根據(jù)法國(guó)研究者的結(jié)果,,某些多灶性運(yùn)動(dòng)神經(jīng)病患者,無(wú)論有無(wú)外在傳導(dǎo)阻滯癥狀,,都可以對(duì)免疫球蛋白IV作出反應(yīng),。
該研究的首席科學(xué)家Emilien Delmont博士說(shuō):“這表明臨床判斷仍然是診斷多灶性運(yùn)動(dòng)神經(jīng)病的金標(biāo)準(zhǔn)。
因此,,IVIg或許可以作為處方藥供慢性非對(duì)稱運(yùn)動(dòng)肌無(wú)力的備選病人使用,。即使這些病人經(jīng)神經(jīng)傳導(dǎo)測(cè)試檢測(cè)其外周神經(jīng)分布并無(wú)傳導(dǎo)阻滯癥狀。
在今年8月的《Neurology,》雜志上,,法國(guó)尼斯市巴斯德醫(yī)院的Delmont博士和他的同事們提到,,他們研究了20名存在傳導(dǎo)阻滯癥狀和13名不存在這種癥狀的病人,根據(jù)研究數(shù)據(jù)得出的這個(gè)結(jié)論,。隨訪這些病人已經(jīng)超過(guò)4年時(shí)間,。所有病人均患有非對(duì)稱的運(yùn)動(dòng)肌無(wú)力但并沒(méi)有感覺(jué)、球麻痹和呼吸方面的癥狀,,且不牽涉上位神經(jīng)元,。
組間并沒(méi)有觀察到顯著區(qū)別,但在無(wú)傳導(dǎo)阻滯癥狀的病人中,,牽涉中位神經(jīng)元的情況顯著較少,。近端肌無(wú)力也存在這種情況。
的治療效果兩組很相近不存在傳導(dǎo)阻滯癥狀的病人中有8(61%)人對(duì)這種治療有反應(yīng),,而存在這種癥狀的病人中有14(70%)人有反應(yīng),。
馬里蘭州巴爾地摩市約翰霍普金斯大學(xué)醫(yī)學(xué)院的Vinay Chaudhry博士,也是這篇文章的共同通訊作者,,他說(shuō)他認(rèn)同這種方法,。
他說(shuō):“[glow=255,red,2][glow=255,red,2]關(guān)于IVIg的實(shí)驗(yàn)證實(shí)它可以用于在多灶性外周神經(jīng)分布處患有進(jìn)展性非對(duì)稱末端肌無(wú)力的病人即使這些病人在神經(jīng)傳導(dǎo)測(cè)試中發(fā)現(xiàn)并無(wú)神經(jīng)阻滯癥狀。”
備注:
多灶性運(yùn)動(dòng)神經(jīng)病是近年來(lái)才被認(rèn)識(shí)的少見(jiàn)的獲得性免疫介導(dǎo)的神經(jīng)病,,多為緩慢隱匿性起病,,以非對(duì)稱性肌無(wú)力、肌萎縮,、肌束顫動(dòng),、腱反射降低為特征,無(wú)呼吸肌麻痹,、無(wú)腦神經(jīng)受累,、無(wú)感覺(jué)障礙、無(wú)錐體束征,。有文獻(xiàn)報(bào)道多灶性運(yùn)動(dòng)神經(jīng)病可以用大量免疫球蛋白注射治療,,約80%~90%患者癥狀可緩解。
英文原文:
Immunoglobulin May Be Useful in Multifocal Motor Neuropath
Certain patients with multifocal motor neuropathy with or without apparent conduction block respond to IV immunoglobulin (IVIg) therapy, according to French researchers.
"This study shows that clinical judgment remains the gold standard in diagnosis of multifocal motor neuropathies," lead investigator Dr. Emilien Delmont told Reuters Health.
"Hence, a trial of IVIg may be prescribed for selected patients presenting with chronic asymmetric motor weakness with a peripheral nerve distribution without any conduction block being detected in the nerve conduction study," he added.
In the August issue of Neurology, Dr. Delmont of Hopital Pasteur, Nice and colleagues note that they came to this conclusion after studying data on 20 such patients with conduction block and 13 without. They had been followed for more than 4 years. All had asymmetric motor weakness without sensory, bulbar or respiratory signs, and no upper motor neuron involvement.
Few significant differences were seen between groups, but involvement of the median nerve was significantly less frequent in patients without conduction block. This was also true of proximal weakness.
The efficacy of IVIG was similar in both groups, with responses in 8 (61%) of patients without conduction block and 14 (70%) with conduction block.
Dr. Vinay Chaudhry of Johns Hopkins University School of Medicine, Baltimore, Maryland, author of an accompanying editorial, told Reuters Health that he agreed with the approach.
"A trial of IVIg may be justified in patients with progressive distal asymmetric weakness in a multifocal peripheral nerve distribution even if they don't have conduction block on nerve conduction studies," he said.