Role of the repeat expansion size in predicting age of onset and severity in RFC1 disease.

RFC1 disease, caused by biallelic repeat expansion in RFC1, is clinically heterogeneous in terms of age of onset, disease progression and phenotype. We investigated the role of the repeat size in influencing clinical variables in RFC1 disease. We also assessed the presence and role of meiotic and somatic instability of the repeat. In this study, we identified 553 patients carrying biallelic RFC1 expansions and measured the repeat expansion size in 392 cases. Pearson's coefficient was calculated to assess the correlation between the repeat size and age at disease onset. A Cox model with robust cluster standard errors was adopted to describe the effect of repeat size on age at disease onset, on age at onset of each individual symptoms, and on disease progression. A quasi-Poisson regression model was used to analyse the relationship between phenotype and repeat size. We performed multivariate linear regression to assess the association of the repeat size with the degree of cerebellar atrophy. Meiotic stability was assessed by Southern blotting on first-degree relatives of 27 probands. Finally, somatic instability was investigated by optical genome mapping on cerebellar and frontal cortex and unaffected peripheral tissue from four post-mortem cases. A larger repeat size of both smaller and larger allele was associated with an earlier age at neurological onset [smaller allele hazard ratio (HR) = 2.06, P < 0.001; larger allele HR = 1.53, P < 0.001] and with a higher hazard of developing disabling symptoms, such as dysarthria or dysphagia (smaller allele HR = 3.40, P < 0.001; larger allele HR = 1.71, P = 0.002) or loss of independent walking (smaller allele HR = 2.78, P < 0.001; larger allele HR = 1.60; P < 0.001) earlier in disease course. Patients with more complex phenotypes carried larger expansions [smaller allele: complex neuropathy rate ratio (RR) = 1.30, P = 0.003; cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) RR = 1.34, P < 0.001; larger allele: complex neuropathy RR = 1.33, P = 0.008; CANVAS RR = 1.31, P = 0.009]. Furthermore, larger repeat expansions in the smaller allele were associated with more pronounced cerebellar vermis atrophy (lobules I-V ß = -1.06, P < 0.001; lobules VI-VII ß = -0.34, P = 0.005). The repeat did not show significant instability during vertical transmission and across different tissues and brain regions. RFC1 repeat size, particularly of the smaller allele, is one of the determinants of variability in RFC1 disease and represents a key prognostic factor to predict disease onset, phenotype and severity. Assessing the repeat size is warranted as part of the diagnostic test for RFC1 expansion.

Initial Experience With 6D Skull Tracking and Intrafractional Motion Monitoring in the United Arab Emirates' First CyberKnife® Radiosurgery Center.

Introduction The introduction of the CyberKnife® system has marked a significant advancement in the field of radiosurgery, offering unparalleled precision in targeting and treating cranial and extracranial lesions. This paper details the first experience from the United Arab Emirates in implementing 6D skull tracking and intrafractional motion monitoring in CyberKnife® radiosurgery. The study aims to evaluate the system's efficacy in tracking and adjusting patient movement during treatment, enhancing treatment accuracy and patient safety. Methods and materials This retrospective study analyzed 732 images from six patients treated at the UAE's first CyberKnife® center. Patients were divided into two groups based on their treatment regimens: Patients 1 to 4 (P1 to P4) received multifractionated stereotactic radiotherapy, while Patients 5 and 6 (P5 and P6) underwent single-fraction stereotactic radiosurgery (SRS). The movements recorded included supero-inferior, lateral, antero-posterior, roll, pitch, and yaw. Statistical tools were employed to interpret the data, including heat maps, box-and-whisker plots, and correlation analysis. Results The study's results indicate varied patterns of intrafractional movement across the different axes and between the two treatment groups. Multifractionated therapy patients exhibited a specific range and frequency of movements compared to those undergoing single-fraction treatment. The most significant movements were observed in the supero-inferior and lateral axes. Discussion The findings suggest that the CyberKnife® system's real-time tracking and adaptive capabilities are crucial in managing patient movements, especially in prolonged treatment sessions. The differences in movement patterns between multifractionated and single-fraction treatments underscore the need for tailored approaches in intrafractional motion monitoring. Conclusion The initial experience of the UAE's first CyberKnife® center demonstrates the system's effectiveness in addressing intrafractional movements, enhancing the precision and safety of radiosurgery treatments. This study contributes valuable insights into optimizing treatment protocols and underscores the importance of continuous monitoring and adaptive strategies in advanced radiosurgery.

