Epidemiological aspects of multiple sclerosis: a comparative study of four centres in Europe

Epidemiological data of 931 patients with definite and probable multiple sclerosis in four areas in Europe (Groningen, G?ttingen, Darmstadt and Baranya) were collected and compared. The same criteria and scoring methods were used. Clear differences between the areas appeared: a high percentage of severely disabled patients were encountered in the northern part (Groningen) and a high percentage of signs and symptoms related to supraspinal lesions in brainstem, cerebellum and hemispheres were found in the south east (Hungary). In G?ttingen, in the central part of the total area, a relatively higher percentage of younger MS patients with minimal disability were found. However, it still has to be determined to which extent these differences are real or caused by information bias.     

A clinical comparative study of multiple sclerosis and neuro-Behçet's syndrome.

Clinical comparisons were made between Japanese patients with multiple sclerosis (66 cases) and neuro-Behçet's syndrome (23 cases). Those with neuro-Behçet showed marked male predominance, while those with multiple sclerosis showed slight female preponderance. Both showed encephalomyelopathy disseminated in time and space. Patients with multiple sclerosis, however, showed a more polyphasic course, whilst those with neuro-Behçet showed a more progressive one. In multiple sclerosis optic neuritis, acute transverse myelitis, painful tonic seizures, mental disturbance and internuclear ophthalmoplegia were common. On the other hand, in neuro-Behçet the main neurological manifestation was progressive pseudobulbar palsy. Serum and CSF showed more inflammatory changes in neuro-Behçet than in multiple sclerosis. Clinical estimation suggested that in multiple sclerosis the main lesions were in the optic nerve, tegmentum of the brain stem and spinal cord, whereas in neuro-Behçet they were in the basal parts of the brain stem.     

Some recent advances in the clinical aspects of multiple sclerosis

Tallis R.C. (1980) Neuropathology and Applied Neurobiology 6,325–335
Annotation. Some recent advances in the clinical aspects of multiple sclerosis Recent work on the clinical aspects of multiple sclerosis is reviewed with particular regard to symptomatology : New approaches to clinical symptoms and the identification of more subtle impairments are illustrated by recent studies of visual function in M.S. patients; pathophysiology : It is now widely appreciated that the dysfunction observed in patients is not determined solely by histologically demonstrable demyelination. The function of the demye-linated neuron is highly variable, being dependent upon factors which may change from day to day. Recent ideas about 'neuro-electric blocking factors' and other factors that may influence demyelinated neurons and hence symptoms are discussed; diagnosis : tests on C.S.F., electro-physiological and psychophysiological tests and computer tomography as aids to diagnosis and the controversy over 'specific' blood tests are reviewed; course and prognosis : Long term follow-up studies confirm that, in a significant proportion of cases, the course of M.S. may be benign and have identified some early prognostic indices; Treatment : The results of trials of symptomatic (spinal cord stimulation) and would-be curative therapies (such as dietary supplementation with poly-unsaturated fatty acids and immunosuppression) are briefly discussed.     

A clinical patho-anatomical study of clinically silent multiple sclerosis

This is the first study on the frequency, size, number, and location of plaques in clinically silent MS. Among the present 18 patients in whom MS was unexpectedly diagnosed at autopsy, it had been clinically silent in 13. An estimate of the prevalence of silent MS is about 25% of that diagnosed in vivo. In the silent group, the MS plaques were located mainly in the periventricular areas, and this may explain the silent nature of the disease.     

Personality aspects in multiple sclerosis

To test the claim that peculiar personality bias is detectable in multiple sclerosis (MS) we used the Szondi test to investigate the psychodynamic aspects of 110 MS patients in comparison with 200 healthy subjects. MS patients appeared to have a greater need for love in a passive form than normal people, rigid defense mechanisms, difficulty in resolving their inner conflicts either by sublimation or by internalization of satisfactory new emotional experiences, feelings of autoaggressiveness, and many symptoms of depression. Some of these aspects correlate with the severity of the disease, others seem to date back to early childhood as peculiar personality patterns. An investigation of childhood events in 110 controls confirmed that MS patients had had many more unhappy experiences in childhood than might commonly be expected. Further, the oft-reported psychiatric troubles preceding MS clinical onset suggest that at least in some MS patients there are specific gaps in personality structure dating back to early phases of their development

