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Objective Neurological symptoms of COVID-19 patients have been recently described. However, no comprehensive data have been reported on pre-existing neurological comorbidities and COVID-19. This study aims at evaluating the prevalence of neurological comorbidities, and their association with COVID-19 severity. Methods We evaluated all consecutive patients admitted to the Emergency Room (ER) of our hospital between the 3rd March and the 14th April 2020, and diagnosed with COVID-19. Data on neurological and non-neurological diseases were extracted, as well as data on demographic characteristics and on severity degree of COVID-19. The prevalence of neurological comorbidities was calculated, and multivariate binary logistic regression analyses were used to estimate the association between neurological diseases and COVID-19 severity. Results We included 344 patients. Neurological comorbidities accounted for 22.4% of cases, with cerebrovascular diseases and cognitive impairment being the most frequent. Neurological comorbidity resulted independently associated with severe COVID-19 (OR 2.305; p = 0.012), as well as male gender (p = 0.001), older age (p = 0.001), neoplastic diseases (p = 0.039), and arterial hypertension (p = 0.045). When neurological comorbidity was associated with non-neurological comorbidities, the OR for severe COVID-19 rose to 7.394 (p = 0.005). Neurological patients, in particular cerebrovascular and cognitively impaired ones, received more respiratory support indication. Conclusion Neurological comorbidities represent a significant determinant of COVID-19 severity, deserving a thorough evaluation since the earliest phases of infection. The vulnerability of patients affected by neurological diseases should suggest a greater attention in targeting this population for proactive viral screening.

We describe a 30-year-old woman with an infantile-onset epilepsy due to a left temporal gliotic area who developed a nonconvulsive status epilepticus (NCSE) during tiagabine (TGB) adjunctive therapy. The ictal EEG recording showed a de novo right temporal focus not previously evident. After the i.v. administration of 4mg lorazepam, the NCSE episode rapidly resolved and her usual left temporal EEG abnormalities reappeared. To our knowledge this is the first case of paradoxical seizure exacerbation associated with TGB therapy in which the clinical and EEG features are congruous with a new contralateral focus.

Prospective, observational study. To assess the spinal cord function intraoperatively in subjects during spine stabilization for spinal cord trauma, by recording muscular (m-MEPs) and epidural motor evoked potentials (e-MEPs, D wave) along with cortical and epidural somatosensory evoked potentials (e-SEPs) and predicting the outcome of spinal cord injury (SCI). Regional Trauma Center, Torino, Italy. Fifty-five patients were intraoperatively studied during posterior spine stabilization surgery for traumatic SCI. In all, 21 of these had complete SCI, 14 an incomplete SCI—6 of them with central cord syndrome and 1 with central cord plus Brown Sequard syndrome—and 20 patients were neurologically uncompromised. The neurophysiologic profile of the complete SCI was the absence of both m-MEPs and e-MEPs caudally to the lesion site, associated with a lack of cortical and e-SEPs cranially to the lesion site. None of these patients recovered motor function in the follow-up. A clearly detectable caudal D wave was associated with motor recovery even in deeply paraparetic patients. In one neurologically incomplete patient a reversible deterioration of m-MEPs and e-MEPs was observed during the compression-distraction manoeuvre. Intraoperative neurophysiological evaluation of SCI patients can provide information about spinal cord function that is not retrievable by other clinical means and can correctly predict neurological outcome. Intraoperative testing during early stabilization of the spine of deeply paraparetic SCI patients provides additional information about their neurological profile.

The prediction of neurologic outcome in comatose patients after cardiac arrest (CA) has been investigated and the findings were summarized in a practice parameter of the American Academy of Neurology (1). These guidelines did not include the EEG as a reliable outcome indicator but recent studies on continuous EEG recorded during TH have re-evaluated the role of EEG (2–4). These studies underline the utility of a simplified classification system of EEG patterns and point to which pattern attains reliable predictive value both for good and bad outcomes. These reports are of high clinical relevance because, until today, the possibility of identifying early prognostic indicators of good outcome in patients surviving to CA has not been investigated. At the best of our knowledge, only few multicentre prospective studies (4–6) analyzed the prognostic value of EEG and SEP recorded in the same patient after CA as single predictor but none analyzed the diagnostic accuracy of the association of these two tests. We present the study protocol of Italian Multicentre Study about prognostic value of EEG and SEP in comatose patients after CA (ProNeCA) proposed by study group of “Intraoperative and Intensive Care Clinical Neurophysiology” of the Italian Society of Clinical Neurophysiology.

We report here a study of peripheral type benzodiazepine receptors (pBZr) in mononuclear cells (MNC) from blood of patients with multiple sclerosis (MS) during periods of stable and active disease and from normal controls. Most active MS patients were retested in a longitudinal study, both during a treatment with high dose steroids and while medication free. Active MS produces a significant decrease of receptor density compared with the controls whereas remission of the disease shows no effect. Four weeks of steroid treatment restore binding density to normal levels, and two weeks of drug withdrawal result in a small, but significant increase in number of the binding sites compared with the control value. We suggest that the number of pBZr in blood MNC might change during the clinical course and steroid therapy of MS.

