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Dünyada birçok kimyasal madde bulunmakta ve bu kimyasallarýn bir kýsmýnýn doz ya da maruz kalýnan süre iliþkili toksik etkileri bulunmaktadýr. Özellikle iyatrojenik nedenli olanlarýn etkileri daha iyi tanýmlanabilmektedir. Ancak, laboratuvarlarda toksik olduðu tespit edilen maddelerin günlük hayatta da (örneðin; iþyerlerinde) toksik etkiye sahip olduðunu kanýtlamak zordur. Günümüzde nörotoksik ajanlarýn patofizyolojik etkileri hala tam bilinmemektedir. Bu derlemede bilimsel olarak kanýtlanmýþ çevresel ve mesleksel ajanlarýn sinir sistemi üzerine olan nörotoksik etkilerine deðinilecektir.

There are numerous chemicals throughout the world, some of which have a dose- or exposure-related toxic effect. The effects of iatrogenic causes in particular can be better defined. It is hard to prove that in laboratories toxic substances have toxic effects in everyday life, however (e.g. workplaces). Today, the pathophysiological effects of neurotoxic agents are still unknown. This review discusses the neurotoxic effects on the nervous system of scientifically established environmental and occupational agents.

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Amaç: Putamendeki azalmýþ dopamin reseptör sayýsý, huzursuz bacak sendromu (HBS) patofizyolojisi için önemli bir neden olarak kabul edilmiþtir. Retina dopamin, retina hücreleri arasýnda iletiþim kurarak kontrast duyarlýlýðýnda (CS) önemli bir rol oynar. Ayrýca retinal dopaminerjik nöronlarýn diðer retina nöronlarýný koruduðu öne sürülmektedir. Bu çalýþmada HBS hastalarýnýn retina tabakalarýnýn kalýnlýðýný ve CS fonksiyonunu (CSF) araþtýrmayý amaçladýk.
Gereç ve Yöntem: Bu çalýþma ileriye dönük ve olgu kontrollü olup, katýlýmcý sayýsý 68’dir. HBS’li (n=35) hasta ve saðlýklý katýlýmcý (n=33) olmak üzere iki grup hasta vardýr.
Optik koherens tomografi, merkezi maküler kalýnlýk, retina sinir lifi tabakasý kalýnlýðý (RSLT) ve retina ganglion hücresi iç pleksiform tabaka kalýnlýðýný (GCIPL) ölçmek için kullanýldý. RSLT kalýnlýðý ortalama ve sektörel (üst, alt, nazal ve temporal) olarak ölçüldü.
Bulgular: Grup 1’de ortalama ve üst sektörel RSLT kalýnlýðý, grup 2’ye göre istatistiksel olarak anlamlý derecede daha ince idi (sýrasýyla; p=0,03 ve p=0,01). Bununla birlikte, diðer RSLT sektörlerinin kalýnlýklarý grup 1 ve grup 2 arasýnda farklý deðildi. Grup 1 ve grup 2 arasýnda GCIPL ve merkezi maküla kalýnlýklarý açýsýndan istatistiksel olarak anlamlý bir fark yoktu. Öte yandan, 1,5 derece baþýna döngü (cpd), 3 cpd, 6 cpd, 12 cpd ve 18 cpd gibi tüm uzamsal frekanslarda CSF’nin ortalama deðerleri grup 2’de grup 1’e kýyasla istatistiksel olarak anlamlý derecede yüksekti (tüm uzaysal alanlarda p<0,001) frekanslar).
Sonuç: RLS hastalarýnda retina katmanlarýnýn kalýnlýðý ve CSF’nin azalmasý retinal dopaminerjik disfonksiyonu destekleyebilir. Ek olarak non-invaziv olarak uygulanan bir OKT incelemesi HBS hastalarýnýn taný ve takibinde fayda saðlayabilir.

