Cluster Analysis Revealed Two Hidden Phenotypes of Cluster Headache.

Objective: To investigate the possible subgroups of patients with Cluster Headache (CH) by using K-means cluster analysis. Methods: A total of 209 individuals (mean (SD) age: 39.8 (11.3) years), diagnosed with CH by headache experts, participated in this cross-sectional multi-center study. All patients completed a semi-structured survey either face to face, preferably, or through phone interviews with a physician. The survey was composed of questions that addressed sociodemographic characteristics as well as detailed clinical features and treatment experiences. Results: Cluster analysis revealed two subgroups. Cluster one patients (n = 81) had younger age at diagnosis (31.04 (9.68) vs. 35.05 (11.02) years; p = 0.009), a higher number of autonomic symptoms (3.28 (1.16) vs. 1.99(0.95); p < 0.001), and showed a better response to triptans (50.00% vs. 28.00; p < 0.001) during attacks, compared with the cluster two subgroup (n = 122). Cluster two patients had higher rates of current smoking (76.0 vs. 33.0%; p=0.002), higher rates of smoking at diagnosis (78.0 vs. 32.0%; p=0.006), higher rates of parental smoking/tobacco exposure during childhood (72.0 vs. 33.0%; p = 0.010), longer duration of attacks with (44.21 (34.44) min. vs. 34.51 (24.97) min; p=0.005) and without (97.50 (63.58) min. vs. (83.95 (49.07) min; p = 0.035) treatment and higher rates of emergency department visits in the last year (81.0 vs. 26.0%; p< 0.001). Conclusions: Cluster one and cluster two patients had different phenotypic features, possibly indicating different underlying genetic mechanisms. The cluster 1 phenotype may suggest a genetic or biology-based etiology, whereas the cluster two phenotype may be related to epigenetic mechanisms. Toxic exposure to cigarettes, either personally or secondarily, seems to be an important factor in the cluster two subgroup, inducing drug resistance and longer attacks. We need more studies to elaborate the causal relationship and the missing links of neurobiological pathways of cigarette smoking regarding the identified distinct phenotypic classes of patients with CH.

Insight into pain syndromes in acute phase of mild-to-moderate COVID-19: Frequency, clinical characteristics, and associated factors.

BACKGROUND: Pain has been frequently described as a clinical feature of COVID-19, and the main pain syndromes that have been associated with the acute phase of this disease so far are headache, myalgia, arthralgia, and neuropathic pain. Understanding the characteristics of pain symptoms is crucial for a better clinical approach. METHODS: Patients who were diagnosed as having COVID-19 using reverse transcription-polymerase chain reaction were included in the study. Patients were asked to complete a 51-item questionnaire via a phone interview, which included questions on demographics, acute COVID-19 symptoms, the presence of pain symptoms, and their characteristics in the acute phase of COVID-19. RESULTS: A total of 222 out of 266 patients with COVID-19 participated in the study, yielding a response rate of 83.5%. A total of 159 patients reported at least one kind of pain syndrome with a prevalence of 71.6%. Myalgia was reported in 110 (49.6%) patients, headache in 109 (49.1%), neuropathic pain symptoms in 55 (24.8%), and polyarthralgia in 30 (13.5%) patients. A total of 66 patients reported only one type of pain, 46 reported two types, 42 reported three types, and five patients reported all four types of pain. Logistic regression analysis showed that there were significant associations between these pain syndromes and a strong association was found between neuropathic pain and headache. CONCLUSION: Pain is a frequently observed symptom of mild-to-moderate COVID-19. There are significant relationships between pain syndromes in COVID-19, which may be due to a sequence of common etiologic factors. SIGNIFICANCE: This study described the main pain syndromes associated acute phase of mild-to-moderate COVID-19 and its associated features. Headaches and pain of neuropathic characteristics were prevalent in this sample.

The expression and prognostic value of miR-146a and miR-155 in Turkish patients with multiple sclerosis.

