The relationship between cartilage thickness and muscle thickness or leg length discrepancy in poliomyelitis sequelae.

OBJECTIVES: To evaluate the relationship between cartilage loss and differences in muscle thickness and/or leg length in poliomyelitis sequelae (PMS). Our study is the first to evaluate the relationship between cartilage loss and both muscle atrophy and leg length discrepancy in the same population. METHODS: 37 patients with PMS and 38 healthy controls were included. Talar and distal femoral cartilage thicknesses and gastrocnemius medialis and quadriceps femoris muscle thicknesses were measured via ultrasound. Leg length differences and manual muscle strength were also evaluated. RESULTS: The mean muscle thicknesses and cartilage thicknesses were thinner in the more affected legs than in the less affected legs in the patient group. All of the ultrasonographic measurements were thinner in the less affected legs of the patient group than in the right legs of the control group, except for the knee cartilage thicknesses. While there was a correlation between the cartilage thickness difference and the muscle thickness difference between the less and more affected legs in the patient group, there was no correlation between the cartilage thickness difference and leg length differences. CONCLUSIONS: Patients with PMS are predisposed to osteoarthritis. Talar and knee cartilage thicknesses may be more associated with the muscle thickness than the leg length discrepancy in PMS.

Rapid eye movement sleep without atonia constitutes increased risk for neurodegenerative disorders.

OBJECTIVES: REM (rapid eye movement) sleep without atonia (RSWA) is a polysomnographic finding used in diagnosis of REM sleep behavior disorder (RBD). Clinical significance of idiopathic RSWA (iRSWA) unaccompanied by RBD is not known. We designed a prospective study to investigate whether iRSWA constitutes an increased risk for developing neurodegenerative disorders. MATERIALS AND METHODS: Between January 2010 and December 2014, a total of 4362 patients underwent a full-night video-polysomnography. Upon detailed clinical and polysomnographical examination, patients with iRSWA and idiopathic RBD (iRBD) were enrolled into this study and followed up at every six months for at least 4 years up to 9 years. RESULTS: We had a total of 31 patients with iRBD and 67 patients with iRSWA. Mean age was higher in iRBD group than those in iRSWA group (P = .016). Restless legs syndrome/Willis-Ekbom disease was significantly more common in patients with iRBD than those in patient with iRSWA (P < .001). Eighteen patients with iRSWA (26.8%) developed iRBD after 2.6 + 2.2 years. Six patients with iRSWA (8.9%) developed neurodegenerative disorders following 2.4 + 1.5 years; four were diagnosed as Parkinson's disease (PD) and two developed probable Alzheimer-type dementia. In patients with iRBD, eight patients (25.8%) developed neurodegenerative disorders-all was Parkinson's disease-following 2.6 + 2.2 years. Development of neurodegenerative diseases was positively correlated with age (P < .001) and periodic leg movements in sleep in both groups (P < .010). CONCLUSIONS: These results show that iRSWA may also be accepted as a risk factor in the development of PD or neurodegenerative diseases. Advanced age and periodic leg movements in sleep seem to be correlated with higher risk.

Genital automatisms: Reappraisal of a remarkable but ignored symptom of focal seizures.

OBJECTIVES: Genital automatisms (GAs) are uncommon clinical phenomena of focal seizures. They are defined as repeated fondling, grabbing, or scratching of the genitals. The aim of this study was to determine the lateralizing and localizing value and associated clinical characteristics of GAs. METHODS: Three hundred thirteen consecutive patients with drug-resistant seizures who were referred to our tertiary center for presurgical evaluation between 2009 and 2016 were investigated. The incidence of specific kinds of behavior, clinical semiology, associated symptoms/signs with corresponding ictal electroencephalography (EEG) findings, and their potential role in seizure localization and lateralization were evaluated. RESULTS: Fifteen (4.8%) of 313 patients had GAs. Genital automatisms were identified in 19 (16.4%) of a total 116 seizures. Genital automatisms were observed to occur more often in men than in women (M/F: 10/5). Nine of fifteen patients (60%) had temporal lobe epilepsy (right/left: 4/5) and three (20%) had frontal lobe epilepsy (right/left: 1/2), whereas the remaining two patients could not be classified. One patient was diagnosed as having Rasmussen encephalitis. Genital automatisms were ipsilateral to epileptic focus in 12 patients and contralateral in only one patient according to ictal-interictal EEG and neuroimaging findings. Epileptic focus could not be lateralized in the last 2 patients. Genital automatisms were associated with unilateral hand automatisms such as postictal nose wiping or manual automatisms in 13 (86.7%) of 15 and contralateral dystonia was seen in 6 patients. All patients had amnesia of the performance of GAs. CONCLUSION: Genital automatisms are more frequent in seizures originating from the temporal lobe, and they can also be seen in frontal lobe seizures. Genital automatisms seem to have a high lateralizing value to the ipsilateral hemisphere and are mostly concordant with other unilateral hand automatisms. Men exhibit GAs more often than women.

