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Background The National Institutes of Health Stroke Scale (NIHSS) is the most widespread clinical scale used in patients presenting with acute stroke. The merits of the NIHSS include simplicity, quickness, and agreement between clinicians. The clinical evaluation on posterior circulation stroke remains still a limit of NIHSS. Methods We assessed the application of a new version of NIHSS, the e-NIHSS (expanded NIHSS), adding specific elements in existing items to explore signs/symptoms of a posterior circulation stroke. A total of 22 consecutive patients with suspected vertebrobasilar stroke were compared with 25 patients with anterior circulation stroke using NIHSS and e-NIHSS. Results We compared the NIHSS and e-NIHSS scores obtained by the 2 examiners, in patients with posterior circulation infarct (POCI), using the Wilcoxon test. Patients with POCI evaluated with e-NIHSS had an average of 2 points higher than patients evaluated with classical NIHSS. The difference was statistically significant ( P Conclusions The NIHSS is a practical scale model, with high reproducibility between trained, different examiners, focused on posterior circulation strokes, with the same total score and number of items of the existing NIHSS. The e-NHISS could improve the sensitivity of NIHSS in posterior circulation stroke and could have an impact on clinical trials, as well as on outcomes. Further studies are needed to investigate a larger number of patients and the correlation between the e-NIHSS score and neuroimaging findings.

Refractory status epilepticus (RSE) is a particular critical condition characterized by seizures that continue despite the use of first- and second-line therapies and by high mortality. To date, only one prospective study investigated clinical features and prognostic factors in RSE. In this study, we performed a one-year prospective survey to identify clinical features, outcomes, and variables associated with the development of RSE in the adolescent and adult population of Modena, northern Italy. We observed 83 episodes of SE in 83 patients. In 31% of the cases, third-line therapy (anesthetic drug) was needed. Among this group, 14% resolved and were classified as RSE, while, in 17%, seizures recurred at withdrawal of anesthetics and were classified as super-RSE. The development of RSE/super-RSE was associated with a stuporous/comatose state at presentation and with the absence of a previous history of epilepsy. Refractory status epilepticus/super-refractory status epilepticus showed a worse outcome compared with responsive SE: 54% versus 21% for 30-day mortality; 19% versus 56% for a return to baseline condition. This prospective study confirms stupor/coma at onset as a relevant clinical factor associated with SE refractoriness. We observed a rate of RSE comparable with previous reports, with high mortality and morbidity. Mortality in the observed RSE was higher than in previous studies; this result is probably related to the low rate of a previous epilepsy history in our population that reflects a high incidence of acute symptomatic etiologies, especially the inclusion of patients with postanoxic SE who have a bad prognosis per se. This article is part of a Special Issue entitled "Status Epilepticus".

Background During resting-state EEG–fMRI studies in epilepsy, patients’ spontaneous head–face movements occur frequently. We tested the usefulness of synchronous video recording to identify and model the fMRI changes associated with non-epileptic movements to improve sensitivity and specificity of fMRI maps related to interictal epileptiform discharges (IED). New methods Categorization of different facial/cranial movements during EEG–fMRI was obtained for 38 patients [with benign epilepsy with centro-temporal spikes (BECTS, n = 16); with idiopathic generalized epilepsy (IGE, n = 17); focal symptomatic/cryptogenic epilepsy (n = 5)]. We compared at single subject- and at group-level the IED-related fMRI maps obtained with and without additional regressors related to spontaneous movements. As secondary aim, we considered facial movements as events of interest to test the usefulness of video information to obtain fMRI maps of the following face movements: swallowing, mouth–tongue movements, and blinking. Results Video information substantially improved the identification and classification of the artifacts with respect to the EEG observation alone (mean gain of 28 events per exam). Comparison with existing method Inclusion of physiological activities as additional regressors in the GLM model demonstrated an increased Z-score and number of voxels of the global maxima and/or new BOLD clusters in around three quarters of the patients. Video-related fMRI maps for swallowing, mouth–tongue movements, and blinking were comparable to the ones obtained in previous task-based fMRI studies. Conclusions Video acquisition during EEG–fMRI is a useful source of information. Modeling physiological movements in EEG–fMRI studies for epilepsy will lead to more informative IED-related fMRI maps in different epileptic conditions.

