIH consists of a headache associated with CSF pressure greater than 250 mm CSF (25 cm CSF) in adults, and greater than 280 mm CSF (28 cm CSF) in children, checked by lumbar puncture (LP). […] If CSF pressure is high and the patient has signs, symptoms, and/or findings on MRI, then the diagnosis is confirmed. […] A brain MRI with contrast should be a first line test, which helps to exclude other obvious causes of papilledema and elevated CSF pressure (such as brain tumor), and results can support a diagnosis of IIH. […] Brain MRV should always be done with the MRI to ensure there is no blood clot (cerebral venous thrombosis) in the venous drainage pathways, or significant narrowing (stenosis) of the venous sinuses.
- #26 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/1214410-overview
A subsequent article further refined the diagnostic criteria by adding the following two criteria: The diagnostic lumbar puncture should be performed with the patient in the lateral decubitus position. MRI and MRV should be considered the ideal imaging studies to rule out intracranial venous sinus thrombosis. However, some patients cannot undergo MRI (eg, ferromagnetic foreign body, non-MRI compatible aneurysm clip or cardiac pacemaker), so CT scanning and CT venography (CTV) may be necessary as alternative neuroimaging studies.
- #27 EM@3AM: Idiopathic Intracranial Hypertension – emDocshttps://www.emdocs.net/em3am-idiopathic-intracranial-hypertension/
A 31-year-old female presents with headache. […] Initial VS include T 36.0, HR 75, BP 120/65, RR 18, SpO2 97% RA. […] Fundoscopic exam reveals bilateral papilledema. […] Idiopathic Intracranial Hypertension (IIH) is a disorder characterized by signs of increased intracranial pressure (headaches, vision loss, and papilledema) with no other cause detected on neuroimaging or other evaluations. […] Evaluation: History and exam are often benign, but it is vital to consider the disease. […] Obtain: Labs â CBC, BMP, LFTs, HCG for females. […] Neuroimaging â MRI with MRV preferred imaging, but suitable to obtain CT in patients not able to tolerate MRI. […] Official Diagnosis (modified Dandy criteria): symptoms of increased ICP, no localizing signs with exception of 6th nerve palsy, patient is awake/alert, normal central imaging findings with no evidence of thrombosis, LP opening pressure > 25 cm but otherwise normal CSF, no other explanation for increased ICP. […] The diagnosis of IIH is made by a lumbar puncture with an increased opening pressure. […] Making the diagnosis is critical as it affects management and appropriate management can avoid the most common complication which is vision loss.
- #28 Pseudotumor Cerebri Patient Guide – Brigham and Women’s Hospitalhttps://www.brighamandwomens.org/neurology/neuro-ophthalmology/pseudotumor-cerebri
The second test that is required is a lumbar puncture (also known as a spinal tap.) This test is performed by inserting a small needle into the back to measure the pressure of the cerebrospinal fluid. A small sample of the fluid is collected to check if there is any infection or inflammation that has caused elevated pressure. In patients with Pseudotumor Cerebri, the pressure is elevated but the cerebrospinal fluid itself is normal.
- #29https://step2.medbullets.com/neurology/120306/idiopathic-intracranial-hypertension-pseudotumor-cerebri
The indication for lumbar puncture is performed after secondary causes of increased intracranial pressure have been excluded on neuroimaging. […] Elevated opening pressure is a finding in lumbar puncture. […] Ophthalmic examination is indicated to determine the extent of optic nerve damage from the increased intracranial pressure.
