OBJECTIVE Holmes tremor (HT) is a rare and debilitating movement disorder comprising both rest and action tremor, and it is known for its resistance to treatment. Its most common causes include ischemic or hemorrhagic insults and trauma. Mechanistically, the combined rest and action tremor is thought to require a double lesion of both the dopaminergic nigrostriatal system and the dentatorubrothalamic pathways, often near the midbrain where both pathways converge. The aim of this study was to characterize HT as a presenting sign in cases of hydrocephalus and to discuss potential pathomechanisms, clinical presentations, and treatment options. METHODS MEDLINE and Web of Science were searched for cases of HT with hydrocephalus from database inception to August 2021, and these were compiled along with the authors' own unique case of treatment-responsive HT in a child with low-pressure obstructive hydrocephalus secondary to a tectal tumor. Patient characteristics, presenting signs/symptoms, potential precipitating factors, interventions, and patient outcomes were recorded. RESULTS Nine patients were identified including the authors' video case report. All patients had a triventriculomegaly pattern with at least a component of obstructive hydrocephalus, and 4 patients were identified as having low-pressure hydrocephalus. Parinaud's syndrome and bradykinesia were the most commonly associated signs. Levodopa and CSF diversion were the most commonly used and effective treatments for HT in this population. This review was not registered and did not receive any funding. CONCLUSIONS HT is a poorly understood and probably underrecognized presentation of hydrocephalus that is difficult to treat, limiting the strength of the evidence in this review. Treatment options include CSF diversion, antiparkinsonian agents, antiepileptic agents, deep brain stimulation, and MR-guided focused ultrasound, and aim toward the nigrostriatal and dentatorubrothalamic pathways hypothesized to be involved in its pathophysiology.
e congratulate the Neurosurgical Focus Editorial Board for this landmark issue.However, it is timely to examine why it is extraordinary and describe the gender landscape for women neurosurgery leaders.A volume on male leaders in neurosurgery would be simply "International leaders in neurosurgery, past and present," and many of the statements below would be absurd if gender were reversed.Compare "…the greatest perceived barrier to women wanting to pursue…a career in surgery was in the incongruity with motherhood…" with "…the greatest perceived barrier to men wanting to pursue…a career in surgery was in the incongruity with fatherhood….
The use of social media has continued to grow in medicine and surgery and the scholarly evaluation of its role, as well as potential drawbacks, is prudent. We read with interest a recently published article on social media of vascular surgeons.1 As fellow surgeons, social media users, and researchers, we have some concerns regarding the methodology and conduct of the study, the ethics of the study, and, finally, its conclusions.
Enterogenous cysts are rare benign congenital tumours of the central nervous system. The aim of management is complete resection to minimise the chance of recurrence. To date, management of recurrence has favoured further surgical resection. We describe the case of a recurrent enterogenous cyst of the cervical spine, initially treated with decompression via laminectomy and fenestration. Magnetic Resonance Imaging (MRI) follow up has demonstrated spontaneous recurrence and deflation of the cyst on multiple occasions. We propose that conservative management of recurrent enterogenous cysts may be a valid management option following fenestration or partial resection of the cyst, and that recurrence may not always warrant further surgical intervention.
