Anonymous
Not logged in
Talk
Contributions
Create account
Log in
Search
Editing
Intracranial hypertension
From MEpedia, a crowd-sourced encyclopedia of ME and CFS science and history
Namespaces
Page
Discussion
More
More
Page actions
Read
Edit
Edit source
History
Warning:
You are not logged in. Your IP address will be publicly visible if you make any edits. If you
log in
or
create an account
, your edits will be attributed to your username, along with other benefits.
Anti-spam check. Do
not
fill this in!
{{NeedsImage}} '''Intracranial hypertension''' (IH) is a condition of increased [[cerebrospinal fluid]] pressure inside the skull.<ref name="NORD" /> It results from increases in the volume of the brain, blood or spinal fluid within the fixed volume of the cranium (skull).{{Citation needed|reason= | date = 22 December 2019}} The previous names for intracranial hypertension, pseudotumor cerebri and benign intracranial hypertension, are regarded as inaccurate and downplaying the severity of intercranial hypertension.<ref name="NORD" /> ==Types== *'''Idiopathic Intercranial Hypertension''' or IIH, formerly known as Primary intracranial hypertension *'''Secondary Intercranial Hypertension''': has an identifiable caused for example drugs (such as [[tetracycline]], loral or intrathecal steroids, [[growth hormone]] treatments and others), [[sleep apnea]] and certain systemic diseases such as [[Systemic lupus erythematosus|lupus]], leukemia, kidney failure (uremia), and others.<ref name="NORD" /> ==Signs and symptoms== * [[Headache]] (worse when lying down)<ref name="Soler1998">{{Cite journal | last = Soler | first =D. | last2 = Cox | first2 = T. | last3 = Bullock | first3 = P. | last4 = Calver | first4 =D.M. | last5 = Robinson | first5 = R.O. | date = 1998-01-01 | title = Diagnosis and management of benign intracranial hypertension | url =https://adc.bmj.com/content/78/1/89|journal=Archives of Disease in Childhood|language=en|volume=78|issue=1 | pages = 89–94|doi=10.1136/adc.78.1.89|issn=0003-9888|pmid=9534686}}</ref> * [[Dizziness]] * [[Nausea]] / [[vomiting]] * Altered vision * Pulse synchronous [[tinnitus]] * [[Neck pain|Stiff neck]] * Back and arm pain<ref name="Dankaerts2018" /><ref name="NORD" /> * Pain behind the eyes * [[Photophobia]] * [[Exercise intolerance]]<ref name="NORD">{{Cite news | url=https://rarediseases.org/rare-diseases/idiopathic-intracranial-hypertension/ | title = Idiopathic Intracranial Hypertension|work=NORD (National Organization for Rare Disorders)|access-date=2018-11-12|language=en-US}}</ref> * Memory difficulties * Back pain, radiculopathy (radiating pain)<ref name="Dankaerts2018" /> The most common sign is [[papilledema]] (swelling of the optic nerve sheath). ==Diagnosis== Arachnoiditis, a progressive inflammatory disorder affecting the middle membrane surrounding the spinal cord and brain (arachnoid membrane), epiduritis (involving inflammation of the tough, outer canvas-like covering surrounding the brain and spinal cord) and [[meningitis]] should be ruled out during diagnosis.<ref name="NORD" /> There are many tools that can be used in the diagnosis of intracranial hypertension. The most typical method is a [[lumbar puncture]], during which the opening pressure is measured. Opening pressures of 20 H<sub>2</sub>O or greater are considered abnormal in non-obsese people, 25 H<sub>2</sub>O or greater in obese people. However, as pressure can fluctuate and change with position, a more accurate method is a 24 hour intracranial bolt test. This involves inserting an intracranial pressure monitor directly into the cranium to continuously measure pressure over the course of a day. Normal is 7-15 mm Hg in a supine adult.<ref name="Pickard2004">{{Cite journal | last = Pickard | first = J.D. | last2 = Czosnyka | first2 = M. | date = 2004-06-01 | title = Monitoring and interpretation of intracranial pressure | url = https://jnnp.bmj.com/content/75/6/813|journal=Journal of Neurology, Neurosurgery & Psychiatry|language=en|volume=75|issue=6 | pages = 813–821|doi=10.1136/jnnp.2003.033126|issn=0022-3050|pmid=15145991}}</ref> An MRI can also aid in diagnosis. While generally considered benign, an empty sella can suggest intracranial hypertension, particularly in patients manifesting the symptoms of intracranial hypertension.