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BACKGROUND AND AIMS: This study aimed to identify the clinical characteristics and electrodiagnostic subtypes of Guillain-Barré syndrome (GBS) in Istanbul. METHODS: Patients with GBS were prospectively recruited between April 2019 and March 2022 and two electrodiagnostic examinations were performed on each patient. The criteria of Ho et al., Hadden et al., Rajabally et al., and Uncini et al. were compared for the differentiation of demyelinating and axonal subtypes, and their relations with anti-ganglioside antibodies were analyzed. RESULTS: One hundred seventy-seven patients were included, 69 before the coronavirus disease 2019 pandemic (April 2019-February 2020) and 108 during the pandemic (March 2020-March 2022), without substantial changes in monthly frequencies. As compared with the criteria of Uncini et al., demyelinating GBS subtype diagnosis was more frequent according to the Ho et al. and Hadden et al. criteria (95/162, 58.6% vs. 110/174, 63.2% and 121/174, 69.5%, respectively), and less frequent according to Rajabally et al.'s criteria (76/174, 43.7%). Fourteen patients' diagnoses made using Rajabally et al.'s criteria were shifted to the other subtype with the second electrodiagnostic examination. Of the 106 analyzed patients, 22 had immunoglobulin G anti-ganglioside antibodies (14 with the axonal subtype). They had less frequent sensory symptoms (54.5% vs. 83.1%, p = 0.009), a more frequent history of previous gastroenteritis (54.5% vs. 22.9%, p = 0.007), and a more severe disease as compared with those without antibodies. INTERPRETATION: Serial electrodiagnostic examinations are more helpful for accurate subtype diagnosis of GBS because of the dynamic pathophysiology of the disease. We observed no significant increase in GBS frequency during the pandemic in this metropolis.
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Síndrome de Guillain-Barré , Humanos , Estudios Prospectivos , Conducción Nerviosa/fisiología , Electrodiagnóstico/métodos , Gangliósidos , AnticuerposRESUMEN
Objectives Although age is a risk factor, carpal tunnel syndrome (CTS) can also affect younger individuals, particularly those involved in activities or occupations that require repetitive hand movements, forceful gripping, or prolonged wrist flexion/extension. This case-control study aimed to examine the prevalence of CTS symptoms and frequent object dropping among a group of young dentists who are exposed to CTS risk factors. Additionally, other reported risk factors for CTS, such as sex, obesity, and square wrist sign, were also investigated. Methods A total of 74 dentists (48 women, mean age 28.5 years), who are working at Istanbul Faculty of Dentistry, the largest dental school in Istanbul, which is the biggest city in Turkey, were included in the study. Additionally, 61 age- and sex-matched controls (38 women, mean age 27.9 years) were also recruited. The Edinburgh Hand Preference Questionnaire, Boston Carpal Tunnel Syndrome Questionnaire (BCTQ), a questionnaire for object dropping and occupational hand usage, anthropometric measurements of the hands, clinical neurologic examination, and electromyography intended for the detection of CTS were performed. Results The dentists had a higher total weekly hand usage duration compared to the controls (66.3 vs 44.8 hours, p<0.001). BCTQ scores and the frequency of object dropping were also significantly higher in dentists compared to controls (respective p values: 0.011, 0.003). Positive correlations were found between BCTQ scores, hand usage durations, and object dropping (respective p values: 0.001, <0.001). BCTQ scores were higher in women than in men (p=0.027). Electrophysiologic evidence of CTS was found in one dentist. Conclusions Symptoms of CTS may manifest in individuals at a younger age than predicted, primarily influenced by their occupation and the duration of hand usage. Dentists, in particular, report a higher incidence of complaints related to object dropping, which can be attributed to their frequent use of specialized tools and engagement in delicate tasks, resulting in heightened awareness. However, it can also potentially serve as an indicator of CTS.
