皮质类固醇对多发性硬化IgG指数和IgG合成率的影响

采用Beckman免疫化学仪(ICS—Ⅱ),对21例经皮质类固醇(激素)治疗的多发性硬化(MS)患者(治疗组)和19例未经激素治疗的MS患者(未治疗组)进行IgG指数和IgG合成率测定。结果发现:治疗组IgG指数和IgG合成率均明显低于未治疗组,差异有极显著性意义(P<0.01)。用于MS诊断时,治疗组IgG指数和IgG合成率的敏感性亦明显低于未治疗组(P<0.01)。提示激素可降低中枢神经系统内IgG自身合成量,并可降低IgG指数和IgG合成率诊断MS的敏感性。     

寡克隆带和IgG鞘内合成率对多发性硬化的诊断价值

目的探讨寡克隆带(OCBs)和IgG鞘内合成率(IgGSyn)对多发性硬化(MS)诊断的敏感性、特异性,以及定性和定量指标的相关性。方法选取30例MS(MS组)、40例神经系统炎性疾病(NID组)和22例神经系统非炎性疾病(NNID组)患者,应用速率散射比浊法测定血清和脑脊液(CSF)中免疫球蛋白G(IgG)、白蛋白(Alb)水平,等电聚焦结合银染色法检测CSF中OCBs,计算IgGSyn,并对其敏感性、特异性和阳性结果似然比(PRLR)进行分析。结果OCBs阳性率和IgGSyn异常率MS组与NID组比较差异无显著性;MS组、NID组与NNID组比较差异有极显著性(均P<0.01)。MS组和NID组中,OCBs阳性者与阴性者IgGSyn值差异无显著性。对MS诊断的敏感性、特异性和PRLR,OCBs分别为63.3%、77.7%和2.8;IgGSyn为46.7%、75.2%和1.9。结论OCBs和IgGSyn检测结果的不完全一致性提示中枢神经系统内存在不同的体液免疫反应机制,综合分析OCBs和IgGSyn,对MS诊断具有参考价值。     

The intrathecal synthesis of virus-specific oligoclonal IgG in multiple sclerosis

A highly sensitive antigen-mediated capillary blot technique was developed for the detection of virus-specific oligoclonal IgG in paired CSF and serum samples from patients with various neurological diseases. In multiple sclerosis, intrathecal synthesis of oligoclonal antibodies was present against measles (70%), rubella (60%), varicella zoster (40%) and mumps (30%); in most cases (75%), such synthesis involved two or more viruses. In contrast, antibodies against a non-neurotropic virus (cytomegalovirus) were rarely produced in CSF from MS patients (5%). However, this ‘polyspecific’ reaction was not restricted to MS samples but was also observed in neurolupus and in the late phase of infectious diseases of the central nervous system. These anti-viral antibodies could be produced without de novo replication of the corresponding viral genome and are likely mere bystanders of an ongoing immune response.     

Isotachophoresis evaluation of synthesis of intrathecal IgG subfractions in multiple sclerosis

Summary Cerebrospinal fluid (CSF) and serum samples from patients with multiple sclerosis and other neurological diseases were examined by capillary isotachophoresis (ITP). The percentage and the rate of synthesis of CSF IgG which migrated slowly with ITP were calculated.CSF specimens of most patients with multiple sclerosis contained increased percentages of slowly migrating IgG (slow IgG), corresponding to IgG oligoclonal bands in the high-alkaline region on isoelectric focusing. The patients with multiple sclerosis were found to have increased intrathecal synthesis of slow IgG, which correlated closely with the rate of intrathecal CNS IgG synthesis calculated by Tourtellotte's formula.

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Zusammenfassung Liquor und Serum von Patienten mit Multipler Sklerose und anderen neurologischen Erkrankungen wurden mittels Kapillar-Isotachophoresis (ITP) untersucht. Es wurde berechnet, welchen Anteil das IgG im Liquor, welcher langsam mit der ITP wanderte, ausmachte und wie seine Synthesegeschwindigkeit war. Bei den meisten Patienten mit Multipler Sklerose enthielt der Liquor einen erhöhten Anteil von langsam wandernden IgG, welches oligoclonalen Banden im hochalkalischen Bereich beim isoelectric focusing entsprach. Es wurde gefolgert, daß die Patienten mit Multipler Sklerose eine erhöhte intrathekale Synthese des langsamen IgG aufwiesen, wobei dies eng korrelierte mit dem Anteil der intrathekalen Liquor-IgG-Synthese, wie sie nach der Tourtellotteschen Formel errechnet werden kann.