- #30 Idiopathic Intracranial Hypertension, Pseudotumor cerebri – EyeRounds.org – Ophthalmology – The University of Iowahttp://eyerounds.org/cases/99-pseudotumor-cerebri.htm
Idiopathic Intracranial Hypertension (IIH) is a condition in which the cerebrospinal fluid (CSF) is under high pressure in the absence of an intracranial mass, venous sinus thrombosis or other primary cause (see differential diagnosis). It is also known as pseudotumor cerebri because patients present with signs and symptoms of a brain tumor without a brain tumor being present. […] First, imaging studies of the patient’s head (via magnetic resonance imaging or computed tomography) must be normal except for the signs of raised intracranial pressure (empty sella, flattened globes, collapsed lateral sinus). Next, the cerebrospinal fluid pressure is measured by a lumbar puncture and the pressure must be elevated. The lumbar puncture should be performed with the patient in a supine position for most accurate measurement of the intracranial pressure. Laboratory studies on the cerebrospinal fluid must be normal. All of the above criteria (the Modified Dandy Criteria, see table) must be met in order to diagnose IIH. […] Diagnosis: Idiopathic Intracranial Hypertension (Pseudotumor cerebri)
- #31 Idiopathic Intracranial Hypertension – Neurologic Disorders – Merck Manual Professional Editionhttps://www.merckmanuals.com/professional/neurologic-disorders/headache/idiopathic-intracranial-hypertension
Idiopathic intracranial hypertension causes increased intracranial pressure without a mass lesion or hydrocephalus, probably by obstructing venous drainage; cerebrospinal fluid (CSF) composition is normal. […] Diagnosis of idiopathic intracranial hypertension is suspected clinically and established by brain imaging (preferably MRI with magnetic resonance venography) that has normal results (except for narrowing of the venous transverse sinus). If not contraindicated, lumbar puncture with cerebrospinal fluid (CSF) testing is then done. Elevated opening pressure and normal CSF composition suggests idiopathic intracranial hypertension. […] Consider idiopathic intracranial hypertension if patients, particularly women with excess body weight, have a daily generalized headache with or without visual symptoms; check visual fields and optic fundi. […] Diagnose based on results of brain imaging (preferably MRI with venography) and, if not contraindicated, lumbar puncture; consider chronic meningitis.
- #32 Pseudotumor cerebri Information | Mount Sinai – New Yorkhttps://www.mountsinai.org/health-library/diseases-conditions/pseudotumor-cerebri
Pseudotumor cerebri syndrome; Benign intracranial hypertension […] Idiopathic intracranial hypertension (IIH) is a condition in which the pressure inside the skull is increased. The brain is affected in a way that the condition appears to be, but is not, a tumor. […] The diagnosis is made when other health conditions are ruled out. These include conditions that may cause increased pressure in the skull, such as: Hydrocephalus, Tumor, Venous sinus thrombosis, Infection. […] Treatment is aimed at the cause of IIH. The main goal of treatments is to preserve vision and reduce the severity of headaches. […] People will need to have their vision closely monitored. There can be vision loss, which is sometimes permanent. Follow-up MRI or CT scans may be done to rule out problems such as tumors or hydrocephalus (buildup of fluid inside the skull). […] The condition sometimes disappears on its own within 6 months. Symptoms can return in some people. A small number of people have symptoms that slowly get worse and lead to blindness. […] Vision loss is a serious complication of this condition.
- #33 A practical approach to, diagnosis, assessment and management of idiopathic intracranial hypertension | Practical Neurologyhttps://pn.bmj.com/content/14/6/380
The diagnosis can be difficult and the consequences of error can lead either to the neglect of a serious treatable cause of raised intracranial pressure, blindness or inappropriate treatment of patients who do not have IIH. […] Although there is insufficient literature to generate an evidence-based management strategy for IIH, experienced clinicians can manage it well. […] Pseudotumour cerebri describes raised intracranial pressure and papilloedema in the absence of a space-occupying lesion and can be caused by several conditions. […] Idiopathic intracranial hypertension is a diagnosis of exclusion where NO cause can be found. […] A single lumbar puncture opening pressure reading can be misleading. […] Monitoring of visual fields can be problematic in many patients who have functional overlay. […] Treat the patient. High-risk patients lose vision rapidly.