The impact of headache disorders: a prospective analysis of headache referrals to outpatient and inpatient neurology and emergency services in an Irish University teaching hospital.

BACKGROUND: Headache represents a significant proportion of disability globally in general practice, neurology outpatient settings, and emergency departments. There is scant literature regarding the impact of headache on healthcare services in Ireland. AIMS: We aimed to investigate headache burden across the emergency department, inpatient stays, and neurology outpatient department referrals in an Irish University teaching hospital. METHODS: We prospectively collected data regarding emergency department presentations, inpatient neurology consultations, and neurology outpatient referrals for patients with headache between 13th January and 8th March 2020. Data were analyzed using descriptive statistics. RESULTS: There were 180 emergency department attendances, 50 inpatient consultations, and 76 outpatient referrals with headache. Neurological examinations were often incomplete; neuroimaging was commonly employed. Migraine was the most frequent headache diagnosis at discharge in the emergency department and among inpatients after neurology review. Diagnostic uncertainty was identified-33% of patients left the emergency department with no diagnosis, and "unknown/unspecified headache" was recorded on 49% of outpatient referrals and 30% of inpatient consult requests. Medication overuse headache coexisted with migraine in nine patients in the inpatient group. Prophylaxis had been trialed in 56% of patients with migraine referred to outpatients. CONCLUSIONS: Primary headache disorders have a large impact on hospital services. Diagnostic uncertainty is common; neuroimaging is relied upon. Appropriate care pathways, education, and resource allocation should be prioritized.

Late-onset Tay-Sachs disease presenting with a neuromuscular phenotype-a case series.

BACKGROUND AND PURPOSE: Tay-Sachs disease is a rare and often fatal, autosomal recessive, lysosomal storage disease. Deficiency in ß-hexosaminidase leads to accumulation of GM2 ganglioside resulting in neuronal swelling and degeneration. Typical onset is in infancy with developmental regression and early death. Late-onset Tay-Sachs disease (LOTS) is extremely rare, especially in the non-Ashkenazi Jewish population, and is characterized by a more indolent presentation typically encompassing features of cerebellar and anterior horn cell dysfunction in addition to extrapyramidal and neuropsychiatric symptoms. CASES: A case series of four unrelated patients of non-Ashkenazi Jewish origin with a predominantly, and in some cases pure, neuromuscular phenotype with evidence of a motor neuronopathy on electromyography is presented. Cerebellar atrophy, reported to be a ubiquitous feature in LOTS, was absent in all patients. CONCLUSION: This case series provides evidence to support a pure neuromuscular phenotype in LOTS, which should be considered in the differential diagnosis of anterior horn cell disorders.

Health-Related Quality of Life in Patients with Inherited Ataxia in Ireland.

Inherited cerebellar ataxias (CA) are heterogeneous progressive neurological conditions associated with significant functional limitations. This study aimed to assess the implications of inherited CA on patients' self-reported quality of life (QoL) and impairments in work and activities. 129 individuals with ataxia responded to a survey focused on QoL. Health-related QoL was measured using the RAND 36-Item Short Form Survey. An adaptation of the validated Work Productivity and Activity Impairment questionnaire was used to assess the effect of health on work productivity and ability to perform activities over the past week. Nine percent of respondents were currently employed. Individuals with inherited ataxia experienced significant activity impairment, and 75% required professional or informal care. Health-related quality of life (HRQoL) was significantly worse in all areas for the individuals with inherited ataxia compared with Irish population normative values. Participants with Friedreich's ataxia (n = 56) demonstrated worse physical functioning then those with undetermined ataxia (n = 55). Female gender, younger age at symptom onset, current employment, retirement due to age or ataxia, and living in a long-term care facility were associated with higher sub-scores in different domains of HRQoL, while disease duration correlated with worse physical functioning sub-scores. This study is the first cross-sectional study on HRQoL in patients with inherited ataxia in Ireland. It highlights high rates of unemployment, difficulty with daily activities and physical functioning limitations, which is worse than comparative international studies. Given the limited therapeutic options currently available, optimising HRQoL is an important aspect of managing ataxia.