<ref name="emptysella">{{Cite web | url = https://rarediseases.org/rare-diseases/empty-sella-syndrome/ | title = Empty Sella Syndrome|website=NORD (National Organization for Rare Disorders)|language=en-US|access-date=2019-06-22}}</ref> An empty sella is when the sella, a bony space which holds the [[pituitary gland]], appears “empty” (dark/black) on an MRI. This is because, due to high pressure, the space has been filled with cerebrospinal fluid, flattening the pituitary gland. Patients can also have excess spinal fluid in their optic nerve sheath, which can cause pain behind the eyes and papilledema. Finally, an MR venogram (a type of MRI that uses contrast to visualizes the veins in the brain) can detect bilateral [[transverse venous sinus]] stenosis (TSS), a narrowing of two veins in the back of the head that drain blood from the brain. TSS is found in 83% of cases of intracranial hypertension (compared to 3% of controls).<ref name="Campeau2017">{{Cite journal | last = Campeau | first = N. | last2 = Port | first2 = J. | last3 = Black | first3 =D.F. | last4 = Morris | first4 = P.P. | date = 2017-03-01 | title = Transverse Sinus Stenosis Is the Most Sensitive MR Imaging Correlate of Idiopathic Intracranial Hypertension | url =http://www.ajnr.org/content/38/3/471|journal=American Journal of Neuroradiology|language=en|volume=38|issue=3 | pages = 471–477|doi=10.3174/ajnr.A5055|issn=0195-6108|pmid=28104635}}</ref> It is not known whether TSS is cause or effect, but there is growing evidence that stenting one of the transverse sinus veins can improve or resolve intracranial hypertension.<ref name="Higgins2002">{{Cite journal | last = Higgins | first = J Nicholas P | last2 = Owler | first2 = Brian K | last3 = Cousins | first3 = Claire | last4 = Pickard | first4 = John D | date = 2002-01-19 | title = Venous sinus stenting for refractory benign intracranial hypertension | url =http://www.sciencedirect.com/science/article/pii/S0140673602074408|journal=The Lancet|volume=359|issue=9302 | pages = 228–230|doi=10.1016/S0140-6736(02)07440-8|issn=0140-6736}}</ref><ref name="Pickard2003">{{Cite journal | last = Pickard | first = J.D. | last2 = Sarkies | first2 = N. | last3 = Owler | first3 = B.K. | last4 = Cousins | first4 = C. | last5 = Higgins | first5 = J. N.P. | date = 2003-12-01 | title = Idiopathic intracranial hypertension: 12 cases treated by venous sinus stenting | url =https://jnnp.bmj.com/content/74/12/1662|journal=Journal of Neurology, Neurosurgery & Psychiatry|language=en|volume=74|issue=12|pages=1662–1666|doi=10.1136/jnnp.74.12.1662|issn=0022-3050|pmid=14638886}}</ref><ref name="Halmagyi2011">{{Cite journal | last = Halmagyi | first = G.M. | last2 = Owler | first2 = B.K. | last3 = Hanlon | first3 = M. | last4 = Dunne | first4 = V. | last5 = Allan | first5 = R. | last6 = McCluskey | first6 = P.J. | last7 = Macdonald | first7 = J. | last8 = Thurtell | first8 = M.J. | last9 = Parker | first9 = G.D. | date = 2011-09-01 | title = Transverse Sinus Stenting for Idiopathic Intracranial Hypertension: A Review of 52 Patients and of Model Predictions | url = http://www.ajnr.org/content/32/8/1408|journal=American Journal of Neuroradiology|language=en|volume=32|issue=8|pages=1408–1414|doi=10.3174/ajnr.A2575|issn=0195-6108|pmid=21799038}}</ref> == Risk factors == * Young, overweight, women in their reproductive years (ages 20-45) are most at risk of intercranial hypertension, although any age, gender or weight range can be affected.