Smoking History and Clinical Features of Cluster Headache:Results from the Korean Cluster Headache Registry

Background@#and Purpose Epidemiologic data suggest that cluster headache (CH) is significantly associated with cigarette smoking. The aim of this study was to determine differences in features between patients with a smoking history and those who are never-smokers, using data from a prospective multicenter registry. @*Methods@#Data used in this study were obtained from the Korean Cluster Headache Registry that collected data from consecutive patients diagnosed with CH. We compared clinical and demographic features between ever-smokers (current or former smokers) and never-smokers. @*Results@#This study enrolled 250 patients who were diagnosed with CH, of which 152 (60.8%) were ever-smokers and 98 (39.2%) were never-smokers. The age at CH onset was significantly lower in the never-smoker group than in the ever-smoker group [27.1±12.9 years vs. 30.6± 10.9 years (mean±standard deviation), p=0.024]. Seasonal rhythmicity (58.1% vs. 44.7%, p= 0.038) and triptan responsiveness (100% vs. 85.1%, p=0.001) were higher in never-smokers, while other clinical features such as pain severity, duration, attack frequency, and associated autonomic symptoms did not differ significantly between the groups. The male-to-female ratio was markedly higher in ever-smokers (29.4:1) than in never-smokers (1.7:1). @*Conclusions@#Most of the clinical features did not differ significantly between patients with a smoking history and never-smokers. However, the age at CH onset, sex ratio, and seasonal rhythmicity were significantly associated with smoking history.

Smoking History and Clinical Features of Cluster Headache:Results from the Korean Cluster Headache Registry

Background@#and Purpose Epidemiologic data suggest that cluster headache (CH) is significantly associated with cigarette smoking. The aim of this study was to determine differences in features between patients with a smoking history and those who are never-smokers, using data from a prospective multicenter registry. @*Methods@#Data used in this study were obtained from the Korean Cluster Headache Registry that collected data from consecutive patients diagnosed with CH. We compared clinical and demographic features between ever-smokers (current or former smokers) and never-smokers. @*Results@#This study enrolled 250 patients who were diagnosed with CH, of which 152 (60.8%) were ever-smokers and 98 (39.2%) were never-smokers. The age at CH onset was significantly lower in the never-smoker group than in the ever-smoker group [27.1±12.9 years vs. 30.6± 10.9 years (mean±standard deviation), p=0.024]. Seasonal rhythmicity (58.1% vs. 44.7%, p= 0.038) and triptan responsiveness (100% vs. 85.1%, p=0.001) were higher in never-smokers, while other clinical features such as pain severity, duration, attack frequency, and associated autonomic symptoms did not differ significantly between the groups. The male-to-female ratio was markedly higher in ever-smokers (29.4:1) than in never-smokers (1.7:1). @*Conclusions@#Most of the clinical features did not differ significantly between patients with a smoking history and never-smokers. However, the age at CH onset, sex ratio, and seasonal rhythmicity were significantly associated with smoking history.

Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report -

Background@#Hereditary angioedema (HAE) is a rare disease caused by the deficiency of C1 esterase inhibitor. HAE has a risk of life-threatening complications such as capillary leak syndrome (CLS) and disseminated intravascular coagulation (DIC).Case: A 42-year-old man with HAE presented for deceased-donor kidney transplantation. Prophylactic fresh frozen plasma (FFP) was given before surgery because of the risk of edema development. With careful management during anesthesia, there were no problems during surgery. However, generalized edema, hypotension, hypoalbuminemia, massive drainage of serosanguineous fluids from the intraabdominal space, and DIC occurred on the day after surgery. CLS was suspected and sustained hypotension with generalized edema became worse despite treatment with albumin, danazol, FFP, and vasoactive drugs. The patient’s condition worsened despite intensive care and he died due to shock. @*Conclusions@#The anesthesiologist should prepare for the critical complications of HAE and prepare the appropriate treatment options.

