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Background. MicroRNAs are endogenous, small noncoding RNA molecules that play a pivotal role in the regulation of gene expression. MicroRNAs are involved in many biological processes such as proliferation, cell differentiation, neovascularization, and apoptosis. Studies on microRNA expression may contribute to a better understanding of the pathomechanism of chronic inflammatory demyelinating polyneuropathy (CIDP) and consequently enable the development of new therapeutic measures using antisense miRNAs (antagomirs). In this study, we evaluated the level of miR-31-5p in the serum of patients with CIDP and its correlation with the miR-31-5p level and clinical presentation and electrophysiological and biochemical parameters. Methods. The study group consisted of 48 patients, mean age 61.60 ± 11.76 , who fulfilled the diagnostic criteria of a typical variant of CIDP. The expression of miR-31-5p in patient serum probes was investigated by droplet digital PCR. The results were correlated with neurophysiological findings and the patient’s clinical and biochemical parameters. Results. The mean copy number of miRNA-31 in 100 μl serum was 1288.64 ± 2001.02 in the CIDP group of patients, while in the control group, it was 3743.09 ± 4026.90 . There was a significant positive correlation (0.426) between IgIV treatment duration and miR-31-5p expression. Patients without IgIV treatment showed significantly lower levels of miR-31 compared to the treated group ( 259.44 ± 304.02 vs. 1559.48 ± 2168.45 ; p = 0.002 ). The group of patients with body weight > 80 kg showed statistically significantly lower levels of miRNA-31-5p than the patients with lower body weight ( 934.37 ± 1739.66 vs. 1784.62 ± 2271.62 , respectively; p = 0.014 ). Similarly, the patients with elevated cerebrospinal fluid (CSF) protein levels had significantly higher miRNA-31-5p expression than those with normal protein levels ( 1393.93 ± 1932.27 vs. 987.38 ± 2364.10 , respectively; p = 0.044 ). Conclusion. The results may support the hypothesis that miR-31-5p is strongly involved in the autoimmune process in CIDP. The positive correlation between higher miR-31-5p levels and duration of IVIg treatment may be an additional factor explaining the efficacy of prolonged IVIg therapy in CIDP.

The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is based on a combination of clinical, electrodiagnostic and laboratory features. The different entities of the disease include chronic immune sensory polyradiculopathy (CISP) and autoimmune nodopathies. It is debatable whether CIDP occurring in the course of other conditions, i.e., monoclonal IgG or IgA gammopathy, should be treated as a separate disease entity from idiopathic CIDP. This study aims to evaluate the molecular differences of the nodes of Ranvier and the initial axon segment (AIS) and juxtaparanode region (JXP) as the potential cause of phenotypic variation of CIDP while also seeking new pathomechanisms since JXP is sequestered behind the paranode and autoantibodies may not access the site easily. The authors initially present the structure of the different parts of the neuron and its functional significance, then discuss the problem of whether damage to the juxtaparanodal region, Schwann cells and axons could cause CIDP or if these damages should be separated as separate disease entities. In particular, AIS’s importance for modulating neural excitability and carrying out transport along the axon is highlighted. The disclosure of specific pathomechanisms, including novel target antigens, in the heterogeneous CIDP syndrome is important for diagnosing and treating these patients.

Rozna odpowiedź na leczenie u pacjentow z przewleklą zapalną poliradikuloneuropatią demielinizacyjną (CIDP, chronic infl ammatory demyelinating polyradiculoneuropathy ) moze sugerowac odmienności w etiopatogenezie u poszczegolnych chorych. W artykule omowiono typową i atypową postac CIDP, ze szczegolnym uwzglednieniem statusu pacjentow pod wzgledem wykrywanych przeciwcial, z nastepczym wyodrebnieniem roznych postaci choroby z przewidywaną odpowiedzią na leczenie. Nieprawidlowe rozpoznanie CIDP ma znamienne implikacje dla rokowania i leczenia. Oznaczenie przeciwcial patogennych dla CIDP pomaga w planowaniu terapii. Istotne jest wyodrebnienie grupy chorych z krotkim czasem trwania okna skuteczności leczenia immunoglobulinami, a takze grupy z opornością na immunoglobuliny. W pierwszej grupie mozna osiągnąc efekt terapeutyczny po zwiekszeniu dawki i frakcjonowaniu leku.