Analysis of heart rate variability and risk factors for SUDEP in patients with drug-resistant epilepsy.

BACKGROUND: Cardiac problems have been suggested as causes of sudden unexpected death in epilepsy (SUDEP). Our aim was to investigate possible associations of cardiac autonomic functions based on heart rate variability (HRV) parameters with risk factors of SUDEP in patients with drug-resistant epilepsy. METHODS: Forty-seven patients with drug-resistant seizures and 45 healthy control subjects were enrolled in the study. Interictal time domain parameters of HRV were evaluated with 24-hour Holter recordings. Potential SUDEP risk in patients with epilepsy was estimated using an inventory of seven validated SUDEP risk factors (The SUDEP-7 inventory). RESULTS: When compared with the healthy controls, all time domain measures (SDNN-24, SDNN-index, SDANN-index, RMSSD and pNN50) were significantly suppressed in the patient group. Scores of the SUDEP-7 inventory ranged from 1 to 9 with a median 4 out of a maximum possible risk score of 10. Maximum heart rate value in 24-hour Holter recordings and epilepsy duration were correlated with the SUDEP-7 scores (r=0.3, p=0.03). We found no significant association with HRV measures and SUDEP-7 risk factors. One patient diagnosed with Dravet syndrome died of SUDEP, which was autopsy confirmed; his SUDEP-7 inventory score was 7, HRV measures were significantly diminished, and his maximum heart rate (HR) was 208beats/min (maximum HR is between 104 and 188beats/min in normal subjects). CONCLUSION: Patients with drug-resistant epilepsy present with significantly lower HRV measures, which may increase the risk for sudden cardiac death. Increased heart rate and diminished HRV measures may constitute one of the possible mechanisms underlying SUDEP and should be diagnosed in patients with epilepsy.

The clinical significance and electrophysiologic findings of fixation-off and closure of the eyes sensitivity: Data from a prospective unselected population.

PURPOSE: Electroencephalography (EEG) findings related to the eye-closing motion can be defined in two ways: 'sensitivity to eyes closed' (SEC) and 'eye closure sensitivity (ECS).' Fixation-off sensitivity (FOS) is a different phenomenon induced by the elimination of central vision/fixation. The purpose of our study was to determine the frequencies of SEC, ESC, and FOS, and to analyze the relationship between eyes closure and the fixation-off phenomenon and clinical importance in an unselected population. METHODS: We prospectively evaluated 200 routine interictal EEGs by adding a standardized FOS examination protocol between June and September 2015. Goggles covered with semitransparent tape were used to evaluate FOS. We determined SEC when the epileptiform discharges appeared during eye closure and continued during the eye closed state, whereas ECS was defined as transient epileptic abnormalities following the closure of the eyes lasting for 1-4 sec. The patients were evaluated in terms of demographic characteristics, clinical features, and the relationship between SEC, ECS, and FOS. RESULTS: We detected SEC in 9 (4.4 %) and ECS in 11 (5.4 %) patients. FOS was detected in four (44.4 %) of the patients who showed SEC, all of whom had occipital epileptiform discharges. A statistically significant correlation was found between FOS and treatment resistance in the SEC group (p < 0.001). In logistic regression analysis, occipital lobe epilepsy (p < 0.001) and age under 20 years (p = 0.004) were found as risk factors for SEC. Another interesting finding was the suppression of epileptic discharges with fixation-off in three of 11 patients with ECS. CONCLUSIONS: According to the results of our study, FOS is related to treatment resistance. Therefore, FOS should be evaluated in patients with SEC.

