A Case of Bing-Neel Syndrome Presenting Like Giant Cell Arteritis.

ABSTRACT: A 55-year-old woman presented with new-onset headache, scalp tenderness, shoulder arthralgias, night sweats, and loss of appetite. She was diagnosed with giant cell arteritis by her primary care physician and commenced on oral corticosteroids. However, her headache, scalp tenderness, and night sweats persisted. She then developed right Horner syndrome and trigeminal hypoesthesia. Extensive blood work-up revealed mildly elevated inflammatory markers and a paraproteinemia. Subsequent bone marrow biopsy showed lymphoplasmacytic lymphoma, with 10% of hemopoiesis, and staging led to the diagnosis of Waldenstrom macroglobulinemia without nodal or central nervous system (CNS) lesions. Immunohistochemical staining of a temporal artery biopsy showed perivascular lymphoplasmacytic cells and paraprotein deposits. She was diagnosed with CNS involvement of her macroglobulinemia-Bing-Neel syndrome (BNS). Identification of rare CNS involvement of lymphoma is challenging when a patient is already on steroid immunosuppression. In the absence of clear diagnostic criteria, the rare and heterogenous BNS remains a clinical diagnosis.

Multiple lumbar punctures aiming to relieve headache results in iatrogenic spinal hematoma: a case report.

BACKGROUND: Multiple lumbar punctures have historically been a strategy to relieve headaches associated with idiopathic intracranial hypertension despite limited clinical evidence of long-term efficacy. Lumbar puncture is typically a straightforward procedure with minimal complications reported, however, serious complications can occur. Lumbar-puncture-related spinal hematomas are rare but can lead to irreversible paralysis. CASE PRESENTATION: We report a case of a 28-year-old Caucasian woman who was treated with multiple lumbar punctures to manage headache, thought to be attributed to idiopathic intracranial hypertension. The patient developed a lumbosacral epidural hematoma following a lumbar puncture, which led to incomplete cauda equina syndrome. Multiple lumbar punctures had been the long-term management for the patient's chronic headaches associated with her diagnosis of idiopathic intracranial hypertension. She had no risks of an underlying coagulopathy. Following a lumbar puncture, she re-presented with lower back pain and bilateral paresthesia. Over the subsequent 48 hours, this progressed to urinary incontinence and saddle paresthesia. Imaging revealed an epidural hematoma, which was conservatively managed. She continued to report saddle paresthesia and urinary incontinence 7 months following the lumbar puncture. Between 1974 to 2022, our literature search found 41 case reports detailing lumbar-puncture-related spinal hematomas. It is an established but rare complication of lumbar puncture and there are limited studies looking at the incidence of its occurrence. Whilst coagulopathy has been found to be a risk factor, it is unclear if the gauge of the needle is relevant. Case evidence suggests there may be no significant difference in outcomes between surgical and conservative management of spinal hematomas. This case highlights that lumbar punctures can be invasive, with potentially serious complications. A lumbar puncture should therefore only be performed when clinically justified. CONCLUSIONS: This case highlights a rare complication of lumbar puncture and emphasizes the importance of a risk-benefit discussion for each procedure. Spinal hematoma following lumbar puncture is a rare complication but with potentially devastating consequences. Within the setting of idiopathic intracranial hypertension, the evidence base for the long-term benefit of headache relief by repeat lumbar puncture is low.

Reconfiguration from emergency to urgent elective neurosurgery for glioblastoma patients improves length of stay, surgical adjunct use, and extent of resective surgery.

Background: Glioblastoma (GB) is the most common intrinsic brain cancer and is notorious for its aggressive nature. Despite widespread research and optimization of clinical management, the improvement in overall survival has been limited. The aim of this study was to characterize the impact of service reconfiguration on GB outcomes in a single centre. Methods: Patients with a histopathological confirmation of a diagnosis of GB between 01/01/2014 and 31/12/2019 were retrospectively identified. Demographic and tumour characteristics, survival, treatment (surgical and oncological), admission status, use of surgical adjunct (5-aminolevulinic acid, intra-operative neuro-monitoring), the length of stay, extent of resection, and surgical complications were recorded from the hospital databases. Results: From August 2018 the neurosurgical oncology service was reconfigured to manage high-grade tumours on an urgent outpatient basis by surgeons specializing in oncology. We demonstrate that these changes resulted in an increase in elective admissions, greater use of intra-operative adjuncts resulting in the improved extent of tumour resection, and a reduction in median length of stay and associated cost-savings. Conclusions: Optimizing neuro-oncology patient management through service reconfiguration resulted in increased use of intra-operative adjuncts, improved surgical outcomes, and reduced hospital costs. These changes also have the potential to improve survival and disease-free progression for patients with GB.

Eosinophilic Granulomatosis with Polyangiitis Presenting as Unilateral Acute Anterior Ischaemic Optic Neuropathy.

Eosinophilic granulomatosis with polyangiitis (eGPA) is a rare vasculitis of small-medium sized vessels that can cause both anterior and posterior ischaemic optic neuropathies. Herein, the authors present a rare case of eGPA presenting initially as an acute unilateral anterior ischaemic optic neuropathy from short posterior ciliary artery vasculitis. The diagnosis presented a challenge as clinical and histopathological evidence suggested allergic rhinosinusitis, and no invasive fungal sinusitis was found. The high serum eosinophilia, asthma, optic neuropathy and paranasal sinus abnormalities fulfilled the criteria for a diagnosis of eGPA. Furthermore serum was positive for myeloperoxidase antibodies. Subsequently the case was successfully treated with oral glucocorticoids and intravenous rituximab.

Enlarged and Enhancing Optic Nerves in Advanced Glial Fibrillary Acidic Protein Meningoencephalomyelitis.