The spectrum of immune-mediated neuropathies is broad and the different subtypes are still being researched. With the numerous subtypes of immune-mediated neuropathies, establishing the appropriate diagnosis in normal clinical practice is challenging. The treatment of these disorders is also troublesome. The authors have undertaken a literature review of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), Guillain–Barre syndrome (GBS) and multifocal motor neuropathy (MMN). The molecular, electrophysiological and ultrasound features of these autoimmune polyneuropathies are analyzed, highlighting the differences in diagnosis and ultimately treatment. The immune dysfunction can lead to damage to the peripheral nervous system. In practice, it is suspected that these disorders are caused by autoimmunity to proteins located in the node of Ranvier or myelin components of peripheral nerves, although disease-associated autoantibodies have not been identified for all disorders. The electrophysiological presence of conduction blocks is another important factor characterizing separate subgroups of treatment-naive motor neuropathies, including multifocal CIDP (synonyms: multifocal demyelinating neuropathy with persistent conduction block), which differs from multifocal motor neuropathy with conduction block (MMN) in both responses to treatment modalities and electrophysiological features. Ultrasound is a reliable method for diagnosing immune-mediated neuropathies, particularly when alternative diagnostic examinations yield inconclusive results. In overall terms, the management of these disorders includes immunotherapy such as corticosteroids, intravenous immunoglobulin or plasma exchange. Improvements in clinical criteria and the development of more disease-specific immunotherapies should expand the therapeutic possibilities for these debilitating diseases.

Objectives Primary Sjögren’s syndrome (pSS) is an autoimmune, multisystem exocrinopathy characterized by dysfunction of the exocrine glands. Central nervous system (CNS) involvement is estimated to be present in 10–60% patients with pSS. The present study aimed to evaluate brainstem auditory evoked potentials (BAEP) in pSS patients without central nervous system involvement, and without otolaryngological abnormalities. Method Thirty-six pSS patients (35 women, 1 man, mean age 48 years old) and 40 healthy volunteers were investigated. BAEP, brain imaging, laboratory parameters, and clinical neurological and otolaryngological examinations were performed. Results Abnormal BAEP were recorded in 16.7% patients. The mean wave BAEP I and V latency and mean wave III-V and I-V interpeak latencies were significantly longer in pSS patients than the controls. There were no statistically significant correlations between BAEP parameters and laboratory tests. None of the patients was found having the abnormalities on brain imaging. Conclusions This study confirms that in pSS patients without clinical central nervous system impairment, auditory pathway disturbances could be found. Disorders of brain bioelectrical activity may be a consequence of ongoing autoimmune process. Key Points• BAEP abnormalities confirmed the clinically observed involvement of the central nervous system in patients with pSS.• Brain bioelectrical activity dysfunctions in pSS patients may be a consequence of ongoing inflammatory and/or immunological processes.

Introduction Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disease of the peripheral nervous system, sometimes including the central nervous system. The aim of the study was the assessment of the prevalence of central sensory impairment and its reliance on peripheral nerve damage in patients with CIDP. Material and methods Multimodal (visual—VEP, brainstem auditory—BAEP, somatosensory—SEP) evoked potentials (EPs) were studied in 24 patients diagnosed with CIDP. The results were compared with neurographic parameters of sensory responses. The control group consisted of 35 healthy volunteers selected with respect to age and sex. Results Mean latency of most components of EP were considerably prolonged in patients compared with the control group. There were no correlations between the P100 VEP latency and the peripheral sensory parameters. Statistically significant negative correlations were obtained between BAEP and SEP responses and the amplitude and sensory conduction velocity of peripheral nerves. The inter-latencies were also longer. Conclusions The authors indicated to the possibility of central sensory involvement in patients with CIDP, especially based on the prolonged inter-latency of BAEPs with simultaneously confirmed root affection. The severity of central damage correlates with the degree of peripheral nerve impairment.

Strokes are the main cause of death and long-term disability worldwide. A stroke is a heterogeneous multi-factorial condition, caused by a combination of environmental and genetic factors. Monogenic disorders account for about 1% to 5% of all stroke cases. The most common single-gene diseases connected with strokes are cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) Fabry disease, mitochondrial myopathy, encephalopathy, lactacidosis, and stroke (MELAS) and a lot of single-gene diseases associated particularly with cerebral small-vessel disease, such as COL4A1 syndrome, cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL), and Hereditary endotheliopathy with retinopathy, nephropathy, and stroke (HERNS). In this article the clinical phenotype for the most important single-gene disorders associated with strokes are presented. The monogenic causes of a stroke are rare, but early diagnosis is important in order to provide appropriate therapy when available.