Objective: The decreased number of dopamine receptors in the putamen are considered to play an important role in the pathophysiology of restless legs syndrome (RLS). Retinal dopamine plays an important role in contrast sensitivity (CS) by establishing communication between retinal cells. It is also suggested that retinal dopaminergic neurons protect other retinal neurons. In this study, we aimed to investigate the thickness of retinal layers and CS function (CSF) of patients with RLS.
Materials and Methods: A total of 68 participants were included in this prospective case-control study. The participants were divided into two groups as those with RLS (n=35) in group 1 and control subjects (n=33) in group 2. Optical coherence tomography was used to measure central macular thickness, retinal nerve fiber layer thickness (RNFL), and retinal ganglion cell inner plexiform layer thickness (GCIPL). RNFL thickness was measured as average and as sectoral (superior, inferior, nasal and temporal). Additionally, the CS test measured at all spatial frequencies of 1.5, 3, 6, 12 and 18 cycle per degree (cpd) was performed in all participants.
Results: The average and superior sectoral RNFL thicknesses in group 1 were statistically significantly thinner than group 2 (p=0.03 and p=0.01 respectively). However, the thicknesses of other RNFL sectors were not different between group 1 and group 2. There were no statistically significant differences between group 1 and group 2 regarding the thicknesses of GCIPL and central macula. The mean values of CSF at all spatial frequencies of 1.5 cpd, 3 cpd, 6 cpd, 12 cpd and 18 cpd were statistically significantly higher in group 2 in comparison to group 1 (p<0.001 for all spatial frequencies).
Conclusion: The decreased thickness of retinal layers and reduced CSF in patients with RLS support retinal dopaminergic dysfunction.

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Amaç: Bu çalýþmada yoðun bakým ünitesine (YBÜ) yatan nörolojik manifestasyonlarý olan koronavirüs hastalýðý-2019 (COVID-19) hastalarýnýn verilerini deðerlendirmeyi amaçladýk.
Gereç ve Yöntem: Çalýþmaya nöroloji YBÜ’de nörolojik manifestasyonlar ile yatan COVID-19 hastalarýnýn verileri dahil edildi. Hastalarýn demografik özellikleri, risk faktörleri, laboratuvar sonuçlarý, tedavi yöntemleri, mekanik ventilasyon süreçleri, O2 ihtiyacýný karþýlayan non-invazif yöntemlerin kullanýmý, taburculuk ve 3. ayda klinik sonlaným oranlarý, ölüm oranlarý deðerlendirildi.
Bulgular: Çalýþmaya nörolojik prezentasyonu olan toplam 25 COVID-19 hastasý dahil edildi. Hastalarýn yaþ ortalamasý 66,76±12,93 (36-83) idi. Yüzde 52 oranýný erkek cinsiyet oluþturuyordu. Hastalarýn %96’sýnda polimeraz zincir reaksiyonu testi pozitif, %92 oranýnda akciðer tutulumu vardý. Hastalarýn komorbitesini %48 hipertansiyon, %28 diabetes mellitus ve koroner arter hastalýðý, %20 serebrovasküler hastalýk, %20 kanser, %12 kronik obstriktif akciðer hastalýðý oluþturdu. Hastalarýn %72’si inme ile prezente olan COVID-19 grubundan oluþuyordu. Yüzde 12 ensefalopati tablosunda gelen COVID-19 hastalarýndan, %8 epilepsi ve miyastenia gravis semptomlarý ile gelen hastalardan oluþuyordu. Hastalarýn ortalama YBÜ yatýþ süresi 13,16±12,44 gündü. Hastalarýn %56’sý yüksek mekanik ventilatör ayarlarýnda, senkronize intermittent mekanik ventilasyon modunda takip edildi. Entübasyon gün sayýsý 9,5±12,26 idi. Hastalarýn tümüne favipravir ve antiagregan, %92’sine steroid ve antibakterial tedavi, %52’sine antiagregan, %20’sine plazma tedavisi, %8’ine sitokin filtiresi ve immünoglobulin tedavisi, %4’üne monoklonal antikor tedavisi uygulandý. Hastalarýn yatýþý sürecinde ölümle sonuçlanma oraný %48, iyi klinik sonlaným oraný %36, saptandý.
Sonuç: Nörolojik belirtilerle gelen COVID-19 hastalarýnýn hastane içi tedavi ve takip sürecinde, ayný zamanda taburculuklarýnda kötü klinik sonlaným oranlarý yüksek görülmektedir.

