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Objective: The most common genetic cause of amyotrophic lateral sclerosis (ALS) is the C9orf72 expansion. A high incidence of this expansion has been detected in Sweden and Finland. This Norwegian population-based study aimed to identify the prevalence, geographic distribution, ancestry, and relatedness of ALS patients with a C9orf72 expansion (C9 pos ). Further, we compared C9 pos and C9 neg patients' clinical presentation, family history of ALS and other neurodegenerative disorders, and sociodemographic status., Methods: We recruited ALS patients from all 17 Departments of neurology in Norway. Blood samples and questionnaires regarding clinical characteristics, sociodemographic status and family history of ALS, and other neurodegenerative disorders were collected. The C9orf72 expansion was examined for all patients., Results: The study enrolled 500 ALS patients, 8.8% of whom were C9 pos , with half being sporadic ALS cases. The proportion of C9 pos cases differed between regions, ranging from 17.9% in the Northern region to 1.9% in the Western region. The majority of C9 pos patients had non-Finnish European descent and were not closely related. C9 pos patients exhibited a significantly shorter mean survival time, had a higher frequency of relatives with ALS or dementia, and were more often unmarried/single and childless than C9 neg patients., Conclusion: C9 pos patients constitute a large portion of the Norwegian ALS population. Ancestry and relatedness do not adequately explain regional differences. Relying on clinical information to identify C9 pos patients has proven to be challenging. Half of C9 pos patients were reported as having sporadic ALS, underlining the importance of carefully assessing family history and the need for genetic testing.

Objective: There is still disagreement about whether to routinely use spectrophotometry to detect xanthochromia in cerebrospinal fluid (CSF) or whether visual inspection is adequate. We aimed to evaluate the diagnostic accuracy of these methods in detecting an aneurysmal subarachnoid hemorrhage in patients with sudden onset severe headache., Background: When a patient presents to the emergency department with a headache for which there is suspicion of a subarachnoid hemorrhage, the gold standard to rule this out is to perform a CSF analysis for xanthochromia with or without spectrophotometry if the cranial non-contrast computed tomography (CT) upon admission is negative., Methods: Having applied the gold standard, we retrospectively included patients with acute headache who underwent both CT scan and CSF spectrophotometry at our hospital in the period 2002-2020. Patients were excluded if the cranial CT was interpreted as positive, there was a bloody CSF, or if visual assessment data of the CSF was unavailable. We scrutinized the patients' medical records and evaluated the benefit of spectrophotometry compared to visual inspection. The net bilirubin absorbance cut-off for support of subarachnoid hemorrhage was set at >0.007 absorbance units. The spectrophotometry was also considered positive if the net bilirubin absorbance was ≤0.007 and net oxyhemoglobin absorbance was ≥0.1 absorbance units. We calculated and compared the sensitivity and specificity of CSF spectrophotometry and visual inspection of the CSF., Results: In total, 769 patients, with a mean age of 42.3 ± (standard deviation [SD] = 17.3) years, were included. The headache onset was classified as a thunderclap headache in 41.5%, and 4.7% had a sudden loss of consciousness. Fifteen patients (2%) were finally diagnosed with a subarachnoid hemorrhage, six (0.8%) had an aneurysmal subarachnoid hemorrhage, seven (0.9%) had a perimesencephalic hemorrhage, one (0.1%) had a cortical cerebral sinus venous thrombosis, and one (0.1%) had a spinal epidural hematoma. Four patients (0.5%) had a subarachnoid hemorrhage that was not detected by visual inspection, and two were caused by an aneurysmal rupture. One of these two patients died just before intervention, and the other underwent coiling for an anterior communicating aneurysm. The number needed for lumbar puncture to detect a subarachnoid hemorrhage was 51, but 128 to detect an aneurysmal hemorrhage. The corresponding numbers needed for CSF spectrophotometric analysis were 192 and 385, respectively. Spectrophotometry was positive in 31 patients (4.0%), of whom 18 (2.3%) also had visually detected xanthochromia (11 true positive). The mean net bilirubin absorbance in the 13 samples with visually clear CSF was 0.0111 ± (SD = 0.0103) absorbance units, compared to 0.0017 ± (SD = 0.0013) in the CSF with negative spectrophotometry. The corresponding figures for net oxyhemoglobin absorbance were 0.0391 ± (SD = 0.0522) versus 0.0057 ± (SD = 0.0081). The sensitivity of spectrophotometric xanthochromia detection was 100% (95% confidence interval [CI], 78-100), compared to 73% (95% CI, 45-92) for visual xanthochromia detection. The specificity of spectrophotometric xanthochromia detection was 98% (95% CI, 97-99) compared to 99% (95% CI, 98-100) for visual xanthochromia detection. Both methods had high negative predictive values: 100% (95% CI, 99.5-100) versus 99.5% (95% CI, 98.6-99.9), respectively., Conclusions: Both visual inspection and spectrophotometry have high diagnostic accuracy for detecting CSF xanthochromia, but the lower sensitivity of visual assessment makes it unreliable, and we recommend the use of spectrophotometry in clinical practice., (© 2024 The Author(s). Headache: The Journal of Head and Face Pain published by Wiley Periodicals LLC on behalf of American Headache Society.)

Introduction: Active patient participation is an important factor in optimizing post-stroke recovery, yet it is often low, regardless of stroke severity. The reasons behind this trend are unclear., Purpose: To explore how people who have suffered a stroke, perceive the transition from independence to dependence and whether their role in post-stroke rehabilitation influences active participation., Methods: In-depth interviews with 17 people who have had a stroke. Data were analyzed using systematic text condensation informed by the concept of autonomy from enactive theory., Results: Two categories emerged. The first captures how the stroke and the resultant hospital admission produces a shift from being an autonomous subject to "an object on an assembly line." Protocol-based investigations, inactivity, and a lack of patient involvement predominantly determine the hospital context. The second category illuminates how people who have survived a stroke passively adapt to the hospital system, a behavior that stands in contrast to the participatory enablement facilitated by community. Patients feel more prepared for the transition home after in-patient rehabilitation rather than following direct discharge from hospital., Conclusion: Bodily changes, the traditional patient role, and the hospital context collectively exacerbate a reduction of individual autonomy. Thus, an interactive partnership between people who survived a stroke and multidisciplinary professionals may strengthen autonomy and promote participation after a stroke.

