Microangiopathy with encephalopathy, hearing loss and retinal arteriolar occlusions: two new cases

J Neurol Sci. 1988 Sep;86(2-3):239-50. doi: 10.1016/0022-510x(88)90102-5.


Two young women developed encephalopathy, hearing loss and retinal arteriolar occlusions. Their behaviour became immature and cognitive functions were severely impaired. One of the patients underwent brain biopsy, which showed several microinfarcts in both white and grey matter and microangiopathic changes, with thickened arteriolar segments staining intensely for laminin and fibronectin. These findings support the concept of a new type of microangiopathy involving the brain, inner ear and retina.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Arterial Occlusive Diseases / complications*
  • Arterial Occlusive Diseases / diagnosis
  • Arterial Occlusive Diseases / diagnostic imaging
  • Brain / pathology
  • Cerebrovascular Circulation
  • Cerebrovascular Disorders / complications*
  • Cerebrovascular Disorders / diagnosis
  • Cerebrovascular Disorders / pathology
  • Female
  • Hearing Loss / complications*
  • Hearing Loss / diagnosis
  • Humans
  • Radiography
  • Retina / blood supply*