Biomedical subjects

Laura Tassi

Publications and source records attributed to Laura Tassi.

17 recordsLinked to original sources

PubMed2006

Relationship of epileptic discharges to arousal instability and periodic leg movements in a case of nocturnal frontal lobe epilepsy: a stereo-EEG study.

We describe the case of a patient with nocturnal frontal lobe epilepsy, presenting with periodic leg movements during sleep and complaining of excessive daytime sleepiness. With the support of intracerebral electroencephalogram recordings and the corroboration of the postoperative outcome, periodic leg movements during sleep and excessive daytime sleepiness appeared to be associated to enhanced arousal instability induced by by recurrent epileptic discharges not detectable on scalp electroencephalogram.

Adult↗

PubMed2006

NMDA receptor composition differs among anatomically diverse malformations of cortical development.

Altered excitatory synaptic activity is likely a key factor in the neuronal hyperexcitability of developmental cerebral malformations. Using a combined morphologic and molecular approach, we investigated the NMDA receptor and related protein composition in human epileptic patients affected by periventricular nodular heterotopia, subcortical band heterotopia, or focal cortical dysplasia. Our results indicate that expression levels of specific NMDA receptor subunits are altered in both cerebral heterotopia and cortical dysplasia. A selective increase in the NR2B subunit was present in all cortical dysplasia, whereas the expression level of NR2A and NR2B subunits was significantly downregulated in all patients with heterotopia. NR2B upregulation in cortical dysplasia was greater in the total homogenate than the postsynaptic membrane fraction, suggesting that mechanisms other than increased ionic influx through the postsynaptic membrane may sustain hyperexcitability in dysplastic neurons. In cerebral heterotopia, the NR2A and NR2B downregulation was accompanied by less evident reduction of the SAP97 and PSD-95 proteins of the MAGUK family, thus suggesting that NMDA impairment was associated with altered molecular structure of the postsynaptic membrane. Our results demonstrate that diverse human developmental malformations are associated with different alterations of the NMDA receptor, which may contribute to the genesis of epileptic phenomena.

Adolescent↗

PubMed2006

Periventricular nodular heterotopia: classification, epileptic history, and genesis of epileptic discharges.

PURPOSE: Periventricular nodular heterotopia (PNH) is among the most common malformations of cortical development, and affected patients are frequently characterized by focal drug-resistant epilepsy. Here we analyzed clinical, MRI, and electrophysiologic findings in 54 PNH patients to reevaluate the classification of PNH, relate the anatomic features to epileptic outcome, and ascertain the contribution of PNH nodules to the onset of epileptic discharges. METHODS: The patients were followed up for a prolonged period at the Epilepsy Center of our Institute. In all cases, we related MRI findings to clinical and epileptic outcome and analyzed interictal and ictal EEG abnormalities. In one patient, EEG and stereo-EEG (SEEG) recordings of seizures were compared. RESULTS: We included cases with periventricular nodules, also extending to white matter and cortex, provided that anatomic continuity was present between nodules and malformed cortex. Based on imaging and clinical data, patients were subdivided into five PNH groups: (a) bilateral and symmetrical; (b) bilateral single-noduled; (c) bilateral and asymmetrical; (d) unilateral; and (e) unilateral with extension to neocortex. The latter three groups were characterized by worse epileptic outcome. No differences in outcome were found between unilateral PNH patients regardless the presence of cortical involvement. Interictal as well as ictal EEG abnormalities were always related to PNH location. CONCLUSIONS: The distinctive clinical features and epileptic outcomes in each group of patients confirm the reliability of the proposed classification. Ictal EEG and SEEG recordings suggest that seizures are generated by abnormal anatomic circuitries including the heterotopic nodules and adjacent cortical areas.

Adolescent↗

PubMed2006

Architectural (Type IA) focal cortical dysplasia and parvalbumin immunostaining in temporal lobe epilepsy.