Background Internal carotid artery occlusion (ICAO) is defined as “untouchable” by all specialists; no treatment is indicated because intervention risks (carotid endarterectomy (CEA) or endovascular treatment) are usually much more than benefits. 1 , 2 We report the case of a patient admitted to our hospital with an atherothrombotic ischemic stroke due to symptomatic acute ICAO, who developed a recurrent stroke with hemispheric hypoperfusion and was treated in the emergency department with ICAO revascularization after 60 days of occlusion finding. Case Description D.G., a 62-year-old man, came to our attention for a transient episode of left weakness and hypoesthesia. The electrocardiogram revealed a new diagnosis of atrial fibrillation. CT angiography showed right ICAO; computed tomography and magnetic resonance imaging studies with perfusion imaging revealed a severe hemispheric hypoperfusion. Full anticoagulation therapy was started, and antihypertensive therapy was reduced to help collateral circulation. Some weeks later, the patient was readmitted to the stroke unit for 2 episodes of left-hand weakness. Cerebral angiography confirmed right ICAO from the proximal tract to the siphon. After some days, the patient suffered a femoral hemorrhagic lesion, with active bleeding, and was treated with surgical intervention. On the following day, the patient presented with left hemiplegia with hemianesthesia (National Institutes of Health Stroke Scale score = 14). The patient was treated in the emergency department with a complex endovascular treatment with complete recanalization of ICAO by positioning 3 stents through the intravenous infusion of abciximab. After intensive rehabilitation, at the 3- and 6-month follow-up evaluations, the patient regained autonomy. Conclusion In literature, treatment of chronic ICAO is not indicated. Endovascular recanalization may be beneficial to patients with chronic cerebral hypoperfusion due to ICAO, when all conservative medical therapies have failed.

Introduction Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder due to heterozygous pathogenic variants in transforming growth factor beta (TGFβ) signaling-related genes. LDS types 1–6 are distinguished depending on the involved gene. LDS is characterized by multiple arterial aneurysms and dissections in addition to variable neurological and systemic manifestations. Patient 1: a 68-year-old man was admitted due to an aphasic transient ischemic attack (TIA). Brain CT-scan and CT angiography revealed a chronic and asymptomatic right vertebral artery dissection. Stroke diagnostic panel was unremarkable. His history showed mild stroke familiarity. At age of 49, he was treated for dissecting-aneurysm of the ascending aorta and started anticoagulation therapy. Seven years later, he underwent surgery for dissecting aneurysm involving aortic arch, descending-thoracic aorta, left subclavian artery, and both iliac arteries. Patient 2: a 47-year-old man presented a left hemiparesis due to right middle cerebral artery (MCA) and anterior cerebral artery (ACA) occlusion caused by right internal carotid artery (ICA) dissection after sport activity. Despite i.v. thrombolysis and mechanical thrombectomy, he developed malignant cerebral infarction and underwent decompressive hemicraniectomy. Digital subtraction angiography showed bilateral carotid and vertebral kinking, aneurysmatic dilatation on both common iliac arteries and proximal ectasia of the descending aorta. His father and his uncle died because of an ischemic stroke and a cerebral aneurysm rupture with a subarachnoid hemorrhage (SAH), respectively. Discussion in both cases, considering the family history and the multiple dissections and aneurysms, LDS molecular analysis was performed. In patient 1, the novel NM_005902.3 (SMAD3): c.840T > G; p.(Asn280Lys) likely pathogenic variant was identified, thus leading to a diagnosis of LDS type 3. In patient 2, the novel NM_004612.2 (TGFBR1): c.1225T > G; p.(Trp409Gly) likely pathogenic variant was found, allowing for a diagnosis of LDS type 1. Conclusion LDS is characterized by genetic and clinical variability. Our report suggests that this genetically-determined connective tissue disorder is probably underestimated, as it might firstly show up with cerebrovascular events, although mild systemic manifestations. These findings could lead to identify people at risk of severe vascular complications (i.e., through genetic consult on asymptomatic relatives), in order to perform adequate vascular assessments and follow-up to prevent complications such as stroke.

