Focal non-motor seizures and subsequent focal motor seizures as the main clinical expression of GLUT-1 deficiency.

The seizure type most frequently described in GLUT1 deficiency is generalized (mainly absence). We report the case of a young boy who, as the main clinical manifestation presented with focal non-motor, and then focal motor seizures. At the age of 3 months episodes of face pallor/cyanosis and hypotonus lasting about 1 min, occurred. They were initially misdiagnosed as gastroesophageal reflux. These episodes disappeared spontaneously at 6 months of age. At 12 months, episodes similar to the previous ones reappeared. A few months later, a cluster of several episodes manifest as impaired responsiveness and vomiting occurred. The patient initially performed long-term video-EEG monitoring (LTVEM) however, no seizures were captured. During a second hospitalization for LTVEM, a focal to bilateral clonic seizure was recorded. Brain MRI was normal. Next Generation Sequencing (NGS) panel for genes associated with epilepsy showed a de novo mutation of SCL2A1 gene. The CSF showed glucose of 41 mg/dL, and the CSF/serum glucose ratio was equal to 0.46. The ketogenic diet was started with optimal efficacy in seizure control. Meal-sensitivity in childhood onset focal seizures may be associated with GLUT-1 deficiency syndrome that can be confirmed by biochemical analysis on blood and CSF following diagnostic genetic study.

Diagnostic and therapeutic pathway for pain in a Continuing Care setting: a survey at an Italian Continuing Care Service.

Background: Law no. 38 of 15 March 2010 ensures and governs the access to the palliative care and pain management network for patients who require it. The professional roles involved in the project have been identified by the law, with the specific experience and expertise in the field of palliative care and pain management, by allocating a meaningful role to general practitioners (GPs). For this reason, an important direct training plan has been drawn up that GPs can count on for dedicated refresher courses to increase and deepen their knowledge in this specific clinical field. If the role of the GPs in the pain management and palliative care network was well-defined by the law, we cannot say the same for the Continuing Care Physician (CCP), a role that only partially overlaps that of the GP. The study observed the response of a Continuing Care Service (CCS) to the demand for services from patients with pain-related problems. The role of the CCP is, therefore, outlined in the pain therapy care network by observing the services provided to patients experiencing pain that is understood as being a non-deferrable problem. Methods: A survey was conducted at the CCSs site in Aquila, AS-01 Abruzzo. For this reason, the attending physician records the data of patients who consult the CCSs for pain-related problems on an appropriate questionnaire. The survey period covered a total of 68 days (1 January - 8 March 2020). Results. One hundred sixty five sheets were completed; females were more represented than males (57.6% v 42.4%) and the 36-65 age group appears most greatly represented (47.9%). One of the most frequent reasons for consulting the service is "musculoskeletal pain" (58.2%), followed by abdominal pain (15.8%). In the majority of patients, pain lasted from days (53.9%), with an average of approximately 3 days (3.1± 2.9), or hours in 40% of cases, with an average of over 6 hours (6.54±3.1). 88.5% of patients defined the level of pain experienced as "severe" (NRS=7-10), and the intensity of the pain associated with its repetitiveness (80.3% vs 92.6%) as "severe", with a statistically significant difference in relation to nonrepetitiveness cases (p=0.02). 66.1% of patients said that they had taken analgesics independently, with nonsteroidal anti-inflammatory drugs (NSAIDs) the most frequently taken (53.5%). Patients who turned to the CCS received a pharmacological prescription in almost all cases. NSAIDs, specifically, were the most prescribed medicines (64.8%), followed by muscle relaxants (29.7%). Tramadol was the most represented among opioids, which was prescribed in 7.9% of cases. Just 6.1% of patients were entered into the regional pain management network. Conclusions: The results of the survey show that a large number of patients turn to the CCS to resolve painful symptoms of various natures. The study offers some food for thought concerning the role of CCPs and the importance of providing for their inclusion in the pain therapy clinical and training pathways provided for by Law 38/2010. This would ensure its more effective implementation and, therefore, better care for patients experiencing painful pathologies.

Diagnosis of pediatric anti-NMDAR encephalitis at the onset: A clinical challenge.