PURPOSE: We analyzed 26 surgically treated patients operated on for intractable epilepsy associated with type IA (architectural) cortical dysplasia, to investigate neuropathologic and immunocytochemical features, particularly of the gamma-aminobutyric acid (GABA)ergic system, and to compare the findings with those observed in normal cortex. METHODS: Routinely stained slides and serial sections immunostained for neurofilaments (SMI 311), microtubule-associated protein-2 (MAP-2), neuron-specific nuclear protein (NeuN), glial fibrillary acidic protein (GFAP), parvalbumin (PV), calbindin (CB), and calretinin (CR) were processed. Some sections were processed by using single-immunoperoxidase procedures; others were processed for double immunofluorescence labelling and observed by confocal microscopy. The density of inhibitory PV-immunoreactive interneurons was quantitatively assessed in all patients and control cases by using a two-dimensional cell-counting technique on PV immunostained sections. RESULTS: The density of PV-immunoreactive interneurons was significantly reduced in this group of patients, whereas CB- and CR- positivity appeared similar to those in normal cortex. In five cases, architectural abnormalities, in addition to those that defined type 1A dysplasia, were present and characterized by abnormal clusters of neurons and laminar cellular loss in superficial cortical laminate. CONCLUSIONS: The reduction of PV expression in type IA cortical dysplasia suggests an impairment of the GABAergic system as a possible mechanism for the epileptogenicity; in addition, PV immunoreactivity can be helpful in the neuropathologic characterization of this form of cortical dysplasia.

Adolescent↗

PubMed2005

Negative myoclonus induced by cortical electrical stimulation in epileptic patients.

Negative myoclonus (NM) is a motor disorder characterized by a sudden and abrupt interruption of muscular activity. The EMG correlate of NM is a brief (<500 ms) silent period (SP) not preceded by any enhancement of EMG activity (i.e. myoclonus). This study investigated the role of premotor cortex (PMC), primary motor cortex (MI), primary somatosensory area (SI) and supplementary motor area (SMA) in the pathophysiology of cortical NM by means of intracerebral low frequency (1 Hz) electrical stimulation. In three drug-resistant epileptic patients undergoing presurgical evaluation, we delivered single electric pulses (stimulus duration: 3 ms; stimulus intensity ranging from 0.4 to 3 mA) to PMC (2 patients), MI (1 patient), SI and SMA through stereo-EEG electrodes; surface EMG was collected from both deltoids. The results showed that (i) the stimulation of PMC or MI could evoke a motor evoked potential (MEP) either at rest or during contraction, in this latter case followed by an SP; however, in two patients, at the lowest stimulus intensities (0.4 mA), 50% of stimuli could induce a pure SP, i.e. not preceded by an MEP; raising the intensity of stimulation (0.6 mA), the SPs showed an antecedent MEP in >80% of stimuli; (ii) the stimulation of SI at low stimulus intensities (from 0.4 to 0.8 mA) induced in two patients only SPs, never associated with an antecedent MEP, whereas in the third subject the SPs could be inconstantly preceded by an MEP; by incrementing the stimulus intensity (up to 3 mA), in all three patients the SPs tended to be preceded, although not constantly, by an MEP; stimulus intensity affected SP duration (i.e. the higher the intensity, the longer the SP), without influencing the latency of onset of the SPs; (iii) the stimulation of SMA induced only pure SPs, at all stimulus intensities up to 3 mA; as for SI, increment of stimulus intensity was paralleled by an increase in SP duration, without influencing the onset latency of SPs. We conclude that single electric pulse stimulation of PMC, MI, SI and SMA through stereo-EEG electrodes can induce pure SPs, not preceded by an MEP, which clinically appear as NM, suggesting therefore that these cortical areas may be involved in the genesis of this motor phenomenon. However, it must be pointed out that SMA stimulation induced only pure SPs, regardless of the stimulus intensity, whereas occurrence of pure SPs following stimulation of PMC, MI, and SI depended mainly on the intensity of stimulation.

Adolescent↗

PubMed2005

The temporopolar cortex plays a pivotal role in temporal lobe seizures.

