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Infected Rathke's cleft cysts (RCC) are extremely rare with only a few published cases. We report the case of a 31-year-old man who presented with headaches, visual disturbance, and hypopituitarism secondary to an infected RCC with extension of abscesses along the optic tract. Magnetic resonance imaging showed ring enhancing cystic lesions within an expanded sella with suprasellar and intraparenchymal extension. The radiological appearance suggested a high-grade optic glioma, but an endoscopic transsphenoidal biopsy revealed frank pus in the pituitary fossa, which subsequently grew Staphylococcus aureus. Pathological examination of the cyst wall showed an inflamed RCC. Following a prolonged course of intravenous antibiotics, the infection resolved and vision improved. RCC abscesses are rare and the intracranial extension of the infection in our case makes it unique.

Summary Pituitary adenomas are intracranial neoplasms, usually demonstrating a benign phenotype. We present the case of 21-year-old male with an 18-month history of reduced visual function (acuity and field) in the left eye. Based on neuroimaging and endocrine profile, a giant prolactinoma causing hypogonadotropic hypogonadism was diagnosed and cabergoline was commenced. After a month of treatment, the tumour size reduced, and visual function improved to normal; however, he developed Foster Kennedy syndrome with a swollen right optic disc. After almost 1 year of follow-up, he regained full visual functioning. Two years since his diagnosis, his prolactin remains normal with no adverse effects or further visual complications. Learning points Foster Kennedy syndrome is a rare entity but can be a feature of pituitary adenomas. Visual deterioration secondary to a compressive optic neuropathy can be reversible, provided that diagnosis and treatment are prompt. This case highlights the importance of frequent monitoring of visual function during follow-up of these lesions, particularly when there are deficits at diagnosis.

Background: The impact on clinical outcomes of patient selection using perfusion imaging for endovascular thrombectomy (EVT) in patients with acute ischemic stroke presenting beyond 6 hours from onset remains undetermined in routine clinical practice. Methods: Patients from a national stroke registry that underwent EVT selected with or without perfusion imaging (noncontrast computed tomography/computed tomography angiography) in the early ( Results: We included 4249 patients, 3203 in the early window (593 with perfusion versus 2610 without perfusion) and 1046 in the late window (378 with perfusion versus 668 without perfusion). Within the late window, patients with perfusion imaging had a shift towards better functional outcome at discharge compared with those without perfusion imaging (adjusted common odds ratio [OR], 1.45 [95% CI, 1.16–1.83]; P =0.001). There was no significant difference in functional independence (29.3% with perfusion versus 24.8% without; P =0.210) or in the safety outcome measures of symptomatic intracerebral hemorrhage ( P =0.53) and in-hospital mortality (10.6% with perfusion versus 14.3% without; P =0.053). In the early time window, patients with perfusion imaging had significantly improved odds of functional outcome (adjusted common OR, 1.51 [95% CI, 1.28–1.78]; P =0.0001) and functional independence (41.6% versus 33.6%, adjusted OR, 1.31 [95% CI, 1.08–1.59]; P =0.006). Perfusion imaging was associated with lower odds of futile recanalization in both time windows (late: adjusted OR, 0.70 [95% CI, 0.50–0.97]; P =0.034; early: adjusted OR, 0.80 [95% CI, 0.65–0.99]; P =0.047). Conclusions: In this real-world study, acquisition of perfusion imaging for EVT was associated with improvement in functional disability in the early and late time windows compared with nonperfusion neuroimaging. These indirect comparisons should be interpreted with caution while awaiting confirmatory data from prospective randomized trials.

