The Current State of Neurosurgery in Iceland.

BACKGROUND: Although surgical conditions account for 32% of the global burden of diseases, approximately 5 billion people worldwide lack access to timely and affordable, surgical and anesthetic services. Disparities in access to surgical care are most evident in low- and middle-income countries, often resulting from a lack of surgical infrastructure. However, the establishment of surgical infrastructure, particularly for specialty surgical services including neurosurgery, is challenging in countries with small populations, irrespective of income classification, due to the distribution of high costs among a lesser number of individuals. One such nation is Iceland. Despite high-income status, high quality of life, literacy, and educational attainment, the population of Iceland has often lacked access to local neurosurgical care, with the establishment of the domestic neurosurgical system in 1971 and continued externalization of complex neurosurgical procedures to neighboring nations and neurosurgeons. METHODS: A narrative review was conducted. RESULTS: This article provides the first-ever examination of neurosurgery in Iceland. We discussed the history and the social, political, and economical contexts in Iceland. We examined the history of neurosurgery in Iceland, which provided brief biographic sketches of pioneers who have catalyzed the establishment of neurosurgical care and training in Iceland, and characterize the current state of neurosurgery in Iceland. CONCLUSIONS: Recommendations derived from the experiences of Icelandic neurosurgeons may guide the international community in future initiatives.

[The incidence of sinus thrombosis in Iceland 2008-202].

BACKGROUND: Cerebral venous sinus thrombosis (CSVT) is the cause of 0.5%-1% of all strokes. CSVT can cause haemorrhage, cerebral infarction and increased intracranial pressure. Due to a variety of symptoms, CSVT can be difficult to diagnose. The purpose of this study was to examine the incidence of CSVT in Iceland 2008-2020, risk factors, symptoms, treatment and outcome. METHODS: A retrospective reviewing of medical records of those diagnosed with CSVT from 1. January 2008 to 31. December 2020, was performed. Sex, age at diagnosis, symptoms, known risk factors, imaging results, treatment and outcome were studied. Statistical processing was performed with Excel and Rstudio. RESULTS: Overall, 31 patients were diagnosed with CSVT (22 women). The mean incidence was 0.72/100.00 per year. The mean age was 34.3 years (14-63 years). The most common symptom was headache (87%), other symptoms included focal symptoms and seizures. The most common risk factor was the use of oral contraceptives (73%). Four patients had no risk factor. The most commonly affected sinus was the transverse-sinus (74%). All patients were treated with anticoagulants. Most received heparin or low-molecular-weight heparin then succeeded by warfarin or NOACs. Three months after diagnosis, 87% of the patients scored 0-2 on the modified Rankin Scale. One patient died as a result of CSVT. DISCUSSION: The incidence of CSVT in Iceland is in accordance with other studies. Headache was the most common symptom and oral contraceptives the most common risk factor among women. Most patients made a good recovery, which suggests a timely diagnosis and appropriate treatment for CSVT in Iceland.

Rare SLC13A1 variants associate with intervertebral disc disorder highlighting role of sulfate in disc pathology.

Back pain is a common and debilitating disorder with largely unknown underlying biology. Here we report a genome-wide association study of back pain using diagnoses assigned in clinical practice; dorsalgia (119,100 cases, 909,847 controls) and intervertebral disc disorder (IDD) (58,854 cases, 922,958 controls). We identify 41 variants at 33 loci. The most significant association (ORIDD = 0.92, P = 1.6 × 10-39; ORdorsalgia = 0.92, P = 7.2 × 10-15) is with a 3'UTR variant (rs1871452-T) in CHST3, encoding a sulfotransferase enzyme expressed in intervertebral discs. The largest effects on IDD are conferred by rare (MAF = 0.07 - 0.32%) loss-of-function (LoF) variants in SLC13A1, encoding a sodium-sulfate co-transporter (LoF burden OR = 1.44, P = 3.1 × 10-11); variants that also associate with reduced serum sulfate. Genes implicated by this study are involved in cartilage and bone biology, as well as neurological and inflammatory processes.

Intraventricular Vancomycin Treatment for Shunt-related Ventriculitis Caused by Methicillin-resistant Staphylococcus Aureus in a Preterm Infant: A Case Report.

