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To study the evolution of sensory-motor nerves in the upper and lower limbs in neurologically healthy preterm infants and to use sensory-motor studies to compare the rate of maturation in preterm infants at term age and full-term healthy neonates. The study comprised 26 neurologically normal preterm infants born at 23–33 weeks of gestational age, who underwent sensory nerve conduction and motor nerve conduction studies from plantar medial and median nerves and from tibial and ulnar nerves, respectively. We repeated the same neurophysiological studies in 19 of the preterm infants every 2 weeks until postnatal term age. The data from the preterm infants at term was matched with a group of ten full-term babies a few days after birth. The motor nerve conduction velocity of the tibial and ulnar nerves showed progressive increases in values in relation to gestational age, but there was a decrease of values in distal latencies and F wave latencies. Similarly, there was a gradual increase of sensory nerve conduction velocity values of the medial plantar and median nerves and decreases in latencies in relation to gestational age. At term age, the preterm infants showed significantly lower values of conduction velocities and distal latencies than the full-term neonates. These results were probably because the preterm infants had significantly lower weights, total length and, in particular, distal segments of the limbs at term age. The sensory-motor conduction parameters were clearly related to gestational age, but extrauterine life did not affect the maturation of the peripheral nervous system in the very preterm babies who were neurologically healthy.

Background Refractory chronic migraine (rCM) [1] is a debilitating neurological disorder, characterized by headache on ≥ 15 days per month for > 3 months, resistant to conventional symptomatic and/or prophylactic polytherapy. The effectiveness of the OnabotulinumtoxinA (OnabotA) was demonstrated in PREEMPT trials and approved in 2010 for CM treatment [2,3]. The prophylactic pharmacological actions of OnabotA include: a direct antinociceptiveanalgesic effect for primary peripheral afferent terminals by inhibiting release of nociceptive mediators (glutamate, substance P, CGRP) [4] and an indirect effect presumed to involve inhibition of peripheral and central sensitization in trigeminovascular neurons. The purpose of this study was to evaluate the efficacy, safety and tolerability of OnabotA as a prophylactic therapy in patients with rCM and observe the influence of the type of pain on the effectiveness of the treatment itself.

Aim of this study is to evaluate the relationships between a new classification of somatosensory-evoked-potentials in cascade (SEPs-C) with simultaneous video-electroencephalogram (VEEG) obtained by 1-h integrated neurophysyiological monitoring (1 h-IMNA) and clinical assessment by Sarnat–Sarnat (SS), Cerebral arteries Resistance-Index (RI) using pulsed-Doppler and Magnetic Resonance Imaging (MRI) as early predictors of a 1-year outcome in Hypoxic-ischemic-encephalopathy (HIE). 1 h-IMNA of VEEG and SEPs (bilateral median nerve stimulation) were assessed in 8 (4M-4F) asphyxiated term-infants within 1 and 4 weeks from birth. In all infants SS, RI and MRI were early performed. 1 h-IMNA and RI were also assessed in 20 healthy term-infants, as control-group. Trend of SEPs-C was evaluated in relation to behavioural-states during the VEEG-recording (wakefulness, quiet/active sleep). In control-group SEPs-C were present and modulated. In the 8 asphyxiated infants were identified 4 cortical SEPs’ patterns: bilateral absence/pathological-alteration (A–A), monolateral absence/pathological alteration (A–N), hypovoltated/non-modulated (h/nm), normal (NN) in agreement with RI and MRI. The recognize of SEPs-C pattern during 1 h-IMNA, considering hypovoltage and/or modulation in relation to behavioural-state, especially if concording with RI and MRI’s patterns, could implement the early prognostic-value to identify the moderate asphyxiated newborns. This proposal of neonatal SEPs’classification and its relations with IR and RMI call for further studies as early predictors of HIE, especially if mild.

The purpose of this study was to evaluate the efficacy, safety and tolerability of onabotulinumtoxinA as prophylactic therapy in patients with “refractory” chronic migraine (CM). We analyzed 76 patients (12M, 64F; mean age 52 years- 23–82) with CM resistant to conventional syntomatic and/or prophylactic polytherapy, afferent to the Regional Reference Center of Chemical denervation (RRCCd) of Careggi Hospital, in 2011–2014 period. Each patient was treated (after informed consent) with onabotulinum-toxin A (dose-range 155–195U) every 3 months, according to Blumenfeld’s protocol. We considered the following parameters: frequence (F) and intensity (I) of migraine attacks and drug’s use (D). We employed an ANOVA for repeated measures to test for changes across time. All 76 patients received the toxin treatment at least four time: 44/76 responded to treatment (38F-6M).The parameters F, I, D showed a progressive and gradual decline with time ( p OnabotulinumtoxinA represents a safe, tollerated and effective prophylactic treatment in “refractory” chronic migraine.