Prospective, observational study. Regional Trauma Center, Torino, Italy. Complex spinal surgery carries a significant risk of neurological damage. The aim of this study is to determine the reliability and applicability of multimodality motor-evoked potentials (MEPs) and somatosensory-evoked potentials (SEPs) monitoring during spine and spinal cord surgery in our institute. Recordings of MEPs to multipulse transcranial electrical stimulation (TES) and cortical SEPs were made on 52 patients during spine and spinal cord surgery under propofol/fentanyl anaesthesia, without neuromuscular blockade. Combined MEPs and SEPs monitoring was successful in 38/52 patients (73.1%), whereas only MEPs from at least one of the target muscles were obtained in 12 patients (23.1%); both MEPs and SEPs were absent in two (3.8%). Significant intraoperative-evoked potential changes occurred in one or both modalities in five (10%) patients. Transitory changes were noted in two patients, whereas three had persistent changes, associated with new deficits or a worsening of the pre-existing neurological disabilities. When no postoperative changes in MEP or MEP/SEP modalities occurred, it was predictive of the absence of new motor deficits in all cases. Intraoperative combined SEP and MEP monitoring is a safe, reliable and sensitive method to detect and reduce intraoperative injury to the spinal cord. Therefore, the authors suggest that a combination of SEP/MEP techniques could be used routinely during complex spine and/or spinal cord surgery.

Palatal myoclonus (PM) is characterized by rhythmic involuntary jerking movement of the soft palate and the pharyngopalatine arch, often involving the diaphragm and laryngeal muscles.1 The two distinct clinical entities, symptomatic PM and essential PM, arise from different pathophysiologic mechanisms.2 Symptomatic PM is usually associated with lesions located in the myoclonic triangle (the Guillain–Mollaret triangle). It consists of the red nucleus, inferior olive, contralateral cerebellar dentate nucleus, and the connecting pathways, namely the central tegmental tract, the inferior cerebellar peduncle, and superior cerebellar peduncle.3 Symptomatic PM may be isolated or more rarely associated with movements of the extremities such as tremor, choreoathetosis, and myoclonus.4,5 A 64-year-old man was referred for acute onset of motor impairment of the right arm. On admission neurologic examination revealed clumsiness of the right arm and right dysmetria on finger-to-nose test. On the fifth day the patient developed myoclonic movements …

A mutation in the sodium channel SCN1A was identified in a small Italian family with dominantly inherited generalized epilepsy with febrile seizures plus (GEFS+). The mutation, D1866Y, alters an evolutionarily conserved aspartate residue in the C-terminal cytoplasmic domain of the sodium channel alpha subunit. The mutation decreased modulation of the alpha subunit by beta1, which normally causes a negative shift in the voltage dependence of inactivation in oocytes. There was less of a shift with the mutant channel, resulting in a 10 mV difference between the wild-type and mutant channels in the presence of beta1. This shift increased the magnitude of the window current, which resulted in more persistent current during a voltage ramp. Computational analysis suggests that neurons expressing the mutant channels will fire an action potential with a shorter onset delay in response to a threshold current injection, and that they will fire multiple action potentials with a shorter interspike interval at a higher input stimulus. These results suggest a causal relationship between a positive shift in the voltage dependence of sodium channel inactivation and spontaneous seizure activity. Direct interaction between the cytoplasmic C-terminal domain of the wild-type alpha subunit with the beta1 or beta3 subunit was first demonstrated by yeast two-hybrid analysis. The SCN1A peptide K1846-R1886 is sufficient for beta subunit interaction. Coimmunoprecipitation from transfected mammalian cells confirmed the interaction between the C-terminal domains of the alpha and beta1 subunits. The D1866Y mutation weakens this interaction, demonstrating a novel molecular mechanism leading to seizure susceptibility.

"Generalized epilepsy with febrile seizures plus" (GEFS+) syndrome has been recently described. This term defines a heterogeneous group of generalized epilepsies observed in several members of large pedigree studies. The syndrome spectrum has been widened by including others forms of generalized epilepsy. We report two Italian families in which the fathers showed febrile seizure plus (FS+), and two sons had severe myoclonic epilepsy of infancy (SMEI). The clinical setting of each epileptic member of the family will be discussed, focusing on the relationship with the GEFS+ group, confirming its wide clinical spectrum. In fact, GEFS+ is different from most other epilepsy syndromes as it is defined not by a set of associated symptoms but by the genetic transmission of a predisposition to febrile convulsions and other seizures, with a variable expression in several members of the same pedigree, perhaps due to ionic channel dysfunction. SMEI could represent the most severe end of the spectrum.