[The hereditary vessel disease CADASIL].

Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a hereditary small vessel disease of the brain characterized by progressive white matter lesions, subcortical infarcts, and cognitive decline. This autosomal dominant disorder is caused by mutations in the NOTCH3 gene located on chromosome 19, resulting in the accumulation of granular osmiophilic material within the walls of small arteries and arterioles. Clinically, CADASIL typically manifests in mid-adulthood with recurrent ischemic events, migraine with aura, mood disturbances, and cognitive impairment. Neuroimaging plays a crucial role in the diagnosis of CADASIL, with characteristic findings including white matter hyperintensities particularly in the anterior temporal lobe and external capsule.

[Transient Global Amnesia in the Reykjavik area].

BACKGROUND: Transient Global Amnesia (TGA) is a benign syndrome characterized by sudden anterograde memory loss, that resolves spontaneously within 24 hours. TGA appears without other focal neurological symptoms. The aim of this study was to study TGA in the greater Reykjavik-area. METHODS: We retrospectively analysed the medical history of patients with a diagnosis of TGA (ICD-10 G45.4) at the University Hospital in Iceland in 2010-2021. Medical records were reviewed, and information about year and age at diagnosis, sex, symptoms, precipitating events, imaging results and risk factors were collected. Statistical processing was performed with Excel and Rstudio. RESULTS: Overall, 348 attacks of TGA were identified with a mean frequency of 29 attacks/year, where 9.9% had an earlier history of TGA. The mean age was 64.1, with 50% of subjects between 58-70 years old. The sex distribution was equal (49.9% female). Possible precipitating events were found in 53.7% of cases, with physical activity being the most common one (24.4%), followed by sudden temperature change and emotional stress. In 96% of patients a computerized tomography was performed (no sign of acute changes were found), and magnetic resonance imaging (MRI) in 36.2% of cases. MRI showed restricted diffusion in the hippocampal area in 10.3% of cases. DISCUSSION: TGA is not a rare but a benign syndrome. Our findings regarding age, sex distribution and precipitating events were in accordance with other studies. TGA is thought to result from a temporary hippocampal dysfunction supported by the clinical presentation and MRI findings. The cause of TGA is however still unknown.

[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.

[Syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis ­ two cases and review]

Here we describe two cases of HaNDL (Headache with Neurological Deficits and cerebrospinal fluid Lymphocytosis). A thirty year old man with episodes of headache with lateralizing symptoms and confusion and a 41 year old man with headache, aphasia and right hemiparesis. Symptoms resolved completely in both patients. Considerable cerebrospinal fluid lymphocytosis was present but no signs of CNS infection and MRIs of the brain were normal. Although the cause of HaNDL is unknown, it is thought to be triggered by a viral infection by some. The prognosis is excellent and symptoms normally resolve within 1-3 weeks. It is important to rule out more serious etiologies like stroke, subarachnoid hemorrhage or central nervous system infections.

Long-term use of rituximab increases T cell count in MS patients.

Rituximab has been used to treat MS patients in Iceland for over a decade. However, long-term effect of rituximab on leukocyte populations has not yet been elucidated. By retrospective analysis of flow cytometric data from 349 patients visiting the neurological ward at The National University Hospital of Iceland from 2012 to 2023 for rituximab treatment, the long-term effect of rituximab and whether the effect was dose dependent (1000mg vs 500mg) was evaluated. No difference was detected in efficacy of B cell depletion in patients treated with 500mg as an initial dose of rituximab when compared to 1000mg. Long-term use of rituximab led to an increase in T cell count (p=0,0015) in patients receiving 3-8 doses of rituximab (1.5-8 years of treatment). The increase occurred in both CD4+ (p=0,0028) and CD8+ T cells (p=0,0015) and led to a decrease in the CD4/CD8 ratio (p=0,004). The most notable difference lies in reshaping the balance between näive and effector CD8+ T cells. The clinical implications of long-term treatment with rituximab and its effect on the T cell pool needs to be explored further. Since no difference in B cell depletion was detected between the two patient groups, 1000mg as an initial dose might be excessive, suggesting a personalized dosing regimen might have therapeutic and financial advantages.

Trigeminal neuralgia and its comorbidities: a nationwide disease trajectory study.