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INTRODUCTION: Tibial somatosensory evoked potentials (SEP) are used to identify the neurological status and tethered cord (TC) in patients with spina bifida (SB). Its significance in contributing to the interpretation of urodynamics to determine bladder status is unknown. This study aimed to determine the correlation between SEP and urodynamics in children with SB. MATERIAL AND METHODS: SEP and urodynamic results, for differential diagnosis of TC, were evaluated. SEP scores were correlated with urodynamic findings. SEP results were scored from 1 to 6, with 1, denoting a favorable score and 6, an unfavorable score. Age, gender, detrusor, and sphincter activities in urodynamics were noted. Results were analyzed using the χ2 test and logistic regression analysis. Receiver operating characteristic (ROC) curve was formed to get a valid threshold for the SEP score to predict the urodynamic condition. RESULTS: There were 44 SB patients for whom SEP was done for differential diagnosis of TC. Fifteen patients who did not meet the inclusion criteria were excluded from the study. SB aperta was present in 17 patients and occulta in 12, respectively. The patients had a mean age of 6.6 ± 3.2 years. There were 13 boys and 16 girls. A strong correlation was found between high SEP scores and detrusor sphincter dyssynergia (p < 0.001). A SEP score over 3.5 was found to be 93% sensitive and 73% specific to predict this correlation. There was no relationship between detrusor activity and SEP scores (p = 0.18). DISCUSSION: Tibial SEP is an important noninvasive adjunct tool for the diagnosis of TC in patients with SB. Urodynamic studies are the gold standard in the evaluation of bladder status in neurogenic bladder dysfunction due to SB. Detrusor sphincter dyssynergia may be regarded as a sign of severe spinal cord injury in these patients. CONCLUSION: Our findings suggest that in children with neurogenic bladder, high SEP scores may predict the presence of detrusor sphincter dyssynergia but not the status of detrusor function while providing pathophysiological evidence for neural injury.
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Defectos del Tubo Neural , Traumatismos de la Médula Espinal , Disrafia Espinal , Vejiga Urinaria Neurogénica , Masculino , Femenino , Humanos , Niño , Preescolar , Vejiga Urinaria Neurogénica/diagnóstico , Vejiga Urinaria Neurogénica/etiología , Vejiga Urinaria , Disrafia Espinal/complicaciones , Potenciales Evocados Somatosensoriales , Urodinámica/fisiología , AtaxiaRESUMEN
OBJECTIVES: This prospective study was designed to assess whether patients with skeletal deformities show characteristic masseter inhibitory reflex (MIR) and blink reflex (BR) patterns. A secondary aim was to investigate whether these reflexes change following bilateral sagittal split osteotomy (BSSO). MATERIALS AND METHODS: Fourteen consecutive patients who underwent single-jaw BSSO and 14 class I subjects who constituted the control group were enrolled into the study. MIR and BR, obtained by the stimulation of supraorbital (SBR) and mental nerves (MBR), were electrophysiologically recorded. Sensory impairment in the mental nerve distribution was clinically tested. Three evaluation periods were specified as immediately before (T0), 1 month (T1) and 6 months (T2) after surgery. RESULTS: MIR early silent period duration was significantly shorter in the patients at T0 (pâ¯< 0.001). Sensory deficits developed on 23 sides after BSSO, of which, 17 recovered after 6 months. At T1, MBR was inelicitable bilaterally in 3 patients and unilaterally in 2 patients. These responses were still unrecordable bilaterally in 1 patient, and unilaterally in 4 patients at T2. MIR were unrecordable on 18 sides at T1 and recovered on 11 sides at T2. There were no parallels between the clinical sensory deficits and the abnormal results of the reflexes. CONCLUSIONS: Shorter MIR in patients with dentofacial abnormalities may be a reflection of an adapted trigeminal reflex mechanism. Although MBR and MIR abnormalities do not develop parallel to the clinical sensory deficits, their course might provide insights into the disturbed trigeminal reflex pathways.