Corticomotor Excitability in Two Kinds of Motor Neuron Diseases: A Study on the Patients With Amyotrophic Lateral Sclerosis and Poliomyelitis Survivors.

PURPOSE: To examine upper motor neuron functions comparatively in patients with amyotrophic lateral sclerosis (ALS) and poliomyelitis survivors using transcranial magnetic stimulation (TMS) methods. METHODS: Single- and paired-pulse TMS with conventional methods and the triple-stimulation technique were performed by recording from the abductor digiti minimi and abductor pollicis brevis muscles in 31 patients with ALS, 18 patients with poliomyelitis survivors, and 21 controls. Nine patients were diagnosed as having postpoliomyelitis syndrome after a 6-month follow-up. RESULTS: Triple-stimulation technique and some of conventional TMS studies were able to distinguish ALS from both poliomyelitis survivors and controls. A reduced ipsilateral silent period in abductor pollicis brevis muscles was the only parameter to show a significant difference when comparing thenar and hypothenar muscles in ALS. No significant difference was present in any TMS parameters between the postpoliomyelitis syndrome and non-postpoliomyelitis syndrome groups. CONCLUSIONS: Conventional TMS and particularly triple-stimulation technique studies are helpful in disclosing upper motor neuron dysfunction in ALS. The results of this study might favor the cortical hypothesis for split hand in ALS, but they revealed no significant indication for upper motor neuron dysfunction in postpoliomyelitis syndrome.

Depression is a major determinant of sleep abnormalities in patients with epilepsy.

INTRODUCTION: We aimed to identify sleep disorders in patients with epilepsy and compare this group with a healthy population. We also analyzed the features of sleep disorders in patients with epilepsy to demonstrate the effect of seizures and seizure types on sleep. METHODS: Our study assessed 43 patients with epilepsy and 53 age- and gender-matched healthy controls. The demographic and clinical data of all participants were recorded. The Epworth Sleepiness Scale, Pittsburgh Sleep Quality Index (PSQI), International Restless Legs Syndrome Study Group Rating Scale, Berlin Questionnaire, and Beck Depression Inventory (BDI) were administered to all study subjects. The interview used to evaluate insomnia is based on the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition - DSM-5 diagnostic criteria. RESULTS: Twenty-four patients (55.8%) and 26 controls (49.1%) are women. The mean age of patients and controls was 34.2±11.37 (16-71) and 34.6±11.28 (16-77), respectively. Patients with epilepsy had depression more often than controls, a result that was statistically significant (p<0.0001). We found no statistically significant difference between sleep parameters of patients and controls with normal BDI scores (p>0.05). Patients with depression had worse results on the Berlin Questionnaire and PSQI total score, with statistical significance (p=0.002). Nocturnal seizures, seizure type, and drug treatment had no effect on sleep (p>0.05). CONCLUSION: We concluded that depression rather than epilepsy negatively affects sleep, suggesting that all patients should be asked about their mood and sleep complaints.

Electrophysiological Findings of Subclinical Lower Motor Neuron Involvement in Degenerative Upper Motor Neuron Diseases.

INTRODUCTION: The present study is an examination of possible subclinical involvement of lower motor neuron (LMN) in patients with primary lateral sclerosis (PLS) and hereditary spastic paraparesis (HSP) electrophysiologically. METHODS: Nine PLS patients and 5 HSP patients were prospectively analyzed. Jitter measurement with concentric needle electrode (25 mm, 30 G) (CN-jitter) recorded from right extensor digitorum muscle during voluntary contraction with 1 kHz high-pass frequency filter set. European Myelopathy Score (EMS) was used to evaluate disability. The relationship between disability score and jitter values was investigated. RESULTS: HSP patients had suffered from the disease for longer period of time (p<0.001). Mean jitter values of patients with PLS and HSP were 26.5±12.1 µs and 30.8±34.8 µs, and the number of individual high jitters (>43 microseconds) observed in the PLS and HSP groups was 16/180 and 9/100, respectively without a significant intergroup difference. The ratio of patients with an abnormal jitter study were higher in HSP group (60%) compared to PLS (22%) (p<0.05). Potential pairs with blocking were present in HSP group (7 of 100 potential pairs) but not seen in PLS patients. EMS values were significantly lower in patients having potential pairs with high jitter and blocking compared to those without high jitter and blocking. CONCLUSION: The present study has demonstrated that early signs of LMN dysfunction can be detected electrophysiologically by CN-jitter in patients with UMN involvement. These electrophysiological findings in these patients with longer disease duration and lower clinical scores may be explained by spreading of the disease to LMNs or transsynaptic degeneration and its contribution in disease progression.

