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The Boston Naming Test (BNT) is a neuropsychological tool that is widely utilized in clinical practice to detect naming difficulties (i.e., anomia) in patients with language disorders and to evalua...

Background and aim In the scientific literature, there is unanimous consensus that hospitalization in stroke unit (SU) is the most important treatment for stroke patients. In this regard, the Act number 70/2015 by the Italian government identified specific skills that contribute to a classification of SU and outlined a “hub and spoke” stroke network. The aim of our study was to check the coverage of requirements of first and second level SU in the national territory and to shed light on any deficit or misdistribution of resources. Material and methods In 2019, a survey on the current situation related to stroke care in Italy was carried out by the Italian Society of Neurology (SIN), The Italian Stroke Organization (ISO), and the Association for the Fight against Stroke (A.L.I.Ce). Results First level SU was found to be 58 against a requirement, according to the Act 70/2015, of 240. Second level SU was found to be 52 compared with an expected requirement of 60. Neurointerventionists were 280 nationally, with a requirement of 240. A misdistribution of resources within individual regions was often seen. Conclusions The survey demonstrated a severe shortage of beds dedicated to cerebrovascular diseases, mainly because of lack of first level SU, especially in central and southern Italy. It also suggests that the current hub and spoke system is not yet fully implemented across the country and that resources should be better distributed in order to ensure uniform and fair care for all stroke patients on the whole territory.

Objective:\ud It is not known whether patients with atrial fibrillation (AF) with ischemic stroke despite oral anticoagulant therapy are at increased risk for further recurrent strokes or how ongoing secondary prevention should be managed.\ud \ud Methods:\ud We conducted an individual patient data pooled analysis of 7 prospective cohort studies that recruited patients with AF and recent cerebral ischemia. We compared patients taking oral anticoagulants (vitamin K antagonists [VKA] or direct oral anticoagulants [DOAC]) prior to index event (OACprior ) with those without prior oral anticoagulation (OACnaive ). We further compared those who changed the type (ie, from VKA or DOAC, vice versa, or DOAC to DOAC) of anticoagulation (OACchanged ) with those who continued the same anticoagulation as secondary prevention (OACunchanged ). Time to recurrent acute ischemic stroke (AIS) was analyzed using multivariate competing risk Fine-Gray models to calculate hazard ratios (HRs) and 95% confidence intervals (CIs).\ud \ud Results:\ud We included 5,413 patients (median age = 78 years [interquartile range (IQR) = 71-84 years]; 5,136 [96.7%] had ischemic stroke as the index event, median National Institutes of Health Stroke Scale on admission = 6 [IQR = 2-12]). The median CHA2 DS2 -Vasc score (congestive heart failure, hypertension, age≥ 75 years, diabetes mellitus, stroke/transient ischemic attack, vascular disease, age 65-74 years, sex category) was 5 (IQR = 4-6) and was similar for OACprior (n = 1,195) and OACnaive (n = 4,119, p = 0.103). During 6,128 patient-years of follow-up, 289 patients had AIS (4.7% per year, 95% CI = 4.2-5.3%). OACprior was associated with an increased risk of AIS (HR = 1.6, 95% CI = 1.2-2.3, p = 0.005). OACchanged (n = 307) was not associated with decreased risk of AIS (HR = 1.2, 95% CI = 0.7-2.1, p = 0.415) compared with OACunchanged (n = 585).\ud \ud Interpretation:\ud Patients with AF who have an ischemic stroke despite previous oral anticoagulation are at a higher risk for recurrent ischemic stroke despite a CHA2 DS2 -Vasc score similar to those without prior oral anticoagulation. Better prevention strategies are needed for this high-risk patient group.