Multiple sclerosis (MS) is an inflammatory, autoimmune demyelinating, and neurodegenerative disorder of the central nervous system. Interactions between environmental factors, predisposition genes, and determining genes appear to be involved in its etiology. Epigenetic mechanisms such as microRNA-mediated gene regulation can determine the susceptibility and severity of autoimmune diseases. Therefore, to determine the role of miR-146a and miR-155 in MS and its developmental stages, the expression levels in the serum of MS and clinically isolated syndrome (CIS) patients were compared with those of healthy controls. In the present study, the expression levels of miR-146a and miR-155 were assessed using quantitative Real-Time PCR in blood samples of 15 CIS patients and 61 relapsing-remitting multiple sclerosis (RRMS) patients alongside 32 healthy patients as controls. Furthermore, any associations with the clinicopathologic variables of the patients were also evaluated. Dysregulations were found only in the miR-146a and miR-155 expressions in the RRMS-Control group. When the RRMS patients were evaluated in terms of the characteristics of sex, annual attack rate, age of diagnosis, duration of follow-up, and immunomodulatory treatments used, no significant differences were observed. However, significant dysregulations were identified in miRNA expression in the vitamin D level, EDSS values, and the number of attacks. ROC curve analysis showed that miR-146a and miR-155 were significant in the RRMS-Control group for the area under the curve (AUC). It is possible that miR-146a may be associated with vitamin D deficiency and disease disability, while miR-155 may be associated with the number of attacks.

Contribution of functional dopamine D2 and D3 receptor variants to motor and non-motor symptoms of early onset Parkinson's disease.

In the present study, we focused on investigating the contribution of functional dopamine D2 and D3 receptor variants to motor and/or non-motor symptoms of early onset Parkinson's disease (EOPD). Three functional single nucleotide polymorphisms (SNPs), DRD3 rs6280, DRD2 rs2283265 and DRD2 rs1076560, were genotyped in 128 Turkish EOPD patients and then, statistical analysis was conducted for the potential impacts of SNPs on clinical parameters. All three SNPs were found to be statistically significant in terms of PD-related pain: DRD3 [rs6280; risk allele "T" for pain; p = 0.031; odds ratio (OR)=4.25], DRD2 [rs2283265; risk allele "A" for pain; p = 0.001; OR=8.47] and, DRD2 [rs1076560; risk allele "A" for pain; p = 0.022; OR=4.58]. Additionally, bilateral disease [p = 0.011; OR=5.10] and gender [risk group "female"; p = 0.003; OR=8.53] were also identified as significant univariate risk factors for PD-related pain. Based on logistic regression analysis conducted with the significant univariate risk factors, this the first report to clarify that a female patient with bilateral PD and DRD2 rs2283265 polymorphism has a significant risk for PD-related pain. Our findings might contribute to improve life quality by offering treatment options for pain in PD patients with these clinical and genetic features.

Sex Differences of Migraine: Results of a Nationwide Home-based Study in Turkey.

INTRODUCTION: The prevalence of migraine was found to be more than three-fold higher in women as compared with men, and in addition to differences in prevalence rates, the characteristics and associated features might also differ between the sexes. The aim of this study was to compare sex-specific features of migraine and demographic parameters in a nationwide population-based study in Turkey. METHODS: Among 5323 subjects, a total of 871 patients who were diagnosed as having definite migraine according to the diagnostic criteria of the International Classification of Headache Disorders-III (ICHD-III) were included in our study. The demographic characteristics, associated features, and triggers of migraine were examined with regard to sex. RESULTS: The study group comprised 640 women (73.5%) and 231 men (26.5%), with a female to male ratio of 2.8:1. Attack duration, mean migraine disability assessment scores (MIDAS), frequencies of nausea, vomiting, osmophobia, vertigo/dizziness, and allodynia were found significantly different between women and men. When we compared these parameters between men and postmenopausal women, all these parameters were still significant except nausea. Odor was statistically more frequent as a reported trigger in women, whereas excessive sleep was a statistically more frequent triggering factor in men. The rates of depression and allergy were significantly higher in women when compared with men. CONCLUSION: Longer attack duration, higher MIDAS scores, and the frequencies of nausea, vomiting, osmophobia, vertigo/dizziness, and allodynia were more significant in women and this variance in sex persisted after menopause. Also, some trigger factors and co-morbidities differed between the sexes. These findings might result from complex genetic factors besides sociocultural influences, biologic, and sociocultural roles. Future studies should continue to explore biologic and genetic factors with respect to sex in migraine.

