Challenges in multiple sclerosis; how to define occurence of progression.

The challenges in MS are related to number of controversies in various aspects of disease but the relationship between relapses and disability progression, or aspects of MS as an inflammatory and/or neurodegenerative disease are extremely important because of its implications on prognosis and therapy of MS. MS was classically regarded as white matter inflammatory disease, while disability progression, brain and spinal cord atrophy were regarded as a consequence of global inflammation of NAWM and secondary involvement of grey matter. More recent histopathology studies, but also new, modern MRI techniques changed this view in MS as a prominent grey and white matter disease. Inflammatory demyelination of grey matter occurs early in MS sometimes even before occurrence of white matter lesions. Inspite of early therapy of MS with immunomodulatory drugs disability progression and neurodegeneration are still important and common part of MS pathogenesis. This indicate that treatment is not adequate to the predicted severity of MS, or perhaps to the basic pathogenetic mechanisms in MS. Beside acute clinical symptoms, conclusions about the severity of the disease are reflection of MRI sensitivity to detect focal WM lesions and insensitivity to detect grey matter lesions which correlate better with clinical symptoms. All presented studies and evaluations point to the necessity of changing the established diagnostic evaluation and treatment in MS. At the earliest stage of MS as well as in follow up of disease it would be necessary to apply a new MRI techniques more available for clinical practice such as DIR brain MR imaging at 3T because of their sensitivity to detect grey matter lesions. In patient with present cortical lesions even in earliest stages of MS depending on severity of grey matter involvement more efficacious therapy like second or even third line therapy should start.

Disseminated encephalomyelitis and multiple sclerosis: two different diseases - a critical review.

The practice of initiating immunomodulatory treatment immediately after a clinically isolated syndrome (CIS) suggestive of multiple sclerosis (MS) emphasizes the need to distinguish between disseminated encephalomyelitis (DEM) and MS. Their clinical, genetic, imaging, and histopathological characteristics establish that they are distinct disease entities. Acute and recurrent DEM are more common in children, but also occur in adults. DEM is polysymptomatic and includes signs and symptoms rarely encountered in MS, such as fever, alterations of the state of consciousness, cognitive and aphasic symptoms, and meningism. Cerebrospinal oligoclonal bands are rare. Magnetic resonance imaging (MRI) is the best means of distinguishing between DEM and MS. In the former, the lesion load is heavy, thalamus or basal ganglia are often affected, and early in the disease most of the lesions are usually larger than those of MS and enhance with gadolinium. The MRI spinal cord lesions are longer than three vertebral segments, and define neuromyelitis optica (NMO). Antibodies against aquaporin-4 are present in some NMO, but are also found in cases of MS and DEM. Most NMO are forms of DEM, not MS, and are identical with the 'Oriental' or 'optico-spinal' form of MS.

Avellis syndrome due to borreliosis.

Avellis syndrome is a rare form of alternating hemiparesis that is usually because of atherosclerosis. We report a 67-year-old man who developed paresthesiae of the left arm, dysphagia and dysphonia. The clinical picture, magnetic resonance imaging and cerebrospinal fluid findings were consistent with Avellis syndrome caused by brain stem arteritis because of late stage Borrelia burgdorferi infection, an extremely unusual aetiology for Avellis syndrome; this may well be the first such instance. It may be unrecognized in elderly patients with other risk factors for cerebrovascular disease.

Recurrent optic neuromyelitis with endocrinopathies: a new syndrome or just a coincidence?

The spectrum of optic neuromyelitis (ONM) ranges from monophasic or recurrent idiopathic forms of the disease, to ONM associated with autoimmune disorders. A distinct form of the disease, called recurrent ONM with endocrinopathies, characterized by spinal cord involvement (cavitations with syringomyeloid sensory disturbance), rapid evolution to blindness and paraplegia, characteristic cerebrospinal fluid (CSF) findings, and association with hypothalamus-pituitary dysfunction, has recently been described. The first case of ONM with endocrinopathies in a female Caucasian from Europe is presented, supporting the existence of this syndrome as a separate entity.

[The laboratory diagnosis of multiple sclerosis]. / Laboratornye metody v diagnostike rasseiannogo skleroza.

The importance of laboratory methods for multiple sclerosis (MS) diagnosis and differential diagnosis is often overestimated now. The role of several methods including MRI, evoked potentials, examination of the cerebrospinal fluid and some others methods are discussed in this review. Several conditions may in some patients mimick the appearance of MS and it is easy to understand why there is a tendency among many clinicians to embark on extensive- and expensive-laboratory investigations to establish the correct diagnosis at early stages of the disease. Disorders like cerebrovascular diseases, vasculitis, Lyme disease, neurosarcoidosis, acute disseminated encephalomyelitis, progressive multifocal leukoencephalopathy, HIV-associated encephalitis may cause very close changes of the results of these examinations. A detailed, exhaustive history and the neurological examination, along with a careful scrutiny of the actual MRI films by the neurologist experienced in the diagnosis of MS, will obviate the need for additional tests in the overwhelming majority of cases. So, the MS diagnosis and differential diagnosis is still based mainly of the data of clinical observation.

Diagnostic criteria for multiple sclerosis.

Over a hundred years ago, Charcot set down what he considered to be some of the clinical characteristics of multiple sclerosis (MS). His triad was not specific but it was the first attempt to separate this disease from the many others affecting the nervous system. The history of clinical diagnostic criteria demonstrates the evolution from rather tentative classifications of restricted value to the more elaborate 1983 scheme which incorporates some laboratory procedures under the rubric paraclinical tests, considered to be extensions of the neurological examination, as well as a new category based on the presence of specific abnormalities of the cerebrospinal fluid (CSF). It is curious that until then the term definite MS had been avoided except for autopsy-proven cases, perhaps a wise move, since exact diagnosis may require long term observation. All the proposed schemes have been based on the twin principles of dissemination in both time and space. The diagnosis of MS must remain a clinical one, supported but not supplanted by the increasingly popular magnetic resonance imaging, which is non-specific and is frequently overinterpreted by radiologists lacking appropriate clinical information. Reliance on the MRI as the principal if not exclusive basis for the diagnosis leads to error in as many as one third of cases. This assumes a great deal of importance considering that such non-MS patients may be counted in epidemiological surveys and included in therapeutic trials for disease-modifying drugs, or eventually treated with these very expensive drugs with still controversial long term efficacy. Not surprisingly, attempts to develop reliable criteria for the MRI diagnosis of MS have been unsuccessful in view of the lack of specificity of that procedure. Great care should be taken to exclude the presence of extrinsic cervical spine lesions which might impinge on the cord, leading to the formation of plaques, or mimic the course of MS. An MRI of the cervical spine is recommended in all patients suspected of having MS who have symptoms suggestive of spinal cord involvement. The diagnosis of MS is, and will remain, based on clinical criteria which codify the characteristic dissemination in time and space of MS.