Neonatal encephalopathy is a significant cause of infant mortality and morbidity with risk of neurological sequelae in the survivors of neonates admitted to Neonatal (N) Intensive Care Unit (ICU). The EEG and Evoked Potentials (EPs) are very informative in the ICU. In particular, it is known that the SomatoSensory (SS) EPs are the best single indicator of early prognosis in adults and children patients with traumatic and/or hypoxic-ischemic coma compared to the Glasgow Coma Scale (GCS) and CTscan. Most paediatric studies excluded newborns in an attempt to eliminate the age effects, because of the structural and functional immaturity of somatosensory system. In fact, newborns differ from adults and paediatric patients for many aspects: hypoxic-ischemic aetiology, SSEPs normative data, grading and predictive values, timing and techniques recording, clinical scales of evaluation. Recently a diagnostic and predictive role of early SSEPs has been established in perinatal hypoxic-ischemic. We reported a literature review of early diagnostic/prognostic role of SSEPs and our preliminary neurophysiological data of prospective study in mild or severe perinatal hypoxic-ischemic insult.

We used combined Electroneurography and Muscular-UltraSound study (c-ENG-MUS) to evaluate the morpho-functional neuromuscular aspects in healthy neonatal and adult-juvenile populations. The Tibial Anterior muscle was evaluated in 17 neonates, 10 Full-terms (38–40 ws)/ 7 preterms (32–36 ws) and in 11 young adults (19–45 ys) by c-ENG-MUS, measuring: (1) Onset-Peak (OP) and peak-to-peak (PP) amplitudes of maximum Motor-Compound-Action-Potential (mCMAP); (2) maximum Muscular Thickness (mMT); (3) MT at mCMAP’s detection point (mCMAP-MT). In preterms we performed serial studies until the term-age. (Study approved by the Local-Ethics-Committee). In newborns the OP- and PP-mCMAP amplitudes didn’t correlate linearly, unlike the adults; in the newborns’ and adults’ populations the mCMAP-MT and mMT values linearly relate. In newborns, the mCMAP’s amplitude and MT’s absolute value were about half of the adults’ ones. The detection points of mCMAP and mMT didn’t correspond. In preterms the values of mCMAP-amplitude and mMT proportionally increased to the postconceptional-age. The values of mCMAP-amplitude, mMT and mCMAP-MT significantly differed in neonates (full-terms and preterms) respect the adults because of the effect of age. There was no correlation between the mCMAP-amplitude and mMT (no significant site-effect). c-ENG-MUS is a quiclky and easy technique to evaluate the neuromuscular functional and morphological maturational processes.

The computer technologies advancement with the spread of hospital wireless networks has introduced the new concept of Telemedicine. This has increased the responsibility of Neurophysiological Technologist(NT), called in the “front line” to interact with the Neurophysiologist in another forum. Provide our management experience in Neonatal-ICU that connects quickly the work of the neonatologist’s ICU with the neurophysiologist in his clinic, using the wireless system. Standardised collection, agreed with the neurophysiologist, medical history and the Video Capture(VC) of locomotor repertoire of the neonate, prior to the neurophysiological examination. Improving of the skills of the NT in early recognition of pathological movements to enhanced their monitoring and VideoEEG enforcement. For each newborne “tailored”, anamnestic, somatic and clinical data on-board-computerized were collected.; before the 1 h-VideoEEG-recording, we performed Short-VC(5′-10′) viewable both off and on-line, to evaluating neonates’ spontaneous movements and subsequent neurological assessment simplified by Mercuri’s table (2005) sharing of two parts for the anamnestic collection and for a first clinical approach of neurological newborn, useful for improving the interpretation of neurophysiological data. The competence and reliability of the NT can implement the synergy of different professional figures in the diagnosis and treatment in Neonatal ICU using “WI-FI”.

Neuromuscular diseases, in paediatric age, represent a diagnostic and therapeutic challenge: they are progressive, invalidant and often their clinical presentation, like the equines-foot, constitute a bridge between Neurology and Orthopedy. The aims of this study are:1) present epidemiology of clinical cases observed in our Neuro- Orthopaedic outpatient experience from 2009 to 2014; 2) propose a diagnostic and therapeutic flow-chart to a better overview and management of pediatric equines-foot. We clinically evaluated 600 new patients: 480 showed a mono/bi-lateral motor-deficit of lower limbs. Of these 480: 14 were identified as cerebral palsy, 70 needed further neurophysiological (ENG/EMG) and/or neuromuscular- ultrasound-study to set the diagnosis. In 27 on 70 patients, a neuromuscular and/or spinal-cord disease was confirmed with the help of MRI, genetic and immunohistochemistry tests. In remaining 43 cases the negativity of neurophysiological study directed the diagnosis to orthopaedic-sphere with the exception of 4 clinical uncertain cases which required clinical follow-up. Through this diagnostic algorithm each neurological subject was directed to a focused neuro-rehabilitation (physiotherapy, botulinum toxin,supports) and/or to surgery. A Neuro-Orthopaedic diagnostic and therapeutic flow-chart to overview the paediatric equinus-foot facilitates the management of patient establishing area of competence, therapeutic and/or rehabilitative-priority and optimizing human ansi?d scientific resources.