Introduction: The aim of this study is a comprehensive analysis of the parameters of exogenous evoked potentials (visual, brainstem auditory, and somatosensory) in patients with myasthenia gravis (MG), a prototype of both neuromuscular junction disease and autoimmune disease. The study also seeks to isolate electrophysiological changes that may indicate disorders within the central and/or peripheral nervous system. Methods: A total of forty-two consecutive patients with myasthenia gravis (24 women, 18 men) were included in the study. All of the patients underwent EP examination. MR images were also analyzed. Results: In the group of MG patients, the latency of P100 (113.9 ± 13.9, p &lt, 0.0001) VEP, wave III (3.92 ± 0.29, p = 0.015), wave V (5.93 ± 0.32, &lt, 0.0001), interlatency III–V (2.00 ± 0.12, 0.0001), interlatency I–V (4.20 ± 0.28, 0.001) BAEP, and all components of SEP (N9, P10, N13, P16, N20, P22) were significantly longer. Mean wave I and V amplitude BAEP were relatively lower. Conclusions: The results of the study suggest the presence of disturbances in the bioelectric activities of the central and peripheral nervous system in MG patients.

The goal of this study was to analyse, in relation to electrophysiological results, the distribution of lymphocyte subpopulations and the level of cytokines in patients with the typical form of chronic demyelinating inflammatory polyneuropathy (CIDP) before immunoglobulin treatment. The study group consisted of 60 patients (52 men, eight women), with a mean age 64.8 ± 11.2, who fulfilled the diagnostic criteria for the typical variant of CIDP, with (23 patients) and without (37 patients) diabetes mellitus. We analysed the results of the neurophysiological tests, and correlated them with the leukocyte subpopulations, and cytokine levels. In CIDP patients, IL-6, IL-2, IL-4 and TNF-α levels were significantly increased compared to the control group. Fifty patients had decreased levels of T CD8+ lymphocytes, and 51 patients had increased levels of CD4+ lymphocytes. An increased CD4+/CD8+ ratio was also found. Negative correlations were observed mainly between compound muscle action potential (CMAP) amplitudes and cytokine levels. The study enabled the conclusion that electrophysiological parameters in CIDP patients are closely related to the autoimmune process, but without any clear differences between patients with and without diabetes mellitus. Correlations found in the study indicated that axonal degeneration might be independent of the demyelinating process and might be caused by direct inflammatory infiltration.

The effects of epilepsy on sleep and the activating effects of sleep on seizures are well documented in the literature. To date, many sleep-related and awake-associated epilepsy syndromes have been described. The relationship between sleep and epilepsy has led to the recognition of polysomnographic testing as an important diagnostic tool in the diagnosis of epilepsy. The authors analyzed the available medical database in search of other markers that assess correlations between epilepsy and sleep. Studies pointing to microRNAs, whose abnormal expression may be common to epilepsy and sleep disorders, are promising. In recent years, the role of microRNAs in the pathogenesis of epilepsy and sleep disorders has been increasingly emphasized. MicroRNAs are a family of single-stranded, non-coding, endogenous regulatory molecules formed from double-stranded precursors. They are typically composed of 21–23 nucleotides, and their main role involves post-transcriptional downregulation of expression of numerous genes. Learning more about the role of microRNAs in the pathogenesis of sleep disorder epilepsy may result in its use as a biomarker in these disorders and application in therapy.

Cerebral small vessel disease (CSVD) is the most common, chronic and progressive vascular disease. The changes affect arterioles, capillaries and small veins supplying the white matter and deep structures of the brain. It is the most common incidental finding on brain scans, especially in people over 80 years of age. Magnetic resonance imaging (MRI) plays a key role in the diagnosis of CSVD. The nomenclature and radiological phenotypes of CSVD were published in 2013 based on the unified position of the so-called Centres of Excellence in Neurodegeneration. The disease is characterized by a diverse clinical and radiological picture. It is primarily responsible for stroke incidents, gait disturbances, depression, cognitive impairment, and dementia in the elderly. The CSVD contributes to about 20% of strokes, including 25% of ischemic strokes and 45% of dementias. Common causes of CSVD include arteriosclerosis, cerebral amyloid angiopathy (CAA), genetic small vessel angiopathy, inflammation and immune-mediated small vessel diseases, and venous collagenosis. There is no causal treatment and management is mainly based on combating known risk factors for cardiovascular disease (CVD).