- #34 Diagnostic Criteria For Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) — Ophthalmology Reviewhttps://www.ophthalmologyreview.org/articles/diagnostic-criteria-ptc
Pseudotumor cerebri syndrome (PTC, also referred to as idiopathic intracranial hypertension [IIH]) is classically taught as presenting in young, overweight women of childbearing age, with a history of headaches and findings of bilateral optic nerve swelling, associated with an elevated intracranial pressure. […] However, as with every „textbook” definition of a disease, there are atypical cases (children, men, thin people, older people), and so I am often confronted with some interesting diagnostic challenges when I am referred a patient that does not fit the typical picture of PTC who has bilateral optic nerve swelling. […] […] There are a lot of different aspects to cover with PTC, which I plan to eventually discuss (similar to my plans to cover the entire breadth of ophthalmology, even if it takes me the next 20 years). So stay tuned for more articles covering PTC in the future!
- #35 Idiopathic intracranial hypertension: consensus guidelines on management | Journal of Neurology, Neurosurgery & Psychiatryhttps://jnnp.bmj.com/content/89/10/1088
The aim was to capture interdisciplinary expertise from a large group of clinicians, reflecting practice from across the UK and further, to inform subsequent development of a national consensus guidance for optimal management of idiopathic intracranial hypertension (IIH). […] Over 20 questions were constructed: one based on the diagnostic principles for optimal investigation of papilloedema and 21 for the management of IIH. […] For optimal investigation of patients with papilloedema, there must be clear communication between clinicians for seamless joint investigation between the various specialities. […] The diagnostic criteria mandate a cut-off opening pressure of 25cm CSF for diagnosing IIH. […] The LP opening pressure should not be interpreted in isolation when diagnosing IIH. […] Clinical uncertainty exists, and IIH can be misdiagnosed.
- #36 How Is Pseudotumor Cerebri Syndrome Diagnosed and Treated?https://www.brainandlife.org/articles/pseudotumor-cerebri-syndrome
Because headaches are common in young women and the headaches of pseudotumor cerebri syndrome may resemble migraines the diagnosis may be missed initially if a careful history is not taken and the eyes are not carefully examined for swelling of the optic nerves. […] The diagnosis is less straightforward when the person’s headache has been present for over a year and/or swelling of the optic nerves is not present. […] Treatments include medications, surgery, and addressing any secondary causes such as high sodium, obesity, or medications known to produce high spinal fluid pressure. […] The main goal of treatment is to prevent or reverse visual loss. […] Once diagnosed, it is important to have regular follow-up visits with a neurologist and an ophthalmologist or a neuro-ophthalmologist.
- #37https://journals.lww.com/neur/fulltext/2021/69002/idiopathic_intracranial_hypertension___challenges.28.aspx
Idiopathic intracranial hypertension (IIH) is defined as a syndrome of raised intracranial pressure with normal imaging of the brain and cerebrospinal fluid (CSF) composition. […] The modified Dandy criteria, updated by Friedman in 2002 and 2013, are advocated for the diagnosis of IIH. […] Lumbar puncture and CSF pressure are the gold standards for IIH diagnosis, but pressures can fluctuate and false negatives are common. […] MRI brain is a sensitive tool for identifying IIH. […] The objectives in the investigation of IIH are to establish the existence of raised ICP, evaluate severity, exclude other causes of raised ICP, and identify etiological factors responsible for the IIH. […] The opening pressure does not always give the true steady-state pressure due to natural fluctuations. […] In patients with no evidence of venous sinus thrombosis, signs of elevated CSF pressure on MRI can help in the diagnosis of IIH.
