Abstract:
:Behcet's disease (BD) is a rare condition, more commonly seen in Turkey, the Middle East, the Mediterranean, and the Far Eastern countries. It is a multifaced disease involving the central nervous system (CNS), skin, joints, intestines and blood vessels. CNS involvement occurs in 10-25% of the patients with BD. We report two cases with Neuro-Behcet's disease. Both patients were females and they were in their second decade of life (early forties) when they suffered from relapsing retinitis and uveitis which led to blindness, despite treatment with steroids. They presented with clinical signs of brainstem syndrome, meningoencephalitis and the initial radiological investigations of our patients revealed encephalitic lesions in the brainstem. In the first case, an intracerebral abscess developed 4 years later, and it was evacuated under stereotactic guidance. The most common neuropathologic findings in Neuro-Behcet's disease are focal necrotic lesions in the brain. However, the association of Behcet's disease (BD) with cerebral abscess is very rare. There were only three reported cases of such. Ten years after the initial diagnosis of BD, our second patient suffered from subarachnoid hemorrhage due to a ruptured aneurysm of the superior cerebellar artery, which was then resected successfully. Intracranial aneurysm in patients with Behcet's disease has previously been documented in eight patients. To our knowledge, this is the only case of Neuro-Behcet's disease associated with an aneurysm of the superior cerebellar artery.
journal_name
Clin Neurol Neurosurgjournal_title
Clinical neurology and neurosurgeryauthors
Ho CL,Deruytter MJdoi
10.1016/j.clineuro.2004.07.002keywords:
subject
Has Abstractpub_date
2005-06-01 00:00:00pages
310-4issue
4eissn
0303-8467issn
1872-6968pii
S0303-8467(04)00091-5journal_volume
107pub_type
杂志文章,评审abstract::Charcot-Marie-Tooth disease (CMT) is a group of clinically and genetically heterogeneous neuropathies classically divided into demyelinating (CMT1) and axonal forms (CMT2). The most common demyelinating form is CMT1A, due to a duplication in the gene encoding the peripheral myelin protein 22 (PMP22). Less frequently, ...
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journal_title:Clinical neurology and neurosurgery
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journal_title:Clinical neurology and neurosurgery
pub_type: 杂志文章
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journal_title:Clinical neurology and neurosurgery
pub_type: 杂志文章,评审
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journal_title:Clinical neurology and neurosurgery
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pub_type: 杂志文章
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journal_title:Clinical neurology and neurosurgery
pub_type: 杂志文章,评审
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pub_type: 杂志文章
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journal_title:Clinical neurology and neurosurgery
pub_type: 杂志文章
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pub_type: 杂志文章,评审
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journal_title:Clinical neurology and neurosurgery
pub_type: 杂志文章
doi:10.1016/j.clineuro.2004.02.024
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pub_type: 杂志文章,评审
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pub_type: 杂志文章
doi:10.1016/j.clineuro.2007.07.006
更新日期:2007-11-01 00:00:00
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pub_type: 杂志文章
doi:10.1016/0303-8467(94)90117-1
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pub_type: 杂志文章
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更新日期:2002-09-01 00:00:00
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