Factors Associated with Incidental Neuroimaging Abnormalities in New Primary Headache Patients

Background@#and PurposeDeciding whether or not to perform neuroimaging in primary headache is a dilemma for headache physicians. The aim of this study was to identify clinical predictors of incidental neuroimaging abnormalities in new patients with primary headache disorders. @*Methods@#This cross-sectional study was based on a prospective multicenter headache registry, and it classified 1,627 consecutive first-visit headache patients according to the third edition (beta version) of the International Classification of Headache Disorders (ICHD-3β). Primary headache patients who underwent neuroimaging were finally enrolled in the analysis. Serious intracranial pathology was defined as serious neuroimaging abnormalities with a high degree of medical urgency. Univariable and multivariable logistic regression analyses were conducted to identify factors associated with incidental neuroimaging abnormalities. @*Results@#Neuroimaging abnormalities were present in 170 (18.3%) of 927 eligible patients. In multivariable analysis, age ≥40 years [multivariable-adjusted odds ratio (aOR)=3.37, 95% CI=2.07–6.83], male sex (aOR=1.61, 95% CI=1.12–2.32), and age ≥50 years at headache onset (aOR=1.86, 95% CI=1.24–2.78) were associated with neuroimaging abnormalities. In univariable analyses, age ≥40 years was the only independent variable associated with serious neuroimaging abnormalities (OR=3.37, 95% CI=1.17–9.66), which were found in 34 patients (3.6%). These associations did not change after further adjustment for neuroimaging modality. @*Conclusions@#Incidental neuroimaging abnormalities were common and varied in a primary headache diagnosis. A small proportion of the patients incidentally had serious neuroimaging abnormalities, and they were predicted by age ≥40 years. These findings can be used to guide the performing of neuroimaging in primary headache disorders.

Development and Validation of the Cluster Headache Screening Questionnaire

BACKGROUND AND PURPOSE: Cluster headache (CH) is frequently either not diagnosed or the diagnosis is delayed. We addressed this issue by developing the self-administered Cluster Headache Screening Questionnaire (CHSQ). METHODS: Experts selected items from the diagnostic criteria of CH and the characteristics of migraine. The questionnaire was administered to first-visit headache patients at nine headache clinics. The finally developed CHSQ included items based on the differences in responses between CH and non-CH patients, and the accuracy and reliability of the scoring model were assessed. RESULTS: Forty-two patients with CH, 207 migraineurs, 73 with tension-type headache, and 18 with primary stabbing headache were enrolled. The CHSQ item were scored as follows: 3 points for ipsilateral eye symptoms, agitation, and duration; 2 points for clustering patterns; and 1 point for the male sex, unilateral pain, disability, ipsilateral nasal symptoms, and frequency. The total score of the CHSQ ranged from 0 to 16. The mean score was higher in patients with CH than in non-CH patients (12.9 vs. 3.4, p 8 points, the CHSQ had a sensitivity, specificity, positive predictive value, and negative predictive value of 95.2%, 96%, 76.9%, and 99.3%, respectively. CONCLUSIONS: The CHSQ is a reliable screening tool for the rapid identification of CH.

Familial Creutzfeldt–Jakob Disease with a PRNP Mutation at Codon 180 Presented with Visual Hallucinations and Illusions

No abstract available.

Sugammadex associated profound bradycardia and sustained hypotension in patient with the slow recovery of neuromuscular blockade: A case report

BACKGROUND: New complications associated with sugammadex have been increased since its widespread use. We report a case of an 80-year-old male who experienced profound bradycardia and sustained hypotension after administration of sugammadex. CASE: Following administration of 200 mg sugammadex after laparoscopic cholecystectomy, sudden bradycardia (29 beats/min) developed for 10 seconds and his train-of-four (TOF) ratio remained at 0.2 for 5 min. An additional 200 mg sugammadex was administered and profound bradycardia (21–30 beats/min) and hypotension (60/40 mmHg) developed. Atropine at 0.5 mg was administered, but the effect lasted only 30 s. Profound bradycardia occurred four more times at 30 s intervals, and ephedrine and phenylephrine were injected intermittently to increase the patient's heart rate and blood pressure. The TOF ratio became 0.9 about 10 min after administration of additional sugammadex. CONCLUSIONS: Awareness must be heightened regarding the possibility of sugammadexinduced bradycardia and hypotension, and more attention should be paid to patients with slow recovery times following muscle relaxation, despite the use of sugammadex.