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Sommario Con lo scopo di verificare se esistono delle peculiarità psicologiche nei pazienti con sclerosi multipla (SM), sono stati sottoposti al test di Szondi 110 casi con diagnosi certa e i risultati sono stati analizzati in rapporto a quelli di 200 persone sane di controllo. Nei soggetti con SM sono stati riscontrati un bisogno affettivo passivo più elevato che nei normali, meccanismi di difesa rigidi, difficoltà a tirarsi fuori dai propri conflitti intrapsichici sia attraverso la sublimazione che mediante la introiezione di nuove esperienze affettive, sentimenti autoaggressivi e una forte componente depressiva. Alcuni di questi aspetti sono risultati correlabili con la gravità della malattia, altri invece sono apparsi indipendenti e, dato che i soggetti con SM avevano avuto più esperienze frustranti nella prima infanzia rispetto alle persone di controllo, potrebbero essere indizio di screzi particolari della personalità. Ciò darebbe tra l'altro ragione delle reazioni psicopatologiche che spesso precedono o accompagnano la manifestazione clinica della malattia.

     

Psychological aspects of multiple sclerosis

A significant incidence and prevalence of psychological disorders in multiple sclerosis (MS) has been reported. Their underlying mechanisms and the extent to which they are reactive to psychosocial factors or symptoms of the pathological process itself, remain unclear. Depression is the predominant psychological disturbance with lifetime prevalence around 50% and annual prevalence of 20%. Depression is commoner during relapses, may exacerbate fatigue and cognitive dysfunction and no firm evidence exists of its induction by interferon; instead, treating depression improves adherence to disease-modifying drugs. Anxiety is also frequent, occurs in newly diagnosed patients, and its co-morbidity with depression has been suggested to increase the rate of suicidal ideation. The relationship between stress and MS is an attractive issue because some studies pointed to an association between stressful life-events and MS onset/relapses; however, the evidence supporting this hypothesis is not conclusive so far. Other psychiatric illnesses, as bipolar affective disorder, pathological laughing and crying or psychosis occur less frequently in MS. Therapeutic strategies include psychotherapy, cognitive behavioural therapy, strengthen of coping, and specific medications. The "art" of the MS team in providing the best individualized care is emphasized, aiming to reduce the burden of the disease and improve the patients' quality of life.     

Psychiatric aspects of multiple sclerosis

A patient who presented with depressive and hysterical symptoms of several years' duration and who proved to have multiple sclerosis is presented. Two further cases whose initial symptoms of dissociation and major depression were prodromes of multiple sclerosis are also presented. A discussion of the factors leading to the correct diagnosis in these cases and reference to relevant literature on psychiatric presentations of multiple sclerosis are presented to illustrate the need for psychiatrists' awareness of multiple sclerosis as a causative factor in patients with a wide variety of psychiatric syndromes.     

Cyclophosphamide in chronic progressive multiple sclerosis: a comparative study

No effective treatment is presently available for progressive multiple sclerosis (MS). Cyclophosphamide (CFX), a cytotoxic immunosuppressive drug widely used in systemic dysimmune diseases, has been proposed for the treatment of multiple sclerosis with different schedules and controversial results. To evaluate the safety and clinical efficacy of CFX, we compared three different treatment schedules in patients with progressive MS: induction followed by bimonthly boosters for one year (17 patients); bimonthly boosters for one year without previous induction (15 patients); and monthly boosters for one year (21 patients). Survival analysis showed that the percentage of stable patients was significantly higher in the first and third treatment schedule groups. Myelotoxicity occurred in patients treated with induction and boosters (Group A). A high incidence of broncopneumonia was observed in patients undergoing the second treatment schedule (Group B). No major effects were observed in patients treated with monthly boosters (Group C). Response to treatment was limited to secondary progressive form. This study suggests that monthly treatment with CFX might be safely administered in progressive MS patients; its clinical efficacy must be confirmed by an appropriately designed clinical trial.     

Psychiatric aspects of multiple sclerosis

A consecutive sample of 50 patients with a diagnosis of definite multiple sclerosis was evaluated by means of the Clinical Interview Schedule (CIS) and other psychopathological instruments. A global prevalence of psychopathology of 54% was found, with the prevalence of depression being 22%. Moreover, 46% of the sample presented signs indicative of neuropsychological deterioration in the Benton Visual Retention Test. The association between the presence of psychopathology and the social and neurological characteristics of the sample was also investigated, with results of low significance.     