The emergence of coronavirus disease 2019 (COVID-19) and the ensuing pandemic have altered every aspect of our healthcare system, including the care delivered to those patients who do not contract the virus. However, some non-COVID-19 patients will be more affected than others. In addition to appropriate restrictions in place as of 2 April 2020 in Australia, which limit elective surgical procedures to only those in category 1 (urgent, admission within 30 days) and high priority category 2 (semi-urgent, admission within 90 days), there are now operations which are deemed too unsafe to be performed, even if urgent. The early experience in China and Italy has revealed an increased risk of contagion among ear, nose and throat surgeons and their teams performing aerosolgenerating procedures such as those involving the sinuses. This has implications for patients awaiting neurosurgery for lesions in the pituitary fossa and anterior skull base, which are frequently approached through trans-sphenoidal and other endonasal transsinus surgical corridors. Throughout the world, there has been a strong recommendation to avoid trans-sphenoidal approaches for pituitary tumours. International specialist societies including our own Neurosurgery Society of Australasia have recommended ‘serious consideration be given to avoiding a trans-sphenoidal approach due to extremely high viral transmission risk’. Fortunately, most pituitary tumours are slowgrowing benign lesions and close monitoring with regular radiological imaging and visual assessment remains a viable option for pituitary tumour patients. A small proportion of pituitary tumours however will require surgical intervention acutely due to deteriorating visual acuity or visual fields across serial ophthalmological assessment, as well as pituitary apoplexy causing rapid loss of vision including blindness. In these situations, consideration must be given to the surgical alternative of an open craniotomy to allow access to the pituitary tumour and decompression of the optic apparatus. Whilst the risk profile of a craniotomy is higher compared to that of a trans-sphenoidal approach, the public health risk to the entire surgical team (doctors, nurses and technicians) and subsequently their contacts in proceeding with an aerosol-generating trans-sphenoidal surgery far outweighs the individual risk to the patient. An even smaller proportion of pituitary tumours may present with acute medical manifestations due to hormonal imbalances. Where possible, these should be managed medically under close supervision of an endocrinologist. Fulminant Cushing’s disease is the one rare situation whereby a short period of medical management might fail, and in this situation careful discussion with the treating medical and surgical team must be undertaken in the context of the COVID-19 pandemic to guide surgical approach. We are not suggesting that a craniotomy for a pituitary tumour is superior to a trans-sphenoidal approach. Indeed, our extensive experience across three high-volume pituitary centres confirms that visual and endocrine outcomes are optimized via the transsphenoidal approach. Likewise, we are not suggesting that all pituitary tumours undergo a craniotomy; however, in the current climate of COVID-19, we would strongly recommend a protocol of close monitoring of pituitary tumour patients and proceeding with a craniotomy for those patients who warrant urgent surgical intervention. Finally, it is important to consider the psychology of the patient throughout this situation. Many pituitary tumour patients are well informed having been down a long journey to diagnosis, and to be told their surgical treatment will be delayed (indefinitely) or be considered for a craniotomy may impact on their mental well-being. Having held several discussions with our current pituitary patients, it is important to reassure them of the safety of the current approach, to empathize with their situation and to be in regular contact with them during this period. Whilst we are acting for the good of the overall community, it can be a long and lonely path to walk for the individual patient affected by these decisions.
Research on the epidermal growth factor (EGF) family and the family of receptors (EGFR) has progressed rapidly in recent times. New crystal structures of the ectodomains with different ligands, the activation of the kinase domain through oligomerisation and the use of fluorescence techniques have revealed profound conformational changes on ligand binding. The control of cell signaling from the EGFR-family is complex, with heterodimerisation, ligand affinity and signaling cross-talk influencing cellular outcomes. Analysis of tissue homeostasis indicates that the control of pro-ligand processing is likely to be as important as receptor activation events. Several members of the EGFR-family are overexpressed and/or mutated in cancer cells. The perturbation of EGFR-family signaling drives the malignant phenotype of many cancers and both inhibitors and antagonists of signaling from these receptors have already produced therapeutic benefits for patients. The design of affibodies, antibodies, small molecule inhibitors and even immunotherapeutic drugs targeting the EGFR-family has yielded promising new approaches to improving outcomes for cancer patients. In this review, we describe recent discoveries which have increased our understanding of the structure and dynamics of signaling from the EGFR-family, the roles of ligand processing and receptor cross-talk. We discuss the relevance of these studies to the development of strategies for designing more effective targeted treatments for cancer patients.