BACKGROUND Cerebrovascular disease is an important cause of epilepsy. The incidence may significantly vary (from 2.3% to 43%). Post-stroke seizures occur within 2 weeks of stroke onset (as early-onset seizures) or 2 weeks after a stroke (as late-onset seizures). OBJECTIVES To retrospectively evaluate and differentiate predictive factors for post-stroke seizures. MATERIAL AND METHODS We retrospectively analyzed the medical histories of 164 adult patients diagnosed with post-stroke seizures but no epilepsy recognized prior to the stroke who were hospitalized at the Neurology Clinic of Wroclaw Medical University between 2012 and 2018. The seizures were classified according to the criteria of the International League Against Epilepsy (ILAE) from 2017. The relevant demographic data, type of stroke (ischemic/hemorrhagic), time of occurrence of seizures in relation to the type of stroke, score on the modified Rankin Scale, presence of cardiovascular risk factors, electroencephalography (EEG) recording, and antiepileptic treatment (AED) were collected. In the case of ischemic stroke (IS), the size of the stroke lesion was rated on the ASPECTS scale. RESULTS The study involved 164 patients (average age = 68.83 years), including 86 men (average age = 66.2 years). In 20 out of 164 patients, the seizures were associated with hemorrhagic stroke (HS); in 144 out of 164 patients, the post-stroke epilepsy was associated with IS. Generalized tonic-clonic seizures occurred in 101 out of 164 patients, focal aware seizures occurred in 19 out of 164 patients and focal impaired-awareness seizures occurred in 44 out of 164 patients. CONCLUSIONS Our study has confirmed that generalized seizures occur mostly after an IS and are late complications of it. Early-onset seizures occur mostly after HS associated with severe disability. Seizures are more likely to happen due to the cortical location of the stroke. There is a shift from generalized to focal seizures with an increase in the extent of IS as evaluated using the ASPECTS scale.

Purpose: Acute cerebral venous thrombosis (CVT) is a rare condition that can lead to a serious clinical state; thus, prompt diagnosis and treatment are mandatory. Head computed tomography (CT) plays a crucial role in the initial prompt diagnosis in the emergency setting. The aim of the study was to retrospectively analyse emergency head CT studies and the rate of incorrect diagnoses and main sources of pitfalls. Material and methods: Retrospective analysis of 31 emergency CT studies (22 without contrast, 19F/12M, age range: 4-94 years) of patients with confirmed acute CVT. Results: Thrombosed dural sinuses were found in 24/31 (77.4%) cases, thrombosed veins in 7/31 (22.6%) cases, no lesions within vessels in 2/31 (6.5%) cases. Haemorrhagic brain lesions were found in 9/31 (29%) cases, hypodense oedema in 6/31 (19.6%) cases, brain swelling in 1/31 (3.2%) cases, and no parenchymal lesions were revealed in 15/31 (48.4%) cases. Correct diagnosis of CVT was established in 15 cases (48.4%); however, it was incorrect in 16 cases (51.6%). Incorrect cases consist of 4 groups: 1 - with both vascular and parenchymal lesions that were overlooked (50%), 2 - with vascular lesions only, which were either overlooked, misinterpreted, or covered by artefacts (31.3%,), 3 - with parenchymal lesions only, which were misinterpreted (12.5%), and 4 - with no lesions present in the emergency head CT (6.2%). Conclusions: The high rate of incorrect diagnoses of acute CVT based on emergency head CT requires constant training of radiologists and their close cooperation with clinicians because a delayed diagnosis may be lethal to the patient.

Background and Purpose: The purpose of this study was to assess the correlation of cTn levels in the acute phase of ischemic stroke with the extent of stroke, the degree of disability assessed on the modifi ed Rankin Scale, and their prognostic signifi cance.

Background The aim of the study was to assess microstructural changes within strategic brain regions in multiple sclerosis (MS) patients, using diffusion tensor imaging (DTI), with regard to various aspects of disability. Material and methods The study comprised 50 patients with relapsing-remitting MS (37 women, 13 men, mean age 36.4 yrs) and 27 age- and sex-matched controls. Using DTI, fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values were obtained within corpus callosum (CC), both thalami (TH) and middle cerebellar peduncles (MCP). Disability was assessed using Expanded Disability Status Scale (EDSS), MS Functional Composite (MSFC), Symbol Digit Modalities Test (SDMT) and Fatigue Severity Scale (FSS). DTI indices were compared between the patients and controls and in the MS group - referred to disability measures. Results Significant decrease in FA and increase in ADC within CC and both TH were found in MS patients compared to the controls. DTI indices within CC and TH correlated significantly with SDMT score, and within TH and MCP - with MSFC manual dexterity measure. Conclusions Changes in DTI measures in normal appearing white and grey matter in the MS patients indicate subtle alterations of the tissue integrity. An occult damage to the strategic brain regions may contribute to various aspects of disability due to MS.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common form of autoimmune polyneuropathy. It is a chronic disease and may be monophasic, progressive or recurrent with exacerbations and incomplete remissions, causing accumulating disability. In recent years, there has been rapid progress in understanding the background of CIDP, which allowed us to distinguish specific phenotypes of this disease. This in turn allowed us to better understand the mechanism of response or non-response to various forms of therapy. On the basis of a review of the relevant literature, the authors present the current state of knowledge concerning the pathophysiology of the different clinical phenotypes of CIDP as well as ongoing research in this field, with reference to key points of immune-mediated processes involved in the background of CIDP.