Spatiotemporal Trends Spanning Three Decades Show Toxic Levels of Chemical Contaminants in Marine Mammals.

Despite their ban and restriction under the 2001 Stockholm Convention, persistent organic pollutants (POPs) are still widespread and pervasive in the environment. Releases of these toxic and bioaccumulative chemicals are ongoing, and their contribution to population declines of marine mammals is of global concern. To safeguard their survival, it is of paramount importance to understand the effectiveness of mitigation measures. Using one of the world's largest marine mammals strandings data sets, we combine published and unpublished data to examine pollutant concentrations in 11 species that stranded along the coast of Great Britain to quantify spatiotemporal trends over three decades and identify species and regions where pollutants pose the greatest threat. We find that although levels of pollutants have decreased overall, there is significant spatial and taxonomic heterogeneity such that pollutants remain a threat to biodiversity in several species and regions. Of individuals sampled within the most recent five years (2014-2018), 48% of individuals exhibited a concentration known to exceed toxic thresholds. Notably, pollutant concentrations are highest in long-lived, apex odontocetes (e.g., killer whales (Orcinus orca), bottlenose dolphins (Tursiops truncatus), and white-beaked dolphins (Lagenorhynchus albirostris)) and were significantly higher in animals that stranded on more industrialized coastlines. At the present concentrations, POPs are likely to be significantly impacting marine mammal health. We conclude that more effective international elimination and mitigation strategies are urgently needed to address this critical issue for the global ocean health.

Reproductive biology of male common dolphins (Delphinus delphis) in New Zealand waters.

Reproductive parameters were assessed in 64 male common dolphins (Delphinus delphis) examined post-mortem from strandings and bycatch in New Zealand between 1999 and 2020. The stages of male sexual maturation were assessed using morphological measurements and histological examination of testicular tissue. Age was determined via growth layer groups (GLGs) in teeth. The average age (ASM) and length (LSM) at attainment of sexual maturity were estimated to be 8.8 years and 198.3 cm, respectively. Individual variation in ASM (7.5-10 years) and LSM (190-220 cm) was observed in New Zealand common dolphins. However, on average, sexual maturity was attained at a similar length but at a marginally younger age (< 1 year) in New Zealand compared to populations in the Northern Hemisphere. All testicular variables proved better predictors of sexual maturity compared to demographic variables (age and total body length), with combined testes weight the best outright predictor of sexual maturity. Reproductive seasonality was observed in male common dolphins, with a significant increase in combined testes weight in austral summer. This aligns with most other studied populations, where seasonality in reproduction is typically observed. Given the known anthropogenic impacts on New Zealand common dolphins, we recommend that these findings be used as a baseline from which to monitor population-level changes as part of conservation management efforts. Supplementary Information: The online version contains supplementary material available at 10.1007/s00227-023-04266-5.

DNMT1-associated sensory neuropathy and cerebellar ataxia: A novel variant and review of genotype-phenotype correlation.