Recently, during the pandemic infection of the novel SARS-CoV-2, some cases of Guillan-Barré Syndrome (GBS) have been reported. The aim of this work is to report the natural history of patients with GBS, both COVID and not-COVID related, hospitalized in Liguria region, during lock down period, in order to assess clinical features of both groups and possible managements pitfalls due to pandemic emergency. Fifteen GBS patients were admitted to the Hospitals of Liguria, from February 15th to May 3rd 2020, six with SARS-CoV-2 infection and nine without infection. In COVID-19 related GBS five patients presented with classical GBS and one with variant. Two patients presented neurologic symptoms during or shortly after the viral syndrome, suggesting the pattern of a para-infectious profile. Multi-organ involvement, delay in the diagnosis, incomplete work up and start of therapy, were registered in 50% of cases with a GBS-Disability scale ≥4 at follow-up evaluation. In not-COVID-19 related GBS, main problem was diagnostic delay. In three patients the first neurological observation took place after a mean of 33,6 days. Moreover, five patients went to emergency room after an average of 30 days since the onset of neurological symptoms because of fear of contagion. In conclusion, not only SARS-CoV-2 infection can cause GBS, but it can also, due to effects of pandemic on the health organization, affect the outcome of patients with not COVID-19 related GBS., Highlights • Covid-19 and GBS. • Diagnostic and therapeutic delay. • Management pitfalls.

We present the neuropathological description of an autoptic case of fatal rebound of disease activity after fingolimod discontinuation in a multiple sclerosis patient. MRI prior to the fatal outcome showed several large tumefactive demyelinating lesions. These lesions were characterized by prominent astrocytic gliosis, with a remarkable preponderance of large hypertrophic reactive astrocytes showing intense expression of sphingosine-1-phosphate receptor 1. Prominent astrocytic gliosis was also diffusely observed in the normal-appearing white matter. Dysregulated sphingosine-1-phosphate signaling on astrocytes following fingolimod withdrawal might represent a possible contributing mechanism to disease rebound and might account for the unusual radiological and neuropathological features observed in the present case.

Background The monoclonal antibody natalizumab (NTZ) is a highly effective treatment for patients with multiple sclerosis (MS). However, this drug is associated with increased risk of developing Progressive Multifocal Leukoencephalopathy (PML), an opportunistic infection of central nervous system (CNS) caused by the John Cunningham polyomavirus (JCV). Objective To describe the 12-month clinical course of 39 patients with MS (28 women, 11 men) who developed NTZ-related PML after a mean exposure of 39 infusions. Methods An Italian independent collaborative repository initiative collected and analyzed socio-demographic, clinical, magnetic resonance imaging (MRI) data and number of JCV-DNA copies detected on cerebrospinal fluid (CSF) samples of patients diagnosed as affected by NTZ-related PML. The evolution of disability, measured by the Expanded Disability Status Scale, was assessed at NTZ start, at PML diagnosis and after 2, 6 and 12 months from PML diagnosis. The effect of clinical and paraclinical characteristics at PML diagnosis on the final outcome was also investigated. Results Ten patients (25.6%) were diagnosed before 24 NTZ infusions. In six cases (15.4%) the PML suspect was made on the basis of highly suggestive MRI findings in absence of any detectable change of clinical conditions (asymptomatic PML). In patients with symptomatic PML, the diagnosis was quicker for those who presented with cognitive symptoms (n = 12) rather than for those with other neurological pictures (n = 21) (p = 0.003). Three patients (7.7%) died during the 12-month observation period, resulting in a survival rate of 92.3%. Asymptomatic PML, more localized brain involvement and gadolinium-enhancement detected at MRI, as well as lower viral load were associated with a better disability outcome (p-values

[This corrects the article on p. e377 in vol. 4, PMID: 28804745.].

