Case Report

Intracerebral hemorrhage in Adult with Unilateral Moyamoya disease

Poppy K. Sasmita
FK Unika Atma Jaya/RS Mitra Keluarga Kelapa Gading

Abstrak
Latar Belakang: Moyamoya disease adalah suatu vaskulopati serebral yang jarang dan
berhubungan dengan adanya stenosis-oklusi progresif bilateral dari arteri karotis interna terminal
dengan terbentuknya kolateral pembuluh darah.
Deskripsi kasus: seorang wanita berusia 26 tahun datang ke rumah sakit dengan keluhan nyeri
kepala hebat, muntah dan penurunan kesadaran. Pada pemeriksaan fisik didapatkan kesadaran
somnolen dan kelemahan pada anggota gerak kiri dengan kekuatan 4. Pada pemeriksaan CT
Scan kepala didapatkan adanya perdarahan di nucleus caudatus dan crus anterior capsula interna
kanan disertai perdarahan intraventrikuler (seluruh sistem ventrikel) dengan midline shift
minimal ke kiri. Pada pemeriksaan Digital Substraction Angiography (DSA) didapatkan stenosis
pada arteri karotis interna kanan disertai gambaran puff of smoke. Selama perawatan dilakukan
pemeriksaan CT Angiography kepala untuk mengevaluasi pembuluh darah intrakranial dan
ekstrakranial. Pasien dirawat selama 14 hari dan pulang dalam keadaan compos mentis tanpa
hemiparese. Satu bulan setelah dirawat dilakukan operasi cerebral bypass procedure. Enam
bulan pasca operasi dilakukan pemeriksaan CT Angiography kepala dengan hasil parenkim otak
dan perfusi otak normal.
Kesimpulan: walaupun jarang, perdarahan otak dapat disebabkan karena moyamoya disease
unilateral.
Kata kunci: perdarahan otak, Moyamoya disease, DSA, CT Angiography

Abstract
Background: Moyamoya disease is an uncommon cerebral vasculopathy associated with bilateral
progressive occlusion of the terminal internal carotid arteries with formation of collateral blood
vessels.
Case description: A 26-year-old woman came to the hospital with severe headache, vomiting and
loss of consciousness. On physical examination patient looked somnolence and had weakness in
the left limbs with strength of 4. Head Computed Tomography (CT) scan showed bleeding at
right nucleus caudatus, right anterior crus of internal capsule with intraventricular hemorrhage
(entire ventricular system) with minimal midline shift to the left. On examination Subtraction
Digital Angiography (DSA) showed stenosis of the right internal carotid artery with "puff of
smoke" appearance. During hospitalization, head CT Angiography was performed to evaluate
the intracranial and extracranial blood vessels. Patients hospitalized for 14 days and went home
with compos mentis and without weakness in the left limbs. Patient had cerebral bypass surgery
procedure one month later. Six months after surgery, the patient was evaluated with head CT
Angiography and showed normal brain parenchyma and brain perfusion.
Conclusion: Although a rare case, intracerebral hemorrhage can caused by unilateral Moyamoya
disease.
Keywords: Intracerebral hemorrhage, Moyamoya disease, DSA, CT Angiography