Paediatric neuromuscolar diseases are clinically heterogeneous, progressive and disabling, often requiring invasive, uncomfortable and expensive investigations. Ultrasonographic evaluation of neuromuscular diseases is an highly specific and sensitive first level screening-tool but only few paediatric normative data were reported. We performed a wide muscular ultrasound (MUS) study on 120 healthy children (2–16 y old), evaluating muscular echogenicity and thickness. All values were indipendently analyzed off-line and measured by two Neurosonologists (SL and EM) upon training and test standardization. The agreement between the two measurements was evaluated by the statistic Bland–Altman test. We report the statistical results obtained with measurements performed by two operators in 20 children. For each subject we analyzed both echogenicity and thickness of 5 muscles: Tibialis Anterior, Rectus Femoris, Biceps brachialis, Flexores of Forearm and Gastrocnemius medialis. The statistical Bland Altman analysis showed no significant differences between the measurements performed by two operators with a negligible bias for both echogenicity’s and thickness’ measurements of all evaluated muscles. These results suggest that our muscular ultrasound (MUS) study protocol, performed after a preliminary training and test standardization, is a reliable technique useful to collect paediatric normative data.

The role of ElectroEncephaloGraphy (EEG) and Somatosensory-Evoked-Potentials (SEP) in Neonatal as well as Adult-ICU is validated. To evaluate brain-functions in newborns with high risk of encephalic damage we also proposed an Integrated Multiparametric-Neurophysiological-Assessment (IMNA) simultaneously recording VEEG, CFM, Density-Spectral-Analisys and SEPs. We describe the case of a term-newborn (Apgar- Index 6–9, pH 7,03) in which an 1-h-IMNA was performed at 24 h from birth, to best define the encephalic damage. Results:An early encephalic ultrasound study showed only poor evidences. During IMNA baby incidentally presented short episodes of tonic–clonic motions of left arm. These clinical signs were associated with short right emispheric EEG hypovoltages while the cascade-SEPs monitoring showed a sudden transient change in voltage: right giant SEPs traces simultaneous to seizures. After phenobarbital administration seizures stopped and the right SEPs traces return to normality. An early encephalic MRI showed a right parietal ischemic lesion and also a right extracerebral hemorrhagic injury. The latest one appeared reduced in a further MRI. No further seizures were reported. 1 h-IMNA monitoring, especially cascade-SEPs, is a useful tool to perform an early evaluation and assess prognosis of neonatal hypoxic brain damage; it also allows to correlate neurophysiological parameters? trends to clinical events.

ElectroEncephaloGraphy and Somatosensory Evoked Potentials play an important role in diagnosis/prognosis of brain damage validated by international consensus to Adult-ICU monitoring. Video-EEG and Cerebral-Function-Monitoring are widely used in Neonatal-ICU while SEPs are still underused to early hypoxic brain damage evaluation. We propose an Integrated Multiparametric Neurophysiological Assessment (IMNA) simultaneously recording VEEG, CFM, Density-Spectral-Analysis and SEPs, to evaluate neonatal brain functions. Twelve healthy fullterm-neonates and 32 hypoxic newborns (2 severe, 30 moderate) were studied, within 48 h after birth, with IMNA short monitoring (1 h): VEEG (8 channels), CFM–DSA (4 channels) and cascade-SEPs (Cc-Fz; Cc-Ci; Cv7-Fz) with alternate stimulus (1 Hz) from median nerve. 1 h-IMNA-monitoring was globally alterate in severe-hypoxic neonates, in moderate-hypoxia the VEEG–CFM–DSA were fundamentally normal while cascade-SEPs showed a morphological variability. IMNA was normal and in particular cascade-SEPs was present with behavioral-states related modulation in all healthy fullterm-neonates. 1 h-IMNA-monitoring was able to evaluate a sleep-cycle (VEEG), cortical amplitude and frequency spectral activity (CFM–DSA); the cascade-SEPs were early reliable parameters, suggestive in brain neonatal damage, also moderate. 1 h-IMNA monitoring, especially cascade-SEPs, is a useful tool to early evaluation of various severity neonatal hypoxic brain damage and could be able to identify different outcomes.