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Osteotomía , Reflejo , Humanos , Tronco Encefálico/fisiología , Mandíbula/cirugía , Nervio Mandibular , Estudios Prospectivos , Reflejo/fisiología , Deformidades Dentofaciales/cirugíaRESUMEN
OBJECTIVE: Cutaneous allodynia (CA) is a common symptom in migraine. Its incidence is more frequent in the chronic migraine (CM). CA usually occurs during pain attacks. However, it can also be interictal and its frequency and severity seem to be correlated with the duration of the disease. Several quantitative sensory testing (QST) studies have revealed variable results about mechanical and thermal allodynia accompanying migraine. This study aimed to investigate the effects of CA and onabotulinumtoxinA (BoNT-A) injection on the thermal thresholds measured by QST in patients with CM. The effects of BoNT-A on headaches, CA, and other accompanying symptoms of migraine were also evaluated. METHODS: Single BoNT-A injections were performed in 22 female cases (mean age: 38.1 ± 7.2 years) with CM. Patients were evaluated at 1-7 days before, 28-35, and 84-91 days after the injection. The 22 healthy women in the control group (mean age: 36.6 ± 7.6 years) were examined once. Headache and its characteristics, medication intake, allodynia, presence of anxiety, and depression symptoms were evaluated through relevant scales. The heat (HDT) and cold (CDT) detection thresholds on the forehead and hand were measured bilaterally with QST. The presence of brush allodynia for patients was examined by applying a 4 × 4 gauze pad over the same areas. RESULTS: The patients in the CM group had migraine for an average of 22.5 ± 6.1 years and CM for 6.1 ± 3.2 years. The average number of painful days per month was 22.1 ± 4.0 days. All the patients had migraine attacks with CA (mean 5.6/month). The average allodynia symptom checklist (ASC-12) score was 7.8 ± 6.2. Thermal thresholds measured in the patients with CM were similar to those of the controls. Thermal thresholds did not show significant differences between the symptomatic and the asymptomatic sides at the last migraine attack. There was also no correlation between the allodynia revealed by the physical examination and the thermal thresholds detected by QST. The ASC-12 score decreased significantly with BoNT-A injection (p = 0.030), but no significant change was observed in thermal thresholds after this treatment. CONCLUSION: There was no significant correlation between CA and thermal thresholds. BoNT-A was successful in relieving headache and other associated symptoms, including CA, but had no significant effect on QST parameters.
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Toxinas Botulínicas Tipo A , Trastornos Migrañosos , Adulto , Toxinas Botulínicas Tipo A/uso terapéutico , Femenino , Cefalea/complicaciones , Humanos , Hiperalgesia/diagnóstico , Hiperalgesia/tratamiento farmacológico , Hiperalgesia/epidemiología , Persona de Mediana Edad , Trastornos Migrañosos/complicaciones , Trastornos Migrañosos/tratamiento farmacológico , Dolor/tratamiento farmacológicoRESUMEN
Motor sequence learning (MSL) paradigms are often used to investigate the neural processes underlying the acquisition of complex motor skills. Behavioral and neuroimaging studies have indicated an early stage in which spatial learning is prominent and a late stage of automatized performance after multiple training periods. Functional magnetic resonance imaging (fMRI) studies yielded both decreased and increased activations of the sensorimotor and association areas. However, task-negative and task-positive intrinsic connectivity networks (ICNs), the default mode (DMN) and dorsal attention (DAN) networks involved in governing attention demands during various task conditions were not specifically addressed in most studies. In the present fMRI study, a visually guided MSL (VMSL) task was used for bringing roles of visuospatial and motor attention into foreground in order to investigate the role of attention-related ICNs in MSL. Seventeen healthy, right-handed participants completed training and test sessions of VMSL during fMRI on the 1st day. Then, after daily training for three consecutive days outside the scanner, they were re-tested during the 5th day's scanning session. When test session after early learning period was compared with training session, activation decrease was observed in the occipito-temporal fusiform cortex, while task-related suppression of DMN was reduced. Reduced deactivation after early learning was correlated with decreased error rates. After late learning stage we observed activation decreases in bilateral superior parietal lobules of task-positive DAN, dorsal precunei, and cerebellum. Reduced activity in left posterior parietal and right cerebellar regions were correlated with gains in speed, error rate, respectively. This dissociation in activity changes of DMN and DAN related areas suggests that DAN shows high contribution during both early and late MSL stages, possibly due to attention requirement for automatization of spatial and temporal aspects of motor sequence. In contrast, spatial learning occurring during early MSL stage was sufficient for releasing DMN resources.