The persistence versus interchangeability of migraine and tension-type headaches in a 5-year population-based validated survey.

OBJECTIVE: The aim of this population-based validated study was to determine the course of tension-type headache and migraine and to evaluate the predictors of persistence. METHODS: We evaluated the course of headache in a large population from the first assessment in 2008 through a second assessment in 2013. Then we examined the factors associated with persistent migraine and persistent tension-type headache. RESULTS: Our study in 2013 revealed that only 42.9% of definite migraineurs in 2008 received the same diagnosis again, and of the remaining migraineurs 23.3% were newly diagnosed as definite tension-type headache; 11.6% evolved into probable tension-type headache, 6.4% changed to probable migraine, and 15.8% were headache free. The 17.7% of patients with definite tension-type headache in 2008 were newly diagnosed as having probable tension-type headache, 14.7% as having definite migraine, 6.4% as having probable migraine, and 28.9% as headache free in 2013, and only 32.3% received the definite tension-type headache diagnosis again. Binary logistic regression analysis showed nausea, throbbing and severe headache were the significant parameters for persistent migraine. A multiple regression analysis model with stepwise variable selection revealed that nausea, throbbing and severe headache and osmophobia remained in the final model as predictors of migraine persistence. We found no predictive factor for persistent tension-type headache. CONCLUSION: Migraine and tension-type headache did not seem to show a simple bidirectional linear worsening from headache-free state to definite migraine or vice versa, hence the transitions between them are more chaotic, reflecting that there are still unknown modifiers and modulators. Certain headache characteristics of migraine might predict persistent migraine.

Evaluation of anxiety and depression scales and quality of LIFE in cervical dystonia patients on botulinum toxin therapy and their relatives.

OBJECTIVE: In this study, quality of life and psychiatric comorbid disorders were investigated in patients with cervical dystonia and their spouses and we also investigated the effect of botulinum toxin (BTX) treatment on these parameters. MATERIAL AND METHOD: Thirty patients with cervical dystonia (CD) on BTX treatment and their spouses (n = 30) were included. Beck Depression Scale (BDS), State-Trait Anxiety Inventory I and II (STAI-I, STAI-II), Hospital Anxiety Scale (HAS), Hospital Depression Scale (HDS) for psychiatric comorbid disease assessment, Toronto Western Spasmodic Torticollis Scale (TWSTRS) for disease activity assessment, and Craniocervical Dystonia Questionnaire (CDQ-24), Cervical Dystonia Impact Profile (CDIP-58), and Short Form 36 (SF-36) questionnaires for quality of life assessment were used. BDS, STAI-I and STAI-II, HAS, HDS, and SF-36 scales were also obtained from the spouses. The same tests were applied both before and 8 weeks after the BTX treatment. CONCLUSION: In our study, an increase in psychiatric comorbid disorders such as depression and anxiety was observed and the quality of life was adversely affected in all areas in patients. In the spouses of the patients, the rates of psychiatric comorbid disorders such as depression and anxiety were found to be increased when compared to healthy subjects while vitality, mental health, and general health perception were found to be negatively affected. Patients showed improvements in anxiety level, disease activity, and overall quality of life scales after BTX treatment.

Epigenetic approach to early-onset Parkinson's disease: low methylation status of SNCA and PARK2 promoter regions.