BACKGROUNDS: To investigate the clinical and instrumental features at the onset addressing to the diagnosis of anti-NMDAR encephalitis. METHODS: Twenty children (age: 15 months-17 years; 7 males, 13 females) with initial suspected diagnosis of autoimmune encephalitis, observed between January 2008 and March 2018, were included. The final diagnosis was anti-NMDAR encephalitis in 7 children, other/probable autoimmune encephalitis in 7 children, and primary psychosis in the remaining 6 children. RESULTS: At the clinical onset, anxiety disorder was the main symptom that helped in distinguishing the group of psychotic children from children with non-infectious encephalitis (P = 0.05 OR = 0.001), while epileptic seizures strongly predicted anti-NMDAR encephalitis (P = 0.04 OR = 28.6). At the onset, anti-NMDAR encephalitis could be distinguished from other/probable autoimmune encephalitis for the presence of sleep/wake rhythm alteration (P = 0.05 OR = 15). Among the symptoms occurring during the hospitalization, movement disorders (P = 0.031 OR = 12) were predictive of non-infectious encephalitis rather than primary psychosis. More specifically, the occurrence of language impairment (P = 0.03 OR = 33), epileptic seizures (P = 0.04 OR = 28.6) and catatonia (P = 0.03, OR = 33), were predictive of anti-NMDAR encephalitis. Also at this stage, anxiety disorder (P = 0.03 OR = 0.033) was predictive of primary psychosis. CONCLUSION: Our findings suggest that at the clinical onset epileptic seizures and sleep/wake rhythm alteration represent the main features addressing to the diagnosis of anti-NMDAR encephalitis rather than primary psychosis and other/probable autoimmune encephalitis, while anxiety disorder could be a solid predictor of primary psychosis.

Management of psychogenic non-epileptic seizures: a multidisciplinary approach.

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.

Experimental Determination of Irreversible Entropy Production in out-of-Equilibrium Mesoscopic Quantum Systems.

By making use of a recently proposed framework for the inference of thermodynamic irreversibility in bosonic quantum systems, we experimentally measure and characterize the entropy production rates in the nonequilibrium steady state of two different physical systems-a micromechanical resonator and a Bose-Einstein condensate-each coupled to a high finesse cavity and hence also subject to optical loss. Key features of our setups, such as the cooling of the mechanical resonator and signatures of a structural quantum phase transition in the condensate, are reflected in the entropy production rates. Our work demonstrates the possibility to explore irreversibility in driven mesoscopic quantum systems and paves the way to a systematic experimental assessment of entropy production beyond the microscopic limit.

Febrile infection-related epilepsy syndrome (FIRES) with super-refractory status epilepticus revealing autoimmune encephalitis due to GABAAR antibodies.

BACKGROUND: Febrile infection-related epilepsy syndrome (FIRES) has been described as an epileptic encephalopathy of unknown etiology affecting previously healthy children following febrile illness. Despite large investigations on autoimmune pathogenesis no membrane antibodies has been associated since now. CASE STUDY: We report a 13 years-old girl with negative history for neurological or autoimmune disease that developed at the sixth day of high fever a super-refractory status epilepticus. All investigations, including the most common antibodies related to immune-mediated encephalitis were negative. Seizures continued despite several therapeutic trials with anesthetics (midazolam, propofol) and antiepileptic agents as well as i.v. immunoglobulins but responded, at day 10 from the onset, to ketamine and high dose i.v. steroids. Due the high suspicion of autoimmune encephalitis we tested patient's CSF and plasma on mouse brain with positive response. We subsequently detected a high titre of GABAAR antibodies. After the resolution of the status epilepticus the patient achieved complete recovery of neurological functions. CONCLUSION: this is the first reported case of a FIRES-like condition due to autoimmune encephalitis mediated by GABAAR antibodies. Our case suggests that GABAAR antibodies should be investigated FIRES.

An algorithm for the reconstruction of high-energy neutrino-induced particle showers and its application to the ANTARES neutrino telescope.