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Imagen por Resonancia Magnética , Lóbulo Parietal , Encéfalo , Mapeo Encefálico , Cerebelo , Humanos , Aprendizaje , Destreza Motora , Red Nerviosa/diagnóstico por imagen , Vías NerviosasRESUMEN
Background: Diagnostic evaluation of patients with parenchymal Neuro-Behçet's disease (NBD) requires magnetic resonance imaging (MRI), neuro-ophthalmologic, and neuropsychological evaluation. In this study, we aimed to find out the ideal diagnostic method that most closely reflects the progress in cognitive disability and brain atrophy in NBD. Methods: In this matched case-control study, we included patients with parenchymal NBD, Behçet's disease without neurological involvement (BD), rheumatoid arthritis, and healthy controls. Detailed ophthalmological examination, pattern-reversal visual evoked potentials (prVEP) test, optical coherence tomography (OCT), brain MRI volumetry and cognitive evaluation tests were performed. Disability status was assessed by revised EDSS. Results: Sixty-eight individuals (35 female, 33 male) were recruited. Mean ACE-R scores were significantly lower in the NBD group (NBD vs. healthy, 80±14.4, 93±4.9, p=0.002). prVEP values were similar across groups, but retinal nerve fiber layer thickness (RNFLT) were more frequently abnormal in the NBD group. We found considerable volume reduction in the brainstem, cerebellum, hippocampus, and thalamus in the NBD group. Regarding prVEP, 120 minutes P100 amplitude (p<0.001, r=0.97) and 60 minutes P100 amplitude values (p=0.006, r=0.90) were positively correlated with the total cerebral white matter volume. Conclusion: Our results confirmed previous observations on cognitive dysfunction in patients with NBD. We reported MRI volumetry data of patients with parenchymal neuro-Behçet's disease for the first time and elucidated novel brain regions with atrophy. Clinically determined scores and OCT failed to predict the status of brain atrophy. prVEP P100 amplitude may be used as a surrogate marker of cerebral white matter involvement in NBD.
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Síndrome de Behçet , Atrofia/patología , Síndrome de Behçet/complicaciones , Síndrome de Behçet/diagnóstico por imagen , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Humanos , Laboratorios , Imagen por Resonancia MagnéticaRESUMEN
Neurogenic heterotopic ossification (NHO) is an abnormal development of bone in extra-skeletal tissues, related to neurological disease. NHO is frequently seen after traumatic brain injury or spinal cord injury. NHO may also occur as a rare complication of Guillain Barre Syndrome (GBS). Here, we present a 39 year old man with an acute onset of GBS who developed NHO around both hips two months after the disease onset. Our patient had a history of mechanical ventilation, incomplete tetraplegia and prolonged immobilisation. The pathogenesis of NHO is unclear. Various risk factors have been associated with the development of NHO; prolonged coma, long-term sedation, spasticity, degree of paralysis. NHO is a rare complication of GBS and physicians should be aware that it can develop especially in patients with severe paralysis and in need of mechanical ventilation. Pain and restriction of movements, especially in the hips, should bring NHO to the mind.