Genetic repeat expansions cause neuronal degeneration in amyotrophic lateral sclerosis as well as other neurodegenerative disorders such as spinocerebellar ataxia, Huntington's disease and Kennedy's disease. Repeat expansions in the same gene can cause multiple clinical phenotypes. We aimed to characterize repeat expansions in a Norwegian amyotrophic lateral sclerosis cohort. Norwegian amyotrophic lateral sclerosis patients ( n = 414) and neurologically healthy controls adjusted for age and gender ( n = 713) were investigated for repeat expansions in AR , ATXN1 , ATXN2 and HTT using short read exome sequencing and the ExpansionHunter software. Five amyotrophic lateral sclerosis patients (1.2%) and two controls (0.3%) carried ≥36 repeats in HTT ( P = 0.032), and seven amyotrophic lateral sclerosis patients (1.7%) and three controls (0.4%) carried ≥29 repeats in ATXN2 ( P = 0.038). One male diagnosed with amyotrophic lateral sclerosis carried a pathogenic repeat expansion in AR , and his diagnosis was revised to Kennedy's disease. In ATXN1 , 50 amyotrophic lateral sclerosis patients (12.1%) and 96 controls (13.5%) carried ≥33 repeats ( P = 0.753). None of the patients with repeat expansions in ATXN2 or HTT had signs of Huntington's disease or spinocerebellar ataxia type 2, based on a re-evaluation of medical records. The diagnosis of amyotrophic lateral sclerosis was confirmed in all patients, with the exception of one patient who had primary lateral sclerosis. Our findings indicate that repeat expansions in HTT and ATXN2 are associated with increased likelihood of developing amyotrophic lateral sclerosis. Further studies are required to investigate the potential relationship between HTT repeat expansions and amyotrophic lateral sclerosis., Competing Interests: The authors report no competing interests., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Guarantors of Brain.)

Background: Migraine is a common disorder, particularly affecting women during their reproductive years. This female preponderance has been linked to exposure to female sex hormones., Methods: We used self-reported data from women born in 1943-1965 enrolled in the Norwegian Women and Cancer Study to examine the differences between women with migraine and women without migraine in a prospective design with respect to both endogenous and exogenous female sex hormone exposure., Results: In total, 62,959 women were included in the study, of whom 24.8% reported previous migraine (n = 15,635). Using a Cox proportional hazards model, we found that higher age at menarche reduced the risk of migraine (hazards ratio (HR) = 0.96, 95% confidence interval (CI) = 0.95-0.98) and that oral contraceptive use and parity increased the risk of migraine (HR = 1.12, 95% CI = 1.06-1.18 and HR = 1.37, 95% CI = 1.29-1.46, respectively)., Conclusions: Older age at menarche appears to reduce migraine risk, whereas oral contraceptive use and having children appear to increase the risk. Further research is required to investigate the causality of these associations., Competing Interests: Declaration of conflicting interestsThe authors declare that there are no potential conflicts of interest with respect to the research, authorship and/or publication of this article.

Migraine or migraine-like symptoms can contribute to a delayed stroke diagnosis. However, migraine with aura is a common stroke mimic and often the basis for acute thrombolytic therapy. It is probably also the reason why many patients are misdiagnosed with a transient ischemic attack. In this clinical review, we explain the factors that could differentiate a transient ischemic attack from a migraine with aura.

Background: Research on stroke rehabilitation often addresses common difficulties such as gait, balance or physical activity separately, a fragmentation contrasting the complexity in clinical practice. Interventions aiming for recovery are needed. The purpose of this study was to investigate effects of a comprehensive low-cost physical therapy intervention, I-CoreDIST, vs. usual care on postural control, balance, physical activity, gait and health related quality of life during the first 12 weeks post-stroke., Methods: This prospective, assessor-masked randomized controlled trial included 60 participants from two stroke units in Norway. Participants, who were randomized to I-CoreDIST ( n  = 29) or usual care physical therapy ( n  = 31), received 5 sessions/week when in-patients or 3 sessions/week as out-patients. Primary outcomes were the Trunk Impairment Scale-modified Norwegian version (TISmodNV) and activity monitoring (ActiGraphsWgt3X-BT). Secondary outcomes were the Postural Assessment Scale for Stroke, MiniBesTEST, 10-meter walk test, 2-minute walk test, force-platform measurements and EQ5D-3L. Stroke specific quality of life scale was administered at 12 weeks. Linear regression and non-parametric tests were used for statistical analysis., Results: Five participants were excluded and seven lost to follow-up, leaving 48 participants in the intention-to-treat analysis. There were no significant between-group effects for primary outcomes: TIS-modNV ( p  = 0,857); daily average minutes of sedative ( p  = 0.662), light ( p  = 0.544) or moderate activity ( p  = 0.239) and steps ( p  = 0.288), or secondary outcomes at 12 weeks except for significant improvements on EQ5D-3L in the usual care group. Within-group changes were significant for all outcomes in both groups except for activity levels that were low, EQ5D-3L favoring the usual care group, and force-platform data favoring the intervention group., Conclusions: Physical therapy treatment with I-CoreDIST improved postural control, balance, physical activity and gait during the first 12 weeks after a stroke but is not superior to usual care., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2022 Sivertsen, Arntzen, Alstadhaug and Normann.)

Background and Purpose: Physiotherapy, with an emphasis on high intensity, individually tailored, and person-centered treatment, is an effective route for recovery after a stroke. No single approach, however, has been deemed paramount, and there is limited knowledge about the patient experience of assessment, goal-setting, and treatment in physiotherapy. In this study, we seek to report patient experiences of I-CoreDIST-a new physiotherapy intervention that targets recovery-and those of usual care. The purpose is to investigate how individuals with stroke experience the bodily and interactive course of physiotherapy during their recovery process., Methods: A qualitative study, nested within a randomized controlled trial, consisting of in-depth interviews with 19 stroke survivors who received either I-CoreDIST or usual care. Data were analyzed using systematic text condensation, and this analysis was informed by enactive theory., Results: Interaction with the physiotherapist, which was guided by perceived bodily changes, fluctuated between being, on the one hand, formal/explicit and, on the other, tacit/implicit. The experiences of participants in the intervention group and the usual care group differed predominantly with regards to the content of therapy sessions and the means of measuring progress; divergences in levels of satisfaction with the treatment were less pronounced. The perception of positive bodily changes, as well as the tailoring of difficulty and intensity, were common and essential features in generating meaning and motivation. An embodied approach seemed to facilitate sense-making in therapy situations. In the interaction between the participants and their physiotherapists, trust and engagement were important but also multifaceted, involving both interpersonal skills and professional expertise., Conclusion: The embodied nature of physiotherapy practice is a source for sense-making and meaning-construction for patients after a stroke. Trust in the physiotherapist, along with emotional support, is considered essential. Experiencing progress and individualizing approaches are decisive motivators., (© 2022 The Authors. Physiotherapy Research International published by John Wiley & Sons Ltd.)