AIM: Hereditary sensory neuropathy (HSN) 1E is a neurodegenerative disorder caused by pathogenic variants in DNA methyltransferase 1 (DNMT1). It is characterised by sensorineural deafness, sensory neuropathy and cognitive decline. Variants in DNMT1 are also associated with autosomal dominant cerebellar ataxia, deafness and narcolepsy. METHODS: A 42-year-old man presented with imbalance, lancinating pain, numerous paucisymptomatic injuries, progressive deafness since his mid-20s, mild cognitive decline and apathy. Examination revealed abnormalities of eye movements, distal sensory loss to all modalities, areflexia without weakness and lower limb ataxia. MRI brain and FDG-PET scan demonstrated biparietal and cerebellar atrophy/hypometabolism. Whole exome sequencing detected a heterozygous likely pathogenic missense variant in DNMT1, c.1289G > A, p.Cys430Tyr. Cochlear implant was performed at 44 years for the bilateral high frequency sensorineural hearing loss with improvement in hearing and day-to-day function. RESULTS AND CONCLUSION: We describe a novel variant in DNMT1 and confirm that an overlapping HSN1E-cerebellar phenotype can occur. Only one prior case of cochlear implant in HSN1E has been reported to date but this case adds to that literature, suggesting that cochlear implant can be successful in such patients. We further explore the clinical and radiological signature of the cognitive syndrome associated with this disorder.

Export pathways of biosolid derived microplastics in soil systems - Findings from a temperate maritime climate.

The environmental fate of microplastics (MPs) added to agricultural soils remains poorly understood, particularly regarding their mobility in soils. Here we investigate the potential for MP export from soil to surface waters and groundwater in two agricultural settings with a 20-year history of biosolid treatment. A third site where biosolids had never been applied served as a reference (Field R). The potential for MP export along overland and interflow pathways to surface waters was determined from MP abundances in shallow surface cores (10 cm) along ten down-slope transects (five each for Field A and B), and through MP abundances in effluent from a sub-surface land drain. The risk of vertical MP migration was assessed from 2 m cores, and from MP abundances in groundwater sampled from the core boreholes. XRF Itrax core scanning was conducted on two of the deep cores to capture high-resolution optical and 2-D radiographic imaging. Results suggest limited MP mobility at depths >35 cm, with MPs largely recovered in surface soils characterised by lower compaction. Furthermore, abundances of MPs across the surface cores were comparable, with no evidence of MP accumulations observed. Average MP abundance in the top 10 cm of soil across Field A and B was 365 ± 302 MP kg-1, with 0.3 MP l-1 and 1.6 MP l-1 recovered from the groundwater and field drainpipe water samples, respectively. MP abundances were significantly higher in fields treated with biosolids than in Field R (90 ± 32 MP kg-1 soil). Findings suggest ploughing is the most significant driver of MP mobility in upper soil layers, however the potential for overland or interflow movement cannot be excluded, particularly for fields that may be artificially drained.

Frequency of inter-specialty consensus decisions and adherence to advice following discussion at a weekly neurovascular multidisciplinary meeting.

BACKGROUND/AIMS: Data are limited on the frequency of 'consensus decisions' between sub-specialists attending a neurovascular multidisciplinary meeting (MDM) regarding management of patients with extracranial carotid/vertebral stenoses and post-MDM 'adherence' to such advice. METHODS: This prospective audit/quality improvement project collated prospectively-recorded data from a weekly Neurovascular/Stroke Centre MDM documenting the proportion of extracranial carotid/vertebral stenosis patients in whom 'consensus management decisions' were reached by neurologists, vascular surgeons, stroke physicians-geriatricians and neuroradiologists. Adherence to MDM advice was analysed in asymptomatic carotid stenosis (ACS), symptomatic carotid stenosis (SCS), 'indeterminate symptomatic status stenosis' (ISS) and vertebral artery stenosis (VAS) patients, including intervals between index event to MDM + / - intervention. RESULTS: One hundred fifteen patients were discussed: 108 with carotid stenosis and 7 with VAS. Consensus regarding management was noted in 96.5% (111/115): 100% with ACS and VAS, 96.2% with SCS and 92.9% with ISS. Adherence to MDM management advice was 96.4% (107/111): 100% in ACS, ISS and VAS patients; 92% (46/50) in SCS patients. The median interval from index symptoms to revascularisation in 50-99% SCS patients was 12.5 days (IQR: 9-18.3 days; N = 26), with a median interval from MDM to revascularisation of 5.5 days (IQR: 1-7 days). Thirty patients underwent revascularisation. Two out of twenty-nine patients (6.9%) with either SCS or ISS had a peri-procedural ipsilateral ischaemic stroke, with no further strokes/deaths during 3-months follow-up. CONCLUSIONS: The high frequency of inter-specialty consensus regarding management and adherence to proposed treatment supports a collaborative/multidisciplinary model of care in patients with extracranial arterial stenoses. Service development should aim to shorten times between MDM discussion-intervention and optimise prevention of stroke/death.