<ref name="NORD" /> * [[Ehlers-Danlos syndrome]]<ref name="Henderson2017" /> ==Causes== Some causes of intracranial hypertension include: * Venous stenosis * [[craniocervical instability]] * [[Chiari malformation]] * and many more When the cause of increased pressure is unknown it is called idiopathic intracranial hypertension (IIH). It was previously known as pseudotumor cerebri, as the symptoms can mimic that of a brain tumor, even though no tumor is present. It is considered to be a rare disease, affecting just 1 in 100,000 but milder forms may simply go unrecognized. == Treatment == Treatment approaches may depend on the cause and whether it can be identified. In idiopathic intercranial hypertension, treatments can include reducing cerebospinal fluid volume, e.g., through drug treatments like [[acetazolamide]] ([[Diamox]]) or [[methazolamide]] (Neptazane), or surgical treatments like a shunt, or improving venous outflow (blood draining from the brain) by stenting veins in the brain or neck that may be narrowed.<ref name="Higgins2002" /><ref name="Pickard2003" /><ref name="Halmagyi2011" /><ref name="treatments">{{Cite web | url = https://ihrfoundation.org/treatment/medication-and-surgery | title = Medication and Surgery {{!}} Medication and Surgery {{!}} Treatment | last = | first = | authorlink = | date = | website = Intracranial Hypertension Research Foundation|archive-url=|archive-date=|url-status=|access-date=2022-02-01}}</ref> [[Ketamine]] is not a standard treatment for intracranial hypertension but was shown to reduce ICP by 30% in a controlled trial of 82 pediatric patients in a trauma setting.<ref name="Joseph2009">{{Cite journal | last = Bar-Joseph | first = Gad | last2 = Guilburd | first2 = Yoav | last3 = Tamir | first3 = Ada | last4 = Guilburd | first4 = Joseph N. | date = Jul 2009 | title = Effectiveness of ketamine in decreasing intracranial pressure in children with intracranial hypertension | url =https://www.ncbi.nlm.nih.gov/m/pubmed/19569909/|journal=Journal of Neurosurgery. Pediatrics|volume=4|issue=1 | pages = 40–46|doi=10.3171/2009.1.PEDS08319|issn=1933-0707|pmid=19569909}}</ref> ==Related conditions== {{Video|id=https://www.youtube.com/watch?v=FV92prEwfUY|service=youtube|dimensions=550|description=Dr. Kenneth Liu describes stenting in EDS patients with intracranial hypertension.|alignment=right|urlargs=}}It has been observed by some clinicians that ME/CFS and [[Ehlers-Danlos syndrome]] patients may have borderline or subclinical intracranial hypertension and benefit from IIH treatments such as Diamox, venous stents, or shunts. While these treatments are rarely used by ME/CFS clinicians they are more commonly employed in the clinical care of EDS patients. ===ME/CFS === A case study of a woman presenting with symptoms of CFS and pressure [[headache]], who was diagnosed with borderline intracranial hypertension, found that her CFS symptoms resolved with the placement of a transverse sinus stent.<ref name="Tanaka2002">{{Cite journal | title = Impaired postural cerebral hemodynamics in young patients with chronic fatigue with and without orthostatic intolerance | date = 2002-04-01 | url = https://www.sciencedirect.com/science/article/pii/S0022347602157878|journal=The Journal of Pediatrics|volume=140|issue=4 | pages = 412–417 | last = Tanaka | first = Hidetaka | last2 = Matsushima | first2 = Reiko | last3 = Tamai | first3 = Hiroshi | last4 = Kajimoto | first4 = Yoshinaga|language=en|doi=10.1067/mpd.2002.122725|issn=0022-3476}}<span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=The+Journal+of+Pediatrics&rft.atitle=Impaired+postural+cerebral+hemodynamics+in+young+patients+with+chronic+fatigue+with+and+without+orthostatic+intolerance&rft.volume=140&rft.issue=4&rft.pages=412-417&rft.date=2002-04-01&rft_id=info%3Adoi%2F10.1067%2Fmpd.2002.122725&rft.issn=0022-3476&rfr_id=info%3Asid%2Fme-pedia.org%3AIntracranial+hypertension" class="Z3988"></span></ref> A cross-sectional study of twenty patients presenting at a headache clinic found that a large proportion of patients had borderline intracranial hypertension, with four meeting the diagnostic criteria for IIH (mean cerebrospinal fluid pressure was 19 cm H<sub>2</sub>O (range 12–41 cm H<sub>2</sub>O); however, none had clinical signs of IIH. Cerebrospinal fluid drainage via lumbar puncture improved symptoms in 17/20 patients.