The Myasthenia Gravis-specific Activities of Daily Living scale as a useful outcome measure and in routine clinical management in Polish patients

Introduction The Myasthenia Gravis-Activities of Daily Living scale (MG-ADL) is a short, and easy to use disease-specific quality of life during daily routine tool in myasthenia gravis. Objectives The purpose of our work was to evaluate neurological condition patients with myasthenia gravis using the form MG-ADL in order to enable the introduction in common use of an instrument which allows for the assessment of patients with myasthenia gravis. Patients and Methods The total number of 50 patients with MG were qualified for the examination. Each patient underwent neurological examination and completed the quality of life evaluation questionnaire MQ-ADL. Additionally, each patient was asked to evaluate the quality of his/her life by means of questionnaire MG-QOL 15 and MG Composite in Polish language version. Results Our analysis showed a positive correlation with other scales used - MG-QOL 15, MGFA, MG Composite. The intensification of neurological symptoms showed significant relation with obtained higher number of points in MG-ADL questionnaire. The MG-ADL was found to have high internal consistency, test–retest reliability, and concurrent validity. Conclusion We confirmed reliability and dependability of the questionnaire in the the test-retest assessment. The MG-ADL is accepted to be a reliable and valuable tool for measuring disease-specific QOL in Polish patients with MG

Shoulder pain as one of the symptoms of syringomyelia

Syringomyelia is typically a progressive chronic condition caused by a disruption of normal cerebrospinal fluid flow. Earlier diagnosis is associated with better outcomes, because although the progression of neurological deficits usually stabilizes after intervention, many patients still remain at least partially symptomatic. In this paper, we describe the case of a 33-year-old female patient with syringomyelia. The patient reported to the Department of Neurology due to sensory disorders and shoulder pain for several months. On admission, neurological examination revealed right hand muscular deficit, abolition of sensation of temperature and asymmetry of tendon reflexes. A few weeks earlier, outpatient magnetic resonance imaging (MRI) of the cervical spine showed the features of Arnold-Chiari malformation, syringomyelia and C5/C6 and C6/C7 discopathy. During hospitalization, MRI of the thoracic spine was performed, and the syringomyelic cavity C2–Th6 was revealed. The patient in stable condition was discharged home and referred to the neurosurgery department. The patient underwent a medial sub-occipital craniectomy and, a month later, was admitted to the neurological rehabilitation department due to paresis and sensory disturbances of right upper limb. As a result of the physiotherapy, the right arm's motor function and general physical condition improved

Left-hemisphere ischemic stroke as a complication of cutaneous lupus erythematosus

We describe the case of a 33-year-old patient with cutaneous lupus erythematosus admitted to the Department of Neurology due to the weakness of right limbs and speech disorders after night sleep. On admission, neurological examination revealed: no verbal contact; mixed aphasia with motor predominance; wide, symmetrical and reactive pupils; conjugate gaze palsy in a horizontal direction; right-sided hemianopsia and right-sided pyramidal tract syndrome with right limbs paralysis and bilaterally positive Babinski's sign. Magnetic resonance imaging revealed changes in the type of fresh ischemia in the left hemisphere of the brain – in the temporal lobe / basal ganglia on the left side hyperintensive areas were visible. As a result of the therapy and rehabilitation, the patient's general and neurological condition was improved. The patient was discharged home with recommendations in good general condition

SARS-CoV-2 neurotropism and other possible causes of olfactory disorders in COVID-19

Coronavirus disease 2019 (COVID-19) is an acute infectious respiratory disease (AIRD) caused by infection with the new severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The first cases were diagnosed and reported in Wuhan, central China, in November 2019. The disease initially occurred locally. However, the number of infected individuals increased dynamically and spread worldwide. The most common symptoms of the SARS-CoV-2 infection include malaise, fever, dry cough and dyspnoea. Over time, reports of new COVID-19 symptoms included taste and smell disorders. A potential cause of these disorders is related to neurotropism, i.e. the affinity of SARS-CoV-2 to the nervous system. Angiotensin-converting enzyme 2 receptor is essential in the pathogenesis of SARS-CoV-2 infection. The receptor is found in many tissues and organs, including the olfactory epithelium, neurons and neuroglial cells. Another potential cause is neuroinvasiveness, i.e. the ability of the virus to invade the central nervous system, and thereby damage its structures. As a result, olfactory disorders may occur. Other concepts, such as the inflammatory response of the body and the concept of stroke or damage to olfactory supporting cells, are also considered

