Ryszard Podemski

Different patterns of activation markers expression and CD4+ T-cell responses to ex vivo stimulation in patients with clinically quiescent multiple sclerosis (MS)

Patients with relapsing-remitting (RR) and secondary progressive (SP) forms of multiple sclerosis (MS), although in long-term clinical remission, showed different patterns of increased expressions of the activation markers: CD69, CD40L, and both membrane/surface and cytoplasmic CTLA-4 (mCTLA-4 and cCTLA-4, respectively) in freshly isolated peripheral blood (PB) CD4+ T cells compared with controls. Also observed were dysregulated responses to ex vivo stimulation in both groups of MS patients accompanied by increased IFN-gamma synthesis. Our findings may suggest that the mechanisms leading to each clinical form of the disease may be heterogeneous.

Antibodies to 46-kDa retinal antigen in a patient with breast carcinoma and cancer-associated retinopathy

Cancer-associated retinopathy (CAR) is a rare paraneoplastic syndrome usually associated with small-cell lung carcinoma and serum autoantibodies against recoverin. We report the breast cancer woman with visual impairments and electrophysiological abnormalities characteristic of CAR. Her serum contained high-titer antibodies against α-enolase but not against other retinal proteins. This suggests that anti-enolase antibodies could be responsible for the development of CAR symptoms.

Parry–Romberg syndrome: clinical, electrophysiological and neuroimaging correlations

Parry–Romberg syndrome (PRS) is a rare disorder, described in the nineteenth century by Caleb Parry and Moritz Romberg, characterized by acquired and slowly progressive atrophy of one side of the face. The pathogenesis of PRS is still unclear. Immune-mediated processes are thought to be a basic factor in PRS etiology, but autonomic nervous system might also be impaired. A case of PRS in a 26-year-old woman with coexisting disturbances in the lower left limb is presented. The multimodal electrophysiological studies were done, including electroencephalography, visual, brain auditory, somatosensory and trigeminal somatosensory evoked potentials, blink reflex, standard neurographic and electromyographic examinations, quantitative sensory tests and autonomic tests. Neuroimaging studies consisted of brain MR, single voxel proton MR spectroscopy, diffusion tensor imaging with fiber tractography. Based on multimodal electrophysiological and neuroimaging studies, it was concluded that the impairment in PRS is multisystemic, i.e., motor, sensory, and autonomic. A cortical origin of the symptoms is possible.

Neuropsychological testing and event-related potentials in the assessment of cognitive performance in the patients with multiple sclerosis—A pilot study

AIMS The aim of the study was to evaluate cognitive impairment in multiple sclerosis (MS) patients using neuropsychological testing (NT) and auditory event-related potentials (ERPs) with reference to clinical variables, with an attempt to re-assess NT and ERP results after a year. METHODS The study comprised 21 patients with MS. ERPs results were compared to age-matched controls. Correlations were searched among ERPs and NT results, duration of MS and disability. NT and ERPs were repeated after a year and their results were compared with the initial ones. RESULTS In NT, 90-100% of patients showed impaired memory and attention. Latencies of ERPs were prolonged in patients compared with controls. NT results correlated with clinical variables and N2 parameters. Results of NT, but not ERPs, improved after a year. CONCLUSIONS MS patients present with moderate cognitive impairment and ERP abnormalities, with dysfunction of subcortical-prefrontal circuit as their possible background. NT are more useful than ERP in monitoring cognitive performance in MS patients.