Neuroimaging Findings of First-Visit Headache Patients

BACKGROUND: Neuroimaging can play a crucial role in discovering potential abnormalities to cause secondary headache. There has been a progress in the fields of headache diagnosis and neuroimaging in the past two decades. We sought to investigate neuroimaging findings according to headache disorders, age, sex, and imaging modalities in first-visit headache patients. METHODS: We used data of consecutive first-visit headache patients from 9 university and 2 general referral hospitals. The International Classification of Headache Disorders, third edition, beta version was used in headache diagnosis. We finally enrolled 1,080 patients undertook neuroimaging in this study. RESULTS: Among 1,080 patients (mean age: 47.7±14.3, female: 60.8%), proportions of headache diagnosis were as follows: primary headaches, n=926 (85.7%); secondary headaches, n=110 (10.2%); and cranial neuropathies and other headaches, n=43 (4.1%). Of them, 591 patients (54.7%) received magnetic resonance imaging (MRI). Neuroimaging abnormalities were found in 232 patients (21.5%), and their proportions were higher in older age groups and male sex. Chronic cerebral ischemia was the most common finding (n=88, 8.1%), whereas 76 patients (7.0%) were found to have clinically significant abnormalities such as primary brain tumor, cancer metastasis, and headache-relevant cerebrovascular disease. Patients underwent MRI were four times more likely to have neuroimaging abnormalities than those underwent computed tomography (33.3% vs. 7.2%, p <0.001). CONCLUSIONS: In this study, the findings of neuroimaging differed according to headache disorders, age, sex, and imaging modalities. MRI can be a preferable neuroimaging modality to identify potential causes of headache.

Wernicke encephalopathy as initial presentation of lymphoma

No abstract available.

Clinical Features of Cluster Headache Patients in Korea

Cluster headache (CH) is a rare underdiagnosed primary headache disorder with very severe unilateral pain and autonomic symptoms. Clinical characteristics of Korean patients with CH have not yet been reported. We analyzed the clinical features of CH patients from 11 university hospitals in Korea. Among a total of 200 patients with CH, only 1 patient had chronic CH. The average age of CH patients was 38.1 ± 8.9 years (range 19–60 years) and the average age of onset was 30.7 ± 10.3 years (range 10–57 years). The male-to-female ratio was 7:1 (2.9:1 among teen-onset and 11.7:1 among twenties-onset). Pain was very severe at 9.3 ± 1.0 on the visual analogue scale. The average duration of each attack was 100.6 ± 55.6 minutes and a bout of CH lasted 6.5 ± 4.5 weeks. Autonomic symptoms were present in 93.5% and restlessness or agitation was present in 43.5% of patients. Patients suffered 3.0 ± 3.5 (range 1–25) bouts over 7.3 ± 6.7 (range 1–30) years. Diurnal periodicity and season propensity were present in 68.5% and 44.0% of patients, respectively. There were no sex differences in associated symptoms or diurnal and seasonal periodicity. Korean CH patients had a high male-to-female ratio, relatively short bout duration, and low proportion of chronic CH, unlike CH patients in Western countries.

Comprehensive Application of the International Classification of Headache Disorders Third Edition, Beta Version

The purpose of this study was to test the feasibility and usefulness of the International Classification of Headache Disorders, third edition, beta version (ICHD-3beta), and compare the differences with the International Classification of Headache Disorders, second edition (ICHD-2). Consecutive first-visit patients were recruited from 11 headache clinics in Korea. Headache classification was performed in accordance with ICHD-3beta. The characteristics of headaches were analyzed and the feasibility and usefulness of this version was assessed by the proportion of unclassified headache disorders compared with ICHD-2. A total of 1,627 patients were enrolled (mean age, 47.4±14.7 yr; 62.8% female). Classification by ICHD-3beta was achieved in 97.8% of headache patients, whereas 90.0% could be classified by ICHD-2. Primary headaches (n=1,429, 87.8%) were classified as follows: 697 migraines, 445 tension-type headaches, 22 cluster headaches, and 265 other primary headache disorders. Secondary headache or painful cranial neuropathies/other facial pains were diagnosed in 163 patients (10.0%). Only 2.2% were not classified by ICHD-3beta. The main reasons for missing classifications were insufficient information (1.6%) or absence of suitable classification (0.6%). The diagnoses differed from those using ICHD-2 in 243 patients (14.9%). Among them, 165 patients were newly classified from unclassified with ICHD-2 because of the relaxation of the previous strict criteria or the introduction of a new diagnostic category. ICHD-3beta would yield a higher classification rate than its previous version, ICHD-2. ICHD-3beta is applicable in clinical practice for first-visit headache patients of a referral hospital.

