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An epileptic seizure is one of the causes of so-called "transient neurological events" (TNEs). The differential diagnosis of a TNE relies mainly on history and physical examination. Laboratory markers are less frequently useful. After diagnosing an epileptic seizure, a distinction must be made between an acute symptomatic and an unprovoked seizure, since they have different treatments and prognosis. History, physical examination and other examinations (laboratory and imaging) are paramount in this distinction. After the diagnosis of a first unprovoked seizure, an EEG should be requested which may aid in establishing the diagnosis, evaluating the recurrence risk or ascertaining the self-limited nature of the seizure. 3T-MRI with an epilepsy protocol can be considered when CT has not clarified the aetiology. The decision to treat should be discussed with the patient/relatives, taking into account the risk of recurrence, the clinical characteristics (aetiology, seizure type, age, job, epileptic seizure schedule, comorbidities and polymedication), probability of AED side effects, and stigmatization. Nowadays, the chosen regimen is usually monotherapy with a second-generation AED that better suits the patient's characteristics, comorbidities and concurrent medication. Counselling should include first aid, precipitating factors, sport and physical exercise in order to avoid possible driving restrictions, the need for therapy compliance, and risk of recurrence and SUDEP.

Two young males with refractory epilepsy of unknown aetiology were referred for vagus nerve stimulation (VNS). Sleep disturbances emerged following VNS parameter changes. In Patient 1, video-polysomnogram (PSG) disclosed snoring and catathrenia in non-REM sleep. Central apnoea also occurred, but more rarely. In Patient 2, video-PSG showed mixed apnoea with desaturation and episodes of stridor followed by a catathrenia-like sound. A drug-induced sleep endoscopy (DISE) revealed, during VNS OFF time, glossoptosis, "trap door" of the epiglottis, and paresis of the left side of the larynx and ipsilateral vocal cords. During ON time, there were periods of pharyngeal collapse, in which video-PSG revealed patterns suggestive of both obstructive and central sleep apnoea. All these sleep-related phenomena were coincident with VNS ON time. In the first patient, VNS parameter adjustment was sufficient to successfully reverse all the symptoms, whereas the other patient required concomitant treatment with continuous positive airway pressure. The data broaden our knowledge about sleep disorders related to VNS, in particular stridor and catathrenia. We suggest that central sleep apnoea may be associated with laryngeal occlusion. DISE may be considered in selected cases as a valuable clinical tool to evaluate, in a single session, the effectiveness of multiple VNS parameter changes on respiration and laryngeal side effects. [Published with video sequences].

De novo absence status is clinically characterized by a confusional syndrome and neurophysiologically by the presence of periodic spike/polyspike-and-wave discharges on EEG. The treatment should be started promptly, and fast recovery is usually seen. However, cognitive symptoms can be very difficult to detect, and no consensus exists on how cognitive improvement can be clinically monitored. We report a patient with absence status epilepticus, whose therapeutic response was monitored neurophysiologically with EEG and clinically with a cognitive test; the Montreal Cognitive Assessment (MoCA). Based on this case report, we describe the use of the MoCA for monitoring cognitive function in a patient with absence status epilepticus. MoCA was evaluated on three occasions, with a total score ranging from 9, before treatment, to 23, when an EEG with no epileptiform discharges was obtained. We suggest that MoCA may be a useful tool to monitor cognitive improvement in absence status epilepticus.

This study aimed to characterize, clinically and neurophysiologically, a series of patients with gelastic seizures (GS), including both adults and children. We retrospectively collected patients with GS from epilepsy clinics of five tertiary hospital centres within a single country. Patients were selected through relatives'/caregivers' descriptions, home video and/or video-EEG monitoring. GS were identified through ictal semiology. Thirty-five patients were enrolled; 62.9% had initial GS in infancy, 14.3% in adolescence and 22.8% at adult age. Twenty-six had abnormal MRI: eight presented with hypothalamic hamartoma (HH) and 16 non-HH lesions that included different structural aetiologies and genetic, metabolic and immune aetiologies. All patients with HH had their first GS in infancy or adolescence. For the remaining aetiologies, GS started in infancy in 59.3%, in adolescence in 11.1% and at adult age in 29.6%. Video-EEG data was available for analysis in 11 patients, including seven patients with a non-HH MRI lesion. The ictal onset topography on scalp video-EEG was usually concordant with the MRI lesion (in 6/7 patients) and the most frequent ictal onset was fronto-temporal. In two patients, both video-EEG and MRI suggested a parietal and occipital epileptogenic zone. Aetiologies and patterns of affected topography unrelated to HH are common in patients with GS, and all age groups may manifest with this type of ictal semiology. This ictal manifestation has no lateralizing value and, despite a clear preponderance for hypothalamic, frontal and temporal lobe origins, other brain areas, namely the parietal and occipital lobes, should be considered.