     

多发性硬化脑脊液寡克隆区带及24小时鞘内IgG合成率检测的比较

采取垂直平板聚丙烯酰胺凝胶电泳法、火箭免疫电泳法对30例多发性硬化病人进行脑脊液寡克隆区带及24小时鞘内IgG合成率的检测,结果发现寡克隆区带、合成率与多发性硬化的发病年龄、病程、病程进展类型、病残程度无统计学上的相关性,寡克隆区带阳性率46.7％,合成率增高占60％,两者合计异常率可达73.3％,提示我国多发性硬化寡克隆区带阳性率、合成率增高均较国外低,因而在我国对多发性硬化的实验室诊断两者同时检测更有意义。     

Azathioprine reduces intrathecal IgG synthesis in multiple sclerosis

Intrathecal IgG synthesis and CSF oligoclonal bands were reexamined after 18-24 months in 66 patients with multiple sclerosis; 40 of them received azathioprine (AZA) 2.5 mg/Kg/die; all received a course of dexamethasone (DEXA) during clinical relapses. The IgG Index was significantly reduced in the group treated with AZA, especially in patients with short disease duration, low disability and high IgG index. Changes observed in CSF banding pattern were not significant. These results suggest an effect of AZA on IgG synthesis, as reported by in vitro studies.     

IgG ratios and oligoclonal IgG in multiple sclerosis and other neurological disorders

The findings are reported of various CSF abnormalities, including IgG indices and oligoclonal IgG, in 160 patients with multiple sclerosis of differing diagnostic certainty and 146 patients with other neurological disorders.

An abnormal IgG index, defined as the ratio of IgG/albumin in CSF to that in serum, has been found in 77.7% of definite MS cases, falling to a figure of 32.1% in the single lesion group. A tendency, reported previously, for IgG levels to be higher in disabled patients, particularly those with a short history or early onset, has been confirmed.

Oligoclonal IgG, on the other hand, has been found in 56% of definite MS cases, less frequently than in most other reported series. Analysis of the literature suggests considerable variability in the finding of oligoclonal IgG in other than definite MS, and in other neurological disorders. The possibility that subjective factors are partly responsible for this variability, rather than discrepancies in patient selection requires consideration, and suggests that CSF electrophoresis and IgG estimations are complementary aids in the diagnosis of multiple sclerosis.

Differences have been expressed regarding the relationship of oligoclonal IgG to clinical parameters of the disease. Further sequential analysis of the development and variability of the oligoclonal pattern in MS is required.     

中国同心圆硬化72例临床特点分析

目的了解我国同心圆硬化患者的临床特点。方法检索维普电子期刊全文数据库1989年-2015年间收录的同心圆硬化病例报告文献,并分析所报道病例的临床特点。结果共纳入46个相关研究,包括72例患者,其中男性35例,女性37例,发病年龄最小为17岁,最大63岁,平均发病年龄为(37.88±9.85)岁。2例发病前有疫苗接种史,另2例发病前有感冒前驱感染史。53例(73.61%)患者以运动障碍为首发症状,19例以头痛、头晕或言语不利等为首发症状。52例患者进行了脑脊液检查,其中30例正常、5例脑脊液压力增高、13例脑脊液蛋白增高、10例脑脊液寡克隆区带(OB)阳性。同心圆硬化病灶累及部位最多见于额叶,其余累及部位还包括顶叶及半卵圆中心等。大多数均为多发病灶,仅3例为单发病灶。个别同心圆硬化可伴随甲状腺功能减退(2例)与周围神经损害(1例)。结论中国同心圆硬化以中青年常见,男女发病无明显差异,大多数无诱发因素,常见首发症状包括肢体障碍、头晕、头痛,实验室检查常无明显异常,脑脊液寡克隆区带出现率较低,诊断依赖于头部MRI。     

