Myelin oligodendrocyte glycoprotein (MOG)-associated disease (MOGAD) is a rare, antibody-mediated inflammatory demyelinating disorder of the central nervous system (CNS) with various phenotypes starting from optic neuritis, via transverse myelitis to acute demyelinating encephalomyelitis (ADEM) and cortical encephalitis. Even though sometimes the clinical picture of this condition is similar to the presentation of neuromyelitis optica spectrum disorder (NMOSD), most experts consider MOGAD as a distinct entity with different immune system pathology. MOG is a molecule detected on the outer membrane of myelin sheaths and expressed primarily within the brain, spinal cord and also the optic nerves. Its function is not fully understood but this glycoprotein may act as a cell surface receptor or cell adhesion molecule. The specific outmost location of myelin makes it a potential target for autoimmune antibodies and cell-mediated responses in demyelinating processes. Optic neuritis seems to be the most frequent presenting phenotype in adults and ADEM in children. In adults, the disease course is multiphasic and subsequent relapses increase disability. In children ADEM usually presents as a one-time incident. Luckily, acute immunotherapy is very effective and severe disability (ambulatory and visual) is less frequent than in NMOSD. A critical element of reliable diagnosis is detection of pathogenic serum antibodies MOG with accurate, specific and sensitive methods, preferably with optimized cell-based assay (CBA). MRI imaging can also help in differentiating MOGAD from other neuro-inflammatory disorders. Reports on randomised control trials are limited, but observational open-label experience suggests a role for high-dose steroids and plasma exchange in the treatment of acute attacks, and for immunosuppressive therapies, such as steroids, oral immunosuppressants and rituximab as maintenance treatment. In this review, we present up-to-date clinical, immunological, radiographic, histopathological data concerning MOGAD and summarize the practical aspects of diagnosing and managing patients with this disease.

The impact of choroid plexus with its blood–cerebrospinal fluid barrier in the ischemic stroke pathology is poorly explored. Transthyretin (TTR) is a protein synthesized in liver and just in choroid plexus. The current study was designed to assess the prognostic value of serum TTR for functional outcome (at the time of hospital discharge) and long-term (one-year) overall mortality in ischemic stroke patients. We conducted a prospective observational study. Patients (n = 81) with acute (< 24 hours of symptoms onset) ischemic stroke consecutively admitted to Stroke Unit were included. An unfavorable outcome was defined as a modified Rankin Scale (mRS) score ≥ 3. The relationships between serum TTR levels and clinical outcome were analyzed using multivariate analysis. One-year mortality was analyzed by Kaplan–Meier survival curves stratified by mean value of TTR. Compared with patients with mRS <3, patients with an unfavorable outcome at hospital discharge had significantly lower TTR levels on admission (P < 0.0001). In non-survivals serum TTR levels were significantly lower compared with patients who survive one year of observation (P = 0.009). Using multivariate analysis, transthyretin emerged as an independent predictor for unfavorable outcome at the day of hospital discharge (adjusted odds ratio = 0.96; 95% CI: 0.9–0.99, P <0.05). A one-year mortality of patients with the lower TTR levels was significantly higher than in patients with TTR levels above mean value (P = 0.02). Serum level of TTR at admission was a predictor of functional outcome after ischemic stroke and was also associated with one-year mortality in stroke survivals.

Background: Hyperechogenicity of the substantia nigra (SN) measured by transcranial sonography (TCS) is a characteristic feature observed in patients with Parkinson’s disease (PD). To our knowledge, no SN hyperechogenicity data are available for Polish population. Moreover most of studies come from few centres, which used the one type of ultrasound device. The main aim of the study was to investigate the association between PD and SN hyperechogenicity measured by sonographic machine, not assessed so far. Materials and methods: In this study cross-sectional study SN hyperechogenicity was evaluated in 102 PD patients and 95 control subjects. Midbrain was visualised by Aloka Prosound 7 ultrasound device. SN area measurement, the relation to the clinical features of PD, inter- and intra-observer reliability were evaluated. Results: We confirmed that SN echogenicity is significantly increased in PD patients compared to control subjects (p < 0.001). The area under curve for PD patients vs. controls was 0.93. Receiver operating characteristic analysis indicated a cut-offs for SN echogenicity at 0.19 cm² with accuracy equal to 90%, specificity — 86% and sensitivity — 93.7%. The SN hyperechogenicity was not related to PD clinical findings. Reliability was good if an experienced sonographer performed the SN measurements. Conclusions: This study shows that the SN abnormality observed by TCS isa specific feature, which can be helpful in the process of PD diagnosing. Background: Hyperechogenicity of the substantia nigra (SN) measured by transcranial sonography (TCS) is a characteristic feature observed in patients with Parkinson’s disease (PD). To our knowledge, no SN hyperechogenicity data are available for Polish population. Moreover most of studies come from few centres, which used the one type of ultrasound device. The main aim of the study was to investigate the association between PD and SN hyperechogenicity measured by sonographic machine, not assessed so far. Materials and methods: In this study cross-sectional study SN hyperechogenicity was evaluated in 102 PD patients and 95 control subjects. Midbrain was visualised by Aloka Prosound 7 ultrasound device. SN area measurement, the relation to the clinical features of PD, inter- and intra-observer reliability were evaluated. Results: We confirmed that SN echogenicity is significantly increased in PD patients compared to control subjects (p < 0.001). The area under curve for PD patients vs. controls was 0.93. Receiver operating characteristic analysis indicated a cut-offs for SN echogenicity at 0.19 cm² with accuracy equal to 90%, specificity — 86% and sensitivity — 93.7%. The SN hyperechogenicity was not related to PD clinical findings. Reliability was good if an experienced sonographer performed the SN measurements. Conclusions: This study shows that the SN abnormality observed by TCS isa specific feature, which can be helpful in the process of PD diagnosing.

The role of the thyroid gland in ischemic stroke pathology is not well understood. As thyroid hormones modulate the extracellular matrix, we explored the possible link between them and secreted protein acidic and rich in cysteine like 1 (SC1) - one of the extracellular matrix molecules. In the 81 patients with acute ischemic stroke, serum SC1 levels were much higher compared with 30 control subjects: 4.47 vs 2.43 ng/mL (p < 0.001). Serum levels of free thyroxine (fT4) were higher in stroke subjects compared to those of controls (p = 0.03). In stroke patients, TSH concentration was lower than in the control group (p = 0.03). SC1 levels positively correlated with fT4 levels (p = 0.02) and negatively with TSH (p = 0.03) in stroke patients. Our results confirmed the association between thyroid hormones and SC1 - extracellular matrix protein. (C) 2018 Polish Neurological Society. Published by Elsevier Sp. z o. o. All rights reserved.