Lethal bilateral cerebral infarction caused by Moyamoya disease

K. Numata, M. Suzuki, R. Mashiko, Y. Tokuda
2016 QJM: Quarterly journal of medicine  
A 35-year-old woman with schizophrenia was brought to the emergency department after being found unconscious on the floor by her mother in winter season. The patient had been in her usual state of health until 3 days prior to admission. On examination she was comatose and had severe hypothermia (29.0 C), hypotension, and bradycardia. Diffusion-weighted magnetic resonance (MR) imaging revealed symmetrical hyper-intensity lesions in the territories of bilateral internal carotid arteries (ICA) (
more » ... gure 1A ). MR angiography showed the diminished perfusion of bilateral anterior cerebral arteries and middle cerebral arteries ( Figure 1B ). Based on the blood supply noted only from the external carotid artery, she was diagnosed with stage VI Moyamoya disease, which is characterized by progression of cerebral infarction via bilateral stenoocclusive changes at and around the ICA bifurcation along with abnormal netlike-vessels in the basal regions ( Figure 1A and B) . 1,2 The patient died several days after admission. References 1. Houkin K, Aoki T, Takahashi A, Abe H. Diagnosis of Moyamoya disease with magnetic resonance angiography. Stroke 1994; 25:2159-64. 2. Yamada I, Suzuki S, Matsushima Y. Moyamoya disease: diagnostic accuracy of MRI. Neuroradiology 1995; 37:356-61. Figure 1. (A) Diffusion-weighted MR imaging of the brain, showing symmetrical hyper-intensity lesions in the circulatory territories of bilateral internal carotid arteries. (B) MR angiography of the brain, showing the diminished perfusion of bilateral anterior cerebral arteries and middle cerebral arteries in coronal.
doi:10.1093/qjmed/hcw037 pmid:26980787 fatcat:ov276ogczrh7va7fcvod2bkajy