Visual evoked potentials (VEP) are changes in potentials that arise in the central nervous system. In the interpretation of the VEP test results, it is assumed that the elongation of the latency time is caused by the demyelination of the nerve fibers, and the axon damage is responsible for the decrease in the amplitude. The observed VEP deviations are not specific for specific diseases, but indicate disturbances in visual conductivity. VEP may play a diagnostic role in the early detection of visual involvement. The aim of the study was the functioning of visual pathway assessment on the basis of visual evoked potentials (VEP) examination, in patients with primary Sjögren’s Syndrome (pSS), without focal symptoms of central nervous system disorder. The effect of disease activity, as assessed by clinical parameters and antibody levels (anti-Ro52, SSA, and SSB), on the central nervous system was also evaluated. Thirty-two consecutive patient with pSS (31 females, 1 male) were included in the study. VEP was performed at baseline, and after 6 (T6) years. Their results were compared longitudinally between the baseline and T6, depending on the duration of the disease and treatment. The immunological activity of pSS was also analyzed. The group of patients showed a significant prolongation of the P100 implicit time (105.5 ± 5.1 vs. 100.6 ± 3.9, p = 0.000) and a significant higher the P100-N145 amplitude (12.3 ± 4.1 vs. 9.4 ± 3.0, p = 0.000). Abnormalities in electrophysiological parameters of VEP at baseline correlated with presentation of anti-Ro52 antibodies and aching joints. At baseline, the P100 implicit time was shorter for the patients with pSS than for those at T6 (105.50 ± 5.1 vs. 109.37 ± 5.67, p = 0.002). pSS patients without CNS involvement presented with dysfunction of visual pathway, as revealed by VEP abnormalities. Relationships were found between VEP parameters and with present of anti-Ro52 antibodies and aching joints. VEP may be a useful method for assessment and monitoring of subclinical visual deficit in the course of pSS.

Objectives Cognitive impairment and fatigue are regarded as important aspects of multiple sclerosis. The aim of this study was to evaluate cognitive performance, the level of fatigue and parameters of event-related potentials (ERP) in patients diagnosed with clinically isolated syndrome (CIS). Patients and methods The study comprised 44 patients with CIS and 45 healthy controls. Cognitive performance was assessed using the Brief Repeatable Battery of Neuropsychological Tests (BRBNT), fatigue - using the Fatigue Severity Scale (FSS) and Modified Fatigue Impact Scale (MFIS). Auditory ERP were performed and the parameters of N200 and P300 components were analyzed. Neuropsychological and electrophysiological measures were referred to clinical and radiological features of the disease activity. Results Forty five% of patients failed in at least one test from BRBNT, mainly within the domains of memory and attention. In 18% of patients FSS corresponded with moderate or severe fatigue. The mean latency of N200 and P300 was significantly longer and amplitude of P300 was lower in those patients with CIS than in the controls. Significant correlations were found between the results of MFIS and tests evaluating verbal memory and attention, as well as between N200 latency and results of tests for verbal memory. Conclusions Cognitive performance and fatigue deserve attention from the earliest clinical stage of MS. Abnormalities of event-related potentials in CIS suggest early impact of the disease on functional neural networks.

Background. Transient global amnesia (TGA) is a rare, benign condition characterised by a sudden deficit of anterograde and retrograde memory that usually lasts for a few hours and is not accompanied by other focal neurological symptoms or signs. Its aetiology is still unclear. Various events or activities may trigger TGA. Evidence of seasonal variations in the appearance of TGA is inconsistent. Methods. We retrospectively analysed the medical history of 114 adult patients with diagnosed TGA, hospitalised at two neurology departments in Wroclaw from 2008 to 2014. We reviewed risk factors, trigger points, and occurrence in each month of the year in our patient population. Results. Over this seven-year period, 114 patients were diagnosed with TGA. The annual occurrence ranged from 13 to 22 hospitalisations. The mean age of the patients was 64 years. There were 36 TGA events in men and 78 in women. TGA occurred most frequently in spring (36%) and summer (30%), with the incidence peaking during March. Conclusions. Our findings suggest that there is a relationship between the season of the year and the probability of TGA.