Rapid and thorough assessment of acute neuropsychiatric symptoms is essential for effective treatment. Here we describe a patient with a distinctive but relatively rare disease.

Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects motor neurons. In Europe, disease-causing genetic variants have been identified in 40-70% of familial ALS patients and approximately 5% of sporadic ALS patients. In Norway, the contribution of genetic variants to ALS has not yet been studied. In light of the potential development of personalized medicine, knowledge of the genetic causes of ALS in a population is becoming increasingly important. The present study provides clinical and genetic data on familial and sporadic ALS patients in a Norwegian population-based cohort., Methods: Blood samples and clinical information from ALS patients were obtained at all 17 neurological departments throughout Norway during a 2-year period. Genetic analysis of the samples involved expansion analysis of C9orf72 and exome sequencing targeting 30 known ALS-linked genes. The variants were classified using genotype-phenotype correlations and bioinformatics tools., Results: A total of 279 ALS patients were included in the study. Of these, 11.5% had one or several family members affected by ALS, whereas 88.5% had no known family history of ALS. A genetic cause of ALS was identified in 31 individuals (11.1%), among which 18 (58.1%) were familial and 13 (41.9%) were sporadic. The most common genetic cause was the C9orf72 expansion (6.8%), which was identified in 8 familial and 11 sporadic ALS patients. Pathogenic or likely pathogenic variants of SOD1 and TBK1 were identified in 10 familial and 2 sporadic cases. C9orf72 expansions dominated in patients from the Northern and Central regions, whereas SOD1 variants dominated in patients from the South-Eastern region., Conclusion: In the present study, we identified several pathogenic gene variants in both familial and sporadic ALS patients. Restricting genetic analysis to only familial cases would miss more than 40 percent of those with a disease-causing genetic variant, indicating the need for genetic analysis in sporadic cases as well., (© 2022 The Author(s). Published by S. Karger AG, Basel.)

Objective: We aimed to determine the effectiveness of natalizumab (NTZ) by assessing overall No Evidence of Disease Activity 3 (NEDA-3) in a local Norwegian cohort. Background: NTZ is an immunomodulating drug used in the treatment of multiple sclerosis (MS). It has typically been used as a second-line treatment, but certain patients with high disease activity have started directly with NTZ. Methods: This retrospective cohort study includes all patients who received NTZ for relapsing-remitting MS at Nordland Hospital in the period 2008-2018. In June 2019, status for every patient was assessed, and a survival curve was used to show the cumulative probability of achieving NEDA-3 over time. Results: The cohort consisted of 66 patients, 49 women and 17 men with a mean age of 40.0 ± 10.8 years. Each patient received on average 45.8 ± 36.4 NTZ infusions. Mean age and Expanded Disability Status Scale (EDSS) at first infusion was 34.8 ± 10.5 and 3.2 ± 1.9, respectively. Prior to NTZ treatment, 83% had used other disease modulating drugs and 65% were anti-JC virus (JCV) seronegative. During the study period, seven patients converted to seropositive. In 2019, 40 patients had switched or stopped treatment: 19 due to positive JCV serostatus, 9 due to disease activity, 7 due to adverse effects or complications (1 progressive multifocal leukoencephalopathy), 2 due to pregnancy, and 3 due to autologous hematopoietic cell transplantation abroad. Three patients experienced rebound in the wake of discontinuation (7.5%). Of the patients receiving NTZ for more than 3 years ( n = 33), 50% had achieved NEDA-3 after 3 years. Compared to those with evidence of disease activity (EDA), these NEDA-3 patients had significant lower EDSS score before first NTZ treatment ( p = 0.04). They were also slightly, but not significantly, younger at debut of their MS, at the diagnosis and at first NTZ treatment. Of all the patients who ever started on NTZ, 23% had achieved NEDA-3 5 years later. The mean EDSS in 2019 was 3.6 ± 2.5. Conclusion: Despite the high rate of treatment switch, mainly due to the risk of PML, almost one in four who started on NTZ achieved NEDA-3 after 5 years, and the overall disease progression was low in the total cohort. Treating less advanced disease seems to predict better long-term stability., Competing Interests: EB has received honoraria for giving lectures from Sanofi. KA has received honoraria for giving lectures from Biogen, Allergan, Roche, Teva, and Novartis. He has also served in an advisory board for Biogen Norway, and has enrolled patients in the tysabri observational program (TOP) study. The remaining author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Jaklin, Benjaminsen and Alstadhaug.)

Background: Knowledge of comorbid disorders is important to optimize therapy for multiple sclerosis (MS), but data are limited. The aim of this study was to assess comorbidity in persons with MS living in Nordland County on January 1, 2017., Methods: Data were retrieved from the Norwegian Patient Registry (2008-2017) and validated through review of electronic hospital charts (1970-2017). Comorbidity was defined as any distinct disorder, classified in the International Classification of Diseases (ICD-10), that had existed or occurred after the diagnosis of MS was established., Results: Data from 637 subjects were reviewed, and 97.5% were registered with at least one comorbid condition. Malignant melanoma was found in 0.5%, and non-melanoma skin cancers in 1.9%. In female subjects, breast cancer was found in 3.3%. Hypothyroidism was confirmed in 3.1%, type-1 diabetes in 0.3%, type-2 diabetes in 3.9%, psychosis in 0.6%, epilepsy in 2.8%, myocardial infarction in 1.7%, subarachnoid hemorrhage in 0.2%, cerebral infarction in 0.6%, pulmonary embolism in 0.9%, inflammatory bowel disease in 1.3%, and rheumatoid arthritis in 0.6%., Conclusion: Compared to reports from other Norwegian epidemiological studies, a higher proportion of inflammatory bowel disease and epilepsy was found. This is in accordance with findings from other studies. The prevalence of non-melanoma skin cancers was significantly higher than in the general Norwegian population as they were reported by The Cancer Registry of Norway., (Copyright © 2020. Published by Elsevier B.V.)

