Ketogenic dietary therapies in epilepsy: recommendations of the Italian League against Epilepsy Dietary Therapy Study Group

A stepwise increase in the utilization of ketogenic dietary therapies for drug-resistant epilepsy has been observed in Italy in the last decade, although it is still considered often underused in many centers when compared to other countries. The Dietary Therapy Study Group of the Italian League against Epilepsy proposes practical recommendations to improve shared knowledge and facilitate the application of ketogenic dietary therapies, optimizing its efficacy and tolerability. The experts involved (11 child neuropsychiatrists, two adult neurologists, one psychologist, one pharmacologist, one pediatric endocrinologist, one representative of patients' associations, and three dietitians and clinical nutritionists) responded to a survey on current clinical practice issues and were asked to discuss controversial topics related to supplementation, long-term maintenance, transition, and a multidisciplinary approach to ketogenic dietary therapies. Practical indications for patient selection, diet initiation, management, side effects prevention, and follow-up are provided

Facial Expression of Emotion in Human Frontal and Temporal Lobe Epileptic Seizures

While there is evidence of an impaired perception of emotion in verbal and facial expression in epileptic patients with unilateral focal resection of frontal, temporal, or parieto-occipital cortex, there is up to now a lack of research on the encoding aspects of facial expressions during seizure. In this study the video recordings of 146 seizures of 20 patients with temporal lobe epilepsy (12 females and 8 males) and 9 patients with frontal lobe epilepsy (2 females and 7 males) were analyzed using the Facial Action Coding System (FACS). Seizures were recorded in a standard hospital setting. Each video was paired with an EEG recording in order to ascertain the relationship between the clinical manifestations and the ictal discharge. The hypothesis was that, during the seizure, in addition to well-established facial expressions such as the “blank stare” during a “petit mal” absence with impaired consciousness, and the grimaces (unilateral or bilateral jerking and tonic contractions of the facial musculature), the facial displays can show a coherent pattern that is comparable to the facial expressions of emotions as they appear in normal subjects. Coherent patterns of facial expressions of emotions during the ictal event can emerge as a result of the activation of selective inborn motor patterns. Further displays of emotional patterns during temporal lobe seizures are recognizable in the ictal laughing, (gelastic seizure), characterized by forced and unmotivated laughter; and the dacrystic seizure, characterized by forced and unmotivated crying. These date confirm a crucial role of the limbic system both in the recognition and expression of emotion

Ictal pattern of EEG and muscular activation in symptomatic infantile spasms: a videopolygraphic and computer analysis

PURPOSE: To investigate ictal muscular phenomena characterizing symptomatic infantile spasms (ISs) and their relation to ictal EEG. METHODS: Four children with severe encephalopathy, neurologic impairment, and refractory ISs related to different dysplastic lesions, underwent videopolygraphic recordings collecting surface electromyogram (EMG) activity from several cranial and limb muscles to evaluate the pattern of muscular recruitment, duration, and side-to-side asymmetry of ISs. Acquired data were stored for off-line analysis by a computerized polygraphic system. RESULTS: Spasms were characterized by a complex pattern of muscular activation. A constant or rostrocaudal propagation pattern was lacking in all patients. Intervals between the onset of EMG activity in different muscles in each spasm were very long: </=100-200 ms. Two patients, with hemispheric cortical dysplasia and agenesis of the corpus callosum, had asymmetric and asynchronous spasms in which the EMG onset of the muscles contralateral to the affected hemisphere constantly anticipated that of the ipsilateral ones. Backaveraging of EEG activity disclosed a high-amplitude EEG complex on the same side as the dysplastic lesion, preceding or succeeding the contralateral muscle activity onset. In the other two patients with diffuse cortical dysplasia, no EEG transient was related to EMG activity onset. CONCLUSIONS: Despite clinical similarity between spasms in the same patient, our data demonstrate the complexity and heterogeneity of these motor phenomena. Our findings cannot be explained simply in terms of cortical or reticular generators like other motor phenomena such as cortical myoclonus or startle reflex

Pathology-Based Approach to Seizure Outcome after Surgery for Pharmacoresistant Medial Temporal Lobe Epilepsy

BACKGROUNDS: Hippocampal sclerosis (HS) is the most common cause of drug-resistant medial temporal lobe epilepsy (MTLE). Structural abnormalities such as HS, granule cell pathology (GCP) and focal cortical dysplasia (FCD), have been histopathologically classified possibly allowing more accurate prognostic seizure and neuropsychological outcome assessment. We correlated seizure outcome with comprehensive temporal lobe pathological findings, identified according to the most recent classification systems of HS, GCP and FCD. METHODS: All the 83 patients submitted to anterior temporal lobectomy (ATL) for drug-resistant MTLE and with a proven diagnosis of HS between April 2001 and May 2014 were collected. Patients were divided in two main groups: 1) Isolated HS with/without GCP (HS +/- GCP) 2) HS associated with FCD with/without GCP (HS+FCD +/- GCP). Patients were followed up at least 1 year and seizure outcome was reported in accordance with Engel classification. RESULTS: Group I: HS +/- GCP: Statistical analysis confirmed a better outcome in HS + GCP patients than in HS-noGCP (p < 0,05). Moreover, a better outcome for the patients affected by GCP type I was observed (p < 0,05). Group II: HS+FCD +/- GCP: Patients with HS variant type I presented a better seizure outcome than the patients with HS type II (Engel class IA HS type I vs. type II: 69% vs. 40%). CONCLUSIONS: A pathology based approach to epilepsy surgery might improve the interpretation of the results, could predict which cases will enjoy a better seizure outcome and could help to the comprehension of the causes of failures

Epilepsy associated tumors: Review article

Long-term epilepsy associated tumors (LEAT) represent a well known cause of focal epilepsies. Glioneuronal tumors are the most frequent histological type consisting of a mixture of glial and neuronal elements and most commonly arising in the temporal lobe. Cortical dysplasia or other neuronal migration abnormalities often coexist. Epilepsy associated with LEAT is generally poorly controlled by antiepileptic drugs while, on the other hand, it is high responsive to surgical treatment. However the best management strategy of tumor-related focal epilepsies remains controversial representing a contemporary issues in epilepsy surgery. Temporo-mesial LEAT have a widespread epileptic network with complex epileptogenic mechanisms. By using an epilepsy surgery oriented strategy LEAT may have an excellent seizure outcome therefore surgical treatment should be offered early, irrespective of pharmacoresistance, avoiding both the consequences of uncontrolled seizures as well as the side effects of prolonged pharmacological therapy and the rare risk of malignant transformation