Contactin-1 links autoimmune neuropathy and membranous glomerulonephritis.

Membranous glomerulonephritis (MGN) is a common cause of nephrotic syndrome in adults, mediated by glomerular antibody deposition to an increasing number of newly recognised antigens. Previous case reports have suggested an association between patients with anti-contactin-1 (CNTN1)-mediated neuropathies and MGN. In an observational study we investigated the pathobiology and extent of this potential cause of MGN by examining the association of antibodies against CNTN1 with the clinical features of a cohort of 468 patients with suspected immune-mediated neuropathies, 295 with idiopathic MGN, and 256 controls. Neuronal and glomerular binding of patient IgG, serum CNTN1 antibody and protein levels, as well as immune-complex deposition were determined. We identified 15 patients with immune-mediated neuropathy and concurrent nephrotic syndrome (biopsy proven MGN in 12/12), and 4 patients with isolated MGN from an idiopathic MGN cohort, all seropositive for IgG4 CNTN1 antibodies. CNTN1-containing immune complexes were found in the renal glomeruli of patients with CNTN1 antibodies, but not in control kidneys. CNTN1 peptides were identified in glomeruli by mass spectroscopy. CNTN1 seropositive patients were largely resistant to first-line neuropathy treatments but achieved a good outcome with escalation therapies. Neurological and renal function improved in parallel with suppressed antibody titres. The reason for isolated MGN without clinical neuropathy is unclear. We show that CNTN1, found in peripheral nerves and kidney glomeruli, is a common target for autoantibody-mediated pathology and may account for between 1 and 2% of idiopathic MGN cases. Greater awareness of this cross-system syndrome should facilitate earlier diagnosis and more timely use of effective treatment.

Adjunctive cenobamate in highly active and ultra-refractory focal epilepsy: A "real-world" retrospective study.

OBJECTIVE: Recent clinical trials have shown that cenobamate substantially improves seizure control in focal-onset drug-resistant epilepsy (DRE). However, little is known about cenobamate's performance in highly active (≥20 seizures/month) and ultra-refractory focal epilepsy (≥6 failed epilepsy treatments, including antiseizure medications [ASMs], epilepsy surgery, and vagus nerve stimulation). Here, we studied cenobamate's efficacy and tolerability in a "real-world" severe DRE cohort. METHODS: We conducted a single-center retrospective analysis of consecutive adults treated with cenobamate between October 2020 and September 2022. All patients received cenobamate through an Early Access Program. Cenobamate retention, seizure outcomes, treatment-emergent adverse events, and adjustments to concomitant ASMs were analyzed. RESULTS: Fifty-seven patients received cenobamate for at least 3 months (median duration, 11 months). The median cenobamate dose was 250 mg/day (range 75-350 mg). Baseline demographics were consistent with highly active (median seizure frequency, 60/month) and ultra-refractory epilepsy (median previously failed ASMs, nine). Most (87.8%) had prior epilepsy surgery and/or vagus nerve stimulation. Six patients stopped cenobamate due to lack of efficacy and/or adverse events. One patient died from factors unrelated to cenobamate. Among patients who continued cenobamate, three achieved seizure freedom (5.3% of cohort), 24 had a 75%-99% reduction in seizures (42.1% of cohort), and 16 had a 50%-74% reduction (28.1% of cohort). Cenobamate led to abolition of focal to bilateral tonic-clonic seizures in 55.6% (20/36) of patients. Among treatment responders, 67.4% (29/43) were treated with cenobamate doses of ≥250 mg/day. Three-fourths of patients reported at least one side-effect, most commonly fatigue and somnolence. Adverse events most commonly emerged at cenobamate doses of ≥250 mg/day. Side-effects were partially manageable by reducing the overall ASM burden, most often clobazam, eslicarbazepine, and perampanel. SIGNIFICANCE: Patients with highly active and ultra-refractory focal epilepsy experienced meaningful seizure outcomes on cenobamate. Emergence of adverse events at doses above 250 mg/day may limit the potential for further improvements in seizure control at higher cenobamate doses.