Objective: To assess the incidence of postdural puncture headache (PDPH) using 22-gauge atraumatic needle (Sprotte, 22GS) compared with 22-gauge traumatic needle (Quincke, 22GQ)., Background: Diagnostic lumbar puncture (dLP) is commonly complicated by PDPH. Despite evidence to support the use of 22GS, European neurologists seem to keep using 22GQ., Methods: This was a randomized, double-blind study. Adults (age: 18-60 years) scheduled for dLP were included. dLP and CSF acquisition were performed in accordance with highly standardized procedures. Patients were followed up on days 2 and 7., Results: In total, 172 patients were randomized and lumbar punctured, and 21 were excluded due to wrong inclusion (n = 11), needle switch (n = 7), failed dLP (n = 1), withdrawal (n = 1), and missed follow-up (n = 1). Among the remaining 151 patients (mean age: 40.7 ± 12.4 years), 77 had dLP using 22GQ and 74 using 22GS. Incidence of PDPH among patients punctured with 22GS (18%) was significantly lower (p = .004) than among patients punctured with 22GQ (39%). Relative risk was 0.45, 95% CI 0.26-0.80. Patients with PDPH had significantly lower weight (p = .035), and there was no significant difference related to age (p = .064), sex (p = .239), height (p = .857), premorbid episodic migraine (p = .829), opening pressure (p = .117), operators (p = .148), amount of CSF removed (p = .205), or number of attempts (p = .623)., Conclusions: The use of 22GS halves the risk of PDPH compared with 22GQ. This study provides strong support to make a change in practice where traumatic needles are still in regular use., (© 2020 The Authors. Brain and Behavior published by Wiley Periodicals LLC.)

Background: Room tilt illusion (RTI) is a rare symptom of higher vestibular dysfunction, consisting of a transient vertical rotation of the visual scene in the sagittal or coronal plane, most often 90 o or 180 o , without any alteration in shape, size and color of objects., Case Presentation: A 63-year-old woman with a history of hypertension and chronic obstructive pulmonary disease went through an uncomplicated aortobifemoral graft surgery due to aortoiliac occlusive disease. Post-operatively she experienced five episodes, lasting from 10 to 30 min, with RTI; 90 o forward rotation of the visual scene in the sagittal plane. Work-up revealed subclavian steal grade 3, and transient ischemia of the central vestibular system of the brainstem was the presumed mechanism., Conclusion: The course of episodic RTIs is often benign, but RTI may represent ischemia in the posterior cerebral circulation. Both stroke and otoneurologic workup are recommended. To our knowledge, this is the first case of RTI associated with subclavian steal reported.

Background: The actions of caffeine as an antagonist of adenosine receptors have been extensively studied, and there is no doubt that both daily and sporadic dietary consumption of caffeine has substantial biological effects on the nervous system. Caffeine influences headaches, the migraine syndrome in particular, but how is unclear. Materials and Methods: This is a narrative review based on selected articles from an extensive literature search. The aim of this study is to elucidate and discuss how caffeine may affect the migraine syndrome and discuss the potential pathophysiological pathways involved. Results: Whether caffeine has any significant analgesic and/or prophylactic effect in migraine remains elusive. Neither is it clear whether caffeine withdrawal is an important trigger for migraine. However, withdrawal after chronic exposure of caffeine may cause migraine-like headache and a syndrome similar to that experienced in the prodromal phase of migraine. Sensory hypersensitivity however, does not seem to be a part of the caffeine withdrawal syndrome. Whether it is among migraineurs is unknown. From a modern viewpoint, the traditional vascular explanation of the withdrawal headache is too simplistic and partly not conceivable. Peripheral mechanisms can hardly explain prodromal symptoms and non-headache withdrawal symptoms. Several lines of evidence point at the hypothalamus as a locus where pivotal actions take place. Conclusion: In general, chronic consumption of caffeine seems to increase the burden of migraine, but a protective effect as an acute treatment or in severely affected patients cannot be excluded. Future clinical trials should explore the relationship between caffeine withdrawal and migraine, and investigate the effects of long-term elimination., (Copyright © 2019 Alstadhaug and Andreou.)

Background: Health registries may yield important data for epidemiological studies. However, in order to be a valuable source for information, the registered data have to be correct., Objectives: The aim of the study was to validate data from the Norwegian Patient Registry (NPR) regarding multiple sclerosis (MS)., Materials and Methods: We obtained data on individuals residing in Nordland County and registered with a MS diagnosis in the NPR or in local hospital records. The NPR data included a unique 11-digit personal identity number that made it possible to identify the individuals medical records. For each individual registered with MS in the NPR, the hospital record was scrutinized in order to confirm or rule out the diagnosis., Results: In Nordland County, 657 individuals had MS 1 January 2017. Of these, 637 were recorded with a correct diagnosis of MS in the NPR, while 59 were recorded incorrectly. Incorrect registration was due to a diagnosis that did not fulfill the diagnostic criteria, later investigation had ruled out MS or it was an error in the diagnostic code registration process. Twenty individuals were not registered with MS in the NPR. These were patients who received their diagnosis before data in the NPR were person identifiable (before 2008), and who later had no MS-registered contact with public specialist healthcare services. The sensitivity is 0.97, and the positive predictive value is 0.92., Conclusion: Data from the NPR gave a good estimate of the occurrence of MS, but nearly one in 10 registered diagnoses was not correct., (© 2019 The Authors. Brain and Behavior published by Wiley Periodicals, Inc.)

Objective: There are some indications of increasing incidence of amyotrophic lateral sclerosis (ALS). Awareness of cognitive impairment in ALS has increased in recent years. We describe the epidemiology and clinical features of ALS in a county in northern Norway over a period of 15 years., Methods: All patients with motor neuron disease (MND) living in Nordland County in the period 2000-2015 were identified and the medical records were scrutinized. The average annual incidence was calculated for the whole period and for five-year periods. Prevalence point was 1 January 2015., Results: We identified 74 cases with MND. The crude point prevalence was 4.1 per 100,000. The average annual incidence was 2.1 per 100,000 for the whole period, 2.0 in the period 2000-2004, 2.3 in 2005-2009, and 2.0 in 2010-2014. All except one of the 22 patients with other forms of MND developed ALS during the course of the disease. The mean survival time was 38 months, patients with bulbar symptoms at diagnosis had a mean survival time of 29 months and those with solely spinal symptoms had a mean survival time of 50 months. Seven patients were diagnosed with frontotemporal dementia (FTD)., Conclusion: The incidence was stable during the study period. Other forms of MND converts to clinical ALS given time. Survival time is almost two years shorter in patients with bulbar symptoms at the first examination, compared to those with solely symptoms from spinal muscles. FTD was found in 9% of the patients.