<ref name="Higgins2013">{{Cite journal | last = Higgins | first = Nicholas | last2 = Pickard | first2 = John | last3 = Lever | first3 = Andrew | date = 2013-11-21 | title = Lumbar puncture, chronic fatigue syndrome and idiopathic intracranial hypertension: a cross-sectional study | url = http://journals.sagepub.com/doi/pdf/10.1177/2042533313507920|journal=JRSM Short Reports|language=en|volume=4|issue=12 | pages = 204253331350792|doi=10.1177/2042533313507920|issn=2042-5333|pmc=3899735|pmid=24475346}}</ref> Researchers speculate that a subset of CFS patients may have borderline cases of idiopathic intracranial hypertension without papillodema, that is, swelling of the optic nerve.<ref name="Higgins2017">{{Cite journal | last = Higgins | first = J. Nicholas P. | last2 = Pickard | first2 = JohnD. | last3 = Lever | first3 = Andrew M.L. | date = Aug 2017 | title = Chronic fatigue syndrome and idiopathic intracranial hypertension: Different manifestations of the same disorder of intracranial pressure? | url = https://www.ncbi.nlm.nih.gov/pubmed/28735654|journal=Medical Hypotheses|volume=105 | pages = 6–9|doi=10.1016/j.mehy.2017.06.014|issn=1532-2777|pmid=28735654}}</ref> The 2019 Bertilsson/Bragée study<ref name="Bragee2019">{{Cite journal | last = Bragée | first = Björn | last2 = Michos | first2 = Anastasios | last3 = Drum | first3 = Brandon | last4 = Fahlgren | first4 = Mikael | last5 = Szulkin | first5 = Robert | last6 = Bertilson | first6 = Bo C. | date = 2020-08-28 | title = Signs of Intracranial Hypertension, Hypermobility, and Craniocervical Obstructions in Patients With Myalgic Encephalomyelitis/Chronic Fatigue Syndrome | url = https://www.frontiersin.org/article/10.3389/fneur.2020.00828/full|journal=Frontiers in Neurology|volume=11 | pages = 828|doi=10.3389/fneur.2020.00828|issn=1664-2295}}</ref> found evidence of IH in ME/CFS patients meeting the [[Canadian Consensus Criteria]]. Of 205 patients who underwent brain MRIs, 171 (83%) had an optic nerve sheath diameter/eyeball transverse diameter quotient (ONSD/ETD) >0.22, where the maximum found in healthy patients is 0.23.<ref name="Kim2017">{{Cite journal | last = Kim | first = Dong Hwan | last2 = Jun | first2 = Jin-Sun | last3 = Kim | first3 = Ryul | date = Dec 2017 | title = Ultrasonographic measurement of the optic nerve sheath diameter and its association with eyeball transverse diameter in 585 healthy volunteers | url = http://www.nature.com/articles/s41598-017-16173-z|journal=Scientific Reports|language=en|volume=7|issue=1|pages=15906|doi=10.1038/s41598-017-16173-z|issn=2045-2322|pmc = 5698472|pmid=29162911|quote=|via=}}</ref> Eighty-seven patients (42%) had an ONSD/ETD > 0.25, which is the threshold for pathological.<ref name="Du2020">{{Cite journal | last = Du | first = Jie | last2 = Deng | first2 = Yanjun | last3 = Li | first3 = Hua | last4 = Qiao | first4 = Shigang | last5 = Yu | first5 = Mengnan | last6 = Xu | first6 = Qingya | last7 = Wang | first7 = Chen | date = Apr 2020 | title = Ratio of Optic Nerve Sheath Diameter to Eyeball Transverse Diameter by Ultrasound Can Predict Intracranial Hypertension in Traumatic Brain Injury Patients: A Prospective Study | url = http://link.springer.com/10.1007/s12028-019-00762-z|journal=Neurocritical Care|language=en|volume=32|issue=2 | pages = 478–485|doi=10.1007/s12028-019-00762-z|issn=1541-6933|pmc=|pmid=|quote=|via=}}</ref> Of 125 patients who underwent a cervical spine MRI, 100 (80%) has some form of obstruction capable of increasing pressure- e.g. spondylolisthesis, osteophytes, cysts, syrinxes. 13.2% had tonsillar herniations severe enough to be considered a [[Chiari malformation|Chiari Malformation]].<ref name="Bragee2019" /> ===Ehlers-Danlos syndrome=== A number of EDS neurosurgeons have observed an association between intracranial hypertension and EDS.<ref name="Henderson2017">{{Cite journal | last = Henderson | first = Fraser C. | last2 = Austin | first2 = Claudiu | last3 = Benzel | first3 = Edward | last4 = Bolognese | first4 = Paolo | last5 = Ellenbogen | first5 = Richard | last6 = Francomano | first6 = Clair A. | last7 = Ireton | first7 = Candace | last8 = Klinge | first8 = Petra | last9 = Koby | first9 = Myles | date = 2017 | title=Neurological and spinal manifestations of the Ehlers–Danlos syndromes | url = https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.c.31549|journal=American Journal of Medical Genetics Part C: Seminars in Medical Genetics|language=en|volume=175|issue=1|pages=195–211|doi=10.1002/ajmg.c.31549|issn=1552-4876}}</ref> Penn State vascular neurosurgeon, Dr. [[Kenneth Liu]], has presented case studies of patients with EDS whose symptoms improve with venous stenting.