COVID-19 and autoimmune diseases of the nervous system — an update

Introduction. Due to a similar pathomechanism, COVID-19 infection may significantly affect the course of autoimmune diseases (AIDs). In our review, we aimed to assess the severity of SARS-CoV-2 infection, response to treatment, and the impact of COVID-19 infection on the course of the underlying disease in patients with neuroimmune diseases. State of the art. In the time of the COVID-19 pandemic, it was important to determine the influence of COVID-19 infection on the course of autoimmune diseases due to the weakened immune system and immunosuppressive therapies. Clinical implications. Many reports have indicated that in patients with AIDs, the existence of the disease is not associated with a worse prognosis in the course of the viral infection. Patients in advanced stages of the disease, elderly patients, and those with comorbidities are at risk of more frequent hospitalisations and higher mortality in the course of COVID-19. Moreover, some drugs used in AIDs have been tested for their efficacy in SARS-CoV-2 infection. Episodes of newly diagnosed myasthenia gravis, Guillain-Barré syndrome, acute disseminated encephalomyelitis (ADEM), and neuromyelitis optica spectrum disorder (NMOSD) secondary to COVID-19 or vaccination have also been reported. Vaccination against this pathogen is highly recommended in most patients with AIDs. Future directions. Despite many studies on the association between COVID-19 and neuroimmune diseases, more specific data is needed. The approach to patients with AIDs should be individual, since many issues remain unresolved despite the long-lasting pandemic

Validity and reliability of the Polish version of myasthenia gravis – Quality of life questionnaire – 15 item

Aim The myasthenia gravis-quality of life questionnaire 15 item (MG-QOL15) is a short, and easy to use disease-specific quality of life (QOL) tool in myasthenia gravis. The aim of this study was to validate and adapt the Polish version of the MG-QOL15. Materials and methods The total number of 50 patients with MG were qualified for the examination. Each patient underwent neurological examination and completed the quality of life evaluation questionnaire MQ-QOL 15 after translation and back-translation. Additionally, each patient was asked to evaluate the quality of his/her life by means of questionnaire SF-36 in Polish language version. Results The MG-QOL15 was found to have high internal consistency, test–retest reliability, and concurrent validity. Conclusion The MG-QOL15 is accepted to be a valid, reliable, valuable tool for measuring disease-specific QOL in Polish patients with MG

What you cannot get from routine MRI of MS patient and why – The growing need for atrophy assessment and seeing beyond the plaque

Multiple sclerosis is a disease that still has not been fully understood and calls for better diagnostic procedures for the improvement of everyday patient care and drug development. Routine magnetic resonance examinations reveal demyelinating focal lesions, but they do not correlate sufficiently with the patients’ disability and cognitive impairment. For more than 100 years it has been known that demyelination affects not only white but also grey matter of the brain. Recent research has confirmed the serious consequences of grey matter pathology. Over the last several years, atrophy of the brain and especially of its grey matter has become a most promising marker of the patients’ clinical status. The paper discusses the concept and importance of atrophy assessment in relation to the standard magnetic resonance results

Improvement of quality of life after therapeutic plasma exchange in patients with myasthenic crisis

