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Biomedical subjects

G Lo Russo

Publications and source records attributed to G Lo Russo.

At least 19 recordsLinked to original sources

Surgical treatment of drug-resistant nocturnal frontal lobe epilepsy.

Of the cases with nocturnal frontal lobe epilepsy (NFLE) approximately 30% are refractory to antiepileptic medication, with several patients suffering from the effects of both ongoing seizures and disrupted sleep. From a consecutive series of 522 patients operated on for drug-resistant focal epilepsy, 21 cases (4%), whose frontal lobe seizures occurred almost exclusively (>90%) during sleep, were selected. All patients underwent a comprehensive pre-surgical evaluation, which included history, interictal EEG, scalp video-EEG monitoring, high-resolution MRI and, when indicated, invasive recording by stereo-EEG (SEEG). There were 11 males and 10 females, whose mean age at seizure onset was 6.2 years, mean age at surgery was 24.7 years and seizure frequency ranged from <20/month to >300/month. Nine patients reported excessive daytime sleepiness (EDS). Prevalent ictal clinical signs were represented by asymmetric posturing (6 cases), hyperkinetic automatisms (10 cases), combined tonic posturing and hyperkinetic automatisms (4 cases) and mimetic automatisms (1 case). All patients reported some kind of subjective manifestations. Interictal and ictal EEG provided lateralizing or localizing information in most patients. MRI was unrevealing in 10 cases and it showed a focal anatomical abnormality in one frontal lobe in 11 cases. Eighteen patients underwent a SEEG evaluation to better define the epileptogenic zone (EZ). All patients received a microsurgical resection in one frontal lobe, tailored according to pre-surgical evaluations. Two patients were operated on twice owing to poor results after the first resection. Histology demonstrated a Taylor-type focal cortical dysplasia (FCD) in 16 patients and an architectural FCD in 4. In one case no histological change was found. After a post-operative follow-up of at least 12 months (mean 42.5 months) all the 16 patients with a Taylor's FCD were in Engel's Class Ia and the other 5 patients were in Engel's Classes II or III. After 6 months post-surgery EDS had disappeared in the 9 patients who presented this complaint pre-operatively. It is concluded that patients with drug-resistant, disabling sleep-related seizures of frontal lobe origin should be considered for resective surgery, which may provide excellent results both on seizures and on epilepsy-related sleep disturbances. An accurate pre-surgical evaluation, which often requires invasive EEG recording, is mandatory to define the EZ. Further investigation is needed to explain the possible causal relationships between FCD, particularly Taylor-type, and sleep-related seizures, as observed in this cohort of NFLE patients.

Adolescent↗

Stereo-EEG in children.

BACKGROUND: Stereotactic placement of intracerebral multilead electrodes for chronic EEG recording of seizures or stereoelectroencephalography (SEEG) was introduced 50 years ago at Saint Anne Hospital in Paris, France for the presurgical evaluation of patients with drug-resistant focal epilepsy. SEEG explorations are indicated whenever the noninvasive tests fail to adequately localize the epileptogenic zone (EZ). INDICATIONS: Currently, approximately 35% of our operated-on children require a SEEG evaluation. Arrangement of electrodes is individualized according to the peculiar needs of each child, to verify a predetermined hypothesis of localization of the EZ based on pre-SEEG anatomo-electro-clinical findings. Multilead intracerebral electrodes are designed to sample cortical structures on the lateral, intermediate, and mesial aspect of the hemisphere, as well as deep-seated lesions. Stereotactic stereoscopic teleangiograms and coregistered 3-D MRI are employed to plan avascular trajectories and to accurately target the desired structures. Pre-SEEG stereotactic neuroradiology and electrode implantation are usually performed in separate procedures. Electrodes are removed once video-SEEG monitoring is completed. INTRACEREBRAL ELECTRICAL STIMULATIONS: Intracerebral electrical stimulations are used to better define the EZ and to obtain a detailed functional mapping of critical cortical and subcortical regions. MORBIDITY: Surgical morbidity of SEEG is definitely low in children. SEEG-GUIDED RESECTIVE SURGERY: In 90% of evaluated children, SEEG provides a guide for extratemporal or multilobar resections. SEEG-guided resective surgery may yield excellent results on seizures with 60% of patients in Engel's Class I.

Brain Mapping↗

Treatment of human immunodeficiency virus-associated facial lipoatrophy with lipofilling and submalar silicone implants.

