Clinical and pathological features of Devic’s neuromyelitis optica and multiple sclerosis in Chinese patients

BACKGROUND:Devic's neuromyelitis optica (DNMO) and multiple sclerosis in Asian populations have been considered to be the same disease. However, there is an increasing number of studies suggesting that DNMO and multiple sclerosis are different diseases.OBJECTIVE:Little information is available regarding comparisons of DNMO patients between China and other countries, as well as clinical manifestations of Chinese patients with DNMO and multiple sclerosis. The present study performed a multi-center, pathological, retrospective analysis.DESIGN, TIME AND SETTING:A retrospective analysis of clinical data from seven patients with DNMO diagnosed between 1957 and 1998.PARTICIPANTS:Data from Chinese DNMO patients was provided by the Shanghai Second Medical University, Sun Yat-sen University of Medical Sciences and the First Affiliated Hospital of Harbin Medical University in China.METHODS:Clinical and pathological data from Chinese patients with DNMO were retrospectively analyzed. The clinical characteristics of DNMO were compared between Chinese and Caucasian patients. In addition, clinical and pathological differences between DNMO and multiple sclerosis Chinese patients were compared.MAIN OUTCOME MEASURES:Clinical and pathological features of Chinese patients with DNMO.RESULTS:All seven Chinese patients with DNMO exhibited abrupt onset of vision disturbance, with a disease course of 3 clays to 9 years. DNMO recurred in two of the patients. Demyelinating lesions were observed in all patients, with necrotic lesions and gitter cells in five patients, collagenous hyperplasia in one patient, and perivascular inflammatory cell infiltration in six patients. Comparison between Chinese and Caucasian DNMO patients revealed no significant differences in age at onset, clinical onset, duration, or interval between optic neuritis and myelitis. Compared with Chinese multiple sclerosis patients, Chinese DNMO patients presented with fewer recurrences, higher occurrence of necrosis, perivascular inflammatory cell infiltration and gitter cells, and a lower occurrence of collagenous hyperplasia.CONCLUSION:There was no difference in DNMO clinical features between Chinese and Caucasian patients. However, the clinical and pathological features of DNMO were different compared with multiple sclerosis in Chinese patients. Results suggested that the characteristics of DNMO in Chinese patients were significantly different than multiple sclerosis.     

Clinical studies on multiple sclerosis

Probable and possible MS cases with a debut during the years 1950–1964 within the city of Gothenburg were identified (312 cases). This corresponds to an incidence of 5.3 per 100,000. The material was stratified according to diagnostic probability into three categories. For the final analysis cases with the lowest diagnostic probability were omitted (about 9 %). The follow-up was completed during 1977, i.e. 13–27 years from onset. A longitudinal analysis of each case was based upon a mixed prospective/retrospective study in which the authors personally examined the majority of the cases during most of the years. The female/male ratio was 1.5–1.6. The mortality rate was higher for males due in part to an earlier progressive development of multiple sclerosis and in part to a higher trend for acquisition of other mortal diseases. Bouts, as the first manifestation of the disease, were more frequent in young ages than in old, and more frequent among females than in males. The average bout frequency decreased significantly with the duration of the disease as well as with the age of onset. The opposite trend was characteristic for development of a progressive course. Among symptoms at onset, those indicating lesions of long sensory and/or motor tracts (particularly the sensory) dominated. Such initial symptoms were seen in 30–40 % of the younger patients and 70 % of the older patients. The rest was divided between cases with optic nerve lesions (20–30 % among younger, 12–15 % among older patients) and brain stem lesions (approx. 25 % among younger, 10–19 % among older patients). The occurrence of various symptoms during the first two decades of the disease was also analyzed and the pattern of symptoms presented graphically. These results will be treated further in subsequent studies.     

Neuromyelitis optica (Devic's syndrome): not always multiple sclerosis

A patient is presented with clinical signs and symptoms of neuromyelitis optica (Devic's syndrome). The clinical findings, the cerebrospinal fluid findings and the Magnetic Resonance Imaging findings in our patient demonstrate that neuromyelitis optica is not always a form of Multiple Sclerosis. It can also be a manifestation of other demyelinating diseases, for example Disseminated Encephalomyelitis.     

Case of multiple sclerosis with Devic's syndrome and secondary cerebellar signs]

The authors describe a 43-year-old woman treated for multiple sclerosis during 11 years in whom at the end of her life tetraplegia, bulbar syndrome and cerebellar syndrome developed. Postmortem examination of the brain disclosed changes compatible with the diagnosis of multiple sclerosis, diffuse sclerosis and neuromyelitis optica. The variability of the demyelinating process is discussed. In this case this variability was responsible for successive appearance of various nosological entities depending on the "balance of power" between the factors damaging myelin and the resistance of the organism.     

