Search

Background: Gender-affirming hormone therapy is critical to the management of transgender persons. Cyproterone acetate (CPA) is a synthetic, progesterone-like compound commonly used in high doses a...

Extent of resection is a major determinant of outcomes following pituitary surgery. Intra-operative magnetic resonance imaging (iMRI), provides an immediate assessment of the extent of resection, allowing further tumour resection during the same procedure. However, such systems are expensive and significantly increase operative time, prompting some authors to question the additional benefit conferred by iMRI when combined with endoscopy. Our aim was to assess the impact of combining 3 T iMRI with endoscopy in patients with pituitary tumours.We retrospectively reviewed a prospectively maintained database to identify patients who underwent iMRI guided endoscopic resection of pituitary tumours between May 2017 and November 2018 (iMRI cohort). This cohort was compared with a pre-iMRI cohort of patients who underwent endoscopic resection of pituitary adenomas. Operative time, extent of resection, control of endocrine disease and post-operative complications were recorded and analysed.Thirty-seven patients were included in each cohort. iMRI facilitated additional tumour resection in 6/37 (16%) of cases. In 4/37 cases (11%), iMRI prompted a return to theatre but no further tumour could be identified. The overall GTR rate, following iMRI was 24/37 (65%) as compared to 21/37 (57%) in the pre-iMRI cohort. Cure of endocrine disease associated with hormonally active tumours was achieved in 9/11 (82%) of cases in the iMRI cohort. The mean operative time in the iMRI cohort was 327 minutes (five hours 27 minutes).3 T iMRI provides immediate identification of residual tumour following endoscopic pituitary surgery. This allows for resection of surgically accessible residual disease during the same procedure and is likely to reduce the requirement for later re-intervention. However, the use of iMRI in this setting is associated with significant resource allocation issues which must be considered prior to the widespread adoption of this technique.

