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.
Multiple sclerosis is a common cause of disability among young and middle-aged people. Despite the modern possibilities of diagnostics and therapy, over time, the disease acquires a secondary progressive character. Rehabilitation of patients at all stages of the disease plays an important role in improving well-being, improving the quality of life, adapting the patient and restoring motor skills. However, there is currently no clear recommendation for the application of specific techniques in each case. The aim of this work was to analyze the available methods of rehabilitation therapy, to highlight the most used and promising ones. Due to the progressive course of the disease, the benefits of rehabilitation measures are usually higher at the initial stages. Nevertheless, nowadays there is a large number of works devoted to rehabilitation measures in patients with moderate and high levels of disability. It has been shown that both inpatient and outpatient rehabilitation has a positive effect on the quality of life and improvement of clinical indicators. Our review describes the main techniques with recommendations for the scheme of application. A comprehensive assessment of the patient's health status, a multidisciplinary team and a personalized approach increase the quality and effectiveness of rehabilitation measures. We also describe our own experience in the treatment of spasticity in patients with a secondary progressive multiple sclerosis.
The review is devoted to up-to-date data on epidemiology, aspects of the pathogenesis of neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD). The authors consider a role of myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) in the syndromes phenotypically similar to NMO and NMOSD. Special attention is drawn to the methods of MOG-IgG antibodies detection and indications for testing. The approaches and management for treatment and prevention of NMO relapses, risks of complications during pregnancy and immediately after delivery, as well as methods for their prevention and treatment, are described.
AIM:To analyze clinical, neuroimaging and laboratory characteristics of acute disseminated encephalomyelitis (ODEM) in adults with an analysis of the frequency of diagnostic errors at the initial examination stage.MATERIAL AND METHODS:The study included 23 patients hospitalized with a diagnosis of ODEM. The analysis of clinical characteristics, MRI results and cerebrospinal fluid (CSF) was performed. The diagnosis of the disease in each case was specified after a full examination and dynamic observation.RESULTS:The diagnosis of ODEM was confirmed only in 16 cases. In 6 cases, the final diagnosis of multiple sclerosis (MS) was made, and one case of Susak syndrome, primary CNS lymphoma and levamisole-associated multifocal inflammatory leukoencephalopathy was also detected. It has been shown that ODEM in adults is characterized by the acute development of multifocal brain lesions in combination with clinical manifestations of encephalopathy, which in half of cases is preceded by an infectious disease. There were no specific clinical, neuroimaging and laboratory features, which could allow differentiation of ODEM from onset of MS.CONCLUSION:To make a correct diagnosis in the first episode of acute multifocal brain lesion, a dynamic observation, including repeated MRI, is needed. The development of specific biomarkers may be of great importance for the early differential diagnosis of demyelinating diseases.
Poster: ECR 2018 / C-2849 / DTI and perfusion changes in multiple sclerosis patients by: I. Krotenkova , V. Bryukhov, M. Zakharova, M. Krotenkova, L. Askarova; Moscow/RU
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.
Primary central nervous system lymphoma (PCNSL) is a rare aggressive extranodal non-Hodgkin lymphoma. Difficulties in diagnosing PCNSL are associated with the absence of pathognomonic clinical and neuroimaging findings of this disease. The article describes the clinical case of a female patient with autopsy-confirmed multifocal large-cell B-cell CNS lymphoma misdiagnosed as acute disseminated encephalomyelitis (ADEM). Clinical and neuroimaging characteristics of PCNSL and ADEM as well as the role of diagnostic methods in establishing the correct diagnosis are discussed.
In the paper, the results of 1-year treatment with avonex, a newimmunomodulator from the group of interferons b, in 34patients with relapsing remitting multiple sclerosis have beenreported. In the treated patients it was shown significantdecrease of the activity of the pathological process, which wasevidenced by reduction of the severity of exacerbations and therelapse rate. The decrease of disability of patients with multiplesclerosis (by EDSS score) was revealed. In general, one cannotice good tolerability of avonex.
Results of the two-year treatment with avonex of 34 patients withrelapsing remitting multiple sclerosis are summarized. This studyprovides evidence for the significant decrease in activity of thepathological process in patients treated with avonex, which is confirmedby the reduction of the relapse rate and the increase of the proportion of patients remaining exacerbation-free. The decreaseof the EDSS score has also been shown. In general, avonex was toleratedwell in patients under study.
Nowadays neurodegeneration in multiple sclerosis is closely associated with irreversible disability progression. However, the involvement sequence of different regions in atrophy is still unclear. The aim of our study was to determine the relationship between brain and spine atrophy and neurological disability in relapsing-remitting multiple sclerosis (RRMS) patients. 45 RRMS patients underwent brain and spine MRI with voxel based morphometry and neurological examination with EDSS and were compared to 20 healthy controls. As a result we determined a distinct patterns of atrophy distribution in MS patients and the sequence of involvement of different central nervous system regions. A strong correlation between disability progression rate and gray matter atrophy rate was detected.
Objective. To evaluate the jugular veins (IJV) using duplex sonography in patients with multiple sclerosis (MS). Material and methods. The study included 21 MS patients with different disease course, degree of disability and disease duration. A control group consisted of 8 healthy volunteers. Results. IJV changes that meet the CCSVI criteria have not been found. These findings are consistent with the results of recently published large studies. Possible reasons of non-confirmation of this hypothesis are discussed. Conclusion. Currently vascular intervention in MS is not recommended.
Trigeminal neuralgia is a rare symptom of multiple sclerosis affecting the disability. Multiple sclerosis related trigeminal neuralgia has been attributed to a demyelinating lesion in the pons. When the adequate pain drug-relieve therapy is not possible or when the patient becomes refractory to the treatment or can not continue pharmacological treatment because of the side effects, surgical intervention, including percutaneous radiofrequency rhizotomy is being discussed. Literature review and the data upon the efficiency and safety of this neurosurgical treatment in 16 patients with multiple sclerosis have been analyzed. Percutaneous radiofrequency rhizotomy has been proved to be a safe, reproducible and effective method of the symptomatic surgical treatment of trigeminal neuralgia in patients with multiple sclerosis in cases of the intolerance/inefficiency of the pharmacological therapy.