Several schemes for the diagnosis and clinical classification of multiple sclerosis (MS) have been advanced [l}. The best known is that published by Schumacher et alC31. The criteria for this scheme were established in order to select patients for participation in therapeutic trials, and pertain only to what might be called definite MS. No provision was made for incorporating supportive laboratory data into the diagnostic criteria.As no reliable specific laboratory test for the diagnosis of MS has been discovered, the diagnosis remains a clinical one, and there is still a need for clinical diagnostic criteria. However, several laboratory and clinical procedures have been developed within the last decade which aid greatly in demonstrating neurological dysfunction attributable to lesions, and even the lesions themselves.One problem with the various published diagnostic classifications is their discrepant terminology: what is considered "probable" in one is called "definite" in another. Another problem is that all the proposed schemes require much subjective judgment, a difficulty which cannot be completely overcome but can be diminished by adding to the clinical evaluation the results of laboratory, neuroimaging, neuropsychological, and neurophysiological procedures. Today there is a need for more exact criteria than existed earlier in order to conduct therapeutic trials in multicenter programs, to compare epidemiological surveys, to evaluate new diagnostic procedures, and to estimate the activity of the disease process in MS. Method and ProcedureOn April 26 and 27, 1982, the following persons participated The participants reviewed in detail historical and clinical symptomatology in MS; immunological observations; cerebrospinal fluid (CSF) tests; neurophysiological procedures including visual, brainstern auditory, trigeminal, and somatosensory evoked potential measurements; the evoked blink reflex; a variety of physiological and psychophysiological procedures; neuropsychological assessment; tissue imaging procedures such as computer assisted tomography (CT scanning) and nuclear magnetic resonance (NMR); and urological studies of bladder, bowel, and sexual dysfunction. This re-~
Epidemiological studies of multiple sclerosis (MS) have shown the importance of genetic susceptibility factors that are modified by as yet unknown environmental influences. The often-cited interrelation between prevalence and latitude is no longer viable, with the important but unexplained exception of Australia and New Zealand. The standardization and increasing use of uniform diagnostic criteria lend new credibility to the results of MS surveys. More precise criteria are offered for symptoms of disease onset, Devic's syndrome, and progressive disease. The role of magnetic resonance imaging in the diagnosis of MS, and the pitfalls of its abuse, are reviewed. Reports of epidemics of MS are examined and discarded because they are based on the biologically meaningless date of diagnosis or that of onset, rather than when MS was probably acquired, that is, before puberty. The concept of onset-adjusted prevalence suggests that patients who are symptomatic should be included retrospectively in epidemiological surveys, even before they have been formally diagnosed as having MS, but individuals who were symptomatic before moving to a study area should not. The importance of ethnic homogeneity of patients and control subjects cannot be overemphasized in prevalence and risk factor studies. In the latter, close attention must be paid to biological plausibility and to prudent statistical interpretation. The hypothesis of the MS trait describes a systemic, asymptomatic condition that does not affect the nervous system, and may explain the low concordance of the disease in monozygotic twins.
Objectives: To report the clinical features and outcome of 24 Brazilian patients with optic neuromyelitis syndrome (ONM); discuss the underlying pathological events associated with the ONM syndrome; review the nosological situation of ONM in the group of inflammatory and demyelinating diseases of the central nervous system. Patients and Methods: Patients with ONM treated at the Hospital da Lagoa, Rio de Janeiro were studied. Demographic, clinical, magnetic resonance imaging, cerebrospinal fluid, and pathological data were analysed. Results: The study consisted of 20 women, four men of whom 10 were white and 14 Afro-Brazilians. Clinical course was recurrent in 22 cases and monophasic in two. Neurological manifestations at inclusion were: sensory impairment (66%), bilateral (41.6%) or unilateral blindness (20.8%), paraplegia or quadriplegia (37.5%). The EDSS was moderate/severe in 70.8%. The underlying pathological events were respectively pulmonary tuberculosis and upper respiratory infection in the two monophasic cases; in the 22 recurrent ONM patients: pulmonary tuberculosis (3), neurocysticercosis (1), polyarteritis nodosa (1), antinuclear antibody and rheumatoid factor (1), antiphospholipid antibody primary syndrome (1), diabetes mellitus (1), hypothyroidism (1), and amenorrhea-galactorrhea (4). Normal cerebrospinal fluid was found in 52% and an inflammatory profile in 48%. Only four recurrent ONM white patients had brain and spinal cord magnetic resonance imaging and cerebrospinal fluid findings compatible with the diagnosis of multiple sclerosis. Large lesions were seen in 62% of spinal magnetic resonance images. Six of 12 recurrent ONM Afro-Brazilian died. There were no statistical differences in the demographic data of the two ethnic groups. Afro-Brazilians were significantly more severely impaired and had a higher mortality rate than the white patients. Conclusion: These cases were classified as follows: two monophasic acute disseminated encephalomyelitis; one recurrent disseminated encephalomyelitis; three recurrent ONM associated with Hughes syndrome, autoantibodies and polyarteritis nodosa; six recurrent ONM with endocrinopathies; and finally, four muliple sclerosis cases. The remaining cases were not associated with any other condition. It would seem clear that ONM is a syndrome rather than a single disease.
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