Changes in motor unit bioelectrical activity recorded at two different sites in a muscle.

INTRODUCTION: The objective of this study was to compare the properties of bioelectrical signals of motor units recorded at different sites in the muscles of controls, patients with myopathy and patients with motor neuron disease (MND). METHODS: Five controls, 10 patients with myopathy and 11 patients with MND were included. Electrophysiologic tests were performed in the biceps brachii (BB) muscle from two recording sites. Site 1 was near the belly of the muscle and Site 2 was 5cm distal from Site 1, near the tendon. Multi-motor unit potential (MUP) analysis, jitter analysis, and peak number count were calculated from the signals recorded using a concentric needle electrode (CN). RESULTS: At Site 2, duration was longer, number of phases was higher and amplitudes were smaller in MUPs compared with those recorded at Site 1. This significant difference between recording site and patient groups was related to neurogenic muscles. Jitter analysis showed no significant difference except an intergroup difference between the patient groups and controls. The peak number calculated using the CN was greater when recorded from Site 1 in concordance with MUP analysis. CONCLUSION: Duration of MUP was longer and amplitude was smaller when the recording electrode was placed distally along the muscle near the tendon in neurogenic muscles, probably related to increased temporal dispersion. However, changing the position of the needle did not provide further information in distinguishing myogenic muscles.

Repeater F-waves in amyotrophic lateral sclerosis: Electrophysiologic indicators of upper or lower motor neuron involvement?

OBJECTIVE: To extract insight about the mechanism of repeater F-waves (Frep) by exploring their correlation with electrophysiologic markers of upper and lower motor neuron dysfunction in amyotrophic lateral sclerosis (ALS). METHODS: The correlations of Frep parameters with clinical scores and the results of neurophysiological index (NI), MScanfit MUNE, F/M amplitude ratio (F/M%), single and paired-pulse transcranial magnetic stimulation (TMS), and triple stimulation technique (TST) studies, recorded from abductor digiti minimi (ADM) and abductor pollicis brevis (APB) muscles of 35 patients with ALS were investigated. RESULTS: Frep parameters were correlated with NI and MScanfit MUNE in ADM muscle and F/M% in both muscles. None of the Frep parameters were correlated with clinical scores or TST and TMS measures. While the CMAP amplitudes were similar in the two recording muscles, there was a more pronounced decrease of F-wave persistence in APB, probably heralding the subsequent split hand phenomenon. CONCLUSION: Our findings suggest that the presence and density of Freps are primarily related to the degree of lower motor neuron loss and show no correlation with any of the relatively extensive set of parameters for upper motor neuron dysfunction. SIGNIFICANCE: Freps are primarily related to lower motor neuron loss in ALS.

[Comparison of triptans, NSAID and combination in migraine attack treatment]. / Migren atak tedavisinde triptan, NSAII ve kombinasyon tedavisinin karsilastirmasi.

