CONTEXT Neurofibromatosis type 1 (NF-1) is the most prevalent autosomal dominant genetic disorder among humans. Moyamoya disease is a cerebral vasculopathy that is only rarely observed in association with NF-1, particularly in the pediatric age range. The present study reports an occurrence of this association in an infant. CASE REPORT An eight-month-old female presented convulsive seizures w...

BACKGROUND AND PURPOSE Although studies have evaluated the differential imaging of moyamoya disease and atherosclerosis, none have investigated the added value of vessel wall magnetic resonance imaging (MRI). This study evaluates the added diagnostic value of vessel wall MRI in differentiating moyamoya disease, atherosclerotic-moyamoya syndrome (A-MMS), and vasculitic-MMS (V-MMS) with a multico...

We report the case of a 17-year-old Korean girl with systemic lupus erythematosus (SLE) who presented with sudden weakness of the right-sided extremities and dysarthria. Oral prednisolone was being taken to control SLE. Results of clinical and laboratory examinations did not show any evidence of antiphospholipid syndrome or thromboembolic disease nor SLE activity. Cerebral angiography showed st...

BACKGROUND Parainfectious vascular events are a known complication of bacterial meningitis, typically occurring within two weeks of disease onset. Delayed vascular complications are rare. We present a case of progressive vasculopathy following bacterial meningitis. CASE DESCRIPTION A 20 year old woman developed progressive vasculopathy after successful treatment of pneumococcal meningitis. Wi...

Background: Parainfectious vascular events are a known complication of bacterial meningitis, typically occurring within two weeks of disease onset. Delayed vascular complications are rare. We present a case of progressive vasculopathy following bacterial meningitis. Case description: A 20 year old woman developed progressive vasculopathy after successful treatment of pneumococcal meningitis. Wi...

An 11-year-old boy with hemoglobin sickle disease (HbSD), bilateral stenosis of the intracranial carotid arteries, and moyamoya syndrome had recurrent ischemic strokes with aphasia and right hemiparesis. His parents (Jehovah's Witnesses) refused blood transfusions. After bilateral extracranial-intracranial (EC-IC) bypass surgery, hydroxyurea treatment increased hemoglobin F (HbF) levels to more...

A 7-year-old white girl presented with left hemiparesis and ischemic stroke secondary to moyamoya syndrome, a progressive cerebrovascular occlusive disorder of uncertain but likely multifactorial etiology. Past medical history revealed hearing loss and developmental delay/ intellectual disability. Routine karyotype demonstrated extra chromosomal material on 6p. Single nucleotide polymorphism mi...

BACKGROUND AND PURPOSE Intracranial aneurysms and aneurysmal subarachnoid hemorrhage may occur more frequently in sickle-cell disease (SCD), and this could be related to the sickle genotype and moyamoya syndrome seen in SCD. METHODS Records from a total of 1002 patients with SCD attending 2 specialized adult hematologic services were retrospectively reviewed. We analyzed data of a cohort of 7...