Objective: This study aims to evaluate data from coronavirus disease-2019 (COVID-19) patients with neurological manifestations hospitalized in the intensive care unit (ICU).
Materials and Methods: The study included data from COVID-19 patients with neurological manifestations hospitalized in ICU. Patients’ demographic characteristics, risk factors, laboratory results, treatment methods, mechanical ventilation methods, use of non-invasive techniques to meet O2 requirements, clinical outcome at discharge and after three months, and mortality rates were evaluated.
Results: The study included 25 patients. Mean age of the patients was 66.76±12.93. Fifty two percent of the patients were male. Of the patients 96% had a positive polymerase chain reaction test, and 92% had lung involvement. The comorbidities of the patients were hypertension (48%), diabetes mellitus (28%), coronary artery disease (28%), cerebrovascular disease (20%), cancer (20%), and chronic obstructive pulmonary disease (12%). Seventy two percent of the patients presented with stroke. Of the patients %12 presented with encephalopathy, whereas patients with epilepsy and myasthenia gravis accounted for 8%. Patients’ average length of stay in ICU was 13.16±12.44 days. Pressure-synchronized intermittent mandatory ventilation mode was used in 56% of patients. Number of intubation days was 9.5±12.26. All patients were treated with favipiravir and antiaggregant, 92% with steroids and antibacterial therapy, 52% with antiaggregant, 20% with plasma therapy, 8% with cytokine filter and immunoglobulin therapy, and 4% with monoclonal antibody therapy. During hospitalization, mortality rate was 48%, good clinical outcome rate was 36%.
Conclusion: The rates of poor clinical outcomes are seen to be high during in-hospital treatment and follow-up of COVID-19 patients presenting with neurological symptoms, as well as at discharge.

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Amaç: El becerisinde bozulma, Parkinson hastalýðý (PH) olan bireylerde en fazla engelleyici semptomlardan biri olmasýna raðmen, el becerisini deðerlendirmek için güvenilir ve geçerli hasta tarafýndan bildirilen sonuç ölçümü (PROM) bulunmamaktadýr. Bu nedenle, bu çalýþma: (1) PH’de el beceri ölçümü-36’nýn (EBÖ- 36) test-tekrar test güvenilirliðini; (2) EBÖ-36 puanlarýndaki minimal saptanabilir deðiþimi (MSD); (3) EBÖ-36 puanlarýnýn eþzamanlý ve bilinen gruplar geçerliliðini; ve (4) saðlýklý bireylerden PH’leri en iyi ayýran kesme puanýný araþtýrmayý amaçladý.
Gereç ve Yöntem: Bu kesitsel bir çalýþmaydý. EBÖ-36, sýnýf içi korelasyon katsayýsý (ICC), Bland-Altman grafikleri ve MSD ile hesaplanan test-tekrar test güvenilirliðini belirlemek için 4 hafta sonra tekrarlandý. Eþ zamanlý geçerlilik, EBÖ-36 ve dokuz delikli peg testi, Hareket Bozukluklarý Derneði Birleþik Parkinson Hastalýðý Derecelendirme ölçeði (HBD-BPHDÖ), HBD-BPHDÖ II, HBD-BPHDÖ III, Hoehn ve Yahr evresi ve 8 maddelik PH anketi arasýndaki korelasyonlar kullanýlarak deðerlendirildi. Bilinen gruplar geçerliliði, Parkinson hastalarý ve saðlýklý bireyler arasýnda EBÖ-36 puanlarýnýn karþýlaþtýrýlmasýyla belirlendi. Alýcý iþletim karakteristiði analizi Parkinson hastalarýný saðlýklý bireylerden en iyi ayýrt eden EBÖ-36’nýn kesme puanýný belirlemek için kullanýldý.
Bulgular: Otuz altý Parkinson hastasý ve 32 saðlýklý birey dahil edildi. Mükemmel test-tekrar test güvenilirliði bulundu (ICC: 0,953). Bland-Altman grafiði yüksek bir uyum gösterdi. MSD 2,33’tü. EBÖ-36 diðer sonuç ölçümleri ile orta ila yüksek korelasyonlara sahipti (korelasyon katsayýlarý -0,473 ile -0,763 arasýndaydý, p<0,05 hepsi için). EBÖ-36’da; Parkinson hastalarý saðlýklý bireylerden daha düþük puanlara sahipti (p<0,001). 76,50’lik kesme puaný, saðlýklý bireylerden Parkinson hastalarýný en iyi ayýrt eder.
Sonuç: EBÖ-36, Parkinson hastalarýnda el becerisini deðerlendirmek için güvenilir ve geçerli bir ölçümdür. Ayrýca, Türk popülasyonunda Parkinson hastalarý için el becerisinin deðerlendirilmesinde klinik olarak kullanýlabilir tek PROM’dur.