Background and aim The effect of epigenetic modifications in the genes related to Parkinson's disease (PD) is still unclear. In the present study, we investigated methylation status of SNCA and PARK2 genes in patients with early-onset Parkinson's disease (EOPD). Materials and methods The promoter region methylation status of SNCA and PARK2 genes was evaluated by methylation specific-PCR (MSP) in 91 patients with EOPD and 52 healthy individuals. Results The methylation of SNCA and PARK2 promoter regions were significantly lower in EOPD patients compared to the control group (P = 0.013 and P = 0.03, respectively). We also found that the methylation status of the SNCA might be associated with positive family history of PD (P = 0.042). Conclusion Although it should be supported by further analysis, based on the results of the present study, the methylation status of SNCA and PARK2 genes might contribute to EOPD pathogenesis.

Mutation analysis of the PARKIN, PINK1, DJ1, and SNCA genes in Turkish early-onset Parkinson's patients and genotype-phenotype correlations.

OBJECTIVE: Variations in PARK genes (PRKN, PINK1, DJ-1, and SNCA) cause early-onset Parkinson's disease (EOPD) in different populations. In the current study, we aimed to evaluate the frequencies of variations in PARK genes and the effects of these variations on the phenotypes of Turkish EOPD patients. METHODS: All coding regions and exon-intron boundaries of the PRKN, PINK1, DJ-1, and SNCA genes were screened by heteroduplex analysis followed by direct sequencing of the detected variants in 50 Turkish EOPD patients. These variants were evaluated using SIFT, PolyPhen, HSF, and LOVD web-based programs. RESULTS: The frequency of EOPD-associated variations in the PRKN gene was 34%. Among these variations, p.A82E in exon 3 and p.Q409X in exon 11 was determined to be pathogenic. We also defined previously unknown cryptic variations, including c.872-35 G>A and c.872-28T>G in exon 8 of PRKN and c.252+30 T>G and c.322+4 A>G in exons 4 and 5 of DJ1, respectively, that were associated with EOPD. Although no significant association was observed between the PARK gene mutations and clinical features (P>0.05), the alterations were related to the clinical symptoms in each patient. CONCLUSION: An increasing number of studies report that PRKN, PINK1, DJ1 and SNCA mutations are associated with early-onset Parkinson's disease; however, a limited number of studies have been conducted in Turkey. Additionally, our study is the first to evaluate the frequency of SNCA mutations in a Turkish population. The aim of this study was determine the frequency distributions of the PRKN, PINK1, DJ1, and SNCA gene mutations and to analyze the relationships between these genetic variations and the clinical phenotype of EOPD in Turkish patients.

Association of MDR1, CYP2D6, and CYP2C19 gene polymorphisms with prophylactic migraine treatment response.

Prophylactic therapy response varies in migraine patients. The present study investigated the relationship between the resistance to the drugs commonly used in prophylactic therapy and the possible polymorphic variants of proteins involved in the metabolism of these drugs. Migraine patients with the MDR1 3435TT genotype exhibited a better treatment response to topiramate than migraine patients with the CC and CT genotypes (p=0.020). The MDR1 C3435T polymorphism was also found to be a higher risk factor for topiramate treatment failure in a comparison of the number of days with migraine (ß2=1.152, p=0.015). However, there was no significant relationship between the treatment response to topiramate and either the CYP2D6 or CYP2C19 polymorphism, and there were no significant correlations between the treatment responses to amitriptyline, propranolol, and valproic acid and the MDR1, CYP2D6 and CYP2C19 gene polymorphisms. This is the first study to investigate the effect of the polymorphic variants on prophylactic therapy response in migraine patients.

Characterization of Migraineurs Having Allodynia: Results of a Large Population-based Study.