Transcranial direct current stimulation (tDCS) has been suggested to improve language function in patients with post-stroke aphasia. Most studies on aphasic patients, however, were conducted with a very limited follow-up period, if any. In this pilot, single-blind study on chronic post-stroke aphasic patients, we aimed to verify whether or not tDCS is able to extend its beneficial effects for a longer period of time (21 weeks after the end of stimulation). Three aphasic patients underwent anodal tDCS (A-tDCS, 20 min, 1.5 mA) and sham stimulation (S-tDCS) over the left frontal (perilesional) region, coupled with a simultaneous naming training (on-line tDCS). Ten consecutive sessions (five days per week for two weeks) were implemented. In the first five sessions we used a list of 40 figures, while in the subsequent five sessions we utilized a second set of 40 figures differing in word difficulty. At the end of the stimulation period we found a significant beneficial effect of A-tDCS (as compared to baseline and S-tDCS) in all our subjects, regardless of word difficulty, although with some inter-individual differences. In the follow-up period, the percentage of correct responses persisted significantly better until the 16th week, when an initial decline in naming performance was observed. Up to the 21st week, the number of correct responses, though no longer significant, was still above the baseline level. These results in a small group of aphasic patients suggest a long-term beneficial effect of on-line A-tDCS.

The aim of this observational study was to assess the efficacy of tetrahydrocannabinol-cannabidiol oromucosal spray (THC:CBD, Sativex) on spasticity using the stretch reflex in subjects with multiple sclerosis (MS). Numeric rating scale (NRS) for spasticity, modified Ashworth scale (MAS) and the stretch reflex were assessed before and during treatment in 57 MS patients with spasticity eligible for THC:CBD treatment. A significant reduction of stretch reflex amplitude, as well as significant reductions of NRS and MAS scores were observed. There was a low concordance between the three measures (stretch reflex, NRS and MAS), likely related to the different aspects of muscle hypertonia assessed. Stretch reflex responders were taking a significantly higher number of puffs, while no differences were found in the responders by the other scales, suggesting that higher dosage would add benefit if tolerated. The present study confirms the efficacy of cannabinoids in reducing spasticity in patients with MS, suggesting a higher sensitivity and specificity of the stretch reflex compared to other measures. As an objective and quantitative measure of spasticity, the stretch reflex is particularly useful to assess the effects of cannabinoids on spinal excitability and may have a role in future pharmacological studies.

A study of "primary" (VEPs) and "cognitive" (ERPs) visual evoked potentials was carried out in a group of non-demented Afro-American Parkinson's disease (PD) patients. Current studies suggest that differences exist in the clinical manifestations of PD in Caucasian and non-Caucasian populations. Two horizontal sinusoidal gratings differing in spatial frequency, i.e., 1 and 4 cycles per degree (cpd), were presented in an "odd-ball" paradigm to 17 patients with PD and 17 age-matched control subjects. While the 1 cpd stimulus, is not expected to reveal retinal dopaminergic deficency, but only visuocognitive deficits, the 4cpd may give direct information of both "retinal" and "cognitive" visual deficits. We measured the latencies and amplitudes of N70, P100 and P300 components, and derived the "normalized" measures of P300-N70 latency difference (Central Processing Time-CPT70), the P300-P100 latency difference (CPT100) and the P300 amplitude responses normalized to either N70 and P100 amplitude (Amplitude Ratios AR70 and AR100). Our results do show that cognitive electrophysiological deficits in younger PD patients exist in non-Caucasians, perhaps to an even greater degree than in Caucasians, and confirm that absolute and normalized ERP amplitude and latency abnormalities are a distinguishing feature of younger PD patients from controls. In particular P300 measures are abnormal for 1 cpd pattern. A negative correlation exists between P300 amplitude and the motor score. By comparing the results for 1 and 4cpd stimuli it can be concluded that "primary" and "cognitive" visual abnormalities are independently affected in PD, implying that visuo-cognitive abnormalities are not passively determined by retinal dopaminergic deficiency.

Neurosyphilis is still a significant medical problem in developing countries and syphilitic ocular manifestations are often not diagnosed due to the lack of typical characteristics. We describe the case of a 59-year-old homosexual man with a 1-month history of decreased vision acuity in his left eye who was diagnosed with neurosyphilis and received treatment with intravenous penicillin G (16 million units in divided daily doses), with great improvement of visual acuity and CSF examination findings. The interest of this case is not only represented by the unusually early ocular involvement, but also by the rapid evolution of the disease into the secondary stage in a man who had had one at-risk homosexual relationship only 3 months before the onset symptoms. We also support the view that the presence of ocular involvement in syphilitic patients is suggestive of involvement of the CNS and should be considered synonymous with neurosyphilis.

