Giuseppe Didato

Sudden unexpected death in epilepsy (SUDEP) and sleep

The risk of sudden unexpected death is considered to be notably higher in patients with epilepsy with respect to the general population. Sudden unexpected death in epilepsy (SUDEP) is probably caused by the peri-ictal concurrence of a number of different predisposing and precipitating factors. Among these, the presence of a seizure before the fatal event is the only feature that seems to be constantly present. Different mechanisms, namely cardiac arrhythmias, respiratory dysfunctions, dysregulation of systemic or cerebral circulation have been suggested as potential physiopathological mechanisms. Moreover, clinical data seem to suggest that SUDEP could occur preferentially during sleep. In order to assess a possible relationship between sleep and SUDEP, we have analyzed studies in which sufficient information about the circumstances of deaths was available. Our analysis confirms that the relationship between sleep and SUDEP is not given by chance as the percentage of possible sleep-related SUDEP is higher than 40% in the majority of studies. We will discuss the possible longstanding and precipitating mechanisms involved in the interaction between sleep and epilepsy likely to favour SUDEP occurrence. In this perspective, possible preventive measures will be hypothesized.

Insular‐opercular seizures manifesting with sleep‐related paroxysmal motor behaviors: A stereo‐EEG study

Purpose:  Sleep‐related complex motor seizures are a common feature of nocturnal frontal lobe epilepsy. Nevertheless, recent studies also suggest that sleep‐related hypermotor seizures can originate in the insula. The present study describes the electroclinical features of eight drug‐resistant epileptic patients with insular‐opercular seizures manifesting with nocturnal complex motor seizures.

Taylor's focal cortical dysplasia increases the risk of sleep-related epilepsy.

Purpose:  To analyze the topography of the epileptogenic zone (EZ) and the etiologic substrate as risk factors for sleep‐related focal epilepsy.

7T MRI features in control human hippocampus and hippocampal sclerosis: An ex vivo study with histologic correlations

Hippocampal sclerosis (HS) is the major structural brain lesion in patients with temporal lobe epilepsy (TLE). However, its internal anatomic structure remains difficult to recognize at 1.5 or 3 Tesla (T) magnetic resonance imaging (MRI), which allows neither identification of specific pathology patterns nor their proposed value to predict postsurgical outcome, cognitive impairment, or underlying etiologies. We aimed to identify specific HS subtypes in resected surgical TLE samples on 7T MRI by juxtaposition with corresponding histologic sections.

Type II focal cortical dysplasia: Ex vivo 7T magnetic resonance imaging abnormalities and histopathological comparisons

In the present report, the correlations between ex vivo high‐resolution imaging and specific histological and ultrastructural patterns in type II focal cortical dysplasia (FCD) have been studied to explain the differences in the magnetic resonance imaging (MRI) detection of dysplasia and to contribute to the presurgical imaging evaluation of this pathology.

Type II FCD: ex vivo 7 Tesla MRI abnormalities and histopathological comparisons

In the present report, the correlations between ex vivo high‐resolution imaging and specific histological and ultrastructural patterns in type II focal cortical dysplasia (FCD) have been studied to explain the differences in the magnetic resonance imaging (MRI) detection of dysplasia and to contribute to the presurgical imaging evaluation of this pathology.

Refractory epilepsy and encephalocele: Lesionectomy or tailored surgery?

We read with great interest the paper entitled ‘‘Tailored surgery for drug-resistant epilepsy due to temporal pole encephalocele and microdysgenesis’’ by Giulioni et al. The authors describe two cases of encephalocele associated with cortical microdysgenesis and assert the necessity to consider an extensive surgical resection in subjects with small encephaloceles, due to the possible concomitance of malformations of cortical development. We recently observed two patients that may represent two opposite situations of patients. The first subject is a woman who experienced a tonic– clonic seizure during sleep at age 14 years, and was started with carbamazepine (CBZ) up to 1200 mg/day. No seizures appeared for 8 years. At age 22, she started to experience brief, weekly complex partial seizures, described as loss of consciousness, gestural and bipedal automatisms, followed by confusional status. Seizures persisted despite treatment with numerous antiepileptic drugs in various combinations. At age 35, when she came to our observation, physical and neurologic examinations were normal. Video-EEG monitoring allowed recording a nocturnal seizure originating from wide area of the right frontal lobe. MRI showed a small encephalocele associated with a linear cortical hyperintensity in T2-weighted sequences, localized in right anterior frontal region (Fig. 1A). Neuropsychological examination showed impairment in visuospatial memory, constructive apraxia and difficulties in planning actions, consistent with a right frontotemporal impairment. At age 36, she underwent lesionectomy and resection of adjacent cortex (Fig. 1B). Histologic examination showed encephalocele and an associated type IB focal cortical dysplasia (FCD), extending beyond the resection margin. Surgery was uncomplicated, without any additional post-operative neurologic or neuropsychological deficits, but seizure frequency was not modified (Engel class IV) at 5 years of follow-up. Patient refused further surgical interventions. The second patient is a 20-year-old man who came to our observation at age 14 for rare seizures characterized by déjà-vu, dreaming state and sometimes oral automatisms. Treatment with carbamazepine resulted in seizure freedom for 14 months, then he started experiencing weekly complex partial seizures with cephalic aura, loss of consciousness, oral and gestural automatisms, dystonia and clonic movements of right upper limb, followed by confusion state. Adjunctive treatment with topiramate and, subsequently, lacosamide did not modify seizure frequency. Standard brain MRI was deemed normal. At age 20 he was evaluated for epilepsy surgery. Prolonged video-EEG monitoring allowed detecting three seizures originating from left temporal lobe. High-resolution CT scan (Fig. 1B) showed a small bony defect in left mesial portion of middle cranial fossa. MRI targeted at temporal pole (Fig. 1C) evidenced a tiny encephalocele

