目的:探讨多发性硬化(multiple sclerosis,Ms)的临床特点。方法:回顾性分析61例经临床证实的 MS 的临床特点及治疗转归。结果:本组资料显示 MS 多见于青壮年女性,女性与男性之比为2.2:1;73.77%的患者发病无诱,多为急性起病,首发症状为...目的:探讨多发性硬化(multiple sclerosis,Ms)的临床特点。方法:回顾性分析61例经临床证实的 MS 的临床特点及治疗转归。结果:本组资料显示 MS 多见于青壮年女性,女性与男性之比为2.2:1;73.77%的患者发病无诱,多为急性起病,首发症状为视力障碍,其次为肢体无力、肢体麻木和共济失调;病损部位以视神经损害最常见,其次为脊髓、脑干、大脑半球白质。痛性强直性痉挛发作和 Lhermitte 氏综合征少见。脑脊液压力升高7.84%,蛋白质升高48.08%,IgG 指数升高占8.5%,寡克隆(OB)阳性34.1%。诱发电位检查,视觉施发电位(VEP)阳性>上肢体感诱发电位(SEP)>下肢 SEP>上肢 SSEP>听脑干诱发电位(BAEP)。磁共振成像(MRI)阳性>CT 扫描。皮质激素对复发缓解型 MS 发作期有效,但不能阻止其复发。结论:根据临床特征,结合脑脊液免疫学、神经电生理及头颅、脊髓影像学检查能显著提高临床确诊率。对 MS 的临床治疗仍存在亟待解决的问题。展开更多
We report the case of a 57-year-old man who presented bilateral subacute and painless optic neuropathy after meningopolyradiculitis revealing a primary human immunodeficiency virus infection. Both antiretroviral and s...We report the case of a 57-year-old man who presented bilateral subacute and painless optic neuropathy after meningopolyradiculitis revealing a primary human immunodeficiency virus infection. Both antiretroviral and steroid treatments were ineffective. Clinical symptoms and evolutive pattern were consistent with a mechanism of microvascular ischaemia of the optic nerve head. Optic neuropathies related to HIV infection are rare compared to those resulting from opportunistic infections. There are several pathophysiological mechanisms involved.展开更多
Background: Human herpesvirus 6 (HHV-6), a widesp- read virus and causative agent of exanthema subitum in children, has been asso ciated with a number of neurologic disorders including cranial nerve palsies, se izures...Background: Human herpesvirus 6 (HHV-6), a widesp- read virus and causative agent of exanthema subitum in children, has been asso ciated with a number of neurologic disorders including cranial nerve palsies, se izures, encephalitis, meningitis, and multiple sclerosis. Patient: A 31-year-old man presented with bilateral optic neuropathy, disc edema, and unilateral ton ic pupil, which were found to be associated with acute HHV-6 infection. The pat ient had been suffering from juvenile diabetes for 5 years. One week after onset of intravenous antiviral therapy with foscarnet, disc edema subsided, and tonic pupil reaction was no longer detectable. Conclusions: HHV6 infection may play a role as a causative agent in patients with optic neuropathy and tonic pupil.展开更多
患者男,46岁。因双眼下方视物模糊15 d伴双眼酸楚于2019年3月11日到河北省眼科医院就诊。患者有强直性脊柱炎(AS)病史10余年,未曾系统治疗。7 d前在当地医院确诊为“双眼前部缺血性视神经病变”,未予治疗。眼科检查:右眼视力0.8,左眼视...患者男,46岁。因双眼下方视物模糊15 d伴双眼酸楚于2019年3月11日到河北省眼科医院就诊。患者有强直性脊柱炎(AS)病史10余年,未曾系统治疗。7 d前在当地医院确诊为“双眼前部缺血性视神经病变”,未予治疗。眼科检查:右眼视力0.8,左眼视力0.25,矫正均不能提高。右眼、左眼眼压分别为18、13 mm Hg(1 mm Hg=0.133 kPa)。双眼眼前节检查未见异常,直接对光反射迟钝,晶状体及玻璃体无明显混浊。眼底检查,双眼视盘水肿、边界不清,视盘周围见线状出血,视网膜静脉充盈纡曲;黄斑水肿,中心凹反光不见;右眼鼻上方视网膜可见大量类圆形出血斑,左眼鼻侧视网膜可见少量类圆形出血斑(图1A,1B)。展开更多
Understanding the spectrum of idiopathic inflammatory demyelinating d isorders (IIDD) is a fundamental issue for the diagnosis and treatment of these disorders as well as for the approach to their pathogenesis. The sp...Understanding the spectrum of idiopathic inflammatory demyelinating d isorders (IIDD) is a fundamental issue for the diagnosis and treatment of these disorders as well as for the approach to their pathogenesis. The spectrum of IIDD is usually classified according to clin ical course and lesion distribution. We compared the demographic features, clini cal characteristics, laboratory findings, and genetic backgrounds between 193 Ja panese patients with and without clinically or radiographically fulminant attack s who all satisfied the diagnostic criteria for multiple sclerosis (MS). “Fulmi nant attacks”in the current study represent attack related clinically or radio logically severe relapses but do not necessarily mean severe disability. Patient s with fulminant attacks were clinically and immunogenetically distinct from tho se free of such attacks, and the previously described characteristics of the opt icospinal form of MS (OSMS) or neuromyelitis optica (NMO) were mostly shared by patients with fulminant attacks. HLA profiles were similar among patients with f ulminant attacks irrespective of the lesion distributions. The GG homozygous and G alleles of the CTLA4 gene A/G coding SNP at position 49 in exon 1 were signif icantly more common in patients with fulminant attacks than in those without. At tack related severity may be an important factor if validated by prospective st udies defining criteria and establishing relationships to disease course and tre atment regimens.展开更多
