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Objectives There is a clinical variability and heterogeneity among Facioscapulohumeral Muscular Dystrophy (FSHD) patients. Escalation after menopause in women, early onset in men suggests that estrogen might be a protective factor on the course of FSHD. In spite of few molecular studies supporting the protective role of estrogen in FSHD in vitro, there is no study revealing the effect of estradiol on the protein levels of DUX4, β-catenin and PAX3/PAX7. In present study, we investigated the effect of estradiol treatment on the expressions of DUX4, β-catenin and PAX3/PAX7 protein levels. Materials and Methods Primary myoblasts of 63 and 71 years old (63yM/71yM) males; 47 years old (47yF) female FSHD patients were used. Cells were processed under these conditions; (i) untreated, (ii) 10 nM-30 min estradiol and (iii) 10 nM-4 h estradiol treated. The expression of DUX4, PAX3/PAX7 and β-catenin were examined by western-blotting. Results Expression of DUX4 significantly downregulated after 4 h treatment of estradiol while PAX3/PAX7 56 kDa variant expression upregulated in 71yM cells. β-catenin and PAX3 expression was variable among the samples. Conclusion Our results suggest that estrogen might be a palliative treatment option via downregulation of DUX4 protein in DUX4 expressing FSHD patients.

Objectives The relation between increase of tonus and joint movement velocity is controversial in Parkinson's rigidity. It is accepted that the increase of tonus in rigidity is constant during joint movement, and does not change within all limits of movement. However, there is thoughtful evidence that the change in tonus in rigidity has a correlation with joint movement velocity and amplitude of movement. The pendulum movement that is formed by triggering of the patellar T reflex allows the examination of phasic stretching reflexes and physiological changes of passive stretching. Therefore, the velocity and amplitude properties of tonus in Parkinson's rigidity can be scanned together. Materials and methods Patellar T reflex-triggered patellar pendulum was recorded in 40 Parkinson's patients. The velocity and amplitude changes in the pendulum were observed according to the rigidity scale. Muscle action potentials were recorded from the rectus femoris muscle and biceps femoris muscles simultaneously via superficial recording electrodes. Knee joint angle changes were recorded with a goniometer. The kinesiological and electromyographic features were compared with those of the control subjects. Results The number of pendulums decreased significantly, the angle of joint movement decreased, the peak time decreased and the angular velocity slowed down significantly in the Parkinson's group. While the latency of the patellar T reflex did not change significantly, its amplitude decreased, and the onset time of joint movement measured by accelerometer was prolonged. Conclusions Parkinson's rigidity has a velocity-dependent component, and this correlates negatively with the rigidity scale.

© 2020 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made., Objective: To investigate disease spread in amyotrophic lateral sclerosis (ALS), and determine the influence of lower (LMN) and upper motor neuron (UMN) involvement. Methods: We assessed disease spread in ALS in 1376 consecutively studied patients, from five European centers, applying an agreed proforma to assess LMN and UMN signs. We defined the pattern of disease onset and progression from predominant UMN or lower motor neuron (LMN) dysfunction in bulbar, upper limbs, lower limbs, and thoracic regions Non-linear regression analysis was applied to fit the data to a model that described the relation between two random variables, graphically represented by an inverse exponential curve. We analyzed the probability, rate of spread, and both combined (area under the curve). Results: We found that progression was more likely and quicker to or from the region of onset to close spinal regions. When the disease had a limb onset, bulbar motor neurons were more resistant. Furthermore, in the same time frame more patients progressed from bulbar to lower limbs than vice-versa, whether predominantly UMN or LMN involvement. Patients with initial thoracic involvement had a higher probability for rapid change. The presence of predominant UMN signs was associated with a faster caudal progression. Interpretation: Contiguous progression was leading pattern, and predominant UMN involvement is important in shortening the time for cranial-caudal spread. Our results can best be fitted to a model of independent LMN and UMN degeneration, with regional progression of LMN degeneration mostly by contiguity. UMN lesion causes an acceleration of rostral-caudal LMN loss., This is an EU Joint Programme - Neurodegenerative Disease Research (JPND) project. The project is supported through national funding organizations under the aegis of JPND - www.jpnd.eu. This project was also partially supported by FCT funding to Neuroclinomics2 (PTDC/EEI-SII/1937/2014).

