Frontal Functional Connectivity of Electrocorticographic Delta and Theta Rhythms during Action Execution Versus Action Observation in Humans

We have previously shown that in seven drug-resistant epilepsy patients, both reaching-grasping of objects and the mere observation of those actions did desynchronize subdural electrocorticographic (ECoG) alpha (8–13 Hz) and beta (14–30) rhythms as a sign of cortical activation in primary somatosensory-motor, lateral premotor and ventral prefrontal areas (Babiloni et al., 2016a). Furthermore, that desynchronization was greater during action execution than during its observation. In the present exploratory study, we reanalyzed those ECoG data to evaluate the proof-of-concept that lagged linear connectivity (LLC) between primary somatosensory-motor, lateral premotor and ventral prefrontal areas would be enhanced during the action execution compared to the mere observation due to a greater flow of visual and somatomotor information. Results showed that the delta-theta (<8 Hz) LLC between lateral premotor and ventral prefrontal areas was higher during action execution than during action observation. Furthermore, the phase of these delta-theta rhythms entrained the local event-related connectivity of alpha and beta rhythms. It was speculated the existence of a multi-oscillatory functional network between high-order frontal motor areas which should be more involved during the actual reaching-grasping of objects compared to its mere observation. Future studies in a larger population should cross-validate these preliminary results

Multiple spinal meningiomas after tamixifen therapy: a case report

We describe a rare case of multiple spinal meningiomas and evaluate the possible relationship with tamoxifen treatment. We observed a 74-year-old woman who showed a spastic paraparesis gradually developed in the last year. The patient underwent left mastectomy for a breast cancer ten years earlier and was treated with tamoxifen for four years after surgical intervention. Magnetic resonance imaging revealed three spinal meningiomas at C6-C7, D6-D7 and D9 levels. Taking in account the tumor-inducing properties of tamoxifen and the extreme rarity of multiple spinal meningiomas, we suggest that tamoxifen may be the cause play a role in the genesis of the spinal meningiomatosis in the observed patient. Therefore, we propose the long-term clinical and neurological surveillance of patients who assumed tamoxifen, even for a short time, in order to survey the possible appearance of secondary tumours

Drug-resistant temporal lobe epilepsy due to cavernous malformations.

OBJECT: Supratentorial cavernous angiomas may be associated with drug-resistant focal epilepsy. Surgical removal of the malformation may result in seizure control in a number of patients, although in most studies a long history and high frequency of attacks have been recognized as indicators of unfavorable seizure outcome. In the literature, there are no clear indications regarding the optimal diagnostic presurgical workup and the surgical strategy for this particular subgroup of patients with symptomatic epilepsy. In this paper the authors focus on the preoperative workup and the surgical management of the disease in eight consecutive patients undergoing surgery for drug-resistant temporal lobe epilepsy (TLE) due to cavernous malformations (CMs), and the relevant literature on this issue is also reviewed. METHODS: Preoperatively, all patients were assessed using a noninvasive protocol aimed at localizing the epileptogenic zone on the basis of anatomical, electrical, and clinical criteria. The presurgical assessment yielded an indication for lesionectomy in two cases, lesionectomy plus anteromesial temporal lobectomy in four cases, and lesionectomy plus extended temporal lobectomy in two cases. At follow-up examinations, seizure, neuropsychological, and psychiatric outcomes were all evaluated. Seven patients were categorized in Engel Class IA (seizure free), and one was categorized in Engel Class IB (occasional auras only). No adverse effects on neuropsychological or psychosocial functioning were observed. CONCLUSIONS: Epilepsy surgery can be performed with excellent results in patients with intractable TLE caused by CMs. Noninvasive presurgical evaluation of these patients may enable a tailored approach, providing complete seizure relief in most cases

Surgical treatment of drug resistant temporal lobe epilepsy due to hippocampal sclerosis: results in a consecutive series of 139 patients with long-term follow-up

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Severe and rapidly-progressive Lafora disease associated with NHLRC1 mutation: a case report.

Lafora disease (LD), also known as progressive myoclonic epilepsy-2 (EPM2), is a rare, fatal autosomal recessive disorder typically starting during adolescence in otherwise neurologically normal individuals. It is clinically characterized by insidious of progressive neurological features including seizures, action myoclonus, visual hallucination, ataxia and dementia. Mutations in the laforin (EPM2A) gene on chromosome 6q24 or in the malin gene (NHLRC1) on chromosome 6p22 are responsible of LD phenotype. Diagnostic workup includes genetic analysis as well as axillary skin biopsy with evidence of typical periodic acid-Schiff (PAS)-positive polyglucosan inclusion bodies (Lafora bodies) in the apocrine glands and/or in the eccrine duct. Usually, genotype-phenotype correlations do not reveal substantial differences between patients carrying EPM2A and NHLRC1 mutations, but a few specific NHLRC1 mutations appear to correlate with a late onset and slow progressing LD. We report a case of LD due to compound heterozygote NHLRC1 mutation in an adolescent presenting with severe and atypical electro-clinical features, mimicking an autoimmune encephalopathy, and a rapidly progressive clinical course

Localizing significance of temporal intermittent rhytmic delta activity (TIRDA) in drug-resistant focal epilepsy

Objective: Temporal intermittent rhythmic delta activity (TIRDA) is an EEG pattern characterized by sinusoidal trains of activity, ranging from 1 to 3.5 Hz, and well localized over the temporal regions. It is considered to be an indicator of temporal lobe epilepsy (TLE), but full agreement between different authors has still not been reached. The aim of this study was therefore to assess the role of TIRDA in localizing the epileptogenic zone, which was estimated using anatomo-electro-clinical correlations obtained from non-invasive pre-surgical investigations in a large group of patients affected by drug-resistant partial epilepsy. Methods: The occurrence of TIRDA was investigated using a prolonged Video-EEG recording of 129 patients affected by drug-resistant partial epilepsy that underwent a non-invasive pre-surgical protocol. Patients were divided into 3 groups: TLE only, extratemporal epilepsy, and multilobar epilepsy including temporal lobe. According to the epileptogenic zone identified using anatomo-clinical-radiological correlations, 3 different subgroups of TLE were identified. mesial, lateral, and mesio-lateral, Statistical analysis was performed in order to evaluate the relationship between TIRDA and the epileptogenic zone, and neuroradiological, neuropathological, EEG interictal and fetal findings. Results. The pattern of TIRDA was observed in 52 out of the 129 (40.3%) patients studied. Significant correlations were found between TIRDA and: (i) mesial and mesio-lateral TLE; (if) mesial temporal sclerosis; (iii) interictal epileptiform discharge localized over the anterior temporal regions; and (iv) 5-9 Hz temporal fetal discharge. Conclusions: Our research shows that TIRDA plays a role in localizing the epileptogenic zone, suggesting that this pattern might be considered as an EEG marker of an epileptogenesis that involves the mesial structures of the temporal lobe. However, further studies investigating the relationship between intracranial EEG monitoring and simultaneous scalp EEG recording are needed in order to confirm our findings and improve our understanding of the significance of TIRDA