文摘目的:探讨多发性硬化(multiple sclerosis,Ms)的临床特点。方法:回顾性分析61例经临床证实的 MS 的临床特点及治疗转归。结果:本组资料显示 MS 多见于青壮年女性,女性与男性之比为2.2:1;73.77%的患者发病无诱,多为急性起病,首发症状为视力障碍,其次为肢体无力、肢体麻木和共济失调;病损部位以视神经损害最常见,其次为脊髓、脑干、大脑半球白质。痛性强直性痉挛发作和 Lhermitte 氏综合征少见。脑脊液压力升高7.84%,蛋白质升高48.08%,IgG 指数升高占8.5%,寡克隆(OB)阳性34.1%。诱发电位检查,视觉施发电位(VEP)阳性>上肢体感诱发电位(SEP)>下肢 SEP>上肢 SSEP>听脑干诱发电位(BAEP)。磁共振成像(MRI)阳性>CT 扫描。皮质激素对复发缓解型 MS 发作期有效,但不能阻止其复发。结论:根据临床特征,结合脑脊液免疫学、神经电生理及头颅、脊髓影像学检查能显著提高临床确诊率。对 MS 的临床治疗仍存在亟待解决的问题。
文摘We report the case of a 57-year-old man who presented bilateral subacute and painless optic neuropathy after meningopolyradiculitis revealing a primary human immunodeficiency virus infection. Both antiretroviral and steroid treatments were ineffective. Clinical symptoms and evolutive pattern were consistent with a mechanism of microvascular ischaemia of the optic nerve head. Optic neuropathies related to HIV infection are rare compared to those resulting from opportunistic infections. There are several pathophysiological mechanisms involved.
文摘Background: Human herpesvirus 6 (HHV-6), a widesp- read virus and causative agent of exanthema subitum in children, has been asso ciated with a number of neurologic disorders including cranial nerve palsies, se izures, encephalitis, meningitis, and multiple sclerosis. Patient: A 31-year-old man presented with bilateral optic neuropathy, disc edema, and unilateral ton ic pupil, which were found to be associated with acute HHV-6 infection. The pat ient had been suffering from juvenile diabetes for 5 years. One week after onset of intravenous antiviral therapy with foscarnet, disc edema subsided, and tonic pupil reaction was no longer detectable. Conclusions: HHV6 infection may play a role as a causative agent in patients with optic neuropathy and tonic pupil.
文摘患者男,46岁。因双眼下方视物模糊15 d伴双眼酸楚于2019年3月11日到河北省眼科医院就诊。患者有强直性脊柱炎(AS)病史10余年,未曾系统治疗。7 d前在当地医院确诊为“双眼前部缺血性视神经病变”,未予治疗。眼科检查:右眼视力0.8,左眼视力0.25,矫正均不能提高。右眼、左眼眼压分别为18、13 mm Hg(1 mm Hg=0.133 kPa)。双眼眼前节检查未见异常,直接对光反射迟钝,晶状体及玻璃体无明显混浊。眼底检查,双眼视盘水肿、边界不清,视盘周围见线状出血,视网膜静脉充盈纡曲;黄斑水肿,中心凹反光不见;右眼鼻上方视网膜可见大量类圆形出血斑,左眼鼻侧视网膜可见少量类圆形出血斑(图1A,1B)。
文摘Understanding the spectrum of idiopathic inflammatory demyelinating d isorders (IIDD) is a fundamental issue for the diagnosis and treatment of these disorders as well as for the approach to their pathogenesis. The spectrum of IIDD is usually classified according to clin ical course and lesion distribution. We compared the demographic features, clini cal characteristics, laboratory findings, and genetic backgrounds between 193 Ja panese patients with and without clinically or radiographically fulminant attack s who all satisfied the diagnostic criteria for multiple sclerosis (MS). “Fulmi nant attacks”in the current study represent attack related clinically or radio logically severe relapses but do not necessarily mean severe disability. Patient s with fulminant attacks were clinically and immunogenetically distinct from tho se free of such attacks, and the previously described characteristics of the opt icospinal form of MS (OSMS) or neuromyelitis optica (NMO) were mostly shared by patients with fulminant attacks. HLA profiles were similar among patients with f ulminant attacks irrespective of the lesion distributions. The GG homozygous and G alleles of the CTLA4 gene A/G coding SNP at position 49 in exon 1 were signif icantly more common in patients with fulminant attacks than in those without. At tack related severity may be an important factor if validated by prospective st udies defining criteria and establishing relationships to disease course and tre atment regimens.