ABSTRACT: There is a limited understanding of risk factors and comorbidities in trigeminal neuralgia, a disease characterized by paroxysms of severe unilateral facial pain and a higher incidence in women. We aim to identify temporally associated comorbidities involving trigeminal neuralgia by analyzing nationwide disease trajectories. Using data from 7.2 million unique individuals in the Danish National Patient Register between 1994 and 2018, each individual diagnosed with trigeminal neuralgia was compared with 10,000 matched controls to identify co-occurring diseases. The sequential disease associations were identified in sex-stratified disease trajectories. A Cox-regression analysis investigated whether treatment with carbamazepine or oxcarbazepine, as compared with gabapentin, pregabalin, or lamotrigine, was associated with stroke risk. Finally, we investigated the stroke polygenic risk score and its association with stroke incidence in a subset of genotyped individuals with trigeminal neuralgia. We included 7141 individuals with trigeminal neuralgia (64.2% female, mean age at diagnosis 58.7 years) and identified 18 diseases associated with subsequent trigeminal neuralgia. After diagnosis, trigeminal neuralgia was associated with 9 diseases, including ischemic stroke (relative risk 1.55). Carbamazepine or oxcarbazepine treatment increased the ischemic stroke risk (hazard ratio 1.78; 95% confidence interval 1.47-2.17); however, the polygenic risk of stroke showed no association. In the Danish population, a trigeminal neuralgia diagnosis is temporally associated with 27 diseases revealed in systematic disease trajectories. Trigeminal neuralgia itself and its first-line treatment, but not a stroke polygenic risk score, was associated with an increased risk of ischemic stroke indicating that vascular risk factors should be routinely assessed in individuals with trigeminal neuralgia.

[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.

[Cerebral ischemia/infarction - epidemiology, causes and symptoms]. / Heilablóðþurrð/heiladrep: Faraldsfræði, orsakir og einkenni.

Eight of ten strokes are due to cerebral ischemia, two from cerebal hemorrhage. Stroke is the most common cause of disability, the second commonest cause of dementia and the fourth commonest cause of death in the developed world. The incidence of stroke is 150-200/100.000 individuals/ year. One of every seven individuals suffers from stroke in their lifetime. In this article the epidemiology, risk factors, pathophysiology and symptoms of cerebral ischemia will be reviewed.

[Cerebral ischemia/infarction - diagnosis and treatment]. / Heilablódþurrd/-drep - greining og medferd.

Four hundred individuals suffer from ischemic stroke every year in Iceland, more than one daily. Cerebral ischemia is an emergency. Around two million brain cells die every minute after an occlusion of a cerebral artery. Therefore, it is of utmost importance that the patient is transported quickly to hospital, not least to receive thrombolytic treatment. Even though thrombolytic treatment can be given up to four and a half hours after the ictal event, time is brain and the effect of thombolysis reduces dramatically as times passes. For every two patients who are treated inside one hour, one recovers fully. When the treatment is administred between three and four and a half hours only one in 14 recovers fully. All patients with an acute stroke should be admitted to a stroke unit where a multidisciplinary focus on stroke causation and treatment is present, with emphasis on early rehabilitation. Secondary preventive treatment focusing on anti-thrombotic, hypertensive, diabetic, cholesterol lowering treatment, carotic endarterectomy and life style changes should be initiated as soon as possible.

[Cerebral sinus thrombosis - an uncommon but important differential diagnosis to headache, stroke and seizures. Cases and overview]. / Stokkasegi - sjaldgæfur sjúkdómur en mikilvæg mismunagreining við höfuðverk, heilablóðfall og flog. Tilfellaröð með yfirliti.

Thrombosis of the cerebral veins and sinuses is an unusual but important cause of increased intracranial pressure and stroke, especially in the young and middle aged. Pregnant women, especially during the puerperium, and individuals with thrombophilia are a special risk group. What makes the diagnosis difficult is the vast range of symptoms including: headache, nausea, vomiting, blurry vision, reduction of consciousness, aphasia and motor and sensory disturbances. We present four cases which reflect the diverse clinical presentation of the disease.

[Brain abscess - overview]. / Heilaigerd - yfirlitsgrein.