- #38 Idiopathic Intracranial Hypertension: pseudotumor cerebrihttps://webeye.ophth.uiowa.edu/eyeforum/article/iih/pc-medical-tx.htm
Weight loss is the cornerstone of therapy for idiopathic intracranial hypertension. […] In our experience, acetazolamide appears to be an effective treatment for idiopathic intracranial hypertension. […] The trial has two aims. One is to determine if acetazolamide (Diamox) with a low sodium weight reduction diet is superior to placebo with the diet. […] The mechanism of action of acetazolamide is likely multifactorial. […] Acetazolamide appeared to be an effective medication in their patients with results occurring over several months. […] It has been documented that furosemide (Lasix) can lower intracranial pressure. […] Furosemide has also been used to treat IIH. […] Acetazolamide and furosemide in combination was significantly more effective in achieving normal ICP than antituberculous drugs alone.
- #39 Idiopathic Intracranial Hypertensionhttps://practicalneurology.com/articles/2020-may/idiopathic-intracranial-hypertension
The ICHD-3 criteria for IIH-related headache also require elevation in CSF pressure. […] The headache can be accompanied by pulsatile tinnitus and papilledema but these are not required for the diagnosis, nor is a response to lumbar puncture. […] Surgical intervention should be considered for individuals who are at immediate risk of vision loss and have medically refractory disease. […] There are 3 main surgical options for IIH treatment. These include CSF-diverting procedures: LP or ventriculoperitoneal (VP) shunting, optic nerve sheath fenestration (ONSF), and venous sinus stenting. […] Despite improving visual outcomes by reducing ICP, many people with IIH continue to have disabling headache even after ICP returns to normal. […] Ultimately, treatment of headache in IIH is based on the headache phenotype, which is typically migrainous, and data on specific therapies for headache in IIH are limited.
- #40 Idiopathic intracranial hypertension doc – Bobby Jones CSFhttps://bobbyjonescsf.org/physician-information/idiopathic-intracranial-hypertension/
Among the most telling findings is swelling of the optic nerve at the back of the eye, the only part of the brain that can be seen on physical exam. Swelling of the optic nerve head, called papilledema, represents the swelling that is occurring in the brain. […] The most commonly used medication for the initial treatment of IIH is acetazolamide. […] The first step in treatment of IIH is determining its possible from the many listed above. […] If the pressure is 25 mmH2O or greater, and a Chiari I malformation is not present, a spinal fluid shunt can be considered. […] In the Chiari Pseudotumor Syndrome, both a Chiari I malformation and IIH are present in the same patient. […] Recognition of IIH, an increasingly frequent condition, deserves increased attention at all levels of patient care.
- #41 Fulminant Idiopathic Intracranial Hypertension – EyeWikihttps://eyewiki.org/Fulminant_Idiopathic_Intracranial_Hypertension
A complete ocular examination including a dilated fundus examination, visual field examination, and optic nerve photographs should be performed. […] Neuroimaging, specifically magnetic resonance imaging (MRI) of the brain and orbits with and without contrast as well as magnetic resonance venography (MRV) of the brain, are the recommended imaging modalities. […] The management of FIH differs from typical IIH because of the increased risk of permanent vision loss. […] Rapid diagnosis and management of FIH is necessary to prevent permanent vision loss. […] Prompt treatment is required to prevent permanent vision loss and includes temporizing medical measures until surgical intervention can be performed.
- #42 Intracranial Hypertension (Pseudotumor Cerebri): Diagnosis & Treatment | Nationwide Children’s Hospitalhttps://www.nationwidechildrens.org/conditions/intracranial-hypertension-pseudotumor-cerebri
Idiopathic intracranial hypertension, sometimes called pseudotumor cerebri, is a condition in which the cerebro-spinal fluid is not able to drain normally. […] The term pseudotumor is sometimes used because the extra fluid mimics the effects of a brain tumor by pushing against the brain. […] Common symptoms of intracranial hypertension are headache and vision problems. […] The exact cause is not known. However, we do know if the body makes more cerebrospinal fluid than the amount absorbed, it causes the pressure inside the skull to increase. […] For symptoms of intracranial hypertension, an ophthalmologist (eye doctor) usually looks for swelling of the optic nerve in the back of the eye, and for signs of vision loss. […] Intracranial hypertension is diagnosed if the symptoms listed above are present and there is elevated pressure inside the skull. […] The increased pressure causes headaches and injury to the optic nerves. […] If treated, the outcome is good. If not treated, permanent blindness can occur. […] With treatment, in most cases, this condition goes away. However, increased pressure can return months or even years later.
