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ObjectiveThe current research aimed to analyze the alterations within the motor cortex and pyramidal pathways and their association with the degree of damage within the peripheral nerve fibers in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). To achieve that goal, we investigated the microstructural changes within the pyramidal white matter tracts using diffusion tensor imaging (DTI) parameters, evaluated metabolic alterations in both precentral gyri using magnetic resonance spectroscopy (MRS) ratios, and correlated them with the neurographic findings in patients with CIDP.MethodsThe spectroscopic ratios of NAA/Cr, Cho/Cr, and mI/Cr from both precentral gyri and the values of fractional anisotropy (FA), axial diffusivity (AD), and mean diffusivity (MD) from both of the corticospinal tracts were correlated with the results of neurological and neurographic findings. The comparison of DTI parameters between the patients and controls was performed using Student’s t-test or the Mann–Whitney U test. Due to the lack of normal distribution of most variables, Spearman’s Rho rank coefficient was used to test all correlations. All analyses were performed at a significant level of alpha = 0.05 using STATISTICA 13.3.ResultsCompared to the control group (CG), the patient group showed significantly lower ratios of NAA/Cr (1.66 ± 0.11 vs. 1.61 ± 0.15; p = 0.022), higher ratios of ml/Cr in the right precentral gyrus (0.57 ± 0.15 vs. 0.61 ± 0.08; p = 0.005), and higher levels of Cho/Cr within the left precentral gyrus (0.83 ± 0.09 vs. 0.88 ± 0.14, p = 0.012). The DTI parameters of MD from the right CST and AD from the right and left CSTs showed a strong positive correlation (0.52–0.53) with the sural sensory nerve action potential (SNAP) latency of the right sural nerve. There were no other significant correlations between other DTI and MRS parameters and neurographic results.SignificanceIn our study, significant metabolic alterations were found in the precentral gyri in patients with CIDP without clinical symptoms of central nervous system involvement. The revealed changes reflected neuronal loss or dysfunction, myelin degradation, and increased gliosis. Our results suggest coexisting CNS damage in these patients and may provide a new insight into the still unknown pathomechanism of CIDP.

ObjectiveThere is an ongoing search for markers useful in monitoring and predicting disease activity at the early stage of multiple sclerosis (MS). The goals of this study were to prospectively evaluate the changes in parameters of multimodal evoked potentials (EP) and cognition within a 3-year follow-up period in patients with clinically isolated syndrome (CIS), and to assess the prognostic value of baseline findings with regard to the disease outcomes.MethodsIn 29 patients (20 women, nine men, mean age 31.1) multimodal (visual, brainstem auditory, somatosensory, event-related) EP and neuropsychological tests (NT) were performed at baseline (T0) and after 1 (T1) and 3 (T3) years. Their results were compared longitudinally between baseline, T1, and T3. Baseline results confirmed conversion of CIS into multiple sclerosis (MS) and disability level at T1 and T3 using multiple comparisons and a logistic regression model.ResultsApart from mean N13/P16 SEP (somatosensory evoked potentials) amplitude (lower at T1 and T3 than at baseline (T0 1.02 ± 0.37 μV, T1 0.90 ± 0.26 μV, T3 0.74 ± 0.32 μV, p < 0.05 for both comparisons), no significant changes of EP or NT parameters were found in longitudinal assessment. Baseline P300 Pz latency was longer for the patients with MS than for those with CIS at T1 (352.69 vs. 325.56 ms). No predictive value was shown for any of the analyzed baseline variables with regard to conversion from CIS into MS.SignificanceBaseline ERP abnormalities were associated with their short-term conversion into MS. ERP are worth considering in multimodal EP evaluation at the early stage of MS.

ObjectivesThe motor neuron survival protein, which is deficient in spinal muscular atrophy (SMA), performs numerous cellular functions. Currently, SMA is believed to be a multi-organ disease, including lesion of various structures of the central and peripheral nervous systems. Motor nerve damage, especially in milder SMA types, is controversial. This prompted the conduct of the electrophysiological studies in adults with SMA types 2 and 3 presented in this paper.MethodsThe study group consisted of 44 adult patients with SMA types 2 and 3. All patients underwent neurological examination with Hammersmith Functional Motor Scale-Expanded (HFMSE) assessment. Standard electrophysiological studies in the ulnar nerve and conduction velocity distribution (CVD) tests were performed in all patients and controls.ResultsA prolongation of the distal latency and lowering of the motor potential amplitude with no changes in CVD were found in the whole patient group. There were no dependencies on the number of gene copies. Patients with low HFSME value had slower standard conduction velocity, CVD in upper and median quartiles, and narrower CVD spread; in milder SMA, CVD spread was greater than in controls.InterpretationThe significant reduction in motor response amplitude in SMA seems to be primarily related to motor neuron loss and directly proportional to its severity. The coexisting rearrangement in the peripheral nerve structure is present in SMA, and this could be partially caused by a coexisting demyelinating process. Nerve remodeling mainly affects large fibers and occurs in more severe SMA types with significant disability.

BackgroundThe gold standards for the diagnosis and treatment of carpal tunnel syndrome (CTS) and its outcome are undecided. Using clinical and electrophysiological methods, we tried to establish which fibers achieved full postoperative recovery, and the possibility of using non-standard electrophysiological tests as outcome predictors.MethodsThe study group consisted of 35 patients and controls. The Historical–Objective Scale, standard neurography, conduction velocity distribution tests (CVD), and quantitative sensory testing (QST) were performed before and after CTS surgery.ResultsClinical improvement was observed on average in 54.3% of the patients, higher in less advanced CTS. All parameters improved significantly after surgery, except for CVD; most remained worse than in the controls. Only QST parameters fully returned to normal limits. Patient age and CTS severity were important in the estimation of the risk of no improvement.ConclusionsThe efficiency of minimally invasive CTS surgery is higher in younger patients with less advanced CTS. Complete recovery was present only in small fibers; larger fibers could most likely be responsible for residual signs. We did not notice any benefits in CTS diagnosis using methods of small fiber assessment. QST seemed to be useful in the diagnosis of residual signs, and in deciding upon possible reoperation.

IntroductionSimilarities in morphology, physiological function, and neurophysiological findings between median and ulnar nerves are not unequivocal. Our previous study confirmed differences in motor fiber parameters between these nerves in healthy persons. We made an attempt to assess and compare the physiological parameters of different sensation modalities (temperature, pain, and vibration) in median and ulnar nerves.MethodsThe study was performed in 31 healthy, right-handed volunteers: 17 women, 14 men, mean age 44.8 ± 15.5 years. Standard sensory conduction tests in the median and ulnar nerves were performed together with the estimation of vibratory, temperature, and warm- and cold-induced pain thresholds in the C7 and C8 dermatomes on the palm, using quantitative sensory testing.ResultsThere were no statistically significant differences in the standard sensory conduction test in the median and ulnar nerves across the whole group: between right and left hands, and between women and men. We revealed differences in the temperature and pain thresholds between these nerves, mainly in low temperature perception. There were no differences in estimated thresholds between sides or in female and male groups. The vibratory limits did not differ significantly between nerves, and subgroups.ConclusionThe study confirmed the differences in the physiological sensory perception between the median and ulnar nerves. The median nerve is more sensitive to temperature stimulation than the ulnar nerve, but simultaneously less sensitive to pain-inducing temperature stimuli. These findings should be considered during the examination of hand nerve pathology.