<ref>{{Cite web | url = https://vimeo.com/190164038 | title = Venous Stenting in Intracranial Hypertension | last = Liu | first = Kenneth | date = |website=|archive-url=|archive-date=|url-status=|access-date=}}</ref> == Notable studies == * 2018, The link between idiopathic intracranial hypertension, fibromyalgia, and chronic fatigue syndrome: exploration of a shared pathophysiology<ref name="Dankaerts2018">{{Cite web | url = https://www.dovepress.com/the-link-between-idiopathic-intracranial-hypertension-fibromyalgia-and-peer-reviewed-article-JPR | title = The link between idiopathic intracranial hypertension, fibromyalgia, and chronic fatigue syndrome: exploration of a shared pathophysiology | last = Dankaerts | first = Wim | authorlink= | last2 = Bruyninckx | first2 = Frans | authorlink2 = | date = 2018-12-10 | website = Journal of Pain Research|language=English|doi=10.2147/jpr.s186878|pmc=|pmid=30573989|archive-url=|archive-date=|url-status=|access-date=2019-01-03 | last3 = Stalmans | first3 = Ingeborg | last4 = Vansant | first4 = Greet | last5 = Rasschaert | first5 = Ricky | last6 = Hulens | first6 = Mieke}}</ref> - [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6292399/ (Full text)] == See also == * [[Chiari malformation|Arnold-Chiari Malformation]] * [[Cerebral Venous Stenosis]] * [[Empty sella syndrome]] * [[Fibromyalgia]] == Learn more == *[https://ihrfoundation.org/ Intercranial Hypertension Research Foundation] * Video: [https://vimeo.com/190164038 Venous Stenting in Intracranial Hypertension], Dr. Kenneth Liu ==References== {{reflist}} [[Category:Diagnoses]] [[Category:Potential comorbidities]] [[Category:Neurological diseases and disorders]]
Summary:
Please make sure your edits are consistent with
MEpedia's guidelines
.
By saving changes, you agree to the
Terms of use
, and you irrevocably agree to release your contribution under the
CC BY-SA 3.0 License
and the
GFDL
. You agree that a hyperlink or URL is sufficient attribution under the Creative Commons license.
Cancel
Editing help
(opens in new window)
Templates used on this page:
Template:Category handler
(
edit
)
Template:Citation needed
(
edit
)
Template:Cite journal
(
edit
)
Template:Cite news
(
edit
)
Template:Cite web
(
edit
)
Template:Colon
(
edit
)
Template:Digits
(
edit
)
Template:Fix
(
edit
)
Template:Fix/category
(
view source
) (protected)
Template:GetFallback
(
edit
)
Template:LangSwitch
(
edit
)
Template:Last word
(
edit
)
Template:Main other
(
edit
)
Template:NeedsImage
(
edit
)
Template:Reflist
(
edit
)
Template:Str len
(
edit
)
Template:Uselang
(
edit
)
Template:Video
(
edit
)
Template:Year
(
edit
)
Module:Arguments
(
edit
)
Module:Category handler
(
edit
)
Module:Category handler/blacklist
(
edit
)
Module:Category handler/config
(
edit
)
Module:Category handler/data
(
edit
)
Module:Category handler/shared
(
edit
)
Module:Check for unknown parameters
(
edit
)
Module:Citation/CS1
(
edit
)
Module:Citation/CS1/COinS
(
edit
)
Module:Citation/CS1/Configuration
(
edit
)
Module:Citation/CS1/Date validation
(
edit
)
Module:Citation/CS1/Identifiers
(
edit
)
Module:Citation/CS1/Utilities
(
edit
)
Module:Citation/CS1/Whitelist
(
edit
)
Module:Namespace detect/config
(
edit
)
Module:Namespace detect/data
(
edit
)
Module:No globals
(
edit
)
Module:String
(
edit
)
Module:Unsubst
(
edit
)
Module:Yesno
(
edit
)
This page is a member of 3 hidden categories:
Category:All articles with unsourced statements
Category:Articles that need an image or photo
Category:Articles with unsourced statements from 2019
Navigation
Navigation
Skip to content
Main page
Browse
Become an editor
Random page
Popular pages
Abbreviations
Glossary
About MEpedia
Links for editors
Contents
Guidelines
Recent changes
Pages in need
Search
Help
Wiki tools
Wiki tools
Special pages
Page tools
Page tools
User page tools
More
What links here
Related changes
Page information
Page logs