- #43 Content – Health Encyclopedia – University of Rochester Medical Centerhttps://www.urmc.rochester.edu/encyclopedia/content.aspx?contenttypeid=134&contentid=57
Idiopathic intracranial hypertension (IIH) is a disorder related to high pressure in the brain. It causes signs and symptoms of a brain tumor. It’s also sometimes called pseudotumor cerebri or benign intracranial hypertension. […] The symptoms of IIH mimic those of a true brain tumor. The main sign is unusually high pressure inside the skull. This is known as intracranial hypertension. […] A physical exam and a few tests can help identify IIH. Diagnosis involves ruling out other health problems. These include a brain tumor. You may need these tests: Brain imaging, such as MRI or CT scans; Spinal tap (lumbar puncture) to withdraw a sample of fluid from around the spine for testing pressure; Exam to test vision and check the back of your eye. […] Seeing a healthcare provider right away to diagnose symptoms and begin treatment can help to prevent complications.
- #44 Idiopathic Intracranial Hypertensionhttps://practicalneurology.com/articles/2020-may/idiopathic-intracranial-hypertension
Treatment of idiopathic intracranial hypertension should be started as soon as diagnosis is confirmed to prevent vision loss. […] The diagnosis of IIH is made using the Friedman criteria, whereas the International Classification of Headache Disorders (ICHD-3) outlines the diagnostic criteria of the associated headache. […] If papilledema is present, the next diagnostic step is to obtain an MRI with and without contrast and venography to exclude the presence of a structural abnormality or cerebral venous sinus thrombosis (CVST). […] If a secondary cause of papilledema is not identified, a lumbar puncture must be performed. Opening pressure of 25 cm or more cerebrospinal fluid (CSF) and otherwise normal CSF confirms the diagnosis of IIH. […] In IIHWOP, the diagnosis can be made if all the above criteria are met except papilledema and cranial nerve 6 palsy is present.
- #45 Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) – EyeWikihttps://eyewiki.org/Pseudotumor_Cerebri_(Idiopathic_Intracranial_Hypertension)
The goal of treatment is to alleviate symptoms of ICP and preserve vision. Although diagnostic lumbar puncture may provide symptom relief the latter is often transient and requires combination with further long-term therapy. […] Medical therapy is usually considered among patients with mild to moderate disease. […] Surgical management should be the option of choice among patients with refractory headaches or more severe/ rapidly progressive visual field loss when all other options have failed to prevent progressive visual loss. […] The course of the disease is variable and may vary from weeks to years. To date there are no prospective studies that have evaluated the natural history of the disease. Following treatment there is usual improvement and/or disease stabilization. Nonetheless, many patients may not fully recover and demonstrate persistent visual field defect, disc edema or elevated opening pressures on lumbar puncture.
- #46 Pseudotumor cerebri (idiopathic intracranial hypertension)https://johnsonmemorial.org/jmh-health/disease-conditions/con-20197162
Pseudotumor cerebri (idiopathic intracranial hypertension) occurs when the pressure inside your skull (intracranial pressure) increases for no obvious reason. […] To diagnose your condition, your doctor will review your symptoms and medical history, conduct a physical examination, and order tests. […] If pseudotumor cerebri is suspected, a doctor trained in eye conditions (ophthalmologist) will look for a distinctive type of swelling affecting the optic nerve in the back of your eye. […] Your doctor is likely to order an MRI or CT scan. These tests can rule out other problems that can cause similar symptoms, such as brain tumors and blood clots. […] Your doctor might order a lumbar puncture to measure the pressure inside your skull and analyze your spinal fluid. […] Once you’ve had pseudotumor cerebri, you’ll need to have your vision checked regularly to monitor changes.
