[Acute hemorrhagic leukoencephalitis with a subacute onset]. / Ostryi gemorragicheskii leikoentsefalit Khersta, variant podostrogo techeniya.

Acute hemorrhagic leukoencephalitis (AHLE), also called Hurst's encephalitis, is a rare demyelinating disease of the central nervous system characterized by rapid progression and acute inflammation of the white matter of the brain and spinal cord. AHLE is currently considered as a rare, most severe variant of acute disseminated encephalomyelitis. Clinically AHLE is characterized by a fulminant course with a rapid development of encephalopathy and multifocal neurological symptoms. AHLE is associated with high mortality rate that requires immediate and aggressive treatment initiation. This article describes a case of AHLE with an atypical course, a subacute form, which is extremely rarely described in the literature, with the progressive symptoms' development over several months. Due to delayed treatment initiation, unfortunately, a fatal outcome has been observed. Subsequent histological examination of the autopsy material confirmed the presence of a subacute form of AHLE in the patient.

[Hereditary optic neuropathy associated with demyelinating diseases of the central nervous system]. / Nasledstvennaya opticheskaya neiropatiya v sochetanii s demieliniziruyushchimi zabolevaniyami tsentral'noi nervnoi sistemy.

Demyelinating optic neuritis and hereditary optic neuropathy (HON) take a leading place among the diseases, the leading clinical syndrome of which is bilateral optic neuropathy with a simultaneous or sequential significant decrease in visual acuity. Optic neuritis can occur at the onset or be one of the syndromes within multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), and myelin oligodendrocyte glycoprotein (MOG) antibody disease (MOGAD). HON are a group of neurodegenerative diseases, among which the most common variants are Leber's hereditary optic neuropathy (LHON), associated with mitochondrial DNA (mtDNA) mutations, and autosomal recessive optic neuropathy (ARON), caused by nuclear DNA (nDNA) mutations in DNAJC30. There are phenotypes of LHON «plus¼, one of which is the association of HON and CNS demyelination in the same patient. In such cases, the diagnosis of each of these diseases causes significant difficulties, due to the fact that in some cases there are clinical and radiological coincidences between demyelinating and hereditary mitochondrial diseases.

[Optic neuropathies as an interdisciplinary subject of research]. / Opticheskie neiropatii kak predmet mezhdistsiplinarnogo izucheniya.

Despite the wide range of clinical, instrumental and laboratory methods used in modern ophthalmology, the problem of diagnosing optic neuropathy and identifying its etiology remains relevant. A complex multidisciplinary approach involving various specialists is required in the differential diagnosis of immune-mediated optic neuritis, for example in multiple sclerosis, neuromyelitis optica spectrum disorder, and MOG-associated diseases. Of special interest is differential diagnosis of optic neuropathy in demyelinating diseases of the central nervous system, hereditary optic neuropathies and ischemic optic neuropathy. The article presents a summary of scientific and practical results of differential diagnosis of optic neuropathies with various etiologies. Timely diagnosis and early therapy start reduces the degree of disability in patients with optic neuropathies of different etiologies.

Levamisole-associated multifocal inflammatory encephalopathy: clinical and MRI characteristics, and diagnostic algorithm.

Levamisole-associated multifocal inflammatory encephalopathy (LAMIE) is a devastating adverse effect of levamisole (LEV) treatment. In Russia, people often use LEV without a doctor's prescription for anthelmintic prophylaxis. LAMIE often misdiagnosed as the first episode of MS or acute disseminated encephalomyelitis (ADEM). The aim of our study was to describe clinical, laboratory and morphological characteristics of LAMIE, magnetic resonance imaging (MRI) patterns and create an algorithm for the differential diagnosis. This study was a prospective observational study with retrospective analysis of cases. It was performed at two hospitals with ambulatory service for MS. We included 43 patients with LAMIE with follow-up was from 1 year to 5 years. Age was 19-68 y.o. with female predominance. The most typical manifestations of LAMIE were cerebellar, pyramidal and cognitive symptoms, and majority of patients had biphasic course of the disease. Three main types of MRI patterns were described: ADEM-like, MS-like, atypical demyelination. About 40% of patients had CSF specific oligoclonal bands synthesis, but only 20 % of them converted to MS during the period from 1 month until 2 years. The CSF albumin levels and immunoglobulin G index were elevated in LAMIE patients compared to reference values. We described results of brain biopsy in two cases. Therefore LAMIE should be considered in patients with demyelinating or inflammatory conditions with biphasic onset of the disease and variable MRI presentation.

[Quantitative susceptibility mapping in assessment of inflammation and neurodegeneration in multiple sclerosis]. / Kolichestvennoe kartirovanie vospriimchivosti v otsenke vospalitel'nogo i neirodegenerativnogo protsessov pri rasseyannom skleroze.