SMA is a neuromuscular disease and occurs primarily through autosomal recessive inheritance. Identification of deletions in the SMN1 gene especially in the exon 7 and exon 8 regions (hot spot), are used in carrier testing. The exact copy numbers of those exons in the SMN1 and SMN2 genes in 113 patients who presented with a pre-diagnosis of SMA were determined using MLPA method. We aimed to reveal both the most common copy number profiles of different SMA types. It was found that the frequency of homozygous deletions in SMN1 was 15.9%, while heterozygous deletions was 16.9%. The most common SMN-MLPA profile was 0-0-3-3. In the cases with homozygous deletion, SMA type III diagnosis was observed most frequently (44%), and the rate of consanguineous marriage was found 33%. Two cases with the same exonic copy number profile but with different clinical subtypes were identified in a family. We also detected distinct exonic deletion and duplication MLPA profiles for the first time. We created "the SMA signature" that can be added to patient reports. Furthermore, our data are important for revealing potential local profiles of SMA and describing the disease in genetic reports in a way that is clear and comprehensive.

After tissue or limb loss, the development of sensation and perception of the lost or deafferent tissue is defined as a phantom phenomenon. We investigated the presence of phantom phenomena in individuals who underwent a full face transplant as well as those who had a hand transplant. Specifically, we investigated sensory perception of the face on the fingers and sensory perception of the fingers on the face in three full face and four hand transplant patients. In all seven individuals, we used a brush to separately stimulate the right and left sides of the face or the palmar and dorsal faces of the hand. We then asked the individuals if they felt a sensation of touch on any other part of their body and, if so, to describe their perceptions. Changes in the regions of the primary sensory cortex representing the hand and face were defined using fMRI obtained via tactile sensory stimulation of the clinical examination areas. Two of the full face transplant patients reported sensory perceptions such as a prominent sensation of touch on their faces during sensory stimulation of their fingers. Three of the hand transplant patients reported sensory perceptions, which we referred to as finger patches, during sensory stimulation of the face area. In fMRI, overlaps were observed in the cortical hand and face representation areas. We consider the phantom hand and phantom face phenomena we observed to be complementary due to the neighborhood of the representations of the hand and face in the somatosensory cortex.

© 2021 Elsevier B.V. All rights reserved., Background: The role of cardiovascular risk factors in amyotrophic lateral sclerosis (ALS) is controversial. A favourable profile has been found in ALS patients, but previous studies have not specifically considered the profile in different disease phenotypes. Methods: Demographic data, smoking habits, lifetime exercise, and medical history including diabetes mellitus, arterial hypertension, hypercholesterolemia, hypertriglyceridemia, stroke, and cardiac events, were analysed in ALS patients and in controls with other neurological disorders, utilising a standardized questionnaire applied by the same neurologist. In ALS patients the results were analysed according to their different phenotypes. Univariate analyses and multinomial logistic models were applied to estimate the odds ratios (ORs) and confidence intervals (CIs) for covariates, to test potential modifiers and their effects. Results: 500 consecutively assessed adult ALS patients (mean age 65.6, 47% women, and 136 bulbar-onset) and 327 age and gender-matched controls were studied. Patients with spinal-onset ALS took more exercise (p = 0.012), reported less hypertension (p = 0.002) and had fewer cardiac events (p = 0.012). Multinomial regression analysis showed that men without hypertension have a higher risk of having spinal-onset ALS (p < 0.001) while female with hypertension have a higher risk of having bulbar-onset ALS (p = 0.033). Conclusions: Risk-factors in ALS can be influenced by gender and phenotype. This study suggests that men with spinal ALS are healthier, exercise more and have lower rate of hypertension, but females with bulbar-onset ALS are more prone to hypertension. The complex interplay between exercise, diet and comorbidities with ALS phenotype requires further investigation., This study is included in the ONWebDUALS project (JPND-PS/0001/2013) initiative. This is an EU Joint Program – Neurodegenerative Disease Research (JPND) project – Fundação para a Ciência e a Tecnologia (JPND-PS/0001/2013).

© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ., Amyotrophic lateral sclerosis (ALS) is a rare and progressive neurodegenerative disease mainly affecting upper and lower motor neurons but also causing multisystem involvement, in particular, associated with cognitive changes. Minor sensory fibre dysfunction has been described in the past and confirmed in recent studies. In a multicentre study investigating a population of 88 patients with ALS, the ESTEEM group (a European Telematic Project for quality assurance within Clinical Neurophysiology) reported sensory polyneuropathy (PNP) in 12.5% of the patients, not influenced by age, disease duration and onset region. In this study, we aimed to readdress prevalence of and risk factors for PNP in a larger population of patients with ALS. A large number of variables, including gene mutations, were assessed.