Progressive multifocal leukoencephalopathy is a rare, opportunistic infection of the central nervous system caused by the John Cunningham virus (JCV). There is no effective antiviral treatment available, and restoring immunocompetence is essential for survival. If this occurs too quickly, however, the inflammatory response may prove fatal. This is an up-to-date review of the disorder, intended for clinicians responsible for immunomodulatory therapy.

Purpose: The prevalence of epilepsy among patients with multiple sclerosis (MS) has been found higher than in the general population. Although cortical pathology may be involved, the causal link between MS and epileptic seizures is still unclear. We aimed to identify and describe the patients with active epilepsy in a previously described population based MS-cohort., Methods: Medical records of all patients with MS in Nordland County on January 1, 2010, were scrutinizing for evidence of comorbid seizures and epilepsy., Results: Among 431 patients with MS, we identified 19 (4.4%) with a history of seizures or epilepsy. Fourteen (3.2%) of these had active epilepsy defined as use of antiepileptic drugs or seizures within the last 5 years. One patient got epilepsy before other signs of MS. In patients with relapsing-remitting MS (RRMS) at onset and active epilepsy (n=10), 70% had converted to secondary progressive (SPMS) at prevalence date, compared to only 35% of those without active epilepsy (p=0.02). 43% had converted to SPMS before they got epilepsy. Attack semiology or electroencephalogram recordings indicated a focal onset of seizures in 12 of 14 (86%) with active epilepsy., Conclusion: The frequency of active epilepsy among MS patients in Nordland was 3.2%, approximately 4.5 times higher than in the general Norwegian population. RRMS patients with active epilepsy had more likely converted to SPMS than patients without active epilepsy. With a high frequency of focal epilepsy, the study supports that focal MS brain pathology is the cause of the comorbid epilepsy., (Copyright © 2017 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Objective: To investigate associations between photophobia and seasonal variation of migraine., Methods: In this cross-sectional study, migraineurs consecutively recruited were referred to a specialist center located above the Arctic Circle at 68-71 degrees North during a 2.5-year period. Data were obtained through a structured interview., Results: In total, 302 migraineurs with a mean (±SD) age of 35.5 (±12.6) years were included. Patients who reported seasonal variation of migraine (n = 90; 29.8%) also reported more often interictal photophobia than the others (61/90, 67.8% vs 92/212, 43.4%, P < .0001). Patients reported sunlight or other bright light to trigger migraine attacks in 74.4% with seasonal migraine (SM) compared with 40.6% in patients with non-seasonal migraine (NSM) (P < .0001), but there were similar frequencies of attacks reported to be triggered by sleep, menstruation, and other precipitating factors. After adjusting for migraine with aura, migraine disability, chronic migraine, interictal photophobia, and insomnia, sunlight or other bright light, photophobia was still associated with SM (OR; 3.47, CI [95%]; 1.83-6.59, P < .0001)., Conclusions: Migraineurs in a subarctic area reporting seasonal variation of attack frequency also report increased interictal photophobia independent of other clinical factors. Chronobiological mechanisms and/or increased activity in the visual system may be responsible for this phenomenon., (© 2017 American Headache Society.)

Objectives We determined headache patients' satisfaction with telemedicine and assessed how telemedicine influenced headache burden, compliance with diagnosis and treatment, and need for follow-up consultations. Methods During 2.5 years, patients from Northern Norway referred with non-acute headaches for a specialist consultation at Tromsø University Hospital were consecutively randomised to either telemedicine or traditional visits. Baseline data were recorded and compared to data from a three-month follow-up questionnaire (see Supplementary material). The following were evaluated: (1) satisfaction with the consultation; (2) headache status; subjective improvement, average pain intensity, treatment, headache days per month, and Headache Impact Test (HIT-6); and (3) treatment compliance and follow-up visits. Results Out of 402 consultations, 348 (86.6%) answered the questionnaire. Satisfaction was similar in the telemedicine and the traditional group (88.8% vs. 92.3%; p = 0.35). Subgroup analyses were not prespecified, but there were no differences in satisfaction among females, migraineurs, rural patients and urban patients. Improvement from baseline after three months was reported equally in the telemedicine and the traditional groups. There were also no differences in treatment compliance, but rural telemedicine patients had less-frequent headache visits at three months' follow-up (28.9% vs. 48.7%, p = 0.002). Conclusion Telemedicine is non-inferior to traditional consultations in patient satisfaction, specialist evaluation, and treatment of non-acute headaches. ClinicalTrials.gov ID: NCT02270177.

Medication overuse headache is a secondary headache-a worsening of a pre-existing headache (usually a primary headache) owing to overuse of one or more attack-aborting or pain-relieving medications., Competing Interests: Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article.