We describe two patients with Parkinson's disease (PD) who developed clinical criteria of pathological gambling addiction in the setting of increased dopamine replacement therapy (levodopa and dopamine agonist medications). The second patient showed also signs of dopamine dysregulation syndrome, with an addiction to dopaminergic medication. Neither patients responded to the standard therapy for gambling behavior, but dramatically improved after bilateral subthalamic nucleus (STN) deep brain stimulation (DBS) and early postoperative withdrawal of dopaminergic therapy. The possible therapeutic role of subthalamic nucleus deep brain stimulation (STN-DBS) on such a disabling behavior needs to be investigated prospectively.

Objectives : We investigated whether the transient pattern onset and offset visual evoked potential (VEP) can distinguish between patients with Parkinson's disease (PD) and normal subjects. Methods : Two horizontal sinusoidal gratings differing in spatial frequency, i.e. 1 and 4 cycles per degree, were presented to 17 patients with PD and 16 age-matched control subjects. We analyzed the responses in the time-domain and measured the latencies and amplitudes of N1 and P1 to the onset and the offset of the stimulus; we also derived the measures of offset N1 and P1 amplitude responses ‘normalized’ to onset N1 and P1 amplitude values, respectively (amplitude ratios). Results : Absolute and normalized offset P1 amplitude is a distinguishing feature of PD patients from controls. Offset P1 amplitude was significantly larger in PD patients than in controls, particularly to the lower spatial frequency stimulus ( P P Conclusions : We conclude that the pattern onset/offset VEP amplitude provides a simple measure to evaluate visual processing deficits in PD and could contribute to an understanding of the pathophysiology of these changes.

It has been reported that non-Caucasian populations often suffer from an atypical type of Parkinson's disease (PD) characterized by poor levodopa response, early cognitive impairment and autonomic dysfunction. We tested the effect of a well known antiparkinsonian compound, amantadine, in 23 Afro-American patients with PD in a time-limited (six months), open-label, clinical and electrophysiological (simultaneously recorded primary and cognitive visual evoked potentials) trial. Patients were given amantadine either as monotherapy (first group) or added to levodopa treatment (second group). Amantadine produced a significant (p < 0.05) shortening of the latency of the event related potential (P300) obtained in a visual discrimination paradigm, while the timing of primary visual evoked potentials was little or not at all affected. Amantadine also showed significant beneficial effects (p < 0.01) on the motor score of both groups as assessed by the Rated Parkinson's Disease Neurological Exam, including items related to autonomic dysfunction. These findings suggest that amantadine alone and as adjuvant to levodopa can significantly improve both the speed of visual cognitive processing and the clinical score in non caucasian patients with PD. For these populations amantadine can be thus considered a helpful therapeutical option.

OBJECTIVES: To investigate the time dynamics and phase relationship with the stimulus of the onset/offset visual evoked potentials (VEPs), P300 and gamma band oscillatory responses to visual (contrast) stimulation. Gamma band oscillatory activity mediates in sensory and cognitive operations, with a role in stimulus-related cortical synchronization, but is reportedly reduced in the time window of the P300 response. METHODS: Ten healthy volunteers were studied. VEPs and P300 were obtained in a stimulus condition combining standard contrast stimulation and a visual odd-ball paradigm. Visual stimuli were gratings with a sinusoidal luminance profile (9.0 degrees central retina; 1.3 cycles/degree; 70% contrast) that were presented monocularly in onset/offset mode, with vertical orientation (frequent stimulus; 80%) or with a 15 degrees rotation to the right (infrequent, target stimulus). The total signal activity (temporal spectral evolution), the activity phase-locked to the stimulus onset (rectified integrated average), and the 'locking index' (ratio of the activity phase-locked to the stimulus to the total signal activity) were computed over time and across frequencies on the signals recorded at occipital (visual responses) and central locations (P300). RESULTS: Oscillatory activity centered around approximately 20.0-35.0 Hz and phase-locked to the stimulus was recorded at occipital locations with time dynamics anticipating the conventional VEPs. Phase-locking was higher after frequent than in response to target stimuli and after the stimulus offset compared to onset, while the phase-locking of the VEP frequency components was higher after the stimulus onset. The low frequency components of the P300 recorded at Cz (below approximately 8.0-10.0 Hz) were almost totally phase-locked to the stimulus, while the gamma band activity at the P300 location did not vary over time in amplitude or phase-locking and was mostly non-locked to the target stimulus. CONCLUSIONS: These observations add to the evidence of a role of the gamma band oscillatory responses (centered at approximately 20.0-35.0 Hz) in visual information processing and suggest that the increment in gamma band activity during cognitive operations also depends on task characteristics, vigilance or selective attention, and brain functional state. The visual P300 appears to reflect low frequency synchronization mechanisms.