Spinal epidural lipomatosis (SEL) results from an abnormal accumulation of unencapsulated fat within the epidural space and is a rare cause of spinal cord compression, which needs to be considered with a high index of suspicion. It most commonly occurs secondary to chronic corticosteroid use and endocrinopathies. Idiopathic cases are highly associated with obesity. We report an unusual case of idiopathic thoracic SEL in a 69-year-old male, with an adjacent infiltrative Hodgkin's lymphoma and associated vertebral crush fracture, which resulted in ataxia and sensory loss. Magnetic resonance imaging scans displayed extensive SEL and an infiltrative disease process causing thoracic cord compression. Surgical decompression confirmed the presence of extensive epidural lipomatosis and Hodgkin's lymphoma and subsequently led to improvement in neurological symptoms. To our knowledge, this is the first reported case of concomitant SEL with an adjacent Hodgkin's lymphoma resulting in cord compression.
Gender perceptions in surgery are changing. The archetypal surgeon of yesteryear was a very masculine male. Being a feminine surgeon was once considered an oxymoron; though, with the increasing feminisation of the profession, being a woman and a capable surgeon are no longer mutually exclusive. There are however, significant obstacles to a fully inclusive perception of surgeons that reaches beyond gender and encompasses a diversity of masculinities and femininities. These obstacles, and indeed advancements, will be explored in this article, with particular attention to how gender insights will challenge the way surgeons and trainees participate in surgical education and the future workforce. Traditionally, women have been underrepresented across all medical specialties, especially surgery. Occupational gender disproportion has occurred for many years. As societies begin to understand gender equality, women are making some gains in gender parity in many industries around the developed world. Female applicants to medical schools in the USA rose from 9% in 1965, to 46% in 2005. Most medical schools in Australia and New Zealand now have a slight female predominance. While these gains are a sign of progress, the changes at the undergraduate level are yet to have a significant impact on the proportion of surgeons who are female. Women make up 28% of current trainees, but only 10% of surgeons in Australia and New Zealand. There are many reasons for the paucity of female surgeons; the projected ‘future self’ of medical students and prevocational doctors has been explored in great detail in the literature, with papers published across surgery, education, social sciences, psychology and humanities. A common theme in this literature is that female medical students often struggle to project themselves actually being a surgeon because of a lack of well-balanced female role models, widely held notions that leading a balanced, full life and being a surgeon are not compatible, and that family priorities must take a back seat during the long years of training required. With the increasing prevalence of graduate-entry medical education, this means that these training years often continue into the mid-to-late30s and potentially early 40s – a time in life when opportunities for reproduction through traditional means become biologically nonnegotiable. The time taken to train in surgery is comparable to other specialties; however, surgery continues to hold the reputation for the most arduous and inflexible training. Given these perceptions, coupled with a complete lack of practical institutional and jurisdictional policies on parental leave or flexible training, medical students and junior doctors cannot be blamed for shunning surgery. However, it is not women alone who are responsible for this trend away from surgery – in a study of over 12 000 American medical graduates annually over a 13-year period, Lambert and Holmboe demonstrated both ‘men and women expressed similar and significant rates of declining interest in specialties with uncontrollable lifestyles’. Much of the literature in this area cites the lack of suitable female surgeons with a balanced lifestyle as being a significant barrier to recruiting females to surgery. While current female surgeons have not needed widespread gender-specific role models to achieve career success, female medical students and junior doctors often want to see suitable female surgeons so they can project their own values onto a model that already exists in order to have their possible future career actually embodied before they feel comfortable to imagine themselves in a similar role. Nevertheless, there is an emerging body of work that demonstrates a gradual improvement in the gender balance in surgery. In the article by Borman, she argues women are joining the mainstream of surgery and becoming an important current within it. It is certainly our experience that the surgical workforce in Australia