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Multiple sclerosis (MS) is a chronic central nervous system inflammatory disease. Fibrinogen/Albumin ratio (FAR) has been studied as an inflammatory marker in the past, and its significant relationship with inflammation has been shown. In our study, we examined serum levels of albumin, fibrinogen and FAR in patients presenting with MS attack. We investigated its usability in the diagnosis and management of MS attacks.This retrospective study included 40 patients admitted to hospital with MS attack and 40 control patients. All patients' demographics, medical history, the mean Expanded Disability Status Scale (EDSS), imaging findings and laboratory tests were extracted from medical records. Patients' fibrinogen, albumin levels were recorded in the blood tests performed before steroid administration, and FAR values were calculated. The patients' and controls' results were compared.Fibrinogen and FAR values were significantly higher in the patient group (p0.001 in both). There was no difference between the patient and control groups in terms of albumin (p = 0,16). No significant relationship was found between parameters such as EDSS, disease duration, smoking status and FAR value (p0.05 in all).Serum fibrinogen and FAR levels in the patients presenting with an attack were significantly higher than the control group. FAR value did not vary with disease duration and EDSS score. Based on this information, FAR may be a useful indicator for MS attack regardless of EDSS and disease duration.

Introduction: Parkinson’s disease (PD) is the second common neurodegenerative disease following Alzheimer’s disease. Multifactorial risk factors including environmental, nutritional and genetic factors may affect the frequency, risks, and genetic features of PD in different geographic and ethnic regions. Background: Our aim was to investigate the sociodemographic and disease characteristics, cognitive performances of patients with PD in Nusaybin, a southeastern city, and Hendek a northwestern city which have different geographical-cultural status, and to investigate the possible effects of regional differences on disease characteristics. Method: This retrospective study includes the data of patients with the diagnosis of PD who presented to outpatient Neurology clinics of Hendek State Hospital which is a city in the northwestern region of Turkey between April 2017 and June 2018 and patients who presented to the outpatient Neurology clinics of Nusaybin State Hospital, which is a city in the southeastern region of Turkey between October 2015 and January 2017. SPSS.23c (Statistical package for Windows) database was used for statistical analysis. Results: Our study included 114 cases with Parkinson’s disease in Hendek and 96 cases in Nusaybin.The majority of patients had idiopathic PD (IPD) in both provinces (96.9% in Hendek, and 92.1% in Nusaybin). Educational status of female patients was significantly lower in Nusaybin when compared with Hendek (9.3% and 63.3%, respectively). Family history for IPD was significantly higher in Hendek than Nusaybin (31.7% and 9.9%, respectively). There was a positive correlation between educational status and cognitive performances of the patients in both groups. Negative correlation was found between standardized mini mental test (s-mmt) scores and stage, severity of IPD in both groups. Conclusion: The socio-cultural status of our country decreases by going from west to east. Thus knowledge about PD, accessibility to the hospitals and health centers with the opportunity to get optimum treatments may show variabilities in between the regions. We believe that our study provides a comparison of patients with PD in two different regions of our country with different geographic, genetic characteristics, and development levels.

