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Hereditary cerebral hemorrhage with amyloidosis


Other Names for this Disease
  • Hereditary cerebral hemorrhage with amyloidosis, Dutch type (subtype)
  • Hereditary cerebral hemorrhage with amyloidosis, Icelandic type (subtype)
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Where can I find information about the symptoms of hereditary cerebral hemorrhage with amyloidosis - Dutch type?

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What is cerebral amyloid angiopathy (CAA)?

Cerebral amyloid angiopathy (CAA) is a neurological condition in which amyloid protein is deposited onto the walls of the arteries of the brain (and less frequently, veins).  Although CAA often does not cause symptoms, it may cause bleeding into the brain (hemorrhagic stroke), dementia, or neurologic episodes in some patients.  The majority of CAA cases occur in individuals who do not have a family history. However, two familial forms of CAA have been identified. [1][2]
Last updated: 2/5/2009

What is hereditary cerebral hemorrhage with amyloidosis - Dutch type?

Hereditary cerebral hemorrhage with amyloidosis - Dutch type is an autosomal dominant neurological disorder characterized by recurrent hemorrhagic strokes and dementia. The condition is caused by the deposition of an amyloid-beta precursor protein in the central blood vessels and plaques in the brain.[3] This condition was named for the families in two coastal villages in the Netherlands who were first documented with the condition.[4] 
Last updated: 2/5/2009

What symptoms may be associated with hereditary cerebral hemorrhage with amyloidosis - Dutch type?

Approximately 87% of individuals with hereditary cerebral hemorrhage with amyloidosis - Dutch type have intracranial hemorrhage (bleeding in the brain) and 13% have infarcts (stroke).[2] The first stroke usually occurs between the ages of 45 and 65 years, and is not caused by hypertension or hemorrhagic diathesis (bleeding tendency).[5] Nausea, vomiting, progressive headache, focal neurological signs (double or decreased vision, speech difficulties, confusion, delirium, weakness or paralysis, sensation changes or loss of sensation, progressive intellectual deterioration and memory disturbance) and impairment of consciousness are the most frequent signs and symptoms.[5][1][6] Psychiatric abnormalities, including dementia are also common, with some patients developing dementia without intracranial hemorrhage.[2][6]


Last updated: 2/5/2009

What causes hereditary cerebral hemorrhage with amyloidosis - Dutch type?

The clinical symptoms of hereditary cerebral hemorrhage with amyloidosis - Dutch type are caused by the build-up of a protein called amyloid within the arterial walls of the brain.[1][6] This protein build-up causes bleeding into the brain. The symptoms occur because bleeding in the brain harms brain tissue.[1]

Hereditary cerebral hemorrhage with amyloidosis-Dutch type is an autosomal dominant disorder with complete penetrance (all individuals who inherit the mutated gene will develop the condition). The likely genetic defect is in the amyloid protein precursor protein (APP) gene on chromosome 21.[2]


Last updated: 2/5/2009

How might hereditary cerebral hemorrhage with amyloidosis - Dutch type be treated?

There is no known effective treatment for hereditary cerebral hemorrhage with amyloidosis - Dutch type. Treatment is supportive and based on the control of symptoms. In some cases, rehabilitation is needed for weakness or clumsiness. This can include physical, occupational, or speech therapy. Occasionally, some patients are good candidates for medications that can help improve memory.[1] The management of intracranial hemorrhage (ICH) related to hereditary cerebral hemorrhage with amyloidosis - Dutch type is identical to the standard management of ICH. The main objectives include reversing anticoagulation, managing intracranial pressure, and preventing complications.[7]
Last updated: 2/5/2009

References