Amyotrophic Lateral Sclerosis is one major disease in the group of neurodegenerative conditions. As with most other neurodegenerative diseases, clinical signs of the disease usually show among the elderly population, and most commonly around 60-65 years of age. Therefore the disease is not expected to impact the fertility of ALS patients. When examined from an evolutionary medicine and evolutionary biology perspective, there should be no selection pressure on the patient population due to the late onset of ALS.

Sudden foot dorsiflexion lengthens soleus muscle and activates stretch-based spinal reflexes. Dorsiflexion can be triggered by activating tibialis anterior (TA) muscle through peroneal nerve stimulation or transcranial magnetic stimulation (TMS) which evokes a response in the soleus muscle referred to as Medium Latency Reflex (MLR) or motor-evoked potential-80 (Soleus MEP80), respectively. This study aimed to examine the relationship between these responses in humans. Therefore, latency characteristics and correlation of responses between soleus MEP80 and MLR were investigated. We have also calculated the latencies from the onset of tibialis activity, i.e., subtracting of TA-MEP from MEP80 and TA direct motor response from MLR. We referred to these calculations as Stretch Loop Latency Central (SLLc) for MEP80 and Stretch Loop Latency Peripheral (SLLp) for MLR. The latency of SLLc was found to be 61.4 ± 5.6 ms which was significantly shorter (P = 0.0259) than SLLp (64.0 ± 4.2 ms) and these latencies were correlated (P = 0.0045, r = 0.689). The latency of both responses was also found to be inversely related to the response amplitude (P = 0.0121, r = 0.451) probably due to the activation of large motor units. When amplitude differences were corrected, i.e. investigating the responses with similar amplitudes, SLLp, and SLLc latencies found to be similar (P = 0.1317). Due to the identical features of the soleus MEP80 and MLR, we propose that they may both have spinal origins.

The suppression of oxidative-stress induced neurotoxicity by antioxidants serves as a potential preventive strategy for neurodegenerative diseases. In this study, we aimed to investigate the cell protective and antioxidant effects of masitinib and cromolyn sodium against toxin-induced neurodegeneration. First, human neuroblastoma SH-SY5Y cells were differentiated into neuron-like (d)-SH-SY5Y cells. The differentiated cells were confirmed by immuno-staining with anti-PGP9.5 antibody, a neuronal marker. Cell culture groups were formed, and a neurotoxin, 1-methyl-4-phenyl1,2,3,6-tetrahydropyridine (MPTP) was applied on cells followed by masitinib and/or cromolyn sodium treatments. Survival rate of cells were detected by MTT assay. Anti-inflammatory Transforming Growth Factor-β1 (TGF-β1) and nitric oxide (NO) levels and total oxidant and antioxidant capacities (TOC and TAC) in cell conditioned media (CM) were measured. Morphological analysis and apoptotic nuclear assessment of cells were also noted. When (d)-SH-SY5Y cells were exposed to neurotoxin, cell viability rates of these cells were found to be decreased in a concentration-dependent manner. CM of toxin applied group displayed higher levels of TOC/TAC ratios and NO levels compared to control (p 0.01). Both masitinib and cromolyn sodium protected cells from toxin-induced cell death as revealed by ameliorated rates of viability, reversed toxin-induced elevation of TOC/TAC ratios, and decreased NO levels in their CM (p 0.01). Combined treatment significantly reduced TOC/TAC ratios and NO levels more effectively compared to mono-treatments. Both drugs also increased TGF-β1 levels significantly in cell CM. When these agents were tested for therapeutic effects against toxin-induced cell degeneration, better viability results were obtained by both masitinib and cromolyn sodium treatment, with significantly better amelioration provided by combined application of these drugs (p 0.01). This study demonstrated new findings that combined treatment with cromolyn sodium, an FDA-approved drug of asthma, and masitinib, an orally administered drug with a low toxicity, exert neuroprotective and additive therapeutic effects. We propose that combination therapy of masitinib and cromolyn sodium may represent an innovative treatment in neurodegenerative diseases. Combination therapy may be more advantageous that it enables combined application of lower doses of both drugs, providing less side effects.

