Search

Introduction: Inflammatory demyelinative diseases of the central nervous system are mostly idiopathic and represent the major cause of neurological disability in young adults. These diseases differ in terms of clinical symptoms, severity, pathological characteristics and epidemiology. However, there are also significant similarities between these diseases, which sometimes bring to a misleading diagnosis. Neuromyelitis optica (NMO) is a demyelinative disease in which the optic nerve and the spinal cord are predominantly affected. The detection of specific antibodies to aquaporin-4 (NMO-IgG) led to a modification of the diagnostic criteria for NMO., Methods: We performed a retrospective study on NMO-IgG positive patients referred to the Department of Neurology MS Center (2006-2011) with suspected NMO. Based on the presenting symptomatology of the patients, we identified the cases with optic neuritis and various parameters that may differentiate between NMO and MS. NMO-IgG were evaluated by ELISA., Results: A total of 50% of the 107 patients with NMO-IgG fulfilled the revised criteria of NMO; 38 patients had a single attack of optic neuritis or long lesion in the spinal cord and 15 patients presented with an opticospinal type of MS. The visual acuity following a single attack of optic neuritis remained significantly lower in NMO patients as compared to MS patients. Most of the NMO patients with NMO-IgG had additional attacks of optic neuritis within a short time from the initial event., Conclusions: The finding of NMO-IgG in patients with optic neuritis foreshadows a bad prognosis and relapses. These patients are at high risk of experiencing a second event in the central nervous system and fulfilling the clinical criteria for NMO. Due to the difference in the severity of inflammation of the optic nerve between NMO and MS, it is highly recommended to seek a laboratory check-up for NMO-IgG in serum, immediately after the first event, in order to determine the necessity and the kind of treatment for the patient.

Paraneoplastic Neurologic Diseases (PND) are remote complications of certain neoplastic diseases characterized by distinct neurological deficits in the central as well as the peripheral nervous system. The neurological disorders are usually clinically evident before the cancer is identified. PND are rare syndromes believed to have an immunological link where antibodies or activated T cells are acting against self antigens shared by the tumor cells and neurons. Most of these onconeural antigens are located in the cytoplasm-nuclear compartment of the cell, whereas others are located at the membrane and act as receptors or ion channels. This short review discusses the autoimmune mechanisms involved in PND in general and in opsoclonus-myoclonus-ataxia in particular. The article attempts to clarify why in most PND the immune effector mechanisms against onconeural antigens detected in sera of patients may not be the direct cause of the neurological deficit. We elaborate on the difference between the immune response against cytoplasmic-nuclear antigens as opposed to membranal antigens with regard to the fact that: a) the nervous system is considered immune privileged; b) neither the tumors nor the neurons have an optimal expression of MHC class I antigens and onconeural epitopes on the membrane. The availability of onconeural antigens to the immune effectors mechanisms is probably the main reason why there are few animal models for PND.

Primary Sjogren's syndrome has a wide clinical spectrum, ranging from an exocrinopathy to a systemic process and even extending to B-lymphocyte neoplasia. We describe a patient with this syndrome who developed central nervous system involvement manifested by involuntary dystonic movements of the mouth and larynx. We believe that the neurological involvement in this patient is part of his basic disease. Further studies are required to define more accurately the frequency, severity and spectrum of the neurological manifestations associated with primary Sjogren's syndrome.

A synthetic copolymer of amino acids, copolymer 1 (Cop 1), proved to be very effective in suppressing experimental autoimmune encephalomyelitis (EAE), an animal model for multiple sclerosis (MS). It is 1 of a series of synthetic amino acid copolymers which simulate the basic protein constituent of the myelin sheath and the autoantigen responsible for induction of EAE. It cannot induce EAE but it does suppress it in a variety of animals. The immunological cross-reaction between Cop 1 and the basic protein is the basis for this suppressive activity. In view of the resemblance between EAE and MS, clinical trials with Cop 1 in MS were started. The first preliminary clinical trial was at this hospital and involved 4 patients with severe MS. A double-blind, randomized placebo-controlled pilot trial was carried on for 2 years in 50 patients with the exacerbating-remitting form of MS but with a Kurtzke Disability Status Scale rating of no more than 6. There were statistically significant differences in the number of patients with exacerbations in the placebo group, 23, as compared to only 16 in the Cop 1-treated group. As to exacerbations per patient, the 2-year averages were 2.7 and 0.6, respectively. Side-effects of Cop 1 were minimal. These results suggest that Cop 1 may be beneficial when given early in the exacerbating-remitting form of MS. Further trials, necessary to establish its efficacy in MS, are being run at the Albert Einstein College of Medicine in NY.