title: [A case of non-atherothrombotic cerebroretinal small vessel disease].
abstract: BACKGROUND
  There is an increasing interest in the relation between retinal artery abnormalities and cerebral small-vessel diseases (SVD), because retinal vessels share common properties with cerebral small vessels. We report a case of juvenile cerebrovascular disease presenting retinal vessel abnormalities, which clinically resembled cerebral autosomal dominant arteriopathy with stroke and ischemic leukoencephalopathy (CADASIL) but in which Notch3 gene mutations were not detected.
  
  
  METHODS
  A 42-year old woman was hospitalized at the department of Neurology in our hospital, complaining of headache and dysarthria. MRI showed bilateral spotted white matter lesions in the paraventricular area and the temporal lobe, and an ovoid lesion in the right corona radiata. Despite steroid pulse therapy, she developed right incomplete hemiparesis and new lesions were detected in the anterior temporal pole and external capsule. Her genetic analysis showed no mutations in the Notch 3 gene. Ophthalmological examination revealed arterial sheathing in the peripapillary region. Fluorescein angiography showed narrowing of the retinal arterioles and distinguished a peripheral vascular network.
  
  
  CONCLUSIONS
  In this case, ophthalmological examination revealed retinal vessel abnormalities in a relatively young woman with no risk factors such as hypertention or artheriosclerosis, presenting recurrent subcortical strokes. This actual case indicates the association between retinal vessel abnormalities and cerebral SVDs.
authors: Department of Ophthalmology, Osaka Red Cross Hospital. naokosp@kuhp.kyoto-u.ac.jp
authors: Naoko
authors: N
authors: Arakawa
authors: Satoshi
authors: S
authors: Kashii
authors: Kazuo
authors: K
authors: Washida
authors: Daita
authors: D
authors: Kaneda
authors: Hirokazu
authors: H
authors: Masai
authors: Kano
authors: K
authors: Hiroi
authors: Hogara
authors: H
authors: Taguchi
authors: Kazuya
authors: K
authors: Fujiwara