Comparative epidemiological studies of multiple sclerosis in South Africa and Japan.

Evidence is presented pointing to an increased incidence of multiple sclerosis having taken place in South Africa during the period 1964 to 1970 and in Tokyo, Japan, during the period 1966 to 1972. There is a possibility that these changes in incidence point to the introduction during the years immediately after the second world war, of an infective element-probably from a high-risk area-to Tokyo and South Africa. If this is so, it lends support to the theory of an infective basis for multiple sclerosis and that the suspected infection in prepubertal susceptibles may produce the symptoms of multiple sclerosis years later. Other factors relevant to multiple sclerosis such as environmental changes, improved diagnostic techniques, and susceptibility are discussed.     

Oligoclonal bands in Devic's neuromyelitis optica and multiple sclerosis: differences in repeated cerebrospinal fluid examinations

We studied repeated cerebrospinal fluids of patients with Devic's neuromyelitis optica (NMO) and multiple sclerosis (MS). Variations of oligoclonal bands (OBs) had opposite trends in the two groups. In MS, OBs were detected in 399 of 411 patients (97%) and never disappeared. In NMO, OBs were detected in three of 11 patients (27%) and always disappeared. The hypothesis that NMO and MS follow distinct pathogenetic pathways is supported by our findings, which can be useful for the differentiation of NMO from MS.     

Correlation of immunological studies and disease progression in chronic progressive multiple sclerosis

Thirty untreated patients with clinically definite chronic progressive multiple sclerosis were matched with 10 patients with clinically stable definite multiple sclerosis and 16 patients with other neurological diseases. A group of 12 normal control (NC) volunteers was matched to these groups. All patients with chronic progressive multiple sclerosis and normal control subjects were analyzed for the concanavalin A suppressor assay, mitogen stimulation, and phenotyping of peripheral blood mononuclear cells. In addition, serum was analyzed for interleukin-2 levels. Results of mitogen stimulation studies did not distinguish the groups. Concanavalin A-induced suppression was significantly decreased in the patients with chronic progressive multiple sclerosis (p less than 0.01). Phenotyping of fresh cells showed an elevated CD4: CD8 ratio in the patients with chronic progressive multiple sclerosis. Neither phenotyping nor concanavalin A-induced suppression correlated with or predicted the degree of disability, but the serum levels of interleukin-2 correlated inversely with disability (p less than 0.01) and directly with a poor prognosis after 18 months of observation (p less than 0.05). Serum levels of interleukin-2 decreased as the disease progressed.     

Association studies in multiple sclerosis

In the search for candidate loci having a role in susceptibility to common diseases such as multiple sclerosis (MS), studies often look for an ‘association’ between one allele at a putative candidate susceptibility locus and the disease, e.g. MS. However, reproducibility of results from association studies has been difficult. In MS, despite numerous studies, association has been reproducibly confirmed only for the major histocompatibility complex, the MHC. The present paper is designed to review the allelic associations which have been reported in MS and suggest possible reasons for the difficulties in replicating these studies. We also outline some suggestions for improving the validity of future association studies in MS and in other complex traits.     

Clinical course and prognosis of 27 patients with childhood onset multiple sclerosis in Japan

To investigate whether outcomes of childhood onset multiple sclerosis (MS) have changed or not, the clinical courses of childhood onset MS in 27 patients at our hospital and those reported by Fukuyama in 1991 were compared. The ratios of our patients with poor prognosis in walking and vision were decreased. Widespread use of high-dose corticosteroid therapy and interferon therapy may be one cause of the low frequency of severe sequelae in our study.     

Seasonal variation of multiple sclerosis exacerbations in Japan

Abstract. Several reports have described the seasonal variation of multiple sclerosis (MS) attacks in the European countries and in the US. Some have insisted that attacks occurred more frequently in winter or spring. We investigated the possibility of a seasonal variation in the frequency of MS attacks among patients in Japan. A total of 172 MS exacerbations in 34 MS patients were analyzed retrospectively. Attacks were divided into two groups: opticospinal type and brain type. The 12 months of the year were assigned to 6 groups based on average monthly temperature. Of the 172 MS exacerbations, 123 were opticospinal type and 49 were brain type of attacks. The total number of attacks was significantly more frequent in the warmest (July and August) and coldest (January and February) months. The heat of summer in warmer, low latitude areas may be a risk factor for MS attacks.