Moyamoya disease is characterized by a progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Although its etiology is still unknown, recent genome-wide and locus-specific association studies identified RNF213 as an important susceptibility gene of moyamoya disease among East Asian population. A polymorphism in c.14...

A 54-year-old woman presented to our hospital with progressive motor weakness of the right arm. She had a medical history of systemic lupus erythematosus (SLE) and hypothyroidism. Magnetic resonance imaging indicated a watershed infarction of the left hemisphere. Cervical echogram indicated severe stenosis of the internal carotid artery (ICA) without wall thickening. Cerebral angiography indica...

BACKGROUND Moyamoya syndrome associated with protein S deficiency is rarely encountered and is usually reported in paediatric cases with cerebral ischaemia. CASE REPORT A 32-year-old woman had symptoms of sudden-onset severe headache, projectile vomiting, impaired consciousness, and slight neck stiffness. The computed tomography scan of her brain showed primary intraventricular haemorrhage, a...

Terson syndrome was originally used to describe a vitreous hemorrhage arising from aneurysmal subrarachnoid hemorrhage. Terson syndrome can be caused by intracranial hemorrhage, subdural or epidural hematoma and severe brain injury but is extremely rare in intraventricular hemorrhage associated with moyamoya disease. A 41-year-old man presented with left visual disturbance. He had a history of ...

BACKGROUND AND PURPOSE Moyamoya disease has been classically described by the Asian experience, yet clinical aspects of moyamoya phenomena in the United States remain vastly undefined. The multifocal occlusive arterial disorder may be linked with numerous conditions; however, later stages of this syndrome share common vascular pathophysiology. This study is aimed at characterizing inpatient moy...

Neurofibromatosis type 1 is a multisystem genetic disease of autosomal dominant transmission that reveals important cutaneous manifestations such as café-au-lait spots, multiple neurofibromas, and ephelides in skin fold areas, as well as hamartomatous lesions in the eyes, bones, glands, and central nervous system. Moyamoya disease is a rare progressive vaso-occlusive disorder that occurs with i...

Progressive vascular changes are described in an adult with moyamoya syndrome and similar cases are reviewed. Angiographic findings support the concept of a progressive acquired stenosis of the internal carotid artery as the primary abnormality in this syndrome.

A 16-year-old boy, who had sudden left-sided hemiplegia, died two weeks following onset of symptoms. A right carotid angiogram showed stenosis at the termination of the internal carotid artery. The middle cerebral artery had a beaded appearance and some of its branches were occluded. A basal "moyamoya" syndrome and transdural anastomoses were present. At autopsy, multiple intracranial dissectin...

CONTRIBUTION OF AUTHORS Adnan I Qureshi, Waqas I Gilani MD, Sarwat I. Gilani MD, Malik M. Adil MD . Equally assisted in the synthesis and discussion of ideas, and share equal responsibility for the information written in the manuscript above. CONFLICT OF INTEREST No conflict of interests. RUNNING TITLE Cephalometric Features of Moyamoya Disease. BACKGROUND Moyamoya disease is highly preva...