INTRODUCTION Superior canal dehiscence syndrome (SCDS) is a rare disorder characterized by an array of audiovestibular symptoms due to a dehiscence of bone overlying the superior semicircular canal (SSC). In the presence of debilitating symptoms, surgical management, to plug or resurface the SCC is performed. Although computed tomography (CT) may overestimate the size or presence of a dehiscence due to a partial volume effect, it remains an invaluable diagnostic tool. OBJECTIVES To assess for correlation between the arc and length of dehiscence and clinical symptomology. METHOD A single-center, single-operator retrospective analysis of 42 patients who underwent trans mastoid plugging of SCC with confirmed radiological dehiscence of their SSC between January 2008 and July 2019 was undertaken. Patients were assessed based on seven predefined clinical symptoms. Length and arc of dehiscence's were evaluated by means of high resolution (0.5 mm) CT (HRCT), using multiplanar reconstruction (MPR). Receiver operating characteristics (ROC), and more specifically the area under the ROC curve (AUROC) were used to assess for statistical significance. RESULTS Our results demonstrate overall very little correlation between the arc and size of the dehiscence and symptoms. The only statistically significant correlation we found was between length of dehiscence and the presence of aural fullness. CONCLUSION SCDS is a debilitating condition with an array of symptoms on presentation. While dehiscence length demonstrated a correlation with aural fullness, no other symptomology in patients with radiologically evident SCDS demonstrated a statistically significant correlation either against the length or arc of dehiscence.

Fungal rhinosinusitis (FRS) is inflammation of the paranasal sinus mucosa due to fungal infections, which can be invasive or non-invasive. The occurrence of a sphenoid mucocele with a fungal ball is rare. We report a case of sphenoid sinus mucocele with a fungal ball caused by Scedosporium apiopermum in a 32-year-old female who presented to the Emergency Department with persistent headache not relieved on medications. The radiological images showed a mucocele with clival osteomyelitis. Urgent endoscopic examination and debridement was undertaken which demonstrated a mucocele with fungal ball. Microbiological examination confirmed it to be Scedosporium apiopermum.

Purpose Highlight and characterize manifestations, diagnostic/management approaches and outcomes in a contemporary cohort of patients with pituitary metastases (PM) from a large European pituitary center—over 10 years. Methods Retrospective review of PM cases between 1/2009 and 12/2018. Clinical, laboratory, imaging data at PM detection and during follow-up were analysed. Results 18 cases were identified (14 females; median age at diagnosis 61.5 years). Most common primary malignancies were lung (39%) and breast (32%). Most frequent presenting manifestation was visual dysfunction (50%). Gonadotrophin, ACTH, TSH deficiency were diagnosed in 85%, 67%, 46% of cases, respectively; diabetes insipidus (DI) was present in 17%. 33% of cases were detected during investigation for symptoms unrelated to PM. PM management included radiotherapy (44%), transsphenoidal surgery (17%), transsphenoidal surgery and radiotherapy (6%) or monitoring only (33%). One-year survival was 49% with median survival from PM detection 11 months (range 2–47). Conclusions In our contemporary series, clinical presentation of PM has evolved; we found increased prevalence of anterior hypopituitarism, decreased rates of DI and longer survival compared with older literature. Increased availability of diagnostic imaging, improvements in screening and recognition of pituitary disease and longer survival of patients with metastatic cancer may be contributing factors.