and New Zealand is changing. In their work assessing medical student perceptions compared with the realities for female surgeons, Park et al. were able to demonstrate a clear difference in perceptions versus realities when it came to issues of rewarding family life, happy marriage, or having children. If gains are to continue to be made, medical students and prevocational doctors demand to see and experience, through their observations of female trainees and surgeons, practical improvements in these areas. The aspirations of medical students and prevocational doctors impact hugely, but somewhat invisibly, on eventual selection into surgical training. Training institutions, such as the Royal Australasian College of Surgeons (RACS), rarely see who is not applying, only who is applying to their training schemes. The numbers and reasons for not applying to surgery are relatively difficult to assess. The best objective measure we have for this concept is the feminisation of other ‘procedural specialties’, for example, obstetrics and gynaecology (O&G). In O&G, 80% of trainees and 43% of consultants are female. By comparison, 28% of surgical trainees and 10% of surgeons are female. Despite the well-established gender parity in medical schools, females are choosing specialties other than surgery at a greater rate than they are choosing surgery. By inference, surgery is missing out; ‘if women continue to reject surgical careers, recruitment will be limited to a decreasing pool of male applicants’. Despite these silent non-applicants and their impact on the selection pool for surgical training, surgery continues to enjoy popularity among medical students and prevocational doctors as it is seen as one of the most prestigious specialties within the medical profession PERSPECTIVES
ANZ Journal of SurgeryVolume 81, Issue 4 p. 289-290 Trigeminal neuralgia due to severe vascular compression of the trigeminal nerve Reuben D. Johnson DPhil, FRCS (Neuro.Surg.), Reuben D. Johnson DPhil, FRCS (Neuro.Surg.) Department of Neurosurgery, Royal Melbourne Hospital,Parkville, Melbourne, Victoria, AustraliaSearch for more papers by this authorRuth Mitchell MB ChB, Ruth Mitchell MB ChB Department of Neurosurgery, Royal Melbourne Hospital,Parkville, Melbourne, Victoria, AustraliaSearch for more papers by this authorNiki Maartens FRCS (SN), FRACS, Niki Maartens FRCS (SN), FRACS Department of Neurosurgery, Royal Melbourne Hospital,Parkville, Melbourne, Victoria, AustraliaSearch for more papers by this author Reuben D. Johnson DPhil, FRCS (Neuro.Surg.), Reuben D. Johnson DPhil, FRCS (Neuro.Surg.) Department of Neurosurgery, Royal Melbourne Hospital,Parkville, Melbourne, Victoria, AustraliaSearch for more papers by this authorRuth Mitchell MB ChB, Ruth Mitchell MB ChB Department of Neurosurgery, Royal Melbourne Hospital,Parkville, Melbourne, Victoria, AustraliaSearch for more papers by this authorNiki Maartens FRCS (SN), FRACS, Niki Maartens FRCS (SN), FRACS Department of Neurosurgery, Royal Melbourne Hospital,Parkville, Melbourne, Victoria, AustraliaSearch for more papers by this author First published: 21 March 2011 https://doi.org/10.1111/j.1445-2197.2011.05676.xCitations: 1Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume81, Issue4April 2011Pages 289-290 RelatedInformation
Gliomatosis cerebri (GC) is an uncommon brain tumour defined as a diffuse neoplastic glial cell infiltration of the brain, involving more than two cerebral lobes and, occasionally, the infratentorial structures or the spinal cord. GC of the oligodendroglial phenotype is extremely rare, especially in the paediatric setting. We describe an unusual case of oligodendroglial GC diagnosed in a 16-year-old boy with Ollier disease. To our knowledge this is the first case of GC reported in a child with Ollier disease.
Although prostate adenocarcinoma is the most commonly diagnosed cancer in men, intracranial metastases are rare. We describe a 72-year-old patient with known metastatic prostate cancer, presenting with a dural-based parafalcine lesion on radiological imaging, following a seizure. Total macroscopic excision of the lesion was achieved at surgery, with histopathology confirming prostate adenocarcinoma embedded in an atypical (World Health Organization Grade II) meningioma, fulfilling all the criteria for true tumour-to-tumour metastasis. To our knowledge, this is the first report of prostate cancer metastasising to an atypical meningioma.
Olfactory neuroblastomas are malignant, neuroectodermal nasal tumours. We describe a 62-year-old patient with a recurrent olfactory neuroblastoma invading a radiation-induced meningioma. Given the temporal and spatial relationship between the primary lesion and the described recurrence, this was due to metastatic rather than direct contiguous spread, fulfilling all the criteria for true tumour-to-tumour metastasis. To our knowledge, this is the first description of an olfactory neuroblastoma metastasising to a meningioma.