Benign childhood epilepsy with centrotemporal spikes (BECTS) has been investigated through EEG–fMRI with the aim of localizing the generators of the epileptic activity, revealing, in most cases, the activation of the sensory–motor cortex ipsilateral to the centrotemporal spikes (CTS). In this case report, we investigated the brain circuits hemodynamically involved by CTS recorded during wakefulness and sleep in one boy with CTS and a language disorder but without epilepsy. For this purpose, the patient underwent EEG–fMRI coregistration. During the “awake session”, fMRI analysis of right-sided CTS showed increments of BOLD signal in the bilateral sensory–motor cortex. During the “sleep session”, BOLD increments related to right-sided CTS were observed in a widespread bilateral cortical–subcortical network involving the thalamus, basal ganglia, sensory–motor cortex, perisylvian cortex, and cerebellum.In this patient, who fulfilled neither the diagnostic criteria for BECTS nor that for electrical status epilepticus in sleep (ESES), the transition from wakefulness to sleep was related to the involvement of a widespread cortical–subcortical network related to CTS. In particular, the involvement of a thalamic–perisylvian neural network similar to the one previously observed in patients with ESES suggests a common sleep-related network dysfunction even in cases with milder phenotypes without seizures. This finding, if confirmed in a larger cohort of patients, could have relevant therapeutic implication.

Patients with chronic medial temporal lobe epilepsy (MTLE) can be impaired in different tasks that evaluate emotional or social abilities. In particular, the recognition of facial emotions can be affected (Meletti S, Benuzzi F, Rubboli G, et al. Neurology 2003;60:426-31. Meletti S, Benuzzi F, Cantalupo G, Rubboli G, Tassinari CA, Nichelli P. Epilepsia 2009;50:1547-59). To better understand the nature of emotion recognition deficits in MTLE we investigated the decoding of basic emotions in the visual (facial expression) and auditory (emotional prosody) domains in 41 patients. Results showed deficits in the recognition of both facial and vocal expression of emotions, with a strong correlation between performances across the two tasks. No correlation between emotion recognition and measures of IQ, quality of life (QOLIE-31), and depression (Beck Depression Inventory) was significant, except for a weak correlation between prosody recognition and IQ. These data suggest that emotion recognition impairment in MTLE is not dependent on the sensory channel through which the emotional stimulus is transmitted. Moreover, these findings support the notion that emotional processing is at least partly independent of measures of cognitive intelligence.

We report the case of a 69-year-old man with a 7-month history of severe progressive supranuclear gaze palsy associated with mild cognitive decline and sleep disturbances, but not parkinsonism. After a period spent consulting a range of different specialists, the appearance of brachial myoclonus prompted his referral to a movement disorders specialist. Duodenum biopsy confirmed the suspicion of neuro-Whipple disease. Antibiotic therapy was started but the delay in the diagnosis proved fatal to this patient. This noteworthy case shows unusual neurological features of a rare but treatable disease, often misdiagnosed as progressive supranuclear palsy.

Lesion and neuroimaging studies have demonstrated that the mesial temporal lobe is crucial for recognizing emotions from facial expressions. In humans, bilateral amygdala damage is followed by impaired recognition of facial expressions of fear. To evaluate the influence of unilateral mesial temporal lobe damage we examined recognition of facial expressions and functional magnetic resonance (fMRI) brain activation associated with incidental processing of fearful faces in thirteen mesial temporal lobe epilepsy (MTLE) patients (eight with right MTLE, five with left MTLE). We also examined the effect of early versus later damage, comparing subjects with hippocampal-amygdalar sclerosis (MTS) and seizures occurring before five years of age to epilepsy patients with late onset seizures. Fourteen healthy volunteers participated as controls. Neuropsychological testing demonstrated that the ability of right MTLE patients to recognize fearful facial expressions is impaired. Patients with early onset of seizures were the most severely impaired. This deficit was associated with defective activation of a neural network involved in the processing of fearful expressions, which in controls and left MTLE included the left inferior frontal cortex and several occipito-temporal structures of both hemispheres.

The role of the Apolipoprotein E (APOE) alleles in syndromes associated with focal cerebral atrophy (fronto-temporal dementia, primary progressive aphasia, corticobasal degeneration) is still controversial. We studied the APOE allele distribution in 39 patients with clinically diagnosed syndromes associated with focal cerebral atrophy (FCA), in 50 patients with early-onset probable Alzheimer's disease (EOAD), and in 60 patients with late-onset probable AD (LOAD). The APOE genotype was determined from a blood sample, using polymerase chain reaction and restriction enzyme digestion. The APOE epsilon4 allele frequency was significantly higher in the EOAD (21.0%) and LOAD (33.3%) groups, but not in the FCA group (5.1%), as compared with controls. In our population, the epsilon2 allele frequency was significantly higher in patients with FCA (12.8%) than in controls (4.8%). These results show that the APOE epsilon4 allele is not a risk factor for syndromes associated with FCA. The potential role of the epsilon2 allele in these syndromes needs further investigation.

