Impact of remoteness and rurality on the treatment and survival of patients with glioblastoma in the north of Scotland.

Background: The geographical catchment area served by the Neurosurgical Unit in Aberdeen, Scotland is the largest in the United Kingdom. We examined whether a distance-decay effect on survival exists for patients diagnosed with glioblastoma, who have to travel substantial distances for neurosurgical and oncological treatment in the north of Scotland. Methods: Electronic medical records of adult patients with glioblastoma, referred for treatment between 2007 and 2018, who underwent surgical resection were reviewed. Travel time by car (as a measure of distance travelled) was calculated from the patients' home to their general practice (GP) and to their main neuro-oncological centre. Results: There were 122 patients; 71 (58.2%) were male and the mean age was 57.8 years. The urban-rural split was 61.5% and 38.5%, respectively. Median driving time to the neuro-oncological centre was 36 min and to the GP this was 6 min. Most patients underwent either sub-total (49.6%) or gross total (46.3%) surgical resection. Post-operative treatments included: radiotherapy only (15.6%), chemotherapy only (6.6%), and chemotherapy with radiotherapy (63.1%). Temozolomide was used in 70.5% of patients. Seventeen patients did not receive any post-operative chemo-radiotherapy. The median survival time was 345 days. There was no statistically significant association between distance travelled and survival time in days. MGMT methylation status, extent of resection, Charlson co-morbidity index and treatment received significantly affected survival. Conclusions: There was no evidence of disadvantage on survival time for patients living further from their neuro-oncological centre compared to those who live nearer.

Tectal Plate Glioma: A Clinical and Radiologic Analysis of Progression and Management in Adults.

BACKGROUND: Tectal plate gliomas (TPGs) are a heterogeneous group of uncommon brain tumors. TPGs are considered indolent and are usually managed conservatively but they have the potential to transform into higher-grade tumors. The aims of this study were to investigate the natural history of adult TPG, treatment outcomes, and overall survival. METHODS: A retrospective cohort analysis was performed of adult patients with TPG between 1993 and 2021. Baseline clinical, radiologic, and management characteristics were collected. The primary outcome was tumor progression, defined as increasing size on radiologic assessment or new gadolinium contrast enhancement. Secondary outcomes included management and mortality. RESULTS: Thirty-nine patients were included, of whom 23 (52.2%) were men. Median age at diagnosis was 35 years (interquartile range, 27-53). Radiologic tumor progression was observed in 8 patients (20.5%). The 10-year progression-free survival was 72.6% (95% confidence interval [CI], 0.58-0.91). The 10-year overall survival was 86.5% (95% confidence interval, 0.75-1.0). Cerebrospinal fluid diversion procedures were used in 62% of the cohort (n = 24). Seventeen patients (43.6%) underwent at least 1 endoscopic third ventriculostomy, whereas only 6 patients (15.4%) underwent at least 1 ventriculoperitoneal shunt. CONCLUSIONS: TPG has an overall favorable clinical prognosis, although progression occurs in 1 in 5 patients. Showing accurate factors by which patients with TPG may be risk stratified should be a key area of further research. A follow-up duration of 10 years would be a reasonable window based on the radiologic progression rates in this study; however, larger cohort studies are needed to answer both questions definitively.

Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension.

BACKGROUND: We aimed to create a multidisciplinary consensus clinical guideline for best practice in the diagnosis, investigation and management of spontaneous intracranial hypotension (SIH) due to cerebrospinal fluid leak based on current evidence and consensus from a multidisciplinary specialist interest group (SIG). METHODS: A 29-member SIG was established, with members from neurology, neuroradiology, anaesthetics, neurosurgery and patient representatives. The scope and purpose of the guideline were agreed by the SIG by consensus. The SIG then developed guideline statements for a series of question topics using a modified Delphi process. This process was supported by a systematic literature review, surveys of patients and healthcare professionals and review by several international experts on SIH. RESULTS: SIH and its differential diagnoses should be considered in any patient presenting with orthostatic headache. First-line imaging should be MRI of the brain with contrast and the whole spine. First-line treatment is non-targeted epidural blood patch (EBP), which should be performed as early as possible. We provide criteria for performing myelography depending on the spine MRI result and response to EBP, and we outline principles of treatments. Recommendations for conservative management, symptomatic treatment of headache and management of complications of SIH are also provided. CONCLUSIONS: This multidisciplinary consensus clinical guideline has the potential to increase awareness of SIH among healthcare professionals, produce greater consistency in care, improve diagnostic accuracy, promote effective investigations and treatments and reduce disability attributable to SIH.

Spinal cord compression secondary to extramedullary haematopoiesis in transformed polycythaemia rubra vera.