Subcortical band heterotopia is a neuronal migration disorder that may cause refractory epilepsy. In these patients, resective surgery has yielded inadequate results. Deep brain stimulation of the anterior nuclei of the thalamus has been used for the treatment of refractory epilepsy with good results. We describe the first two patients with subcortical band heterotopia who were submitted to deep brain stimulation of the anterior nuclei of the thalamus, with evaluation of seizure outcome after 12 and 18 months of follow-up. At these times, both showed a >50% decrease in seizure frequency and an increase in seizure freedom. Both patients had a depressive syndrome after surgery that responded fully to anti-depressive medication in one patient and partly in the other. In both, deep brain stimulation of the anterior nuclei of the thalamus was associated with good seizure outcome. This procedure can therefore be considered in the treatment of patients with subcortical band heterotopia and refractory epilepsy. Depression may be a transient adverse event of the surgery or stimulation, however, its aetiology is probably multifactorial.

Limbic encephalitis with LGI1 antibodies may cause drug-resistant temporal lobe epilepsy. We report a case of a young man with progressive drug-resistant focal epilepsy, hyperhidrosis, and memory impairment associated with a left mesial temporal lesion. Epilepsy surgery was performed with the provisional diagnosis of cortical dysplasia or tumour. A neuropathological study following amygdalohippocampectomy revealed limbic encephalitis and LGI1 antibodies were identified in the serum. Two and a half years after surgery, the patient remains seizure-free without medication, with normal memory and without hyperhidrosis. Although immunosuppression is the first-line therapy for autoimmune limbic encephalitis, this case suggests that, in selected cases, a lasting response can be achieved with surgery.

Aim: Although surgery for temporal lobe epilepsy (TLE) harbours a good prognosis, post-operative seizures may occur. Long-term, post-operative seizure follow-ups are rare but necessary to properly define outcome., Methods: Longitudinal, long-term, post-operative seizure follow-up in TLE patients with outcome analysed using Engel's classification. Three groups were considered according to the type of resection: isolated amygdalohippocampectomy (IAH), further divided into anterior and complete, AH plus focal neocortical resections (AH + FR) and focal neocortical resections (FR)., Results: Eighty-nine patients were enrolled (61 in the IAH group, 24 in the AH + FR group, and four in the FR group), with a mean follow-up time of 46.7 months. For the three groups together, 90.9% and 86.7% of the patients were in Engel class I for six months and five years, respectively. Kaplan-Meir analysis of the IAH and AH + FR groups showed that, while 82.2% of patients of the IAH group tended to remain in class I within 84 months after surgery, 86.7% of the AH + FR group tended to remain in class I within 12 months. Kaplan-Meier analysis of the IAH sub-groups showed that more patients (91.0%) with anterior resection tended to remain in class I, although for a longer period of time (36 months), compared to those with complete resection (84.0% of patients and 12 months, respectively). For the IFR group, only three patients were in Engel class I for long-term follow-up., Conclusions: High rates of seizure freedom were obtained and stably maintained for years. The reasons for better long-term prognosis of the anterior IAH group are so far unclear, the IFR group was too small to draw any conclusive data.

Gastrointestinal symptoms are considered manifestations of ictal phenomena. Eructations are only rarely described. The present case illustrates eructation as an ictal sign in a patient with symptomatic refractory epilepsy and a left temporal lesion submitted to video-EEG monitoring. The ictal EEG reveals a generalized attenuation of background activity followed by a lateralised right anterior temporal theta rhythmic activity. Eructations take place during the initial generalized ictal EEG changes. The rarity of the ictal eructation is emphasised, and the potential localizing and lateralizing value are discussed in the present case.

Singing is a rare ictal symptom of focal epilepsy. We report a case of a left-handed woman, with no musical education, experiencing refractory seizures consisting of the singing of a popular Portuguese folksong, with simultaneous, rhythmic hand clapping. A left mesial temporal sclerosis was successfully treated by selective amygdalohippocampectomy. As in most previously reported cases, the epileptogenic zone was located in the left hemisphere, although the ictal onset zone could not be lateralized on scalp EEG. This clinical case supports the evidence that cerebral music production seems to require both hemispheres, in accordance to the concept that brain specializations for music is associated with a dynamic, well-orchestrated interaction of music-specific neural networks. [Published with video sequences].