Bilimsel zemin: Türk Nöroloji Board Kurulu 2001 yýlýnda kuruldu ve
çalýþmalarýna baþladý. Bu kurulun önemli komisyonlarýndan olan sýnav
komisyonu Ege Týp Eðitimi Anabilim Dalý ile iþbirliði içinde 03 Ekim 2004
tarihinde 40. Ulusal Nöroloji Kongresi sýrasýnda ilk Türk Nöroloji Derneði
Yeterlilik Yazýlý Sýnavý’ný gerçekleþtirdi. Bu sýnavýn hazýrlýk, uygulama ve
deðerlendirme aþamalarý, bu süreçte olan diðer nöroloji dernek yeterlilik
kurullarýna yol göstermek ve nöroloji topluluðunun bu sürece bakýþ
açýsýný yansýtmasý nedeniyle yayýmlanmak üzere hazýrlandý.

Scientific background: The Turkish Neurological Society Executive
Committee began efforts to establish the Turkish Neurology Proficiency
Board in 2001, and this was achieved in 2004. The Examination
Committee, which is one of the key committees of this board, realized the
first Turkish Neurological Society written examination for proficiency with
the cooperation of Ege Medical Training Department in 2004 during the
40th National Neurology Congress. The preparation, application, and
evaluation stages of this examination have been prepared for publication,
to serve as a guide for the other neurology boards involved in this process,
and to reflect the view of the Turkish Neurological Society.

Bilimsel zemin: Spinoserebellar ataksiler (SCA'lar) otozomal dominant geçiþ gösteren, genetik ve klinik olarak heterojen yapýya sahip bir nörodejeneratif hastalýk grubudur. Belli coðrafi bölgelerde yapýlan toplum çalýþmalarý SCA alt-tiplerinin görülme sýklýðýnýn dünya genelinde 3/100 000 olduðunu gösterir. Temelde denge ve koordinasyon kaybý ile tanýmlanan SCA'lar progresif, geç-baþlangýçlý hastalýklardýr. SCA alttiplerinin klinik bulgularý büyük oranda örtüþtüðü ve her bir SCA alt-tipi içerisinde bile klinik çeþitlilik gözlendiði için, sadece klinik özelliklere dayanarak SCA tanýsý koymak güçtür. Bu baðlamda, SCA'la rýn genetik etyolojilerinin belirlenmesi, bu hastalýk grubunun tanýmlanmasý ve sýnýflandýrýlmasý, hastalara kesin taný konulabilmesi için önem kazanýr. Günümüzde geni ve mutasyonu tanýmlanmýþ olan 12 otozomal dominant serebellar ataksinin moleküler tanýsý yapýlabilmektedir. Yukarýdaki sayýya gün geçtikçe yeni ataksi genlerinin eklenmesi moleküler taný için, klinik özelliklerin, nöropatolojik bulgularýn, aile öyküsünün, hastalýk baþlangýç yaþýnýn ve toplumlara özgü SCA daðýlýmýnýn önemine iþaret eder. Yorum: SCA 'larýn seyrini durduracak tedavi yöntemleri henüz geliþtirilememiþ olsa da, moleküler analiz klinik tanýnýn doðrulanmasý, klinik sürecin kabaca tahmin edilmesi, aile planlamasýnýn yapýlabilmesi için gereklidir. Boðaziçi Üniversitesi'ndeki deneyimimiz, klinisyen-biyolog iþbirliðinin doðru ve kesin SCA tanýsý koymadaki önemini göstermektedir.

Scientific background: Spinocerebellar ataxias (SCAs) are a clinically and genetically heterogeneous group of neurodegenerative disorders that are inherited in an autosomal dominant manner. Population studies in certain geographical areas throughout the world indicate that the prevalence of SCA is 3/100 000. SCAs which are characterized by loss of balance and coordination, are progressive and late-onset disorders. Since the clinical symptoms of SCA subtypes significantly overlap, and since there is a high clinical variation even in each SCA subtype, the diagnosis of SCA patients, based on only clinical features, becomes difficult and complex. In this respect, the identification of the genetic etiology of SCAs is significant for understanding and classifying this group of disorders, and for definite diagnosis of patients. Today, molecular diagnosis of 12 autosomal dominant cerebellar ataxias with defined gene and mutations can be performed. The increase in the number of new ataxia genes suggests the guidance of well-defined clinical signs, neuropathological findings, family history, age of onset and population-specific SCA prevalence in molecular diagnosis. Conclusion: Although there has been no established therapy to prevent the progression of SCA yet, molecular analysis is required for the confirmation of cfinical diagnosis, the rough prediction of cfinical course and the family planning. Boðaziçi University experience reveals the importance of clinician-bench scientist cooperation and an intense dialogue for correct and definite SCA diagnosis.