The possible meaning of fractional anisotropy measurement of the cervical spinal cord in correct diagnosis of amyotrophic lateral sclerosis

Diagnosis of amyotrophic lateral sclerosis (ALS) is based on clinical criteria and electrophysiological tests (electromyography, and transcranial magnetic stimulation). In the search for ALS biomarkers, the role of imaging procedures is currently emphasized, especially modern MR techniques. MR procedures were performed on 15 ALS patients and a sex- and age-matched control group. The MR examinations were performed with a 1.5-T MR unit, and the protocol consisted of sagittal T1-weighed images, sagittal and axial T2-weighed images, and sagittal T2-weighed FAT SAT images followed by an axial diffusion tensor imaging (DTI) sequence of the cervical spinal cord. FA values in individual segments of the cervical spinal cord were decreased in the ALS group in comparison with the control group. After comparing FA values for anterior, posterior, and lateral corticospinal columns, the greatest difference was observed between the C2 and C5 segments. Spinal cord assessment with the use of FA measurements allows for confirmation of the motor pathways lesion in ALS patients. The method, together with clinical criteria, could be helpful in ALS diagnosis, assessment of clinical course, or even the effects of new drugs. The results also confirmed the theory of the generalized character of ALS.

PD-1 gene polymorphic variation is linked with first symptom of disease and severity of relapsing-remitting form of MS

Multiple sclerosis (MS) is an inflammatory disease of the central nervous system (CNS), where inflammation, demyelination together with the axonopathy are the cardinal features on pathologic ground, with a combined genetic and environmental background. The associations of PD-1 single nucleotide polymorphisms (SNPs): PD-1.3 (in intron 4), PD-1.5 and PD-1.9 (both in exon 5) with clinical manifestation of MS in 479 south Polish individuals including 203 MS patients were analyzed. Presence of PD-1.5T allele was linked with the first manifestations of disease: diplopia and pyramidal signs - favored pyramidal signs but protected against of diplopia development. Farther, PD-1.3G/PD-1.5C/PD-1.9C haplotype significantly favored whereas GTC protected against diplopia. Besides, GTT haplotype strongly favored non-severe RRMS outcome and ATC haplotype was specific only for these MS patients. Our population-based case-control study, investigating selected three PD-1 SNPs: PD-1.3, PD-1.5 and PD-1.9, revealed that polymorphic variation may be rather disease-modifying than MS risk factor.

Profile of autonomic dysfunctions in patients with primary brain tumor and possible autoimmunity

OBJECTIVE Cerebral lesion due to different neurological conditions could be complicated by autonomic dysfunction, reported in the literature as a sympathetic hyperactivity. The mechanisms of dysautonomia still remains partial. The aim of the study was to assess the profile of autonomic dysfunction in patient with primary brain tumors, with attempt to estimate the additional factors in pathogenesis of dysautonomia. MATERIAL AND METHODS Neurological examinations, the Lows autonomic disorder questionnaire, electrophysiological autonomic tests (Heart Rate Variability test at rest and during deep breathing, spectral analysis of R-R intervals, sympathetic skin response test), studies of peripheral nerves, blood sampling collection for antibodies were done in 33 patients with recognized primary brain tumors. RESULTS The averaged Lows Questionnaire score in the patients group was significantly higher than in the controls, systolic blood pressure was increased, heart rate tended to be higher without significance, but heart rate variability was severe low, LF/HF ratio also tended to be higher in the patients group. In SSR test the amplitude of responses from hand and foot was significantly lower without changes in their latencies. We found changes in the electrophysiological tests of peripheral nerves, and positive anti-neural antibodies in 5 patients. CONCLUSIONS The results of the study indicated to the sympathetic nervous system hyperactivity in patients with primary brain tumors. Local brain lesion with high intracranial pressure, additional peripheral nerve damage probably in the course of autoimmunity, and direct influence of autoimmunity to the central part of autonomic nervous system are possible in the pathogenesis of dysautonomia.

Evaluation of the Degradation of the Selected Projectile, Commissural and Association White Matter Tracts Within Normal Appearing White Matter in Patients with Multiple Sclerosis Using Diffusion Tensor MR Imaging - a Preliminary Study.

