Electrodiagnostic methods to verify Guillain-Barré syndrome subtypes in Istanbul: A prospective multicenter study.

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.

Determination of tibial somatosensory evoked potentials predicts detrusor sphincter dyssynergia in children with neurogenic bladder dysfunction.

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.

Neurogenic heterotopic ossification in Guillain-Barre syndrome: a rare case report.

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.

Prospective Analysis of the Swallowing Reflex After Sagittal Split Osteotomy: Comparison with Normal Volunteers.

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.

Multidisciplinary management of a partially edentulous patient with oromandibular dystonia: A clinical report.

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.

Exonic duplication CNV of NDRG1 associated with autosomal-recessive HMSN-Lom/CMT4D.

PURPOSE: Copy-number variations as a mutational mechanism contribute significantly to human disease. Approximately one-half of the patients with Charcot-Marie-Tooth (CMT) disease have a 1.4 Mb duplication copy-number variation as the cause of their neuropathy. However, non-CMT1A neuropathy patients rarely have causative copy-number variations, and to date, autosomal-recessive disease has not been associated with copy-number variation as a mutational mechanism. METHODS: We performed Agilent 8 × 60 K array comparative genomic hybridization on DNA from 12 recessive Turkish families with CMT disease. Additional molecular studies were conducted to detect breakpoint junctions and to evaluate gene expression levels in a family in which we detected an intragenic duplication copy-number variation. RESULTS: We detected an ~6.25 kb homozygous intragenic duplication in NDRG1, a gene known to be causative for recessive HMSNL/CMT4D, in three individuals from a Turkish family with CMT neuropathy. Further studies showed that this intragenic copy-number variation resulted in a homozygous duplication of exons 6-8 that caused decreased mRNA expression of NDRG1. CONCLUSION: Exon-focused high-resolution array comparative genomic hybridization enables the detection of copy-number variation carrier states in recessive genes, particularly small copy-number variations encompassing or disrupting single genes. In families for whom a molecular diagnosis has not been elucidated by conventional clinical assays, an assessment for copy-number variations in known CMT genes might be considered.

Dropping Objects in Carpal Tunnel Syndrome: Clinical and Electrophysiological Features.

Background Dysfunction in both afferent sensory and efferent motor components of the median nerve may contribute to the manifestation of the symptom of dropping objects (DO) in carpal tunnel syndrome (CTS). The objective of this study was to compare the clinical and electrophysiological characteristics of CTS patients with dropping objects (wDO) and those without dropping objects (w/oDO). The study evaluated the correlation between DO symptoms and median partial conduction block, as well as the reduction in median motor conduction velocity at the wrist. Methodology An examination for CTS and the Boston Carpal Tunnel Syndrome Questionnaire (BCTQ) were performed. Median and ulnar sensory and motor nerve conduction studies were conducted in both upper extremities, and the percentage decrease in the median compound motor action potential amplitude at the wrist level was calculated. Results The study included 201 female participants aged between 22 and 88 years, comprising 57 patients with CTSwDO, 78 patients with CTSw/oDO, and 66 healthy controls. In the CTSwDO group, compared to the CTSw/oDO group, BCTQ scores were significantly higher, the prevalence of sensory deficits was more pronounced, and a larger number of hands, both dominant and non-dominant, were classified as stage 3 or higher in the clinical CTS classification. However, no correlation was found between the presence of DO symptoms and any neurophysiological findings. Conclusions DO may be a symptom of clinical severity in CTS, as previously reported. No neurophysiological parameter that could be associated with DO was identified.

Prevalence of Carpal Tunnel Syndrome Symptoms Among Young Dentists.

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.

Effects of sagittal split osteotomy on brainstem reflexes.

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 (p < 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.

Cutaneous allodynia and thermal thresholds in chronic migraine: The effect of onabotulinumtoxinA.

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.

Default mode and dorsal attention network involvement in visually guided motor sequence learning.

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.

Nerve conduction studies in Charcot-Marie-Tooth disease in a cohort from Turkey.