Purpose: Clival Chordomas are locally aggressive tumours which pose a significant treatment challenge. Endoscopic endonasal approach for clival chordomas is correlated with higher resection rates and lower morbidity rates in comparison to open approaches. We present our initial single institution experience and short-term patient outcomes following endoscopic endonasal approach for resection of clival chordomas. Materials and methods: This is a retrospective analysis of ten patients undergoing endoscopic endonasal approach for clival chordomas in our neurosurgical unit over a 6 year period between August 2010 and September 2016. The procedures were performed using two surgeons, four hands, binostril endoscopic endonasal approach with a Karl Storz® endoscope and intraoperative BrainLab® image guidance. Results: Overall 15 endoscopic endonasal approach resections of clival chordoma were performed in 10 patients with median follow up period of 39.5 months (range 9-76). Gross total resection was achieved in 4 cases (40%), near total resection in 4 cases (40%) and subtotal resection in 2 cases (20%). 5 cases (50%) required revision resections. Cerebrospinal fluid leak occurred in 2 patients. 1 case of meningitis occurred in a patient with revision surgery. There were no new neurological deficits post operatively with 3 patients demonstrating resolution of diplopia post operatively. No recurrence occurred following gross total resection. 1 out of 4 cases of near total resection showed evidence of progression during the follow up period. Both cases of subtotal resection demonstrated evidence of progression with one dying of unrelated cause during the follow up period. Conclusion: Endoscopic endonasal approach represents a safe technique for debulking and resection of clival chordomas. Due to the rarity of clival chordomas, it is important that patients with this pathology are managed in high volume skull base centres where a multi-disciplinary team approach is available.

BACKGROUND AND PURPOSE: The introduction of liquid embolic agents has revolutionized endovascular approach to cranial vascular malformations. The aim of the study was to retrospectively assess the efficacy and safety of Precipitating Hydrophobic Injectable Liquid (PHIL), a new nonadhesive liquid embolic agent, in the treatment of patients with cranial dural arteriovenous fistulas. The primary end point was the rate of complete occlusion of dural arteriovenous fistulas. Secondary end points included the incidence of adverse events and clinical status at 3-month follow-up. MATERIALS AND METHODS: This was a retrospective multicenter study. Twenty-six consecutive patients with dural arteriovenous fistulas (de novo or previously treated) treated by injection of PHIL only or with PHIL in combination with other embolization products (such as Onyx or detachable coils) were included in the study. Recruitment started in August 2014 and ended in September 2015. RESULTS: Twenty-two (85%) patients were treated with PHIL only, with 3 patients treated with both PHIL and Onyx, and 1, with both PHIL and coils. Immediate complete angiographic occlusion was achieved in 20 (77%) patients. Of the 6 patients with residual fistulas, 3 were retreated with PHIL and 1 achieved angiographic cure. An adverse event was seen in 1 patient who developed worsening of preexisting ataxia due to acute thrombosis of the draining vein. CONCLUSIONS: PHIL appears to be safe and effective for endovascular treatment of cranial dural arteriovenous fistulas. Short-term angiographic and clinical results are comparable with those of Onyx, with the added advantage of easier preparation and improved homogeneous cast visualization. The use of iodine as a radio-opacifier also produces considerably less artifacts on CT compared with tantalum-based embolic materials.

Chronic low back pain is extremely common with a life time prevalence estimated at greater than 70%. Facet joint arthrosis is thought to be the causative aetiological substrate in approximately 25% of chronic low back pain cases. Facet joint injection is a routine intervention in the armamentarium for both the diagnostic and therapeutic management of chronic low back pain. In fact, a study by Carrino et al. reported in excess of 94,000 facet joint injection procedures were carried out in the US in 1999. Although generally considered safe, the procedure is not entirely without risk. Complications including bleeding, infection, exacerbation of pain, dural puncture headache, and pneumothorax have been described. We report a rare case of a 47-year-old female patient who developed a left L4/5 facet septic arthrosis with an associated subdural empyema and meningitis following facet joint injection. This case is unique, as to the best of our knowledge no other case of subdural empyema following facet joint injection has been reported in the literature. Furthermore this case serves to highlight the potential serious adverse sequelae of a routine and apparently innocuous intervention. The need for medical practitioners to be alert to and respond rapidly to the infective complications of facet joint injection cannot be understated.

Subarachnoid and intraparenchymal haemorrhages due to cerebral mycotic aneurysms are rare and associated with high mortality. Streptococcus agalactiae, is a rare cause of endocarditis and there are no reported cases of cerebral mycotic aneurysms secondary to this organism. We report a rare case of streptococcus agalctiae induced intracranial mycotic aneurysm.

