The term idiopathic means that it occurs for unclear reasons, intracranial means inside the head, and hypertension means high pressure. The recent discoveries of the glymphatic and lymphatic systems of the brain have helped advance our understanding of csf physiology and may allow new insights in the understanding of idiopathic intracranial hypertension iih. Sep 11, 2018 the recent discoveries of the glymphatic and lymphatic systems of the brain have helped advance our understanding of csf physiology and may allow new insights in the understanding of idiopathic intracranial hypertension iih. Pdf idiopathic intracranial hypertension devin binder. Benign intracranial hypertension also known as pseudotumor cerebri and use of combined and progestogenonly contraceptive methods. Pseudotumor cerebri literally translates to false brain tumor. The multicenter trial also examined a number of secondary outcomes. Idiopathic intracranial hypertension iih, also known as pseudotumor cerebri is a condition in which the pressure in your head is elevated. Idiopathic intracranial hypertension treatment of idiopathic intracranial hypertension should be started as soon as diagnosis is confirmed to prevent vision loss. It is a disorder defined by clinical criteria that include symptoms and signs isolated to those produced by increased intracranial pressure icp. Pdf idiopathic intracranial hypertension researchgate. This is a primary reason why chronic ih is often misdiagnosed. Idiopathic intracranial hypertension iih is a disorder of elevated intracranial pressure. The main symptoms are headache, vision problems, ringing in the ears with the heartbeat, and shoulder pain.
This disorder presents more commonly in obese, young women, presenting commonly as headaches and papilledema. Idiopathic intracranial hypertension iih is a challenging disorder with a rapid increasing incidence due to a close relation to obesity. Intracranial hypertension can be primary and idiopathic in obese women of childbearing age. Idiopathic intracranial hypertension iih is a disorder of elevated intracranial pressure icp of unknown origin. May 31, 2017 idiopathic intracranial hypertension iih a disorder of unknown aetiology that predominantly affects obese women of childbearing age. Weight management in idiopathic intracranial hypertension. Introduction idiopathic intracranial hypertension iih is also called pseudotumor cerebri. The primary issue is chronically raised intracranial pressure icp, and the most crucial neurologic symptom is papilledema, which may result in progressive optic atrophy and blindness. Idiopathic intracranial hypertension practical neurology. Jul 07, 2017 people suffering from idiopathic intracranial hypertension often go about their routine lives and activities without changing their diet. The main goals of treatment are to preserve visual function and alleviate symptoms, which can usually be achieved with a combination of weight loss, medical therapies, and surgical.
The onset of symptoms is often insidious and patients may see many different specialists before the iih diagnosis is settled. In some paediatric reports, adrenal insufficiency has been reported in association also with idiopathic intracranial hypertension iih. Exploring the current management idiopathic intracranial. Mar 21, 2016 idiopathic intracranial hypertension iih is a challenging disorder with a rapid increasing incidence due to a close relation to obesity. The idiopathic intracranial hypertension treatment trial. Pdf idiopathic intracranial hypertension vinay shah. Pseudotumor cerebri, idiopathic intracranial hypertension.
Methods between september 2015 and october 2017, a specialist interest group including neurology, neurosurgery. The clinical presentation is dominated by progressive visual disturbances, which. The high pressure is caused by a buildup of cerebrospinal fluid csf. Idiopathic intracranial hypertension in pregnancy federal. Although it can affect any age and gender, it is classically seen in obese or overweight women of childbearing age 1. Idiopathic intracranial hypertension pseudotumor cerebri the basics summary and recommendations the major morbidity associated with idiopathic intracranial hypertension iih is loss of vision, although persistent headaches are also a source of disability and decreased quality of life. Idiopathic intracranial hypertension iih, also called pseudotumor cerebri, is a condition in which high pressure inside your head can cause headache and problems with vision. Its also called idiopathic intracranial hypertension.
Idiopathic intracranial hypertension definition of. Adult patients who present with papilloedema and symptoms of raised intracranial pressure need urgent multidisciplinary assessment including neuroimaging, to exclude lifethreatening causes. Idiopathic intracranial hypertension iih, previously known as pseudotumor cerebri and benign intracranial hypertension, is a condition characterized by increased intracranial pressure pressure around the brain without a detectable cause. Secondary intracranial hypertension can be seen with cerebral venous thrombosis chapters 414 and 421 chapter 414 chapter 421, masses in the brain chapter 195, hydrocephalus, or other intracranial processes. This is a pdf file of an unedited manuscript that has been accepted for. This disorder presents more commonly in obese, young women, presenting commonly as. Idiopathic intracranial hypertension iih, or pseudotumor cerebri, formerly called benign intracranial hypertension, is a challenging condition with. Idiopathic intracranial hypertension iih is a rare condition where intracranial hypertension is found in the context of normal brain parenchyma and no mass lesion, ventriculomegaly, underlying infection, or malignancy. The term pseudotumor cerebri was later introduced in 1904, and later still benign intracranial hypertension in 1955 not to be confused with benign intracranial hypotension 15. Where there is no apparent underlying cause for the raised intracranial pressure, patients are considered to have idiopathic intracranial hypertension iih. Idiopathic intracranial hypertension genetic and rare.
