Old names for ih include benign intracranial hypertension and pseudotumor cerebri. Because the symptoms of iih can resemble those of a brain. Diagnosis and prognosis, brain, volume 97, issue 2, 1 june 1974, pages 289300. Benign intracranial hypertension in adult after tetracycline therapy. The intracranial hypertension research foundation is the only nonprofit organization in the world. 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. 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 symptoms, diagnosis. Idiopathic intracranial hypertension was first described in 189711 as serous meningitis, followed by various names such as otitic hydrocephalus, toxic hydrocephalus, etc, pseudotumour cerebri,12 and the commonly known benign intracranial hypertension. Idiopathic intracranial hypertension is also called pseudotumor cerebri syndrome and benign intracranial hypertension.
The most common symptoms are headaches and vision loss. Mr imaging of idiopathic intracranial hypertension american. The term, iih, has now replaced the name benign intracranial hypertension bih. A survey was sent to doctors who manage idiopathic intracranial hypertension iih regularly. As you observe, benign intracranial hypertension bih is by no means a universally benign condition, being a recognised cause of usually reversible visual loss. A practical approach to, diagnosis, assessment and management. This disorder presents more commonly in obese, young women, presenting commonly as. According to the dandy criteria, an iih diagnosis is appropriate if a person.
Idiopathic intracranial hypertension is common in obese women and can lead to significant visual impairment. Benign intracranial hypertension also known as pseudotumor cerebri and use of combined and progestogenonly contraceptive methods. Aan members 800 8791960 or 612 9286000 international nonaan member subscribers 800 6383030 or 301 2232300 option 3. Pdf diagnosis and management of benign intracranial. The investigation and management of iih is complex involving many specialities. Welcome to the intracranial hypertension research foundation.
Intracranial hypertension ih is a buildup of pressure around the brain. Papilledema and idiopathic intracranial hypertension. Drugs used in childhood idiopathic or benign intracranial. Diagnosisandmanagementofbenignintracranial hypertension. This case demonstrates typical appearances of benign intracranial hypertension, one of the more common causes of an empty sella. Clinical findings headache, nausea, vomiting, pulsatile tinnitus, double vision and other visual symptoms. Evaluation and management of adult idiopathic intracranial. This paper summarises the first consensus guidelines for idiopathic intracranial hypertension as an infographic. First described more than 100 years ago, the cause of the disorder remains unknown. Benign intracranial hypertension was initially associated with impaired cerebrospinal fluid absorption resulting from lateral sinus thrombosis after acute otitis media, hence the term post otitic hydrocephalus. It is most common in women between the ages of 20 and 50. Methods between september 2015 and october 2017, a specialist interest group including neurology. Increased pressure within the brain in the absence of a tumor. The modified dandy criteria consist of, the classic findings of headache, pulsatile tinnitus, papilledema, and elevated cerebrospinal fluid csf pressure, however, these are rarely collectively present in any one patient.
Benign intracranial hypertension ciprofloxacin administration. Idiopathic intracranial hypertension iih has been defined as a condition in which there is increased intracranial pressure icp without a spaceoccupying lesion or hydrocephalus and with a normal cerebrospinal fluid csf composition. Update on idiopathic intracranial hypertension michael wall, md idiopathic intracranial hypertension iih is a disorder of overweight women in the childbearing age characterized by increased intracranial pressure with its associated signs and symptoms in alert and oriented patients. Iih is commonly associated with obesity, younger age and females. Idiopathic intracranial hypertension iih, also known as pseudotumor cerebri, is a disorder of increased intracranial pressure that occurs mainly in overweight women of childbearing years, often in the setting of weight gain. This patient went on to have csf pressures measured via a lumbar puncture which demonstrated elevated pressures 30cm h20. A practical approach to, diagnosis, assessment and. Methods between september 2015 and october 2017, a specialist interest group including neurology, neurosurgery. Idiopathic intracranial hypertension iih is defined as a syndrome of signs and symptoms of increased intracranial pressure without causative lesions on magnetic resonance imaging mri or computed tomography1. Surgical treatment of benign intracranial hypertension. In the literature, iih primarily affects young obese women and the estimated incidence in this. 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.
Symptoms may include headache, nausea, vomiting, pulsating intracranial noises, singing in the ears, double vision, loss of visual accuracy, and even blindness. Idiopathic intracranial hypertension national eye institute. Idiopathic intracranial hypertension nord national. Intracranial hypertension causes, risk factors, symptoms. Dec 12, 2019 idiopathic intracranial hypertension iih, previously referred to as pseudotumor cerebri, is a disorder of increased intracranial pressure. It is a diagnosis of exclusion, and radiologic examinations are traditionally performed to help exclude lesions that produce intracranial hypertension. Iih, also known as pseudotumor cerebri and benign intracranial hypertension, is a syndrome characterized by increased csf pressure and papilledema in patients without focal neurologic findings, except for occasional cn vi palsy. Benign intracranial hypertension is a diagnosis of exclusion characterised by the signs, symptoms and proof of raised intracranial pressure in the absence of localising neurological signs, obstruction or deformation of the ventricular system, in an alert and orientated patient modified dandy criteria, table 1, 14. A neurological disorder characterised by an idiopathic increase in intracranial pressure in the absence of organic disease. Idiopathic intracranial hypertension in pregnant women. Idiopathic intracranial hypertension in a 24yearold woman. Idiopathic intracranial hypertension the lancet neurology. Benign intracranial hypertension pubmed central pmc. This is rare and sometimes its not clear why it happens.