Background Up to 60% of non-functioning pituitary adenomas stain for gonadotrophins on immunocytochemistry but do not secrete excess gonadotrophins. Functioning gonadotroph adenomas with clinical manifestations are extremely rare and majority of these are FSH secreting macroadenomas. Diagnosis may be difficult in pre-menopausal women with high oestrogen and variable FSH levels but in men, the unusually high gonadotrophins give the clues for diagnosis. Clinical Case We present the case of a 37-year-old Caucasian male with clinical manifestations of an FSH-secreting pituitary macroadenoma. He had sexual dysfunction with loss of libido for a year followed by bilateral testicular pain and enlargement (right >left). He was initially investigated under urology and had repeated treatment for suspected epididymitis. The diagnosis came to light many months later when he presented to emergency department with headaches and visual disturbance. Brain imaging confirmed a pituitary macroadenoma (3×2.5×3cm) with optic chiasm compression. He had bilateral superior temporal visual field defects. Pituitary profile showed unusually high FSH 200 IU/L (0.7-11.1), normal LH 7.6 IU/L (0.8-7.6) and a high normal testosterone 27 nmol/L (8.5-29). Prolactin was slightly elevated at 474mIU/L (0-350), cortisol and TSH were in normal range. He had polycythaemia (Haemoglobin 181g/l and HCT 0.51 L/L) and old blood results indicate his haemoglobin was above the upper limit of normal for previous 10 years. This gentleman had a successful transsphenoidal hypophysectomy with near complete resection of pituitary adenoma. His vison improved immediately after surgery. Histology of the resected adenoma showed gonadotroph differentiation and immunoreactivity predominantly with FSH and also with LH and prolactin stains. A day after surgery, his FSH levels dropped to 18 IU/L and LH was undetectable. Symptoms of hypogonadism had gradually worsened post-surgery and 2 months later, his pituitary profile showed severe hypogonadotrophic hypogonadism (FSH 3.6 IU/L, undetectable LH and testosterone 0.9 nmol/L). He was started on testosterone replacement which he continues till date. He remains asymptomatic with no testicular pain and the last MR imaging, a year after his surgery showed no recurrence in the pituitary adenoma. Conclusion Testicular enlargement and hypogonadal symptoms with low testosterone levels are recognised features in FSH secreting pituitary adenomas. Our patient had testicular enlargement but consistently high testosterone levels prior to surgery and polycythaemia for many years prior to presentation. This may be due concomitant hypersecretion of both FSH and LH from his pituitary adenoma. The reason for low libido despite high androgen levels was not entirely clear. Symptoms of hypogonadism overlap with many non-gonadal illnesses. A normal testosterone level in most cases would not lead to further endocrine investigations. Our case highlights the need to suspect such rare underlying pituitary pathology when dealing with unusual combination of hypogonadal symptoms, testicular enlargement and normal or high normal testosterone levels. Presentation: Saturday, June 11, 2022 1:30 p.m. - 1:35 p.m., Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Introduction Giant invasive prolactinomas are rare pituitary tumours and have a male preponderance of 9: 1. In majority of cases, dopamine agonists (DA) are the treatment of choice in lowering prolactin and tumour shrinkage. Surgery may be opted in those with acute compressive symptoms or visual loss. Prolactinomas are known to invade the sellar floor, sphenoid sinus and clivus. Spontaneous CSF leak is rare in untreated patients with invasive prolactinomas compared to those on DA who respond with rapid tumour shrinkage that causes unplugging of the conduits resulting in CSF rhinorrhoea. We present 5 male patients with untreated giant invasive prolactinomas with skull base destruction where close surveillance was opted over DA treatment. Results Five male patients with median age of 73±10 years were studied. 1 patient presented with headache, vomiting and 6th nerve palsy which spontaneously resolved over weeks. 2 patients were diagnosed during work up of other hormonal deficiencies and in 2 patients giant tumours were incidentally found on imaging for head injury. Prolactin values ranged from 55,641 to 835,800 mIU/L. Hormonal deficiency was present in 3/5; anterior hypopituitarism (1) and symptomatic secondary hypogonadism (2). MRI imaging in all patients showed extensive skull base bony erosion, with tumour invasion into sphenoid sinus and clivus. Additionally, the patient with largest tumour had invasion into right orbital roof and floor of right anterior cranial fossa. In other patients, tumour had also invaded occipital condyles (1), bilateral cavernous sinus (1). All patients were discussed in tertiary neurosurgical MDT. As the risk of CSF leakage due to tumour shrinkage outweighed the benefits of tumour reduction, a decision not for medical treatment was agreed with patients and planned for active surveillance with MRI scans and regular clinical and visual fields assessments. The mean follow-up period was 4±1 years. One patient was very frail and decided not for radiological surveillance. DA treatment is being considered for the patient with the largest tumour which has grown further causing frontal lobe invasion. There was no significant tumour size increase in other 4 patients and none had spontaneous CSF leak. Discussion Risk stratification for CSF leak with DA treatment is difficult as it depends on the invasion, tumour response to DAs and the extent of underlying bony destruction. This may cause a dilemma on whether or not to treat some patients with DAs. The risk of CSF leak, bacterial meningitis and the subsequent need for urgent surgical repair may outweigh the benefits of tumour reduction with DAs particularly in some patients with low symptom burden. In our experience, patient counselling, active radiological surveillance and considering treatment with change in symptoms (visual deterioration, compressive pathology) could be an appropriate management option in such patients. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Background During the coronavirus disease 2019 (COVID-19) pandemic, concerns have been raised regarding the increased risk of perioperative mortality for patients with COVID-19, and the transmission risk to healthcare workers, especially during endonasal neurosurgical operations. The Pituitary Society has produced recommendations to guide management during this era. We sought to assess contemporary neurosurgical practice and the effects of COVID-19. Methods A multicenter prospective observational cohort study was conducted at 12 tertiary neurosurgical units (United Kingdom and Ireland). Data were collected from March 23 to July 31, 2020, inclusive. The data points collected included patient demographics, preoperative COVID-19 test results, operative modifications, and 30-day COVID-19 infection rates. Results A total of 124 patients were included. Of the 124 patients, 116 (94%) had undergone COVID-19 testing preoperatively (transsphenoidal approach, 97 of 105 [92%]; expanded endoscopic endonasal approach, 19 of 19 [100%]). One patient (1 of 116 [0.9%]) had tested positive for COVID-19 preoperatively, requiring a delay in surgery until the infection had been confirmed as resolved. Other than transient diabetes insipidus, no other complications were reported for this patient. All operating room staff had worn at least level 2 personal protective equipment. Adaptations to surgical techniques included minimizing drilling, draping modifications, and the use of a nasal iodine wash. At 30 days postoperatively, no evidence of COVID-19 infection (symptoms or positive formal testing results) were found in our cohort and no mortality had occurred. Conclusions Preoperative screening protocols and operative modifications have facilitated endonasal neurosurgery during the COVID-19 pandemic, with the Pituitary Society guidelines followed for most of these operations. We found no evidence of COVID-19 infection in our cohort and no mortality, supporting the use of risk mitigation strategies to continue endonasal neurosurgery in subsequent pandemic waves.