Vascular aspects of multiple sclerosis

Three types of vascular dysfunction have been described in multiple sclerosis (MS). First, findings from epidemiological studies suggest that patients with MS have a higher risk for ischaemic stroke than people who do not have MS. The underlying mechanism is unknown, but might involve endothelial dysfunction secondary to inflammatory disease activity and increased plasma homocysteine concentrations. Second, patients with MS have global cerebral hypoperfusion, which might predispose them to the development of ischaemic stroke. The widespread decrease in perfusion in normal-appearing white matter and grey matter in MS seems not to be secondary to axonal degeneration, but might be a result of reduced axonal activity, reduced astrocyte energy metabolism, and perhaps increased blood concentrations of endothelin-1. Data suggest that a subtype of focal MS lesions might have an ischaemic origin, and there seems to be a link between reduced white matter perfusion and cognitive dysfunction in MS. Third, the pathology of MS might be the consequence of a chronic state of impaired venous drainage from the CNS, for which the term chronic cerebrospinal venous insufficiency (CCSVI) has been coined. A number of recent vascular studies do not support the CCSVI theory, but some elements of CCSVI might be explained by slower cerebral venous blood flow secondary to the reduced cerebral perfusion in patients with MS compared with healthy individuals.     

Genetic aspects of multiple sclerosis

A large body of immunological, epidemiological, and genetic data indicate that tissue injury in multiple sclerosis (MS) results from an abnormal immune response to one or more myelin antigens that develops in genetically susceptible individuals after exposure to an as-yet undefined causal agent. A genetic component in MS is indicated by an increased relative risk to siblings compared to the general population, and an increased concordance rate in monozygotic compared to dizygotic twins. The past few years have seen real progress in defining the genetic basis of MS setting the stage for new approaches for the final characterization of the genes involved in MS susceptibility and pathogenesis. Whole genome screens conducted in different populations identified discrete chromosomal regions potentially harboring MS susceptibility genes, however, with the exception of the Major Histocompatibility Complex (MHC) on 6p21, no single locus generated overwhelming evidence of linkage. These results suggest a complex genetic etiology, including multiple genes of small to moderate effect and probable genetic heterogeneity. The identification and characterization of MS susceptibility genes and their correlation with disease phenotypes is likely to define the basic etiology of the disease, improve risk assessment, and influence therapeutics.     

A comparative study of Japanese multiple sclerosis patients with and without oligoclonal IgG bands

The cerebrospinal fluid oligodonal IgG bands (OB) are less frequently observed in Japanese multiple sclerosis (MS) patients compared with Caucasian patients. We studied 40 consecutive Japanese MS patients to investigate the differences in the clinical and magnetic resonance imaging (MRI) features of MS between OB-positive patients and OB-negative ones. Among the 40 patients, 22 (55%) patients were OB-positive by either agarose gel electrophoresis (AGE) or isoelectric focusing (IEF), and 18 (45%) patients were OB-negative by both AGE and IEF. There were differences between the two groups only in the clincal forms of MS, but not in terms of gender, onset age, disease duration, or disease severity. In the OB-negative group, nine (50%) of the patients had the optic-spinal form of MS (OS-MS), but only one patient (4.5%) in the OB-positive group had OS-MS. Although most OB-positive patients showed brain MRI lesions typical of MS, 13 (72%) of the OB-negative patients showed no or few brain MRI lesions and the rest of the OB-negative patients showed atypical MS lesions, such as diffuse white matter lesions or large ring-enhanced lesions. Our results suggest that the majority of OB-negative Japanese MS patents show either no or few brain MRI lesions or atypical brain MRI lesions.     

Epidemiology of multiple sclerosis in Arabs in Kuwait: A comparative study between Kuwaitis and Palestinians

On December 31, 1988 there were 201 registered multiple sclerosis patients in Kuwait, an overall prevalence rate (PR) of 10.2 per 100000; among them were 186 Arabs, of whom 72 were Palestinians and 51 Kuwaitis. Comparison of these two subgroups, who had a similar age distribution revealed that the disease was2 1/2 times more frequent among Palestinians (PR 23.8/100 000) than among Kuwaitis (PR 9.5/100 000). Palestinians also showed significant differences from Kuwaitis in eye color, blood group distribution and HLA-DR and HLA-DQW epitopes frequency. This suggests that genetic rather than environmental factors might be the underlying cause for the high susceptibility to develop MS among Arabs originating from the Eastern Mediterranean basin.