INTRODUCTION: In the demyelinating form of Charcot-Marie-Tooth disease, median motor conduction velocity (MCV) was noted to be around 20 m/s in peripheral myelin protein 22 (PMP22) duplications, in contrast to higher MCVs in connexin 32 gene (Cx32) mutations and lower MCVs in the demyelinating form of myelin protein zero gene (MPZ) mutations. METHODS: Nerve conduction studies were performed in 64 families with both common and rare mutations. RESULTS: Mean MCV of the median nerve was 20 ± 5 m/s in PMP22 duplications, 34 ± 6 m/s in Cx32 mutations, 20 ± 9 m/s in KIAA1985 (SH3TC2) mutations, and 11 ± 8 m/s in MPZ mutations. Conduction was generally uniform; however, conduction blocks were present in 1 patient each with the MPZ mutation and PMP22 duplication, both with unusual phenotypes. CONCLUSION: Our results confirm those of the other investigators. Electrophysiological results of the rare KIAA1985 (SH3TC2) mutation reveal that their MCVs span a broad range and that conduction is uniform.

Restless Legs Syndrome in Patients with Behçet's Disease and Multiple Sclerosis: Prevalence, Associated Conditions and Clinical Features.

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.

Evaluation of Neurological Examination, SEP Results, MRI Results, and Lesion Levels in Patients Who Had Been Operated for Myelomeningocele.

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.

Laboratory and clinical correlates of brain atrophy in Neuro-Behçet's disease.

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.

Effect of the brain-derived neurotrophic factor gene Val66Met polymorphism on sensory-motor integration during a complex motor learning exercise.

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.

Are acquired hepatocerebral degeneration and hepatic myelopathy reversible?

BACKGROUND: Acquired hepatocerebral degeneration (AHD) and hepatic myelopathy (HM) are rare complications of chronic liver disease and are usually resistant to medical therapy. MATERIALS AND METHODS: The clinical and laboratory findings of 14 male and 2 female patients with AHD or HM were evaluated. RESULTS: The prevalence of AHD and HM was 2% inpatient case series in the last 10 years. The median age of the patients (5 Child's B and 11 Child's C) was 48.7 years (28 to 66 y), and the mean known duration of the liver disease was 75 months (24 to 194 mo). The median time of onset of neurologic findings after diagnosis of the liver disease was 14.5 months. Eight patients who had marked spastic paraparesis or tetraparesis were included in the HM group and all others had AHD group. Sixty-nine percent of the patients had a spontaneous or surgical portosystemic shunts, and the remaining dense retroperitoneal collaterals. During the follow-up period of median 29 months (4 to 72 mo), 12 patients died while waiting for liver transplantation, and these patients suffered from the several complications of chronic liver disease more than the living patients. A marked improvement was observed in 2 of the patients (1 with AHD and the other with HM) at 6 and 8 months after the liver transplantation, respectively. CONCLUSIONS: Our data suggest that liver transplantation had an important effect on the improvement in these patients.

The evaluation of interictal focal EEG findings in adult patients with absence seizures.

PURPOSE: To investigate the focal interictal EEG abnormalities in adult patients with absence seizures (ASs) and to identify their clinical, EEG and semiological correlates. METHODS: Fifty patients older than 18 years, diagnosed as having IGE with AS documented with ictal recordings. Interictal focal sharp or spike-waves and strictly focal paroxysmal slow activity were considered as focal EEG features. The patients having focal EEG features were classified as "Group I", whereas the remaining of them was classified as "Group II". RESULTS: We observed focal findings in 34% of the patients, mainly in frontotemporal (41%), and frontal (29%) regions. There were no significant differences with respect to the clinical parameters such as sex, epilepsy duration, positive family history and the age of the onset between the groups. Psychiatric co-morbidities were significantly higher in Group I when compared to Group II (P=0.00). Accompanying automatisms were higher in Group I, whereas eye deviation during absences was higher in Group II. In Group I, the asymmetry of the ictal discharges was more frequently observed. Focal EEG features were more frequently seen in juvenile absence epilepsy syndrome, without reaching a significance level. CONCLUSION: The focal findings in adult absence epilepsy patients could have some unknown etio-pathogenetic and prognostic implications. We emphasize the cautious interpretation of isolated interictal focal EEG abnormalities to prevent a wrong diagnosis of focal epilepsy in patients who may indeed suffer from generalized epilepsy.

The Impact of Affective State on Quality of Life in Focal Epilepsy in Turkey.

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.

Erratum: Evaluation of OnabotulinumtoxinA Treatment in Patients with Concomitant Chronic Migraine and Temporomandibular Disorders.

[This corrects the article on p. 330 in vol. 55, PMID: 30622389.].