Introduction We sought to evaluate quality of life patients with myasthenic crisis before and after therapeutic plasma exchange. Materials and methods In our study we conducted an assessment of the quality of life with the use of the questionnaire SF-36, when executed eleven therapeutic plasma exchange. The assessment was made on baseline and after 4 weeks. We also did neurological clinical evaluation before and after TPE. Results Patients in the study showed significant improvement in quality of life after performed therapeutic plasma exchange. The changes were observed in physical functioning, which confirmed the results of the statistical significance of p<0.05. In the analysis, the assessment of mental functioning not obtained the results of statistical significance, but the results also showed improvement in self-assessment. We observed high correlation between general health and physical mental functioning, between the role limitations due to physical health problems and role limitations due to emotional problems, and general health perception and bodily pain. Conclusions Therapeutic plasma exchange significantly improves the quality of life of patients with myasthenia gravis during the crisis

Secondary progressive multiple sclerosis — from neuropathology to definition and effective treatment

Introduction. There is no single, commonly accepted, standard definition of secondary progressive multiple sclerosis (SPMS), an absence that poses a challenge for clinicians.State of the art. SPMS is characterised by inflammation, neurodegeneration and disease progression with the presence or absence of relapses. No biochemical or radiological biomarkers are currently available to indicate the precise secondary progressive course in individual patients. The retrospective approach to identifying SPMS patients raises many difficulties, especially in terms of determining the time point of progression. Currently, the most precise diagnosis of SPMS is based on the definition proposed by Lorscheider et al., where SPMS is defined as a disability progression by 1 step on the Expanded Disability Status Scale (EDSS) in patients with EDSS ≤ 5.5 or of 0.5 EDSS steps in patients with EDSS ≥ 6 in the absence of a relapse, a minimum EDSS score of 4 and pyramidal functional system (FS) score of 2, and confirmed progression over ≥ 3 months, including confirmation within the leading FS.Clinical implications. The need to establish criteria for the diagnosis of SPMS is currently of crucial importance due to emerging treatment opportunities including siponimod, a sphingosine 1-phosphate (S1P) receptor modulator selective for S1P1 and S1P5 receptors. It is reasonable to introduce drugs at the earliest possible stage of lesion progression to reduce inflammation a nd t o p rotect t he c entral n ervous s ystem ( CNS) a gainst i rreversible n eurodegeneration.Future directions. Further studies with prospective, multicentre and long term follow-up design are needed to provide better insights into SP course in MS patients. This should be supported by radiological, biochemical and pathological evaluations to help establish reliable and sensitive biomarkers to guide clinical practice

Early predictors of injectable disease modifying drugs suboptimal response based on clinical and radiological data assessment in Polish Multiple Sclerosis patients

Background. Prospective database studies can provide useful information regarding ‘real-world’ outcomes and drug efficacy. Objective. To determine the early predictors of suboptimal treatment responses at two and three years under injectable Disease Modifying Therapy (DMT). Methods. This was a multi-centre prospective database study. Adult patients who started injectable DMTs between January 2008 and June 2013 were included. The follow-up continued until July 2014. Suboptimal treatment responses were defined as: the presence of clinical relapse and/or Expanded Disability Status Score (EDSS) progression and/or newly emerging T2 lesions or/and gadolinium enhancing lesions on magnetic resonance imaging (MRI). The parameters were assessed up to 24 months prior to, and every 12 months during, the treatment. Results. Analysis included 297 MS (multiple sclerosis) patients followed for a mean time of 2.3 ± 1.3 years (range 1–5). Within the three years of observation, the persistence and efficacy with injectable DMTs was high. With increased disability, defined by EDSS ≥ 3, the risk of treatment failure increased up to seven times, OR 7.33 in the second year radiological analysis (CI 95% : 1.69–29.2) p < 0.01, similar to over two times in the second year clinical analysis, with the baseline symptomatic hemiparesis OR 2.75 (CI 95% : 1.06–7.06) p 0.034. A high relapse rate one year prior to treatment adversely influenced the treatment success at three years, OR 3.04 (CI 95% : 1.49–8.43) p < 0.01. Conclusions . Injectable DMTs should not be chosen for treatment initiation in motoric disabled patients (EDSS ≥ 3) with a high grade of clinical activity. These drugs are effective in less active relapsing-remitting (RR) MS patients