Hava Ozlem Dede

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.

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.

S101. A novel automatized F-wave MUNE method: A preliminary analysis

Introduction The aim of this study was to test a novel automatized F-wave MUNE method by comparing its results with those elicited with the manual method performed in patients with amyotrophic lateral sclerosis (ALS) and healthy controls. Methods Ninety F-waves elicited with supramaximal stimuli were recorded from thenar and hypothenar muscles in 10 ALS patients and 4 healthy controls. For manual analysis, F-waves with the same amplitude, latency and shape were selected visually by the same examiner. For automatized analysis, similar signals were grouped according to latencies of negative and positive peaks and peak-to-peak amplitudes in MATLAB by using the original software developed for this study. The mean amplitudes of F-waves repeating more than once were defined as sMUPs and MUNE value for each case was calculated by dividing the CMAP amplitude by mean sMUP amplitude. Results Data of two healthy controls and 1 ALS patient were excluded from the analysis since no repeater F-waves were detected by one of the methods. sMUP and F-wave MUNE values calculated by both methods were found to be highly correlated (Spearman’s rho, p μ V and 406.55  μ V, respectively in thenar muscles and 412.53  μ V and 332.77  μ V in hypothenar muscles). Similar F-wave MUNE values were also calculated by using both methods (26.00 and 28.58, respectively in thenar muscles and 36.04 and 30.18 in hypothenar muscles). Conclusion Our automatized F-wave MUNE method seems to be promising in reflecting motor unit numbers in the hand muscles in patients with ALS and healthy controls.

T40. Comparison of MScanFit MUNE and other parameters derived from the CMAPScan in ALS patients

Introduction The aim of this study was to analyse MScanFit MUNE, D50 and step% values elicited from the CMAP scan curves of thenar and hypothenar muscles in patients with amyotrophic lateral sclerosis (ALS) and healthy controls. Methods Thirty-four ALS patients (31 definite, 1 probable and 2 possible, according to Awaji criteria) and 24 healthy controls were included. CMAP scan curves were recorded from both thenar and hypothenar muscles by applying 500 stimuli. Step% and D50 values were calculated semi-automatically and MScanFit MUNE was calculated by using the freeware version of Qtrac software. Results Mean MScanFit MUNE and D50 values were lower and step% values were higher significantly in patients (48.8, 25.0, 32.5 in thenar muscles and 66.5, 28.9, 17.0 in hypothenar muscles, respectively) as compared to healthy controls (112.5, 45.6, 4.0 in thenar muscles and 124.5, 46.6, 1.1 in hypothenar muscles; p p Conclusion Step% and MScanFit MUNE are parameters correlated with each other and they are sensitive tools in revealing the severities and the courses of the electrophysiological abnormalities in ALS.

F52. Repeater F-waves: Electrophysiologic manifestations of upper or lower motor neuron involvement?

Introduction The aim of this study is to compare repeater F-wave parameters with CMAP amplitudes recorded from the hand muscles (as indicators of the amount of lower motor neuron [LMN] loss) and with the triple stimulation technique (TST) ratios in the same muscles (as indicators of upper motor neuron [UMN] involvement) in 19 patients with amyotrophic lateral sclerosis (ALS). Methods Ninety F-waves were recorded from thenar and hypothenar muscles by supramaximal stimuli applied to the relevant nerves at the wrist level. Repeater F-waves (F rep ) were visually defined as those presented with the same amplitude, latency and shapes. Repeater neuron (RN) index, F rep index, persistances of F-waves, F/M amplitude ratio and the neurophysiologic index (NI) were calculated. By depending our laboratory normals, TST ratio values lower than 82% and 86% were accepted as abnormal showing UMN involvement in thenar and hypothenar muscles respectively. Results TST ratios were abnormal in 13 thenar and 11 hypothenar muscles. None of the F rep parameters showed any significant relationship with the abnormal TST ratios, except the lower F rep index in thenar muscles, in patients with UMN involvement according to low TST ratios. However, there was a strong negative correlation with the CMAP amplitudes and F rep index and RN index values in hypothenar muscle (Spearman’s rho, p Conclusion These findings imply that repeater F waves might be a manifestation of LMN loss more than being a result of UMN involvement.