Summary Backround The aim of the study was to assess the impairment of the selected white matter tracts within normal appearing white matter (NAWM) in multiple sclerosis (MS) patients using diffusion tensor imaging (DTI). Material/Methods Thirty-six patients (mean age 33.4 yrs) with clinically definite, relapsing-remitting MS and mild disability (EDSS – Expanded Disability Status Scale 1–3.5) and 16 control subjects (mean age 34.4 yrs) were enrolled in the study. DTI examinations were performed on a 1.5T MR scanner. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values were obtained with a small ROI method in several white matter tracts within NAWM including: the middle cerebellar peduncles (MCP), the inferior longitudinal fasciculi (ILF), inferior frontooccipital fasciculi (IFOF), genu (GCC) and splenium of the corpus callosum (SCC), posterior limbs of the internal capsules (PLIC), superior longitudinal fasciculi (SLF) and posterior cingula (CG). There were no demyelinative lesions within the ROIs in any of the patients. Results A significant decrease in FA was found in MS patients in both the ILFs and IFOFs (p<0.001) and in the left MCP and right SLF (p<0.05), compared to the normal subjects. There were no significant differences in FA values in the remaining evaluated ROIs, between MS patients and the control group. A significant increase in ADC (p<0.05) was found only in the right PLIC and the right SLF in MS subjects, compared to the control group. Conclusions The FA values could be a noninvasive neuroimaging biomarker for assessing the microstructural changes within NAWM tracts in MS patients.

Emotional prosody expression in acoustic analysis in patients with right hemisphere ischemic stroke

OBJECTIVES The role of the right cerebral hemisphere in nonverbal speech activities remains controversial. Most research supports the dominant role of the right hemisphere in the control of emotional prosody. There has been significant discussion of the participation of cortical and subcortical structures of the right hemisphere in the processing of various acoustic speech parameters. The aim of this study was an acoustic analysis of the speech parameters during emotional expression in right hemisphere ischemic strokes with an attempt to reference the results to lesion location. MATERIALS AND METHODS Acoustic speech analysis was conducted on forty-six right-handed patients with right-middle cerebral artery stroke, together with 34 age-matched people in the control group. We compared the results of acoustic studies between patients with varying infarct locations and the control group. RESULTS Variations in fundamental frequency during verbal expression of joy, anger and sadness were significantly smaller in the patient group than in the control group. Cortical lesion caused more restrictions in fundamental frequency variation in the expression of joy and a lower voice intensity in expressions of anger and joy compared to those patients with subcortical lesions. CONCLUSIONS Cortical lesion was associated with a more impaired expression of emotional prosody than subcortical lesion. The results indicate the leading role of the cortical structures of the right hemisphere in the expression of emotional prosody.

Mild cognitive impairment and event-related potentials in patients with cerebral atrophy and leukoaraiosis

ObjectiveThe influence of cerebral atrophy and leukoaraiosis (LA) on the degree and profile of cognitive impairment remains unclear.DesignThe aim of the study was to assess neuropsychological features of cognitive performance and parameters of event-related potentials (ERP) in subjects with generalised cerebral atrophy and LA.SettingDepartment of Neurology, University of Medicine.Patients and participantsForty-two patients with LA and/or cerebral atrophy and twenty controls.Measurements and resultsNeuropsychological testing (NT) included Mini Mental State Examination (MMSE), Auditory Verbal Learning Test (AVLT) and Trail Making Test (TMT). Auditory ERPs were performed and parameters of the N2 and P3 components were compared in the patients and controls. Relationships were analysed between radiological indices of atrophy and LA, and NT and ERP results. Results of NT suggested generalised mild cognitive impairment in all the patients. P3 and N2 latencies were longer in the patients than in controls, especially in the LA subgroup. Correlations were found for indices of atrophy, AVLT and ERP parameters. There was a predominant influence of age upon ERP parameters and radiological indices.ConclusionsCerebral atrophy and LA result in deficits in memory and attention. NT and ERP may be used as complementary methods in the assessment of cognitive impairment in patients with cerebral atrophy and LA.