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Biomedical subjects

Vesna V Brinar

Publications and source records attributed to Vesna V Brinar.

16 recordsLinked to original sources

The differential diagnosis of acute transverse myelitis.

The clinical and paraclinical characteristics of acute transverse myelitis (ATM) were analyzed in 31 patients. In some patients there was clinical evidence of complete transection, in others of only partial lesions. Magnetic resonance imaging (MRI) in the acute phase in the first group was normal, but showed cord atrophy subsequently. It is probable that the clinical picture was due to parenchymatous neuronal lesions, analogous to those of axonal polyneuropathy. In the patients with incomplete transverse lesions, the most common finding was demyelination. In the patients with circumscribed demyelinating lesions, the symptoms and MRI were suggestive of clinically isolated syndromes (CIS) predictive of multiple sclerosis (MS). Extensive demyelination was indicative of acute disseminated encephalomyelitis (ADEM) due to hyperergic vasculopathy or various forms of chronic vasculitis. In two patients with variable clinical symptoms, a vascular malformation was the cause of the clinical presentation, and in one patient demyelination was due to the disc compression.

Adult↗

Psychiatric manifestations of multiple sclerosis and acute disseminated encephalomyelitis.

It is unusual for acute disseminated encephalomyelitis and multiple sclerosis to present as purely psychiatric disorders. We report five patients with such demyelinating diseases and symptoms of psychosis, depression or anxiety. The importance of excluding demyelination as the basis for these psychiatric disturbances is emphasized, especially in the presence of unexplained neurologic findings. The possible relationship between psychiatric symptoms and demyelinating disorders is explored.

Adult↗

CNS demyelination in autoimmune diseases.

Autoimmune diseases represent a diverse group of disorders that have generally of unknown etiology and poorly understood pathogenesis. They may be organ-specific or systemic, giving rise to overlapping syndromes; more than one autoimmune disease may occur in the same patient. Numerous case reports have documented that multiple sclerosis (MS) may be present concurrently with other autoimmune diseases, most commonly rheumatoid arthritis, autoimmune thyroid disease, type I diabetes mellitus and pernicious anemia. Case reports of disseminated encephalomyelitis (DEM) coincidental with other autoimmune diseases are rare. Many of systemic autoimmune diseases cause central nervous system (CNS) demyelination and are frequently then diagnosed as MS, whereas they often are instances of DEM, the result of vascular, granulomatous or postinfectious manifestations. We have reviewed 15 patients with autoimmune diseases and CNS demyelination in order to determine the nature of the demyelinating process.

Adolescent↗

Diagnostic criteria for multiple sclerosis: an historical review.

Starting with Charcot, diagnostic criteria for multiple sclerosis (MS) have evolved to reflect advances in our understanding of the disease and the development of new diagnostic techniques, and from purely clinical considerations to increasing dependency upon imaging of the central nervous system. The MS diagnostic process was revolutionized by the 1981 introduction of magnetic resonance imaging (MRI), but the increasing reliance upon this technique has led to a surge in erroneous diagnoses, mostly because of the failure to distinguish between MS and disseminated encephalomyelitis (DEM), as well as mounting disregard for the data obtained from the traditional history and physical examination. The most recent scheme of McDonald et al. incorporated quantitative MRI criteria of dubious origin and reliability, but failed to provide qualitative, illustrative ones that would help differentiate between MS and DEM. The choice will have to be made by the neurological community between basing the diagnosis of MS on the MRI alone, or to use it as one aspect of a comprehensive clinical diagnostic algorithm. There will never be a substitute for the experienced and astute clinician's 'feel' for the patient.

Brain↗

The nature of multiple sclerosis.

Multiple sclerosis (MS) has recently been classified according to its clinical course. Despite relapses and remissions, its course is invariably progressive, and the observed progression from the remitting-relapsing to the secondary progressive form represents the accumulation of permanent damage to the nervous system. Discussions of the nomenclatural position of Schilder's, Marburg's, and Baló's diseases, ignore the fact that the unique, pathognomonic, sharp-edged plaque of MS, is also the pathologic end-result in the three variants. Devic's disease or neuromyelitis optica (NMO) is quite different and with some exceptions, is a particular form of disseminated encephalomyelitis (DEM). There is no evidence that the 'oriental form of MS' is anything but NMO. The suggestion that MS and DEM are variants of the same condition is contradicted by the fact that the pathological characteristics of the two are quite different. While it is probable that the two share aspects of pathogenesis, the patients differ because of their genetic endowment. This was dramatically demonstrated in a group of Japanese patients who died after anti-rabies vaccination and were found to have the typical sharp-edged lesions of MS. The genetic determinant was also crucial in the marmoset in which EAE uniquely resulted in a chronic relapsing-remitting (RR) disease characterized by the classic sharp-edged lesions of MS. The question 'ADEM: distinct disease or part of the MS spectrum?' can be answered with a resounding no. A new classification is proposed separating the different forms of MS from the various types of DEM.

Brain↗

Diagnostic and therapeutic dilemmas.