OBJECTIVE: Allodynia reflects the clinical correlate of central sensitization, but it is usually neglected in clinical headache management. We aimed to report the prevalence and previously unnoticed associations of allodynia in migraineurs by a nationwide face-to-face questionnaire-based study by physicians. METHODS: A total of 5323 households were examined for headache according to the diagnostic criteria of International Classification of Headache Disorders-II. Detailed headache features, premonitory signs, demographics, socio-economic status, and hormonal status of female individuals were analyzed with regard to the presence of allodynia in patients with definite migraine. RESULTS: Allodynia was present in 61.1% of migraineurs in the general population of Turkey. The duration and severity of attacks (P<0.0001), photophobia (P=0.001), phonophobia, and also osmophobia (P<0.0001), as well as premonitory signs (P=0.018), showed significant associations with allodynia. Migraineurs with aura or family history of migraine more often reported allodynia in comparison with those without (P=0.001 and 0.028, respectively). Allodynic migraineurs had a higher rate of physician consults and high levels on the Migraine Disability Assessment questionnaire, reflecting increased burden of headache. Furthermore, migraineurs with allodynia had high probability of attacks close to menses. Migraine improved during pregnancy, but it worsened after menopause or during oral contraceptive use in individuals experiencing allodynia when compared with those without allodynia. DISCUSSION: The duration, severity, and disability of migraine attacks, photophobia, phonophobia, and osmophobia, as well as premonitory signs, showed significant associations with allodynia in the general population. Moreover, migraineurs with aura or family history of migraine more often reported allodynia, and allodynic migraneurs were more sensitive to hormonal changes. Allodynia, which seems to indicate higher tendency to central sensitization, should be implemented in daily headache practice to predict the prognosis and high levels of migraineous involvement.

Migraine incidence in 5 years: a population-based prospective longitudinal study in Turkey.

BACKGROUND: The incidence of migraine has been investigated only in a few studies worldwide and it is not known in our country. We, therefore, aimed to estimate the migraine incidence in a previously accomplished population-based prevalence study sample of 5323 individuals in the year 2008. METHODS: The former Turkish headache prevalence study has been completed as a nationwide, randomized, home-based study of face-to-face examination by physicians trained for headache diagnosis by using ICHD criteria. Five years after this study an optimized survey including 50 questions was performed to estimate the migraine incidence in migraine-free individuals in the previous study, with a 56.4 % responder rate. Two validation studies for this survey were performed prior and after the study each in 100 subjects by comparing the gold standard of expert diagnosis of headache, showing high rate of reliability (Crohnbach alpha: 0.911 and 0.706, respectively). RESULTS: Migraine incidence was estimated as 2.38 % (2.98 % in women and 1.93 % in men) per year in 2563 migraine-free individuals; if the population at risk is defined as the group without any headaches, the migraine incidence decreased to 1.99 %. The chronic migraine (CM) incidence [without medication overuse (MOH)] was 0.066 % and that of MOH was 0.259 %. We found a significant burden of the disease on the occupational functionality as well as on social and family life, even in the early years of the migraine. The family history of headaches especially in the fathers could be useful to predict new cases of migraine, besides the well-known risk factor, diagnosis of depression, whereas income and education did not seem to relate to migraine onset. CONCLUSIONS: Our study with a large population-based nation-wide sample, using ICHD-II criteria, with structured headache interviews as well as blinded re-validation of the questionnaire diagnoses showed a 2.38 % incidence rate of migraine in Turkey, higher than most of the other previous reports; a finding which could be related to genetic factors and also to the methodological differences in the study designs. Moreover the incidence of CM was found to be 0.066 %.

Population-based study of vestibular symptoms in migraineurs.

CONCLUSION: The differences between migraineurs with vertigo or motion sickness or both, and migraineurs with neither might reflect differences in migraine pathophysiology. OBJECTIVE: To assess vestibular symptoms in 871 definite migraineurs. METHODS: Data were gathered using a structured questionnaire. We considered responses to only 2/150 questions: (1) 'have you had vertigo with or apart from your headaches?' and (2) 'have you experienced motion sickness most of your life?'. The target groups were: (a) migraineurs with either vertigo or motion sickness, 'migraine with vestibular symptoms' (MwVS), their control group being migraineurs with neither vertigo nor motion sickness, 'migraine without vestibular symptoms' (MwoVS); (b) migraineurs who reported vertigo, 'migraine with vertigo' (MwV); their control group being migraineurs without vertigo (MwoV). RESULTS: Among the 871 definite migraineurs, 534 had MwV, 337 had MwoV, 663 had MwVS, and 208 had MwoVS. The MwVS group had more headache, aura, nausea, vomiting, osmophobia, allergy, allodynia, headache increasing with head motion, noise as trigger for headache, days needing analgesics, and higher migraine disability scores than the MwoVS group. The pattern was the same in the MwV vertigo group as in the MwVS group, apart from migraine disability scores, which were no different.