Frequency and relevance of IgM intrathecal synthesis in multiple sclerosis

Using a new ELISA method we have measured the IgM concentration in the serum and the cerebrospinal fluid CSF from 110 neurological patients. Among there, 41 had multiple sclerosis (MS), 48 other inflammatory diseases (OID), including 30 AIDS, and 21 non-inflammatory neurological diseases (NID). A highly significant correlation was established between results with native IgM and the dithiothreitol reduced IgM. An intrathecal synthesis (ITS) of IgM was detected using the CSF IgM/CSF albumin ratio, the IgM index and a quantitative formula in 33 patients: nine MS, 23 OID (including 18 AIDS) and one NID. The frequency of IgM ITS was 22% in MS patients, 48% in the OID (60% in AIDS) and 5% in the NID groups. This ITS was not impaired by an increase in serum IgM concentration or by a blood–CSF barrier damage. These facts confirm that intrathecal immunity is not a “steady-state” related to the general immunity but a specific response restricted to the central nervous system. Conversely, CSF IgM increase and IgM ITS were closely related (p < 10^?6). In addition, IgM ITS and IgG ITS were found to be highly correlated in OID, especially in AIDS patients: such correlation was not observed in the MS group. No significant correlations were observed between IgM ITS and any of the clinical parameters in MS patients. These results suggest the probable specificity of IgM ITS in MS patients.     

IgG subclasses and their intrathecal synthesis in patients with amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis may be an autoimmune disease. In this paper IgG subclasses levels in the CSF and sera and their intrathecal synthesis were studied. IgG subclasses levels were determined by ELISA method using monoclonal antibodies against human IgG subclasses, secondary biotinylated antibody and avidin-biotin-peroxidase complex. There was statistically significant elevation of IgG1 and IgG3 subclasses in the CSF of ALS patients. In sera of patients with ALS, IgG2 level was diminished, but there was no statistical difference in other IgG subclasses. IgG1 and IgG3 indices were elevated in patients with ALS, detecting synthesis of these subclasses in the CNS. General IgG index value did not differ from the control value. The results support the concept that autoimmune mechanisms may play a role in the pathogenesis of ALS.     

Therapeutic trials of multiple sclerosis and intrathecal IgG production

In our clinical trial we have compared the effect of high doses of prednisone, ACTH alone or ACTH and cyclophosphamide on plasma and CSF albumin and IgG levels.We have found that the treatment with high doses of prednisone is more effective in the suppression of CNS IgG synthesis than ACTH, or cyclophosphamide. However the significance of this immunological phenomenon in the pathogenesis of MS is a very complex problem, since we have not observed any correlation between the depression of the intrathecal IgG synthesis and clinical results of MS treatment.This text was presented as communication at the Italo-Polish meeting held in Rome on 20–21 April 1985, arranged by the Società Italiana di Neurologia.     

Antibodies against viruses and structural brain components in oligoclonal IgG obtained from multiple sclerosis brain

Summary Immunoglobulin G (IgG) was isolated from three multiple sclerosis (MS) and two control brains by Protein A Sepharose affinity chromatography and was characterized by thin-layer polyacrylamide gel isoelectric focusing (PAG IEF) and antiserum immunofixation. The three MS brains contained oligoclonal IgG. Immunofixation with measles, herpes simplex, varicella, rubella, mumps and cytomegalovirus as antigens and autoradiography revealed that some of the oligoclonal IgG bands separated by PAG IEF contained antibodies against herpes simplex in one, measles in two and varicella virus in all three MS brains. No antibodies were detected with this technique against structural human (crude saline, lipid-proteolipid, ganglioside, and myelin basic protein extracts of MS and normal human brain) and bovine (purified myelin, myelin basic protein and oligodendrocytes of bovine brain) brain components. The finding of viral antibodies and the absence of antibodies against structural brain proteins in oligoclonal MS brain IgG is similar to that previously recorded in MS cerebrospinal fluid (CSF).