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Síndrome de Guillain-Barré/complicaciones , Síndrome de Guillain-Barré/diagnóstico por imagen , Osificación Heterotópica/diagnóstico por imagen , Osificación Heterotópica/etiología , Adulto , Humanos , MasculinoRESUMEN
INTRODUCTION: To investigate the prevalence and characteristics of Restless Legs Syndrome (RLS) in patients with Behçet's Disease (BD) and Multiple Sclerosis (MS). METHODS: Consecutive patients with BD and MS seen in the outpatient clinic were included in the study. As a control group, volunteer subjects without a known peripheral or central nervous system disorder were included. The BD group was divided into two sub-groups as BD with neurological involvement [Neuro-Behçet's Disease (NBD)] and BD without any neurological involvement (other BD) for further evaluation. Data on demographic characteristics, medical history and family history were collected, and all patients underwent neurological examination. The patients were evaluated for the presence of diagnostic criteria for RLS. The features and severity of RLS were evaluated in patients with RLS using Restless Legs Syndrome Identification Form, and the International Restless Legs Syndrome Study Group (IRLSSG) Rating Scale. The clinical and radiological findings of patients with BD and MS were retrieved from their medical files. RESULTS: The study included a total of 96 patients with BD (mean age 39.9±11.8; 51 males; 41 patients with NBD) and 97 patients with MS (mean age 34.97±4.1 years; 24 males). There were 100 healthy control subjects (mean age 36.18±11.10 years; 46 males). RLS was more prevalent in MS (22.8%) and NBD (22%) groups compared to the control group (10%; p=0.004 and 0.029, respectively) with a statistically significant difference. The prevalence of RLS was higher in MS patients with less disability. Most severe RLS symptoms were observed in the MS group. The rate of sleep disorders was also higher in MS group. Although stress appeared to be a factor worsening RLS in all groups, its prevalence was higher in the MS group (p=0.011). There was no correlation between the distribution of magnetic resonance imaging lesions and RLS in both MS and NBD groups. CONCLUSIONS: It is well established that RLS can accompany disorders involving the peripheral and central nervous systems such as all types of peripheral neuropathy, myelopathy, and Parkinson's disease. The present study showed that MS and NBD also seem to be a risk factor for RLS, being associated with more severe symptoms.
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The brain-derived neurotrophic factor (BDNF) gene Val66Met polymorphism may cause impairment in short-term motor learning by reducing activity-dependent BDNF expression, which causes alterations in synaptic plasticity by changing glutamatergic and GABAergic synaptic transmissions. Sensory-motor integration (SMI) plays an important role in motor learning. In this study, we investigated the role of this polymorphism on SMI during a complex motor learning practice. Forty-three healthy participants performed standardized 5-day basketball shooting exercises under supervision. Electrophysiologic SMI studies were performed before the first day exercise (T0) and after the first and fifth day exercises (T1 and T2, respectively). SMI was studied using electrical median nerve stimulation at the wrist, followed by transcranial magnetic stimulation (TMS) of the contralateral motor cortex with various inter-stimulus intervals (ISIs). Recordings were made from the thenar and forearm flexor muscles. Participants were divided into two groups according to their BDNF genotype. Group 1 consisted of 26 subjects with the Val66Val genotype and group 2 included 17 subjects with the BDNF Met allele. Group 2 had a lower increase in basketball scores at day 5. Moreover, they had higher afferent facilitation for the responses recorded from both thenar and forearm flexor muscles at T1, but these changes could not be maintained until T2. This non-persistent early hyper-responsivity of the sensory-motor cortex in subjects with the BDNF Met allele might be explained by a transient upsurge of cortical excitability to compensate the insufficient cortical plasticity during motor learning, which could be considered as a sign of lower performance in motor skill learning.
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Factor Neurotrófico Derivado del Encéfalo/genética , Potenciales Evocados Motores/fisiología , Aprendizaje/fisiología , Desempeño Psicomotor/fisiología , Adolescente , Adulto , Rendimiento Atlético , Baloncesto/fisiología , Estimulación Eléctrica , Femenino , Voluntarios Sanos , Humanos , Masculino , Polimorfismo de Nucleótido Simple , Estimulación Magnética Transcraneal , Adulto JovenRESUMEN
OBJECTIVE: Myelomeningocele is the most severe and the most frequent form of spina bifida. Most of the myelomeningocele patients undergo operations in new-born age. In terms of life quality and rehabilitation, follow-up's of these patients in the growth and development period after the operation is critical. In our study, our aim is to emphasize the correlation of SEP results with MRI results and clinical features of the myelomeningocele patients. MATERIALS AND METHODS: In our study, we included 36 patients who had undergone myelomeningocele operation and have been followed-up in Istanbul Bilim University Florence Nightingale Hospital, Spina Bifida Research and Treatment Centre. Posterior tibial nerve SEP was performed on each patient and neurological examinations were done in the same session. Results were compared with clinical functional lesion levels, levels of fusion defect and ambulation levels. In order to evaluate SEP results, we used age-related reference values from Boor et al.'s study in 2008. Patients were grouped as normal, unilaterally prolonged, bilaterally prolonged, unilaterally lost, and bilaterally lost. RESULTS: The correlations of posterior tibial nerve SEP results were significant with ambulation levels (r = 0.428, P < 0.01), clinical functional lesion levels (r = 0.477, P < 0.01) and fusion defect levels (r = -0.528 P < 0.05). The lumbar SEP results were only significantly correlated with functional lesion levels (r = 0.443 P < 0.05). CONCLUSIONS: Radiological studies are insufficient when evaluating the functionality of the central nervous system. To fully evaluate the functionality and watch the neurological development with accuracy, especially in operated patients, electrophysiological studies should be an indispensable part of myelomeningocele follow-ups.