The aim of this study is to examine cortical plasticity and to analyze cortical reorganization following hand and facial transplantation, using functional magnetic resonance imaging. Patients who had undergone full-face transplantation, hand transplantation and scapular arm replantation, as well as healthy controls, participated in the study. The perioral area and volar surfaces of the index finger and thumb were stimulated and images were acquired using 3 T functional MRI. The areas of the somatosensory cortex representing the hand and face are different in size and shape due to experience-dependent plasticity. Therefore, a new and more adaptive volume of interest analysis was created whereby the radiuses of the VOI masks were defined by the peak intensity of subsequent clusters. For each control subject, the distribution of activated voxels was observed for various cluster defining thresholds in order to determine the mean number of activated voxels for each stimulation inside the defined region. The determined numbers of voxels per subject were extracted from the defined regions using a binary search algorithm. Subsequently, the distances between the weighted centers of the extracted regions were calculated and compared. In transplant patients, the weighted centers of the hand and face clusters were separated at same-sized volumes. Two of the rehabilitated full-face transplant patients converge to the range of the controls. As a result, the weighted distribution of somatotopy indicated previous and present cortical reorganization. Additionally, referred sensation was assessed in two full-face transplant and one replant patient with activation clusters partially in BA40 in the Inferior Parietal Lobule.

Spasticity is one of the major problems that arise in different neurological diseases and seriously affect the quality of human life. Research on the understanding of mechanism of spasticity remains as important as the studies on the spasticity therapy and rehabilitation. In this study, the spasticity mechanism which develops concerning the upper motor neuron lesions is investigated by modelling “Patella tendon reflex triggered patella pendulum”. The mathematical model based on the pendulum phenomenon is developed by solving the curve-fitting problem as finding the curve that best fits a set of data points. Electrophysiological and dynamic measurement data were taken from 76 spastic subjects and 20 healthy participants. The mathematical model is determined by the morphological properties of the goniometric variations. The results denote that the mathematical model containing two clinically relevant parameters –frequency component of the damped oscillatory motion defined as “f0” with the maximum angle of the reflex defined as “a0” ensures to distinguish spasticity from healthy subjects.

Copyright: © 2019 Barc K et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited., Amyotrophic lateral sclerosis (ALS) is a fast progressing neurodegenerative disease leading to quadriplegia, anarthria and respiratory insufficiency. A large variety of phenotypes and disability progression requires individually tailored management. Identification of predictors of poor prognosis may not only improve management, but also allow for more precise patients' stratification for clinical trials or research studies. The aim of the study was to investigate the influence of emotional lability present at disease onset on ALS progression by exploring its direct impact on the decay of the ALS Functional Rating Scale-Revised (ALSFRS-R). The study was performed in a group of 1145 patients from Germany, Poland, Portugal and Turkey between 2014 and 2018. The analysis showed that the presence of emotional lability at ALS onset was linked to a faster decline of ALSFRS-R (0.70 vs 0.50, p, This study was supported by OnWebDuals project (JNPD 01ED1511B; DZP/2/JPND-III/2015). This is an EU Joint Programme - Neurodegenerative Disease Research (JPND) project. The project is supported through the following funding organization under the aegis of JPND - www.jpnd.eu: Germany, Bundes-ministerium für Bildung und Forschung (BMBF); Poland, Narodowe Centrum Badań i Rozwoju (NCBiR); Portugal, Fundação a Ciência e a Tecnologia (FCT); Sweden, Vetenskapsrådet (VR).

Objectives: This study aims to identify the optimal follow-up method for evaluation of the surgical outcome for carpal tunnel syndrome (CTS). Patients and methods: Between January 2006 and December 2010, 61 hands of a total of 46 patients (7 males, 39 females; mean age 56.0 +/- 10.4 years; range, 20 to 71 years) with a diagnosis of CTS were retrospectively analyzed. All operations were performed by a single surgeon with a mini-incision distal to the transverse carpal ligament. At a mean follow-up of seven years after surgery, electromyography (EMG) was repeated for all patients. The Boston Carpal Tunnel Questionnaire (BCTQ), Boston Symptom Severity Scale, Boston Functional Status Scale, palmar pinch strength, grip strength, and EMG of the patients were compared before and after surgery. Results: The mean follow-up was 84410 (range, 72 to 104) months. There were significant improvements in the Boston Symptom and Functional Scale scores postoperatively, as well as in the grip and pinch strength. After surgery, EMG findings improved in 83.6% of the patients. However, there was no significant correlation between pre- and postoperative Boston Symptom Severity Scale scores, functional status, pinch and grip strengths, and pre- and postoperative EMG results. Conclusion: Our study results demonstrate that the symptom severity and functional status scores of the BCTQ are favorable, and this tool is reliable and easy-to-apply for the diagnosis and follow-up of CTS surgeries.

