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A 63-year-old woman presented with headache, progressive somnolence, neurocognitive decline and urinary incontinence through a year. Medical history was unremarkable except for hypertension and hypercholesterolaemia. Neurological examination was normal. Brain MRI showed findings typical for spontaneous intracranial hypotension (subdural fluid collection, pachymeningeal enhancement, brain sagging) and pituitary tumour. The patient’s complaints improved dramatically but temporarily after treatment with each of repeated targeted as well as non-targeted blood patches and a trial with continuous intrathecal saline infusion. Extensive work up including repeated MRI-scans, radioisotope cisternographies, CT and T2-weighted MR myelography could not localise the leakage, but showed minor root-cysts at three levels. Finally, lateral decubitus digital subtraction dynamic myelography with subsequent CT myelography identified a tiny dural venous fistula at the fourth thoracic level. After surgical venous ligation, the patient fully recovered. Awareness of spontaneous dural leaks and their heterogeneous clinical picture are important and demands an extensive workup.

ObjectivesThe 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.DesignConsensus guideline critically reviewed by the Association of British Neurologists, British Association for the Study of Headache, the Society of British Neurological Surgeons and the Royal College of Ophthalmologists.SubjectsAn initial UK survey of attitudes and practice in IIH was sent to a wide group of physicians and surgeons.MethodsBetween September 2015 and October 2017 a specialist interest group including neurology, neurosurgery, neuro-radiology, ophthalmology, nursing, primary care doctors, and patient representatives met. A comprehensive systematic literature review was performed to assemble the foundations of the statements.ResultsOver twenty questions were constructed: One based on the diagnostic principles for optimal investigation of papilloedema and twenty-one for the management of IIH. 3 main principles were identified:to treat the underlying disease;to protect the vision andto minimise the headache morbidity.Statements presented provide insight to uncertainties in IIH where research opportunities exist.ConclusionsIn collaboration with many different specialists, professions and patient representatives we have developed guidance statements for the investigation and management of adult IIH.

Background Management of Idiopathic Intracranial Hypertension (IIH) aims to reduce intracranial pressure (ICP). Acetazolamide is the most commonly used drug, with class 1 evidence demonstrating modest improvement in patients with mild visual loss. Other drugs used include Topiramate, Furosemide, Amiloride and Octreotide, despite little mechanistic or clinical evidence to support their use. The aim of this study was to ascertain which of these drugs has the greatest effect on lowering ICP in-vivo. Methods Using a validated epidural ICP recording method we measured changes in ICP in conscious female rodents after subcutaneous administration of these drugs at clinically equivalent and supra-clinical doses over 2 hours (peak plasma concentrations). Results At clinical doses, Topiramate lowered ICP by 32% (p=0.0009) compared to a 25% reduction for Acetazolamide (p=0.0081). Post-hoc analysis showed no significant difference between the two (p=0.85). Furosemide, Amiloride and Octreotide had no significant effect. Conclusion Our in-vivo studies have demonstrated that, at clinically equivalent doses, Topiramate significantly lowers ICP and is as effective as acetazolamide. Topiramate may have additional advantages in IIH, including its migraine prevention properties and weight loss effects. These findings support the need for future randomised controlled trials evaluating the therapeutic efficacy of topiramate in IIH.

Current therapies for reducing raised intracranial pressure (ICP) in conditions such as idiopathic intracranial hypertension (IIH) have limited efficacy and tolerability; therefore there is an urgent need to develop novel therapies. Glucagon-like peptide-1 receptor (GLP-1R) agonists are used therapeutically to treat diabetes and promote weight loss but have also been shown to affect fluid homeostasis in the kidney. The GLP-1R is also present in the choroid plexus, therefore we investigated whether Exendin-4, a GLP-1R agonist, is able to modulate cerebrospinal fluid (CSF) secretion at the choroid plexus and subsequently reduce ICP. We used in vitro models of the choroid plexus to demonstrate the presence of the GLP-1R and, after Exendin-4 treatment, showed a two fold increase in cAMP, a downstream signaling molecule. We also determine that treatment with Exendin-4 reduces Na+ K+ ATPase activity, a key regulator of CSF secretion. Finally, we demonstrate that administration of Exendin-4 in normal and raised ICP (hydrocephalic) rats significantly reduces ICP. These findings provide the first proof of concept that GLP-1R agonists can affect CSF secretion and ICP. Repurposing existing GLP-1 drugs may represent a novel therapeutic strategy for conditions of raised ICP including IIH, hydrocephalus and traumatic brain injury.

STUDY OBJECTIVE--The aim was to study the extent and type of health service utilisation, medication habits, and sickness absence due to the primary headaches. DESIGN--This was a cross sectional epidemiological survey of headache disorders in a general population. Headache was diagnosed according to a structured interview and a neurological examination using the criteria of the International Headache Society. SETTING--A random sample of 25-64 year-old individuals was drawn from the Danish National Central Person Registry. All subjects were living in the Copenhagen County. PARTICIPANTS--740 subjects participated (76% of the sample); 119 had migraine and 578 had tension type headache. MAIN RESULTS--Among subjects with migraine 56% had, at some time, consulted their general practitioner because of the migraine. The corresponding percentage among subjects with tension type headache was 16. One or more specialists had been consulted by 16% of migraine sufferers and by 4% of subjects with tension type headache. The consultation rates of chiropractors and physiotherapists were 5-8%. Hospital admissions and supplementary laboratory investigations due to headache were rare (< 3%). Half of the migraine sufferers and 83% of subjects with tension type headache in the previous year had managed with at least one type of drug in the current year. Acetylsalicylic acid preparations and paracetamol were the most commonly used analgesics. Prophylaxis of migraine was used by 7%. In the preceding year 43% of employed migraine sufferers and 12% of employed subjects with tension type headache had missed one or more days of work because of headache. Most common was 1-7 days off work. The total loss of workdays per year due to migraine in the general population was estimated at 270 days per 1000 persons. For tension type headache the corresponding figure was 820. Women were more likely to consult a practitioner than men, whereas no significant sex difference emerged as regards absenteeism from work. CONCLUSIONS--The impact of the headache disorders on work performance in the general population is substantial, and the disorders merit increased attention.

Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype or an atypical disease course should alert the physician to reevaluate a presumed IIH-diagnosis. The authors report a case of a 32-year-old non-obese male with intracranial hypertension, secondary to a syphilitic central nervous system infection, initially misdiagnosed as being idiopathic. Upon relevant antibiotic treatment, signs and symptoms of elevated intracranial pressure resolved completely. Syphilis is a rare, but very important, differential diagnosis that in this case was clinically indistinguishable from IIH.