2019, Vol 19, No 2
Parkinson’s disease – the role of the digestive tract in pathogenesis
Aktualn Neurol 2019, 19 (2), p. 55–61
DOI: 10.15557/AN.2019.0009

Parkinson’s disease is a common neurodegenerative disease in which α-synuclein deposits are aggregated in the brain, especially in the substantia nigra. Many studies indicate that this process may begin in the olfactory bulb and the enteric nervous system, and then spread through the vagus nerve and the olfactory route to further areas of the brain, many years before the onset of motor symptoms. This probably results in a much earlier occurrence of non-motor disorders, such as constipation or hyposmia. Intestinal inflammation in Parkinson’s disease may lead to increased intestinal permeability, referred to as leaky gut syndrome, which leads to microbial translocation and might trigger accumulation of α-synuclein in the enteric nervous system. Recently, more attention is paid to the influence of mutual interactions between the central nervous system and the intestines, which led to the definition of the gut–brain axis, involving neuronal, endocrine and immunological factors. Increased expression of proinflammatory cytokines in the intestines, glial intestinal reaction or the presence of α-synuclein deposits in the enteric nervous system are just some of the evidence for gastrointestinal involvement in the pathogenesis of Parkinson’s disease. Environmental exposure plays a key role in the formation of the composition and functioning of the intestinal microbiome and may increase susceptibility to neurodegenerative diseases such as Parkinson’s disease. Therefore, the intestinal microbiome in Parkinson’s disease has recently become a potential target in preventive strategies.

Keywords: Parkinson’s disease, gut microbiota, gut–brain axis, enteric nervous system
Stiff-person syndrome – a literature review
Aktualn Neurol 2019, 19 (2), p. 62–73
DOI: 10.15557/AN.2019.0010

Stiff-person syndrome, also known as stiff-man syndrome (SMS) or the Moersch–Woltman syndrome, was first described in 1956. The disease is rare. The estimated prevalence in the general population is 1–2 cases/1,000,000, and the annual incidence is 1 case per 1,000,000. It is 2–3 times more common in females. Symptoms usually occur in 20–50-year-olds; childhood-onset stiff-man syndrome accounts for 5% of cases. Based on the pathogenesis of the disorder, paraneoplastic, autoimmune and cryptogenic stiff-person syndrome have been described, whereas clinicians distinguish between classical stiff-person syndrome and its variants. Diagnostic criteria for the syndrome have been developed and it was shown that overlapping autoimmune diseases increase the risk of the disorder. There are two main clinical presentations of the disorder: 1) muscle stiffness in the trunk and limbs due to simultaneous contraction of agonist and antagonist muscles, and 2) the above clinical picture concomitant with superimposed episodic muscle spasms in the absence of pyramidal and extrapyramidal disorders. Although stiff-person syndrome is associated with antibodies against glutamic acid decarboxylase and amphiphysin, their presence is not necessary for the diagnosis. Patients with paraneoplastic syndrome, which accounts for 5–10% of cases, should be always screened for cancer. The treatment should be multidirectional and include: 1) immunomodulation, 2) symptomatic treatment, 3) monitoring, diagnosis and treatment of overlapping autoimmune and/or neoplastic diseases.

Keywords: stiff-person syndrome, stiff-man syndrome, glutamic acid decarboxylase, amphiphysin, diagnostic criteria
Non-pharmacological interventions on cognitive functions in mild cognitive impairment and their efficacy in the light of meta-analyses and systematic reviews
Aktualn Neurol 2019, 19 (2), p. 74–82
DOI: 10.15557/AN.2019.0011

Recent decades have witnessed a growing interest in the possibilities of non-pharmacological modulation of cognitive functions in older patients using cognitive interventions, such as cognitive training, cognitive stimulation and cognitive rehabilitation. These approaches are of particular importance in the group of patients with mild cognitive impairment. Since the increased risk of dementia coincides with the possibility of improving cognitive functioning, mild cognitive impairment is considered a condition potentially prone to intervention. The paper presents a systematic literature review of papers that synthesise the results of research on the efficacy of cognitive interventions in patients with mild cognitive impairment. We included 14 out of 136 publications in the review: 4 meta-analyses and 10 systematic reviews, which yielded a total number of 76 studies. The literature analysis led us to conclude that there seems to be clear evidence indicating beneficial, though modest, effects of cognitive training on cognitive functioning, memory in particular. Although the conclusions are less clear in the case of cognitive rehabilitation, they suggest improved behavioural indicators. Studies on the efficacy of cognitive stimulation in mild cognitive impairment are virtually missing in meta-analyses and systematic reviews. At the same time, authors of most of the analysed papers express some methodological reservations regarding these studies. Furthermore, the used interventions and diagnostic criteria are very heterogeneous, which makes the synthesis difficult. As a result of the current quality of evidence for the efficacy of cognitive interventions in patients with mild cognitive impairment, these interventions are assigned not more than the lowest clinical recommendation level. However, in the light of possible improvement of research methodology and the lack of recommendations for any of the available pharmacological therapies for mild cognitive impairment, cognitive interventions remain a promising approach.