Summary Co-existence of craniopharyngioma and acromegaly has been very rarely reported. A 65-year-old man presented with visual deterioration, fatigue and frontal headaches. Magnetic resonance imaging revealed a suprasellar heterogeneous, mainly cystic, 1.9 × 2 × 1.9 cm mass compressing the optic chiasm and expanding to the third ventricle; the findings were consistent with a craniopharyngioma. Pituitary hormone profile showed hypogonadotropic hypogonadism, mildly elevated prolactin, increased insulin-like growth factor 1 (IGF-1) and normal thyroid function and cortisol reserve. The patient had transsphenoidal surgery and pathology of the specimen was diagnostic of adamantinomatous craniopharyngioma. Post-operatively, he had diabetes insipidus, hypogonadotropic hypogonadism and adrenocorticotropic hormone and thyroid-stimulating hormone deficiency. Despite the hypopituitarism, his IGF-1 levels remained elevated and subsequent oral glucose tolerance test did not show complete growth hormone (GH) suppression. Further review of the pre-operative imaging revealed a 12 × 4 mm pituitary adenoma close to the right carotid artery and no signs of pituitary hyperplasia. At that time, he was also diagnosed with squamous cell carcinoma of the left upper lung lobe finally managed with radical radiotherapy. Treatment with long-acting somatostatin analogue was initiated leading to biochemical control of the acromegaly. Latest imaging has shown no evidence of craniopharyngioma regrowth and stable adenoma. This is a unique case report of co-existence of craniopharyngioma, acromegaly and squamous lung cell carcinoma that highlights diagnostic and management challenges. Potential effects of the GH hypersecretion on the co-existent tumours of this patient are also briefly discussed. Learning points: Although an extremely rare clinical scenario, craniopharyngioma and acromegaly can co-exist; aetiopathogenic link between these two conditions is unlikely. Meticulous review of unexpected biochemical findings is vital for correct diagnosis of dual pituitary pathology. The potential adverse impact of GH excess due to acromegaly in a patient with craniopharyngioma (and other neoplasm) mandates adequate biochemical control of the GH hypersecretion.

A 69-year-old gentleman with metastatic esophageal adenocarcinoma presented with acute abdominal pain to the emergency medicine department and subsequently developed an acute left hemiplegia while in the resuscitation unit. An unenhanced computed tomography (CT) scan of the head showed right frontal cerebral gas emboli while an unenhanced CT scan of the abdomen and pelvis showed extensive portal venous gas and pneumatosis intestinalis, presumed secondary to bowel infarction.

Subarachnoid and intraparenchymal haemorrhages due to cerebral mycotic aneurysms are rare and associated with high mortality.

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.

Context Despite the major risk of regrowth of clinically nonfunctioning pituitary adenomas (CNFAs) after primary treatment, systematic data on the probability of further tumor progression and the effectiveness of management approaches are lacking. Objective To assess the probability of further regrowth(s), predictive factors, and outcomes of management approaches in patients with CNFA diagnosed with adenoma regrowth after primary treatment. Patients, design, and setting Retrospective cohort study of 237 patients with regrown CNFA managed in two UK centers. Results Median follow-up was 5.9 years (range, 0.4 to 37.7 years). The 5-year second regrowth rate was 35.3% (36.2% after surgery; 12.5% after radiotherapy; 12.7% after surgery combined with radiotherapy; 63.4% with monitoring). Of those managed with monitoring, 34.8% eventually were offered intervention. Type of management and sex were risk factors for second regrowth. Among those with second adenoma regrowth, the 5-year third regrowth rate was 26.4% (24.4% after surgery; 0% after radiotherapy; 0% after surgery combined with radiotherapy; 48.3% with monitoring). Overall, patients with a CNFA regrowth had a 4.4% probability of a third regrowth at 5 years and a 10.0% probability at 10 years; type of management of the first regrowth was the only risk factor. Malignant transformation was diagnosed in two patients. Conclusions Patients with regrown CNFA after primary treatment continue to carry considerable risk of tumor progression, necessitating long-term follow-up. Management approach to the regrowth was the major factor determining this risk; monitoring had >60% risk of progression at 5 years, and a substantial number of patients ultimately required intervention.