We investigated the role of the temporal pole (TP) in 48 consecutive patients with drug-refractory temporal lobe epilepsy (TLE). Chronic depth recordings of TP cortex activity were used in association with video recording of ictal symptoms during 48 spontaneous seizures. In 23 cases (48%, group 1) the TP was involved at the onset of the seizure, before or concurrently with the hippocampus. In the remaining 25 patients (52%, group 2) the TP was involved 16.4 +/- 13.8 s after the hippocampus. A past history of febrile seizures was found in both groups, with no statistical difference. Ictal symptoms did not differentiate TP seizures from seizures originating in the hippocampus but the first clinical sign occurred sooner in group 1 compared with group 2 (respectively 10.56 +/- 9 and 25.7 +/- 19 s, respectively, P = 0.005). Loss of awareness also occurred sooner in the case of TP seizures compared with mesiotemporal lobe (MTL) seizures (22.9 +/- 22.6 versus 42.2 +/- 18.6 s, P = 0.0002). MRI data analysis showed that hippocampal sclerosis was present in both groups of patients, although it was more frequent in patients with MTL onset. Anterior temporal white matter changes were found ipsilateral to the epileptogenic area and tended to be more frequent in patients with TP seizures. All the patients underwent tailored anterior temporal lobectomy that included the TP, the hippocampus, the parahippocampal gyrus and the anterior part of the lateral temporal cortex. A better postoperative outcome was achieved in group 1 compared with group 2 (Engel class 1, 95 and 72% respectively, P = 0.04). We conclude that the frequent TP involvement at the onset of seizures could be a supplementary explanation for some failures of selective amygdalohippocampectomy, which should be addressed preferentially to well-selected patients. Moreover, the involvement of the TP cortex at the onset of the seizures is a good predicting factor for postoperative seizure outcome.

Adolescent↗

PubMed2005

Increased discharge threshold after an interictal spike in human focal epilepsy.

It is commonly assumed that interictal spikes (ISs) in focal epilepsies set off a period of inhibition that transiently reduces tissue excitability. Post-spike inhibition was described in experimental models but was never demonstrated in the human epileptic cortex. In the present study post-spike excitability was retrospectively evaluated on intracerebral stereo-electroencephalographic recordings performed in the epileptogenic cortex of five patients suffering from drug-resistant focal epilepsy secondary to Taylor-type neocortical dysplasias. Patients typically presented with highly periodic interictal spiking activity at 2.33 +/- 0.87 Hz (mean +/- SD) in the dysplastic region. During the stereo-electroencephalographic procedure, low-frequency stimulation at 1 Hz was systematically performed for diagnostic purposes to identify the epileptogenic zone. The probability of evoking an IS during the interspike period in response to 1-Hz stimuli delivered close to the ictal-onset zone was examined. Stimuli that occurred early after a spontaneous IS (within 70% of the inter-IS period) had a very low probability of generating a further IS. On the contrary, stimuli delivered during the late inter-IS period had the highest probability of evoking a further IS. The generation of stimulus-evoked ISs is occluded for several hundred milliseconds after the occurrence of a preceding spike discharge. As previously shown in animal models, these findings suggest that, during focal, periodic interictal spiking, human neocortical excitability is phasically controlled by post-spike inhibition.

Adolescent↗

PubMed2005

Biting behavior, aggression, and seizures.