The distinct genetic pattern of ALS in Turkey and novel mutations.

The frequency of amyotrophic lateral sclerosis (ALS) mutations has been extensively investigated in several populations; however, a systematic analysis in Turkish cases has not been reported so far. In this study, we screened 477 ALS patients for mutations, including 116 familial ALS patients from 82 families and 361 sporadic ALS (sALS) cases. Patients were genotyped for C9orf72 (18.3%), SOD1 (12.2%), FUS (5%), TARDBP (3.7%), and UBQLN2 (2.4%) gene mutations, which together account for approximately 40% of familial ALS in Turkey. No SOD1 mutations were detected in sALS patients; however, C9orf72 (3.1%) and UBQLN2 (0.6%) explained 3.7% of sALS in the population. Exome sequencing revealed mutations in OPTN, SPG11, DJ1, PLEKHG5, SYNE1, TRPM7, and SQSTM1 genes, many of them novel. The spectrum of mutations reflect both the distinct genetic background and the heterogeneous nature of the Turkish ALS population.

Impaired cognitive performance and hippocampal atrophy in Parkinson disease.

BACKGROUND/AIM: Dementia is common in Parkinson disease (PD). Since magnetic resonance imaging has been used, hippocampal atrophy has been shown in PD patients with or without dementia. In this study we sought the correlation of cognitive decline with bilateral hippocampal volume in PD patients. MATERIALS AND METHODS: Thirty-three patients with diagnosis of idiopathic PD and 16 healthy subjects were included in this study. PD patients were divided into two groups as normal cognitive function and mild cognitive impairment (MCI). The Mini-Mental State Examination and detailed cognitive assessment tests were performed for all patients for cognitive analyses. Depression was excluded by the Geriatric Depression Scale. RESULTS: The mean onset age of disease was 55 years for PD patients without dementia and 59 for PD patients with MCI. According to the Hoehn-Yahr scales, 24% of patients had grade 1, 58% had grade 2, and 18% had grade 3 disease. Right and left hippocampal volumes decreased along with cognitive test scores in PD patients. Increased right hippocampal volume was correlated with forward number test in the MCI-PD group. CONCLUSION: These findings suggest that memory deficit is associated with hippocampal atrophy in PD patients.

Gender differences in older adults with chronic migraine in Turkey.

AIM: Chronic migraine is a growing and disabling subtype of migraine with different risk factors and clinical features, even in older adults. We sought to define and differentiate clinical features of chronic migraine in older adults. We also aimed to compare major clinical features of chronic migraine in older adults with those in younger people of both sexes. METHODS: We used electronic dataset (Turkish Headache Database) from 13 tertiary headache centers in Turkey. Electronic dataset included detailed headache-defining features according to ICHD-II criteria based on face-to-face interviews and examination by a headache specialist. Using statistical methods, clinical variables of chronic migraine in older adults were compared with those of younger adults. We included 915 patients with chronic migraine (mean age 43.80 ± 13.95 years); 83.3% were females. In total, 301 patients (32.9%) with chronic migraine aged >50 years were compared with 614 patients aged <50 years. RESULTS: There was no significant change in men with increasing age. However, duration of headache history, severity of attacks, previous histories of motion sickness and positive family history of headaches were significantly different in women with increasing age. Further sex-related differences have been shown in parameters such as attack duration, quality and associated nausea. CONCLUSION: Chronic migraine is an infrequent type of migraine and shows age-related changes in some phenotypic characteristics, such as severity of attacks, especially in women aged older than 50 years. Furthermore, positive family history of headaches and history of motion sickness increase the likelihood of developing chronic migraine in older women, indicating involvement of some gender-related, but as-yet unknown, genetic factors.