Objective: To evaluate long-term treatment efficacy and safety of one-time telemedicine consultations for nonacute headaches., Methods: We randomized, allocated, and consulted nonacute headache patients via telemedicine (n = 200) or in a traditional manner (n = 202) in a noninferiority trial. Efficacy endpoints, assessed by questionnaires at 3 and 12 months, included change from baseline in Headache Impact Test-6 (HIT-6) (primary endpoint) and pain intensity (visual analogue scale [VAS]) (secondary endpoint). The primary safety endpoint, assessed via patient records, was presence of secondary headache within 12 months after consultation., Results: We found no differences between telemedicine and traditional consultations in HIT-6 ( p = 0.84) or VAS ( p = 0.64) over 3 periods. The absolute difference in HIT-6 from baseline was 0.3 (95% confidence interval [CI] -1.26 to 1.82, p = 0.72) at 3 months and 0.2 (95% CI -1.98 to 1.58, p = 0.83) at 12 months. The absolute change in VAS was 0.4 (95% CI -0.93 to 0.22, p = 0.23) after 3 months and 0.3 (95% CI -0.94 to 0.29, p = 0.30) at 12 months. We found one secondary headache in each group at 12 months. The estimated number of consultations needed to miss one secondary headache with the use of telemedicine was 20,200., Conclusion: Telemedicine consultation for nonacute headache is as efficient and safe as a traditional consultation., Clinicaltrialsgov Identifier: NCT02270177., Classification of Evidence: This study provides Class III evidence that a one-time telemedicine consultation for nonacute headache is noninferior to a one-time traditional consultation regarding long-term treatment outcome and safety., (Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Background and Purpose: We investigated non-acute headache patients' long-term satisfaction with a telemedicine consultation and consultation preferences in northern Norway. We hypothesized that patients were not less satisfied with telemedicine than traditional consultations. We also examined the influence of gender, age and education on satisfaction., Methods: For 2.5 years, patients were consecutively screened, recruited and randomly assigned to telemedicine or traditional visits with a consultation at a neurological outpatient department. The primary endpoint was frequency of satisfied patients at 3 and 12 months. Secondary endpoints were satisfaction with consultation, communication, information, diagnosis, advice and prescriptions, and preferred visit form at 12 months., Results: Of 402 participants, 279 (69.4%) answered questionnaires at both 3 and 12 month, and 291 (72.4%) responded at 12 months. The long-term satisfaction of telemedicine patients was 124/145 (85.5%) compared with 118/134 (88.1%) in the traditional group (P = 0.653). The groups did not differ with respect to secondary endpoints, but females were more satisfied with telemedicine communication (P = 0.027). In the telemedicine group, 99/147 (67.3%) were indifferent to the type of consultation. Age and education did not alter the primary results., Conclusions: At 1 year after a specialist evaluation for headache, telemedicine patients did not express less satisfaction than those with traditional consultation. Telemedicine specialist consultations may be a good alternative for headache patients in secondary care., (© 2017 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Background: Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic brain infection caused by the human polyomavirus JC (JCPyV). A particular problem with the drug cladribine seems to be prolonged suppression of the CD4+ T-cells, a well-known risk factor for PML., Case Description: A 67-year-old male presented with a 3-weeks history of unsteady gait, dysarthria and a dysfunctional right arm. Seven years earlier, he had been diagnosed with urticaria pigmentosa, and 2 years later aggressive systemic mastocytosis. Cladribine treatment was initiated and regarded effective, but the course was complicated with bouts of severe anemia and recurrent episodes of salmonella associated gastroenteritis. His lymphocyte count fell to 0.1×10 9 /L at its lowest level, but gradually rose. Despite this, in the 6 month wake of the last dose of cladribine given, the patient experienced herpetic stomatitis, had CMV present in blood, and ultimately developed the neurological symptoms. An MRI scan revealed a lesion in the right cerebellar hemisphere compatible with PML, and PCR analysis of the CSF showed positive for JCPyV DNA with a load of 323 950 copies/ml. No pathological cells were seen on CSF flow cytometry. The CD4/CD8-ratio was 0.45 (160 CD4+ cells/mm 3 and 360 CD8+ cells/mm 3 ). The patient passed away 3 weeks later., Conclusion: PML may be the consequence of prolonged lymphopenia due to the use of cladribine., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Background: The feasibility of telemedicine in diagnosing and treating nonacute headaches, such as primary headaches (migraine and tension-type) and medication-overuse headaches has not been previously investigated. By eliminating the need of travel to specialists, telemedicine may offer significant time and money savings., Objectives: Our objective was to estimate the acceptance of telemedicine and investigate the feasibility and cost savings of telemedicine consultations in diagnosing and treating nonacute headaches., Methods: From September 2012 to March 2015, nonacute headache patients from Northern Norway who were referred to neurologists through an electronic application system were consecutively screened and randomized to participate in either telemedicine or traditional specialist visits. All patients were consulted by two neurologists at the neurological department in Tromsø University Hospital. Feasibility outcomes were compared between telemedicine and traditional groups. Baseline characteristics and costs were then compared between rural and urban patients. Travel costs were calculated by using the probabilistic method of the Norwegian traveling agency: the cheapest means of public transport for each study participant. Loss of pay was calculated based on the Norwegian full-time employee's average salary: < 3.5 hours=a half day's salary, > 3.5 hours spent on travel and consultation=one day's salary. Distance and time spent on travel were estimated by using Google Maps., Results: Of 557 headache patients screened, 479 were found eligible and 402 accepted telemedicine participation (83.9%, 402/479) and were included in the final analyses. Of these, 202 received traditional specialist consultations and 200 received telemedicine. All patients in the telemedicine group were satisfied with the video quality, and 198 (99%, 198/200) were satisfied with the sound quality. The baseline characteristics as well as headache diagnostics and follow-up appointments, and the investigation, advice, and prescription practices were not statistically different between the two randomized groups. In addition, telemedicine consultations were shorter than traditional visits (38.8 vs 43.7 min, P<.001). The travel cost per rural individual (292/402, 73%) was €249, and estimated lost income was €234 per visit. The travel cost in the urban area (110/402, 27%) was €6, and estimated lost income was €117 per visit. The median traveling distance for rural patients was 526 km (range 1892 km), and the median traveling time was 7.8 hours (range 27.3 hours). Rural patients had a longer waiting time than urban patients (64 vs 47 days, P=.001), and fewer women were referred from rural areas (P=.04). Rural women reported higher pain scores than urban women (P=.005)., Conclusion: Our study shows that telemedicine is an accepted, feasible, time-saving, and cost-saving alternative to traditional specialist consultations for nonacute headaches., Trial Registration: Clinicaltrials.gov NCT02270177; http://clinicaltrials.gov/ct2/show/NCT02270177 (Archived by WebCite at http://www.webcitation.org/6hmoHGo9Q).