Pitfalls of Atrial Advancement Using a Ventricular Extra-stimulus During Supraventricular Tachycardia

The delivery of single His-refractory ventricular extra-stimulus during supraventricular tachycardia is useful to identify the mechanism of the tachycardia. We present the different responses based on the ventricular extra-stimulus site. Our findings demonstrate that the atrial activation via an accessory pathway was not advanced based on the ventricular pacing site. Therefore, atrioventricular tachycardia could masquerade as atrioventricular nodal reentrant tachycardia.

Cardiovascular Autonomic Dysfunction in Mild and Advanced Parkinson's Disease

OBJECTIVE: The purpose of the present study was to investigate cardiovascular autonomic dysfunction in patients with Parkinson's disease (PD) with mild to severe stages of motor symptoms and to compare cardiovascular autonomic dysfunction between drug-naïve and dopaminergic drug-treated groups. METHODS: This study included 188 PD patients and 25 age-matched healthy controls who underwent head-up tilt-testing, 24-h ambulatory blood pressure (BP) monitoring and 24-h Holter monitoring. Autonomic function test results were evaluated among groups categorized by motor symptom severities (mild vs. moderate vs. severe) and treatment (drug-naïve or dopaminergic drug treatment). RESULTS: Orthostatic hypotension and supine hypertension were more frequent in patients with PD than in healthy controls. The frequencies of orthostatic hypotension, supine hypertension, nocturnal hypertension and non-dipping were not different among groups. Additionally, no significant differences were detected in supine BP, orthostatic BP change, nighttime BP, nocturnal BP dipping, or heart rate variabilities among groups. CONCLUSIONS: Cardiovascular autonomic dysfunction is not confined to moderate to severe PD patients, and starts early in the course of the disease in a high proportion of PD patients. In addition, dopaminergic drug treatments do not affect cardiovascular autonomic function.

Chronic Daily Headache and Medication Overuse Headache in First-Visit Headache Patients in Korea: A Multicenter Clinic-Based Study

BACKGROUND AND PURPOSE: Chronic daily headache (CDH) is defined as a headache disorder in which headaches occur on a daily or near-daily basis (at least 15 days/month) for more than 3 months. Chronic migraine (CM) and medication overuse headache (MOH) are very disabling headaches that remain underdiagnosed. The aim of this study was to establish the frequency of CDH and its various subtypes, and examine the associations with MOH among first-visit headache patients presenting at neurology outpatient clinics in Korea. METHODS: Eleven neurologists enrolled first-visit patients with complaints of headaches into outpatient clinics for further assessment. Headache disorders were classified according to the International Classification of Headache Disorder (third edition beta version) by each investigator. RESULTS: Primary CDH was present in 248 (15.2%) of the 1,627 included patients, comprising CM (143, 8.8%), chronic tension-type headache (CTTH) (98, 6%), and definite new daily persistent headache (NDPH) (7, 0.4%). MOH was associated with headache in 81 patients (5%). The association with MOH was stronger among CM patients (34.5%) than patients with CTTH (13.3%) or NDPH (14.3%) (p=0.001). The frequency of CDH did not differ between secondary and tertiary referral hospitals. CONCLUSIONS: The frequencies of CDH and MOH diagnoses were 15.2% and 5%, respectively in first-visit headache patients presented at secondary or tertiary referral hospitals in Korea. CM was the most common subtype of CDH and was most frequently associated with MOH.

Bilateral Thalamic Lesions in Hashimoto's Encephalopathy

No abstract available.

Blood Pressure and Heart Rate Variability in Alzheimer's Disease

BACKGROUND AND PURPOSE: Altered blood pressure (BP) and heart rate variations (HRVs) have been reported in Alzheimer's disease (AD). However, it is unclear how these two manifestations are associated with AD. Therefore, the objective of this study was to investigate BP and heart rate variability in AD compared to that in normal controls, patients with subjective memory impairment (SMI), and patients with mild cognitive impairment (MCI). METHODS: Case-control comparisons were made among AD (n=37), MCI (n=24), SMI (n=17), and controls (n=25). All patients underwent clinical and neuropsychological assessments with 24-h ambulatory BP and Holter monitoring. RESULTS: Patients with AD had higher pulse pressures than those in other groups. In addition, AD patients experienced blunted nocturnal BP dipping associated with declining cognitive status. AD patients also had larger ranges of HRV in parasympathetic domains compared to other groups, especially at night. CONCLUSIONS: Our results suggest that diurnal sympathetic and parasympathetic cardiac variability were significantly disturbed in mild cholinesterase-naive AD patients. This may be an indirect sign of disturbed integrity to the sleep-wake cycle in mild AD.