Posthemorragic hydrocephalus is a relatively common condition in prematures, often requiring ventriculoperitoneal shunts. We report a case of methicillin-resistant Staphylococcus aureus infection of a ventriculoperitoneal shunt in a premature neonate which failed conventional intravenous treatment. Despite the absence of published guidelines, we used available data and expert advice to treat the patient with intraventricular vancomycin. The treatment was successful in eradicating the infection without observed toxicity. This case highlights the need for international guidelines on intraventricular treatment for neonates.

[Obesity isn't always obesity: Cushing's disease - case report]. / Offita er ekki alltaf offita: Cushing-sjúkdómur, sjúkratilfelli

Woman in her thirties presented to the emergency room with a two-week history of worsening headache and diplopia. For eight years she had suffered from progressive weight gain, diabetes and hypertension that didn't improve with lifestyle modification. A lumbar puncture demonstrated increased intracranial pressure and MRI a pituitary adenoma. Physical examination was consistent with Cushing's syndrome and endocrine workup confirmed Cushing's disease. Treatment was complex, including unsuccessful pituitary surgery and gamma knife radiosurgery, and eventually bilateral adrenalectomy with subsequent development of Nelsons syndrome. This case illustrates the diagnostic delay that many patients with CD suffer from.

[Occlusion of the vertebrobasilar artery. Case presentation and literature review]. / Lokun í botn- og hryggslagæð heila. Sjúkratilfelli og yfirlit

This paper is a case report of a 22 year old, previously healthy woman that presented comatose to the Emergency Room at Landspitali University Hospital Iceland. A CT image of the head on admission revealed a large right cerebellar infarct with oedema compressing the fourth ventricle. A CT angiogram on admission was suspicious for a dissection of the left vertebral artery (confirmed during endovascular treatment) and a total occlusion of the distal third of the basilar artery. Thrombolytic therapy with t-PA was initiated followed by thrombectomy with good recanalization. The following day the patient underwent suboccipital craniotomy for malignant cerebellar infarction. She made a good clinical recovery to a modified Ranking scale of 1 at 90 days after discharge from the hospital. Following the case is a literature review on the clinical aspects of occlusion of the vertebrobasilar system, use and utility of imaging and treatment with (anticoagulation, IV and IA thrombolysis) modalities that have been tried. Finally, the evidence regarding thrombectomy and the role of craniotomy for malignant stroke are reviewed.

[Reversible cerebral vasoconstriction syndrome - a common cause of thunderclap headache].

Reversible cerebral vasoconstriction is characterized by thunderclap headache and vasoconstriction of cerebral arteries, with or without focal neurologic symptoms. The syndrome is three times more common in women with a mean age around 45 years. In approximately 60% of cases a cause can be identified, commonly after intake of vasoactive substances. The pathophysiology of reversible cerebral vasoconstriction syndrome is unknown, though temporary dysregulation in cerebral vascular tone is thought to be a key underlying mechanism. The syndrome typically follows a benign course; however, complications such as ischemic stroke or intracranial hemorrhage can cause permanent disability or death in a small minority of patients. Vascular imaging reveals alternating cerebral vasoconstriction and vasodilation that normalizes within 12 weeks. Calcium channel antagonists such as nimodipine reduce the frequency of thunderclap headaches but do not decidedly affect the risk of cerebral ischemia or hemorrhage. In this article the epidemiology, risk factors, pathophysiology, symptoms, diagnosis and treatment of RCVS is reviewed.

Preparation and Culturing of Human Primary Vascular Cells.

Cerebral amyloid angiopathy (CAA) results from amyloid accumulation within arteries of the cerebral cortex and leptomeninges. This condition is age-related, especially prevalent in Alzheimer's disease (AD), and the main feature of certain hereditary disorders (i.e., HCHWA-I). The vascular smooth muscle cells (VSMCs) appear to play a vital role in the development of CAA, which makes them well suited as an experimental model to study the disease and screen for possible remedies. We describe two different methods for isolating and culturing human VSMCs: First, using the human umbilical cord as an easy source of robust cells, and secondly, using brain tissue that provides the proper cerebral VSMCs, but is more problematic to work with. The umbilical cord also provides human umbilical vascular endothelial cells (HUVEC), useful primary cells for vascular research. Finally, the maintenance, preservation, and characterization of the isolated vascular cells are described.