Editore Mektup, Eriskin yasa dek tani almamis, ayni aileden alti erkek bireyin etkilendigi herediter spastik paraparezi (HSP) olgularini tanimladik. Bu yazida, ender gorulen bu hastaligin bulgulari ve genetik teshisinin ulkemizde zorluklari hakkindaki goruslerimizi literatur esliginde sunuyoruz. HSP, alt ekstremite kaslarinda gucsuzluk ve spastisite ile semptom veren, progresif seyirli nadir bir norojeneratif hastaliktir (1). Klinik ve genetik olarak heterojenite gosterir. Fenotipe gore "saf" HSP ve "komplike" HSP olarak iki genis gruba ayrilir. Genetik olarak otozomal dominant, otozomal resesif, mitokondrial veya X’ e bagli resesif gibi cesitli kalitsal gecis ozellikleri tespit edilebilir (2). Genellikle otozomal dominant gecisli olmasina ragmen, akraba evliliklerinin siklikla goruldugu toplumlarda resesif tip de siklikla ortaya cikmaktadir. Hastaligin klinik tablosu, kortiko-spinal traktusun uzunluga bagimli dejenerasyonuna baglidir. Oncelikle alt ekstremiteleri innerve eden en uzun lifler etkilenmistir. Karakteristik olarak, alt ekstremite kas gucsuzlugu, spastisite ve ekstansor plantar ortaya cikmaktadir. Bunun yaninda bircok olguda, ataksi, agir amiyotrofi, optik atrofi, pigmenter retinopati, mental retardasyon, ekstrapiramidal bulgular, demans, sagirlik, iktiyozis, periferal noropati ve epilepsi gibi bulgular eslik edebilmekte ve bu grup olgular komplike HSP olarak adlandirilmaktadir (3). Bu yazida, ikinci dekaddan sonra sag bacakta incelme ve agri ile semptomatik olan, ilerleyici yurume guclugune sahip 41 yasindaki erkek hastamiz ailesindeki bireylerle birlikte incelendi. Olgunun detayli norolojik ve fizik muayene bulgulari, laboratuvar testleri, goruntuleme ozellikleri, elektrofizyolojik ve genetik incelemeler ile birlikte degerlendirildi. Indeks olgumuz ve ailesindeki etkilenmis alti erkekte mitokondriyal gecis dusundurdu (Sekil 1). Indeks olgumuz, ikinci dekaddan sonra farkettigi sag bacakta incelme, ve son alti ayda ortaya cikan bacaklarda agri ve ilerleyici yurume guclugu ile klinigimize basvurdu. Oz gecmisinde ozellik yoktu. Anne ve babasi ayni koyden idi, ancak aralarinda akrabalik yoktu. Ailesinde babada, uc amcada ve dedede yurume guclugu oldugu ogrenildi. Norolojik muayenesinde; yuksek damak, paraparezi (4/5 motor kuvvet), bilateral alt ekstremitelerde belirgin atrofi, , gastroknemius kaslarinda psodohipertrofi dikkat cekmekteydi. Bilateral pes-cavus, cekic parmak ve spastisite, spastik yuruyus, dort ekstremitede artmis derin tendon refleksleri, bilateral ekstansor taban cildi refleksi mevcuttu. Kas gucu kaybi veya duyusal bulgulari yoktu. Kranyal ve spinal goruntulemesi normal, serum kreatinin kinaz (CK) duzeyi 63 (N: 20-200 mU/L) idi. Akrabalari arasinda 96 yasindaki anneanne, 72 ve 68 yasindaki halalari ve annesinin norolojik muayenesi normal idi. 54 yasindaki babanin yuksek damak, sol alt ekstremitesinde hafif spastisite ve derin tendon reflekslerinin canli olmasi disinda norolojik muayenesi dogal idi. 7-8 yildir yurume guclugu yasayan 61 yasindaki amcanin norolojik muayenesinde, alt