Skin-electroretinography patterns in early onset retinal dystrophies

Abstracts / Clinical Neurophysiology 130 (2018) e1–e19

subjects with normal TPEV had absent or markedly reduced fundamental harmonic and presence of a subharmonic. According to Shpak2 fSSVEPs can be useful in differential diagnosis of optic atrophy in children. These preliminary results suggest that the implementation of flash TVEPs methodology with fSSVEPs can provide further diagnostic information and encourage further studies in optic nerve and visual pathways disorders. doi:10.1016/j.clinph.2018.09.062

Clinical and electroencephalographic risk factors for severe outcome in asphyxiated neonates undergoing therapeutic hypothermia V. Spagnolo, M. Agatiello, D. Trevisanuto, G. Talenti, E. Cainelli, A. Suppiej Padova, Italy Ferrara, Italy In the first days after birth, a major focus of research is to identify infants with hypoxic ischemic encephalopathy at highest risk of death or severe neurological impairment. This is especially crucial in view of the discussion of redirection of care. We aimed to seek associations between some neonatal routinely recorded parameters and the development of severe outcomes. The study group included 83 neonates recruited for TH because of perinatal asphyxia, who underwent continuous EEG/aEEG monitoring. Severe outcome was defined as death or major neurological sequelae at one-year of age. 17% had seizures, mean age at seizure onset was 22.29 h (range 5– 35), mean total period with seizures 21.71 h (range 1–73), seizure burden (%of time seizuring/hour) mean 5.39% (range 0.92%12.17%), seizure frequency mean 1.8 seizure/hours (range 1–4.25). Severe outcome was significantly associated with pH and base excess measured in the first hour of life, mode of delivery, Apgar score, Sarnat & Sarnat score, EEG-confirmed neonatal epileptic seizures, antiepileptic therapy. Seizures with electro-clinical dissociation increased 6-fold the chance of having a severe outcome (OR CI 95%: 1.02–38.87). We suggest a multi-parametric protocol which can be useful to identify neonates at high neurological risk, available before rewarming from TH. doi:10.1016/j.clinph.2018.09.063

Psychopathology and patterns of sensory processing in nonsuicidal self-injured adolescents P. Lanteri, I. Seri, A. Aurigo, S. Rebora, L. Sciarretta Genova, Italy Nonsuicidal self-injury (NSSI) is a multifaceted and growing phenomenon among adolescents. This study aimed at analyzing some of the psychobehavioral correlates of NSSI compare to features of long latency Somatosensory Evoked Potentials (LL-SSEP). We studied 12 adolescents (mean age 14 ys ± 1 ys), presenting NSSI referred to the Infantile Neuropsychiatry Unit of Gaslini Children’s Hospital of Genoa. We divided the sample in two groups (frequent NSSI fNSSI and occasional NSSI - oNSSI) based on the Risk-Taking and Self-Harm Inventory for Adolescents (RITSHIA) for evaluated presence and severity of NSSI (cut off at Self-Harm subscale 16). We evaluated: the Theory of Mind subtest of NEPSY-II battery (TOM) for mentalizing ability, temporal features and topographical distribution bilaterally across the cortex of the LL-SSEP following physiological sequence N60-N70-N140. fNSSI presented lower mentalization ability in 5/6 patients compared to oNSSI (normal TOM score in 6/6 patients). Analyzing LL-SSEP we recorded not significant differences

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in latency of N60 and N70 and amplitude of N140, but fNSSI presented less representation and distribution with less amplitude of N60-N70 and increase latency of N140. Features of LL-SSEP correlate with the ability of mentalization and the intensity of NSSI behavior. doi:10.1016/j.clinph.2018.09.064

Skin-electroretinography patterns dystrophies A. Suppiej, V. Schoch, C. Ceccato

in

early

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Padova, Italy Ferrara, Italy Electroretinography can be performed early in life by noninvasive methodology, using skin electrodes. It provides unique insight into retinal function.1 The aim of this study was to evaluate patterns of rods and cones dysfunction in early onset retinal dystrophies (EORD), using ERG. Forty-eight patients (mean age 17.6 ± 15.4 months) presenting with sensory nystagmus and electro-clinical diagnosis of isolated retinal dystrophy, were reviewed. ERGs were recorded with skin electrodes, without pupillary dilatation and sedation, under photopic and scotopic conditions. Thirteen cases with Leber’s Congenital Amaurosis had severely depressed or flat ERG early in life. Nineteen children with Achromatopsia had stationary cone involvement confirmed by at least one follow up ERG, 8/19 had also a mild stationary reduction of the amplitude of scotopic ERG. Nine cases with rod-cone dystrophy had progressive abnormality with earlier and prevalent scotopic involvement. Seven patients with cone-rod dystrophy had progressive photopic ERG involvement exceeding scotopic ERG involvement. The ERG represents a valid diagnostic tool, particularly in infancy when other diagnostic tests are more challenging. Specific ERG phenotypes support EORD diagnosis allowing timely genetic testing. doi:10.1016/j.clinph.2018.09.065

Amyotrophic Lateral Sclerosis (ALS): Telemedicine system for home care and patient monitoring G. Fanella, G. De Salvo, V. Frasca, M. Calamani, E. Onesti, M. Ceccanti, C. Cucchiella, T. Barone, V. Pozzilli, I. Fiorini, M. Goglia, S. Di Bari, M. Inghilleri Roma, Italy The development of a new domotic healthcare system for patients with Amyiotrophic Lateral Sclerosis (ALS) to improve home-based clinical follow-up through the identification of functional loss worsening and complications, preserving interaction between patients and caregivers. The following electromedical wireless sensors were developed to analyse breathing, nutrition, motility and communication: Pulse Oximeter, surface EMG sensor associated to a dynamometer, Laryngophone, EEG-based BCI device. The project was divided into: (1) User centered design; (2) development of wireless sensors, remote terminal units, central server; (3) pilot clinical project and medical validation. Visual Analogic Scale (VAS) and SUS scales were used to analyse the user-friendliness and the satisfaction of each sensor. The domotic interface was tested on eight patients with ALS. SUS and VAS scores indicate a good system usability, both in satisfaction and in ease of use. The usability of the Laryngophone may be improved, even if ratings of recorded audiofiles performed well. The data collected, born from the collaboration between neurologists and biomedical engineers, led to innovative home medical devices and original algorithms that recognize alterations of vital parameters