Polycythaemia vera (PV) is a clonal proliferative disorder of the bone marrow characterised by autonomous haematopoiesis, which results in a panmyelosis in the peripheral blood. It is typically characterised by an acquired mutation in JAK-2 V617F. Progression to myelofibrosis (MF), characterised by worsening cytopenias and the development of constitutional symptoms, is seen in up to 10% of cases. Extramedullary haematopoiesis (EMH) in the spleen is a common finding in myelofibrotic transformation, but elsewhere in the body it is extremely unusual. We report the case of a 69-year-old male whose PV progressed to secondary MF and who presented with compression of the thoracic spinal cord directly as a result of EMH. Cytogenetic and molecular findings in the bone marrow were in keeping with evolving myeloid disease. He was managed by surgical laminectomy with an excellent outcome. Extramedullary haematopoiesis may be seen in both PV and on transformation to MF. This very rare complication should be borne in mind when managing patients with myeloproliferative disorders.

Histologically proven acute paediatric thoracic disc herniation causing paraparesis: a case report and review of literature.

PURPOSE: We describe an extremely rare and previously unreported presentation of acute progressive paraparesis secondary to traumatic thoracic disc herniation in a child presenting to our institution. METHODS: A 12-year-old girl presented with progressive paraparesis 24 h after falling from standing height while playing at school. She was being lifted up by her friends and fell landing on her feet then rolled onto her back initially with no pain or neurological sequelae. Over the next few hours, she developed back pain followed by progressive paraparesis associated with urinary retention and sensory impairment. RESULTS: MR imaging demonstrated an unusual lateral and dorsally based lesion at T7/8 causing cord compression which was thought to represent an epidural haematoma. Urgent posterior decompressive surgery was performed but no evidence of haematoma was seen, a large well-circumscribed solid piece of soft tissue was found in the extradural space causing significant cord compression. This was sent for histological analysis and subsequently reported as showing cartilaginous disc material. Postoperative MR imaging at 2 weeks and 3 months demonstrates complete resection of this disc material with no significant kyphotic deformity on standing X-ray at 18 months. Complete neurological recovery occurred over the subsequent 3 months following emergent surgery, and at 18-month review, the patient remains asymptomatic and fully independent.

Use of intraoperative Doppler ultrasound with neuronavigation to guide arteriovenous malformation resection: a pediatric case series.

OBJECT: Over the last 20 years, several intraoperative adjuncts, including ultrasonography, neuronavigation, and angiography, have been said to aid the intraoperative localization and resection of cerebral arteriovenous malformations (AVMs). The authors assessed the value of intraoperative Doppler ultrasonography in conjunction with neuronavigation during surgery for cerebral AVMs in the pediatric population. METHODS: The authors reviewed all cranial AVM resections performed by a single surgeon at their institution in the period from 2007 to 2013 and here describe their experience and results in a series of 20 consecutive AVM resections in 19 pediatric patients. Intraoperative Doppler ultrasonography had been used in conjunction with preoperative CT or neuronavigational MRI. Preoperative and postoperative clinical findings, patient age, and Spetzler-Martin AVM grade were identified in all patients. RESULTS: All patients, whose ages ranged from 2 to 16 years, underwent craniotomy and excision of an AVM, which was supratentorial in 18 cases and infratentorial in 2. Patients in 11 cases underwent preoperative embolization, and all other patients underwent cerebral angiography prior to surgery, except for 2 patients who were urgently surgically treated because of low Glasgow Coma Scale scores and associated hematoma. Spetzler-Martin Grades I (3 cases), II (6), III (7), and IV (4) AVMs were represented in this series. Intraoperative Doppler ultrasound provided high-quality images in all cases and demonstrated the location, size, and flow characteristics of the AVM and any associated hematoma. Delayed postoperative cerebral angiography demonstrated successful AVM resection in all cases. An assessment of clinical outcomes revealed no new long-term neurological deficits at 3 months postoperatively. CONCLUSIONS: Intraoperative Doppler ultrasonography is a reliable and useful tool for intraoperative localization and guidance for AVM resection in the pediatric population. When used in conjunction with neuronavigation equipment and modern microscopes, this technique has shown a very high complete resection rate with extremely low associated morbidity.

Occipitocondylar hyperplasia and syringomyelia presenting with facial pain.

The authors describe a unique presentation and long-term management of a rare craniovertebral abnormality in a patient presenting to their institution. This 10-year-old girl presented with right-sided facial pain and subjective dysesthesia of the chest wall without evidence of cervical myelopathy. She was found to have extensive cervicothoracic syringomyelia secondary to compression at the foramen magnum by hypertrophic occipital condyles. Posterior decompression and medial condylectomy was performed, with significant radiological and clinical improvement over the next 5 years of follow-up. The authors discuss the clinical pathophysiology and operative techniques used.