A novel algorithm to reconstruct neutrino-induced particle showers within the ANTARES neutrino telescope is presented. The method achieves a median angular resolution of [Formula: see text] for shower energies below 100 TeV. Applying this algorithm to 6 years of data taken with the ANTARES detector, 8 events with reconstructed shower energies above 10 TeV are observed. This is consistent with the expectation of about 5 events from atmospheric backgrounds, but also compatible with diffuse astrophysical flux measurements by the IceCube collaboration, from which 2-4 additional events are expected. A [Formula: see text] C.L. upper limit on the diffuse astrophysical neutrino flux with a value per neutrino flavour of [Formula: see text] is set, applicable to the energy range from 23 TeV to 7.8 PeV, assuming an unbroken [Formula: see text] spectrum and neutrino flavour equipartition at Earth.

From intravenous to enteral ketogenic diet in PICU: A potential treatment strategy for refractory status epilepticus.

BACKGROUND: Ketogenic diet (KD) has been used to treat refractory status epilepticus (RSE). KD is a high-fat, restricted-carbohydrate regimen that may be administered with different fat to protein and carbohydrate ratios (3:1 and 4:1 fat to protein and carbohydrate ratios). Other ketogenic regimens have a lower fat and higher protein and carbohydrate ratio to improve taste and thus compliance to treatment. We describe a case of RSE treated with intravenous KD in the Pediatric Intensive Care Unit (PICU). CASE REPORT: An 8-year-old boy was referred to the PICU because of continuous tonic-clonic and myoclonic generalized seizures despite several antiepileptic treatments. After admission he was intubated and treated with intravenous thiopental followed by ketamine. Seizures continued with frequent myoclonic jerks localized on the face and upper arms. EEG showed seizure activity with spikes on rhythmic continuous waves. Thus we decided to begin KD. The concomitant ileus contraindicated KD by the enteral route and we therefore began IV KD. The ketogenic regimen consisted of conventional intravenous fat emulsion, plus dextrose and amino-acid hyperalimentation in a 2:1 then 3:1 fat to protein and carbohydrate ratio. Exclusive IV ketogenic treatment, well tolerated, was maintained for 3 days; peristalsis then reappeared so KD was continued by the enteral route at 3:1 ratio. Finally, after 8 days and no seizure improvement, KD was deemed unsuccessful and was discontinued. CONCLUSIONS: Our experience indicates that IV KD may be considered as a temporary "bridge" towards enteral KD in patients with partial or total intestinal failure who need to start KD. It allows a prompt initiation of KD, when indicated for the treatment of severe diseases such as RSE.

Inter-laboratory evaluation of the EUROFORGEN Global ancestry-informative SNP panel by massively parallel sequencing using the Ion PGM™.

The EUROFORGEN Global ancestry-informative SNP (AIM-SNPs) panel is a forensic multiplex of 128 markers designed to differentiate an individual's ancestry from amongst the five continental population groups of Africa, Europe, East Asia, Native America, and Oceania. A custom multiplex of AmpliSeq™ PCR primers was designed for the Global AIM-SNPs to perform massively parallel sequencing using the Ion PGM™ system. This study assessed individual SNP genotyping precision using the Ion PGM™, the forensic sensitivity of the multiplex using dilution series, degraded DNA plus simple mixtures, and the ancestry differentiation power of the final panel design, which required substitution of three original ancestry-informative SNPs with alternatives. Fourteen populations that had not been previously analyzed were genotyped using the custom multiplex and these studies allowed assessment of genotyping performance by comparison of data across five laboratories. Results indicate a low level of genotyping error can still occur from sequence misalignment caused by homopolymeric tracts close to the target SNP, despite careful scrutiny of candidate SNPs at the design stage. Such sequence misalignment required the exclusion of component SNP rs2080161 from the Global AIM-SNPs panel. However, the overall genotyping precision and sensitivity of this custom multiplex indicates the Ion PGM™ assay for the Global AIM-SNPs is highly suitable for forensic ancestry analysis with massively parallel sequencing.

Cross-neutralization of the coagulant activity of Crotalus durissus terrificus venom from the northeast of Argentina by bivalent bothropic antivenom

Cross-neutralization of Crotalus durissus terrificus venom coagulant activity was tested using bivalent horse antivenom against Bothrops alternatus and Bothrops diporus venoms. Our in vitro and in vivo experiments showed that bothropic antivenom neutralizes the thrombin-like activity of crotalic snake venom and this cross-reaction was demonstrated by immunoassays either with whole venom or a purified thrombin-like enzyme. These results suggest common antigenic properties and, consequently, similar molecular structure among venom thrombin-like enzymes. Besides, they provide information that could be further used in the development of new antivenom formulations.

