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It is one of the two types of hereditary cerebral amyloid angiopathy alongside [[familial British dementia]].<ref>{{Cite web|title=Hereditary cerebral amyloid angiopathy: MedlinePlus Genetics|url=https://medlineplus.gov/genetics/condition/hereditary-cerebral-amyloid-angiopathy/|access-date=2021-09-07|website=medlineplus.gov|language=en}}</ref>
The disorder is [[histopathology|histopathologically]] characterized by serve [[cerebral amyloid angiopathy]] with [[neurofibrillary tangle]] deposition in the [[limbic system]] and the [[neocortex]].<ref>{{Cite book|last1=MD|first1=Thomas P. Naidich|url=https://books.google.com/books?id=TUfZSKldJRwC&q=Dementia,+familial+Danish&pg=PA947-IA15|title=Imaging of the Brain: Expert Radiology Series|last2=MD|first2=Mauricio Castillo|last3=MD|first3=Soonmee Cha|last4=MD|first4=James G. Smirniotopoulos|date=2012-10-31|publisher=Elsevier Health Sciences|isbn=978-1-4160-5009-4|language=en}}</ref> It is also [[Neuropathology|neuropathologically]] characterized by diffuse atrophy of [[cerebellum]], [[cranial nerves]], and the [[spinal cord]].<ref>{{Cite book|last1=Pantoni|first1=Leonardo|url=https://books.google.com/books?id=z6lkAwAAQBAJ&q=Dementia,+familial+Danish&pg=PA87|title=Cerebral Small Vessel Disease|last2=Gorelick|first2=Philip B.|date=May 2014|publisher=Cambridge University Press|isbn=978-1-107-03166-1|pages=87|language=en}}</ref>
== Symptoms and signs==
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