PURPOSE: To describe the semiologic features of aggressive behaviors observed in human epileptic seizures with particular reference to the act of biting a conspecific. METHODS: We analyzed the biting behavior (BB) and other aggressive gestures occurring in a group of 11 patients retrospectively selected from >1,000 patients subjected to video-EEG/SEEG monitoring for presurgical evaluation of drug-resistant seizures. RESULTS: Patients displaying BB showed (a) a male sex predominance, (b) heterogeneous etiologies and lesion locations, and (c) seizures involving the frontotemporal regions of both hemispheres. The act of biting was a rapid motor action, lasting approximately 600 ms, occurring in the context of strong emotional arousal, fear, and anger, with various bodily gestures with aggressive connotation. BB was mainly a "reflexive" behavior, in that biting acts were evoked (both during and after seizures) by actions of people in close contact with the patient. The sole intrusion of the examiner's hand in the space near the patient's face was effective in triggering BB. Rarely, self-directed or object-directed biting acts were not triggered by external stimuli. Intracranial data (SEEG) obtained in one subject showed that the amygdala/hippocampal region plus the orbitomedial prefrontal cortex had to be involved by ictal activity to observe BB. CONCLUSIONS: Anatomic and electrophysiologic data in our patients suggest that a model of dual--temporal and frontal--dysfunction could account for the occurrence of ictal/postictal BB. Behavioral data suggest also that BB and related aggressive gestures can be considered as the emergence of instinctive behaviors with an adaptative significance of defense of the peripersonal space.

Adult↗

PubMed2005

Stereoelectroencephalography in the presurgical evaluation of focal epilepsy: a retrospective analysis of 215 procedures.

OBJECTIVE: To report on indications, surgical technique, results, and morbidity of stereoelectroencephalography (SEEG) in the presurgical evaluation of patients with drug-resistant focal epilepsy. METHODS: Two-hundred fifteen stereotactic implantations of multilead intracerebral electrodes were performed in 211 patients (4 patients were explored twice), who showed variable patterns of localizing incoherence among electrical (interictal/ictal scalp electroencephalography), clinical (ictal semeiology), and anatomic (magnetic resonance imaging [MRI]) investigations. MRI scanning showed a lesion in 134 patients (63%; associated with mesial temporal sclerosis in 7) and no lesion in 77 patients (37%; with mesial temporal sclerosis in 14 patients). A total of 2666 electrodes (mean, 12.4 per patient) were implanted (unilaterally in 175 procedures and bilaterally in 40). For electrode targeting, stereotactic stereoscopic cerebral angiograms were used in all patients, coupled with a coregistered three-dimensional MRI scan in 108 patients. RESULTS: One hundred eighty-three patients (87%) were scheduled for resective surgery after SEEG recording, and 174 have undergone surgery thus far. Resections sites were temporal in 47 patients (27%), frontal in 55 patients (31.6%), parietal in 14 patients (8%), occipital in one patient (0.6%), rolandic in one patient (0.6%), and multilobar in 56 patients (32.2%). Outcome on seizures (Engel's classification) in 165 patients with a follow-up period of more than 12 months was: Class I, 56.4%; Class II, 15.1%; Class III, 10.9%; and Class IV, 17.6%. Outcome was significantly associated with the results of MRI scanning (P = 0.0001) and with completeness of lesion removal (P = 0.038). Morbidity related to electrode implantation occurred in 12 procedures (5.6%), with severe permanent deficits from intracerebral hemorrhage in 2 (1%) patients. CONCLUSION: SEEG is a useful and relatively safe tool in the evaluation of surgical candidates when noninvasive investigations fail to localize the epileptogenic zone. SEEG-based resective surgery may provide excellent results in particularly complex drug-resistant epilepsies.

Adolescent↗

PubMed2003

Nocturnal frontal lobe epilepsy: intracerebral recordings of paroxysmal motor attacks with increasing complexity.

PURPOSES: To show the results of the investigation conducted with intracerebral electrodes in a patient with drug-resistant nocturnal frontal lobe epilepsy, characterized by sleep-related paroxysmal motor attacks of increasing complexity ranging from simple and brief stereotyped motor events to paroxysmal arousals and major attacks. METHODS: The patient was studied with long-term video-stereo-electroencephalographic monitoring by means of stereotactically implanted intracerebral electrodes. RESULTS: Video-stereo-electroencephalography demonstrated that minor events and paroxysmal arousals, as well as major attacks, were correlated with a discharge in the right supplementary motor area and central cingulate gyms. The increasing complexity of these sleep-related ictal motor behaviors reflected a different pattern of discharge, with a progressive spread to other frontal and extrafrontal areas in the fully developed attacks. Surgical resection of the right supplementary motor area and central cingulate gyrus was performed. In the 5 years since the operation, the patient has remained completely seizure free. CONCLUSION: This study clearly demonstrates the ictal origin of minor events in nocturnal frontal lobe epilepsy. The increasing complexity of the motor behaviors from minor to major attacks reflects different duration, amplitude, and spread of the epileptic discharge.