The role of extracellular conditions during CaCo-2 cells growth: a preliminary study for numerical model validation.

OBJECTIVES: One important limitation in cell therapy protocols, and regenerative medicine (an innovative and promising strategy for different pathologies treatment), is the lack of knowledge about cells engraftment, proliferation and differentiation. In order to allow an efficient and successful cell transplant, it is necessary to predict the logistics, economic and timing issues during cellular injection. It has been reported that several parameters, such as cells number, temperature and extracellular pH (pH0) value can influence metabolic pathways and cellular growth. Numerical analysis and model can help to reduce and understand the effects of the above environmental conditions on cell survival. The aim of this paper is to develop the first step of cells transplantation in order to identify "in vitro", which parameters can be useful to develop and validate a numerical model, able to evaluate "in vivo" cells engraftment and proliferation. MATERIAL AND METHODS: We studied the variation of extracellular parameters--such as medium volume, buffer system, nutrient concentrations and temperature on human colon carcinoma cells (CaCo-2) "in vitro culture"--pursuing the goal of understanding in deeper details cellular processes such as growth, metabolic activity, survival and pH0. RESULTS: Results showed that CaCo-2 cells growth and mortality increase after two days in culture when cells were suspended in 3.5 ml volume to respect of 10 ml volume. Different temperature values influenced CaCo-2 cells growth and metabolic activity showing a direct relationship with the volume of the medium. CONCLUSIONS: Our results describe as CaCo-2 cell growth, metabolic activity, mortality and extracellular pH were influenced by extracellular parameters, enabling us to develop and validate a numerical model to be use to predict cells engraftment and proliferation.

Childhood refractory focal epilepsy following acute febrile encephalopathy.

BACKGROUND: We describe a group of previously normal children who developed severe focal epilepsy after an acute/sub-acute illness resembling encephalitis. METHODS: This is a retrospective study. An acute phase (encephalitis/encephalopathy period) and a chronic phase (chronic focal resistant epilepsy) were defined. RESULTS: Eight patients were enrolled. The median age at onset was 6.6 years (range 8 months-17.6 years). In the acute phase, fever was the first symptom in all cases and was associated with seizures and status epilepticus. All patients had focal seizures arising in both hemispheres. Seizure onset occurred in the frontal and temporal regions. EEGs showed slowing background activity associated with focal or diffuse slow waves with rare epileptiform abnormalities. Cerebrospinal fluid oligoclonal bands were observed in four out of six patients tested. MRI images showed bilateral peri-insular hyperintensity in four cases. Five patients received corticosteroids, and in four cases, they were given along with intravenous immunoglobulins. The median duration of the acute phase was 19 days (range 15-30 days). During the chronic phase, which followed the acute phase without interval, patients presented with drug-resistant focal seizures and neuropsychological deficits, which ranged from hyperactivity and attention deficits to short-term verbal memory deficit, pervasive developmental disorders, and language delay. CONCLUSION: Considering the clinical presentations, EEG findings, and the associated occurrence of non-specific immunological activations, a possible immune-mediated pathogenesis can be hypothesized, although firm conclusions cannot be drawn out.

Myocardial ischemia in cocaine-user with anomalous origin of the left circumflex coronary artery.