Background CRANIAL (CSF Rhinorrhoea After Endonasal Intervention to the Skull Base) is a prospective multicenter observational study seeking to determine 1) the scope of skull base repair methods used and 2) corresponding rates of postoperative cerebrospinal fluid (CSF) rhinorrhea in the endonasal transsphenoidal approach (TSA) and the expanded endonasal approach (EEA) for skull base tumors. We sought to pilot the project, assessing the feasibility and acceptability by gathering preliminary data. Methods A prospective observational cohort study was piloted at 12 tertiary neurosurgical units in the United Kingdom. Feedback regarding project positives and challenges were qualitatively analyzed. Results A total of 187 cases were included: 159 TSA (85%) and 28 EEA (15%). The most common diseases included pituitary adenomas (n = 142/187), craniopharyngiomas (n = 13/187). and skull base meningiomas (n = 4/187). The most common skull base repair techniques used were tissue glues (n = 132/187, most commonly Tisseel), grafts (n = 94/187, most commonly fat autograft or Spongostan) and vascularized flaps (n = 51/187, most commonly nasoseptal). These repairs were most frequently supported by nasal packs (n = 125/187) and lumbar drains (n = 20/187). Biochemically confirmed CSF rhinorrhea occurred in 6/159 patients undergoing TSA (3.8%) and 2/28 patients undergoing EEA (7.1%). Four patients undergoing TSA (2.5%) and 2 patients undergoing EEA (7.1%) required operative management for CSF rhinorrhea (CSF diversion or direct repair). Qualitative feedback was largely positive (themes included user-friendly and efficient data collection and strong support from senior team members), demonstrating acceptability. Conclusions Our pilot experience highlights the acceptability and feasibility of CRANIAL. There is a precedent for multicenter dissemination of this project, to establish a benchmark of contemporary practice in skull base neurosurgery, particularly with respect to patients undergoing EEA.

Background: The endonasal transsphenoidal approach (TSA) has emerged as the preferred approach in order to treat pituitary adenoma and related sellar pathologies. The recently adopted expanded endonasal approach (EEA) has improved access to the ventral skull base whilst retaining the principles of minimally invasive surgery. Despite the advantages these approaches offer, cerebrospinal fluid (CSF) rhinorrhoea remains a common complication. There is currently a lack of comparative evidence to guide the best choice of skull base reconstruction, resulting in considerable heterogeneity of current practice. This study aims to determine: (1) the scope of the methods of skull base repair; and (2) the corresponding rates of postoperative CSF rhinorrhoea in contemporary neurosurgical practice in the UK and Ireland. Methods: We will adopt a multicentre, prospective, observational cohort design. All neurosurgical units in the UK and Ireland performing the relevant surgeries (TSA and EEA) will be eligible to participate. Eligible cases will be prospectively recruited over 6 months with 6 months of postoperative follow-up. Data points collected will include: demographics, tumour characteristics, operative data), and postoperative outcomes. Primary outcomes include skull base repair technique and CSF rhinorrhoea (biochemically confirmed and/or requiring intervention) rates. Pooled data will be analysed using descriptive statistics. All skull base repair methods used and CSF leak rates for TSA and EEA will be compared against rates listed in the literature. Ethics and dissemination: Formal institutional ethical board review was not required owing to the nature of the study–this was confirmed with the Health Research Authority, UK. Conclusions: The need for this multicentre, prospective, observational study is highlighted by the relative paucity of literature and the resultant lack of consensus on the topic. It is hoped that the results will give insight into contemporary practice in the UK and Ireland and will inform future studies.