Background and purposeThe Pipeline Embolization Device (PED) has become a routine first-line option for treatment of intracranial aneurysms (IAs). We assessed the early safety and technical success of a new version of PED, Pipeline Flex Embolization Device with Shield Technology (Pipeline Shield), which has the same design and configuration but has been modified to include a surface synthetic biocompatible polymer.Materials and methodsThe Pipeline Flex Embolization Device with Shield Technology (PFLEX) study is a prospective, single-arm, multicenter study for the treatment of unruptured IAs using Pipeline Shield. The primary study endpoints included the occurrence of major stroke in the territory supplied by the treated artery or neurologic death at 1 year post-procedure. Secondary endpoints included the rate of Pipeline Shield-related or procedure-related serious or non-serious adverse events. Analyses were conducted to evaluate early safety findings in the 30-day post-procedure period as well as technical procedural success outcomes.ResultsFifty patients with 50 unruptured target IAs were enrolled. Mean aneurysm diameter was 8.82±6.15 mm. Thirty-eight aneurysms (76%) were small (ConclusionsThe results of this first experience with the new Pipeline Flex corroborate the early safety of the device. Mid-term and long-term follow-up examinations will provide data on safety outcomes at the 6-month and 1-year follow-up periods.Clinical trial registrationNCT02390037.

Arteriovenous malformations (AVMs) are the leading causing of intra-cerebral haemorrhage. Stereotactic radiosurgery (SRS) is an established treatment for arteriovenous malformations (AVM) and commonly delivered using Gamma Knife within dedicated radiosurgery units. Linear accelerator (LINAC) SRS is increasingly available however debate remains over whether it offers an equivalent outcome. The aim of this project is to evaluate the outcomes using LINAC SRS for AVMs used within a UK neurosciences unit and review the literature to aid decision making across various SRS platforms. Results have shown comparability across platforms and strongly supports that an adapted LINAC based SRS facility within a dynamic regional neuro-oncology department delivers similar outcomes (in terms of obliteration and toxicity) to any other dedicated radio-surgical platform. Locally available facilities can facilitate discussion between options however throughput will inevitably be lower than centrally based dedicated national radiosurgery units.

Importance We report a new syndrome, which we are calling head-jolting nystagmus , that expands the differential diagnosis of head movement–induced paroxysmal vertigo. Observations Two male patients (65 and 58 years old) described rotational vertigo after violent and brief (1- to 2-second) oscillations of the head (head jolting) that triggered intense horizontal nystagmus lasting 45 seconds. Accelerations of the head required to induce these episodes could only be achieved by the patients themselves. In case 1, the episodes gradually disappeared over a 6-year period. In case 2, magnetic resonance imaging (3-T) suggested a filling defect within the left horizontal semicircular canal. He underwent surgical canal plugging in March 2013 that resolved the symptoms. Conclusions and Relevance We attribute head-jolting nystagmus to dislodged material within the horizontal semicircular canal and provide a mechanistic model to explain its origin.