ekstremitede belirgin spastisite, dort ekstremitede canli derin tendon refleksleri ve bilateral ekstansor taban cildi refleksi mevcut olup, yuruyus spastik idi. 4-5 yildir yurume guclugu yasayan 60 yasindaki amcanin norolojik muayenesinde; torakal kifoskolyoz, yuksek damak, alt ekstremitede hakim spastisite mevcuttu ve derin tendon refleksi dort ekstremitede canli, taban cildi bilateral lakayt idi. 3-4 yildir yurume guclugu yasayan 51 yasindaki amcanin norolojik muayenesinde; yuksek damak, hafif spastik yuruyus disinda ozellik yoktu. Hastalik oykusu olan diger amcaya ulasilamadi. HSP tanisi; normal norolojik gelisimi takip eden, alt ekstremitede hakim spastisite, kas gucsuzlugu, hiperrefleksi ve ekstansor plantar yanitin varligi, olasi edinsel nedenlerin dislanmasi ve/veya aile oykusunun varligi ile konulmaktadir. Inceledigimiz alti olguda HSP ile uyumlu norolojik muayene bulgulari mevcuttu. HSP genleri tarafindan kodlanan proteinler, hucre ici membran ve protein akisinda, aksonal transportta, hucre iskeleti duzenlenmesinde ve mitokondriyal islevlerde rol alir. Spastik paraparezi 7 (SPG7) ve SPG13 mitokondrial hastaliklar grubundandir (4). Pedigri degerlendirildiginde hastaligin mitokondriyal kalitildigi dusunulen ve genetik incelemeye yonlendirilen olgularimizin ekzom analizi surmektedir. HSP’de klinik bulgular genellikle 3-5. dekadda baslar. Bulgular saf HSP ile uyumlu olabilecegi gibi ek norolojik veya sistemik bulgular da gorulebilir. Siklikla Italyan ve Fransiz ailelerde tanimlanmistir. Piramidal bulgular yani sira, beyin sapi bulgulari, derin duyu bozuklugu, alt ekstremitelerde belirgin gucsuzluk, pes kavus, skolyoz gibi iskelet deformiteleri de gorulebilir (4,5). Serum CK duzeyleri artabilir, kranyal manyetik rezonans goruntulemede (MRG) serebral veya serebellar atrofi gorulebilir. Bizim indeks olgumuzda ve ailesindeki bireylerde CK duzeyi ve norogoruntuleme bulgulari normal idi. HSP ayirici tanisinda spinal kord hasarlanmalari, infeksiyoz, iskemik, metabolik, inflamatuvar myelopatiler goz onunde bulundurulmalidir (3). Biz de indeks olgumuz ve diger inceledigimiz aile bireylerinde, oyku ve klinik degerlendirme, vitamin B12, folik asit, ferritin duzeyleri gibi laboratuvar testleri, kranyal ve servikal MRG ile olasi edinsel nedenleri disladik. Klinik yansimalar ve genetik ozellikler birlikte incelendiginde, bolgeler arasinda dagilim farkliligi olmakla birlikte, yalnizca spastisite ile seyreden vakalar cogunlukla OD gecis gosterirken, komplike vakalar genellikle OR gecis gostermektedir (6). Bizim alti HSP olgumuz saf HSP formunda idi. Her kusakta hastalik ortaya cikmisti ancak hic etkilenmis kadin olmamasi nedeniyle mitokondriyal gecis dusunuldu ve genetik incelemeye yonlendirildi. Molekuler biyoloji ve norogenetik alaninda son on yildaki ilerlemeler ile gunumuzde HSP ile ilgili yuze yakin genetik lokus ve gen tanimlanmistir ve calismalar devam etmektedir (6). Bu kadar genis genetik yelpazeye sahip olmasi hastaligin tani konma surecini uzatmakta ve zaman zaman eldeki imkanlar yetersiz kalmaktadir.