Adult↗

PubMed2003

Focal cortical dysplasias: MR imaging, histopathologic, and clinical correlations in surgically treated patients with epilepsy.

BACKGROUND AND PURPOSE: Focal cortical dysplasia (FCD) covers a spectrum of conditions in which the neuropathologic and electroclinic presentations and the surgical outcomes vary. The aim of this study was to identify the MR features of histologic subtypes of FCD that would be useful for differential diagnosis. METHODS: We reviewed the MR data of 49 patients treated surgically for intractable partial epilepsy, who received a histologic diagnosis of FCD not associated with other brain abnormalities except hippocampal sclerosis and who were classified by histologic criteria as having architectural dysplasia (28 patients), cytoarchitectural dysplasia (six patients), or Taylor's FCD (15 patients). RESULTS: From the MR features, it was generally possible to distinguish Taylor's FCD from architectural or cytoarchitectural dysplasias (non-Taylor's FCD). Findings suggesting Taylor's FCD were focal cortical thickening, blurring of the gray-white matter junction, and hyperintensity (on T2-weighted images) of subcortical white matter often tapering toward the ventricle. Focal brain hypoplasia with shrinkage and moderate signal intensity alterations in the white matter core were present in most patients with architectural dysplasia. The lesion was generally extratemporal in Taylor's FCD and temporal in architectural dysplasia. Ipsilateral hippocampal sclerosis was often present in architectural dysplasia (dual abnormality). CONCLUSIONS: In patients with FCD, Taylor's FCD and non-Taylor's FCD can usually be distinguished with MR imaging, although some overlap exists. A provisional MR diagnosis is important for presurgical investigations and surgical planning and may have prognostic implications.

Adolescent↗

PubMed2003

Cortical development and focal cortical dysplasia.

A brief survey of cortical development is presented, focusing on neuronal migration and its alterations. Corticogenesis is achieved through ordered temporospatial steps, via the formation of transient structures, and successive waves of cell proliferation and migration (followed by cell differentiation and maturation), and apoptotic cell death. The appearance of the proliferative ventricular zone and marginal zone, and of the superficial primordial plexiform layer, is followed by the formation of the prospective layer I, of the subplate, whose neurons are destined to die, and of the cortical plate that will give rise to layers II-VI. Cells arising in the ventricular zone migrate radially using radial glia as a scaffold, and are destined to form pyramidal cells. Cortical interneurons are mainly generated in the ganglionic eminence and migrate along axonal substrates following tangential routes. Disorders of this complex process lead to a wide range of alterations, and focal derangements of cortical organization have been grouped under the term focal cortical dysplasia (FCD). As the result of a neuropathological revision of FCD cases with intractable epilepsy, a novel classification comprising three subgroups of FCD has been introduced, and is supported by electroclinical and neuroimaging data, as well as by the postsurgical outcome of patients: i). architectural dysplasia, characterized by altered cortical lamination; ii). cytoarchitectural dysplasia, with the occurrence of giant neurons besides cortical dyslamination; iii). Taylor-type cortical dysplasia, in which altered cortical lamination is consistently associated with the occurrence of giant, dysmorphic and ectopic neurons, and frequently with the so-called balloon cells.

Cell Movement↗

PubMed2003

Neuroimaging of focal cortical dysplasia: neuropathological correlations.