In this study, it is aimed to determine the degree of the development in emotional expression of full face transplant patients from photographs. Hence, a rehabilitation process can be planned according to the determination of degrees as a later work. As envisaged, in full face transplant cases, the determination of expressions can be confused or cannot be achieved as the healthy control group. In order to perform image-based analysis, a control group consist of 9 healthy males and 2 full-face transplant patients participated in the study. Appearance-based Gabor Wavelet Transform (GWT) and Local Binary Pattern (LBP) methods are adopted for recognizing neutral and 6 emotional expressions which consist of angry, scared, happy, hate, confused and sad. Feature extraction was carried out by using both methods and combination of these methods serially. In the performed expressions, the extracted features of the most distinct zones in the facial area where the eye and mouth region, have been used to classify the emotions. Also, the combination of these region features has been used to improve classifier performance. Control subjects and transplant patients' ability to perform emotional expressions have been determined with K-nearest neighbor (KNN) classifier with region-specific and method-specific decision stages. The results have been compared with healthy group. It has been observed that transplant patients don't reflect some emotional expressions. Also, there were confusions among expressions.

PURPOSE Congenital mirror movement disorder (CMMD) is characterized by unintended, nonsuppressible, homologous mirroring activity contralateral to the movement on the intended side of the body. In healthy controls, unilateral movements are accompanied with predominantly contralateral cortical activity, whereas in CMMD, in line with the abnormal behavior, bilateral cortical activity is observed for unilateral motor tasks. However, task-related activities in subcortical structures, which are known to play critical roles in motor actions, have not been investigated in CMMD previously. METHODS We investigated the functional activation patterns of the motor components in CMMD patients. By using linkage analysis and exome sequencing, common mutations were revealed in seven affected individuals from the same family. Next, using functional magnetic resonance imaging (fMRI) we investigated cortical and subcortical activity during manual motor actions in two right-handed affected brothers and sex, age, education, and socioeconomically matched healthy individuals. RESULTS Genetic analyses revealed heterozygous RAD51 c.401C>T mutation which cosegregated with the phenotype in two affected members of the family. Consistent with previous literature, our fMRI results on these two affected individuals showed that mirror movements were closely related to abnormal cortical activity in M1 and SMA during unimanual movements. Furthermore, we have found previously unknown abnormal task-related activity in subcortical structures. Specifically, we have found increased and bilateral activity during unimanual movements in thalamus, striatum, and globus pallidus in CMMD patients. CONCLUSION These findings reveal further neural correlates of CMMD, and may guide our understanding of the critical roles of subcortical structures for unimanual movements in healthy individuals. This work was funded by a grant of the Turkish National Scientific and Technological Council (TUBI-TAK 1001-108S355).

Background We assessed the recovery of 2 face transplantation patients with measures of complexity during neuromuscular rehabilitation. Cognitive rehabilitation methods and functional electrical stimulation were used to improve facial emotional expressions of full-face transplantation patients for 5 months. Rehabilitation and analyses were conducted at approximately 3 years after full facial transplantation in the patient group. We report complexity analysis of surface electromyography signals of these two patients in comparison to the results of 10 healthy individuals. Methods Facial surface electromyography data were collected during 6 basic emotional expressions and 4 primary facial movements from 2 full-face transplantation patients and 10 healthy individuals to determine a strategy of functional electrical stimulation and understand the mechanisms of rehabilitation. A new personalized rehabilitation technique was developed using the wavelet packet method. Rehabilitation sessions were applied twice a month for 5 months. Subsequently, motor and functional progress was assessed by comparing the fuzzy entropy of surface electromyography data against the results obtained from patients before rehabilitation and the mean results obtained from 10 healthy subjects. Results At the end of personalized rehabilitation, the patient group showed improvements in their facial symmetry and their ability to perform basic facial expressions and primary facial movements. Similarity in the pattern of fuzzy entropy for facial expressions between the patient group and healthy individuals increased. Synkinesis was detected during primary facial movements in the patient group, and one patient showed synkinesis during the happiness expression. Synkinesis in the lower face region of one of the patients was eliminated for the lid tightening movement. Conclusions The recovery of emotional expressions after personalized rehabilitation was satisfactory to the patients. The assessment with complexity analysis of sEMG data can be used for developing new neurorehabilitation techniques and detecting synkinesis after full-face transplantation. Electronic supplementary material The online version of this article (10.1186/s12984-018-0356-0) contains supplementary material, which is available to authorized users.

