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Thursday, 28 November 2013

Cerebral aneurysm - Diseases associated with Cerebral aneurysm

Cerebral aneurysm is defined as a cerebrovascular disorder causes of the blood vessel to bulge or balloon out of the wall of a blood vessel as a result of the weaken of blood vessels and veins and occurred mostly at the bifurcations and branches of the large arteries located at the Circle of Willis.
Diseases associated with Cerebral aneurysm
1. Von Recklinghausen's neurofibromatosis
Dr. J Baldauf and the research team at the Department of Neurosurgery, Helios Hospital Berlin report a case of an intracranial aneurysm associated with von Recklinghausen's neurofibromatosis. A 34-year-old woman presented with a history of headaches, unconsciousness and neck rigidity. Widespread cutaneous neurofibromas were found. Investigations revealed an aneurysm of the anterior communicating artery(28)


2. Behçet's disease
Although Cerebral aneurysms in Behçet's disease are very rare. Dr. S Nakasu and the research indicated there is a case of a 57 year old man with Behçet's disease is described, who had a subarachnoid hemorrhage due to rupture of a peripheral middle cerebral artery aneurysm. He underwent a successful aneurysmal clipping. Three years later he had seizures and was found to have a new aneurysm on the contralateral peripheral middle cerebral artery as well as some radiological features of vasculitis. After 3 months of steroid therapy, the aneurysm disappeared. Although surgical treatment is the first choice for ruptured aneurysms, steroid therapy may be effective for unruptured small aneurysms in patients with Behçet's disease(29).

3. Marfan syndrome
Marfan syndrome is an autosomal dominant connective tissue disorder commonly due to mutation of the fibrillin-1 (FBN-1) gene that causes disruption of elastic fibers in large- and medium-size arteries and predisposes to aneurysm formation and arterial dissection. Cardiovascular complications occur in most patients with Marfan syndrome, but interestingly, neurovascular complications of Marfan syndrome are rare(30). 

4. Pseudoxanthoma elasticum
Although intracranial aneurysms have been associated with many hereditary collagen disorders, the incidence of brain aneurysms in pseudoxanthoma elasticum (PXE) appears to be exceedingly low and uncertain. There is a rare case of a sisters with PXE who both developed intracranial aneurysms. This report supports the previously questioned hypothetical association between PXE and intracranial aneurysms(31).

5. Ehlers-Danlos syndrome
Ehlers-Danlos syndrome Type IV is a heritable connective tissue disorder with frequent neurovascular manifestations, such as intracranial aneurysms. Patients with this syndrome have notoriously fragile blood vessels, and the reported mortality rate for any type of vascular surgical procedure is 40%. This syndrome is rare, however, and the complication rate of aneurysm surgery may have been overestimated(32)

6. Hypoplasia and fibromuscular dysplasia
Fibromuscular dysplasia represents one of the more common types of arterial fibrodysplasia, a heterogeneous group of nonatherosclerotic vascular occlusive and aneurysmal diseases. There is a report of  the first case, to the knowledge, of an elderly man with infrarenal aortic fibromuscular dysplasia associated with aortic hypoplasia, without involvement of renal arteries, and contiguous aortoiliac aneurysm(33). 
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Sources
(28) http://www.neurologyindia.com/article.asp?issn=0028-3886;year=2005;volume=53;issue=2;spage=213;epage=215;aulast=Baldauf 
(29) http://jnnp.bmj.com/content/70/5/682.full
(30) http://www.ncbi.nlm.nih.gov/pubmed/21723458
(31) http://www.ncbi.nlm.nih.gov/pubmed/21423077
(32) http://www.ncbi.nlm.nih.gov/pubmed/12188938
(33) http://www.ncbi.nlm.nih.gov/pubmed/19118740