Background Primary Sjogren’s syndrom (pSS) is a chronic autoimmune disease, which, in addition to impaired function of exocrine glands, can affect other organs including nervous system. Objectives The aim of the study was to establish whether in patients with pSS without central nervous system (CNS) involvement, the function of the central portion of the sensory pathway can be challenged. Methods In 33 patients diagnosed with pSS without clinical features of CNS damage and normal head CT scan, somatosensory evoked potentials (SEP) were studied. The results were compared to other clinical parameters of the disease, particularly to immunological status. The control group consisted of 20 healthy volunteers selected with respect to age and sex. Results Mean latency of all components of SEP were considerably prolonged in patients compared to the control group. Mean interpeak latency N20-N13 (duration of central conduction TT) did not differ significantly between the groups. However, in the study group, mean amplitude of N20P22 and N13P16 was significantly higher compared to healthy individuals. In patients with PSS, significant differences in SEP parameters depending on duration of the disease, duration of arthralgia and presence of SSA and SSB antibodies were noted. No significant differences in mean SEP parameters were observed with respect to skin lesions, xerophthalmia, current joint pain and swelling, focus score, levels of C3 and C4 complement components, ESR, CRP and presence of Ro52 antibodies. Conclusions The authors confirmed central nervous system involvement often observed in patients with pSS. They also showed dysfunction of the central sensory neuron as a difference in amplitude of cortical response, which indicates subclinical damage to the CNS. Disclosure of Interest None declared

Endogenous cognitive event-related potentials (CERPs) reflect higher-level processing of sensory information and can be used to evaluate cognitive functions. The aim of this paper was to determine whether there are any abnormalities in the electrophysiological parameters of CERPs in patients with primary Sjögren's syndrome (pSS) but without symptoms of central nervous system (CNS) involvement or mental disorder. The analysis of CERP parameters was then correlated with the clinical status of the patients and with some of the immunological parameters in the patient group.Thirty consecutive patients with pSS (29 females, one male) were included in the study. All the patients underwent CERP examination.There was a significant prolongation of the latency of P300 and N200 potentials in patients with pSS. Abnormalities in electrophysiological parameters of CERPs correlated with the duration of the disease, salivary gland abnormalities, and elevated erythrocyte sedimentation rate (ESR) values. Patients with coexisting chronic fatigue syndrome (CFS) had larger P300 amplitudes. There were no statistically significant changes in the electrophysiological parameters of CERPs in patients with pSS dependent on the presence of peripheral nervous system (PNS) lesions, skin changes, arthritis, abnormalities in white blood cells and the immune system or the levels of blood lipids.The results of the study suggest the presence of a minor cognitive dysfunction in patients with pSS without symptoms of CNS involvement or mental disorder. Cognitive dysfunction correlated with the disease duration time and the severity of inflammatory changes (salivary gland abnormalities and inflammatory markers in the blood). Further and larger longitudinal studies are necessary for confirmation of this correlation.

Background Cerebral venous thrombosis (CVT) is a rare condition which constitutes 0.5-1% of all strokes. The clinical and radiological picture of CVT is non-specific and can mimic other disorders. Objectives The aim of the study was to retrospectively evaluate and correlate clinical and radiological symptoms presented by patients with CVT, both in the initial and follow-up neurological and neuroimaging examinations, with a special emphasis on diagnostic difficulties. Material and methods Material consisted of 11 patients with CVT (7 women, 4 men). The average age was 43.5, ranging from 23 to 69 years. Clinical symptoms, laboratory findings, risk factors and the results of neuroimaging examinations including CT, MRI and DSA were retrospectively analyzed and correlated. Results All subjects developed superficial CVT and 1 also deep CVT, with no parenchymal lesions in 2 cases, non-hemorrhagic infarctions in 3 and hemorrhagic lesions in 6 subjects. The most frequent symptoms were headache, seizures and hemiparesis. The major risk factors were hormonal therapies in women and congenital thrombophilia. Factors influencing the clinical course and outcome the most were location and type of brain lesions, with hemorrhagic cortical infarctions bringing the worst prognosis and being associated with the highest rate of persistent neurological deficits, despite the rate of vessel recanalization. Conclusions In our opinion, quick diagnosis before parenchymal hemorrhagic lesions are visible on CT is of crucial importance and requires a constant alertness and good cooperation of neurologists and radiologists, especially in emergency settings.