Hemifacial spasm, a life-long condition characterized by involuntary unilateral contractions of the facial muscles, is a disabling disorder often resulting in patient irritation and social embarassment. Its probable etiology is neurovascular compression of the facial nerve at its root exit zone. The current medical treatment consists of either baclofen or anticonvulsant drugs, with limitation due to side effects or low efficacy. In recent years botulinum toxin injection and microvascular decompression of the facial nerve have been shown to be highly successful. However, both procedures share some complications and require special techniques. We present 5 patients affected by hemifacial spasm who responded well to the novel anticonvulsant drug gabapentin. Gabapentin was administered at a dose ranging from 900 to 1,600 mg daily, with rapid and clear improvement of spasms and absence of any remarkable adverse effects. Our findings suggest that gabapentin may be an effective treatment for patients with hemifacial spasm with a very good ratio of therapeutic effects to side effects when compared with other drugs currently used.

Aphasia due to simple partial status epilepticus is rare. We report a case of prolonged mixed aphasia without clouding of consciousness in a patient with an old history (10 years) of multiple sclerosis (MS). We found a clear clinical-EEG correlation with a continuous epileptic pattern in the shape of periodic lateralized epileptiform discharges (PLEDs). Clinical, laboratory and neuroradiological data ruled out possible etiological conditions other than MS and suggested that the development of new subcortical demyelinating lesions might play a critical role in seizure production. These findings, besides the role of PLEDs as an intrinsic feature of the status epilepticus condition, are discussed in relation to the literature.

Background To investigate to what extent Alzheimer's Disease (AD) affects Resting State activity, the possible impairment of independent electrophysiological parameters was determined in Eye-open and Eye-closed Conditions. Specifically, Flash-Visual Evoked Potential (F-VEP) and quantitative EEG (q-EEG) were examined to establish whether abnormalities of the former were systematically associated with changes of the latter. Methods Concurrently recorded F-VEP and q-EEG were comparatively analysed under Eye-open and Eye-closed Conditions in 11 Controls and 19 AD patients presenting a normal Pattern-Visual Evoked Potential (P-VEP). Between Condition differences in latencies of P2 component were matched to variations in spectral components of q-EEG. Results P2 latency increased in 10 AD patients with Abnormal Latency (AD-AL) under Eye-closed Condition. In these patients reduction of alpha activity joined an increased delta power so that their spectral profile equated that recorded under Eye-open Condition. On the opposite, in Controls as well as in AD patients with Normal P2 Latency (AD-NL) spectral profiles recorded under Eye-open and Eye-closed Conditions significantly differed from each other. At the baseline, under Eye-open Condition, the spectra overlapped each other in the three Groups. Conclusion Under Eye-closed Condition AD patients may present a significant change in both F-VEP latency and EEG rhythm modulation. The presence of concurrent changes of independent parameters suggests that the neurodegenerative process can impair a control system active in Eye-closed Condition which the electrophysiological parameters depend upon. F-VEP can be viewed as a reliable marker of such impairment.