Hirayama disease (HD) was first reported by Hirayama et al. in 1959. The disease is considered as a type of benign focal motor neuron disease that primarily affects upper limbs of young males. In this case report, we present a man aged 40-years with rapidly progressive weakness and atrophy in his left hand. The findings of nerve conduction studies were consistent with left ulnar neuropathy at the elbow. Flexion magnetic resonance imaging (MRI) revealed minimal enlargement of the posterior epidural space and anterior displacement of the spinal cord. After exclusion of relevant diseases the patient was diagnosed as having Hirayama disease with ulnar neuropathy. Mild ulnar entrapment at the elbow may be considered as a clinical feature of HD. Therefore, it is recommended that young male patients with wasting in upper extremities with findings of ulnar entrapment should not be judged to have ulnar neuropathy before HD has been carefully excluded.

Background: Facioscapulohumeral muscular dystrophy (FSHD) occurs as a consequence of genetic deletion of D4Z4 repeats on chromosome 4q35. Onset of FSHD is earlier in males, suggesting that testosterone may trigger the disease. In accordance, the rapid progression of disease in women after menopause suggests a protective role for estrogen and progesterone. No studies have examined levels of all these hormones in relation with the severity of FSHD. Aims: To evaluate the possible correlation between the severity of FSHD with sex hormones, age, and genetic deletion on chromosome 4q35. Subjects and Methods: D4Z4 repeat units were investigated in 33 patients (19 males/14 females) with FSHD. In the blood samples, luteinizing hormone, follicle-stimulating hormone, free estriol, estradiol, free testosterone and total testosterone, progesterone, 17-OH progesterone, prolactin, albumin, and fibrinogen were measured. The severity of FSHD was identified using a Clinical Severity Score (CSS) scaling system. Spearman's correlation and regression analyses were performed as statistical analyses. Results: Age (P = 0.001, r = 0.541) and total testosterone (P = 0.045, r = 0.351) were positively correlated, and the progesterone/total testosterone (P = 0.025, r = −0.390) and estradiol/total testosterone ratios (P = 0.025, r = −0.389) were negatively correlated with the severity of FSHD. Conclusions: Our results indicate that age, total testosterone, ratios of estradiol and progesterone to total testosterone, but not deletion on chromosome 4q35, have a significant relation with the severity of FSHD. Given that both estrogen and testosterone treatment are considered in therapy, our results suggest that estrogen and progesterone but not testosterone are likely to be more effective on the severity of FSHD.

In our medical school, we changed from a lecture-based method to a team-based learning (TBL) method to teach “polyneuropathies” in the neurology clerkship starting from the 2014 to 2015 academic year. Real patients were used instead of written scenarios in TBL sessions. This study aimed to compare former lecture-based and the current TBL methods in terms of knowledge retention, in-class learner engagement, and learner reactions. First, we determined in-class engagement and satisfaction of the students for the lectures given in the 2013–2014 academic year. The following year, besides the same criteria, we also determined individual (IRAT) and group readiness test (GRAT) scores in the TBL group. End-of-clerkship exam scores for both groups were recorded. Additionally, opinions of patients about their experiences throughout the TBL process were determined. One year later (2015 for lecture and 2016 for TBL), both groups sat for an MCQ test to determine their knowledge retention levels. We found no difference between groups regarding end-of-clerkship exam scores. The mean knowledge retention test score of the TBL group was significantly higher than that of the lecture group (5.85 ± 1.74 vs. 3.28 ± 1.70). The differences between IRAT, GRAT, and retention test scores in the TBL group were significant. The mean student satisfaction score on a five-point scale was 3.01 ± 0.9 (median = 3) in the lecture group and 4.11 ± 1.1 (median = 4) in the TBL group. Our results seem encouraging for use of TBL performed with real patients in neurology education to achieve better long-term knowledge retention and higher in-class engagement and student satisfaction.