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The International League against Epilepsy (ILAE) proposed a diagnostic scheme for psychogenic non-epileptic seizure (PNES). The debate on ethical aspects of the diagnostic procedures is ongoing, the treatment is not standardized and management might differ according to age group. The objective was to reach an expert and stakeholder consensus on PNES management. A board comprising adult and child neurologists, neuropsychologists, psychiatrists, pharmacologists, experts in forensic medicine and bioethics as well as patients’ representatives was formed. The board chose five main topics regarding PNES: diagnosis; ethical issues; psychiatric comorbidities; psychological treatment; and pharmacological treatment. After a systematic review of the literature, the board met in a consensus conference in Catanzaro (Italy). Further consultations using a model of Delphi panel were held. The global level of evidence for all topics was low. Even though most questions were formulated separately for children/adolescents and adults, no major age-related differences emerged. The board established that the approach to PNES diagnosis should comply with ILAE recommendations. Seizure induction was considered ethical, preferring the least invasive techniques. The board recommended looking carefully for mood disturbances, personality disorders and psychic trauma in persons with PNES and considering cognitive-behavioural therapy as a first-line psychological approach and pharmacological treatment to manage comorbid conditions, namely anxiety and depression. Psychogenic non-epileptic seizure management should be multidisciplinary. High-quality long-term studies are needed to standardize PNES management.

Introduction REM sleep behavior disorder (RBD) is a common non motor feature of Parkinson's Disease (PD) affecting about half the patients with this disease. Distinct structural brain tissue abnormalities have been reported in several regions modulating REM sleep of the patients with idiopathic RBD. At the present time, there are no conventional MRI studies investigating patients with PD associated with RBD. Methods Herein, we used voxel-based morphometry (VBM) to detect the neuroanatomical profile of PD patients with and without RBD. Optimized VBM was applied to the MRI brain images in 11 PD patients with RBD (PD-RBD), 11 PD patients without RBD (PD) and 18 age-and sex-matched controls. To corroborate VBM findings we used automated volumetric method (FreeSurfer) to quantify subcortical brain regions volumes. Patients and controls also underwent DAT-SPECT and cardiac MIBG scintigraphies. Results The VBM analysis showed markedly reduced gray matter volume in the right thalamus of PD-RBD patients in comparison with PD patients and controls. Automatic thalamic segmentation in PD-RBD patients showed a bilaterally reduced thalamic volume as compared with PD patients or controls. All PD patients (with and without RBD) showed a reduced tracer uptake on DAT-SPECT and cardiac MIBG scintigraphies as compared to controls. Conclusions Our findings suggest that the presence of RBD symptoms in PD patients is associated with a reduced thalamic volume suggesting a pathophysiologic role of the thalamus in the complex circuit causing RBD.

Objective: To identify clinical and imaging features at presentation that might predict long-term outcome in patients with mild mesial temporal lobe epilepsy (mMTLE), which is defined by at least 24 seizure-free months with or without antiepileptic medication. Methods: In the setting of a prospective, population-based, longitudinal cohort study, we followed up 101 patients, all with mMTLE at enrolment. By protocol, patients underwent clinical evaluation every 3–12 months. Independent t test, Mann-Whitney test, or χ 2 test was used for comparing 2 groups. The incidence rate of refractory MTLE (rMTLE) was expressed as number of cases every 100 person-years. Results: After a mean follow-up of 12.2 ± 3.7 years, 16 patients dropped out and 85/101 (mean age 46.5 ± 13.3 years) were available for the present analysis. Of these, 64/85 (75%) patients remained seizure-free and 21/85 (25%) became refractory (rMTLE), the latter corresponding to 2.0 cases per 100 persons per year. Patients with rMTLE showed a longer duration of epilepsy ( p p = 0.006), more frequent febrile convulsions ( p = 0.02), and hippocampal sclerosis (HS) at MRI ( p = 0.004) as compared to those with mMTLE. Conclusions: mMTLE is a syndrome representing the mildest form of the wide spectrum of MTLE. Earlier age at onset, history of febrile convulsions, longer duration of epilepsy, and the presence of HS on MRI predict a worse outcome.