A 36-year-old woman presented with intermittent fever, nausea and vomiting, generalized polyarthralgias, and bilateral optic disc swelling. She had a history of difficult-to-control myasthenia gravis since the age of 18 years. Lumbar puncture demonstrated a normal opening pressure; cerebrospinal fluid (CSF) was remarkable for high protein, low glucose, and a mononuclear pleocytosis. Although initial MRI of the brain was normal, a repeat study 8 weeks later revealed enlarged and enhancing bilateral intraorbital and intracranial optic nerves. After a nondiagnostic brain biopsy, a CSF sample tested positive for antibodies to glial fibrillary acidic protein (GFAP). Findings in this case indicate that optic nerve swelling encountered in GFAP meningoencephalomyelitis is more likely due to optic nerve inflammation rather than elevated intracranial pressure.

Idiopathic intracranial hypertension: consensus guidelines on management.

The aim was to capture interdisciplinary expertise from a large group of clinicians, reflecting practice from across the UK and further, to inform subsequent development of a national consensus guidance for optimal management of idiopathic intracranial hypertension (IIH). METHODS: Between September 2015 and October 2017, a specialist interest group including neurology, neurosurgery, neuroradiology, ophthalmology, nursing, primary care doctors and patient representatives met. An initial UK survey of attitudes and practice in IIH was sent to a wide group of physicians and surgeons who investigate and manage IIH regularly. A comprehensive systematic literature review was performed to assemble the foundations of the statements. An international panel along with four national professional bodies, namely the Association of British Neurologists, British Association for the Study of Headache, the Society of British Neurological Surgeons and the Royal College of Ophthalmologists critically reviewed the statements. RESULTS: Over 20 questions were constructed: one based on the diagnostic principles for optimal investigation of papilloedema and 21 for the management of IIH. Three main principles were identified: (1) to treat the underlying disease; (2) to protect the vision; and (3) to minimise the headache morbidity. Statements presented provide insight to uncertainties in IIH where research opportunities exist. CONCLUSIONS: In collaboration with many different specialists, professions and patient representatives, we have developed guidance statements for the investigation and management of adult IIH.

An unusual case of leptomeningeal carcinomatosis in a patient with primary adenocarcinoma of the lung.

A 72-year-old man was brought to the emergency department with acute onset confusion and haemoptysis. Chest X-ray showed a possible lung mass, while CT head showed a fluid-filled, space-occupying lesion (SOL) in the right frontal lobe of the brain. MRI head indicated that this SOL had spilt its contents into the subarachnoid and intraventricular spaces. Due to a fluctuating Glasgow Coma Scale (GCS), the patient underwent emergency debulking. Macroscopically, a frail-walled cystic tumour filled with straw-coloured fluid was noted; histology confirmed metastasis from a primary lung adenocarcinoma. Whole brain radiotherapy was given, with a view to commence systemic therapy. The patient, however, deteriorated and unfortunately passed away a few weeks after completing radiotherapy. This patient presented with leptomeningeal metastasis as the first presentation of a lung adenocarcinoma, and had a highly unusual mechanism by which leptomeningeal spread had occurred, with metastatic brain tumour spilling its contents into the meningeal spaces.

Surgery for Acute Subdural Hematoma: Replace or Remove the Bone Flap?

OBJECTIVE: In surgery for acute subdural hematoma (ASDH), the bone flap can be fixed onto the skull, left riding to provide partial skull decompression, or removed. This study assessed whether removing the bone flap improved outcome. METHODS: We conducted an observational study on consecutive patients who were operated for ASDH in our hospital from July 2011 to June 2014. We retrospectively collected data on demographics, injury severity, and predicted and observed outcomes at 6 months. The cohort was divided into 2 groups based on whether the bone flap was replaced (fixed and riding flap craniotomy) or removed (decompressive craniectomy). The differences in functional status, postoperative control of intracranial hypertension, and number of cranial reoperations were analyzed. A subgroup analysis compared decompressive craniectomy with riding flap craniotomy alone. RESULTS: Data were obtained for 99 patients; 69 had decompressive craniectomy, 17 had riding flap craniotomy, and 13 had fixed flap craniotomy. The decompressive craniectomy group had statistically worse injuries, more predicted poor outcomes (69% vs. 57%, P = 0.013), more observed poor outcomes (59% vs. 37%, P = 0.037), equivalent control of intracranial hypertension, and a similar number of reoperations compared with the craniotomy groups. Subgroup analysis between decompressive craniectomy and riding flap craniotomy showed no significant difference in baseline characteristics and outcomes. CONCLUSIONS: Our study concluded that removing the bone flap after ASDH evacuation was not associated with a better outcome. We recommend replacing the bone flap if brain conditions allow. Further research is required to evaluate the role of skull decompression in surgery for ASDH.

Heavier-than-water silicone oil mixture as a long-term tamponade agent: a pilot study.

PURPOSE: To evaluate the tamponade effect on the retina of a heavier-than-water silicone oil mixture and to compare it with the effect of silicone oil. METHODS: Prospective, non-randomised, comparative pilot study. Phakic/pseudophakic patients with retinal detachment undergoing vitrectomy with Densiron 68 or silicone oil were recruited. The 'separation volume', defined as the relative volume of the space between intraocular tamponade agent and retina, was estimated using magnetic resonance imaging in both groups and compared. RESULTS: Nine participants were included; 4 received silicone oil and 5 Densiron 68. The mean separation volume was statistically significantly larger in the silicone oil group (0.477 ± 0.419 cm(3)) than in the Densiron group (0.042 ± 0.013 cm(3); p = 0.014). CONCLUSIONS: In this study Densiron achieved an excellent tamponade effect in the retina.