Objective: Although impaired manual dexterity is one of the most disabling symptoms in patients with Parkinson’s disease (PD), there is no reliable and valid patient-reported outcome measure (PROM) for assessing dexterity. Therefore, this study aimed to investigate: (1) The test-retest reliability of the manual ability measure-36 (MAM-36) in patients with PD; (2) the minimum detectable change (MDC) in the MAM-36 scores; (3) the concurrent and known-groups validity of the MAM-36 scores; and (4) the cut-off score that best discriminates patients with PD from healthy individuals.
Materials and Methods: This was a cross-sectional study. The MAM-36 was repeated after 4 weeks to determine the test-retest reliability which was calculated with the intraclass correlation coefficient (ICC), Bland-Altman plots, and MDC. The concurrent validity was assessed using correlations between the MAM-36 and nine-hole peg test, Movement Disorders Society Unified Parkinson’s Disease Rating scale (MDS-UPDRS), MDS-UPDRS II, MDS-UPDRS III, Hoehn and Yahr stage, and 8-item PD questionnaire. The known-groups validity was determined by comparing the MAM-36 scores between patients with PD and healthy individuals. Receiver operating characteristic analysis was used to determine the cut-off score for the MAM-36 to best discriminate patients with PD from healthy individuals.
Results: Thirty six patients with PD and 32 healthy individuals were included. Excellent test-retest reliability was found (ICC: 0.953). The Bland-Altman plot demonstrated a high agreement. The MDC was 2.33. The MAM-36 had fair to high correlations with the other outcome measurements (correlation coefficients ranged from -0.473 to -0.763, p<0.05 for all). Patients with PD had lower scores than healthy individuals in terms of the MAM-36 (p<0.001). The cut-off score of 76.50 best distinguished patients with PD from healthy individuals.
Conclusion: The MAM-36 is a reliable and valid measurement for assessing manual dexterity in patients with PD. It is also the only clinically available PROM in assessing manual dexterity for patients with PD in the Turkish population.

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Amaç: Leptin, inme de dahil olmak üzere sayýsýz koþuldaki rolü nedeniyle uzmanlarýn dikkatini çekmiþtir. Bununla birlikte, leptini inme ile iliþkilendiren güvenilir kanýtlarýn yokluðunda, bir risk faktörü olarak rolü hala tartýþmalýdýr. Bölgemizdeki iskemik inme hastalarýnda leptin düzeylerini ve diðer kardiyometabolik risk faktörleri ile iliþkisini belirlemeyi amaçladýk.
Gereç ve Yöntem: Bu çalýþma, 100 hasta ile yaþ ve cinsiyet açýsýndan eþleþtirilmiþ 100 saðlýklý kontrolü içeren hastane temelli gözlemsel bir olgu-kontrol çalýþmasýdýr. Lipid profili, insülin seviyesi ve açlýk kan þekeri ölçümüne ek olarak hem hastalarda hem de kontrollerde leptin seviyeleri ölçüldü. Ýnsülin direnci insulin direncinin hemostatik modelle deðerlendirilmesi modeli kullanýlarak hesaplandý. Karotis intima-medya kalýnlýðý (KÝMK), yüksek çözünürlüklü eko-renkli Doppler sistemi (GE LOGIQ S8) ile belirlendi. Nörogörüntülemede yapýldý. Sürekli deðiþkenler için Student’s t-testi, kategorik deðiþkenler için ki-kare testi ve leptin düzeylerinin çeþitli parametrelerle korelasyonunu belirlemek için Pearson korelasyon katsayýsý kullanýldý.
Bulgular: Ortalama leptin düzeyleri hastalarda kontrol grubuna göre anlamlý derecede yüksekti (15,36±5,76 vs. 5,72±1,19 ng/ml; p<0,001). Leptin ile iskemik inmeli hastalarda aterojenik lipid profili, enflamatuvar belirteçler yani beyaz kan hücreleri ve eritrosit sedimentasyon hýzý, açlýk plazma glukozu ve insülin direnci ile pozitif korelasyonlar bulunurken, leptin ile yüksek yoðunluklu lipoproteinler arasýnda ters bir korelasyon vardý. Benzer þekilde hastalarda leptinin sistolik kan basýncý, vücut kitle indeksi ve KÝMK ile pozitif iliþkisi olduðu bulundu.
Sonuç: Hiperleptinemi iskemik inmeli hastalarda sýk görülen bir bulgudur ve iskemik inmeye yatkýnlýk oluþturduðu bilinen çeþitli kardiyovasküler risk faktörleri ile anlamlý þekilde iliþkilidir.