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Zusammenfassung Immunoglobulin G, IgG, wurde aus der Gehirnsubstanz von drei Fällen mit multipler Sklerose (MS) und zwei Kontrollen mittels Affinitätschromatographie mit Protein A-Sepharose isoliert. Das IgG wurde mit isoelektrischer Fokussierung in Polyacrylamidgelplatten (PAG IEF) und anschließender Antiserum-Immunofixation charakterisiert. Bei MS wurde oligoklonales IgG gefunden. Mit Hilfe von Antigen-Immunofixation mit Masern, Herpes simplex, Varizellen, Rubella, Parotitis und Zytomegalievirus und nachfolgender Autoradiographie (radioaktive Zweitantikörper gegen die genannten Antigene) konnte gezeigt werden, daß die oligoklonalen Bande in den drei MS-Gehirnen Antikörper gegen Herpes simplex in einem, Masern in zwei und Varizellen-Virus in allen drei Fällen enthielten. Keine solchen Antikörper wurden entdeckt, wenn die Technik für strukturelle Gehirn-Komponenten vom Menschen (einfache physiologische Salz-, Lipid-Proteolipid-, Gangliosid- und basische Myelo-Protein-Extrakte von MS und normalem Gehirn) und vom Rind (gereinigtes Myelin, basisches Myelo-Protein und Oligo-Dendrozyten aus Gehirnrinde) verwendet wurde.

     

Intrathecal IgG synthesis in multiple sclerosis: Comparison between isoelectric focusing and quantitative estimation of cerebrospinal fluid IgG

Summary Isoelectric focusing of CSF and serum IgG followed by crossed immuno isoelectric focusing and direct immunofixation as well as quantitative assay of IgG and albumin were performed in 64 clinically definite multiple sclerosis patients. Intrathecal IgG synthesis was calculated according to the CSF IgG index and de novo CNS IgG_syn. Isoelectric focusing showed abnormal IgG fractions in CSF indicating increased intrathecal synthesis of oligoclonal IgG in 99% of patients. Only 62% and 70% of multiple sclerosis patients showed values of CSF IgG indices and de novo CNS IgG_syn higher than in controls. Increased intrathecal IgG synthesis was indicated more frequently by de novo CNS IgG_syn in patients with a normal CSF IgG index than by the CSF IgG index in patients with normal de novo CNS IgG_syn. All patients with blood CSF barrier damage had increased de novo CNS IgG_syn, but only 40% had an increased CSF IgG index. Isoelectric focusing seemed to be a more sensitive method to detect an increased intrathecal oligoclonal IgG synthesis than quantitative methods. Identification of abnormal IgG fractions can be performed easily and with more reproducible results by direct immunofixation than by crossed immuno isoelectric focusing. The formula for de novo CNS IgG_syn seemed more sensitive and less influenced by blood-CSF barrier damage than the CSF IgG index to detect increased intrathecal IgG synthesis in multiple sclerosis patients. No correlation was found between the CSF IgG pattern or amounts and age, duration, clinical course or therapy of the disease.

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Zusammenfassung Bei 64 klinisch bestimmten Fällen von multipler Sklerose wurde die isoelektrische Fokussierung von CSF und Serum-IgG, gefolgt von crossed immuno-isoelektrischer Fokussierung und direkter Immunofixierung, sowohl bei der quantitativen Bestimmung als auch bei Albumin, durchgeführt. Die IgG-intrathekal-Synthese wurde entsprechend dem CSF-IgG-Index und de-novo-CNS-IgG_syn berechnet. Bei 99% der Patienten zeigte die isoelektrische Fokussierung abnorme Fraktionen im CSF an, ein Hinweis auf eine erhöhte intrathekale Synthese von oligoklonalem IgG. Nur bei 62–70% der Multiple-Sklerose-Fälle waren die Werte des CSF-IgG-Index und de-novo-CNS-IgG_syn höher als bei Vergleichskontrollen. Eine erhöhte intrathekale Synthese wurde häufiger von de-novo-CNS-IgG_syn bei Patienten mit normalem CSF-IgG-Index als von CSF-IgG-Index bei Patienten mit normalem de-novo-CNS-IgG_syn angezeigt. Bei allen Patienten mit Störung der Blut-CSF-Schranke war de-novo-CNS-IgG_syn erhöht, dagegen wurde bei nur 40% eine Erhöhung des CSF-IgG-Index festgestellt. Im Vergleich zu den quantitativen Verfahren scheint die isoelektrische Fokussierung die genauere Methode zur Feststellung einer erhöhten Intrathekal-oligoklonal-IgG-Synthese zu sein. Abnorme IgG-Fraktionen werden eher durch direkte Immunofixierung einfacher und mit wenig voneinander abweichenden Resultaten festgestellt als durch immunoisoelektrische Fokussierung. Die Formel für die de-novo-CNS-IgG_syn scheint sensibler und weniger durch Störungen der Blut-CSF-Schranke beeinflußbar zu sein als der CSF-IgG-Index bei der Bestimmung erhöhter Intrathekal-IgG-Synthese bei Fällen von multipler Sklerose. Es wurde kein Zusammenhang zwischen der CSF-IgG-Zusammensetzung und den Werten zum Alter, der Krankheitsdauer, dem klinischen Verlauf und der Behandlungstherapie des Kranken gefunden.