The prevalence of anomalous coronary artery is approximately 0.6-1.7% in patients who undergo coronary angiography. These anomalies may cause angina, acute myocardial infarction, syncope and sudden death. Cocaine abuse is now a major health hazard: more than 5 million of Americans are current users. Cocaine abuse can result in coronary artery vasocostriction and the association between cocaine abuse, myocardial ischemia and infarction in the absence of coronary artery disease has been reported. We report the case of a patient with a story of nasally inhaled cocaine abuse presented with exertional chest pain and a perfusion defect of the anterior and inferior-lateral walls of the left ventricle during myocardial perfusion SPECT. The anomalous origin of the left circumflex coronary artery from the righ sinus of Valsalva was detected during coronary angiography and confirmed by the multislice computed tomography (MSCT). Although the coronary angiography is the gold standard of cardiac imaging technique for the diagnosis of coronary artery disease the identification of anomalous coronary arteries is frequently difficult with conventional coronary angiography because of the lack of 3-dimensional (3D) information related to the course of the coronary arteries to the great vessels. The MSCT provides a high spatial resolution, which allows a successful identification of the congenital coronary artery anomalies. This case report provides further a supportive evidence for the role of MSCT in the detection of the coronary artery anomalies.

Elasticity/distensibility of the ascending aorta: basal conditions and simulated conditions from space flights.

INTRODUCTION: The hysto-morfological composition of the ascending aorta wall gives to the vessel its characteristic elasticity/distensibility, which is deteriorated due to both physiological (age) and pathological events (hypertension, diabetes, dyslipidemia). This contributes to reduce the wall elasticity and to occurrence of cardiovascular events. MATERIALS AND METHODS: Thirty young healthy subjects (20 males, 10 females, age <30 yr), were subjected to different postural conditions with and without Lower Body Negative Pressure (LBNP) with conventional procedures, to simulate the microgravity conditions in space flight. During this procedure the cardiovascular parameters and the aorta elasticity were assessed with ecocardiography. RESULTS: The observation of results and statistical comparison showed that despite different hemodynamic conditions and with significant variation of blood pressure related to posture, elasticity/distensibility did not change significantly. DISCUSSION: The elasticity/distensibility of arterial vessels is the result of two interdependent variables such as blood pressure and systolic and diastolic diameters. While blood pressure and heart rate vary physiologically in relation to posture, the compensation of the vessel diameters modifications maintains the aortic compliance invariate. Therefore, in young healthy people, despite the significant postural and the sudden pressure changes (equivalent to parietal stress) aortic compliance does not alter. This behavior might be related to the low rate of cardiovascular events that are present in healthy people aged under 30 yrs.

Non-epileptic paroxysmal manifestations during sleep in infancy and childhood.

Sleep-related paroxysmal disorders in infancy and childhood have recently been reviewed and classified by the American Sleep Disorders Association. Although in some patients diagnosis is easy to achieve, at times a video/EEG recording of the episodes is required because of the similarity to epileptic seizures. These disorders often represent a challenge for paediatricians as an accurate diagnosis is required. We report a review of the literature and a characterisation of our series of patients. Paroxysmal events during sleep are frequently misdiagnosed as psychogenic fits, epileptic seizures or physiologic events with evident disadvantages for children and families. The widespread use of neurophysiological techniques and better knowledge of the semeiology of epileptic seizures have led us to correctly define these events, which have sometimes been erroneously treated in the past.

Indications for surgical treatment of epilepsy in childhood: a clinical and neurophysiological approach.

Resistant epilepsy is defined as the persistence of unacceptable seizures, despite correct drug treatment, or control of seizures at the cost of excessive side effects. About 30% of partial seizures are resistant to treatment; many of them is potentially a candidate for surgical treatment. Selection of patients is based fundamentally on precise identification of the epileptogenic area and on the evaluation that possible removal of that area will not be followed by serious neurological or neuropsychological deficits, or by onset of seizures in another part of the brain. To this end, careful clinical, neurophysiological and functional studies are conducted. Compared with adults, video-electroencephalographic (EEG) study of seizures in childhood is more difficult because of the lack of patient cooperation and, therefore, the lack of every subjective element in the seizures. Furthermore, the criteria for defining drug resistance in childhood are still under discussion, as many epilepsies are age dependent and seizures stop with growth. Despite this, the age at time of surgery has been steadily decreasing, in the conviction that the persistence of intractable partial epilepsy is detrimental to cognitive development and can damage brain areas that are apparently healthy. The clinical and EEG criteria for epilepsies that are secondary to some specific disorders have been defined, e.g. hemimegalencephalia and focal cortical dysplasias often produce epilepsy with onset in the first days of life characterized by partial seizures and subintrant spasms, EEG record of focal or hemispheric burst suppression and drug resistance that can be defined within the first months of life. Prolonged video-EEG monitoring is always necessary to establish congruence between the area in which the seizure originates and the brain lesion evidenced at neuroimaging. Correct monitoring of seizures means close cooperation between neurologist, neurophysiology technician and the patient, if the child is at least 5-6 y old. Absence of cooperation also limits the use of in-depth recording. Neuropsychological assessment of the child can be of great help in defining the area damaged by the epileptogenic site as well as the state of the other brain areas. Close cooperation between neurologist, radiologist, neuropsychologist and brain surgeon is necessary for a case-by-case assessment of indication for surgical treatment.