Objective: Leptin has drawn the attention of experts for its role in a myriad of conditions including stroke. However, in the absence of credible evidence linking leptin to stroke, its role as a risk factor is still debatable. We aimed to determine the leptin levels and its relationship with other cardiometabolic risk factors in patients with ischemic stroke in our region.
Materials and Methods: It was a hospital based case-control observational study involving 100 patients and 100 age- and sex- matched healthy controls. Leptin levels were measured in both patients and controls in addition to estimation of lipid profile, insulin level and fasting blood glucose. Insulin resistance was calculated using the homeostasis model assessment for insulin resistance model. Carotid intima-media thickness (CIMT) was determined by high-resolution echocolour Doppler system (GE LOGIQ S8). Neuroimaging was also performed. Student’s t-test for continuous variables, chi-square test for categorical variables and Pearson correlation coefficient to determine the correlation of leptin levels with various parameters were used.
Results: The mean leptin levels were significantly higher in patients compared to control group (15.36±5.76 vs. 5.72±1.19 ng/ml; p<0.001). Leptin was found to have positive correlations with atherogenic lipid profile, inflammatory markers i.e, white blood cells and erythrocyte sedimentation rate, fasting plasma glucose and insulin resistance in patients with ischemic stroke whereas there was an inverse correlation between leptin and high density lipoproteins. Similarly leptin was found to have positive association with systolic blood pressure, body mass index and CIMT in patients.
Conclusion: Hyperleptinemia is a common finding in patients with ischemic stroke and is significantly associated with various cardiovascular risk factors known to predispose to ischemic stroke.

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Amaç: Friedreich ataksisi (FRDA) progresif yürüyüþ ve ekstremite ataksisi, dizartri, duyu kaybý ve kas güçsüzlüðü ile karakterize en sýk kalýtsal ataksidir. Birçok hastalýkta, özellikle nadir hastalýklarda; hasta ve hasta yakýnlarýnýn yetersiz farkýndalýðý, saðlýk uzmanlarýnýn bilgi birikiminin yeterli olmamasý ve taný testlerine eriþmekte güçlük nedeniyle taný gecikmesi görülür. Bu çalýþmada, FRDA’lý bireylerin taný gecikmesi ve klinik özellikleri araþtýrýlmýþtýr.
Gereç ve Yöntem: Harding klinik taný kriterlerini karþýlayan ve genetik incelemede artmýþ GAA trinükleotid tekrarý olan bireyler çalýþmaya alýnmýþtýr. Hastalarýn baþlangýç semptomlarý, semptom baþlangýç yaþý, semptom baþlangýcýndan tanýya kadar geçen süre ve komorbid durumlar gibi demografik ve klinik verileri kaydedilmiþtir. Ayrýca hastalarýn detaylý nörolojik muayeneleri yapýlmýþ ve ataksi deðerlendirme ve derecelendirme skalasý (SARA) ile kaydedilmiþtir.
Bulgular: Çalýþmaya 12 erkek ve 10 kadýn olmak üzere toplam 22 hasta dahil edildi. Bireylerin yaþ ortalamasý 30,4±6,3 (19-41) idi. Semptom baþlangýç yaþý 19±5,6 (9-32), taný yaþý 22,1±6,1 (13-33) ve semptomlarýn baþlamasýndan tanýya kadar geçen süre 3,03±2,66 (0,2-9) yýl idi. On dokuz olgunun ilk belirtisi (%86,4) gövde ataksisi iken, 3 olgunun (%13,6) ilk belirtisi ekstremite ataksisi idi. Sekiz (%36,4) olgu ambulatuvar iken 14 (%63,6) olgu non-ambulatuvar idi. Ortalama toplam SARA skoru 18,2±6,7 idi [medyan 19,5 (7-30)].
Sonuç: Bu çalýþma ülkemizde FRDA hastalarýnda taný gecikmesini deðerlendiren ilk çalýþmadýr. Her ne kadar FRDA en sýk kalýtsal ataksi olsa da, çalýþmamýzda FRDA’lý hastalarda tanýda önemli bir gecikme olduðu gösterilmiþtir. Toplumun ve saðlýk profesyonellerinin FRDA konusunda farkýndalýklarýný artýracak çalýþmalara ihtiyaç vardýr.

