2017, Vol 17, No 1
Primary progressive multiple sclerosis in the Polish population
Aktualn Neurol 2017, 17 (1), p. 5–14
DOI: 10.15557/AN.2017.0001

Objectives: The aim of the study was the epidemiological analysis and evaluation of selected clinical and sociodemographic factors in Polish patients with primary progressive multiple sclerosis. Methods: The study included patients from 7 provinces in central and eastern Poland registered in the Registry of Patients with Multiple Sclerosis on 31 December 2016. The incidence of various forms of the disease was compared, and clinical, demographic and social disparities between relapsing-remitting and primary progressive multiple sclerosis were analysed. Results: Of 3,199 registered patients, 2,188 persons (66.2%) had the relapsing-remitting form of multiple sclerosis, 774 (24.2%) had the secondary progressive type and 307 (9.6%) suffered from primary progressive disease. The first symptoms of primary progressive multiple sclerosis appeared almost 10 years later than in patients with the relapsing-remitting type (39.2 ± 11.4 vs. 29.8 ± 9.8). The period from the first symptoms to diagnosis was more than twice as long in patients with primary progressive multiple sclerosis (5.8 ± 3.4) as in those with relapsing-remitting disease (2.4 ± 1.6). The average degree of disability in the Expanded Disability Status Scale was similar and amounted to 3.2 ± 2.1 for relapsing-remitting and 3.6 ± 2.4 for primary progressive multiple sclerosis. The relapsing-remitting form was observed more often in women (2.4:1), and the primary progressive form appeared with equal frequency in both sexes (1:1). Disease-modifying treatment was received by 34% of patients with relapsing-remitting and in only 1.9% of patients with primary progressive multiple sclerosis. Conclusions: The primary progressive form affects approximately 10% of Polish patients with multiple sclerosis. The first symptoms appear at about 40 years of age with equal frequency in both sexes, and its diagnosis takes more than twice as much time as in the case of relapsing-remitting multiple sclerosis.

Keywords: multiple sclerosis, clinical course, primary progressive form, epidemiology
The effects of aerobic training on the functional status, quality of life, the level of fatigue and disability in patients with multiple sclerosis – a preliminary report
Aktualn Neurol 2017, 17 (1), p. 15–22
DOI: 10.15557/AN.2017.0002

Objectives: The aim of this paper was to assess the effects of aerobic training on the quality of life and fatigue in patients with multiple sclerosis. Material and methods: A total of 53 patients with known multiple sclerosis (ICD G35.0) who began a standard 4-week rehabilitation programme were included in the study. Patients were divided into two groups: AT (aerobic training) patients (n = 21), who additionally underwent training on a lower limb cycle ergometer (three 10-minute sessions per day with an hour interval), and non-AT group of patients (n = 32). Life quality assessment based on the WHOQOL-BREF scale (World Health Organization Quality of Life), an assessment of motor impairment based on the Expanded Disability Status Scale by Kurtzke (EDSS) and an assessment of the severity of fatigue using the Fatigue Severity Scale (FSS) were performed at baseline and after four weeks. Results: After completing rehabilitation programmes, an improvement in the quality of life was observed both in AT and non-AT group. However, a more significant improvement in the two evaluated aspects, physical (p = 0.001 vs. 0.01) and psychological (p = 0.001 vs. 0.05), was observed in the AT group. However, no improvement in social terms was observed. There was also a reduction in the severity of fatigue (0.03 for AT group vs. 0.15 for non-AT group). There was no statistically significant improvement in the EDSS score in any of the groups. Conclusions: Aerobic training has beneficial effects on the quality of life and the severity of fatigue in patients with multiple sclerosis.

Keywords: multiple sclerosis, aerobic training, rehabilitation, quality of life, fatigue syndrome
The role of female sex hormones in multiple sclerosis and its animal models
Aktualn Neurol 2017, 17 (1), p. 23–33
DOI: 10.15557/AN.2017.0003

Multiple sclerosis is a progressive disease of the central nervous system with a complex immunopathogenesis characterised by inflammation, demyelination and progressive loss of axons. In the studies on the complex and multifactorial pathophysiology of multiple sclerosis, an animal model of the experimental autoimmune encephalomyelitis plays an important role. Clinical and experimental data suggest autoimmune background of inflammatory responses in the central nervous system. Unfortunately, the pathology of multiple sclerosis is still not fully understood, and the currently available drugs are only able to inhibit the progression of the neurological deficits. Epidemiological data clearly show that gender significantly affects the incidence and course of multiple sclerosis. In both clinical and experimental studies, pregnancy and hormonal treatment with female sex hormones have been shown to influence the number of relapses and the progression of neurological deficits. Abundant data suggesting the role of both sex and pregnancy in multiple sclerosis gave rise to research on the role of sex hormones in this disease and new forms of treatment. In this paper, we have reviewed the available literature regarding the effects of female sex hormones on the course of multiple sclerosis, with particular emphasis on the role of estrogens and progestagens. Data from both clinical and experimental studies have been included. Estrogens and progestagens are the two most important groups of female steroid hormones. They are necessary for development of sex organs as well as for fertility and during pregnancy. It is known that these hormones are able to modulate the function of the immune system. In this review, we have focused not only on the immunomodulatory, but also on the neuroprotective and neuroregenerative role of estrogens and progestagens. We have also described the new therapeutic possibilities associated with the hormonal therapy targeting sex hormones.