Experience with immunomodulatory treatments in Rasmussen's encephalitis.

The authors investigated immunomodulatory treatments in 15 patients with Rasmussen encephalitis (RE) (14 with childhood and one with adolescent onset RE). Positive time-limited responses were obtained in 11 patients using variable combinations of corticosteroids, apheresis, and high-dose IV immunoglobulins. Although surgical exclusion of the affected hemisphere is the only treatment that halts disease progression, immunomodulation can be considered when early surgery is not feasible, in late-onset patients with slower disease progression, and in the few cases of bilateral disease.

Diagnostic imaging in 13 cases of Rasmussen's encephalitis: can early MRI suggest the diagnosis?

Rasmussen's encephalitis (RE) is a rare, progressive, chronic encephalitis characterised by drug-resistant epilepsy, progressive hemiparesis and mental impairment. It typically involves only one cerebral hemisphere, which becomes atrophic. We present neuroradiological findings in 13 children with RE. MRI was performed in all patients, fluorodeoxyglucose positron-emission tomography (PET) in three, Tc-99m hexamethylpropylenamine oxime single-photon emission computed tomography (SPECT) in two and proton MR spectroscopy ((1)HMRS) in two. MRI showed progression of the hemisphere atrophy, always prevalent in the region primarily involved (13 patients), spread of the abnormal signal in white matter (11) and cortex (10) and progression of atrophy of the head of the caudate nucleus (nine). Associated secondary changes were: atrophy of the contralateral cerebellar hemisphere (in four patients), the ipsilateral hippocampus (in five) and the brain stem (in five). The earliest CT and MRI abnormalities, seen between 1 day and 4 months after the first seizure (in 12 patients examined, nine of whom had MRI) in one cerebral hemisphere included: high signal on T2-weighted images in the cortex (seven patients) and white matter (nine), cortical atrophy usually involving the frontoinsular region, with mild or severe enlargement of the lateral ventricle (eight) and moderate atrophy of the head of the caudate nucleus (seven). Cortical swelling in the early stage of the disease was recognisable only in two patients. PET revealed hypometabolism, SPECT decreased perfusion, and (1)HMRS reduction of N-acetylaspartate in the affected hemisphere. PET and SPECT were usually performed in the late stages and did not provide specific findings. MRI thus demonstrates the progression of RE and may suggest the diagnosis in the early stages, often before the appearance of neurological deficits. Early diagnosis of RE may be crucial for selecting patients for aggressive medical therapy or major surgical interventions such as hemispherectomy.

Rasmussen's encephalitis: early characteristics allow diagnosis.

OBJECTIVE: To identify early manifestations of Rasmussen encephalitis (RE) that can prompt early and reasonably secure diagnosis, allowing medical or surgical therapies at an early stage when they may be more effective in slowing the disease. METHODS: The authors studied 12 patients with clinical and neuropathologic diagnosis of RE, followed from disease onset, assessing clinical history, imaging, and EEG and focusing on early characteristics. Anti-GluR3 antibody assays were also considered in 11 patients. RESULTS: By 4 months from first symptoms, all cases had 1) refractory focal seizures with a predominant motor component, 2) slow focal activity on EEG contralateral to the motor manifestations, and 3) focal contralateral white matter hyperintensity with insular cortical atrophy on neuroimaging. Less constant or later findings were epilepsia partialis continua, oligoclonal bands, and serum anti-GluR3 antibodies. CONCLUSIONS: The association of partial seizures with focal EEG and neuroimaging changes allows a tentative diagnosis of RE 4 to 6 months after first symptoms.