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).MethodsBetween September 2015 and October 2017, a specialist interest group including neurology, neurosurgery, neuroradiology, ophthalmology, nursing, primary care doctors and patient representatives met. An initial UK survey of attitudes and practice in IIH was sent to a wide group of physicians and surgeons who investigate and manage IIH regularly. A comprehensive systematic literature review was performed to assemble the foundations of the statements. An international panel along with four national professional bodies, namely the Association of British Neurologists, British Association for the Study of Headache, the Society of British Neurological Surgeons and the Royal College of Ophthalmologists critically reviewed the statements.ResultsOver 20 questions were constructed: one based on the diagnostic principles for optimal investigation of papilloedema and 21 for the management of IIH. Three main principles were identified: (1) to treat the underlying disease; (2) to protect the vision; and (3) to 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.

Purpose By incorporating major developments in genetics, ophthalmology, dermatology, and neuroimaging, to revise the diagnostic criteria for neurofibromatosis type 1 (NF1) and to establish diagnostic criteria for Legius syndrome (LGSS). Methods We used a multistep process, beginning with a Delphi method involving global experts and subsequently involving non-NF experts, patients, and foundations/patient advocacy groups. Results We reached consensus on the minimal clinical and genetic criteria for diagnosing and differentiating NF1 and LGSS, which have phenotypic overlap in young patients with pigmentary findings. Criteria for the mosaic forms of these conditions are also recommended. Conclusion The revised criteria for NF1 incorporate new clinical features and genetic testing, whereas the criteria for LGSS were created to differentiate the two conditions. It is likely that continued refinement of these new criteria will be necessary as investigators (1) study the diagnostic properties of the revised criteria, (2) reconsider criteria not included in this process, and (3) identify new clinical and other features of these conditions. For this reason, we propose an initiative to update periodically the diagnostic criteria for NF1 and LGSS.

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Object The authors recently developed and internally validated the ETV Success Score (ETVSS)—a simplified means of predicting the 6-month success rate of endoscopic third ventriculostomy (ETV) for a child with hydrocephalus, based on age, etiology of hydrocephalus, and presence of a previous shunt. A high ETVSS predicts a high chance of early ETV success. In this paper, they assess the clinical utility of the ETVSS by determining whether long-term survival outcomes for ETV versus shunt insertion are different within strata of ETVSS (low, moderate, and high scores). Methods A multicenter, international cohort of children (≤ 19 years old) with newly diagnosed hydrocephalus treated with either ETV (489 patients) or shunt insertion (720 patients) was analyzed. The ETVSS was calculated for all patients. Survival analyses with time-dependent modeling of the hazard ratios were performed. Results For the High-ETVSS Group (255 ETV-treated patients, 117 shunt-treated patients), ETV appeared to have a lower risk of failure right from the early postoperative phase and became more favorable with time. For the Moderate-ETVSS Group (172 ETV-treated patients, 245 shunt-treated patients), ETV appeared to have a higher initial failure rate, but after about 3 months the instantaneous risk of ETV failure became slightly lower than shunt failure (that is, the hazard ratio became < 1). For the Low-ETVSS Group (62 ETV-treated patients, 358 shunt-treated patients), the early risk of ETV failure was much higher than the risk of shunt failure, but the instantaneous risk of ETV failure became lower than the risk of shunt failure at about 6 months following surgery (the hazard ratio became < 1). Conclusions Across all ETVSS strata, the risk of ETV failure becomes progressively lower compared with the risk of shunt failure with increasing time from the surgery. In the best ETV candidates (ETVSS ≥ 80), however, the risk of ETV failure is lower than the risk of shunt failure very soon after surgery, while for less-than-ideal ETV candidates (ETVSS ≤ 70), the risk of ETV failure is initially higher than the risk of shunt failure and only becomes lower after 3–6 months from surgery. These results need to be confirmed by larger, prospective, and preferably randomized studies.

A series of neuroendoscopic third ventriculostomies in children less than 1 year old is reported. Twenty-seven patients underwent the procedure with 21 (77%) failing within a mean of 1.36 months of the procedure. Nineteen were subsequently shunted. The presence or absence of flow through the ventriculostomy and the size of the lateral ventricles on post-operative imaging were not an indicator of success or failure. Only 4 (15%) had a complication of the procedure. Although the majority fail, approximately 1/3 are spared the added morbidity and mortality of having a shunt. With such a low morbidity and zero mortality the procedure has many benefits over shunting. Consequently, neuroendoscopic third ventriculostomy is used in this institution, where possible, rather than a shunt.

BackgroundAtypical meningiomas are an intermediate grade brain tumour with a recurrence rate of 39–58 %. It is not known whether early adjuvant radiotherapy reduces the risk of tumour recurrence and whether the potential side-effects are justified. An alternative management strategy is to perform active monitoring with magnetic resonance imaging (MRI) and to treat at recurrence. There are no randomised controlled trials comparing these two approaches.Methods/DesignA total of 190 patients will be recruited from neurosurgical/neuro-oncology centres across the United Kingdom, Ireland and mainland Europe. Adult patients undergoing gross total resection of intracranial atypical meningioma are eligible. Patients with multiple meningioma, optic nerve sheath meningioma, previous intracranial tumour, previous cranial radiotherapy and neurofibromatosis will be excluded. Informed consent will be obtained from patients. This is a two-stage trial (both stages will run in parallel):Stage 1 (qualitative study) is designed to maximise patient and clinician acceptability, thereby optimising recruitment and retention. Patients wishing to continue will proceed to randomisation.Stage 2 (randomisation) patients will be randomised to receive either early adjuvant radiotherapy for 6 weeks (60 Gy in 30 fractions) or active monitoring.The primary outcome measure is time to MRI evidence of tumour recurrence (progression-free survival (PFS)). Secondary outcome measures include assessing the toxicity of the radiotherapy, the quality of life, neurocognitive function, time to second line treatment, time to death (overall survival (OS)) and incremental cost per quality-adjusted life year (QALY) gained.DiscussionROAM/EORTC-1308 is the first multi-centre randomised controlled trial designed to determine whether early adjuvant radiotherapy reduces the risk of tumour recurrence following complete surgical resection of atypical meningioma. The results of this study will be used to inform current neurosurgery and neuro-oncology practice worldwide.Trial registrationISRCTN71502099 on 19 May 2014.

Noninvasive EM image guidance in shunt surgery reduces poor shunt placement, resulting in a significant decrease in the early shunt revision rate.