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The aim of this study was electromyographic description of changes in swallowing before and after bilateral sagittal split ramus osteotomy. In this prospective study, twenty-eight patients were divided into 3 groups according to the occlusion pattern: Group I (Angle Class III), Group II (Angle Class II), and Control (Class I). Serial cone-beam computed tomography analyses and electromyographic data were collected preoperatively, 1st and 6th months after setback surgery in Group I, and advancement surgery in Group II. Swallowing reflex with 3-20 ml water bolus were studied. Patients were further divided into two subgroups according to the magnitude of relapse. The mean setback of the mandible was 4.62 ± 1.92 mm in Group I, and the mean advancement was 4.19 ± 2.00 mm in Group II. Mandibular relapse rate was 17.40%. Oral preparation phase shortened after surgery in both study groups. Two subjects in Group II and one in Group I had piecemeal deglutition, and two of them became normal postoperatively. Most of the swallowing durations of the relapsed cases were longer than those of stabilized patients. Important clinical considerations are as follows: the oral preparation period becomes shorter after surgery; piecemeal deglutition may disappear after treatment; and individuals with a longer oral period and piecemeal deglutition may have increased tendency to skeletal relapse. This multidisciplinary study enhances our understanding of the adaptive response to the swallowing reflex after orthognathic surgery and provides novel insight into the association between the submental muscle activity and relapse in orthognathic patients.
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Deglución , Osteotomía Sagital de Rama Mandibular , Cefalometría , Estudios de Seguimiento , Voluntarios Sanos , Humanos , Estudios Prospectivos , ReflejoRESUMEN
CONTEXT: Seizures and accompanying situations including social, medical, and psychiatric problems threaten the quality of life (QOL) in patients with epilepsy. The World Health Organization defines health is a state of complete physical, mental, and social well-being, and not merely the absence of disease or infirmity. AIMS: This study examines the prevalence of both depression and anxiety symptoms and also impact of the affective state on QOL in patients with focal epilepsy in Turkey. SETTINGS AND DESIGN: One hundred and five patients with focal epilepsy over 18 years old were included in this study. The patients were classified into four groups according to the presence of AS and seizure control. SUBJECTS AND METHODS: Patients' affective symptoms (AS) and QOL were examined using the Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), and QOL in Epilepsy Inventory-31 (QOLIE-31). STATISTICAL ANALYSIS USED: We used descriptive statistics, Chi-square test, independent samples t-test, one-way analysis of variance, Mann-Whitney U-test, Kruskal-Wallis H-test, and also Pearson's and Spearman's correlation test for correlations. RESULTS: There were positive correlations between total QOLIE-31 score and epilepsy surgery, employment, and seizure freedom, whereas negative correlations were found with antiepileptic drug use, anxiety, and depression. Statistically significant differences were found in QOLIE-31 totals and subscores between Groups 3 and 4 (P < 0.05). CONCLUSIONS: The presence of AS has a negative impact on QOL in patients with focal epilepsy. Physicians should be aware that psychiatric comorbidities in epilepsy have a severe impact and epilepsy treatment requires comprehensive management.