The signs and symptoms of intracranial hypertension are that the patient maintains an alert and oriented mental state, but has no localizing neurologic findings. Csf is a clear liquid that coats your brain and spinal cord. However, certain diet changes can go a long way in aiding in improving the condition to a large extent. Idiopathic intracranial hypertension iih, previously referred to as pseudotumor cerebri, is a disorder of increased intracranial pressure. See idiopathic intracranial hypertension pseudotumor cerebri. The idiopathic intracranial hypertension treatment trial iihtt was the first large, randomized study on the use of acetazolamide and weight loss for treatment of idiopathic intracranial hypertension. People suffering from idiopathic intracranial hypertension often go about their routine lives and activities without changing their diet. Visual acuity is less than 2020 in 10 to 29 percent of patients on presentation 2,3,3537. The clinical and radiologic presentations of iih appear to be related to congestion of the glymphatic system associated with an overflow of the lymphatic csf outflow. This is rare and sometimes its not clear why it happens.
Benign intracranial hypertension bih is a headache syndrome characterised by 1 raised cerebrospinal fluid csf pressure in the absence of an intracranial mass lesion or ventricular dilatation. To characterize the clinical course of iih more completely, we monitored the clinical status, especially visual function, in 50 consecutive newlydiagnosed patients over a period of 2 to 39 months average. This form is known to occur in young, overweight, females in their reproductive years ages 2045. Evaluation and management of adult idiopathic intracranial. Dec 12, 2019 idiopathic intracranial hypertension iih, previously referred to as pseudotumor cerebri, is a disorder of increased intracranial pressure. Pseudotumor cerebri, more commonly referred to as idiopathic intracranial hypertension iih, is characterized by increased intracranial pressure icp with no explanatory findings on imaging studies or in cerebrospinal fluid csf analysis, and may be accompanied by symptoms of chronic headache, tinnitus, papilledema and progressive vision. Headache attributed to benign intracranial hypertension bih. An initial uk survey of attitudes and practice in iih was sent to a wide group of physicians and. The increased intracranial pressure can cause swelling of the optic nerve and result in vision loss. Prognosis and treatment, section on treatment goals and monitoring. The owners of this website hereby guarantee intracrwnienne respect the legal confidentiality conditions, applicable in france, and not to disclose this data to third parties. Jan 28, 2016 idiopathic intracranial hypertension iih is a disorder of unidentified etiology that predominantly influences overweight females of childbearing age. Does benign intracranial hypertension contraindicate use of contraceptive steroids.
Idiopathic intracranial hypertension, also known as benign intracranial hypertension or pseudotumour cerebri, is a condition of unknown cause characterised by raised intracranial pressure and papilloedema. The clinical and radiologic presentations of iih appear to be related to congestion of the glymphatic system associated with an overflow of the lymphatic csf. It most frequently occurs in obese women of childbearing age. Summary idiopathic intracranial hypertension is a disorder producing symptoms and signs of increased intracranial pressure in the absence of an alternative cause. New headache, or a significant worsening of a preexisting headache, caused by and accompanied by other symptoms andor clinical andor neuroimaging signs of idiopathic intracranial hypertension iih, with typical features suggestive of iih. Understanding the complex pathophysiology of idiopathic. Symptoms of iih may include severe headache, nausea. Iih idiopathic intracranial hypertension or bih benign intracranial. Idiopathic intracranial hypertension journal of neurology. The high pressure can cause headache or vision problems. Idiopathic intracranial hypertension iih, also referred to as pseudotumor cerebri or benign intracranial hypertension, is a medical condition characterized by a combination of intractable headaches, papilledema, visual symptoms, tinnitus, and an elevated cerebrospinal fluid csf opening pressure op on lumbar puncture lp in the absence of an intracranial mass. Intracranial hypertension ih is a buildup of pressure around the brain. Idiopathic intracranial hypertension in pregnancy lakshmi thirumalaikumar mrcog,a, kalaivani ramalingam mrcog,b tom hea.
Primary intracranial hypertension, now known as idiopathic intracranial hypertension iih, occurs without known cause. A survey was sent to doctors who manage idiopathic intracranial hypertension iih regularly. Idiopathic intracranial hypertension nord national. Idiopathic intracranial hypertension michigan medicine. Idiopathic intracranial hypertension iih is caused by high pressure in the spaces that surround the brain and spinal cord. Pdf an update on idiopathic intracranial hypertension. Methods between september 2015 and october 2017, a specialist interest group including neurology, neurosurgery, neuroradiology, ophthalmology, nursing, primary care doctors and patient representatives met. Intracranial hypertension an overview sciencedirect topics. Management of patients with idiopathic intracranial hypertension iih should be based on the presence and progression of visual loss. Jul 05, 2019 chronic intracranial hypertension can be caused by many conditions including certain drugs such as tetracycline, a blood clot in the brain, excessive intake of vitamin a, or brain tumor. Idiopathic intracranial hypertension pseudotumor cerebri. Other symptoms idiopathic intracranial hypertension. Idiopathic intracranial hypertension brain oxford academic.