Keywords: mild cognitive impairment, cognitive therapy, cognitive function training, cognitive rehabilitation, cognitive stimulation
Selected versions of the clock test in clinical practice – a comparative analysis of quantitative and qualitative scoring systems
Aktualn Neurol 2019, 19 (2), p. 83–90
DOI: 10.15557/AN.2019.0012

Throughout the years, the clock test has achieved widespread clinical use in assessing cognitive function in patients with dementia. Both the clock test and Mini-Mental State Examination scale are the most common and simple tools for assessing cognitive functions. They are also part of numerous more complex screening methods. There are several versions of clock test, which vary in their scoring systems and can be performed in different ways. Initially, the test was primarily used for the hemispatial neglect. With time, however, it has become one of the main screening tools for cognitive functions in Alzheimer’s disease and vascular dementia. It is also used in other types of dementia, which are dominated by executive function disorders and visuospatial deficits, such as dementia with Lewy bodies and dementia in Parkinson’s disease. The availability of different versions of the clock test allows for the choice of an appropriate scoring system depending on time available, skills, experience and knowledge of the medical personnel. As a psychometric tool, the clock test is a screening method to be used by doctors, which should help quickly and easily identify dysfunctions and their severity. For this reason, most versions of the test are quantitative, the scoring system is simple, and the evaluation procedure is easy to conduct. The aim of this paper was to analyse and compare the quantitative and qualitative scoring systems of this valuable screening tool.

Keywords: dementia, clock test, cognitive function, cognitive impairment
Can dietary interventions delay the conversion to dementia in people with mild cognitive impairment?
Aktualn Neurol 2019, 19 (2), p. 91–96
DOI: 10.15557/AN.2019.0013

The lack of effective causative and symptomatic pharmacotherapy in mild cognitive impairment, which significantly increases the risk of rapid development of dementia, has directed the interests of researchers towards preventive methods. In addition to cognitive, physical and social activity as well as health prevention, dietary interventions are among the most important measures. In addition to promoting the Mediterranean and Norwegian diets, a special role is attributed to specific nutritional ingredients. Current research focuses on the role of caffeine, soy lecithin, ginkgo biloba, ginseng, vitamins B (folic acid and cobalamin) and D (mainly cholecalciferol), and omega-3 fatty acids (eicosapentaenoic acid and docosahexaenoic acid in particular). The research on the role of the above mentioned components did not fully allow for an unequivocal determination of their effects on improving cognitive function. This is due to the heterogeneity of mild cognitive impairment, which results in studies conducted in patients with aetiology other than neurodegenerative as well as healthy or demented subjects. This prevents a complete and accurate assessment of the effect limited to individuals at an increased risk of dementia. Recent data indicate that a combination of certain substances has a protective effect on the development of dementia in patients with mild cognitive impairment to a much greater extent than when these ingredients are consumed separately. Importantly, geographic, cultural, social and economic circumstances of patients do not always allow for effective interventions. The article addresses the issue of the available ready-to-use preparations enhancing cognitive function in patients with mild cognitive impairment in the light of the concept and the diagnosis of these disorders as well as the possibility of preventing dementia.

Keywords: mild cognitive impairment, diet supplements, nutraceutics, prevention, dementia
Role of drinking coffee in the prevention of dementia
Aktualn Neurol 2019, 19 (2), p. 97–102
DOI: 10.15557/AN.2019.0014

The prevention of dementia is an increasingly common and applied strategy to deal with this problem. Researchers are looking for further preventive measures and examine both well-known methods and various substances – including coffee and its ingredients, mainly caffeine. Since presumably caffeine reduces the incidence of diseases that are risk factors for developing dementia, the role of coffee in preventing neurodegenerative diseases is becoming increasingly important. The results of studies evaluating the effects of coffee on neurodegenerative processes remain ambiguous at present, however, the consumption of coffee does not pose a risk for developing dementia. Data on the protective effect of coffee on the known risk factors of the emergence of a clinical manifestation of neurodegenerative diseases, i.e. diabetes mellitus, hypertension, obesity, and depression, clearly indicate a beneficial impact of caffeine and other coffee ingredients on the reduction of the prevalence of these conditions in people regularly drinking coffee. Increasing the amount of coffee consumed translates into a positive effect on lowering the risk but reducing its consumption – increases the risk. Besides proven beneficial effects on the body, coffee also stimulates physical, cognitive and social activity, and improves the effects of the actions taken and it should be remembered that social isolation, sedentary lifestyle and insufficient cognitive activity also contribute to accelerating the development of dementia. The article discusses the role and importance of coffee consumption in the context of risk factors for the development of neurodegenerative diseases.

Keywords: coffee, caffeine, risk factors, prevention, dementia
Neuropsychiatric and neuropsychological deficits in a 33-year-old woman with chorea-acanthocytosis. A case report and a literature review
Aktualn Neurol 2019, 19 (2), p. 103–110
DOI: 10.15557/AN.2019.0015

Neuroacanthocytosis – phenotypically and genetically heterogeneous disorders associated with acanthocytosis – are a group of abnormalities which affect the basal ganglion, causing movement disorders, other neurological symptoms and also cognitive and neuropsychiatric impairments. There are few reports on neuropsychiatric and neuropsychological symptom development in the course of the disease. We described a 33-year-old female patient, who was diagnosed with choreaacanthocytosis associated with neurological, psychiatric and neuropsychological disorders. The neurological disorders included increasing involuntary movements of face and tongue, upper limb chorea, dysarthric speech, slight upper limb deep reflexes, vivid knee jerks, tonic-clonic seizures and absence of Achilles reflexes. Psychiatrically, she was depressed and presented with obsessive thinking. Neuropsychological assessment revealed increasing dysfunctions of attention, immediate memory, learning, verbal fluency, praxia, calculia and difficulties in adjusting behaviour to environmental conditions and in flexible correction of wrong responses. Neuropsychiatric and neuropsychological dysfunctions should be considered in the differential diagnosis to ensure proper diagnosis and management, especially when differentiating disorders in patients with neuropsychiatric symptoms, chorea, or in the case of late (in adulthood) onset of Tourette’s syndrome.

Keywords: acanthocytes, cognition, cognitive decline, movement disorder, neuroacanthocytosis