Age- and sex-specific survivorship of the Southern Hemisphere long-finned pilot whale (Globicephala melas edwardii).

Biodiversity loss is a major global challenge of the 21st century. Ultimately, extinctions of species are determined by birth and death rates; thus, conservation management of at-risk species is dependent on robust demographic data. In this study, data gathered from 381 (227 females, 154 males) long-finned pilot whales (Globicephala melas edwardii) that died in 14 stranding events on the New Zealand coast between 2006 and 2017 were used to construct the first age- and sex-specific life tables for the subspecies. Survivorship curves were fitted to these data using (1) a traditional maximum likelihood approach, and (2) Siler's competing-risk model. Life table construction and subsequent survival curves revealed distinct differences in the age- and sex-specific survival rates, with females outliving males. Both sexes revealed slightly elevated rates of mortality among the youngest age-classes (<2 years) with postweaning mortality rates decreasing and remaining relatively low until the average life expectancy is reached; 11.3 years for males and 14.7 years for females. Overall (total) mortality is estimated to be 8.8% and 6.8% per annum for males and females, respectively. The mortality curve resembles that of other large mammals, with high calf mortality, lower postweaning mortality, and an exponentially increasing risk of senescent mortality. An accelerated mortality rate was observed in mature females, in contrast to the closely related short-finned pilot whale (G. macrorhynchus), which selects for an extension to the postreproductive life span. The reason for the observed differences in the mortality rate acceleration and postreproductive life span between the two pilot whale species have not been established and warrant further investigation. Obtaining robust information on the life history of long-lived species is challenging, but essential to improve our understanding of population dynamics and help predict how future pressures may impact populations. This study illustrates how demographic data from cetacean stranding events can improve knowledge of species survival rates, thus providing essential information for conservation management.

Evaluation of a marine mammal status and trends contaminants indicator for European waters.

Marine mammals are vulnerable to the bioaccumulation, biomagnification and lactational transfer of specific types of pollutants, such as industrial polychlorinated biphenyls (PCBs), due to their long-life spans, feeding at a high trophic level and unique fat stores that can serve as depots for these lipophilic contaminants. Currently, European countries are developing indicators for monitoring pollutants in the marine environment and assessing the state of biodiversity, requirements under both Regional Seas Conventions and European legislation. As sentinel species for marine ecosystem and human health, marine mammals can be employed to assess bioaccumulated contaminants otherwise below current analytical detection limits in water and lower trophic level marine biota. To aid the development of Regional Seas marine mammal contaminants indicators, as well as Member States obligations under descriptor 8 of the EU Marine Strategy Framework Directive, the current study aims to further develop appropriate methodological standards using data collected by the established UK marine mammal pollutant monitoring programme (1990 to 2017) to assess the trends and status of PCBs in harbour porpoises. Within this case study, temporal trends of PCB blubber concentration in juvenile harbour porpoises were analysed using multiple linear regression models and toxicity thresholds for the onset of physiological (reproductive and immunological) endpoints were applied to all sex-maturity groups. Mean PCB blubber concentrations were observed to decline in all harbour porpoise Assessment Units and OSPAR Assessment Areas in UK waters. However, a high proportion of animals were exposed to concentrations deemed to be a toxicological threat, though the relative proportion declined in most Assessment Units/Areas over the last 10 years of the assessment. Recommendations were made for improving the quality of the assessment going forward, including detailing monitoring requirements for the successful implementation of such an indicator.