Hypopituitarism 3 and 12 months after traumatic brain injury and subarachnoid haemorrhage.

OBJECTIVE: High prevalence of hypopituitarism (HP) has been reported after traumatic brain injury (TBI) and subarachnoid haemorrhage (SAH). The aim of the study was to prospectively evaluate the prevalence and progression of HP in patients after TBI and SAH in Icelandic population. DESIGN: A 12 month prospective single-centre study. METHODS AND PROCEDURES: A total of 27 patients were included, 15 patients with TBI and 12 patients with SAH. Pituitary function was evaluated with baseline hormone measurements and diagnostic tests. An insulin tolerance test was used unless contraindicated, then the GHRH-arginine test and Synachten test were used. RESULTS: At three months, 16.7% (2/12) of the patients had HP after TBI and 33.3% (4/12) after SAH. At 12 months, 21.4% (3/14) of patients had HP after TBI and 9.1% (1/11) after SAH. Gonadotropin deficiency was the most common deficiency at 3 months and GH and gonadotropin deficiency at 12 months. CONCLUSIONS: There is a considerable risk of HP after TBI and reason to study pituitary function further in patients with SAH. We believe that neuroendocrine evaluation is important in these patients. Since recovery commonly occurs 12 months after the event, evaluation should be performed after that time if not clinically indicated earlier.

New Directions in 3D Medical Modeling: 3D-Printing Anatomy and Functions in Neurosurgical Planning

This paper illustrates the feasibility and utility of combining cranial anatomy and brain function on the same 3D-printed model, as evidenced by a neurosurgical planning case study of a 29-year-old female patient with a low-grade frontal-lobe glioma. We herein report the rapid prototyping methodology utilized in conjunction with surgical navigation to prepare and plan a complex neurosurgery. The method introduced here combines CT and MRI images with DTI tractography, while using various image segmentation protocols to 3D model the skull base, tumor, and five eloquent fiber tracts. This 3D model is rapid-prototyped and coregistered with patient images and a reported surgical navigation system, establishing a clear link between the printed model and surgical navigation. This methodology highlights the potential for advanced neurosurgical preparation, which can begin before the patient enters the operation theatre. Moreover, the work presented here demonstrates the workflow developed at the National University Hospital of Iceland, Landspitali, focusing on the processes of anatomy segmentation, fiber tract extrapolation, MRI/CT registration, and 3D printing. Furthermore, we present a qualitative and quantitative assessment for fiber tract generation in a case study where these processes are applied in the preparation of brain tumor resection surgery.

New Directions in 3D Medical Modeling: 3D-Printing Anatomy and Functions in Neurosurgical Planning.

This paper illustrates the feasibility and utility of combining cranial anatomy and brain function on the same 3D-printed model, as evidenced by a neurosurgical planning case study of a 29-year-old female patient with a low-grade frontal-lobe glioma. We herein report the rapid prototyping methodology utilized in conjunction with surgical navigation to prepare and plan a complex neurosurgery. The method introduced here combines CT and MRI images with DTI tractography, while using various image segmentation protocols to 3D model the skull base, tumor, and five eloquent fiber tracts. This 3D model is rapid-prototyped and coregistered with patient images and a reported surgical navigation system, establishing a clear link between the printed model and surgical navigation. This methodology highlights the potential for advanced neurosurgical preparation, which can begin before the patient enters the operation theatre. Moreover, the work presented here demonstrates the workflow developed at the National University Hospital of Iceland, Landspitali, focusing on the processes of anatomy segmentation, fiber tract extrapolation, MRI/CT registration, and 3D printing. Furthermore, we present a qualitative and quantitative assessment for fiber tract generation in a case study where these processes are applied in the preparation of brain tumor resection surgery.

Thyrotoxicosis in a 13-year-old girl following pituitary adenectomy for Cushing's disease.

Our objective is to report a case of thyrotoxicosis following pituitary adenectomy for Cushing's disease, the only pediatric case to our knowledge. No thyroid antibodies were detected, and the thyrotoxicosis was successfully treated for 3 months with no relapse after 5 years of follow-up. The cause of thyrotoxicosis remains unknown.