Long-term outcome after limited cortical resections in two cases of adult-onset Rasmussen encephalitis.

Rasmussen encephalitis (RE) is a progressive inflammatory disorder characterized by brain hemiatrophy, unilateral focal deficits, and drug‐refractory focal epilepsy. Epilepsia partialis continua (EPC) is a hallmark of the disease. Several immunomodulatory treatments may slow but not halt the disease progression. The treatment of choice still relies on surgical hemispheric disconnection, which is burdened by heavy neurologic morbidity. More limited cortical resections, although more tolerable, are usually considered to be, at best, only transiently effective in RE. Hemispheric disconnections may be not feasible when neurologic functions are preserved and the dominant hemisphere is affected. Adult patients with a milder RE course that preserves neurologic function for a long period are particularly at risk of developing severe deficits after surgery. In this study we present the histories of two patients with adult‐onset RE who have undergone selective cortical resections to control EPC, avoiding, at the same time, the severe postsurgical deficits that may be induced by hemispheric disconnective surgery. The good result obtained on EPC has been stable over a prolonged period; however, this result was not paralleled by the stop of neurologic progression in one of the two cases.

Epilepsy and NREM-parasomnia: A complex and reciprocal relationship

This section is a new feature of Sleep Medicine allowing readersto submit unique sleep related cases with video segments as a wayto enhance educational value in a manner unattainable throughpaper format. Please see our web site’s (http://ees.elsevier.com/sleep/) Guide for Authors for instructions. We hope this sectionwill be enriched by the contributions of our colleagues who wishto offer stimulating opportunities for discussion and new insightsinto the field of sleep.

Bitemporal epilepsy: A specific anatomo-electro-clinical phenotype in the temporal lobe epilepsy spectrum

PURPOSE Temporal lobe epilepsy (TLE) with bilateral ictal involvement (bitemporal epilepsy, BTLE) is an intriguing form of TLE whose characteristics need to be carefully identified as BTLE patients are not good surgical candidates. The purpose of this study was to define the anatomo-electro-clinical features differentiating BTLE from unilateral TLE (UTLE). METHODS Forty-eight BTLE patients underwent long-term video-EEG monitoring (VEEG) and experienced seizures with bilateral temporal lobe involvement. Their main electro-clinical (demographics, interictal and ictal EEG, ictal signs) and neuro-imaging [brain magnetic resonance imaging (MRI)] data were compared with those of a group of 38 UTLE patients. RESULTS In comparison with the UTLE patients, the BTLE cohort was significantly older at the time of epilepsy onset (p = 0.023), more frequently experienced bilateral asynchronous interictal epileptiform discharges during wakefulness (p = 0.001) and sleep (p < 0.001), bilateral upper limb dystonia (p = 0.005), and auditory auras (p = 0.027), and less frequently showed a recognisable initial ictal EEG pattern of focal flattening or low-voltage fast activity (p < 0.001), post-ictal memory of seizures (p = 0.001), staring (p < 0.001), head deviation (p = 0.004), oro-alimentary automatisms (p = 0.006), and positive brain MRI (p < 0.001). MRI revealed neoplastic lesions (p = 0.007) or alterations other than hippocampal sclerosis (p = 0.028) only in the UTLE patients. CONCLUSION The possibility of recognising BTLE patients during pre-surgical evaluation or being able to suspect bitemporal seizures before VEEG by identifying particular anatomo-electro-clinical patterns is diagnostically important for epileptologists and can help to prevent possible surgical failures.