BackgroundLiquid embolic agents are the preferred embolic material in endovascular treatment of pial and brain arteriovenous malformations and dural arteriovenous fistulas (DAVFs). There is little choice available in interventional neuroradiology practice other than two of the most commonly used liquid embolic agents—n-butyl cyanoacrylate and the Onyx liquid embolic system (ev3 Neurovascular, Irvine, California, USA). PHIL (Precipitating Hydrophobic Injectable Liquid) (Microvention, Inc California, USA) is a new liquid embolic agent, CE marked and available for clinical use in Europe.ObjectiveTo present our preliminary experience using PHIL in treating cranial and spinal DAVFs.MethodsBetween September 2014 and January 2015, eight patients, with five cranial DAVFs and three spinal DAVFs were treated with PHIL as the sole embolic agent used with intent to cure. Clinical presentation, location of DAVF, Borden type, fluoroscopic time, radiation dose, procedural time, injecting microcatheter used, volume of PHIL injected, complications, immediate angiographic data, premorbid and discharge modified Rankin Scale score, and any neurologic deficits were included in the analysis.ResultsSeven patients were successfully treated with complete angiographic exclusion of the fistula in a single sitting. Treatment failed in one patient where only suboptimal microcatheter positioning could be achieved and PHIL failed to penetrate the fistula's nidus. Venous penetration was achieved in all other patients except one with a small fistula, but with adequate fistula penetration by the embolic material. No other technical complication or neurologic deterioration occurred in any of the patients.ConclusionsPHIL liquid embolic agent appears to be an excellent alternative embolic material with certain advantages compared with other available liquid embolic agents. Further studies are required to fully evaluate its safety and efficacy.

A 31 year old lady was admitted with worsening of right sided hemiparesis, on a background five month history of mild right upper limb weakness, and upper limb sensory ataxia. MRI revealed a longitudinally enhancing intrinsic cord lesion from C2-C7 with severe cord expansion, raising initial suspicion for ependymoma. Inflammatory aetiology was considered due to multiple areas of involvement, with T2-weighted signal change within the cervical and thoracic spine, and brain. CSF revealed 4 mononuclear cells, normal protein, normal CSF-serum glucose ratio, T lymphocyte predominance on histology, and no neoplastic cells on flow cytometry. Testing for tuberculosis was negative, including CSF, and extensive imaging did not reveal malignancy. Aquaporin-4 antibodies were positive, and the patient showed marked improvement to intravenous steroids and oral taper, with significant radiological resolution at two months. This case highlights an unusual presentation of NMO, with progressive history over five months, and unusual radiological findings, including severe cord expansion. Positive aquaporin-4 antibody and excellent clinical and radiological response to immunosuppressive therapy confirmed the diagnosis. We took this opportunity to review the clinical presentation and MRI findings in 31 of our five year NMO patient cohort (26 aquaporin-4 antibody positive, 3 anti-MOG positive and 2 seronegative).

Objective Intracranial epidermoids (cholesteatomas) mimic arachnoid cysts in their radiologic characteristics, especially in the cerebellopontine angle. It is essential to differentiate the two conditions because they warrant different therapeutic interventions. The objective of this study is to elucidate the different radiologic characteristics of the conditions. Study design and setting This was a retrospective study of 4 patients referred for a differential diagnosis and management of intracranial cystic lesions to the Departments of Neurotology/Neurosurgery and Neuroradiology in a tertiary referral university hospital. Patients Four patients of different age groups with cystic intracranial lesions, diagnosed epidermoid or arachnoid cysts, were chosen. A retrospective analysis of their case charts, radiologic and surgical interventions, and follow-up records was undertaken. Methods The imaging techniques used included computerized tomographic scans, magnetic resonance imaging (MRI) with T1, T2, proton-density, and gadolinium-enhanced T1 images. In addition, special MRI sequences were used that included fluid-attenuated inversion recovery and echo planar diffusion scanning. All the patients underwent an audiovestibular evaluation. Results Both lesions are characteristically well demarcated and have a homogeneous low density, similar to cerebrospinal fluid on computerized tomographic scan, showing no contrast enhancement. On MRI, epidermoids and arachnoid cysts usually appear hypointense on T1-weighted images and hyperintense on T2-weighted images. On fluid-attenuated inversion recovery, an arachnoid cyst tends to follow cerebrospinal fluid intensity, whereas an epidermoid becomes hyperintense. There are occasions when an epidermoid may appear as a low-intensity lesion on fluid-attenuated inversion recovery. This dilemma is resolved with the use of echo planar diffusion scanning, on which an epidermoid remains bright. Conclusion The authors recommend the use of fluid-attenuated inversion recovery and diffusion sequence MRI when definitive radiologic diagnosis of cystic intracranial lesions becomes difficult with routine computerized tomographic scanning and MRI.

