[A comparative analysis of rebif 22-mcg and copaxone efficacy in multiple sclerosis].

A comparative analysis of efficacy and tolerability of such immunomodulating compounds as rebif 22-mcg and copaxone used in the treatment of multiple sclerosis is presented. The analysis was based on the data obtained in the 2-year study of copaxone (145 patients) and rebif 22-mcg (74 patients) therapy carried out in the Neurology Institute, Russian Academy of Medical Sciences.

[Leber's hereditary optic atrophy: neurological and other non-optic appearances]. / Nasledstvennaia atrofiia zritel'nykh nervov Lebera: nevrologicheskie i drugie vneglaznye proiavleniia.

Comparing to other mitochondrial diseases, multisystemic lesions in Leber's hereditary optic atrophy (LHOA) occur less frequently. However, in some cases there are concomitant manifestations, especially neurological ones. Out of thirteen patients examined in the study, 5 exhibited MRI-detected neurological symptoms and changes, which may have concern to the underlying disease, namely LHOA caused by 11778A mutation. Literature and author's own data on neurological spectrum of LHOA and its possible relation to multiple sclerosis are summarized. A rare combination of LHOA caused by 14484C mutation and diabetes mellitus, described first-ever in the present study, is emphasized.

[Results of a multicenter study of Rebif-22 mcg administration in Russia]. / Rezul'taty multitsentrovogo issledovaniia éffektivnosti preparata Rebif-22 mkg v Rossii.

The results of multicenter study of Rebif 22 micrograms in Russia have been reported. 167 multiple sclerosis patients have received Rebif 22 micrograms three times a week for 1 year. This study provides evidence for reduction of the relapse rate in patients with relapsing/remitting and secondary progressive multiple sclerosis. The tendency to the decrease of the severity of relapses, less need for steroid use and the decrease of EDSS score in patients with relapsing/remitting multiple sclerosis have been shown. In general Rebif 22 micrograms was tolerated well.

[Leber's hereditary optic neuropathy: DNA-diagnosis and clinico-genetic comparisons in 12 families]. / Nasledstvennaia atrofiia zritel'nykh nervov Leber: DNK-diagnostika i kliniko-geneticheskie sopostavleniia v 12 sem'iakh.

Leber's hereditary optic neuropathy (LHON) is a worldwide spread neuro-ophthalmologic disease characterized by immediate pronounced visual reduction with a picture of retrobulbar neuritis, following by optic atrophy. The disease is caused by mitochondrial DNA mutations. Molecular genetic structure of 12 families, including 26 LHON patients, 13 of them being examined, is presented. All of the main primary mutations have been found: the most frequent 11778A (in 10 families), 3460A and 14484C (each in 1 family). In 5 families, the disease was clearly hereditary. Men predominated among the patients, that indicated a reduction of the gene penetrance in women. The most frequent age at the disease onset is 18-25 years. Clinico-genealogical LHON mechanisms correlate with mutation type. Molecular genetic mechanisms of the disease and possible environmental factors, influencing the gene penetrance, are discussed.

[Results of open post-registration clinical trials of copaxone in patients with multiple sclerosis]. / Rezul'taty otkrytykh postregistratsionnykh klinicheskikh ispytanii preparata kopakson u bol'nykh rasseiannym sklerozom.

Results of the 2-year open postregistration study of Copaxone (glatiramer acetate), conducted in 3 Moscow leading medical centres are given. 32 MS patients with remitting-relapsing MS were investigated in accordance with international requirements (neurological scales, MRI of the brain, multimodal evoked potential). Significant reduction of annual relapse rate for 70.3%, stabilization of neurological status and benign safety profile were demonstrated during Copaxone treatment. Practical recommendations for Copaxone use are given.

[Primary meningeal sarcomatosis of the brain and spinal cord with multiple tumor nodes]. / Pervichnyi meningeal'nyi sarkomatoz golovnogo i spinnogo mozga s mnozhestvennymi opukholevymi uzlami.

One case of this rare tumor diagnosed clinically in a female of 24 is reported. Cell composition was the same in both brain and spinal cord. There was a combination of a diffuse pattern of tumor cell arrangement with a formation of nodules and the spread of tumour cells along the perivascular spaces into the brain and spinal cord, along the endoneurium to the cranial and spinal nerve roots and metastasizing through the liquor system.

[The Gerstmann-Sträussler syndrome: new diagnostic possibilities]. / Sindroma Gerstmanna--Shtreusslera: novye vozmozhnosti diagnostiki.

On the basis of clinical picture of the disease, data of CT, MRT, psychological study, original laboratory investigation aimed at indication of changes in transposed neuroglia cells induced by the causal agent of subacute spongious transmissible encephalopathies, the diagnosis of patient K., 49 years old, was considered to be: syndrome of Gertsmann-Sträussler [correction of Herstmann Streussler]. Duration of the disease was 2 years. The case was sporadic. The history of the problem, modern views on etiology, pathogenesis of preventive measures are presented.

[Angiodysgenetic necrotizing myelopathy (the Foix-Alajouanine syndrome)]. / Angiodisgeneticheskaia nekrotiziruiushchaia mielopatiia (sindrom Fua--Alazhuanina).

One observation of a rare disease--angiodysgenetic necrotizing myelopathy--in a 50-year-old patient is described. The diagnosis was established at necropsy and did not confirm suspected spinal cord tumor and myelitis. The disease is angiodysplasia with resulting necrotizing myelopathy. Large dysplastic intra- and extramedullary vessels with a disturbed wall permeability, vascular malformations and necrotic foci in the spinal cord are characteristic for this condition.

[Criteria for the activity of the pathologic process in multiple sclerosis (clinico-computer tomographic correlations)]. / O kriteriiakh aktivnosti patologicheskogo protsessa pri rasseiannom skleroze (kliniko-komp'iuterno-tomograficheskie sopostavleniia).

Eight patients with the clinical signs of disease exacerbation were examined using computer-aided tomography of the head with contrast intensification. In 4 patients with a clinical aggravation expressed in the appearance of new neurological symptoms the authors obtained an effect of contrast intensification. Routine examination of the zones of the white substance revealed drug accumulation in the zones of both subnormal and normal density. There was no drug cumulation in 4 patients whose clinical aggravation was connected with the previous neurological symptomatology. The data obtained are discussed.

[Mental disorders in multiple sclerosis]. / Psikhicheskie narusheniia pri rasseiannom skleroze.

On the basis of clinical and follow up study of 106 patients with multiple sclerosis, including psychological tests and computerized tomography (34 observations), the authors specify the major types of psychic disorders related to this disease and represented in the form of the asthenic, hysteroformative, obsessive, and depressive syndromes, as well as in the form of an initial state with the clinical picture of organic dementia. Correlation of the examination data to computerized tomography findings usually reveals a direct relationship between the degree of psychic disorders and the severity of a cerebral injury. The authors point both to the necessity of controlling the psychic disturbances described and to their importance as a telltale sign in making the differential diagnosis of multiple sclerosis.