Background With the increased rate of childhood cancer survivors, a marked rise in the incidence of secondary intracranial meningiomas has been established. Objective Determine the outcomes of patients diagnosed with radiation-induced meningiomas (RIM). Methods Single-centre retrospective cohort study of patients with new or recurrent RIM (2007–2018). Results 47 patients were identified (21 females). Mean age at radiation was 15 years (SD=14) and the most common indications were leukaemia (n=8) and childhood brain tumours (n=35; medulloblastoma and pilocytic astrocytoma (n=7 each)). 93 de-novo meningiomas were identified. The median latency period between radiation and diagnosis was 28.5 years (IQR 22–37). 61% were asymptomatic whilst the remainder manifested headache (20%), focal neurological deficit (12%) and epilepsy (5%). 32 operated de-novo RIM revealed WHO grade I (n=19; 59%), WHO grade II (n=11; 34%) and 2 intraosseous meningiomas (n=2; 6%). After a median of 5 years (IQR 3–10), 9 (28%) operated RIM recurred/progressed. At recurrence, 5 were operated (3 WHO grade I, 2 WHO grade II and 1 WHO grade III), 2 patients were palliated and 2 remain under active observation. By the end of the study period, 20 patients were harbouring multiple meningiomas and 8 patients were deceased. Conclusion Radiation-induced meningioma should be monitored until symptomatic. Operated RIMs have a high recurrence rate. Further radiotherapy is not effective. Further clinical and genetic analyses as part of an international multi-centre collaboration are planned.

There is a paucity of data describing long-term outcomes of paediatric patients with pituitary adenoma. In this report, we describe clinical features, treatment and outcomes of a paediatric cohort.Retrospective cohort study.Twenty-four white Caucasian patients aged16 years from a single tertiary care centre in the United Kingdom at diagnosis followed for (median, range) 3.3, 0.7-8.4 years.Clinical and radiological data at diagnosis and follow-up.Thirteen patients had prolactinomas (54.1%, age: 15.2 years, 13.2-15.8 years; all females), including ten macroadenomas (11.0-35.0 mm). Patients presented with menstrual disorders (91%), headache (46%), galactorrhoea (46%) and obesity (body mass index [BMI] SDS 2): (38%). Ten patients with prolactinoma were treated with dopamine agonist alone, 3 also required surgery and 2 patients, cabergoline, surgery plus radiotherapy. Five patients had Cushing's disease (20.8%, age: 14.0, 4.0-15.7 years; 2 female), including one macroadenoma (24 mm). Patients presented with obesity (100%), short stature (60%) and headache (40%). Transsphenoidal resection resulted in biochemical cure (09.00 cortisol 50 nmol/L). Two patients relapsed 3- and 6 years following surgery, requiring radiotherapy. One patient also required bilateral adrenalectomy. Six patients had nonfunctioning pituitary adenoma (25.0%, age: 15.8, 12.5-16.0 years; 2 female), including two macroadenomas (20.0-53.0 mm). Patients presented with obesity (67%), visual field defects (50%) and headache (50%). Four required surgical resections; two recurred following surgery and required radiotherapy. On latest follow-up; 13 (54.1%) patients were obese (BMI 3.09 SDS; range: 2.05-3.73 SDS).Obesity is common at diagnosis of pituitary adenoma in childhood and may persist despite successful treatment. Adenomas were larger, more resistant to treatment, and more likely to recur than in adult populations.

We present a 69-year-old transgender woman who underwent gender-affirming surgery in 1998 and gender-affirming hormone therapy (cyproterone acetate (CPA) and estradiol) since this time. Following an MRI scan to investigate tremor in 2013, an incidental left anterior clinoid and right petrous meningioma were identified. Subtotal surgical resection was achieved for the anterior clinoid meningioma (WHO grade 1, meningothelial subtype). At follow-up in 2016, an olfactory groove meningioma and left greater wing of sphenoid meningioma were identified. By 2017, both tumours, along with the petrous meningioma, demonstrated significant growth. In 2018, clinical decline was evident and MRI demonstrated further tumour growth. Surgery was scheduled and the olfactory groove meningioma was completely resected (WHO grade 2, chordoid subtype). Hormones were stopped, after which regression of the petrous meningioma was observed. This case demonstrates an association between high-dose CPA and estradiol and the development, growth and regression of meningiomas in a transgender woman.