Creutzfeldt-Jakob disease: report of four cases and review of the literature.

Creutzfeldt-Jakob disease is a very rare, progressive neurodegenerative disorder that is incurable and always fatal. It is one of the transmissible spongiform encephalopathies caused by prions. Multiple vacuoles in neuropil and neuronal loss in the gray matter gives the classical sponge-like appearance of brain and are responsible for the typical clinical symptoms. In this report, we present 4 cases referred to the neurology department of Uludag University with neurological symptoms. Patients were evaluated with electroencephalogram and magnetic resonance imaging, and performed brain biopsies for further investigation. For definitive diagnosis of Creutzfeldt-Jakob disease, accumulation of prion protein in brain was detected immunohistochemically. Patients died within weeks in consequence of rapid progression of the disease. Although Creutzfeldt-Jakob disease is an infrequent disorder, when a patient presents with characteristic clinical symptoms such as rapidly progressive dementia with myoclonus, the diagnosis of Creutzfeldt-Jakob disease should be taken into consideration.

Sporadic Creutzfeldt-Jacob Disease: An 8-Year Experience from a Single Center in Turkey.

INTRODUCTION: Our aim was to conduct a retrospective review to demonstrate the clinical, radiological, and electrophysiological features of patients with sporadic Creutzfeldt-Jacob disease (sCJD). METHOD: A total of 10 patients (5 female and 5 male, with a mean age of 45 years from a range of 40 to 67 years) out of 8.259 adult patients hospitalized from January 2000 to December 2008 were diagnosed with sCJD. RESULTS: Eight of the patients were diagnosed on the basis of clinical, radiological, electroencephalography (EEG), and cerebrospinal fluid (CSF) findings. Two other patients also had a pathological diagnosis. The most common signs and symptoms were behavioral disturbances, movement disorders, cognitive decline, myoclonus, psychosis, focal neurological deficit, and aphasia. Nine of the patients had periodic sharp wave discharges on EEG. Seven patients were positive for the 14.3.3 protein in the CSF. Five patients had pulvinar signs-a bilateral increased signal in the pulvinar thalami-on cranial magnetic resonance imaging. Eight patients were diagnosed with probable sCJD; two were diagnosed with definite sCJD. All of the patients died as a result of the disease within 24 months after the onset of symptoms. DISCUSSION: sCJD should be considered in the diagnosis of patients who present with rapidly progressive dementia. Clinical and radiological data appear to be sufficient for the diagnosis. However, detailed molecular examinations of the subtypes of the disease may be required for early diagnosis of cases given the wide spectra of CJD.

Impact of sex hormonal changes on tension-type headache and migraine: a cross-sectional population-based survey in 2,600 women.

Sex hormones have some implications on headaches. The objective of the study was to investigate the effects of hormonal changes comparatively on tension-type headache (TTH) and migraine, in a population-based sample. A nationwide face-to-face prevalence study was conducted using a structured electronic questionnaire. 54.3 % of the migraineurs reported that the probability of experiencing headache during menstruation was high, whereas 3.9 % had headache only during menstruation. Forward logistic regression analysis revealed that menstruation was a significant trigger for migraine in comparison to TTH. On the other hand, nearly double the number of TTH sufferers reported "pure menstrual headache" compared to migraineurs (p = 0.02). Menstrual headaches caused significantly higher MIDAS grades. One-third of the definite migraineurs reported improvement during pregnancy and oral contraceptives significantly worsened migraine. Menopause had a slight improving effect on migraine compared to TTH. Sex hormonal changes have major impacts particularly on migraine; however, the effects of hormonal fluctuations on TTH should not be underestimated.