Background and purpose To evaluate if an automatic magnetic resonance imaging (MRI) processing system may improve detection of hippocampal sclerosis (Hs) in patients with mesial temporal lobe epilepsy (MTLE). Methods Eighty consecutive patients with a diagnosis of MTLE and 20 age- and sex-matched controls were prospectively recruited and included in our study. The entire group had 3-T MRI visual assessment of Hs analysed by two blinded imaging epilepsy experts. Logistic regression was used to evaluate the performances of neuroradiologists and multimodal analysis. Results The multimodal automated tool gave no evidence of Hs in all 20 controls and classified the 80 MTLE patients as follows: normal MRI (54/80), left Hs (14/80), right Hs (11/80) and bilateral Hs (1/80). Of note, this multimodal automated tool was always concordant with the side of MTLE, as determined by a comprehensive electroclinical evaluation. In comparison with standard visual assessment, the multimodal automated tool resolved five ambiguous cases, being able to lateralize Hs in four patients and detecting one case of bilateral Hs. Moreover, comparing the performances of the three logistic regression models, the multimodal approach overcame performances obtained with a single image modality for both the hemispheres, reaching a global accuracy value of 0.97 for the right and 0.98 for the left hemisphere. Conclusions Multimodal quantitative automated MRI is a reliable and useful tool to depict and lateralize Hs in patients with MTLE, and may help to lateralize the side of MTLE especially in subtle and uncertain cases.

Background and purpose Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) is a distinct epileptic syndrome with a broad range of severity even amongst affected members of the same pedigree, and the level of pharmacoresistance may reach 30%, close to that seen in sporadic focal epilepsies. Methods To investigate this issue of phenotypic heterogeneity, we prospectively carried out a high-resolution 3-T magnetic resonance imaging (MRI) study in an ADNFLE family containing 10 affected members including one pharmacoresistant patient and carrying the V287L mutation of the CHRN beta2 subunit (CHRNB2). MRI studies were evaluated in a manner blinded to the electro-clinical data. Results The brain MRI showed normal results in all affected individuals except the 22-year-old right-handed woman (member III-7) who had refractory seizures and typical radiological signs of left hippocampal sclerosis. She also had a simple febrile seizure at the age of 10 months. Conclusion The results of this study illustrate that hippocampal sclerosis has offered a fertile substrate for intractable ADNFLE to develop. The present findings also highlight the importance of acquired factors that are directly relevant to the epilepsy phenotype and its severity even in monogenic epilepsies.

Background Partial epilepsy represents almost 70% of the epileptic syndrome. It is characterized by partial seizures that arise from the restricted area of the cerebral cortex. Temporal lobe epilepsy (TLE) is the most frequent form of cryptogenic partial epilepsies [1]. The mesial temporal sclerosis is the pathologic abnormality most frequently detected in post mortem studies of subjects with TLE. Voxel-based morphometry (VBM) is a new MRI tool that has been developed to assess differences of brain tissue volumes between subject groups and has been applied in various studies of patients with TLE [2]. In refractory TLE (rTLE) gray (GM) and white (WM) matter abnormalities are not confined to the hippocampus but also are found in extrahippocampal structures [3]. Less is known about mild TLE (mTLE). In the present work, we used optimized voxel-based morphometry (VBM) to identify GM abnormalities beyond the hippocampus in rTLE and in mTLE with evidence of hippocampal sclerosis (HS). Materials and methods Brain MRI and VBM of GM with modulation was performed on 30 unrelated patients with mTLE (56% women; mean age 35.6 + 15.2 years), 19 patients with rTLE (52% women; mean age 38.4 + 17.4 years) and 37 healthy controls (25 women, mean age 37.3 + 10.6 years). MRI diagnosis of MTS was based on the atrophy of the hippocampal formation and/or mesial temporal hyperintensity on FLAIR or T2 images, or both. Results