The pituitary gland is best imaged with magnetic resonance imaging (MRI) that will delineate the normal gland and identify any areas of abnormality within the gland. Computed tomography (CT) scans can give additional information about the bony anatomy; however, it requires exposure to radiation and is generally reserved for patients not suitable for MRI, i.e., pacemaker. CT is also utilized for navigation planning in endoscopic sinus surgery.

Management of Idiopathic Intracranial Hypertension (IIH) is not standardised. A multidisciplinary Special Interest Group (SIG) was established to develop a uniform investigation and treatment strategy according to disease severity based on current literature and expert consensus. Methods A systematic literature review of “IIH”;“Benign IH” and “pseudotumour cerebri” was followed by a SIG meeting, to identify the population, interventions, controls and outcomes (PICO) questions. Discrepant views were reflected in a questionnaire disseminated though a modified Delphi approach to district general and tertiary hospital interdisciplinary specialists. Results 44/66 questionnaires were returned. 51% would image (CT/MRI) patients with definite papilloedema Conclusions Evidence for IIH management is minimal. Consensus on many aspects of management was demonstrated which will help establish a practical guideline.

We present the rare case of a 74 – year – old woman with extensive intramedullary osteolysis of the petroclival skull base straddling both sides of the basi – occiput and basi – sphenoid. She presented with clinical features of recurrent spontaneous cerebrospinal fluid (CSF) otorrhoea despite three previous attempts at repair of the CSF leak. Recent advances in imaging techniques enable accurate radiological diagnosis of skull base lesions. We performed T1- and T2-weighted magnetic resonance (MR) images, a fluid attenuated inversion recovery (FLAIR) sequence MR, and a diffusion scan to study the characteristics of the skull base pathology. This revealed extensive osteolysis, with cystic spaces within the clivus and the petrous pyramid extending also to the basi – occiput. The leak was sealed using the technique of subtotal petrosectomy with obliteration of the eustachian tube and blind pit closure of the ear canal. The patient has been followed up for six months with no recurrence of symptoms.

Background and aimsWe report a new syndrome (head jolting nystagmus, HJN) that expands the differential diagnosis of head-movement induced paroxysmal vertigo.Methods and resultsTwo male patients (65 and 58 yrs) described rotational vertigo after violent and brief (1–2 s) oscillations of the head (head jolting) that triggered intense horizontal nystagmus lasting 45s. Accelerations of the head required to induce these episodes could only be achieved by the patients themselves. In Patient 1 the episodes gradually disappeared over a 6–year period. In Patient 2, 3–Tesla MRI suggested a filling defect in the left horizontal semicircular canal. He underwent surgical canal plugging that resolved the symptoms.Conclusion and relevanceWe attribute HJN to dislodged material within the horizontal semicircular canal, and provide a mechanistic model to explain its origin.

The WEB is an endovascular flow-disrupting device used in treating wide-necked intracranial aneurysms. Although the device is available in varying sizes, large aneurysms pose a challenge with the need for custom-made devices. We describe the use of coils as an adjunct to the WEB device in successfully treating large aneurysms in two patients, one with an acutely ruptured aneurysm. This novel technique of jailing a microcatheter, deploying the WEB and then coiling the aneurysm saves the need for intracranial stenting, thereby avoiding the need for antiplatelet therapy, which is of benefit in the setting of acute aneurysm rupture.