Objective: Friedreich ataxia (FRDA) is the most frequent hereditary ataxia characterized by progressive gait and limb ataxia, dysarthria, sensory loss, and muscular weakness. Due to insufficient awareness of patients and their relatives, poor knowledge of healthcare professionals, and difficulty in accessing diagnostic tests, delay in diagnosis is seen in many diseases, especially in rare diseases. In this study, the diagnostic delay, and clinical features of individuals with FRDA were investigated.
Materials and Methods: Individuals who fulfilled the criteria for Harding clinical diagnosis and who had increased GAA repetition in the genetic examination were included in the study. Demographic and clinical data of the patients such as initial symptoms, age at symptom onset, time from the onset of symptoms to the diagnosis, and comorbid conditions were recorded. In addition, detailed neurological examinations of the patients were made, and they were evaluated with the scale for the assessment and rating of ataxia (SARA).
Results: A total of 22 patients, 12 males, and 10 females, were included in the study. The mean age of the individuals was 30.4±6.3 (19-41). Age of symptom onset was 19±5.6 (9-32), age at diagnosis was 22.1±6.1 (13-33), and the time from onset of symptoms to diagnosis was 3.03±2.66 (0.2-9) years. While the first symptom of 19 patients (86.4%) was trunk ataxia, the first symptom of 3 patients (13.6%) was extremity ataxia. Eight (36.4%) patients were non-ambulatory and 14 (63.6%) were ambulatory. The mean total SARA score was 18.2±6.7 [median 19.5 (7-30)].
Conclusion: This study is the first study to evaluate the diagnosis delay in patients with FRDA in our country. Although FRDA was the most common hereditary ataxia, in our study, it was shown that there was a significant delay in diagnosis in patients with FRDA. There is a need for studies that will raise awareness of public and health professionals about FRDA.

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Amaç: Bu araþtýrmada yaygýn olarak kullanýlan nöropsikolojik deðerlendirme araçlarýndan sözel akýcýlýk testlerinin güncel norm deðerlerinin oluþturulmasý ve geçerlik-güvenirlik incelemelerinin yapýlmasý hedeflenmiþtir.
Gereç ve Yöntem: Norm deðerlerinin oluþturulmasý amacýyla cinsiyet, yaþ ve eðitime göre tabakalandýrýlmýþ 1.431 saðlýklý bireye fonemik ve semantik akýcýlýk testleri uygulanmýþtýr. Testlerin eþ-zaman geçerlik, iç-tutarlýlýk ve test-tekrar test deðerlendirmeleri yapýlmýþtýr. Cinsiyet, yaþ ve eðitimin sözel akýcýlýk üzerindeki etkileri adýmsal doðrusal regresyon analizi ile incelenmiþtir.
Bulgular: Sonuçlar sözel akýcýlýk testlerinin yüksek düzeyde iç-tutarlýlýk ve kabul edilir düzeyde test-tekrar test güvenirliði olduðunu göstermiþtir. Cinsiyetin sözel akýcýlýk puanlarýna etkisi görülmezken, yaþ ve eðitim fonemik ve semantik akýcýlýðý sýrasýyla %37 ve %25 oranýnda açýklamaktadýr. Yaþ arttýkça sözel akýcýlýk performansý düþmekte, eðitim arttýkça yükselmektedir.
Sonuç: Semantik ve fonemik sözel akýcýlýk testleri norm deðerleri 18-89 yaþ aralýðýndaki bireylerde dört farklý eðitim düzeyinde sunulmuþtur.