Reproductive biology of female common dolphins (Delphinus delphis) in New Zealand waters.

Reproductive biology was assessed in 106 female common dolphins (Delphinus delphis) examined post-mortem from stranding and bycatch events along the New Zealand coastline between 1997 and 2019. The average age (ASM) and length (LSM) at sexual maturity was estimated at 7.5 years and 183.5 cm, respectively. The total number of corpora in mature individuals increased with age and appeared to persist throughout life. Ovarian asymmetry was apparent, with the left ovary displaying higher rates of ovulation, and a maximum of 19 corpora recorded for a 24-year-old female. The estimated ovulation and annual pregnancy rates for mature females were 0.39 year-1 and 30%, respectively. Conception and calving occurred year-round, with a weak seasonal increase observed in late austral spring and early austral summer. As these data did not clearly show whether seasonality was present, the gestation, lactation, and resting periods were calculated as either 12.6 or 12.8 months based on the presence/absence of seasonality, respectively. Similarly, calving interval ranged from 3.15 to 3.2 years, depending upon whether seasonality was considered. The estimated LSM of the New Zealand population aligns with other populations globally, although the estimated ASM is younger by approximately 6 months. Other reproductive parameters align with Northern Hemisphere populations, although demonstrate variation, which may reflect adaptations to local conditions such as water temperature and prey availability. As the species is subject to anthropogenic impacts including pollution and bycatch, we suggest our findings be used as a baseline with which to monitor trends in population parameters. Supplementary Information: The online version contains supplementary material available at 10.1007/s00227-022-04139-3.

Stereotactic radiosurgery with Cyberknife®: first case in the United Arab Emirates (a case report).

A 64-year-old gentleman was referred to the department of oncology with severe pain in the right ear radiating to the right side of the face. Imaging revealed a large extra-axial expansile lesion, surrounding and encasing the right cavernous sinus extending to the right middle cranial fossa. The patient consulted several neurosurgeons and was recommended stereotactic radiosurgery with Cyberknife® as the best non-invasive modality. The proximity to the critical structures, such as the brainstem, made it challenging for any surgical approach. The patient completed stereotactic radiosurgery with Cyberknife® and is doing well one month after treatment.

Age, growth, and sexual dimorphism of the Southern Hemisphere long-finned pilot whale (Globicephala melas edwardii).

Knowledge of population biological parameters can contribute to assessing the resilience of a population in the face of increasing anthropogenic pressures. Southern Hemisphere long-finned pilot whales (Globicephala melas edwardii) are susceptible to high rates of live stranding-related mortality. However, the biological parameters of this population largely are unknown. In this study, age, growth, allometry, and sexual dimorphism are described using teeth and external body measurements obtained from 515 male, 776 female, and 229 individuals of unknown sex, stranded on the New Zealand coastline between 1948 and 2017. Maximum ages of 31 and 38 years were estimated for males (n = 163) and females (n = 239), respectively. Females ranged in length from 160 to 500 cm (modal size class 400-449 cm) and males from 165 to 622 cm (modal size class 500-549 cm). Length-at-birth for both sexes was estimated at 170 cm using a logistic regression model. Growth models for both sexes indicated a preliminary rapid growth phase followed by a second phase of slower growth. For males, a two-phase growth model also indicated a moderate growth spurt around the average age at attainment of sexual maturity (ca.12-13 years). Asymptotic lengths were estimated at 570 and 438 cm for males and females, respectively. We found strong evidence of sexual size dimorphism, with males significantly larger than females for 13 of 14 external measurements. We also found sexual dimorphism with respect to shape, with males having proportionally longer pectoral fins, wider tail flukes, and taller dorsal fins, than females. Estimates of length-at-birth, maximum ages, and sexual shape dimorphism for G. m. edwardii differed from those previously reported for the North Atlantic subspecies (G. m. melas), which may indicate subspecies or population-level differences in morphology, longevity, and sociality.