Brain abscess is a life threatening illness, demanding rapid diagnosis and treatment. Its development requires seeding of an organism into the brain parenchyma, often in an area of damaged brain tissue or in a region with poor microcirculation. The lesion evolves from a cerebritis stage to capsule formation. Brain abscesses can be caused by contiguous or haematogenous spread of an infection, or by head trauma/ neurosurgical procedure. The most common presentation is that of headache and vomiting due to raised intracranial pressure. Seizures have been reported in up to 50% of cases. Focal neurological deficits may be present, depending on the location of the lesion. Treatment of a brain abscess involves aspiration or excision, along with parenteral antibiotic therapy. The outcome has improved dramatically in the last decades due to improvement in diagnostic techniques, neurosurgery, and broad-spectrum antibiotics. The authors provide an overview of the pathogenesis, diagnosis and management of brain abscesses.

[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.

[Restless legs syndrome - a review]. / Fótaóeird - yfirlitsgrein.

UNLABELLED: Restless legs syndrome (RLS) is a common disorder with a prevalence between 10-20% in Iceland. There are two forms of RLS, idiopathic and secondary. Symptom onset of RLS before the age of 45 suggests an idiopathic form with no known underlying cause but inheritance. Symptom onset after age of 45 indicates a secondary form with an underlying cause without inheritance. Causes for secondary forms are for example: iron depletion, uraemia and polyneuropathy. Symptoms of RLS are uncomfortable and unpleasant deep sensations in the legs that are felt at rest, accompanied by an urge to move the legs, typically just before sleep. Accompanying RLS is a sleep disturbance that can lead to daytime somnolence, decreased quality of life, poor concentration, memory problems, depression and decreased energy. Dopamine agonists are currently the first line treatment for RLS. KEYWORDS: restless legs, periodic limb movements, sleep disturbance, dopamine agonists.

[Cervical artery dissection - review]. / Flysjun í slagæðum á hálsi - yfirlitsgrein.

In recent years carotid and vertebral artery dissections have been diagnosed more frequently, probably because new imaging techniques are more reliable and they are certainly less invasive. The cause of cervical artery dissections is largely unexplained but probably involves a combination of genetic and environmental factors such as trauma or infection. Most authors recommend intravenous heparin or low molecular weight heparin followed by oral warfarin to maintain INR between 2-3 for 3-6 months. If the artery has healed after 3-6 months of anti-coagulation all treatment can be stopped but if there is a remaining stenosis the patient can be put on aspirin 75-100 mg a day. The long-term prognosis of cervical artery dissection is favourable in the majority of patients. New dissections are uncommon.

[Spontaneous subarachnoid haemorrhage--review]. / Sjálfsprottin innanskúmsblæding--yfirlitsgrein.

Spontaneous subarachnoid hemorrhage is a bleeding in to the subarachnoid space without trauma. Aneurysms are the underlying cause in 80% of the cases. Among other causes are: arteriovenous malformations, anticoagulation, vasculitis or brain tumor. Spontaneous subarachnoid hemorrhage is a serious disease, where up to half of the patients die. Of those who survive, only half return to work and many have a reduced quality of life. To prevent rebleeding the aneurysm is closed either with endovascular coiling or neurosurgical clipping.

[Health anxiety - increased knowledge and treatment options]. / Heilsukvidi - aukin pekking og medferdarmoguleikar.

Patients with hypochondriasis are preoccupied with the fear or belief that they have a serious, undiagnosed disease. This concern derives from misinterpretations of benign physical sensations, and persists despite appropriate reassurance to the contrary. They have, on average, disproportionately high rates of visits to physicians, specialty consultations, laboratory tests, and surgical procedures, as well as high health care costs. Despite this extensive medical attention, they find their care unsatisfactory, which is understandable, as convincing alternative explanations to their ailments are repeatedly delayed. Physicians, in turn, may feel discouraged and frustrated in relation to these individuals where their best efforts to help often prove ineffective or are even rejected. Recent scientific studies have shown that cognitive behavioural therapy (CBT) and selective serotonin reuptake inhibitors (SSRI) are effective treatment options for health anxiety as demonstrated in clinical trials.

[Conversion disorder - review]. / Hugbrigdaröskun - yfirlitsgrein.