Objective: This study aimed to provide updated normative data for one of the widely used neuropsychological assessment tools, namely verbal fluency tests, and examine their reliability and validity.
Materials and Methods: To establish normative data stratified by gender, age and education, a total of 1,431 healthy participants were administered phonemic and semantic verbal fluency tests. Concurrent validity, internal consistency and test-retest reliability analyses were conducted. The effect of gender, age and education was tested through stepwise linear regression analysis.
Results: The results revealed good internal consistency and acceptable level of test-retest reliability for verbal fluency tests. While gender did not have any effect on test performance, the combined contribution of age and education on phonemic and semantic fluency performance were 37% and 25%, respectively. Verbal fluency performance decreased as a function of age, whereas it increased as a function of education.
Conclusion: The normative data for phonemic and semantic fluency performance between the ages 18 and 89 stratified by four education levels was presented.

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Amaç: Araþtýrmamýzýn amacý Ege Üniversitesi Nöroloji Baþ Aðrýsý Polikliniði’ne 4 saatten kýsa süren baþ aðrýsý ile baþvuran hastalarýn demografik ve klinik özelliklerini, tedavi özelliklerini ve tedavi yanýtlarýný deðerlendirmektir. Bir diðer ana amaç ise kraniyal otonomik bulgulara sahip olan ve sahip olamayan hastalarýn yukarýda belirtilen özelliklerini karþýlaþtýrmaktýr.
Gereç ve Yöntem: Çalýþmamýz retrospektif ve kesitseldir. Bulgular, üçüncül baþ aðrýsý merkezimizde toplanan verilere dayanmaktadýr. Baþ aðrýsý bozukluklarýnýn tanýlarý International Classification of Headache Disorders-3 kýlavuzuna göre konmuþtur. Hastalarýn dosyalarý incelenmiþtir ve bilgileri standart bir olgu rapor formuna kaydedilmiþtir. Hastalarýn demografik özellikleri, klinik özellikleri ve tedavi yanýtlarý not edilmiþtir. Hastalar otonomik bulgulara sahip olan ve sahip olamayan hastalar olarak iki gruba ayrýlmýþtýr ve yukarýda belirtilen özellikleri karþýlaþtýrýlmýþtýr. Trigeminal otonomik sefalji ve trigeminal nevralji (TN) hastalarýnýn tedavi yanýtlarý lojistik regresyon analizi ile deðerlendirilmiþtir. p<0,05 deðerleri istatistiksel olarak anlamlý kabul edilmiþtir.
Bulgular: Altmýþ sekiz hasta dahil edildi. Yirmi beþ hastada trigeminal otonomik sefalji vardý. Yirmi üç hastada TN, 13 hastada gerilim tipi baþ aðrýsý vardý. Yedi hastada baþka birincil baþ aðrýsý vardý. Hastalarýn klinik özellikleri ve tedavi yanýtlarý alt gruplarda farklýydý. Trigeminal otonomik sefalji hastalarýnda tetikleyici faktör tarif etmenin atak tedavisi alma riskini düþürdüðü saptandý [Odds oraný (OR): 0,059, %95 güven aralýðý (GA): (0,005-0,645); p=0,02]. TN hastalarýnda beyin sapý lezyonuna sahip olmak [OR: 24,776, %95 GA: (1,033-60,613); p=0,049] uzun dönemde karbamazepin tedavisinde baþarýsýzlýk riskini artýrýrken günde birden fazla nevralji ataðý yaþamak [OR: 0,58, %95 GA: (0,04-0,832); p=0,036] azaltmaktaydý.
Sonuç: Baþ aðrýsý bozukluklarýnýn doðru teþhisi, temel olarak baþ aðrýsýnýn klinik özelliklerine dayanýr. Etkili bir tedavi için doðru bir teþhis zorunludur, doðru teþhis tümüyle uygun öykü alýnmasýna dayanmaktadýr.