The etiologies of Bell's palsy and brachial neuritis remain uncertain, and the conditions rarely co-occur or reoccur. Here we present a woman in her twenties who had several relapsing-remitting episodes with left-sided facial palsy and brachial neuropathy. The episodes always started with painful left-sided oral blisters. Repeat PCRs HSV-1 DNA from oral vesicular lesions were positive. Extensive screening did not reveal any other underlying cause. Findings on MRI T2-weighted brachial plexus STIR images, using a 3.0-Tesla scanner during an episode, were compatible with brachial plexus neuritis. Except a mannose-binding lectin deficiency, a congenital complement deficiency that is frequently found in the general Caucasian population, no other immunodeficiency was demonstrated in our patient. In vitro resistance to acyclovir was tested negative, but despite prophylactic treatment with the drug in high doses, relapses recurred. To our knowledge, this is the first ever reported documentation of relapsing-remitting facial and brachial plexus neuritis caused by HSV-1., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Background: Cluster headache (CH) attacks are accompanied by cranial autonomic symptoms indicative of parasympathetic hyperactivity and sympathetic dysfunction ipsilateral to the pain. We aimed to assess cranial autonomic function in CH patients during the remission phase of cluster headache., Materials and Methods: During a remission phase, 38 episodic CH patients underwent the following: dynamic pupillometry, measurement of the superficial temporal artery diameter by ultrasound, and measurement of the retinal vessel diameters from digital retinal photographs. Pupillometry was also performed on 30 age- and sex-matched healthy controls., Results: Thirty patients were included (27 men, three women, mean age 50.2 years ± 12.6). Seven patients reported occasional side shift of their headache, but with a clear predominating side. Significantly reduced average pupillary constriction velocity and retinal venular diameter on the CH pain side were found. There was no asymmetry of the superficial temporal artery diameters. Compared to healthy controls, cluster patients displayed bilaterally reduced pupillary average and maximum constriction velocities, reduced constriction in percentage and increased latency of the light reflex., Conclusions: The present findings indicate a bilaterally reduced cranial parasympathetic tone in CH patients in remission phase, with significant lateralization to the CH pain side. This implies a central origin, and a central pathophysiological model of CH is discussed., (© International Headache Society 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Background: Cluster headache (CH) is regarded as a chronobiological disorder. The hypothalamic biological clock may thus be involved in the pathophysiology, but few studies have actually investigated this in CH patients. A variable number tandem repeat (VNTR) polymorphism of the PER3 clock gene has been associated to preferred daily rhythm (chronotype) in several studies. We aimed to study the distribution of PER3 VNTR polymorphisms and chronotypes in a CH population., Methods: We used blood samples from a biobank of CH patients for genetic tests, and invited all tested patients to complete the Horne-Ostberg Morningness-eveningness Questionnaire (MEQ), the Pittsburgh sleep quality Index (PSQI) and the Shift Work Index. Genotypes were compared to a previously tested population of 432 healthy students., Results: One hundred forty nine patients were genotyped, and we found no difference in PER3 VNTR polymorphisms between patients and controls. Seventy-four patients completed the MEQ (54 men, 20 women, mean age 52.3 years ± 13.4), and chronotypes were as follows: 12 % morning-, 37 % intermediate-, and 51 % evening types. Compared with a previous Danish study of CH patients and controls, there were no difference in chronotype distribution. Sixty percent of patients were defined as bad sleepers (PSQI >5), and 51 % of patients currently employed were shift workers., Conclusions: No association between CH, PER3 VNTR polymorphism and chronotype was found in this study.

Background: The risk of multiple sclerosis (MS) increases with increasing latitude. Taking into consideration that Norway has a large latitude range, a south-to-north gradient would be expected. However, previous studies have reported an uneven distribution of the disease in Norway, with a relatively low prevalence in the most northern parts of the country. We describe the incidence and prevalence of MS in a county in the north of Norway over a period of 40 years., Methods: All patients with MS living in Nordland County in the period 1970-2010 were identified by reviewing hospital charts. The patients were included if they met the criteria of definitive or probable MS according to Poser [Ann Neurol 13:227-231, 1983] or MS according to McDonalds [Ann Neurol 50:121-127, 2001]. Point prevalence at the beginning of the decades was calculated. The average annual incidence was calculated for 5-year periods., Results: The total crude prevalence on January 1, 2010 was 182.4 per 100 000. The annual incidence continuously increased from 0.7 per 100 000 in 1970 - 1974 to 10.1 per 100,000 in 2005 - 2009. The time delay from the first symptom to diagnosis was stable from 1975 to 2010. The proportion of primary progressive MS in the prevalence numbers was 38.2% in 1980, and decreases continuously, to 18.6% in 2010. The female to male prevalence ratio has been stable since 1990 at 2.2 to 1., Conclusion: The prevalence and the incidence of MS have steadily increased over a 40 year period. Nordland County is a high-risk area for MS.

Objective: Cluster headache (CH) may cause excruciating pain and not all patients get satisfactory help. Patient dissatisfaction with general practitioners (GPs) and neurologists, and use of complementary and alternative treatment (CAM) may reflect this. The authors studied patient satisfaction with doctors' treatment and use of CAM in a Norwegian CH cohort., Subjects: A total of 196 subjects with a cluster headache diagnosis were identified in the registers of two neurological departments in North Norway., Design: Of these, 70 with a confirmed diagnosis according to the second edition of the International Classification of Headache Disorders (ICHD-2) completed a comprehensive questionnaire with questions concerning satisfaction with doctors' treatment, use of CAM, and effect of both treatment regimes., Results: Satisfaction with doctors' treatment was reported in 44/70 (63%) (GPs) and 50/70 (71%) (neurologists) while 39/70 (56%) were satisfied with both. Too long a time to diagnosis, median four years, was the most commonly reported claim regarding doctors' treatment. Use of CAM was reported in 27/70 (39%), and 14/70 (20%) reported experience with ≥ 2 CAM. Ten patients reported benefit from CAM (37% of "CAM users"). The average cluster period was longer in CAM-users than others (p = 0.02), but CAM use was not associated with age, education, use of medication, effect of conventional treatment, duration of cluster attacks, or time to diagnosis., Conclusion: About two-thirds of CH patients were satisfied with treatment from either GPs or neurologists, and about one-third had used CAM. Despite experiencing diagnostic delay and severe pain, cluster patients seem in general to be satisfied with doctors' conventional treatment.

Importance: No reliable treatment options are known for progressive multifocal leukoencephalopathy with underlying immunodeficiency. We describe successful compassionate use of recombinant human interleukin 7 in a patient with idiopathic CD4+ T-cell lymphocytopenia., Observations: After the diagnoses of progressive multifocal leukoencephalopathy and idiopathic CD4+ T-cell lymphocytopenia were established, a 61-year-old man was treated with recombinant human interleukin 7 on November 1, 2012. Except for an episode of epilepsia partialis continua on January 16, 2013, a gradual clinical improvement was observed until March. Abnormalities shown on magnetic resonance imaging regressed; JC virus DNA in plasma, likely originating from the brain based on sequencing data, cleared; and increases in peripheral CD4+ T cells and JC virus intrathecal antibodies were observed. One year after treatment, the CD4+ T-cell count returned to baseline and the clinical improvement waned, possibly due to the patient's complex epilepsy. On the latest evaluation on January 14, 2014, the patient's condition was unchanged, with no signs of ongoing central nervous system infection., Conclusions and Relevance: The present case argues strongly for proof of the treatment concept. However, deeper insight into the JC virus and its pathogenesis and the immune response during central nervous system infection as well as further clinical studies are needed before recombinant human interleukin 7 can be recommended for the treatment of other cases of immunodeficiency and progressive multifocal leukoencephalopathy.