Harlequin sendromu, yuzde unilateral anhidrozis ve mat cilt rengine kontralateral hiperhidrozis ve kizarikligin eslik ettigi nadir bir sempatik sinir sistemi hastaligidir. Cevresel ve/veya emosyonel stres ile belirginlesen hastalik, cogunlukla tedavi gerektirmez ve benign bir otonom disfonksiyondur. Travmatik ya da dejeneratif surecler tabloya yol acsa da cogunlukla etyolojik neden bulunamaz. 42 yasindaki kadin olgumuz, isi artisi ve stres aninda, goz bulgularinin ptozis, anormal pupil vb. eslik etmedigi, yuz sag yariminda asiri terleme, kizarma ile basvurmusdu. Travma veya cerrahi girisim oykusu olmayan hastanin beyin, servikal ve torakal goruntulemelerinde etyolojik bir neden saptanmadi. Sikayetleri sosyal iliskilerini etkileyecek duzeye ulasan hasta semptomatik tedavi amaciyla sempatik ganglion blokajina yonlendirildi. Olgumuz nadir olarak karsilasilan idyopatik harlequin sendromuna dikkat cekmek amaciyla sunulmustur

Introduction: The relationships between plasma levels of brain natriuretic peptide (BNP) and severity and location of stroke, prognosis, and infarct volume were investigated in acute ischemic stroke patients who presented within the first 24 hours (h) of stroke. Material and methods: Brain natriuretic peptide levels were tested in 40 patients and 30 healthy controls. Infarct volume was automatically calculated by multi-slice computed tomography. Disease severity was assessed using the National Institutes of Health Stroke Scale (NIHSS) at presentation, 24 h, 72 h and the 28th day. Progression was defined as an increase of more than two points in the NIHSS scores. Results: The mean BNP levels were 284.16 ±382.79 at presentation and 273.78 ±451.91 at 72 h in the patient group, whereas the mean BNP level was 25.29 ±13.47 in controls. There was a statistically significant difference between the two groups (p < 0.001). Differences in BNP levels among patient subgroups according to the TOAST and OCSP classifications were not statistically significant (p = 0.534, p = 0.943, respectively). There was no significant correlation between plasma BNP level and infarct volume or NIHSS scores (p = 0.5, p = 0.07). A positive correlation was found between BNP levels and the length of the hospitalization period (p = 0.03 and r = 0.33). There was no statistically significant relationship between elevated plasma BNP levels and progression of disease (p = 0.08). Conclusions: Plasma BNP levels were increased in the acute phase of stroke; therefore, BNP could be used as a biomarker for morbidity and mortality, even in patients without cardiac failure.

Introduction Hereditary ataxias are a group of heterogeneous diseases in regard to their clinical and genetic characteristics. Ataxia that progresses slowly may be accompanied by pyramidal and extrapyramidal findings, articulation disorders, ophthalmic movement disorders, neuropathic complaints, cognitive and behavioral abnormalies, and epilepsy. Definitive diagnosis in hereditary ataxias is based on molecular assays. History, clinical examination, laboratory and neuroimaging assist diagnosis. In our study, thirty-seven patients of suspected hereditary ataxia were examined with their clinical and genetic aspects, and the results compared with literature. Method Our study included 37 patients in 22 families who presented to our center between 2010-2016, and whose familial history and phenotypic features indicated hereditary ataxia. The patients were studied for clinical findings, family tree, neuroimaging, and laboratory findings. Advanced genetic investigations were performed on peripheral venous blood samples for hereditary ataxia. Results Of the 37 patients included in our study, 21 were females and 16 were males. Genetic analyses resulted in spinocerebellar ataxia (SCA) in four families (10 patients), Friedrich ataxia (FA) in three families (eight patients), and recessive ataxia due to point mutation in one family (two patients). SCA subtyping revealed SCA 1, 2, 6 and 8 in our patients. The remaining 16 patients included in our study could not be solved so far and are under investigation. Conclusion Hereditary ataxias are rare neurodegenerative disorders. Large genetic pool, ethnic and local differences complicate diagnosing even further. Our study contributes to the literature by reflecting phenotypic and genotypic characteristics of hereditary SCA patients in our region and reporting rare hereditary ataxia genotypes.