Objective: The aim of our research was to evaluate the demographic and clinical features, treatment characteristics, and responses of the patients who presented to the Ege University Neurology Headache Outpatient Clinic with headaches lasting less than 4 hours. Another primary goal was to compare the characteristics mentioned above of patients with and without cranial autonomic symptoms.
Materials and Methods: Our study was retrospective and cross-sectional. The findings were based on the data collected at our tertiary headache center. Headache disorder diagnoses were made in accordance with the International Classification of Headache Disorders-3 guideline. We reviewed the patients’ charts and recorded the information on a standardized form. The patients’ clinical features, and treatment responses were noted. The patients were divided into two groups as those with and without autonomic findings, and the above-mentioned features were compared. Treatment responses of patients with trigeminal autonomic cephalgia (TAC) and patients with trigeminal neuralgia (TN) were analyzed via logistic regression analysis. Values of p<0.05 were considered statistically significant.
Results: Sixty-eight patients were included. Twenty-five patients had TAC. Twenty-three patients had TN and 13 had tension type headache. Seven had other primary headaches. The patients’ clinical features and treatment responses were different in the subgroups. In patients with TAC, having a triggering factor [Odds ratio (OR): 0.059, 95% confidence interval (CI): (0.005-0.645); p=0.02] reduced the risk of need of attack treatment. In patients with TN, having a brainstem lesion on cranial magnetic resonance imaging sequences [OR: 24.776, 95% CI: (1.033-60.613); p=0.049] increased the risk of long-term treatment failure with carbamazepine, whereas having headache attacks more than once a day [OR: 0.58, 95% CI: (0.04-0.832); p=0.036] decreased the risk.
Conclusion: The correct diagnosis of headache disorders is mainly based on the clinical features of the headache. For an effective treatment initiation, a correct diagnosis is mandatory, entirely dependent on adequate history taking.

Abstract | English Full Text

Beyinde demir birikimi ile seyreden nörodejenerasyon [neurodegeneration with brain iron accumulation (NBIA)], çeþitli klinik belirtiler ve manyetik rezonans görüntülemede (MRG) tespit edilen beyinde demir birikimi ile karakterize, çocuklarý ve yetiþkinleri etkileyen bir dizi kalýtsal bozukluðu kapsar. En sýk görülen NBIA alt tipleri pantotenat kinaz ile iliþkili nörodejenerasyon, fosfolipaz A2 ile iliþkili nörodejenerasyon, yað asidi-2 hidroksilaz ile iliþkili nörodejenerasyon ve mitokondriyal membran proteini ile iliþkili nörodejenerasyondur (MPAN). Burada optik atrofi, ilerleyici biliþsel bozukluk, hareket bozukluðu, T2 aðýrlýklý MRG’de bazal ganglionlarda bilateral hipointensite ile baþvuran ve MPAN için kanýtlanmýþ bir mutasyon tespit edilen bir erkek hastayý sunuyoruz. Beyinde demir birikimi ile seyreden nörodejeneratif bozukluklar 3 ila 30 yýl boyunca teþhis edilmeden kalabilir. Optik atrofi geliþen çocuklarda NBIA düþünülmelidir.

Neurodegeneration with brain iron accumulation (NBIA) encompasses a number of heritable disorders affecting children and adults characterized by diverse clinical manifestations and brain iron deposition detected on magnetic resonance imaging (MRI). The most frequent NBIA subtypes are pantothenate kinase-associated neurodegeneration, phospholipase A2-associated neurodegeneration, fatty acid-2 hydroxylase-associated neurodegeneration and mitochondrial-membrane proteinassociated neurodegeneration (MPAN). Here, we report a male patient presenting with optic atrophy, progressive cognitive and movement impairment, bilateral hypointensity of the basal ganglia on T2-weighted MRI and proven mutation for MPAN. The NBIA disorders can remain undiagnosed for 3 to 30 years. In children developing optic atrophy, NBIA should be taken into consideration.

Abstract | English Full Text

Abstract | English Full Text

Abstract | English Full Text

Abstract | English Full Text