Pediatrik Bir Hastada İskemik İnmeye Neden Olan Primer Santral Sinir Sistemi Vasküliti: Nadir Bir Olgu Sunumu

Yıl 2024, Cilt: 7 Sayı: 1, 37 - 42, 31.03.2024

Furkan Demirkapı , Enis Ademoğlu , Serdar Özdemir

https://doi.org/10.54996/anatolianjem.1211087

Öz

Amaç: Çocukluk çağında inme edinsel beyin hasarına yol açabilen nörolojik bulgu ve semptomlarla karakterize nöropatolojik bir sendromdur. Etiyolojide sıklıkla kardiyoembolik, trombofilik, genetik hastalıklar ve nadiren serebral vaskülitler görülür. Serebral vaskülit terimi sistemik hastalıklara sekonder serebral tutulumu ve yalnızca santral sinir sitemini tutan primer vaskülitleri kapsamaktadır. Primer santral sinir sitemi vasküliti nadir görülmekle birlikte, tanısı sekonder nedenler ve sıklıkla karışan moya moya arteriopatisi, geçici serebral vazokonstrüksiyon sendromu, mikroskobik poliarterit gibi tanıların dışlanmasına dayanır.

Olgu: 14 yaşında erkek hasta bayılması ve o sırada başlayan konuşma bozukluğu olması üzerine acil servise getirildi. Fizik muayenede Glasgow koma skalası skoru 15, motor kuvvetinin sağ alt ve üst ekstremitelerde 1/5, sol alt ve üst ekstremitelerde 5/5, fasiyal asimetri ve konuşmada artikülasyon bozukluğu olduğu görüldü. Sistemik muayenesinde travma bulgusu saptanmadı. Difüzyon ağırlıklı manyetik rezonans görüntülemesinde sol bazal ganglionlar, posterior frontal ve presantral giruslarda akut iskemik inme ile uyumlu difüzyon kısıtlılığı olduğu tespit edildi. Acil serviste enoksaparin (6000 ANTI-XA IU/0.6 ml subkutan 2x1) tedavisi başlanan hasta iskemik inme ön tanısı ile hastaneye yatırıldı. Hastanın bilgisayarlı beyin tomografisi, kontrastlı beyin anjiografi ve manyetik rezonans venografisinde patoloji saptanmadı. Ayrıca beyin manyetik rezonans anjiografi ve dijital subtraksiyon anjiyografide patoloji görülmedi. Olası diğer tanılar dışlandıktan sonra primer santral sinir sistemi vasküliti düşünülerek yüksek doz metilprednizolon tedavisi başlandı.

Sonuç: Çocukluk çağında iskemik inme erişkinlere oranla daha az görülse de nörolojik disfonksiyon, tekrarlayan inme riski ve artmış mortalite ile ilişkilidir. Primer santral sinir sistemi vasküliti tanısı dikkatli bir ayırıcı tanı sürecine dayanan nadir bir pediatrik inme nedeni olduğu hatırlanmalıdır.

Anahtar Kelimeler

Pediatrik, inme, vaskülit

Primary Central Nervous System Vasculitis Causing Ischemic Stroke in a Pediatric Patient: A Rare Case Report

Öz

Aim: Stroke in childhood is a neuropathological syndrome characterized by neurological signs and symptoms that can lead to acquired brain damage. In etiology, cardioembolic, thrombophilic, genetic diseases and rarely cerebral vasculitides are seen. The term cerebral vasculitis includes cerebral involvement secondary to systemic diseases and primary vasculitis involving only the central nervous system. Although primary central nervous system vasculitis (PSSV) is rare, its diagnosis is based on exclusion of secondary causes and frequently confused diagnoses such as moyamoya arteriopathy, transient cerebral vasoconstriction syndrome, and microscopic polyarthritis.

Case: A 14-year-old male patient was brought to the emergency department because of fainting and speech disorder that started at that time. Physical examination revealed a Glasgow coma scale score of 15, motor strength 1/5 in the right lower and upper extremities, 5/5 in the left lower and upper extremities, facial asymmetry and speech articulation disorder. No signs of trauma were detected in the systemic examination. Diffusion-weighted MR imaging was found to be compatible with acute ischemic stroke. The patient, who was treated with enoxaparin (6000 ANTI-XA IU/0.6 ml subcutaneous 2x1) in the emergency department, was hospitalized with a preliminary diagnosis of ischemic stroke. No pathology was detected in the patient's computed tomography (CT), contrast-enhanced cerebral CT angiography (CTA), and magnetic resonance (MR) venography. In addition, no pathology was observed in brain MR angiography (MRA) and digital subtraction angiography (DSA). After excluding other possible diagnoses, primary central nervous system vasculitis was considered and high-dose methylprednisolone treatment was started.

Conlusion: Although ischemic stroke in childhood is less common than in adults, it is associated with neurological dysfunction, risk of recurrent stroke and increased mortality. Primary central nervous system vasculitis is a rare cause of pediatric stroke whose diagnosis is based on careful differential diagnosis.

Anahtar Kelimeler

Pediatric, stroke, vasculitis

Kaynakça

• Ferriero DM, Fullerton HJ, Bernard TJ, et.al. American Heart Association Stroke Council and Council on Cardiovascular and Stroke Nursing. Management of Stroke in NeonatesandChildren: A Scientific Statement FromtheAmericanHeartAssociation/AmericanStrokeAssociation. Stroke. 2019 Mar;50(3):e51-e96.
• Rice CM, Scolding NJ. Thediagnosis of primarycentralnervoussystemvasculitis. PractNeurol. 2020 Apr;20(2):109-114. doi: 10.1136/practneurol-2018-002002. Epub 2019 Oct 24. PMID: 31649101.
• Hajj-Ali RA, Calabrese LH. Central nervoussystemvasculitis: advances in diagnosis. CurrOpinRheumatol. 2020 Jan;32(1):41-46.
• Rohmann JL, Huo S, Sperber PS, Piper SK, Rosendaal FR, Heuschmann PU, Endres M, Liman TG, Siegerink B. Coagulation factor XII, XI, and VIII activity levels and secondary events after first ischemic stroke. J Thromb Haemost. 2020 Dec;18(12):3316-3324.
• Cellucci T, Tyrrell PN, Pullenayegum E, et.al. von Willebrand factor antigen--a possible biomarker of disease activity in childhood central nervous system vasculitis? Rheumatology (Oxford). 2012 Oct;51(10):1838-45.
• Kraemer M, Berlit P. Primary central nervous system vasculitis - An update on diagnosis, differential diagnosis and treatment. J NeurolSci. 2021 May 15;424:117422.
• Salvarani C, Brown RD Jr, Christianson T, et.al. An update of the Mayo Clinic cohort of patients with adult primary central nervous system vasculitis: description of 163 patients. Medicine (Baltimore). 2015 May;94(21):e738.