Focal cortical dysplasia is a well-known cause of intractable epilepsy with early onset of seizures, and is potentially amenable to surgical therapy. It was first described by Taylor in 1971 as a peculiar malformative disorganisation of the neocortex characterised at histology by loss of cortical lamination and accompanied by giant, dysmorphic neurones and, most frequently, by "balloon cells" littered throughout the cortex and sub-cortical white matter. While in the past decades the term "cortical dysplasia" has referred to various malformations of cortical development, such as agyria, pachygyria, polymicrogyria, heterotopia and hemimegalencephaly, it is now widely accepted that the entity identified by Taylor should be considered separately, from both histological and neuroimaging standpoints. More recently, the recognition of various histological subtypes of focal cortical dysplasia characterised by different degrees of cortical disruption with or without cytological abnormalities has generated several classifications that are still unsatisfactory. With better magnetic resonance capability, subtle and very small focal cortical dysplasias may now be visualised and the differential magnetic resonance aspects of the histological subgroups can be established. We will discuss the problem of histopathological classification and magnetic resonance imaging differentiation of the various subtypes of focal cortical dysplasia in the light of personal data collected from a large series of epileptic patients who underwent surgery and had a histological diagnosis of focal cortical dysplasia.

Atrophy↗

PubMed2003

Intra-lesional stereo-EEG activity in Taylor 's focal cortical dysplasia.

Focal cortical dysplasia are a frequent histological finding in epilepsy surgery series. Among the different types of focal cortical dysplasia, distinctive anatomical, electrical and clinical details have been identified for Taylor's focal cortical dysplasia, and in a recent article we reported a better post-surgical outcome in Taylor's focal cortical dysplasia than in other histological subtypes of cortical dysplasias. In the present study, we analysed the intra-lesional electrical activity directly recorded inside Taylor's focal cortical dysplasia during a stereo-EEG diagnostic procedure in 21 patients selected from among the 27 cases in which post-operative neuropathological examination demonstrated this kind of lesion. Our data show the existence of a peculiar interictal pattern characterised by the presence of repetitive and rhythmic spike and poly-spike and wave, frequently associated with short bursts of fusiform micro poly-spikes. Moreover, an almost pathognomonic ictal pattern (mid-amplitude 14-18 Hz rhythmic activity followed by a low voltage recruiting fast activity) is present in 12 of these 21 patients. These electrical peculiarities suggest a high level of epileptogenicity of Taylor's focal cortical dysplasia and could possibly explain the high percentage of post-surgical success among patients with this kind of lesion.

Adult↗

PubMed2003

Focal cortical resection in malformations of cortical development.

Malformations of cortical development may be associated with drug-resistant partial epilepsy suitable for surgical therapy. From the anatomopathological point of view, this categorisation has been used in reference to a wide range of alterations of the cortical mantle. Focal cortical dysplasias represent the main group of malformations of cortical development, but there are also other types of alterations, such as heterotopias, double cortex or polymicrogyria. Defining candidacy for surgical therapy and tailored resection requires thorough pre-surgical evaluation so that the approach will be individualised for each patient. We present our series of 126 patients with malformation of cortical development selected from 321 consecutively operated patients. Within this group encompassing different types of malformation of cortical development, including periventricular heterotopia (nine patients), polymicrogyria (three patients), hemimegalencephaly (one patient) and subcortical band heterotopia (one patient), the largest group was 81 individuals with focal cortical dysplasia. For this last group, we propose a simplified classification defining 42 architectural dysplasias, 12 cytoarchitectural dysplasias and 27 Taylor's focal cortical dysplasias. In addition, at routine neuropathological investigation, the only morphological alteration shown by 31 patients was diffuse neuronal heterotopia. All patients underwent scalp EEG and video-EEG, and 75 patients (59.5%) also underwent stereo-EEG. Magnetic resonance imaging and stereotactic stereoscopic angiography represented the indispensable premises for further studies, in particular stereo-EEG, and for planning surgery and tailoring resection. Magnetic resonance imaging was unhelpful in 17 out of 81 patients with focal cortical dysplasia and in seven out of 31 with neuronal heterotopia, while signal alterations were present in all other cases. Common characteristics corresponding to clinical-histopathological homogeneous subgroups were found within the focal cortical dysplasia group. In patients with architectural dysplasia, the epileptogenic zone was mainly in the temporal lobe and there was a lower seizure frequency than in patients with Taylor's focal cortical dysplasia. Patients with Taylor's type had an epileptogenic zone that was mainly extra-temporal, and a distinctive interictal stereo-EEG. The best outcome was observed in patients with Taylor's type dysplasia: 69% seizure-free (Engel class Ia) after at least 1 year of follow-up, compared with 45% of cytoarchitectural dysplasia and 49% of architectural dysplasia patients.