Quantitative susceptibility mapping (QSM) is a relatively new MRI technique that may potentially help estimate iron concentrations in the brain. It plays a big role in diagnosis of many pathological processes, including multiple sclerosis (MS). Iron metabolism in the brain is a complex and not fully understood process. It is known that the content of iron in the brain increases with age; in addition, its accumulation is often observed in many neurodegenerative diseases, including MS foci, and its amount changes over time. In this regard, the values of magnetic susceptibility obtained using QSM can potentially become a convenient biomarker that reflects the latent activity and progression of MS, which, in turn, can influence the choice of therapy and the tactics of treating patients.

[A case of subtentorial progressive multifocal leucoencephalopathy in a patient treated with natalizumab]. / Sluchai razvitiia progressiruiushchei mul'tifokal'noi leikoéntsefalopatii subtentorial'noi lokalizatsii na fone terapii natalizumabom.

This paper presents a case report of subtentorial progressive multifocal leukoencephalopathy (PML) in a 26-year-old female patient treated with natalizumab. The evolution of clinical features, neuroimaging data and treatment as well as the development of immune reconstitution inflammatory syndrome (IRIS) are described. This case emphasizes the importance to keep accurately the risk management plan during natalizumab treatment. This includes performing MRI scans in order to detect changes typical for PML at the earliest (preclinical) stage in time.

[Radiologically isolated syndrome as a possible preclinical stage of primary-progressive multiple sclerosis]. / Radiologicheski izolirovannyi sindrom - vozmozhnaia doklinicheskaia stadiia pervichno-progressiruiushchego rasseiannogo skleroza.

The article presents a clinical case-report of transformation of radiologically isolated syndrome (RIS) to primary-progressive multiple sclerosis (PPMS). It also provides a review of international and Russian data on the comparison of clinical and MRI characteristics of PPMS with relapsing-remitting multiple sclerosis and secondary-progressive multiple sclerosis and the review of the current data on the risks of transition of RIS to PPMS.

[Brain atrophy and perfusion changes in patients with remitting and secondary progressive multiple sclerosis]. / Atrofiia golovnogo mozga i perfuzionnye izmeneniia u patsientov s remittiruiushchim i vtorichno-progressiruiushchim rasseiannym sklerozom.

AIM: To study the relationship of brain atrophy and changes in perfusion with an increase in the level of disability in patients with multiple sclerosis (MS). MATERIAL AND METHODS: Twenty patients with remitting MS, 20 patients with secondary progressive multiple sclerosis (SPMS) and 20 healthy people were studied. The level of neurological deficit was assessed with EDSS and cognitive status with PASAT. MRI of the brain (standard impulse sequences and 3D-T1-MPR for voxel MRI-morphometry) and perfusion computed tomography with the assessment of visually intact white matter (VIWM) and thalamus were performed. RESULTS: Compared to the control group, patients with MS had a significant atrophy of subcortical gray matter. Patients with SPMS in addition had an atrophy of some cortical areas which was correlated with EDSS scores (p<0.05). The correlation between cognitive impairment and the volume of the left inferior parietal lobule (r=0.677; p=0.011) and worsening of perfusion of VIWM of frontal and parietal lobes, thalamus on both sides was observed in patients with SPMS compared to those with remitting MS. That was correlated with cognitive performance assessed by PASAT. CONCLUSION: Patterns of atrophy distribution in different types of MS were determined. The level of disability is correlated with the severity of brain atrophy. Hypoperfusion of VIWM that was correlated with cognitive impairment was found in patients with SPMS.

[Atypical multiple sclerosis - Balo's concentric sclerosis: two case-reports and a review]. / Atipichnyi rasseiannyi skleroz - kontsentricheskii skleroz Balo: dva klinicheskikh nabliudeniia i obzor.

This article presents two clinical cases of patients diagnosed with Balo's concentric sclerosis. Distinctive features of the pathogenesis in the aspect of differential diagnosis from other forms of multiple sclerosis and possible treatment are discussed.

[A current view on the MRI diagnosis of multiple sclerosis: an update of 2016 revised MRI criteria]. / Sovremennyi vzgliad na MRT-diagnostiku rasseiannogo skleroza: obnovlennye MRT-kriterii 2016 g.

Magnetic resonance imaging (MRI) is the primary method for confirming the clinical diagnosis of multiple sclerosis (MS). The article presents the current data on using MRI of the brain and spinal cord for diagnosis in suspected MS. Special attention is paid to the MRI criteria of McDonald and MAGNIMS for relapsing-remitting MS (RRMS) and primary-progressive MS (PPMS) in the latest revisions of 2010 and 2016. The information provided can help radiologists and neurologists to optimize the use of MRI in clinical practice for diagnosis of MS.