Sequence variant at 8q24.21 associates with sciatica caused by lumbar disc herniation.

Lumbar disc herniation (LDH) is common and often debilitating. Microdiscectomy of herniated lumbar discs (LDHsurg) is performed on the most severe cases to resolve the resulting sciatica. Here we perform a genome-wide association study on 4,748 LDHsurg cases and 282,590 population controls and discover 37 highly correlated markers associating with LDHsurg at 8q24.21 (between CCDC26 and GSDMC), represented by rs6651255[C] (OR=0.81; P=5.6 × 10-12) with a stronger effect among younger patients than older. As rs6651255[C] also associates with height, we performed a Mendelian randomization analysis using height polygenic risk scores as instruments to estimate the effect of height on LDHsurg risk, and found that the marker's association with LDHsurg is much greater than predicted by its effect on height. In light of presented findings, we speculate that the effect of rs6651255 on LDHsurg is driven by susceptibility to developing severe and persistent sciatica upon LDH.

Extramedullary Cavernous Hemangioma with Intradural and Extradural Growth and Clinical Symptoms of Brown-Séquard Syndrome: Case Report and Review of the Literature.

BACKGROUND: Primary spinal tumors are rare. Symptoms depend on the size and location of the tumor. CASE DESCRIPTION: A patient presented with a rare clinical finding, Brown-Séquard syndrome. The symptoms were caused by an extramedullary tumor compressing on the thoracic spinal cord. Pathologic examination showed cavernous hemangioma with growth both intradurally and extradurally. CONCLUSIONS: This is an extremely rare finding; to our knowledge, only 1 case report has been published before in which a spinal cavernous hemangioma had intradural and extradural growth. The clinical symptoms of Brown-Séquard syndrome have not been described before in the findings of spinal cavernous hemangiomas.

[Cerebral arteriovenous malformations - overview].

UNLABELLED: Cerebral arteriovenous malformations (AVMs) are uncommon but can cause intracerebral hemorrhage with grave disability or death. AVMs can even cause focal neurological symptoms, seizures and headache. The treatment of AVMs is complex. The most common treatment forms are microsurgery, stereotactic radiotherapy and endovascular embolization. The best treatment in each case can include a combination of the mentioned treatment forms. New studies indicate that no intervention is the best option in unruptured AVMs. In this article we discuss the epidemiology, diagnosis and treatment of cerebral AVMs. KEY WORDS: cerebral arteriovenous malformations, intracerebral hemorrhage, seizures, headache, surgery, radiation, endovascular embolization. Correspondence: Olafur Sveinsson, olafur.sveinsson@karolinska.se.

[Superior canal dehiscence - Case report].

UNLABELLED: Superior canal dehiscence is a rare syndrome. The symptoms consist of hearing loss, dizziness and autophonia. The patient can be cured with surgery. A 28 year old woman went to several doctors for several months due to diminished hearing, dizziness and autophonia. The symptoms got worse. A work-up led to the diagnosis of superior canal dehiscence in the left ear. She underwent surgery and her symptoms improved. A latency in diagnosis is expected as the syndrome is rare. It's important to think of superior canal dehiscence when patients complain of these symptoms. KEY WORDS: superior canal dehiscence, autophonia, dizziness, hearing deficit. Correspondence: Bryndis Baldvinsdottir, bryndisbaldvins@gmail.com.

[Spontaneus intracerebral haemhorrhage--review]. / Sjálfsprottin heilavefsblæding--yfirlitsgrein.

Spontaneous intracerebral hemorrhage occurs when a blood vessel within the brain parenchyma ruptures without a near related trauma. It is the second most common form of stroke, accounting for approximately 10% to 15% of new strokes. The 30 day mortality is very high (25-50%). Hypertension is the most common cause. Unfortunately, surgery has not proven to be helpful except in certain exceptions such as in large cerebellar hemorrhage. Nonetheless, it is very important that patients with ICH are admitted to an intensive care or a stroke unit with close surveillance of consciousness, focal neurologic symptoms, blood pressure and fluid balance.