Bilimsel zemin: Serebral Venöz Tromboz, klinik seyir olarak pek çok nörolojik hastalýðý taklit edebilir. Aðýr bir bilinç etkilenmesi ve ciddi fokal defisitlere yol açabileceði gibi subklinik, yavaþ seyirli, tek baþýna baþ aðrýsý gibi silik nörolojik belirtiler de oluþturabilir. Erken dönemde çekilen beyin tomografisinin genelde normal olmasý ya da erken tomografi bulgularýnýn gözden kaçabilmesi nedeniyle tanýsý zor olabilir. Tedavi verilmediðinde sakat býrakýcý ve mortal seyredebilecek bir hastalýktýr. Amaç: Bu çalýþmada kliniðimizde Serebral Venöz Tromboz tanýsý almýþ olgularýn klinik ve laboratuvar özellikleri ortaya koyulmaktadýr. Mevcut literatür bilgileri ýþýðýnda klinik yaklaþým tartýþýlmaktadýr. Hastalar ve yöntem: Kliniðimizde dört yýllýk süre içerisinde Serebral Venöz Tromboz tanýsý almýþ 18 olgunun klinik ve demografik özellikleri ve radyolojik bulgularý retrospektif olarak incelenmiþtir. Sonuçlar: En sýk karþýlaþýlan semptom baþ aðrýsý, en sýk görülen patolojik bulgu papil ödem olmuþtur. Mortalite oraný %11.11 bulunmuþtur. En sýk etkilenen dural yapýlar, superior sagittal sinüs, transvers ve sigmoid sinüslerdir. Gebelik ve lupus antikoagülan varlýðý en sýk karþýlaþýlan presipitan durumlardýr. Ýzlenimler: Serebral sinüs trombozu çok silik semptom ve bulgularla seyredebilir. Þüphelenildiðinde ilk tercih edilecek tanýsal iþlem beyin Manyetik Rezonans Görüntüleme ve MR Venografi olmalýdýr. Neden olabilecek trombofilik ve sistemik hastalýklar ayrýntýlý olarak araþtýrýlmalýdýr. Gerektiðinde antikoagülan tedavi güvenilir olarak verilebilir.

Cerebral Vein and Sinus Thrombosis: Clinical Evaluation Scientific background: Cerebral venous thrombosis may mimic many other neurological diseases clinically. Severe changes in consciousness and moderate focal neurological deficits could be seen. The syndrome may also be presented with subclinic, slowly progressive and soft neurological symptoms such as headache. The diagnosis is difficult because the initial computerized tomography is usually normal or the signs are not noticeable. It may be mortal or cause morbidity if the treatment is delayed. Objectives: In this study, we defined the clinical features and radiological findings of patients who have taken the diagnosis of cerebral venous sinus thrombosis. In the aspect of the present literature, clinical approach to Cerebral Sinus Thrombosis is discussed. Patients and Method: This is a retrospective study including 18 patients having the diagnosis of Cerebral Vein and Sinus Thrombosis in our clinic. The patient records and neuroradiology archives of the patients are evaluated. Results: The most common symptom was headache and the most common finding was papillaedema. The mortality rate was 11.11%. The most frequently affected sites were superior sagittal, sigmoid and transverse sinuses. Pregnancy andn lupus anticoagulant were the most common precipitating conditions. Conclusion: Cerebral Sinus Thrombosis may present with mild symptoms and findings. The first step diagnostic procedure is Magnetic Resonance imaging and MR Venography. The anticipating thrombophilic and systemic diseases should be investigated. If necessary, anticoagulant therapy is reliable and beneficial.