Adult↗

PubMed2002

AlphaCaMKII and NMDA-receptor subunit expression in epileptogenic cortex from human periventricular nodular heterotopia.

PURPOSE: Periventricular nodular heterotopia (PNH) is the most common human brain dysgenesis, very frequently characterized by focal drug-resistant epilepsy. To understand the cellular mechanisms underlying its intrinsic hyperexcitability, we investigated the expression of glutamate-receptor subunits and related proteins in four human patients affected by PNH. METHODS: PNH was diagnosed by means of magnetic resonance imaging. The epileptogenic area was revealed by depth electrode recordings and removed during epilepsy surgery. Sections from the removed cerebral tissue were analyzed by means of immunocytochemistry (ICC), with antibodies directed against N-methyl-d-aspartate (NMDA)-receptor subunits, the alpha subunit of the Ca2+/calmodulin-dependent kinase II (alphaCaMKII), and its active phosphorylated form. RESULTS: The ICC data demonstrated that the subcortical heterotopic nodules were consistently characterized by lower expression of alphaCaMKII and its activated form. In more pronounced cases (i.e., when the extension of the nodules to the neocortex determined clear layering abnormalities), the heterotopic tissue also was characterized by a decreased expression of NMDA-receptor subunits, which was particularly evident in the dendritic compartment. CONCLUSIONS: These data suggest the existence of an alteration of alphaCaMKII and the NMDA-receptor complex in the epileptogenic brain tissue of human PNH, which may play a role in the basic mechanisms of hyperexcitability associated with this brain dysgenesis.

Adult↗

PubMed2002

Surgical pathology of drug-resistant partial epilepsy. A 10-year-experience with a series of 327 consecutive resections.

Herein we reviewed the histopathological findings in 327 consecutive surgical specimens from patients with pharmaco-resistant epilepsy (PRE). Three major pathological groups (78.3% of all cases) were identified: Ammon's horn sclerosis (85 cases as an isolated lesion plus 18 as one part of a dual pathology), tumors (94 cases), and malformations (77 cases). Tumors, often associated with cortical dysplasias (CDs), were all of low histological grade and included 61 dysembryoplastic neuroepithelial tumors (DNTs), 29 gangliogliomas, and 4 pleomorphic xanthoastrocytomas (PXAs). Among the malformations were observed 52 CDs, 13 cavernomas, 8 cortical tubers, and 4 cysts. The remaining findings consisted of 16 scars (mostly post-traumatic) and 4 Rasmussen's encephalitis. Fifty-one (15.6%) specimens contained non-specific changes, and histological samples from 215 patients with presurgical implantation of electrodes revealed iatrogenic changes. All these figures are in agreement with the most recent and comparable series, and confirm the high incidence of DNTs that appear clearly as the most common tumoral entity in PRE. Our data also support the hypothesis of a close histogenetic relationship between DNT, ganglioglioma, and PXA, with a putative common origin from pluripotential progenitor CD34 positive cells of the subpial granular layer. As for CDs, our study confirms the clinical relevance of two main subtypes: severe CD (or Taylor's type CD) with neuronal cytomegaly and balloon cells, and non-Taylor's type CDs with a better outcome. Eventually, this series demonstrates that most patients have significant histopathological lesions, among which, aspects that are relevant to the diagnostic surgical pathologist are highlighted [published with a complete image database on CD-Rom].

Adolescent↗