Objectives To analyze growth of residual vestibular schwannoma (VS) following incomplete tumor resection and determine the influence of residual location and size. Design Retrospective case note and scan review. Setting Tertiary skull base unit. Participants Patients with residual tumor following primary surgery for medium and large unilateral growing vestibular schwanomas between 2006 and 2009. Main Outcome Measures Location of residual VS and post-operative growth, comparing those with more (>5%) or less than 5% of tumor residual ( Results Fifty-two patients had visible residual tumor left behind at surgery. Twenty had 5% residual. The residual growth rates were 38% overall, 20% in 5% residuals. There was no significant difference in growth rates at different residual locations. Median follow-up was 6.4 years. Conclusions There is a greater risk of regrowth of residuals > 5%. All positions of residual tumor can regrow, and the preoperative tumor size plays a role in this. Further data is needed to confirm if residual tumor in the fundus is less likely to grow.

It is widely accepted that stereotactic radiosurgery (SRS) is less effective in controlling NF2-related vestibular schwannomas (VS-NF2), than sporadic VS. Concerns persist regarding the risks of malignant transformation and the development of secondary tumours. In recent years there has been an increase in the number of centres offering SRS and fractionated stereotactic radiotherapy (SRT) services for VS. The NF2 National Commissioning Group service framework has recommended limiting radiation therapies for VS-NF2 to SRS (Gamma Knife) provided in Sheffield. The aims of this review were to: 1) Establish the current provision of SRT and SRS services in England. 2) Determine the radiation therapies employed for NF2 patients since 2000. 3) Determine the feasibility of identifying a national cohort of NF2 patients in England who have received radiation therapy since 2000.The lead clinicians managing VS patients in the major regional neurosurgical units in England, and those providing SRS services to NF2 patients were contacted and when possible, databases were examined.A total of 18 NHS centres and 2 private centres were included. This included the four NF2 'hub centres' (Cambridge, London, Oxford and Manchester). Their NF2 databases identified 4, 8, 23 and 42 VS-NF2 treatments with SRS or SRT, respectively since 2000. Eleven centres reported that they had referred VS-NF2 patients exclusively to Sheffield. Each estimated that they had referred no more than one patient per year. The survey identified four Gamma Knife Centres and six Linac SRS/SRT centres with the capacity to treat VS and VS-NF2 patients. Of these, four centres confirmed that they had treated VS-NF2 patients.Since 2000 fewer than 100 SRS and SRT treatments have been undertaken for VS-NF2 patients. Approximately 60% of these have been performed in Sheffield. There is considerable uncertainty regarding the role of radiation therapy in the management of VS-NF2 and consequently a range of views and practice.

Non-traumatic cerebrospinal fluid (CSF) rhinorrhoea is a rare condition. We describe a case of a 62-year-old woman with pneumococcal bacterial meningitis who later was found to have CSF rhinorrhoea secondary to an eroding skull base tumour, which was proven to be pituitary macroadenoma on biopsy. She recovered well from meningitis without any neurological sequelae and underwent trans-sphenoidal surgery for tumour removal as well as dural repair.

DIANA BECK (1902-1956) WAS probably the first female neurosurgeon in the world and played a large part in establishing neurosurgery in the United Kingdom. She served as the consultant advisor in neurosurgery for the Emergency Medical Service in the southwest region during the Second World War and subsequently set up the neurosurgical unit at Frenchay Hospital in Bristol before becoming a consultant at The Middlesex Hospital in London in 1947. There, she was the first woman to be given charge of men in a consultant capacity in a major London teaching hospital. During her 31-year career, she was the only female neurosurgeon in Western Europe and the United States. Miss Beck came to the attention of the general public when she operated on Alan Alexander Milne in 1952. However, she was better known in neurosurgical circles for her interest in intracranial hemorrhage. In this article, some of her original research, case reports, and her seminal paper on the surgical treatment of intracerebral hemorrhage are presented. They are a stark reminder of the limited investigations available at that time. In addition to her neurosurgical achievements, Miss Beck was a remarkable character and teacher. Her Saturday ward rounds proved so popular that they competed successfully with the students' weekend plans. In a specialty in which women continue to represent a disproportionately small percentage of the workforce, Miss Beck was one of our earliest role models.