Objectives: Following translabyrinthine craniotomy the temporal bone defect is commonly obliterated using a free autologous fat graft. In this series the dura was put back in place but not closed primarily. As the fat graft remains in direct contact with the cerebro spinal fluid (CSF) there is potential for dispersal of fat within the CSF space. This paper aims to determine the frequency of such CSF fat dissemination and its clinical significance. Design: A retrospective review of translabyrinthine acoustic neuroma removal with free fat autograft obliteration of the temporal bone defect between the years 1997 and 2000. Setting: Tertiary referral oto-neurosurgical centre. Postoperative magnetic resonance (MR) imaging. Participants: All translabyrinthine patients who had postoperative MR imaging were included. Twenty-six cases were identified. Age range was 13–70 years. Fourteen were male patients. Main outcome measures: Evidence of CSF fat dissemination on MR and patients’ clinical findings. Results: Twenty-two of the 26 scans (85%) demonstrated evidence of fat dissemination into the subarachnoid CSF spaces in the form of microemboli. The cerebellopontine angle was the most common site involved. No evidence of ventricular dilation or any other abnormality was noted. There was no relationship between the presence or extent of fat microembolization and the patients’ clinical course. Conclusions: This study suggests that free fat placed in temporal bone defects commonly migrate into the subarachnoid space and subsequently move around in these spaces. This is not associated with any complications such as hydrocephalus, meningitis or prolonged postoperative headache.

A prospective analysis of patients referred by Otolaryngologists from a tertiary hospital for detailed assessment of the posterior cranial fossa was undertaken. The objectives were to evaluate radiological characteristics of the anterior inferior cerebellar artery (AICA) within the cerebellopontine angle (CPA) and the internal auditory canal (IAC), and to correlate these characteristics with ipsilateral auditory symptoms. Three hundred and thirty-two consecutive adult patients who presented with unilateral auditory symptoms were studied. All patients were referred by the Department of Otolaryngology at City Hospital, Birmingham, from October 1999 to October 2001. Magnetic resonance imaging (MRI) with three-dimensional Fourier Transformation Constructive Interference in Steady State (3D FT-CISS) was the imaging strategy used to investigate each patient. Six hundred and sixty-four sides were studied and the AICA loop was identified in all patients. Using a simple anatomical classification to type the loops: there were 412 type I loops (within the CPA), 202 type II loops (at the porus acousticus, extending up to 50% of IAC) and 50 type III loops (extending beyond 50% of IAC). There was a statistically significant association with unilateral hearing loss and type II and III AICA loops (P = 0.016 and P = 0.006 respectively). An association between the presence of a large diameter vascular loop in the CPA and unilateral auditory symptoms was not found to be significant (P > 0.05).

Maffucci's syndrome is a rare congenital non-inherited condition, characterized by multiple enchondromata, cutaneous haemangiomata and more recently spindle-cell haemangioma-endotheliomata. It is associated with an increased risk of malignancy including intracranial chondrosarcomas. Early diagnosis is crucial; screening patients with Ollier's disease is recommended. The treatment of choice for these intracranial cartilaginous tumours is complete surgical excision but this is often difficult to achieve due to difficult access and relationships with neurovascular structures. An alternative treatment is proton-beam therapy. We report a case of Maffucci's syndrome; illustrating the typical clinical and radiological features as well as the known complications of the condition.

Facial nerve haemangioma is a rare benign neoplasm accounting for 0.7 per cent of all tumours involving the temporal bone. The diagnosis of a facial nerve tumour is often missed or delayed. Early diagnosis is imperative as it influences the eventual outcome for facial nerve function. Prognosis is related to the size of the tumour, the severity and the duration of pre-operative paralysis. The definitive diagnosis of a facial nerve tumour rests exclusively with high resolution imaging of the temporal bone using enhanced magnetic resonance imaging (MRI) and thin-sectioned computed tomography (CT). This case emphasizes the crucial role that high quality imaging can play in the diagnosis of facial nerve tumours, and elegantly illustrates the imaging features of facial nerve haemangiomas.