- #47 Intracranial Hypertension or Pseudotumor Cerebri: The Basics – Migraine Canadahttps://migrainecanada.org/intracranial-hypertension-or-pseudotumor-cerebri-the-basics/
Idiopathic Intracranial Hypertension (IIH), also known as pseudotumor cerebri, is a condition where the pressure inside the skull increases without an obvious cause. […] If symptoms suggest IIH, a neuro-ophthalmologist should evaluate the optic nerves and visual fields for swelling (papilledema) and vision loss. An MRI of the brain and a lumbar puncture to measure intracranial pressure are essential for diagnosis. […] Regular eye exams by an ophthalmologist or optometrist are essential. If symptoms worsen, immediate vision testing is necessary to monitor the optic disk and visual fields.
- #48 Idiopathic Intracranial Hypertension: Symptoms, Treatment, Morehttps://www.healthline.com/health/idiopathic-intracranial-hypertension
Idiopathic intracranial hypertension (IIH) is a disorder associated with increased fluid pressure around your brain. This can cause symptoms such as headaches or vision problems. […] The symptoms of IIH overlap with the symptoms of many other conditions, including brain tumors. Many of the diagnostic tools doctors use for IIH help to rule out other disorders. […] If a healthcare professional believes you might have IIH, some possible diagnostic tests include: physical examination and history, magnetic resonance venography (MRV), computed tomography (CT), lumbar puncture, vision tests performed by an ophthalmologist, complete blood count (CBC). […] If, after testing, your doctor cant diagnose you with anything else and your symptoms still fit the criteria, they may diagnose you with IIH. […] After being treated for IIH, you can expect to have regular examinations with an eye doctor as well as a primary care physician to monitor for signs of permanent vision loss or a recurrence of IIH.
- #49 Idiopathic Intracranial Hypertension: Causes, Diagnosis & Treatmenthttps://www.clevelandclinicabudhabi.ae/en/health-hub/health-resource/diseases-and-conditions/idiopathic-intracranial-hypertension
Idiopathic intracranial hypertension (IIH) is a neurological disorder characterized by an increased pressure within the skull, similar to the pressure caused by a brain tumor. […] IHH is also sometimes referred to as pseudotumor cerebri, which means false brain tumor. […] A comprehensive, multi-step diagnostic approach involving the evaluation of symptoms, neuroimaging, lumbar puncture, and ophthalmologic assessments are essential to confirm a diagnosis of IIH and guide appropriate treatment. […] Neuroimaging plays a crucial role in the diagnosis of IIH. Brain imaging may include: […] A lumbar puncture is another key diagnostic procedure, which provides valuable information about CSF pressure and composition. […] Regular assessments are needed to monitor the effectiveness of treatment and to make adjustments as needed. Managing IIH is often a long-term process, and a personalized approach is key to improving symptoms and preventing vision loss.
- #50 Idiopathic Intracranial Hypertension | North American Neuro-Ophthalmology Societyhttps://www.nanosweb.org/i4a/pages/index.cfm?pageid=4129
Diamox (acetazolamide) is the most common medication used for treating IIH. It is thought to lower brain pressure by reducing fluid (cerebrospinal fluid, or CSF) production. […] IIH may go away over months to years or it may be a lifelong medical problem. IIH can return, and is linked to regaining weight.