Two or three-column neoplastic spinal disease requiring circumferential decompression and instrumented stabilization is commonly treated through combined anterior transcavitary and posterior surgical approaches. An alternative approach advocated in the literature is costotransversectomy and interbody cage insertion. The authors present an effective and less invasive treatment paradigm using a single-stage posterior transpedicular approach (TPA) to circumferential thoracic decompression and fixation, avoiding the morbidity of thoracic or thoraco-abdominal access based on a series of eight patients with upper thoracic neoplastic disease.

Impulse control disorders (ICDs) constitute socially disruptive behaviors such as pathological gambling, impulsive eating, compulsive shopping, and hypersexuality. These conditions are well recognized in patients on dopamine agonist (DA) therapy for Parkinson disease. Dopamine agonists are widely used as first-line agents in the treatment of prolactinomas, but ICDs in this group of patients are relatively rare, perhaps because of lower therapeutic doses used. A review of the literature yielded only a few cases of ICDs in patients on DA treatment for prolactinomas. These symptoms are perhaps underreported because of lack of awareness among patients and health care professionals. Impulse control disorders are recognized psychiatric disorders that have significant psychological and social implications, and patients need to be counselled about this rare possibility when embarking on prolonged DA therapy. We describe a young patient with severe, socially disruptive impulsivity manifesting with pathological gambling who had been on long-term bromocriptine therapy for a macroprolactinoma.

SummaryObjective To study the presentation, management and outcomes and to apply retrospectively the Pituitary Apoplexy Score (PAS) (United Kingdom (UK) guidelines for management of apoplexy) to a large, single-centre series of patients with acute pituitary apoplexy. Design Retrospective analysis of casenotes at a single neurosurgical centre in Liverpool, UK. Results Fifty-five patients [mean age, 52·4 years; median duration of follow-up, 7 years] were identified; 45 of 55 (81%) had nonfunctioning adenomas, four acromegaly and six prolactinomas. Commonest presenting features were acute headache (87%), diplopia (47·2%) and visual field (VF) defects (36%). The most frequent ocular palsy involved the 3rd nerve (81%), followed by 6th nerve (34·6%) and multiple palsies (19%). Twenty-three patients were treated conservatively, and the rest had surgery either within 7 days of presentation or delayed elective surgery. Indications for surgery were deteriorating visual acuity and persistent field defects. Patients presenting with VF defects (n = 20) were more likely to undergo surgery (75%) than to be managed expectantly (25%). There was no difference in the rates of complete/near-complete resolution of VF deficits and cranial nerve palsies between those treated conservatively and those who underwent surgery. Endocrine outcomes were also similar. We were able to calculate the PAS for 46 patients: for the group treated with early surgery mean, PAS was 3·8 and for those managed conservatively or with delayed surgery was 1·8. Conclusions Patients without VF deficits or whose visual deficits are stable or improving can be managed expectantly without negative impact on outcomes. Clinical severity based on a PAS ≥ 4 appeared to influence management towards emergency surgical intervention.