Bilimsel zemin: Nörobehçet sendromunda serumda ve beyin omurilik sývýsýnda (BOS) interlökin 6 (IL-6) ve tümör nekroz faktör a (TNFa) düzeyleri aktif safhada yüksek bulunmuþtur. IL-6'nýn yükselmesi homosisteini aktive etmekte, homosistein ise TNF-a stimulasyonu yapmaktadýr. Proinflamatuar sitokinlerin dýþýnda kemokinlerin de NBS patogenezinde rol aldýklarý düþünülmektedir. Nitekim CXCL8 ve CXCL10 BOS ve serumda yükseldiði gösterilmiþ, MIP-1a ve RANTES ile ilgili çalýþma dikkati çekmemiþtir. Endotelin 1 (ET-1) ve nitrik oksit (NO) etyopatolojik moleküllerin BH'da aktif safhada yükseldiðini gösteren çalýþmalar olmasýna karþýlýk, NBS'da bu konu ile ilgili araþtýrmaya rastlanmamýþtýr.Amaçlar: Çalýþmamýzda NBS patogenezindeki rollerini araþtýrmak üzere nöroinflamatuar mediatörlerden TNF-a, IL-6, daha önce çalýþýlmamýþ olan MIP-1a, RANTES, homosistein, ET-1 ve NO düzeylerini aktif ve inaktif safhalarda kontrollerle karþýlaþtýrmalý araþtýrmak amaçlandý. Gereç ve Yöntemler: 82 NBS, 196 nörolojik komplikasyonu olmayan BH ve 30 normal kontrol bireyde TNF-a, IL-6, MIP-1 a, RANTES, homosistein, ET-1 ve NO'in stabil metabolitlerinden NO2+NO3 düzeyleri incelendi.Sonuçlar: Aktif safhada inaktif safha ve kontrollere göre TNF-a, IL-6, daha önce çalýþýlmamýþ olan MIP-1a, RANTES gibi kemokinler ve homosistein, ET-1 ve NO düzeylerinin yüksek bulundu. Ýzlenimler: Bu immünoinflamatuar moleküllerin NBS patogenezinde karþýlýklý etkileþerek rol aldýklarýný ve bu noktadan hareketle tedavide hedefe spesifik immünoterapötik yöntemlerin yararlý olacaðýný düþündürmektedir.

Neuro-Behçet's Syndrome and Neuroinflammatory Molecules Scientific background: In Neuro-Behçet's Syndrome (NBS) tumor necrosis factor-a (TNF-a) and interleukin 6 (IL-6) levels are found to be high in serum and cerebrospinal fluid (CSF) at active stage. An increase in IL-6 activates homocysteine, which in turn stimulates TNF-a. It is suggested that chemokines alsa play a part in N85 pathogenesis in addition to proinflammatory cytokines. Furthermore, it has been demonstrated that CXCL8 and CXCL10 increase in serum and CSF while any study on MIP-1· and RANTES could not be noted. Although there are some studies that show an increase in etiopathological molecules of Endothelin 1 (ET-1) and nitric oxide (NO) at the active stage in Behçet's Disease (BD), a corresponding study could not be found with respect to NBS. Objectives: The aim was to study the levels of TNF-a and IL-6 of neuroinflammatory mediators as well as those of MIP-1a, RANTES, which had never been studied before, and homocysteine, ET-1 and NO levels at active and inactive stages in comparison to control subjects so as to investigate the roles of the foregoing in the pathogenesis of Neuro-Behçet's syndrome. Material and methods: Levels of TNF-a, IL-6, MIP- 1a, RANTES, homocysteine, ET- 7 and NO2+NO3, i.e. a stable metabolite of NO, were investigated in 82 patients with NBS, in 196 BD patients without any neurological complications, and in 30 normal control subjects. Results: At the active stage, the TNF-a and IL-6 levels and the levels of MIP-1a, RANTES, which had not been studied before, and those of homocysteine, ET-1 and NO were found to be high when compared to inactive stage and the control subjects. Conclusions: it is suggested that the said immunoinflammatory molecules interact with each other and are involved in NBS pathogenesis; and therefore, target-specific immunotherapeutic methods will prove helpful in treatment.

