伴有皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病与多发性硬化症。要么一个,有时两个?

G. Paraskevas, V. Constantinides, E. Kapaki
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引用次数: 4

摘要

脑常染色体显性动脉病变伴皮质下梗死和白质脑病(CADASIL)是遗传性脑血管病、遗传性卒中和遗传性血管性痴呆的最常见原因。CADASIL被误认为多发性硬化症(MS)并不罕见。一种少见但存在的情况是CADASIL和MS(或MS样炎症)同时发生。这些患者可能表现为脊髓病变,脑或脊髓增强病变,脑脊液中寡克隆带阳性,IgG指数高,对皮质类固醇或免疫调节治疗反应良好。CADASIL可通过多种机制触发或调节自身免疫反应,并可能伴随炎症成分或引起ms样疾病。
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Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy vs. multiple sclerosis. Either one or sometimes both?
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL), is the most common cause of inherited cerebral small vessel disease, inherited stroke and inherited vascular dementia. It is not infrequent for CADASIL to be mistaken and mistreated for multiple sclerosis (MS). A much less frequent but existing scenario is the co-occurrence of CADASIL and MS (or MS-like inflammatory condition). Such patients may present with spinal cord lesions, brain or spinal cord enhancing lesions, positive oligoclonal bands and high IgG index in the cerebrospinal fluid and good response to corticosteroids or immunomodulating treatments. CADASIL through various mechanisms may trigger or modulate autoimmune reactions, and either be complicated by an inflammatory component or cause an MS-like disorder.
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