To determine whether preliminary evidence supports X-STOP implants as an effective treatment for lumbar radiculopathy secondary to foraminal stenosis, and if larger formal trials are warranted.Participants had a clinical diagnosis of lumbar radiculopathy supported by MRI findings of foraminal stenosis and relevant nerve root compression. Self-reported disability and pain were measured pre-operation, early and late post-operation using the widely used Oswestry Disablity Index (ODI) and the bodily pain scale of the Medical Outcomes Study 36-item Short Form Health Survey (SF-36BP). The statistical significance (paired samples t test; Wilcoxon signed ranks test), and clinical significance (Cohen's effect size; Standardised response means) of change scores was determined.Fifteen people had X-STOP implants. Data pre- early- and late post-operation were available for ten. Self-reported disability and pain improved substantially by the early post operative measurement. Mean change scores (ODI = 29; SF-36BP = -45), significant at the p0.05 but not significant at the p0.001, were very large and effect sizes exceeded notably criteria for large clinical improvements (0.80). Improvements were maintained at 2-3 years. Both scales had floor and ceiling effects implying changes may be underestimated. There were no surgical complications.In this small study, X-STOP appeared safe and effective. It is less invasive than other established surgical procedures, but does not jeopardise other options in the event of failure. Large scale clinical trials are justified but floor and ceiling effects suggest that the ODI and SF-36 may not be the best choice of outcome measures for those studies.

ObjectiveObesity is highly prevalent among adults with acquired, structural hypothalamic damage. We aimed to determine hormonal and neuroanatomical variables associated with weight gain and obesity in patients following hypothalamic damage and to evaluate the impact of early instigation of weight loss measures to prevent or limit the severity of obesity in these patients.DesignRetrospective study of 110 adults with hypothalamic tumours attending a specialist neuroendocrine clinic. BMI was calculated at diagnosis and at last follow-up clinic visit. Endocrine data, procedures, treatments and weight loss measures were recorded and all available brain imaging reviewed.ResultsAt last follow-up, 82.7% of patients were overweight or heavier (BMI≥25 kg/m2), 57.2% were obese (BMI≥30 kg/m2) and 14.5% were morbidly obese (BMI≥40 kg/m2). Multivariate analysis revealed that use of desmopressin (odds ratio (OR)=3.5;P=0.026), GH (OR=2.7;P=0.031) and thyroxine (OR=3.0;P=0.03) was associated with development of new or worsened obesity. Neuroimaging features were not associated with weight gain. Despite proactive treatments offered in clinic in recent years (counselling, dietetic and physical activity advice, and anti-obesity medications), patients have continued to gain weight.ConclusionsDespite increased awareness, hypothalamic obesity is difficult to prevent and to treat. Improved understanding of the underlying pathophysiologies and multicentre collaboration to examine efficacy of novel obesity interventions are warranted.

Brain biopsy is well established in clinical practice when there is suspicion of CNS malignancy. However, there is little consensus regarding the indications for brain biopsy in non-malignant neurological disease. This is due in no small part to limitations in the available literature pertaining to diagnostic brain biopsies. The published evidence largely comprises small, retrospective, single-centre analyses performed over long time periods, including non-homogeneous patient groups with considerable variation in reported outcomes. Here we present pragmatic guidance for those clinicians considering diagnostic brain biopsy in a patient with non-neoplastic neurological disease and highlight practice points with the aim of maximising the probability of gaining clinically useful information from the procedure.

Brain biopsy is standard clinical practice when CNS malignancy is suspected. Its value has not otherwise been clearly established. We reviewed the indications for, complications and outcome of diagnostic brain biopsies performed between 2003 and 2008 in a single UK centre.Subjects were retrospectively identified using theatre log books and histopathology reports. Case records were analysed by a neurologist and neurosurgeon. Cases were excluded when the pre-operative diagnosis was clearly malignancy or infection.Of all (432) brain biopsies performed, 56 were performed in 52 patients with cryptogenic neurological disease. There were no permanent deficits or deaths. Histopathological reports were classified as definitive (45%), suggestive (20%) or non-diagnostic (36%). Brain biopsy made an immediate contribution to determination of diagnosis in 55% (31 of 56) and a confident diagnosis was eventually made in 40 of 52 patients (77%). Management was altered as a consequence of biopsy in 63%. Successful biopsy of a radiologically identified target increased the proportion of biopsies considered diagnostic to 78% (odds ratio 8.9) whereas non-targeted biopsy was non-diagnostic in 71%. Although a significant proportion of patients died or had progressive disease, this was not uniformly the case; 31% stabilised and 27% improved.We present the highest reported frequency of brain biopsy for cryptogenic neurological disease. The risk associated with the procedure was low and the biopsy results impacted significantly upon diagnosis and management. We therefore propose that the procedure should no longer be considered one of last resort.