Moyamoya disease (multiple progressive intracranial arterial occlusion)

General: Almost exclusively seen in Japanese infants and children; cerebrovascular disorder tha 252d34c t results in occlusion of the large vessels at the base of the brain; slight female prevalence; collateral vascular networks (secondary to bilateral carotid occlusive disease) resembling puffs of smoke (Moyamoya in Japanese).

Ocular: Hemianopsia; nystagmus; papilledema; central retinal vein occlusion; visual field defects; amaurosis fugax; diplopia; optic pallor; ischemic chiasmal syndrome; bilateral renal artery stenosis.

Clinical: Loss of consciousness; seizures; hemiplegia; hemiparesis; intracranial hemorrhage; mental retardation; speech disturbances; unsteady gait; headache; psychiatric manifestations; focal epileptic attacks; chronic cerebrovascular disorder; intracerebral hemorrhage; pituitary adenoma (association).

Ahmadi J, et al. Ischemic chiasmal syndrome and hypopituitarism associated with progressive cerebrovascular occlusive diseases. Am J Neuroradiol 1984; 5:367.

Arita K, et al. Moyamoya disease associated with pituitary adenoma-report of two cases. Neurol Med Chir 1992; 32:753-757.

Nakano T, et al. Moyamoya disease associated with bilateral renal artery stenosis. Acta Paediatr Jpn 35:354-357, 1993.

Noda S, et al. Ocular symptoms of Moyamoya disease. Am J Ophthalmol 1987; 103:812-816.

Slamovits TL, et al. Moyamoya disease with central retinal vein occlusion. J Clin Neuro-Ophthalmol 1981; 1: 123-l27.

Ueki K, et al. Moyamoya disease: the disorder and surgical treatment. Mayo Clin Proc 1994; 69:749-757.

Yamada H. Moyamoya disease in monocular twins: case report. J Neurosurg 1980; 53: 109-l12.