Objective: We aimed to investigate whether Serum Prolidase Activity (SPA) levels could be used as a potential diagnostic and/or prognostic biomarker in Acute Ischemic Stroke (AIS) patients or not. Materials and Methods: SPA levels were prospectively evaluated in 37 patients aged between 20 and 85 y who were admitted within 24 h of the onset of AIS. The control group included 37 healthy volunteers of similar age without any disease. Results: In AIS patients, mean SPA was significantly higher compared to healthy controls (1331 ± 399 pg/ml vs. 1169 ± 221 pg/ml, respectively; p=0.035). SPA was not correlated with age, gender, hypertension, diabetes, total cholesterol, triglycerides, high-density lipoprotein, low-density lipoprotein, hemoglobin, c-reactive protein, or hemoglobin A1c levels (p>0.05 for all comparisons). However, patients with new diagnostic atrial fibrillation had higher levels of prolidase acitvity than the others (1647 ± 403 pg/ml vs. 1270 ± 384 pg/ml, p=0.032). SPA levels were also uncorrelated with National Institutes of Health Stroke Scale, infarct volume, Trial of Org 10172 and the Oxfordshire Community Stroke Project classifications, and duration of hospitalization (p>0.05, for all comparisons). Conclusions: Increased levels of serum prolidase enzyme activity may be an independent predictor of AIS and may contribute to stroke pathophysiology. However, further studies with larger populations are needed to reveal the role of SPA in AIS.

Amaç: Multipl Sklerozda (MS), alt üriner sistem fonksiyon bozuklukları oldukça sık görülmektedir. Çalışmamızda, MS hastalarında alt üriner sistem disfonksiyon semptomları ile hastalığın şiddeti, süresi, alt tipi ve lezyon lokalizasyonu arasındaki ilişkiyi göstermeyi amaçladık.Materyal ve Metod: Son 3 ay içinde nöroloji polikliniğimize başvuran, MS tanılı 42 hastanın demografik özellikleri, MS alt tipi, hastalık süresi, hastalık şiddeti, nörolojik muayeneleri, genişletilmiş özürlülük durum ölçeği (EDSS) puanları, üriner sisteme ait yakınmaları, semptomların süreleri, üriner sistem muayeneleri, fonksiyon testleri (aşırı aktif mesane semptom skoru (OABSS), uluslararası prostat semptom skoru (IPSS)), ürodinamik değerlendirme sonuçları prospektif olarak kaydedildi. İstatistiksel analiz ANOVA, Mann-Whitney U ve non-parametrik Pearson testleri kullanılarak yapıldı. P0,05). İrritatif semptomlar ile cins ve EDSS arasında istatistiksel olarak anlamlı bir farklılık bulunurken (p0,05). İrritatif ve obstrüktif semptomların varlığı ile lezyon lokalizasyonu arasında da istatistiksel olarak anlamlı bir farklılık saptanmadı (p>0,05).Sonuç: Çalışmamız MS’ te hastalık şiddetinin irritatif üriner semptomlar üzerinde önemli bir belirleyici faktör olduğunu göstermektedir., Objective: Lower urinary tract dysfunction (LUTD) are quite commonly seen in Multiple Sclerosis (MS). In this study we aimed to show the relationship between the LUTD symptoms and disease related parameters including the severity, duration, subtypes and the lesion localization of MS.Material and Method: In a prospective study, we recorded demographic information, MS subtypes, disease duration, disease severity, neurological examinations, scores of Expanded Disability Status Scale (EDSS), lower urinary tract symptoms (LUTS), duration of the symptoms, urinary tract examination, urinary function tests (Overactive Bladder Symptom Score (OABSS), International Prostate Symptom Score (IPSS)), urodynamic evaluation results of 42 MS patients whom admitted to our neurology department during the last 3 months. Statistical analysis was performed by using ANOVA, Mann Whitney-U test and non-parametric Pearson test. p0,05). A statistically significant difference was found between the genders, EDSS and irritative symptoms (p0,05). There was no statistically significant difference between the localization of the lesion and presence of the irritative and obstructive symptoms (p>0,05).Conclusion: Our study indicates that, disease severity is an important determinant factor of irritative urinary symptoms in MS patients.