     

Intrathecal IgG synthesis: marker of progression in multiple sclerosis patients

OBJECTIVES: We study the power of IgG synthesis value as a marker of disease activity in multiple sclerosis (MS). MATERIAL AND METHODS: Link index was calculated in 202 MS patients. Time between first, second and third attack and progression index (PI) were compared in patient with normal (NLI) high (HL) or very high Link index (VHLI). RESULTS: Secondary progressive (SP) patients had a higher LI than relapsing-remitting (RR) and primary progressive (PP) courses (1.10 +/- 0.5 for SP vs 0.86 +/- 0.5 for RR and 0.81 +/- 0.5 for PP, P=0.01 and 0.03, respectively). Having a HLI in MS RR and SP patients has no time effect in the development of the second and third attack. PI was higher in patients with VHIL (0.67 +/- 0.7) vs patients with NLI (0.42 +/- 0.4, P=0.008) and with HLI (0.39 +/- 0.3, P=0.001). CONCLUSIONS: This study confirmed that LI is a good marker of subsequent progression of MS.     

Intrathecal synthesis of IgG subclasses in multiple sclerosis and in acquired immunodeficiency syndrome (AIDS)

Serum and cerebrospinal fluid (CSF) of 50 neurological patients (24 multiple sclerosis (MS), ten acquired immunodeficiency syndrome (AIDS) and 16 other neurological diseases (OND)) and ten controls were analyzed by enzyme-linked immunosorbent assay (ELISA) for IgG subclass quantification and for the calculation of intrathecal synthesis (ITS). Total IgG was determined by two methods: electroimmunodiffusion (EID) and ELISA. A highly significant correlation was established between both methods. The existence of ITS was proved by the IgG/albumin ratio, the IgG index, Tourtellotte's formula, and Schuller's formula. In AIDS patients all IgG subclasses showed an increase in the CSF, whereas in sera only the IgG1 was significantly increased. CSF of MS patients showed a predominant increase of IgG1 whereas no significant modification of IgG subclasses was observed in sera. In most of the AIDS patients there was an ITS of IgG1, IgG3 and IgG4, but rarely (3/10) IgG2. In contrast, a polyclonal ITS of IgG was exceptional (1/24) in MS patients. No significant correlation could be established between clinical data and IgG subclass ITS in MS. The variations of each IgG subclass in serum and in ITS were not significantly correlated. Measurement of each IgG subclass and calculation of ITS seems essential in order to analyze any subclass antibody repertory inside the central nervous system.     

寡克隆区带和IgG指数在多发性硬化中的诊断意义

目的研究寡克隆区带（OCBs）和IgG指数（IgGI）对多发性硬化（MS）诊断的敏感性及其影响因素。方法用等电聚焦结合银染色法检测30例MS、40例神经系统炎性疾病（NID）和22例神经系统非炎性疾病（NNID）患者CSF中OCBs，并计算IgG I。结果MS组和NID组比较OCBs阳性率、IgG I异常率均无显著性差异（P〉0．05）；MS组、NID组与NNID组比较。差异均有显著性（P〈0．05）；传统型MS和脊髓型MS比较，差异均无显著性（P〉0．05）。OCBs对MS诊断的敏感性、特异性和阳性结果似然比分别为63．3％、77．7％和2．8；IgG I分别为40．0％、76．7％和1．7。结论本地区MSOCBs阳性率和IgG I异常率较低，可能与遗传背景、疾病类型和药物应用有关，OCBs和IgG I对MS诊断具有相对特异性。     