In modern medicine the term "functional symptoms" is usually used to refer to symptoms where an organic cause cannot be found. Studies have shown that up to half of all patients consulting their family physician and approximately one third of all those attending neurology outpatient clinics present with such symptoms. These patients commonly go between doctors, repeatedly undergo unnecessary tests, even surgery, and various drugs are tried with limited success. These problems tend to be prolonged and greatly reduce the quality of life for the patients involved. Both the DSM IV and ICD 10 classifications include a group for the so-called medically unexplained disorders. Among these disorders is conversion disorder where patients present with neurological symptoms, affecting motor or sensory function, but with no neurological explanation. Here we provide an overview of the current ideas on the aetiology, diagnosis, treatment and prognosis of conversion disorder.

[Central and extrapontine myelinolysis following correction of extreme hyponatremia. Case report and review of the literature]. / Midbrúar- og utanbrúarafmýling í kjölfar leidréttingar svaesinnar blódnatríumlaekkunar. Sjúkratilfelli og yfirlitsgrein.

We report a case of a 43-year-old woman who developed osmotic demyelination syndrome following correction of extreme hyponatremia that was considered to be of chronic nature. The serum sodium level was 91 mmol/L on admission to hospital. It was decided to correct the serum sodium slowly with the goal that the rate of correction would be no more than 12 mmol/l per 24 hours. This was achieved during the first two days of treatment but during the third day the rise in serum sodium was 13 mmol/l. On the 11th day of admission the patient had developed manifestations of pseudobulbar palsy and spastic quadriparesis. Magnetic resonance imaging study confirmed central and extrapontine myelonolysis. The patient received supportive therapy and eventually made full recovery. Current concepts in the pathophysiology of osmotic demyelination syndrome and the treatment of hyponatremia are reviewed. We recommend that the rate of correction of chronic hyponatremia should not exceed 8 mmol/l per 24 hours.

[Euthanasia--a moral choice?]. / Líknardráp--sidferdilegur valkostur?

Euthanasia has been heatedly discussed in Western countries over the last years. Only a few nations have legalized euthanasia or physician assisted suicide with the Dutch at the forefront of that field. Proponents of euthanasia mostly argue for euthanasia on two grounds. Firstly, that the patient has a right to die and secondly, that there is no substantial difference between euthanasia and palliative care. In this paper I will argue against both of the above. I discuss the arguments against euthanasia which are in principle four. Firstly, it is held by many that taking a human life is wrong under all circumstances. Secondly, that it is an unjustifiable demand to ask a person to take another person's life. In relation to that argument, euthanasia is not in accordance with the basic principles of medicine and nursing as they have evolved over the years and could therefore easily disrupt the therapeutic relationship. Thirdly, as shown from Holland there is empirical evidence that euthanasia is not under good enough surveillance and therefore invites misuse. Fourthly, even though euthanasia might possibly be justifiable under certain circumstances, legalisation might well invite abuse because of the message and pressure that the option places on both patients and professionals in terminal care. My answer to the euthanasia demand is palliative care, where dialogue between the patient and doctor is central. But the dialogue cannot be effective, unless both partners are willing and able to engage in sincere and frank conversations.

[Spontaneous intracranial hypotension--a case report and discussion]. / Sjálfsprottinn innankúpulágsrýstingur--sjúkratilfelli og umraeda.

Thirty-three year old woman came to the emergency room with 3 days' history of worsening headache which was relieved by lying down. Examination was normal. Magnetic Resonance Imaging (MRI) of the head showed an enhancement of the meninges. No pressure could be measured during lumbar puncture and cerebrospinal fluid (CSF) was obtained by elevating the patient's head. There was an elevation of protein in the CSF without signs of infection or inflammation. Computerised tomography (CT) myelogram showed a prominent leak from the 2nd right cervical nerve sheath. A blood patch was done at this level with some improvement of symptoms. The patient was readmitted four days later as the headache had worsened. A blood patch was repeated at the same level with limited results. Therefore a fibrinogen patch was done at the same level, of which the effect lasted only 24 hours. A repeated CT myelogram showed a leak from the left 8th cervical and 1st thoracic nerve sheets, but not from the 2nd right cervical nerve sheath. Blood and fibrinogen patches were done at these levels repeatedly with limited results. Therefore, a MRI of cervical-thoracic spine was done which showed signs of previous blood patch at the 1st left thoracic nerve sheath but no sign of a patch over the 8th left cervical nerve sheath. A fibrinogen patch was done at this level. The patient became symptom free and was finally discharged home. We present a case of complicated spontaneous intracranial hypotension and review of the literature.