In the absence of a current therapy to prevent facial lipoatrophy in HIV+ patients treated with HAART, surgical correction of the defect still remains the best option. We evaluate two different surgical techniques for facial contour enhancing and suggest the right choice related to the lipodystrophy severity. Twelve HIV+ patients underwent surgical submalar correction: eight were treated with lipofilling following Coleman's technique and four had bilateral malar silicone implants inserted after determining their positioning with the aid of a new software. Both techniques gave long lasting results in facial contour reshaping ranging from good to very good. No complication was observed. In the mean follow-up period of 2 years no patient felt uncomfortable with his/her image. Both techniques, lipofilling and silicone implant positioning, for managing facial lipoatrophy in HIV+ patients treated with HAART had good results, but the right choice has to be related to the severity of the lipodystrophy in the patient.

Adipose Tissue↗

Intracerebral recordings of minor motor events, paroxysmal arousals and major seizures in nocturnal frontal lobe epilepsy.

The clinical features of nocturnal frontal lobe epilepsy (NFLE) consist of a spectrum of paroxysmal motor manifestations ranging from minor motor events (MMEs) to paroxysmal arousals (PAs) and major seizures. During MMEs and PAs scalp EEG generally does not show definite ictal abnormalities. We describe the clinical and electrophysiological features of three patients affected by drug-resistant NFLE studied with intracerebral electrodes during a presurgical evaluation. The stereo-EEG (SEEG) investigation revealed that MMEs can be fragments of the major seizure and occur during a brief epileptic discharge or on the following arousal. PAs, in the same subject, do not show a definite stereotypy despite the morphological and topographic similarity of the epileptic discharges, thus indicating that other variables may influence the clinical features of PAs.

Adult↗

Sleep-related hyperkinetic seizures: always a frontal onset?

Hyperkinetic seizures are considered a typical manifestation of nocturnal frontal lobe epilepsy (NFLE). Patients with temporal lobe epilepsy with mainly sleep-related seizures have been described; however they commonly lack hyperkinetic activity and seizure frequency is low. We retrospectively analysed our population of 442 consecutive patients surgically treated between January 1996 and January 2004. Among these there were 25 patients with sleep-related hyperkinetic epileptic seizures, with a frontal lobe onset in 18 cases and a temporal lobe onset in 7. Patients with sleep-related hyperkinetic seizures with temporal lobe origin had anamnestic and clinical features strikingly similar to those with a frontal onset, with agitated movements, high seizure frequency and no history of febrile convulsions. We confirm our previous findings that this kind of epileptic manifestation is not only peculiar to frontal lobe epilepsy.

Adolescent↗

Electroclinical, MRI and neuropathological study of 10 patients with nodular heterotopia, with surgical outcomes.

We present the results of a retrospective study on 10 patients operated on for intractable epilepsy associated with nodular heterotopia as identified by high resolution MRI. Seven patients had unilateral heterotopia, one patient had symmetric bilateral heterotopia and two patients had asymmetric bilateral heterotopia. By stereo-electroencephalogram (SEEG) (nine patients) interictal activity within nodules was similar in all cases, and ictal activity never started from nodules alone but from the overlying cortex or simultaneously in nodules and cortex. Excellent outcomes (Engel class Ia, 1987) were achieved in the seven patients with unilateral heterotopia, showing that surgery can be highly beneficial in such cases when the epileptogenic zone is carefully located prior to surgery by MRI and particularly SEEG. For the bilateral cases surgical outcomes were Engel IIa (one patient) or Engel IIIa (two patients). Histological/immunohistochemical studies of resected specimens showed that all nodules had similar microscopic organization, even though their extent and location varied markedly. The overlying cortex was dysplastic in nine patients, but of normal thickness. We suggest that nodule formation may be the result of a dual mechanism: (i) failure of a stop signal in the germinal periventricular region leading to cell overproduction; and (ii) early transformation of radial glial cells into astrocytes resulting in defective neuronal migration. The intrinsic interictal epileptiform activity of nodules may be due to an impaired intranodular GABAergic system.

Adult↗

Sleep-related hyperkinetic seizures of temporal lobe origin.

Sleep-related hyperkinetic seizures are a common feature of nocturnal frontal lobe epilepsy. Although sleep-related seizures with a temporal lobe origin have been reported, they commonly lack hyperkinetic activity. The authors describe three patients with sleep-related seizures characterized by frenetic, agitated, hyperactive movements (bimanual/bipedal activity, rocking, axial, pelvic, and hemiballistic movements), in whom stereo-EEG investigation and surgical outcome demonstrated a temporal lobe origin of the attacks.

Adult↗

A neuropathological, stereo-EEG, and MRI study of subcortical band heterotopia.

The authors performed an MRI, stereo-EEG, and pathology study on a woman with subcortical band heterotopia and partial epilepsy. Clinical manifestations of seizures always started when ictal discharges were present in outer and heterotopic cortices. Simultaneous activation of both cortices and presence of differentiated neurons in the white matter and the heterotopia strongly suggest that the cortices were anatomically and functionally interconnected.