Intrathecal methylprednisolone acetate in multiple sclerosis treatment: effect on the blood-CSF barrier and on the intrathecal IgG production

28 patients with clinically definite multiple sclerosis were treated with intrathecal injections of Methylprednisolone acetate, 40 mg at 4-day intervals. No modifications in the oligoclonal pattern of the CSF IgG were seen following-treatment in any of the cases, although changes in relative band intensity were observed. After 3 intrathecal injections, 54.8% of the cases showed a decrease of more than 15% in the amount of IgG synthesized daily within the central nervous system (de novo CNS IgG SYN). The IgG SYN rate was significantly decreased in 50.0% of the cases after 4 injections and in 91.7% after 5. With regard to blood-CSF barrier function, an increase of more than 15% of the initial value of the Serum/ CSF albumin quotient was recorded in 48.4% of cases after 3 injections, in 34.6% after 4 and in 58.3% after 5 injections.

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Sommario 28 pazienti con sclerosi multipla certa in base a criteri clinici sono stati trattati con iniezioni intratecali di Metilprednisolone acetato. Il pattern oligoclonale delle IgG liquorali non subiva modificazioni in seguito alla terapia, anche se in alcuni casi potevano essere osservate modificazioni nella intensità delle singole bande. Dopo tre iniezioni intratecali la quantità di IgG sintetizzata entro il sistema nervoso centrale nelle 24 ore (de novo CNS IgG SYN) era ridotta di più del 15% nel 54.8% dei casi, mentre dopo quattro e cinque iniezioni una riduzione significativa si osservava rispettivamente nel 50% e 91.7% dei casi. Il quoziente Albuminasiero/ Albumina LCS, considerato quale parametro di funzionalità della barriera ematoliquorale, risultava aumentato di più del 15% rispetto al valore iniziale nel 48.4%, 34.6% e 58.3% dei casi rispettivamente dopo tre, quattro e cinque iniezioni intratecali.

     

Multiple sclerosis intra-blood-brain-barrier IgG synthesis: effect of pulse intravenous and intrathecal corticosteroids

Nine severely disabled clinically definite chronic progressive multiple sclerosis (MS) patients who had at least one determination of intra-blood-brain-barrier (BBB) IgG synthesis rate of greater than 7 mg/day (upper limit of normal=3.3) participated in this study. Seven patients were given 1 gram of methylprednisolone sodium succinate (MP) by intravenous infusion over 30 minutes once a day for 3 days. Statistically significant (p<.05) reduction in intra-BBB IgG synthesis (mg/day) was seeen in 4/7 patients, but in only 2 were normal levels of synthesis rate (<3.3 mg/day) attained. Rebound of IgG synthesis to premedication rates occurred within 30 days in 2/4 patients. There was no change in intensity or pattern of cerebrospinal fluid (CSF) oligoclonal IgG bands by isoelectric focusing, immunofixation, and silver staining. A subsequent course of intrathecal methylprednisolone acetate (MPA) (80 mg twice a week for 5 weeks) was given to 5 of the 7 patients and to 2 additional patients not previously treated. In spite of signs of subarachnoid inflammation, a statistically significant depression of intra-BB synthesis, which far exceeded that from the pulse treatment occurred in all 7, including the 2 patients whose intra-BBB IgG synthesis rates were previously resistant to pulse steroid administration. Normal levels of synthesis were rapidly reached in 4/7 patients; however, an IgG synthesis rebound occurred in 3/7 patiens which was just as rapid. One out of 7 patients showed a temporary reduction in the number of cathodic IgG oligoclonal bands in the CSF. Two patients required discontinuation of treatment due to aseptic meningitis in one and progressive weakness in the other. Clinically, these severely afflicted patients with fixed deficits remained unchanged with either treatment protocol. While MPA and ACTH have similar initial effect on the central nervous systems (CNS) inflammatory response in MS, the well documented risk of serious adversities with MPA prohibit its clinical use in MS in its present form.