Pediatric neuromuscular diseases, clinically heterogeneous, are a diagnostic challenge, and can require invasive and expensive investigations. Two clinical cases studied with integrated electromyography (EMG) and Neuro-Muscular UltraSound (NMUS) are reported: Case 1: A 5 years-old female developing a left equinus-foot within the last year, without any trauma or infection. NMUS-examination shows selective Soleus muscle involvement with increased echogenicity and alterated architecture. The EMG-study is negative for neuropathic/myopathic disorders; the biopsy finds a Soleus’s vascular malformation. Case 2: A 12 years-old male, agonistic karateka, with pes-cavus and right lower limb’s hypometria noted in the last year following an increase in height. NMUS-study shows bilateral involvement of lower limb’s posterior loggias, mainly right, with echogenicity homogeneously increased and preserved architecture. The EMG shows a distal asymmetric polyneuropathy, predominant in the Tibial nerve. Genetic and immunological investigations are in progress. In both clinically similar cases, (paucisymptomatic, monolateral, late-onset presentation), integrated-NMUS-EMG study guided the diagnosis toward: a selective damage with muscle’s morphostructural alteration and negative EMG (case 1); a prevalent chronic damage with muscular structure preserved and EMG-detection of asymmetric neuropathy (case 2). The combined morpho-functional evaluation with integrated NMUS-EMG facilitates pediatric neuromuscular diagnosis, improving the spatial resolution with limited costs and invasiveness.

Neuromuscular ultrasound (NMUS) is a non-invasive, painless and inexpensive technique that quickly screen large areas of muscles but only few data are reported in literature about pediatric physiological muscular structural changes. We collected, by means of a NMUS Health-Technology-Assessment (HTA) study (approved by Local Ethic Committee), pediatric quantitative and qualitative normative data. By NMUS we have bilaterally studied Anterior-Tibial, Long-Toe-Extensor, Rectus-Vastus-Femoris, Forearm-Flexors, Biceps muscles, considering thickness, echogenicity, pennation in 100 healthy children (M49-F51; range 2–16, mean 10,44 years; shared in five-age-groups: (I) 2–5; (II) 6–8; (III) 9–11; (IV) 12–14; (V) 15–16 ys); in about half of them sural and/or ulnar/median perimeter and area were measured. The muscles of I–II were less echogenic than III–IV–V groups. The muscles’ thickness (MT) increased with age especially between IV and V groups. In groups I–II the females’ MT was smaller than the males’s one. MT, BMI, weight were in relationship. The nerves’ area increased from I to V group. During childhood the muscular echogenicity and thickness increased rapidly, the latter especially during the puberty. Also the nerves’ area increased with age. NMUS is a useful tool to know physiological structural changes that are crucial to correctly interpretate and guide electromyographic and neuroimaging evaluation in clinically heterogeneous pediatric neuromuscular diseases.

Standardise an Integrated Multiparametric Neurophysiological Assessment (IMNA) simultaneously recording Video-EEG (VEEG), Cascade-Somatosensory-Evocated-Potentials (CSEPs), Cerebral-Function-Monitoring (CFM) and Density-Spectral-Analysis (DSA); to a complete data’s temporal correlation, using the advantages of each recording. We performed 1 h-short-monitoring-IMNA in 98 newborns (71 fullterms-27 preterms) in Neonatal-ICU; respecting SEPs-topographic derivation cerebral potentials were recorded from front-central-temporal-occipital areas, including the cervical and usual polygraphic ones. We used EBneuro monitoring-device (NeMus2-acquisition-stimulation-hardware, ICU.NET-software), disposable surface-recording electrodes (Ambu-Neuroline), surface-small-size cup (3 mm) AgCl-electrodes (BIONEN), abrasive-gel and conductive-cream (TEN20). The acquisition-panel is composed of modular-windows in demand: (1) VEEG-recording (8bipolar-channels Fp2-C4;C4-O2;Fp2-T4;T4-O2;Fp1-C3;C3-O1;Fp1-T3;T3-O1); parameters (0.3 s;70 Hz;10 μV/mm); impedences (2)-(3) 4bipolars-channels-CFM (scale 20–120 μV)-DSA (visible stripe 0–32 Hz, color-map-interval 0–10 μV 2 ) extrapolated from the EEG-montage. (4) CSEPs (N1–N13 components) in sequence (Cc-Fz;Cc-Ci;Cv7-Fz) obtained by bilaterally alternated-1 Hz-stimulus from median nerve (200 ms;10–20 mA; 50 average), referred to the template-potential. (5) CSEP’s components in trends. 1 h-short-monitoring-IMNA was able to identify a neonate’s sleep-cycle and well tolerated from the newborns (95%), with truthful registrations. IMNA-monitoring was easily performed in Neonatal-ICU, obtaining well recognizable, reliable, on–off-line assessible data, in particular CSEP. This new assessment, using 2 electrodes (Fz-Cv7) and 2 stimulatories in addition respect of the traditional VEEG, is useful to obtain more instrumental data in a little extra-time, extending the neurological informations in Neonatal-ICU.