We report a patient who, after a left parieto-occipital lesion, showed alexia and selective dysgraphia for uppercase letters. He showed preserved oral spelling, associated with handwriting impairment in all written production; spontaneous writing, writing to dictation, real words, pseudowords, and single letters were affected. The great majority of errors were well-formed letter substitutions: most of them were located on the first position of each word, which the patient always wrote in uppercase (as he used to do before his illness). The patient also showed a complete inability to access the visual representation of letters. As demonstrated by a stroke segmentation analysis, letter substitutions followed a rule of graphomotor similarity. We propose that the patient's impairment was at the stage where selection of the specific graphomotor pattern for each letter is made and that the apparent selective disruption of capital case was due to a greater stroke similarity among letters belonging to the same case. We conclude that a visual format is necessary neither for spelling nor for handwriting.

This experiment investigates the kinematic characteristics of the reach to grasp movement of Parkinson's and Huntington's disease subjects under two different experimental conditions. In the first condition subjects were required to perform the movement at a normal speed, while in the second condition they were required to perform the movement as fast as possible. Results showed that the kinematic parameterization of movement in Parkinson's disease subjects did not differ from that of age-matched control subjects for both the normal and the fast condition. However, the performances of Huntington's disease subjects appeared to be different when compared to the other two groups. Differences were mainly related to Huntington's disease patients' inability to properly define the temporal features of the movements.

The presence of a developmental trend in the magnitude of perceptual repetition priming was assessed in two experiments using picture completion. Preschool children, 6-year-old children, and young adults showed the same amount of repetition priming when both a proportional measure of priming (Snodgrass, 1989) was used and spurious explicit memory influences were partialled out from the implicit task. This finding was contrasted with the age-associated improvement in explicit memory as measured by cued and free recall. These results highlight the relevance of controlling explicit memory influence on implicit memory tasks in determining the effect of a variable on implicit processes; moreover they provide further support for the distinction between implicit and explicit memory.

The aim of this paper is to discuss the issue of dissociations found in unilateral spatial neglect according to the modality of space exploration and the nature of the task. For this purpose we present a reanalysis of the data from a recent paper of Gentilini et al. (1989) comparing visual and blindfolded exploration of a computer keyboard and discuss the performance of a left-brain-damaged patient with right visuospatial neglect and left-sided neglect dyslexia. We conclude that unilateral spatial neglect cannot be interpreted as a disruption of a single attentional mechanism, but rather it reflects impaired attentional mechanisms at several levels of cognitive processing.

Background Several studies have reported cognitive impairment in the Parkinsonian (MSA-P) and cerebellar (MSA-C) variants of Multiple System Atrophy, particularly in executive functions. This is attributed to a dysfunction of the cognitive loop linking the prefrontal cortex to basal ganglia. Since a few studies have investigated attentional functions in MSA patients, we assessed cognitive dysfunction and different aspect of attention in patients with MSA-C and MSA-P. Methods Seventeen MSA patients underwent a cognitive assessment of verbal/visual memory, language, visuo-spatial abilities and executive functions. The patients and 32 matched controls were presented with an experimental attentional task designed on Posner’s original Attention Network Test. The task provided three cue conditions (no-cue, temporal-cue, spatial-cue), two target conditions (congruent/incongruent) and two cue validity manipulations (valid/invalid-cue). Comparing reaction times in different experimental conditions allowed assessing the efficiency of the three different attentional components: Alerting, Orienting and Executive Control. Results Patients with MSA-P were impaired in executive functions, verbal memory and visuo-spatial abilities; MSAC patients in space and object perception tasks. MSA patients performed significantly worse than controls in attentional tasks with MSA-C patients showing more errors and longer RTs in incongruent target conditions. Conclusions MSA patients show specific cognitive impairments. MSA-C patients, in particular, are impaired in the attentional tasks. The greater impairment of Executive Control in these patients may be attributed to the widespread degeneration of cerebellum and cerebello-ponto-thalamo fibres projecting to medial areas of prefrontal cortex.