[A DTI study of the spinal cord lesion in patients with multiple sclerosis during the follow-up after relapse]. / Porazhenie spinnogo mozga u patsientov s rasseyannym sklerozom posle obostreniya po dannym diffuzionno-tenzornoi magnitno-rezonansnoi tomografii.

UNLABELLED: Spinal cord involvement is frequent in multiple sclerosis (MS) but the correlation between spinal cord damage on conventional MRI and clinical symptoms is not always obvious. Diffusion tensor imaging (DTI) is a sensitive technique for revealing tissue damage. OBJECTIVE: to investigate spinal cord DTI changes in MS patients during the relapse and in the follow-up. MATERIAL AND METHODS: Data were acquired from 25 patients with relapsing-remitting MS during the relapse characterized by unilateral light hand palsy, in three and twelve months after it. All patients underwent full neurological examination and MRI including conventional head and neck MRI and DTI of the brain and upper spinal cord in the sagittal plane. Twelve healthy subjects entered the control group. RESULTS AND CONCLUSION: Spinal cord sagittal DTI provides a reliable information about significant changes in MS patients compared tothe control group both inside demyelinating lesions and in the normal appearing spinal cord. These differences are preserved both in 3 and 12 months after the relapse and together with clinical recovery create evidence of functional compensatory mechanisms development. A tendency towards DTI parameters normalization together with faster fine motor skills recovery in patients without the asymmetrical decrease in vibration sense shows an important role that afferentation plays in recovery after the relapse.

[Standartization of MRI studies in multiple sclerosis]. / Standartizatsiya MRT- issledovanii pri rasseyannom skleroze.

The use of magnetic resonance imaging (MRI) in patients with multiple sclerosis has markedly increased in recent years. The main task of the MRI studies after the diagnosis of multiple sclerosis is to assess the dynamics of MRI for determining disease progression and monitoring the efficacy of therapy. In this regard, it is very important to obtain the most identical baseline and follow-up MRI that is possible when a single standard protocol is used. This article presents the protocol of brain MRI and spinal cord MRI and interpretation of MRI studies in patients with multiple sclerosis.

[Primary-progressive multiple sclerosis as an atypical demyelinating process]. / Pervichno-progressiruyushchii rasseyannyi skleroz kak atipichnyi demieliniziruyushchii protsess.

This article presents an overview of current data on primary-progressive multiple sclerosis (MS). In this aspect, the authors consider its characteristics in comparison to other MS forms as well as possible markers of the disease, criteria of diagnosis and therapeutic options in the present and the future times.

[Radiologically isolated syndrome - MRI criteria and management tactic]. / Radiologicheski izolirovannyi sindrom (MRT-kriterii i taktika vedeniya bol'nogo).

The use of brain MRI for the diagnosis of neurological diseases has increased significantly over the past two decades. This has led to an increase in the discovery of chance findings, with asymptomatic lesions in the white matter being most frequent. Based on radiological criteria, these findings can be interpreted as demyelinating lesions in some cases. However, in the absence of clinical symptoms characteristic of multiple sclerosis (MS), a diagnosis of MS can't be made in the patients with these incidental findings. These patients now met a diagnosis of radiologically isolated syndrome (RIS). Diagnostic and clinical approaches to patients with RIS are discussed in this article.

[Diffusion tensor magnetic resonance imaging in the diagnosis of visual pathway neurodegeneration in glaucoma].

AIM: To study the state of visual pathways in patients with glaucoma by means of diffusion tensor magnetic resonance imaging (DT-MRI). MATERIAL AND METHODS: DT-MRI was performed in 16 patients: 12 patients with POAG of different stages and 4 controls. Fractional anisotropy (FA) and its longitudinal variation (FA) determined by an original software (Fractional Anisotropy Analyzer) were used to assess the state of the lateral geniculate body and optic radiation. All patients also underwent optical coherence tomography (OCT) of the retina and optic nerve head, Heindelberg retinal tomography (HRT), and Humphrey standard automated perimetry (SAP). RESULTS: There was a significant decrease in FA values in glaucoma patients as compared with the control group (medians of 0.74 and 0.77 respectively, p < 0.01). Moderate and strong correlations of AFA with glaucoma stage as well as morphometric (GCC Average, GLV, FLV, and RNFL Average provided by OCT, rim area provided by HRT) and functional parameters (MD and PSD provided by SAP) of the retina and optic nerve head were also established. CONCLUSION: Reduced fractional anisotropy of the lateral geniculate body and optic radiation in patients with glaucoma may be considered an indicator of axonal degeneration at the level of the visual centers. DT-MRI in vivo helps to determine whether or not the neurodegenerative process spreads to the central nervous system as glaucoma progresses.