title: Diffuse cerebral microhemorrhages in a patient with adult-onset Pompe's disease: a case report.
abstract: BACKGROUND
  Pompe's disease is a glycogen storage disease that manifests as progressive neuropathy, and myopathy. There are a few reports of vasculopathy in this disease, thought to be from small- and medium-vessel arteriopathy. We present a case of late-onset Pompe's disease with microhemorrhages and review of the pertinent literature.
  
  
  METHODS
  We describe a case of microhemorrhages in a patient with known late-onset Pompe's disease.
  
  
  RESULTS
  Our patient was noted to have numerous microhemorrhages concentrated in the posterior circulation distribution in what can best be described as central microhemorrhages, distinct from the pattern seen in amyloid angiopathy. Previous autopsy studies have found vacuoles in the vessel wall, resulting in small aneurysms as a part of the Pompe syndrome.
  
  
  CONCLUSIONS
  There is an accumulating body of evidence that suggests cerebral vasculopathy as one of the primary manifestations of adult-onset Pompe's disease. This is manifested as dolichoectasia of basilar artery, aneurysms, and microhemorrhages that are central in distribution. The primary pathology is thought to be glycogen deposition in small- and medium-sized intracranial vessels. Controlling blood pressure aggressively and screening intracranial vascular imaging are recommended. Further definition of the syndrome is continuing from phenotypic and genotypic dimensions.
authors.affiliation: University of Minnesota Medical Center, Minneapolis, MN, USA.
authors.forename: Divyajot
authors.initials: D
authors.lastname: Sandhu
authors.affiliation: University of Minnesota Medical Center, Minneapolis, MN, USA.
authors.forename: Adam
authors.initials: A
authors.lastname: Rizvi
authors.affiliation: University of Minnesota Medical Center, Minneapolis, MN, USA.
authors.forename: Jae
authors.initials: J
authors.lastname: Kim
authors.affiliation: University of Minnesota Medical Center, Minneapolis, MN, USA.
authors.forename: Rwoof
authors.initials: R
authors.lastname: Reshi