【正文】
,其中約 50%累及中樞神經(jīng)系統(tǒng),也可出現(xiàn)腦神經(jīng)麻痹和多發(fā)性周圍神經(jīng)病等。 SLE患者腦內(nèi)多有血管病變和損害周圍神經(jīng)。一些患者神經(jīng)系統(tǒng)癥狀和體征有自發(fā)性緩解,提示血管病變所致的缺血是可逆性的,并非永久性的損害。 系統(tǒng)性紅斑狼瘡( SLE) 又稱干燥綜合征或干燥性角膜結(jié)膜炎。 Sjogrens綜合征 系指一類由于供給周圍神經(jīng)的血管病變而導(dǎo)致的缺血性神經(jīng)病。常見于結(jié)節(jié)性多動(dòng)脈炎、 TTP、SLE、伯格病( Buerger病或閉塞性血栓性脈管炎)、淀粉樣變性、動(dòng)脈粥樣硬化、糖尿病、機(jī)械性壓迫等。由于病因、病程、病情嚴(yán)重程度、累及范圍不同,故臨床表現(xiàn)也有較大的差別。在慢性閉塞性血管病患者中,可累及單神經(jīng)、多發(fā)單神經(jīng)或多發(fā)性神經(jīng)病,可只累及脊神經(jīng)或(和)腦神經(jīng);以遠(yuǎn)端或近端為主,可對(duì)稱或不對(duì)稱或某些肌群為主;多發(fā)性神經(jīng)病常伴自主神經(jīng)受累。 血管源性神經(jīng)病 主要特征為 P多發(fā)性神經(jīng)病, O臟器腫大, E內(nèi)分泌病、 M蛋白和 S皮膚改變,集英文首字母,稱為 POEMS綜合征??赡苁怯蓾{細(xì)胞產(chǎn)生異常免疫球蛋白血癥導(dǎo)致多系統(tǒng)損害。以感覺 運(yùn)動(dòng)型神經(jīng)病突出,下肢受累較上肢早且重,左右可不對(duì)稱。肌電圖呈神經(jīng)性損害,運(yùn)動(dòng)、感覺傳導(dǎo)速度早期可以減慢。若出現(xiàn)臟器腫大、內(nèi)分泌異常(高血糖也常見)、 M蛋白和骨髓漿細(xì)胞增生、低熱、血沉快、體重下降、下肢水腫、杵狀指及白甲,以及滲出性胸腹水、心包積液(三腔積液),則診斷不難 POEMS綜合征( CrowFukase綜合征) 治 療 Management of Neuropathy ? Once neuropathy is diagnosed, therapy can then be instituted with the goal of both ameliorating symptoms and preventing the progression of neuropathy. Successful management of these syndromes must be geared to the individual pathogenic processes. 是治療本癥的基本原則。 The DCCT research Group reported significant effects of intensive insulin therapy on prevention of neuropathy. (1) 嚴(yán)格控制糖尿病 ?控制好糖尿病,延緩神經(jīng)病變的進(jìn)展 胰島素強(qiáng)化治療 血糖降低過快 胰島素性神經(jīng)炎 ? : This was a stepwise, progressive study that involved treatment of type 2 diabetes patients, with hypotensive drugs, including ACE inhibitors, Ca2+ channel antagonists, hypoglycemic agents, aspirin, hypolipidemic agents, and antioxidants. These findings argue strongly for the multifactorial nature of neuropathy and for the need to address the multiple metabolic abnormalities. ( 2) 多因素綜合干預(yù)治療 ( 3)改善血液循環(huán) ? 抗凝劑 藻酸雙酯鈉、 尿激酶、蝮蛇抗栓酶 ? 鈣拮抗劑尼莫地平、 ? 654立其丁、 ? 凱時(shí) ? 培達(dá) 以往多年試用大量多種 B族維生素包括 B BB B12及復(fù)合維生素 B大都無效。 PKC之類似劑。 Alphalipoic acid Human intravenous immunoglobulin (IVIg) 神經(jīng)鞘營(yíng)養(yǎng)素。73) have been discontinued because of toxicity. There are, however, successors to aminoguanidine that hold promise for this approach (74). ① 物理療法。 (5)對(duì)癥治療 ② 鎮(zhèn)痛劑。 clonidine lidocaine Tramadol Gabapentin加巴噴丁 phenytoin Transcutaneous nerve stimulation calcitonin ☆ Patients must be instructed on foot care with daily foot inspection ☆ They must have a mirror in the bathroom for inspection of the soles of the feet ☆ Providing patients with a monofilament for selftesting reduces ulcers Management of Small Fiber Neuropathies ☆ All diabetic patients should wear padded socks Shoes must fit well with adequate support and must be inspected for the presence of foreign bodies (. nails, pins, teeth etc) before donning . examine the feet and the shoes daily. ☆ Patients must exercise care with exposure to heat (no falling asleep in front of fires) ☆ Emollient creams should be used for the drying and cracking ☆ After bathing feet should be thoroughly dried and p