Background The central nervous system in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is involved in about 5% of patients. The aim of the study was the assessment of the prevalence of central sensory impairment, and its reliance on peripheral nerve damage in patients with CIDP. Materials and methods The study group consisted of 24 patients with mean age of 60.7, who fulfilled the diagnostic criteria of typical form of CIDP at the time of multimodal (visual - VEP, brainstem auditory - BAEP, somatosensory SEP) evoked potentials (EPs) study. Each patient underwent a neurological examination, as well as a CT/MRI scan of the head. The evoked potentials was conducted according to the International Federation of Clinical Neurophysiology (IFCN) guidelines. Results Mean latency of most components of EP were considerably prolonged in patients compared to the control group. Negative correlations of SNAP amplitudes and sensory conduction velocities in peripheral nerves were obtained for I-V and III-V inter-latencies BAEP, for N20, P16, P22 SEP and the central conduction time TT for all sensory fibers of the left upper limb nerves. Conclusions Correlations of SNAP and EP parameters indicate subclinical damage to the central sensory pathways. The CIDP pathomechanism is still unclear and needs further research.

Background Primary Sjogren’s syndrome (pSS) is a chronic autoimmune exocrinopathy characterized by dysfunction of exocrine glands. Central nervous system (CNS) involvement is estimated to be present in 3–60% patients with pSS. The aim of our study was to evaluate parameters of brainstem auditory evoked potentials (BAEP) in pSS patients without clinical neurological deficit, and without otolaryngological abnormalities. Material and methods 36 patients with pSS (35 women, 1 man, mean age 49.3 years old) and 40 healthy controls, sex and age-matched were studied. They did not suffer from disease that could have affected the brain electrical activity. Clinical neurological and otolaryngological examinations, laboratory parameters (C3 and C4 component, ANA antibodies, RF levels), brain imaging (CT or MRI) and BAEP were performed. Statistical analysis was performed using the Statistica 10.0 PL software. Results In none of the patients were the abnormalities on brain imaging found. Abnormal BAEP were recorded in 6 patients (16.6%). pSS patients had a longer mean wave I and V latency and mean wave III-V and I-V interpeak latencies than the controls. There were no correlations between BAEP parameters and laboratory tests. Conclusions 1. In pSS patients without clinical central nervous system impairment the auditory pathway disturbances could be found. 2. Disorders of the brain bioelectrical activity in the course of pSS may be associated with an ongoing autoimmune process. item The analysis of BAEP may be useful methods of assessment of the CNS in patients without clinical neurological symptom.

Background The most frequent clinical presentation of occipital or visual tract lesion is hemianopsia or quadrantanopsia. However, damage to the primary or secondary visual cortex can also manifest as visual hallucinations (photopsiae or complex phenomena). We report visual and somatosensory phenomena following cerebral venous infarction based on a study of a patient with a history of recent head injury. Case presentation We report a 61-year-old man with a history of recent head injury presented with a headache of two weeks duration. He was complaining of transient visual abnormalities, which he described as impaired ability to recognize faces, dark spots moving in the visual field and distorted contours of an objects. Clinical examination showed a balance disorder with no evidence of visual deficit. During further observation the patient started to experience more complex visual and sensory phenomena of: waving of the ceiling, clouds that he could form and feel, he had an impression of incorrect sizes of given objects, he could see a nonexistent pack of cigarettes and the character from the arcade game Pac-Man “eating” an existing drip stand. Conclusions The patient mentioned above possessing simple and complex visual and somatosensory hallucinations and illusions in the course of venous stroke. A possible mechanism involves irritation of cortical centers responsible for visual processing.