The definitive diagnosis is idiopathic intracranial hypertension iih, also known by the older terms benign intracranial hypertension bih or pseudotumor cerebri ptc. Find out who is at risk for iih, how it is diagnosed and treated, and what research is being done. Diagnosis and management of benign intracranial hypertension. It can also be a persistent, longlasting problem, known as chronic ih. Idiopathic intracranial hypertension in pregnancy lakshmi thirumalaikumar mrcog,a, kalaivani ramalingam mrcog,b tom hea. Idiopathic intracranial hypertension headache australia. Correspondence benign intracranial hypertension br med j 1970. Where there is no apparent underlying cause for the raised intracranial pressure, patients are considered to have idiopathic intracranial hypertension iih. Impairment of cerebrospinalfluid reabsorption is the most likely underlying pathophysiological cause of the raised. Benign intracranial hypertension brain oxford academic. Many explanations have been proposed concerning the etiology of benign intracranial hypertension bih.
Common symptoms are headache, pulsatile tinnitus, nausea. Iih is common in obese women and can lead to significant visual impairment. Headache was the presenting symptom in 57% followed in order of frequency by earache, blurred or double vision, and vomiting. The first page of the pdf of this article appears above.
While once called benign intracranial hypertension, to distinguish it from secondary intracranial hypertension produced by a neoplastic malignancy, it is not a benign disorder. The commonest predisposing factor was chronic middleear disease which was found in 39%. 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. Benign intracranial hypertension bih also known as pseudotumor cerebri and empty sella syndrome remains a diagnostic challenge to most physicians. Benign intracranial hypertension is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih. Pseudotumor cerebri and benign intracranial hypertension are both former names for ih, which are now considered inaccurate. These names do not adequately describe the disorder and downplay the seriousness of ih. It can also be a persistent, longlasting problem, known as chronic. Incidence is 1100,000 in normalweight women but 20100,000 in obese women. Many patients suffer from intractable, disabling headaches, and there is a risk of severe, permanent vision loss in approximately 5 percent of patients.
This disorder presents more commonly in obese, young women, presenting commonly as headaches and papilledema. Idiopathic intracranial hypertension iih, previously referred to as pseudotumor cerebri, is a disorder of increased intracranial pressure. Idiopathic intracranial hypertension was first reported in 1893 by heinrich quincke, and terms meningitis serosa. Intracranial hypertension ih is the general term for the neurological disorders in which cerebrospinal fluid csf pressure within the skull is too high. We report the case of a 9yearold male patient with idiopathic intracranial hypertension without papilledema for which mr imaging of the optic nerves and pituitary gland provided important clues for the diagnosis of idiopathic intracranial hypertension and showed a return to normal appearance after normalization of csf pressure. Idiopathic intracranial hypertension iih is caused by high pressure in the spaces that surround the brain and spinal cord. The main symptoms are headache, vision problems, ringing in the ears with the heartbeat, and shoulder pain.
Prior to its diagnosis all other causes of raised intracranial pressure need to be excluded. Mr imaging of idiopathic intracranial hypertension. It can happen suddenly, for example, as the result of a severe head injury, stroke or brain abscess. Benign intracranial hypertension definition of benign. Intracranial hypertension exists when there is a sustained elevation in intracranial pressure icp of more than 15 to 20 mm hg. This means that benign intracranial hypertension, or a subtype of benign intracranial hypertension, affects less than 200,000 people in the us population. It results when the three intracranial componentsblood, brain, and cerebrospinal fluid csfare no longer able to compensate for volume changes occurring within the cranium. 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 radiology reference. Intracranial hypertension, is a neurological disorder characterized by increased intracranial pressure resulting in temporary or permanent loss of vision.
However the placement of intracranial probe is not always possible and noninvasive assessment of intracranial pressure may be useful, particularly in case of emergencies. Idiopathic intracranial hypertension typically occurs in women of childbearing age. Update on idiopathic intracranial hypertension michael wall, md idiopathic intracranial hypertension iih is a disorder of overweight women in the childbearing age characterized by increased intracranial pressure with its associated signs and symptoms in alert. Despite a multitude of proposed links, the aetiology has never been established. 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. Idiopathic intracranial hypertension iih, or pseudotumor cerebri, formerly called benign intracranial hypertension, is a challenging condition with raised intracranial pressure icp in the absence of identifiable cause friedman et al. 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. 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. Intracranial hypertension an overview sciencedirect topics.