Bilimsel zemin: Spinal kord yaralanmalarýnda (SKY) klinik nörolojik deðerlendirme, hastalarýn mevcut durumu ve olasý prognozu hakkýnda ön bilgi verebilmekte, fakat çoðunlukla yetersiz kalmaktadýr. Lezyonun derecesini belirlemek ve klinik deðerlendirmeler sýrasýnda gözden kaçabilecek patolojik deðiþiklikleri saptamak, hastalýðýn prognozu hakkýnda daha fazla bilgi edinebilmek amacýyla, elektrofizyolojik incelemeler ile ortaya çýkarýlacak bulgular oldukça faydalý olabilir.Amaç: Bu çalýþmada SKY'li hastalarda klinik ve elektrofizyolojik bulgularýn karþýlaþtýrýlmasý amaçlanmaktadýr.Gereç ve Yöntemler: SKY'li 31 hastanýn motor ve duysal periferik sinir ileti incelemeleri, geç yanýtlarý, paraspinal, anal sfinkter ve ekstremite iðne elektomiyografisi, posterior tibial sinir skalp somatosensoriyel uyandýrýlmýþ potansiyel (SUP) yanýtlarý, sempatik cilt yanýtlarý (SCY) incelenmiþ ve sonuçlar klinik bulgular ile karþýlaþtýrýlmýþtýr.Bulgular: Alt ekstremite sinir ileti incelemelerinde saptanan patolojiler ile spazm sýklýðý ve hastalýk süresi arasýnda iliþki saptanmadý. H refleksi (H-R) alýnabilen hastalarda spastisite derecesi ve spazm sýklýðý anlamlý olarak fazla bulundu (p<0.05). Spastisite derecesi arttýkça H-R/M yanýtý oranýnda artýþ gözlendi. Hastalarýn %25,B'inde alt ekstremitelerde, %48,4'ünde üst ekstremitelerde tuzak nöropatisi saptandý. SA derecesi ile derin tendon refleksleri ve tonus artýþý arasýnda negatif korelasyon saptandý (p<0.001). SA saptanan hastalarda ortalama hastalýk süresi anlamlý olarak kýsa idi (p<0.05). Anal sfinkterde SA bulgularýnýn þiddeti azaldýkça spastisitenin (p=0.01) ve spazm sýklýðýnýn arttýðý (p= 0.04) saptandý. Paraspinal kaslarda SA bulgularý ne kadar geniþ alanda ise spastisite derecesi (p=0.001) ve spazm sýklýðý o oranda az idi (p= 0.01 ). Sempatik cilt yanýtlarýnýn kaybolduðu seviye, duyu seviyesinden ortalama 2 dermatom farklý bulundu. Klinik olarak tam spinal kord tutulumu olan bir hasta, elektrofizyolojik destekli kýsmi yaralanma grubuna dahil edildi.Sonuç: SKY'li hastalarda elektrofizyolojik incelemelerin hastalýðýn klinik ve prognozu hakkýnda anlamlý bulgular verebileceði ve rehabilitasyonu yönlendirebileceði kanýsýna varýlmýþt ýr.

Scientific background: Neurological examination can provide preliminary informations about the patients and prognosis but it is usually unsatisfactory. Neurophysiological investigations can provide more detailed information about the prognosis of the disease, such as; determining the severity of the lesion and detecting pathological changes that may be underestimated during the clinical evaluations. Objective: The aim of this study is to compare the clinical and the electrophysiological findings in patients with spinal cord injury. Material and methods: In 31 patients with spinal cord injury, we evaluated motor and peripheral nerve conduction velocities, paraspinal, anal sphincter, and extremity electromyographies (EMG); median and posterior tibial nerve somatosensorial responses (SEP); symphathetic clinical responses. Clinical and electrophysiological results were compared with statistical analysis. Results: There was no correlation between the disease duration and spasm frequency in lower extremities, but a significant correlation was found between the spasm frequency and spasticity severity in cases whom H reflexes could be obtained (p<0.05). H-RIM responses were increasing with the severity of spasticity. The electrophysiological findings revealed lower (25.8%) and upper (48.4%) extremity entrapment neuropathies in cases. There was a negative correlation between the deep tendon reflexes, increased tonus and SA severity (p<0.001). The patients with SA had significantly short disease duration (p<0.05). Severity of spasticity (p=0.001) and the frequency of spasms (p=0.04) were increasing with the decreasing severity of SA in anal sphincter. Besides this, we observed that, the more widely dispersed denervation in the paraspinal muscles, the less the level of lower extremity spasticity (p=0.001) and the frequency of the spasms (p=0.07). Symphathetic skin responses were disappeared with an average of two dermatomes below the sensorial level. Only one patient had an electrophysiological partial denervation though his neurological examination showed total spinal involvement.Conclusion: Electrophysiological investigations can yield significant information about the clinical findings and the prognosis of the disease.