Clinical and Genetic Features of Familial Hemiplegic Migraine

Migraine is a common cause of episodic headache disorder, and multiple factors are associated with the pathogenic mechanism underlying migraine. Recent studies suggest that genetic determinants influence migraine, most likely as part of a multifactorial mechanism. Due to the similarity in clinical features and pathophysiological mechanisms between familial hemiplegic migraine (FHM) and migraine with aura, FHM can be used as a model for migraine and aura. Mutations in the CACNA1A gene on chromosome 19p13 (FHM1), in the ATP1A2 gene at 1q23 (FHM2), and in the SCNA1A gene at 2q24 (FHM3) are responsible for most of the representative types of FHM. The recent beta version of the third edition of the International Classification of Headache Disorders contains revised diagnostic criteria for FHM, which include the above-three genetic factors. In this review we discuss the clinical and genetic features of FHM.

Clinical and Genetic Features of Familial Hemiplegic Migraine

Migraine is a common cause of episodic headache disorder, and multiple factors are associated with the pathogenic mechanism underlying migraine. Recent studies suggest that genetic determinants influence migraine, most likely as part of a multifactorial mechanism. Due to the similarity in clinical features and pathophysiological mechanisms between familial hemiplegic migraine (FHM) and migraine with aura, FHM can be used as a model for migraine and aura. Mutations in the CACNA1A gene on chromosome 19p13 (FHM1), in the ATP1A2 gene at 1q23 (FHM2), and in the SCNA1A gene at 2q24 (FHM3) are responsible for most of the representative types of FHM. The recent beta version of the third edition of the International Classification of Headache Disorders contains revised diagnostic criteria for FHM, which include the above-three genetic factors. In this review we discuss the clinical and genetic features of FHM.

Chronic Daily Headache in Korea: Prevalence, Clinical Characteristics, Medical Consultation and Management

BACKGROUND AND PURPOSE: Chronic daily headache (CDH) is a commonly reported reason for visiting hospital neurology departments, but its prevalence, clinical characteristics, and management have not been well documented in Korea. The objective of this study was to characterize the 1-year prevalence, clinical characteristics, medical consultations, and treatment for CDH in Korea. METHODS: The Korean Headache Survey (KHS) is a nationwide descriptive survey of 1507 Korean adults aged between 19 and 69 years. The KHS investigated headache characteristics, sociodemographics, and headache-related disability using a structured interview. We used the KHS data for this study. RESULTS: The 1-year prevalence of CDH was 1.8% (95% confidence interval, 1.1-2.5%), and 25.7% of the subjects with CDH met the criteria for medication overuse. Two-thirds (66.7%) of CDH subjects were classified as having chronic migraine, and approximately half of the CDH subjects (48.1%) reported that their headaches either substantially or severely affected their quality of life. Less than half (40.7%) of the subjects with CDH reported having consulted a doctor for their headaches and 40.7% had not received treatment for their headaches during the previous year. CONCLUSIONS: The prevalence of CDH was 1.8% and medication overuse was associated with one-quarter of CDH cases in Korea. Many subjects with CDH do not seek medical consultation and do not receive appropriate treatment for their headaches.

Concomitant Functional Gastrointestinal Symptoms Influence Psychological Status in Korean Migraine Patients

BACKGROUND/AIMS: Migraine is frequently accompanied by symptoms consistent with functional gastrointestinal disorders (FGIDs). This study evaluated the prevalence of functional gastrointestinal symptoms and assessed the symptoms' relationship with the concomitant functional symptoms of anxiety, depression, and headache-related disability. METHODS: This prospective study included 109 patients with migraine who were recruited from a headache clinic at a teaching hospital. The participants completed a self-administered survey that collected information on headache characteristics, functional gastrointestinal symptoms (using Rome III criteria to classify FGID), anxiety, depression, and headache-related disability. RESULTS: In total, 71% of patients met the Rome III criteria for at least one FGID. In patients with FGID, irritable bowel syndrome was the most common symptom (40.4%), followed by nausea and vomiting syndrome (24.8%) and functional dyspepsia (23.9%). Depression and anxiety scores were significantly higher in patients meeting the criteria for any FGID. The number of the symptoms consistent with FGID in individual patients correlated positively with depression and anxiety. CONCLUSIONS: FGID symptoms defined by the Rome III criteria are highly prevalent in migraine. These symptoms correlate with psychological comorbidities, such as depression and anxiety.