Objectives: The diagnosis of epilepsy can cause many problems, especially when the routine electrophysiological tests are inconclusive. The aim of the present study was to assess the visual evoked potential (VEP) in patients with epilepsy using various kinds of stimulation and activation tests. The VEP parameters were also presented with reference to the type of seizure and abnormalities in electroencephalogram (EEG), including the response to the activation tests. Materials and Methods: This study comprised 81 patients with newly diagnosed epilepsy of unknown etiology before initiation of the treatment. The VEP tests were performed at rest, after hyperventilation, and deprivation of sleep. Visual stimulation included an alternating checkerboard pattern and a uniform flash light (FL) with a frequency of 1.88 and 15 Hz. Results: The VEP parameters obtained with the stimulation of a checkerboard pattern did not differ significantly between the patients and controls. Neither the presence of seizure activity in EEG nor the type of seizure significantly affected the VEP parameters. Using the FL stimulation, a significantly prolonged VEP latency was found at the FL frequency of 1.88 Hz and shortened at the frequency of 15 Hz. These changes were augmented after activation tests. In case of the patients with positive intermittent photic stimulation response in EEG, a significant prolongation of P100 latency was shown at rest and after FL stimulation at the frequency of 1.88 Hz. Conclusion: Standard activation methods significantly affect the VEP parameters in patients with epilepsy. Changes in the VEP parameters depend on the frequency and the type of the stimulus, as well as the activation method used. These findings suggest a disturbed balance between the glutamatergic and GABAergic systems in the visual excitability of neuronal networks in case of patients with epilepsy.

Introduction: Paraneoplastic neurological syndrome is a rare complication of different types of cancer. Non-small cell neuro-endocrine lung cancer is very rare and there are few descriptions of paraneoplastic syndrome in patients with such types of cancer. Paraneoplastic movement disorders, among them chorea, have an exceptionally rare occurrence. Clinical presentation: A 64-year-old woman presented paraneoplastic chorea with non-typical coincidence of anti-Hu and anti-GD1b antibodies. Chorea was the last sign of the disease and was comorbid with encephalitis, chronic polyradiculoneuropathy and autonomic dysfunction; the clinical symptoms were disseminated in time. A very rare type of lung cancer i.e., poorly differentiated non-small cell neuroendocrine cancer was found. Conclusions: The diversity of the clinical picture in this patient with non-small cell neuro-endocrine lung cancer can be explained by the complexity of the neurological pathomechanisms of paraneoplastic syndromes.

Cerebellar stroke is a rare condition with very nonspecific clinical features. The symptoms in the acute phase could imitate acute peripheral vestibular disorders or a brainstem lesion. The aim of this study was to assess the usefulness of the Trial of Org 10172 in Acute Stroke Treatment (TOAST) classification in cerebellar stroke and the impact of clinical features on the prognosis. We retrospectively analyzed 107 patients with diagnosed ischemic cerebellar infarction. We studied the clinical features and compared them based on the location of the ischemic lesion and its distribution in the posterior interior cerebellar artery (PICA), superior cerebellar artery (SCA), and anterior inferior cerebellar artery (AICA) territories. According to the TOAST classification, stroke was more prevalent in atrial fibrillation (26/107) and when the lesion was in the PICA territory (39/107). Pyramidal signs occurred in 29/107 of patients and were more prevalent when the lesion was distributed in more than two vascular regions (p = 0.00640). Mortality was higher among patients with ischemic lesion caused by cardiac sources (p = 0.00094) and with pyramidal signs (p = 0.00640). The TOAST classification is less useful in assessing supratentorial ischemic infarcts. Cardioembolic etiology, location of the ischemic lesion, and pyramidal signs support a negative prognosis.

Objectives Lupus erythematosus (SLE) is a systemic disease with possible nervous system involvement, regarded as unfavorable prognostic factor. Evoked potentials (EP) are electrophysiological markers of disturbed central nervous system (CNS) function, even in subclinical stage of CNS disorders. The purpose of the study was to evaluate parameters of EP in the patients with SLE with or without neuropsychiatric manifestation (NPSLE). Methods The study comprised 53 patients with SLE (49 women, 4 men, aged 21–62, mean 38.3) and 40 healthy, age- and gender- matched controls. 33 patients were classified as having NPSLE. Visual evoked potentials (VEP) and brainstem auditory evoked potentials (BAEP) were performed in all the subjects. Latencies and amplitudes of VEP and BAEP components were compared between SLE patients and controls and between NPSLE and non-NPSLE patients. Results Mean latency of P100 component of VEP for the right eye was significantly longer (104.3 ms vs 102.06 ms; p = 0.05) and mean amplitude of the I component of BAEP for the right ear was significantly lowered (0.25 μ V vs 0.30 μ V; p = 0.04) in the SLE patients in comparison with the controls. No differences were found between NPSLE and non-NPSLE patients. Discussion Asymmetric abnormalities of EP correspond with multifocal brain damage in the course of SLE, although they are not specific for the background of the disease. Conclusions and significance Parameters of VEP and BAEP show asymmetric abnormalities in SLE patients, regardless of the clinical symptoms of CNS involvement. EP may be considered as electrophysiological markers of early CNS dysfunction in the course of SLE.