Reversible serebral vazokonstriksiyon sendromu

Özet

Reversible serebral vazokonstriksiyon sendromu (RCVS), çoğunlukla ani ve şiddetli baş ağrısı ataklarıyla başvuran hastada farklı serebral arter segmentlerinin geçici daralmasıyla seyreder. BTA lümenlerde çok odaklı daralma-genişleme örüntüsünü gösterebilir; erken inceleme normal olabileceğinden tek bir negatif BTA klinik kuşkuyu ortadan kaldırmaz. Kontrastsız BT, RCVS'ye eşlik edebilen subaraknoid veya intrakraniyal kanamayı gösterebilir. Vaskülit gibi benzer damar hastalıklarıyla karışması tanısal gecikmeye ve komplikasyonların gözden kaçmasına neden olabilir.

Faz ve pencere

Kontrastsız

Kontrastsız BT, RCVS'yi doğrudan göstermez; sulkuslarda subaraknoid kan, parankimal hematom veya subdural kanama gibi eşlik eden kanama bulgularını ortaya koyabilir.

BTA tanısal

Birden fazla intrakraniyal arterde ardışık segmental daralmalar ve aralarda daha geniş kalibreli bölümler görülebilir; bu örüntü tanıyı destekler, ancak tanı klinik bulgular ve zaman içindeki damar görüntülemesiyle birlikte konur. Erken evrede lümenler normal görünebilir ve distal dallardaki değişiklikler seçilemeyebilir.

BT bulguları

• Multifokal segmental vazokonstriksiyon — anterior ve posterior dolaşım arterlerinde birbirinden ayrı daralmış lümen bölümleri saptanır.
• İnci dizisi görünümü — damar boyunca dar segmentlerle normal veya görece geniş kalibreli parçalar art arda sıralanır.
• Damar yatağı dağılımı — orta serebral, anterior serebral ve posterior serebral arter dalları birlikte etkilenebilir.
• RCVS’de orta ve büyük çaplı arterlerde segmental daralma ve genişleme alanları görülebilir; bu görünüm çok odaklı bir süreci destekler.
• Erken BTA'nın normal olabilmesi — hastalığın dinamik seyri nedeniyle ilk lümen incelemesinde karakteristik daralma henüz belirginleşmemiş olabilir.
• Konveksite subaraknoid kanaması — kontrastsız görüntüde bazal sisternalardan çok yüksek konveksite sulkuslarında ince kan yoğunluğu görülebilir.
• Eşlik eden kanama, iskemi veya ödem — kortikal subaraknoid ve intraparenkimal kanama görülebilir; bazı hastalarda infarkt veya PRES paterni eşlik eder. İnfarkt DWI ile, ödem ise FLAIR sekanslarında değerlendirilebilir.
• RCVS, tekrarlayan gök gürültüsü baş ağrısı ve orta-büyük serebral arterlerde segmental daralma-genişleme ile karakterizedir; vazokonstriksiyon genellikle 3 ay içinde geriler.

Normalde

RCVS'de intrakraniyal orta-büyük arterlerde segmental daralma ve genişleme alanları görülebilir. RCVS kuşkusunda her arterin kalibresini seri kesitlerde ve karşı hemisferdeki eş düzey dallarla karşılaştır; tek bir bifurkasyon varyantı yerine farklı arter yataklarına dağılmış segmental değişiklik ara.

Ayırıcı tanı

Primer santral sinir sistemi anjiyiti

damar lümenindeki düzensizlik RCVS'yi taklit edebilir; klinik seyir, beyin parankim lezyonlarının dağılımı ve damar duvarı görüntülemesi ayrımda önemlidir.

İntrakraniyal aterosklerotik hastalık

RCVS’den ayrımda damar duvarı görüntülemesindeki duvar tutulumunun dağılımı ve kontrastlanma örüntüsü yardımcı olabilir.

Anevrizmal subaraknoid kanama sonrası vazospazm

kanama bazal sisternalarda yoğunlaşabilir ve daralma kanamanın komşu arter segmentlerinde öne çıkar.

Fibromüsküler displazi

başlıca servikal arterlerin tipik segmentlerinde boncuk dizisi görünümü oluşturabilir; intrakraniyal yaygın geçici vazokonstriksiyonla aynı dağılımda değildir.

Moyamoya hastalığı

ICA terminuslarında ilerleyici daralma ve bazal kollateral damar ağı karakteristiktir.

Serebral venöz tromboz

ani şiddetli baş ağrısı ve konveksite subaraknoid kanamasıyla başvurabilir; klinik kuşkuya göre venöz görüntüleme gerekebilir.

Tuzaklar

• Başlangıç BTA'sının normal olması erken RCVS'yi dışlamaz; damar değişikliklerinin klinik başlangıca göre evrim gösterebileceğini raporda belirt.
• Görüntüleme artefaktları RCVS'deki anjiyografik bulguları örtebilir; bu nedenle damar görüntülerini dikkatle değerlendirmek gerekir.
• Konveksite sulkuslarındaki ince kanı bazal sisternal anevrizmal kanamayla eşitleme; kan dağılımını ve BTA'da anevrizma olup olmadığını birlikte değerlendir.
• Segmental daralma tek başına RCVS'ye özgü değildir; sabit plak, damar duvarı tutulumu ve klinik zaman içindeki gerileme bilgisi ayırıcı tanıda gerekir.
• Erken BTA/MRA normal olabilir; klinik kuşku sürerse seri damar görüntülemesi düşünülebilir, çünkü vazokonstriksiyon erken incelemede gözden kaçabilir ve zaman içinde belirginleşebilir.

Kendini dene

1. Gök gürültüsü baş ağrısı ile başvuran bir hastada orta-büyük serebral arterlerde segmental daralma ve genişleme görülüyor. En olası sendrom hangisidir?

   • RCVS
   • Primer SSS anjiyiti
   • İntrakraniyal ateroskleroz
   • Moyamoya hastalığı
   Cevabı göster

   RCVS. RCVS, gök gürültüsü baş ağrısı ve orta-büyük serebral arterlerde segmental daralma-genişleme ile karakterizedir. Primer santral sinir sistemi anjiyiti ayırıcı tanıdadır; bu hastalıkta beyin MR’ında farklı yaşlarda çoklu infarktlar görülebilir.

2. İlk hafta yapılan DSA normal olabilir; tekrarlanan incelemede bazı daralmalar gerilerken yeni daralma alanları görülebilir. Bu zaman içindeki damar değişkenliğini hangi sendrom açıklar?

   • Reversible serebral vazokonstriksiyon sendromu (RCVS)
   • Diseksiyon
   • Sakküler anevrizma
   • Arteriyel hipoplazi
   Cevabı göster

   Reversible serebral vazokonstriksiyon sendromu (RCVS). RCVS’de ilk hafta DSA normal olabilir; tekrarlanan DSA’da bazı daralmalar gerilerken yeni daralma alanları ortaya çıkabilir.

Kaynaklar

Bu sayfadaki 41 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla otomatik olarak karşılaştırıldı; 15 cümle bu karşılaştırmada düzeltildi (2026-10-09).

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