Background: Histamine has been studied in both health and disease since the initial description a century ago. With its vasodilative effect, it was suggested early on to be involved in the pathophysiology of migraine. Over the past 25 years, much has been learned about histamine as a neurotransmitter in the central nervous system. The role of this neurotransmitter system in migraine has not been previously reviewed., Objective: Discuss a potential role of the brain histaminergic system in migraine., Methods: Unstructured literature search with a no specific hypothesis-driven approach., Results: There is substantial evidence that systemically given histamine may elicit, maintain, and aggravate headache. The mechanisms for this are not known, and histamines do not penetrate the blood-brain barrier (BBB). However, circulating histamine may influence hypothalamic activity via the circumventricular organs that lack BBB. In the rat, prolonged activation of meningeal nociceptors induced by dural mast cell degranulation has been observed. Subcutaneous injections of N-alpha-methyl histamine, a catabolite of histamine with high affinity to the histamine H3 receptor, probably have some migraine preventive effect. A negative feedback on histamine release from mast cells in proximity to C-fiber endings has been a postulated mechanism. Most antihistamines have shown to be ineffective as acute medication for migraine. Two centrally acting potent H1 receptor antagonists (cinnarizine and cyproheptadine) have been reported to be efficacious in preventing migraine. However, the proof for this is limited, and their efficacy has been ascribed other actions than the antihistaminergic. In general, lack of specificity and side effects limit the potential use of centrally acting H1 and H2 antagonists. Brain histamine is synthesized by neurons that are restricted to the posterior basal hypothalamus, more specific to the tuberomamillary nucleus (TMN), and that project practically to the whole central nervous system. The posterior hypothalamus is a suspected locus in quo in several primary headaches. Recently, a positron emission tomography study performed in the prodromal phase of migraine attacks supported the idea of initial involvement of this area. In another recent study, the thalamic nuclei receiving trigeminal output was also shown to have direct connections with the ventral TMN. The central histaminergic system plays an important role in the complex sleep-wake cycle, promoting cortical excitability during wakening and attention, and it consolidates the wake state. The period of the day, in the evenings and during the night, when there is reduced susceptibility for migraine attacks corresponds with less central histaminergic firing. Activation of both the H3 and the H4 receptor promotes inhibitory actions on neurons. The H3 receptor causes autoinhibition of the histaminergic neurons themselves, and centrally acting H3 receptor agonist prodrugs have shown to both inhibit neurogenic inflammation in dura, to induce sleep, and to produce antinociception. There are no registered ongoing studies on H3 and H4 receptor ligands in migraine., Conclusion: The role of the central histaminergic system in migraine is largely unexplored, but findings from preclinical research may be linked to several aspects of the disorder. The histaminergic system of the brain may play an important role, especially in the initial phase of an attack, and histamine H3 and H4 receptor ligands may potentially have migraine prophylactic properties. However, the basis for this is still circumstantial, and the evidence is lacking., (© 2014 American Headache Society.)

Objective: To assess the prevalence of chronic insomnia and the periodicity of headache attacks in an Arctic cluster headache population., Background: Cluster headache is a sleep-related disorder, and attacks have both circadian and circannual rhythmicity., Methods: Through a retrospective hospital chart review, we identified all subjects diagnosed with episodic cluster headache (ICD-10 G 44.0) at the Neurological Departments in Northern Norway (located north of 66°33'N) between January 1, 2000 and December 31, 2010. Patients with a confirmed diagnosis (ICHD-2) received a comprehensive questionnaire covering demographic data, clinical characteristics, sleep, and periodicity of attacks., Results: A total of 196 subjects were registered, and 178 received the questionnaire. The response rate was 88/178 (49%). Fifty-eight men (aged 49.2 ± 13.6) and 12 women (aged 49.7 ± 15.5) were included. Forty percent of the responders suffered from chronic insomnia (Diagnostic and Statistical Manual of Mental Disorders 4th edition). Forty-nine percent of the responders and 42% of the non-responders were shift workers, which is much higher than compared with the general population (24%). Insomnia was significantly associated with shift work and experiencing longer-lasting cluster bouts. One third attributed their insomnia to the cluster headache. Thirty-seven percent reported a seasonal predilection of the cluster periods, and 58% a diurnal periodicity of attacks. Eighty percent often or always had headache attacks during sleep, the most frequent time interval being at 12:00-4:00 am. Shift workers were significantly more likely to see lack of sleep as a cluster attack trigger than daytime workers., Conclusions: Chronic insomnia and shift work seem to be common among Arctic cluster headache patients. The small number of subjects included in this study implies that conclusions should be drawn with caution, but the findings support the idea of cluster headache as a circadian rhythm disorder., (© 2013 American Headache Society.)

Background: Hand knob infarction is a well-known stroke entity. Based on very limited data, embolic stroke mechanism has been considered the most frequent cause; however, prognosis is considered good. We wanted to shed more light on this phenomenon by assessing a cohort of patients referred to a general hospital stroke unit., Methods: Every subject admitted to our stroke unit with an acute isolated hand paresis in the period from 2007 to 2012 was identified prospectively. Patients who had suffered from a stroke in the hand motor cortex or an adjacent area explaining the acute loss of hand function were included in the study. The Trial of Org 10172 in Acute Stroke Treatment criteria were used to classify subtypes of stroke according to etiology. The patients were followed up during autumn 2012., Results: Seventeen subjects were admitted, but in 2 of them symptoms were transitory and magnetic resonance imaging was negative. Two patients were excluded due to persisting sensory deficits. The remaining 13 (11 males and 2 females) patients with an average age of 62.9 (± 13.4) years were included, representing 1.5% of all ischemic strokes diagnosed at the stroke unit in the given period. All patients were right-handed, and the dominant hand was affected only in 4 (31%). The average Medical Research Council's scale score was 3.1 (± 1.4) on admission, and classified as bad. On follow-up, which occurred on average 29.8 (± 19.8) months after the stroke, the score was 4.6 (± 0.4) and was classified as fair to good. No patient experienced a new stroke. The outcome was good to excellent in 10 patients (77%). Two patients died (15%), 1 of probable cardiac arrest and 1 of unknown cause. One patient did not participate in the follow-up. The majority of patients had evidence of both small artery (77%) and large artery (85%) disease. On average, there were 1.6 (± 0.4) new ischemic lesions per patient. Six patients had a solitary lesion (46%). In 5 of them, small artery occlusion was considered the probable stroke mechanism. In 4 cases, the stroke was of undetermined etiology. Three patients had atrial fibrillation, and in 2 of them cardioembolism was the probable stroke mechanism. Two patients with definite large artery atherosclerosis underwent carotid endarterectomy, and 1 of them had comorbid atrial fibrillation., Conclusion: Strokes causing isolated hand paresis seem to have a heterogeneous etiology. Prognosis regarding hand function is good, but long-term outcome depends on stroke etiology and secondary prophylaxis.