Aneurysms greater than 2.5 cm in diameter are classified as giant aneurysms and represent 5-8% of all intracranial aneurysms. Giant intracranial aneurysms demonstrates the clinical course with symptoms related to subarachnoid haemorrhage, mass effect, thromboembolism and seizures. Not only because of their large sizes, but also because of their usually wide and calcified aneurysm neck, existing thrombus within and its proximity to cranial nerves; giant aneurysms causes serious surgical difficulties. Giant aneurysms of the anterior intracranial circulation are rare, slowly progressive vascular abnormalities, often presenting with neuro-ophthalmological symptoms before they rupture. Herein, a case of 77-year-old woman with a giant aneurysm originated from intracavernous segment of internal carotid artery detected in magnetic resonance angiography who presented symptoms due to affected multiple cranial nerves related to mass effect.

McArdle Disease (Glycogen Storage Disorder Type V) is one of the most common inherited genetic alterations known to increase the risk of rhabdomyolysis. It is characterized by glycogen phosphorilase deficiency and is generally inherited autosomal recessively. Altough most of the inherited disorders of enzyme defects in glycogen storage present in childhood, McArdle disease has separate adult-onset forms and the age of onset of the symptoms depends on enzyme activity levels. It mainly presents with progressive muscle weakness and cramps after an exercise and becomes symptomatic in the 2nd or 3rd decades of life. Rhabdomyolysis after intense exercise has been reported. Here we report a 32-year-old male who presented to the emergency room with paralysis, severe bodily pain and hematuria after intense exercise. He was diagnosed with acute renal failure due to rhabdomyolysis. Hemodialysis was finally required despite all other approaches. Muscle biopsy revealed intermyofibrillary and subsarcolemmal glycogen deposits, a marker of Glycogen Storage Disorder Type V (McArdle Disease). The renal transplantation neccessity disappeared as the function tests returned to normal values during follow up. To conclude, we would like to emphasize McArdle disease as a rare cause of acute renal failure due to rhabdomyolysis.

Dermoid cysts are rare cystic tumors originating from ectodermic cells within the intracranial region. These cysts emerge during closure of the neural tube during embryological development. The symptoms are generally incidental and nonspecific. If rupture occurs, the cyst contents propagate through the subarachnoid space and the ventricular system. The dramatic appearance of subarachnoid and cisternal fat droplets facilitates the diagnosis of dermoid cyst rupture by computer tomography and magnetic resonance imaging. Herein, we present a case of ruptured dermoid cyst in an elderly patient. The patient presented with minor symptoms such as nonspecific headache and dizziness; dermoid cyst rupture was diagnosed by observing the fat droplets disseminate into the subarachnoid and cisternal spaces that extend from the left cerebellopontine angle, adjacent to the left carotid channel, to the cavernous sinus, using computer tomography and magnetic resonance imaging.

Fat embolism syndrome is a rarely seen complication of skeletal trauma, and it is seen at a rate of 2–5% after fractures of the long bones of the lower extremities. Its classic triad-+consists of hypoxemia, petechial bleedings on the skin and neurological findings. These neurological findings are highly variable and non-specific, and they can present with lethargy, irritability, delirium, stupor, convulsion or coma. In this report, a male case is presented who was diagnosed with cerebral embolism due to acute cognitive disorder after a segmental tibial fracture.

Of ischaemic stroke patients, about 25% rise from the posterior or vertebrobasilar system. The ischaemia of vertebral arteries may emerge for different vascular pathological reasons, at different localisations and with different clinical findings. Despite its low morbidity and mortality risk, early diagnosis and treatment is of importance. Vertebrobasilar ischaemia symptoms can be observed clinically such as vertigo, tinnitus, double vision, headache, hypokinesis and hearing disorders, etc. In this article, a 42-year-old stroke patient case is presented, who applied to the emergency service with vertigo and then, was diagnosed with bilateral vertebral artery stenosis by means of cranial MR angiography.