Keywords: multiple sclerosis, gender, sex hormones, estrogens, progestagens
A qualitative analysis of the ACE-III profi le in the diff erential diagnosis of dementia syndromes
Aktualn Neurol 2017, 17 (1), p. 34–41
DOI: 10.15557/AN.2017.0004

Addenbrooke’s Cognitive Examination III (ACE-III) is an extended cognitive screening instrument for an early detection of cognitive impairment, initial differential diagnosis of dementia syndromes and monitoring of disease progression. ACE-III assesses attention and orientation, memory, verbal fluency, language and visuospatial function. The scale may be used by physicians and psychologists as either a stand-alone cognitive screening or an introduction to a more in-depth neuropsychological assessment. It comprises a shorter scale, Mini-ACE, which can also be used independently as a very short screening. In this paper, the ACE-III was compared with other most common cognitive screening tools, such as the Mini-Mental State Examination (MMSE) and the Montreal Cognitive Assessment (MoCA). We have presented the clinical utility of ACE-III profile analysis in the differential diagnosis of selected neurodegenerative diseases. We have reviewed ACE-III profiles typical for late and early onset Alzheimer’s disease, frontotemporal lobar degeneration syndromes, Parkinson’s disease and atypical parkinsonian syndromes as well as vascular dementia. Among Alzheimer’s disease atypical variants, posterior cortical atrophy, logopenic variant of primary progressive aphasia and frontal variant have been discussed. As regards the frontotemporal lobar degeneration spectrum, the behavioural variant of frontotemporal dementia, non-fluent and semantic variants of primary progressive aphasia have been presented. Finally, among parkinsonism plus syndromes, dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy and corticobasal degeneration have been reviewed.

Keywords: dementia, cognitive dysfunction, Alzheimer’s disease, frontotemporal lobar degeneration, primary progressive aphasia
Liponerv – new possibilities for helping patients with mild cognitive impairment
Aktualn Neurol 2017, 17 (1), p. 42–48
DOI: 10.15557/AN.2017.0005

Mild cognitive impairment is a clinical state that is defined as intermediate between normal functioning and the first stage of dementia. It involves the deterioration of memory and other cognitive areas. With time some patients develop Alzheimer’s disease-related dementia. As societies age, the prevalence of such symptoms is likely to increase dramatically. Multiple observations indicate an important role of polyunsaturated omega-3 fatty acids in limiting the progression of brain lesions. This is due to the fact that these acids influence pathological processes associated with neurodegeneration such as beta-amyloid accumulation, chronic inflammation, oxidative stress and accelerated apoptosis. In recent years many protective mechanisms of action of fatty acids have been described, including multidirectional inhibition of beta-amyloid toxicity, anti-inflammatory and neuroprotective action of their metabolites, reduction of cellular damage in response to oxidative stress and stimulation of neural cell growth and differentiation. Alpha-lipoic acid is a strong antioxidant which may help to protect polyunsaturated fatty acids and additionally inhibit neuronal damage. Liponerv is a new formulation which contains both acids: DHA (docosahexaenoic acid), EPA (eicosapentaenoic acid), and alpha-lipoic acid. The combination of these two ingredients was evaluated in a pilot clinical study which demonstrated an improvement in everyday cognitive and instrumental abilities of patients with diagnosed Alzheimer’s disease. A number of studies indicate that long-term administration of such a combination may have a positive impact on the condition of brain tissue in individuals without dementia who experience deterioration in their cognitive abilities. Liponerv may be an interesting supplementary therapeutic option in the overall management of these patients. It should undoubtedly be characterised by safety of use and good tolerability, which is indicated by clinical experience with similar products.

Keywords: mild cognitive impairment, polyunsaturated fatty acids, alpha-lipoic acid, anti-inflammatory mediators, oxidative stress, neuroprotection
Autoimmune anti-N-methyl-D-aspartate receptor encephalitis – current state of knowledge overview based on the clinical case
Aktualn Neurol 2017, 17 (1), p. 49–54
DOI: 10.15557/AN.2017.0006

Autoimmune anti-N-methyl-D-aspartate receptor encephalitis was identified in 2007. It is a rare encephalitis (approximately 4%) of the limbic type and is typically diagnosed in young females with paraneoplastic teratomas. Cerebral NMDA receptor blockade leads to characteristic symptoms. GABA neuron inactivation results in psychotic disorders, involuntary movements, fasciculations and nystagmus. Moreover, by affecting the respiratory centre in the brainstem, it can cause respiratory disorders requiring mechanical ventilation. Other symptoms, such as sialorrhea, arrhythmias or arterial hypertension, result from the influence on the autonomous nervous system. NMDA receptor disorders are functional in nature and are mostly reversible. That is why proper diagnosis and prompt treatment, involving tumour removal and immunotherapy, may bring positive therapeutic effects. Approximately 75% of patients with autoimmune anti-NMDA receptor encephalitis have recovered completely or the disease has left only slight consequences. However, it has led to severe deficits in the remaining patients and was fatal in 7% of cases. The paper presents a case of a 23-year-old female with autoimmune encephalitis associated with ovarian teratoma. The disease was diagnosed late and its course was severe with numerous complications and necessity of intensive care. After several months, the patient regained full intellectual functions and intends to continue studying law. The aim of this report was to draw attention to a rare cause of autoimmune encephalitis, which when detected early makes it possible to obtain full recovery. Particular attention should be paid to young women who manifest atypical clinical signs within the central nervous system without tangible changes in imaging. This should prompt the search for a tumour in the abdominal cavity.

Keywords: autoimmune encephalitis, anti-N-methyl-D-aspartate receptor, ovarian teratoma, intensive care