OBJECTIVES: Headache during migraine attack may recur during a single attack. The present study evaluated efficacy of both individual and combined use of agents from nonsteroidal anti-inflammatory drug (NSAID) and triptan groups widely used in treatment of migraine attacks. METHODS: A total of 201 attacks in 67 migraine patients were evaluated. Patients were divided into 3 study groups: those receiving rizatriptan 10mg, tenoxicam 20 mg, and rizatriptan + tenoxicam (combination). Patients evaluated severity of headache based on visual analogue scale (VAS) at moment of drug delivery, after 30 minutes, and after 1, 2, 4, 8 and 24 hours. Attacks were evaluated separately for each treatment alternative, and results were also compared. RESULTS: VAS values were the same at onset of attack, but were lower in rizatriptan and combination groups than in tenoxicam group at 30 minutes and onward. VAS score was above 4 at 60 minutes in tenoxicam group and mean VAS value in this group was found to be significantly higher than values in rizatriptan and combined groups. At 24 hours, VAS scores were similar in combination and tenoxicam groups, while rizatriptan group had higher mean VAS score than the other 2 groups. CONCLUSION: When single drug use fails to provide adequate control, combined use of a rapid-acting triptan and a long-acting NSAID appears to be a suitable treatment option.

Depression is a major determinant of sleep abnormalities in patients with epilepsy / A depressão é um dos principais determinantes das anormalidades do sono em pacientes com epilepsia

ABSTRACT Introduction: We aimed to identify sleep disorders in patients with epilepsy and compare this group with a healthy population. We also analyzed the features of sleep disorders in patients with epilepsy to demonstrate the effect of seizures and seizure types on sleep. Methods: Our study assessed 43 patients with epilepsy and 53 age- and gender-matched healthy controls. The demographic and clinical data of all participants were recorded. The Epworth Sleepiness Scale, Pittsburgh Sleep Quality Index (PSQI), International Restless Legs Syndrome Study Group Rating Scale, Berlin Questionnaire, and Beck Depression Inventory (BDI) were administered to all study subjects. The interview used to evaluate insomnia is based on the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition - DSM-5 diagnostic criteria. Results: Twenty-four patients (55.8%) and 26 controls (49.1%) are women. The mean age of patients and controls was 34.2±11.37 (16-71) and 34.6±11.28 (16-77), respectively. Patients with epilepsy had depression more often than controls, a result that was statistically significant (p<0.0001). We found no statistically significant difference between sleep parameters of patients and controls with normal BDI scores (p>0.05). Patients with depression had worse results on the Berlin Questionnaire and PSQI total score, with statistical significance (p=0.002). Nocturnal seizures, seizure type, and drug treatment had no effect on sleep (p>0.05). Conclusion: We concluded that depression rather than epilepsy negatively affects sleep, suggesting that all patients should be asked about their mood and sleep complaints.

RESUMO Introdução: O objetivo deste estudo foi identificar distúrbios do sono em pacientes com epilepsia e compará-los com uma população saudável. Também foram examinadas as características dos distúrbios do sono em pacientes com epilepsia para demonstrar o efeito e os tipos de convulsões no sono. Métodos: O estudo consistiu em 43 pacientes com epilepsia e 53 controles saudáveis ​​pareados por idade e sexo. Os dados demográficos e clínicos de todos os participantes foram registrados. Todos os participantes do estudo receberam a Escala de Sonolência de Epworth, o Índice de Qualidade do Sono de Pittsburgh (Pittsburch Sleep QUality Index - PSQI), o Questionário do Grupo Internacional de Estudos das Pernas Inquietas, o Questionário de Berlim e o Inventário de Depressão de Beck. A entrevista usada para avaliar a insônia é baseada nos critérios do DSM-V. Resultados: Vinte e quatro pacientes (55,8%) são do sexo feminino e vinte e seis do grupo controle (49,1%) são do sexo feminino. A média de idade dos pacientes e do grupo controle é de 34,2±11,37 (16-71) e 34,6±11,28 (16-77), respectivamente. Pacientes com epilepsia sofrem mais de depressão do que os controles e esse resultado é estatisticamente significativo (p<0,0001). Não houve diferença estatisticamente significativa entre os parâmetros do sono dos pacientes e dos controles que apresentaram escores de Beck normais (p>0,05). Pacientes com depressão apresentam resultados piores no escore total do PSQI de Berlim, o que é estatisticamente significativo (p=0,002). As convulsões noturnas, o tipo de convulsão e o tratamento medicamentoso não afetaram o sono (p>0,05). Conclusão: Determinamos que a depressão, e não a epilepsia, afeta negativamente o sono dos pacientes, sugerindo que todos os pacientes devem ser questionados sobre seu humor e queixas de sono.