Close blood relatives of MS patients have MRI changes suggestive of MS despite the absence of any symptoms. They have also been seen when an MRI is obtained for various reasons in unrelated persons. This raises questions regarding their clinical significance, if any, since typical MS plaques have been found at autopsy in eloquent parts of the nervous system in completely asymptomatic individuals. Do these people have MS and should immunomodulatory treatment be considered? Because they are not ill according to various dictionary definitions, no to both. A higher rate of MS in close blood relatives would be expected due to the known genetic susceptibility, but suggestive MRI changes are quite uncommon, even in unaffected twins. Finally, a review of the four published studies of MS lesions unexpectedly found at autopsy in neurologically asymptomatic persons revealed a prevalence of the disease of 0.1%, the same as the estimated prevalence of clinical MS in the United States.

Adult↗

Sudden onset aphasic hemiplegia: an unusual manifestation of disseminated encephalomyelitis.

The association of the sudden onset of aphasia with hemiplegia, hemisenosry defect, and facial palsy, with MRI evidence of white matter lesions, requires differentiation between multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM). We have observed eight patients with such a syndrome, all of whom were originally diagnosed as multiple sclerosis, but who, on closer examination, turned out to be instances of disseminated encephalomyelitis. The patterns of demyelination seen in T2-weighted MRI are quite different in both conditions. In two of our patients, MRI reverted to normal after the treatment; in others, the images remained unchanged. A review of the reported cases of multiple sclerosis presenting with the acute onset of aphasia, reveals that the majority of them are, in reality, instances of acute disseminated encephalomyelitis with a much better prognosis. Most of these cases are monophasic and immunomodulatory treatment is inappropriate.

Adolescent↗

Non-MS recurrent demyelinating diseases.

The introduction of MRI has shown that the acute, recurrent (R), and multiphasic (M) forms of disseminated encephalomyelitis (DEM) are more common than suspected in adults, and that their MR images are sufficiently characteristic in most instances to make differentiation from multiple sclerosis (MS) possible. In addition, a number of clinical features of DEM are rarely seen in MS: fever, malaise, nausea, vomiting, positional vertigo, convulsions, aphasia, meningism, bilateral optic neuritis, and CSF leukocytosis and elevated protein. CSF oligoclonal bands are usually absent. It is remarkable that confusion between R- and MDEM and MS persists despite the numerous published reports on recurrent DEM dating back 70 years, many illustrating the characteristic MRIs. There are many case reports of DEM erroneously diagnosed as MS, Schilder's, Marburg's, Devic's, and Baló's disease, and, in particular brain tumors. It is probable that acute DEM is occasionally mistaken for a clinically isolated symptom of MS. Possible mechanisms for recurrence include localization at the site of a previous injury to the nervous system, or by the phenomenon of molecular mimicry. The importance of differentiating R- and MDEM from MS is greater today due to the recommendation that immunodulatory treatment be initiated in patients with a clinically isolated syndrome, or when the occurrence of a second clinical episode establishes the diagnosis of MS.

Adult↗

Cerebral demyelination in Wegener's granulomatosis.

A 38-year-old woman with a history of a granulomatous lesion of the nose, developed blurred vision, ataxic gait, and spastic tetraparesis. The presence of demyelination on the brain MRI led to the diagnosis of cerebral demyelination associated with Wegener's granulomatosis. Pulse cyclophosphamide administration resulted in some clinical of improvement of her condition. Demyelinating lesions seen in Wegener's have been ascribed to multiple sclerosis, but in this case, they are much more reminiscent of disseminated encephalomyelitis (DEM). The immunological challenge of the underlying disease, may, in the genetically susceptible person, presumably trigger the appearance of MS lesions. Wegener's granulomatosis must be considered in the differential diagnosis of MS.

Adult↗

Epilepsy and multiple sclerosis.

A review of 29 published clinical series of adult patients who had epileptic seizures and multiple sclerosis (MS) yielded a prevalence of 2.3%, about three to six times that in the general adult population. The probable anatomic basis for the seizures is areas of inflammation, edema, and/or demyelination in the cerebral cortex and the juxtacortical white matter generated by a mechanism that is not completely understood; the fact that these plaques are very common suggests that other factors must operate in view of the rarity of seizures in MS. Seizures have been observed before and presumably marking the clinical onset of the disease, and during acute bouts. In some instances convulsions appear to be the only manifestations of an attack of MS, but there is no general acceptance of seizures as the first and only symptoms of the disease. The coincidental occurrence of both diseases, the nonspecific triggering effect of MS on latent epilepsy, and actual causation by MS are all possible explanations, but the last named is, in our opinion, extremely unusual.

Brain↗

Highlights of the Second International Conference on MS, May 2003, Dubrovnik, Croatia.

The conference was organized by Professors Vesna Brinar of the Faculty of Medicine, University of Zagreb, Croatia and Charles Poser of the Harvard Medical School, Boston, USA. It was attended by almost 200 neurologists from as far afield as South Africa and Russia. The major topics to be explored were: the natural history of multiple sclerosis (MS); development of diagnostic criteria; genetics with special emphasis on the 'MS trait'; pathogenesis with emphasis on repair; nomenclature of MS and its 'variants'; differentiation between MS and disseminated encephalomyelitis; diagnostic and therapeutic dilemmas resulting from MRI abnormalities in asymptomatic people; various treatments; and a general critique of therapeutic trials.

Clinical Trials as Topic↗