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Sommario 9 pazienti affetti da una forma avanzata di MS definita come progressiva cronica che avevano almeno una determinazione dell'indice di sintesi delle IgG di barriera emato-encefalica (BBB) superiore a 7 mg. pro die (limite superiore al normale=3.3) hanno partecipato a questa ricerca. A 7 pazienti è stato somministrato 1 g. di metilprednisolone sodio succinato per via endovenosa per la durata di 30 minuti una volta al giormo per 3 giorni. In 4 su 7 pazienti è stata vista una riduzione statisticamente significativa della BBB mentre solo in due pazienti si raggiunsero i normali livelli di sintesi. In 2 su 4 pazienti il ritorno ai valori pre medicazione avvenne in 30 giorni. Non vi è stata variante delle bande oligoglonali IgG studiate col focusing isoelettrico, l'immunofissazione e la colorazione argentica. Successivamente è stato somministrato del metilprednisolone acetato (MPA) per via intratecale alla dose di 80 mg. due volte alla settimana per cinque settimane a 5 dei 7 pazienti e a 2 pazienti addizionali non trattati prima. Nonostante segni di infiammazione subaracnoidea è stata constatata una importante depressione della sintesi di IgG in tutti e 7 i pazienti compresi i due resistenti alla somministrazione per via venosa. Normali livelli di sintesi sono stati raggiunti rapidamente in 4 pazienti su 7 mentre negli altri 3 si ebbe un rapido ritorno ai valori di partenza. 1 dei 7 pazienti ha dimostrato una temporanea riduzione nel numero delle bande oligoclonali IgG catodiche nel liquor. 2 pazienti hanno dovuto sospendere il trattamento per il verificarsi di una meningite asettica in uno e di un progressivo adinamismo nell'altro. Non vi sono state variazioni del quadro clinico. Si conclude che il metilprednisolone e l'ACTH hanno un effetto iniziale simile sulle risposte infiammatorie del sistema nervoso centrale nei casi di MS, ma il ben documentato rischio di seri controeffetti col MPA ne proibisce l'uso clinico nella MS con le modalità descritte.

     

寡克隆区带和IgG指数对多发性硬化的诊断价值

目的　探讨寡克隆区带 (OCB)和IgG指数对多发性硬化 (MS)诊断的敏感性及特异性。方法　收集 4 8例MS、6 8例神经系统炎性疾病 (NID)及 110例非炎性疾病 (NNID) 3组患者的脑脊液 (CSF)和血清标本 ,分别进行OCB的检测 (等电聚焦 )和IgG指数的计算。并对其阳性结果似然比 (PRLR)进行分析。结果　MS组与NID组比较 ,CSF中OCB阳性率和IgG指数异常率的差异均没有显著性 (均P >0 0 5 ) ;但MS组、NID组与NNID组比较 ,差异均有极显著性 (均P <0 0 0 0 1)。MS组CSF中OCB和IgG指数的敏感性分别为 39 6 %、6 0 4 % ;特异性分别为 80 3%、72 1% ;PRLR分别为 2 0、2 2。当用于判断有无IgG鞘内合成时 ,特异性分别为 97 2 %、92 7% ;PRLR分别为 13 5、7 3。结论　CSF中OCB阳性和IgG指数升高强烈提示有中枢神经系统局部IgG合成 ,对MS有一定的辅助诊断价值     

同心圆硬化的临床及影像特点

目的 总结同心圆硬化临床及影像特点.方法 总结临床确诊的同心圆硬化4例,均行头颅影像、腰穿、神经电生理检查.结果 4例发病平均36.5岁.起病为急性或亚急性.临床表现主要是高级皮层功能下降,性格改变,精神症状,如反应迟钝,记忆力、理解力、计算力下降.幻视,幻听.2例有视力下降,2例肢体轻瘫,1例有阵挛发作.激素治疗有效,4例随访无复发.头颅影像除1例外均为多发病灶,病灶早期可见“煎鸡蛋”样改变或年轮样改变.结论 同心圆硬化急性或亚急性起病,以精神障碍和行为异常为主要临床表现.