It can also be a persistent, longlasting problem, known as chronic. Idiopathic intracranial hypertension is a disorder characterised by raised intracranial pressure that predominantly affects young, obese women. A practical approach to, diagnosis, assessment and. Idiopathic intracranial hypertension, optic nerve sheath fenestration. Sep 11, 2019 pseudotumor cerebri soodoetoomur seruhbry occurs when the pressure inside your skull intracranial pressure increases for no obvious reason. Idiopathic intracranial hypertension pubmed central pmc. Pdf idiopathic intracranial hypertension iih is a condition of unknown etiology often encountered in neurologic practice. Iih idiopathic intracranial hypertension iih idiopathic intracranial hypertension iih, also called pseudotumor cerebri, is a condition in which high pressure inside your head can cause headache and problems with vision.
Iih is common in obese women and can lead to significant visual impairment. Our understanding of this condition has greatly improved in the recent years with neuroimaging features and normal values for lumbar puncture opening pressure now well defined. Update on idiopathic intracranial hypertension ncbi. Idiopathic intracranial hypertension national eye institute. One of the mysteries of this disorder is that not every person will experience the same set of symptoms. Medical definition of idiopathic intracranial hypertension. Other findings on funduscopic examination may include macular exudates and macular edema, choroidal folds across the macula usually resulting from flattening of the globes from increased intracranial pressure. Idiopathic intracranial hypertension iih most commonly occurs in obese females of childbearing age who are also the most likely to be misdiagnosed with iih. Idiopathic intracranial hypertension radiology reference. Headache is the most common symptom, usually presenting as daily and pulsatile. Low energy diet and intracranial pressure in women with. A descriptive study of venous sinus pressures and gradients. Idiopathic intracranial hypertension causes increased intracranial pressure without a mass lesion or hydrocephalus, probably by obstructing venous drainage. Idiopathic intracranial hypertension medical definition.
Idiopathic intracranial hypertension iih is a syndrome characterized by elevated intracranial pressure that usually occurs in obese women in the childbearing years. This paper summarises the first consensus guidelines for idiopathic intracranial hypertension as an infographic. Know the foods to include and foods to avoid in your diet for idiopathic intracranial hypertension. This term was used because symptoms of iih resemble those of brain tumors depsite no tumor being present. The diagnosis and management of idiopathic intracranial. Idiopathic intracranial hypertension presents clinically with signs and symptoms of increased intracranial pressure icp. The first consensus guidelines have been published on investigation and. Apr 12, 2018 idiopathic intracranial hypertension iih, formerly known as pseudotumor cerebri, is a condition that affects the brain. Understanding idiopathic intracranial hypertension the lancet. Methods between september 2015 and october 2017, a specialist interest group including neurology. Idiopathic intracranial hypertension is a secondary headache disorder characterized by headaches and visual symptoms. In a prospective controlled study, 29 patients with established iih as well as 59 control patients underwent ateco mrv.
Idiopathic intracranial hypertension in a 24yearold woman. Idiopathic intracranial hypertension iih is a debilitating disorder characterised by raised intracranial pressure icp, papilloedema with the potential risk of permanent visual loss, and headaches that are profoundly disabling and reduce the quality of life. Because the symptoms of iih can resemble those of a brain. To determine the prevalence and nature of sinovenous obstruction in idiopathic intracranial hypertension iih using autotriggered ellipticcentricordered threedimensional gadoliniumenhanced mr venography ateco mrv. It can also be a persistent, longlasting problem, known as chronic ih. Pdf on dec 4, 2018, dinesh chaudhari and others published a. Idiopathic intracranial hypertension iih, formerly known as pseudotumor cerebri, is a condition that affects the brain. It can happen suddenly, for example, as the result of a severe head injury, stroke or brain abscess. Idiopathic intracranial hypertension is characterized by an increase of intracranial pressure of unknown etiology.
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. Introduction idiopathic intracranial hypertension iih is a disease of. Etiology, risk factor, clinical presentation, fundoscopic examination, papilledema with staging, treatment and intervention. Pseudotumor cerebri symptoms and causes mayo clinic. Find out who is at risk for iih, how it is diagnosed and treated, and what research is being done. Idiopathic intracranial hypertension was first reported in 1893 by heinrich quincke, and terms meningitis serosa. Feb 15, 2016 idiopathic intracranial hypertension iih appears to be due to impaired cerebrospinal fluid csf absorption from the subarachnoid space across the arachnoid villi into the dural sinuses.
782 469 1527 1456 698 1351 1565 1224 16 1163 1452 1312 1 239 1594 742 923 254 904 130 1100 789 751 1351 1432 59 997 16 1332 611 1261 449 920 887 28 150 920 1203 1018 204 448 1386 1290 809 251 171