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[This corrects the article on p. 330 in vol. 55, PMID: 30622389.].
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INTRODUCTION: Diabetes mellitus (DM) affects peripheral nerves inducing diabetic polyneuropathy (DPN). Mitochondrial dysfunction and oxidative stress are potential causes of DPN. METHODS: Nerve conduction studies were performed in 20 type 2 DM patients (11 with DPN) and 15 healthy controls. Perception threshold values of cold, warm and vibration were measured by quantitative sensory testing. Variants of a superoxide dismutase 2 (SOD2) gene single nucleotide polymorphism (SNP) (rs5746136) were determined by polymerase chain reaction (PCR) and following NexteraXT DNA Library. RESULTS: DPN patients showed significantly increased threshold values. DM patients without DPN and healthy controls showed comparable values. TT variant of the SOD2 SNP was more prevalent in DM and DPN patients. DM patients with the TT variant displayed increased perception threshold values. CONCLUSIONS: Quantitative sensory testing is not superior to standard nerve conduction studies in DPN. Carriers of SOD2 SNP manifested increased sensory threshold levels which is important in further corroborating the link between oxidative stress and DPN.
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INTRODUCTION: Electrodiagnostic evaluation provides an important extension to the neurological examination for the evaluation of pediatric neuromuscular disease. Many pediatric neuromuscular diseases are analogous to those seen in the adult. However, the relative frequency of these illnesses varies greatly when different age populations are compared. The purpose of the present study is to provide a retrospective analysis of children referred to our electromyography (EMG) laboratory for electrophysiological examinations. METHODS: We retrospectively reviewed electrodiagnostic records of patients aged between 0-15 years, from January 2004 to June 2013. Patients were classified as having plexopathy, nerve root lesions, polyneuropathy, myopathy, mononeuropathy, anterior horn cell disease, neuromuscular transmission disorder, facial nerve palsy, and other rare disorders. RESULTS: We reviewed totally 5563 pediatric records, which was on the average 578 studies per year. It was about 14% of the all EMG examinations performed in our laboratory. When we looked at all the procedures, 3271 of the records included needle EMGs, 170 of them were single fiber EMGs, 100 of them were repetitive nerve stimulations, and 52 of them were evoked potentials. The results were normal in 55% of the cases. As a result of electrophysiological examinations, the common diagnoses were: plexopathy (28.6%), polyneuropathy (7.4%), and myopathy (6.6%) in patients aged 0-5 years (41.2% of all records); myopathy (9.4%), PNP (8.5%), mononeuropathy (6.4%), and plexopathy (5.9%) in 6-10 years (28.2% of all records); PNP (11.3%), myopathy (6.6%), and mononeuropathy (5.6%) in 11-15 years (30.6% of all records). CONCLUSION: Infants and toddlers mostly suffered from brachial plexopathy which can be prevented by proper obstetrical management. Nerve conduction studies and EMG yielded diagnostic importance for demyelinating neuropathy and myopathy in patients older than 6 years of age.
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Oromandibular dystonia (OMD) is defined as a subset of movement disorders characterized by involuntary muscle contraction in different parts of the oromandibular region. This clinical report presents a multidisciplinary approach to the management of a patient with OMD. The involuntary movement of her mandible and tongue was improved with a mandibular custom occlusal device and maxillary modified removable complete denture together with botulinum toxin A injections.