Event-related functional magnetic resonance imaging was used to investigate the neural mechanisms underlying intertemporal preference for symmetric gains and losses in certain conditions, by asking subjects to choose between two gains or two losses available at different points in time. Our data suggest that a common system is activated when an immediate reward/punishment is available, irrespectively of the impulsive /reflective behaviour performed by the individual.

Heavy drinkers (more than 2 liters of wine per day), without clinical evidence of brain dysfunction, moderate drinkers (1 liter of wine per day) and control subjects (no more than 250 cc of wine per day) were given the Progressive Matrices test and two learning tests, one tapping verbal memory and the other spatial memory. Heavy drinkers' performance was significantly worse than that of the other groups on the Progressive Matrices test and the spatial learning test. With a more lenient method of analysis, the difference between heavy drinkers and controls on the verbal learning test and that between moderate drinkers and controls on the Progressive Matrices test were also significant. When the influence of intelligence on memory performance was partialled out by covarying memory scores for Progressive Matrices scores, all differences on the verbal learning test disappeared whereas the spatial learning test still disciminated heavy drinkers from mild drinkers and controls. The bearing of these findings on the continuum of impairment hypothesis and the right hemisphere vulnerability hypothesis is discussed.

204 Ss entered this research, subdivided in controls ( N = 44) and hemisphere damaged patients ( N = 160). A meaningful and a meaningless patterns version of the Poppelreuter-Ghent Test were employed and the answers were worked out in the frame of the Signal Detectability Theory. No hemispheric asymmetry in the recognition performance turned out, patients with visual field defects being the only impaired Ss. Response criterion differences were completely lacking.

The aim of this study was to compare the performance of young and elderly subjects at two different memory tasks (frequency judgement and word recognition) under two retrieval conditions (forced and unforced choice) in order to determine the source of any incidental age-related memory deficit. Fifty young subjects (aged from 15 to 35 yr) and 50 elderly subjects (aged from 60 to 85) participated in this study. The results showed a significant difference between age groups at word recognition independent of retrieval condition, while frequency judgement was found significantly impaired in elderly as compared with young subjects only when an unforced choice procedure was required. It is suggested that age related memory impairment is not dependent on the nature of the task, but rather to the amount of retrieval information which is given to the subjects.

A 44-year-old woman showed, following an episode of encephalitis, an impoverished knowledge of the meaning and attributes of words and their referents, in spite of intact command of grammatical-syntactic rules and preserved perceptual abilities. There was also a complete loss of the stock of notions she had acquired over her life and constituting the cultural background of a person, in contrast with normal memory for autobiographic events. The case is interpreted as an istance of semantic amnesia. MRI showed damage confined to the antero-medial part of the left temporal lobe.

The performance of unilateral brain-damaged patients on recognition of random shapes was investigated by means of two different experiments. In experiment I interitem similarity and number of response-choice alternatives were varied to evaluate their possible influence on the subjects' performance. In experiment II stimulus exposure time was varied, In both experiments left brain-damaged patients with visual field defect obtained lower scores than control subjects. Right brain-damaged patients with visual field defect obtained scores significantly lower than controls only in the brief exposure condition.

Summary Short-term memory was investigated in 30 control and 125 unilaterally braindamaged patients with a series of tests requiring the immediate reproduction of strings of items of increasing length. In three tests the items were auditorially presented digits or words: one test asked for oral repetition of digits, while the other two required the patient to point to written digits or to pictures. The fourth test aimed at measuring spatial span. On both the Digits Forward test and the two other verbal tests not requiring the use of speech, left brain-damaged patients were impaired in comparison to normals, while the right brain-damaged patients were not. Aphasics had a significantly shorter verbal span than non-aphasic patients with left hemisphere damage. Spatial span, on the other hand, was significantly affected by a lesion posteriorly located in either hemisphere, but not by aphasia. Two patients with an exceedingly poor verbal memory span were observed one suffering from anomic aphasia and the other from conduction aphasia. There were also two right hemisphere damaged patients who showed an extreme reduction of spatial span, which could not be accounted for by space perceptual disorders and contrasted with a normal performance on a spatial long-term memory test.