Bilimsel zemin: Eriþkin yaþta baþlayan nöronal seroid lipofuksinoz (Kufs Hastalýðý) nadir görülen tanýmlanmasý güç bir metabolik nörodejeneratif hastalýktýr.Amaç: Psikiyatrik semptomlar ve ilerleyici biliþsel bozulmasý olan, miyelin kýlýfýnda granüler osmiofilik depolanmalar ve Schwann hücre sitoplazmasýnda parmak izi profili gösterilen bir adult nöronal seroid lipofuksinoz olgusu tanýmlanmaktadýr.Tartýþma: Erken baþlangýçlý davranýþ deðiþikliði ve psikiyatrik semptomlarýn da bulunduðu demans olgularýnda nöbet, motor bulgularýn ve aile anamnezinin varlýðýnda ayýrýcý tanýda nörodejeneratif bir metabolik hastalýk olan Kufs hastalýðý düþünülmelidir.

Scientific background: The adult onset form of neuronal ceroid lipofuscinosis (Kufs Disease) is a rare and poorly characterized entity of metabolic neurodegenerative disease.Objectives: We report a patient with psychiatric symptoms and progressive cognitive decline. We were able to show granular osmiophilic deposits in myelin sheath and fingerprint profile in Schwann celi cytoplasm indicating the presence of adult neuronal ceroid lipofuscinosis.Discussion: Kufs disease should be considered in the differential diagnosis of early onset, progressive cognitive decline with psychiatric symptoms, seizures and motor disturbances especially in patients with positive family history.

Bilimsel zemin: Reversibl posterior lökoensefalopati sendromu (PLES),
baþ aðrýsý bulantý, kusma, bilinç deðiþikliði, görme alaný defekti ve
konvülsiyon ile karakterize kliniko-radyolojik bir sendromdur. Genellikle
malign hipertansiyon, gebelik toksemisi ve immünsüpresif tedaviler ile
iliþkilidir.
Amaç: Baþ aðrýsý, hipertansif atak, kuadranopi kliniði ile baþvuran
hastada, kranial görüntüleme yapmanýn önemi, PLES ayýrýcý tanýsýnda
diffüzyon MR kullanýmýnýn yararý ve nadir görülen bir PLES vakasý
sunulacaktýr.
Bulgular: Hipertansiyon ve böbrek yetmezliði saptanan 35 yaþýndaki
erkek hastada, kranial MR, diffüzyon MR tetkikleri yapýlmýþ ve yaygýn
arka sistem lezyonlarý saptanmýþtýr.
izlenimler: PLES tanýsý koyulan hastanýn kan basýncý kontrolünü takiben
klinik ve radyolojik bulgularý kýsa sürede düzelmiþtir.

Reversible Posterior Leucoencephalopathy (PLES) Syndrome
Scientific background: The posterior leucoencephalopathy (PLES) is a
clinico-radiological syndrome characterised by headache, nausea,
vomiting, disturbances in cognition, depressed level of consciousness,
visual abnormalities and convulsions. It is commonly associated with
malignant hypertension, toxemia of pregnancy or use of
immunosuppressive agents.
O b j e c t i v e: We, here, report a case of reversible posterior
leucoencephalopathy syndrome in a patient submitted with headache,
hypertension, quadranopia and the importance of performing cranial
imaging and the benefit of diffusion MRI in the differential diagnosis of
P L E S.
F i n d i n g s: MRI and diffusion MRI studies have been performed in a 35
year old male patient with hypertension and renal failure, and diffuse
posterior system lesions have been found.
C o n c l u s i o n: The patient’s clinical and radiologic recovery occurs
following control of blood pressure.