- #51 Idiopathic intracranial hypertension: consensus guidelines on management | Journal of Neurology, Neurosurgery & Psychiatryhttps://jnnp.bmj.com/content/89/10/1088
An SIG was formed, including neurology, neurosurgery, neuroradiology, ophthalmology, nursing, primary care doctors and patient representatives. […] Following normal imaging, all patients with papilloedema should have a lumbar puncture to check opening pressure and ensure contents are normal. […] The current Cochrane review on IIH management reported on the use of acetazolamide, a carbonic anhydrase inhibitor, in IIH. […] Acetazolamide could be prescribed for those with IIH symptoms. […] The literature pertaining to shunt type is observational and mainly case series based. […] CSF diversion is generally not recommended as a treatment for headache alone in IIH. […] These statements are not mandatory recommendations but are intended to be used as a guide for doctors who investigate and treat IIH.
- #52 The diagnosis and management of idiopathic intracranial hypertension and the associated headachehttps://pmc.ncbi.nlm.nih.gov/articles/PMC4916517/
The visual prognosis appears to have improved in recent years, but IIH is still a complex disorder to handle, multiple medical specialties need to be involved and the relapse rate is high at up to 40%. […] We suggest organization of specific national IIH teams for awareness, management and research.
- #53https://www.aao.org/eye-health/diseases/what-is-idiopathic-intracranial-hypertension
Idiopathic intracranial hypertension or IIH, previously called „pseudotumor cerebri” is when pressure inside your head rises, causing vision problems, headaches and other symptoms. […] Your ophthalmologist will do a series of tests to diagnose idiopathic intracranial hypertension (IIH). They may include: […] An eye exam. Your ophthalmologist will check your optic nerve for swelling. They will also test to see if you have any blank spots in your field of vision. […] An MRI or CT scan. These scans help to check whether your symptoms are due to IIH or caused by other medical problems. […] A spinal tap. This is when your doctor measures the pressure of your spinal fluid. They will also draw fluid to test it for any problems.
- #54 Idiopathic Intracranial Hypertension | North American Neuro-Ophthalmology Societyhttps://www.nanosweb.org/i4a/pages/index.cfm?pageid=4129
Idiopathic intracranial hypertension (IIH), also called pseudotumor cerebri, is a condition in which there is high pressure in the fluid surrounding your brain, spinal cord, and optic nerves. This can cause headaches and problems with vision. […] Patients with pseudotumor cerebri do not have a tumor. A brain tumor may also cause increased intracranial pressure, which is why this condition is called a pseudotumor – it can cause symptoms like a real tumor, but the tests do not show any tumors (pseudo- means false, so pseudotumor means false tumor). […] There are three important steps that must be done in order to diagnose IIH: A complete eye exam must be performed, including special tests on your visual field and dilation of the pupils to look for swelling of the optic nerves caused by high pressure in the brain (papilledema).
- #55 Idiopathic Intracranial Hypertension New York, NY | Spinal Fluid Shunt New York, NYhttps://www.athospatsalidesmd.com/idiopathic-intracranial-hypertension-interventional-neuroradiologist-new-york-ny.html
Today we know that the majority of patients with IIH have severe stenosis (narrowing) of the large veins in the brain. […] This condition can be easily demonstrated with a non-invasive test called MRV (MRI for the Veins). […] Once a patient has venous sinus stenosis, the removal of CSF from the brain is impaired. […] As CSF is constantly produced, impaired removal of CSF leads to excessive CSF in the brain and leads to increased intracranial pressure. […] A new minimally invasive procedure called Venous sinus stenting has been performed by Dr. Patsalides since 2012 has been shown to be effective in the treatment of patients with IIH. […] Clinical trials conducted at Weill Cornell have shown that venous sinus stent is effective in decreasing intracranial pressure and alleviating symptoms of IIH in carefully selected patients with IIH. […] Study shows Venous Sinus Stenting is effective in decreasing intracranial pressure. […] Study shows Venous Sinus Stenting is effective in improving vision loss. […] Study shows Venous Sinus Stenting is effective in improving pulsatile tinnitus.
- #56 Idiopathic Intracranial Hypertensionhttps://practicalneurology.com/articles/2020-may/idiopathic-intracranial-hypertension