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Toxinas Botulínicas Tipo A/uso terapéutico , Distonía/tratamiento farmacológico , Enfermedades Mandibulares/tratamiento farmacológico , Boca Edéntula , Toxinas Botulínicas Tipo A/administración & dosificación , Femenino , Humanos , Inyecciones Intramusculares , Mandíbula , Músculos Masticadores/efectos de los fármacos , Persona de Mediana Edad , Trastornos del Movimiento/tratamiento farmacológico , Ajuste Oclusal , Enfermedades de la Lengua/tratamiento farmacológicoRESUMEN
Introduction: Migraine and temporomandibular disorders (TMD) are both common diseases and TMD are reported as a risk factor in migraine progression. OnabotulinumtoxinA is used in the treatment of chronic migraine (CM), and also has a potential role in TMD treatment. In this study, it is aimed to compare the efficacy of onabotulinumtoxinA treatment in CM patients with and without TMD. Methods: In this retrospective study, 30 CM patients (age range: 18-65 years), satisfying the inclusion and follow-up criteria in their medical records were investigated. The PREEMPT injection protocol was taken as reference and onabotulinumtoxinA 155-195 U with fixed-dose has been administered into 31 specific sites within the head/neck muscles in included subjects. Two cycles of treatment were assessed in all patients at the baseline and 12 weeks later. The headache diaries, which were completed routinely one month before, and during 6 months follow-up after the treatment, were assessed. The effect of onabotulinumtoxinA treatment was compared between CM patients with and without TMD/bruxism. Results: Of 30 female patients, 17 had concomitant TMD. In week 24, there were significant improvement in the groups with and without TMD regarding to the mean change of frequencies in the days with migraine compared to the initial findings (p<0.001). However, there was no significant difference between the two groups. Conclusions: OnabotulinumtoxinA is an effective and safe treatment for CM. Its efficacy appears to be similar in CM patients with and without TM, speculating that the comorbidity of TMD did not play a role for the treatment response.
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INTRODUCTION: Cramp-fasciculation syndrome (CFS) is a rare peripheral nerve hyperexcitability syndrome. There are only a few reports on clinical and serological profile of a CFS cohort that was followed up by a single outpatient clinic. METHODS: Clinical, electrophysiological, and serological features of 6 CFS patients (5 men, 1 woman; 27-65 years old) were investigated. RESULTS: All patients presented with cramps, fasciculations, muscle pain, and autonomic symptoms, and 2 also reported numbness and burning sensation in limbs, suggestive of neuropathic pain. Antibodies to uncharacterized voltage-gated potassium channel (VGKC)-complex proteins were found in 2 patients and to contactin-associated protein-like 2 (CASPR2) in 1 patient. None of the patients had a tumor. Most of the patients revealed prolonged after-discharges following tibial nerve stimulation. Nerve conduction studies and R-R interval variability tests were normal, whereas sympathetic skin responses were increased in amplitude in 3 seronegative patients. Five patients showed favorable response to carbamazepine or pregabalin treatment, whereas 1 VGKC-antibody-positive patient was resistant to carbamazepine and immunosuppressant treatment. CONCLUSION: Neuropathic pain and VGKC-complex antibodies may be encountered in CFS patients. Although autonomic symptoms are commonly found in CFS, routine autonomic system tests which are done in electrophysiology laboratories might yield normal results.
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INTRODUCTION: We aimed to determine the effect of short day-time sleep on photoparoxysmal epileptic activity in sleep-deprived patients. METHODS: We retrospectively reviewed video-EEG recordings performed between 2003 and 2015. All recordings following at least four hours of sleep deprivation, including intermittent photic stimulation (IPS) both before and after sleep with any form of epileptiform activity were included. The study group was divided into four subgroups: (1) no photoparoxysmal response (PPR) group, with epileptiform activities other than PPRs; (2) increment group, with PPR duration increased by ≥200% after vs. before sleep; (3) no significant change group, with PPR duration increased between 50% and 200% after vs. before sleep; (4) decrement group, with PPR duration increased ≤50% after vs. before sleep. RESULTS: A total number of 5805 EEG recordings from 459 patients was analyzed. Photosensitivity was present in 98 patients (21.4%). The PPRs after sleep were increased in 70% of the photosensitive patients, did not change in 23%, and were decreased in 7%. The increase in duration of PPRs was statistically significant (P<0.001). In our cohort, photosensitivity would have been detected in 67 patients if IPS was applied only before sleep and in 91 patients if IPS was applied only after awakening (P<0.05). CONCLUSIONS: This study demonstrates that photosensitivity is enhanced after awakening from a short sleep following sleep deprivation. Thus, we recommend performing IPS after awakening to increase sensitivity to detect photoparoxysmal epileptiform discharges.