Adult↗

Preserved functional competence of perilesional areas in drug-resistant epilepsy with lesion in supplementary motor cortex: fMRI and neuropsychological observations.

We report a presurgical fMRI study and a longitudinal behavioral and structural MRI study in a 26-year-old right-handed woman with drug-resistant epilepsy of the supplementary motor region with cytoarchitectural dysplasia and minimal cortico-subcortical gliotic damage. fMRI scans were acquired during a silent phonemic verbal fluency task (VF), an automatic counting task (CT), and a finger-tapping motor task (MT). These were all compared with rest. Presurgical neuropsychological assessment was substantially normal with only a minor deficit in the domain of visuo-constructive and complex motor-planning skills. Noticeably, performance on phonemic verbal fluency was normal. Presurgical fMRI results revealed a normal specialization of left SMA and pre-SMA, including a fine-grained somatotopy for mouth and hand representations despite epilepsy. Immediately after surgical removal of the epileptogenic zone (the posterior third of the superior and middle frontal gyri including pre-SMA and part of SMA, and part of the anterior cingulate region--all of which were active presurgically at the fMRI tests), the patient suffered from transcortical motor aphasia temporarily. One year after surgery, she still showed impaired performance in the verbal fluency tasks while naming and comprehension were recovered. The patient was now free from seizures. This fMRI study supports the case that repeated seizures per se may not be sufficient to alter the distribution of neural representations of cognitive function. Selective behavioral impairment after surgical removal of brain areas that were activated during presurgical fMRI permits us to establish a causal link between these activations and task performance. This link could not have been made on the basis of activation patterns or lesion data taken on their own. These findings support the case that some epileptic patients may represent a unique opportunity for cognitive neuroscience studies.

Adult↗

Surgery for drug resistant partial epilepsy in children with focal cortical dysplasia: anatomical-clinical correlations and neurophysiological data in 10 patients.

OBJECTIVE: To analyse a population of children with focal cortical dysplasia operated on for drug resistant partial epilepsy, with emphasis on clinical features, seizure semiology, interictal and ictal EEG and stereo EEG findings, histological and topographical characteristics of the lesions, extension and localisation of cerebral excision, and its postoperative effect on seizure frequency. METHODS: 10 patients were studied, aged between 26 months and 11 years (median 6 years). Magnetic resonance imaging (MRI) abnormalities were unilobar (temporal 3, frontal 2), bilobar (2), or multilobar (1); the two patients with negative MRI suffered from frontal seizures. Presurgical diagnostic steps varied in complexity and invasiveness depending on the anatomical/electrical/clinical features of each patient. In four patients they included only scalp video EEG monitoring, and in six, also invasive recordings using stereotactically implanted intracerebral electrodes. Surgery consisted of corticectomy plus lesionectomy in all cases. RESULTS: 70% of the patients were seizure-free after a minimum postoperative follow up of 25 months. These included three patients with temporal lesions and four of seven patients with other lobar or multilobar extratemporal localisation. One patient had improvement in seizure control. Outcome was poor in multilobar patients, but a class Ia outcome was obtained in one case after partial lesionectomy associated with bilobar corticectomy. All patients showed developmental improvement. CONCLUSIONS: Analysis of the data in these patients allowed the production of an "anatomical-clinical concordance" list, which appeared to be correlated with the diagnostic steps performed. Carrying out a stereo EEG exploration in the most complex cases proved useful in defining the epileptogenic zone in extratemporal and multilobar epilepsies. Stereo EEG recordings facilitated a tailored resection of extralesional cortex.

Cerebral Cortex↗

Focal cortical dysplasia: neuropathological subtypes, EEG, neuroimaging and surgical outcome.

Since the original description by Taylor, the term focal cortical dysplasia has been used to refer to a wide range of alterations of the cortical mantle. More recently, these conditions have been described from neuroimaging, neuropathological and genetic standpoints, generating several classifications. It is widely recognized that these classifications are unsatisfactory. We propose a simplified classification of focal cortical dysplasias based on easily recognized neuropathological characteristics. We retrospectively re-examined histological sections of cortex from 52 of 224 (23%) patients operated on for drug-resistant partial epilepsy in which cortical dysplasia was present but not associated with other brain pathologies except hippocampal sclerosis. Three subgroups were identified: (i) architectural dysplasia (31 patients) characterized by abnormal cortical lamination and ectopic neurones in white matter; (ii) cytoarchitectural dysplasia (six patients) characterized by giant neurofilament-enriched neurones in addition to altered cortical lamination; and (iii) Taylor-type cortical dysplasia (15 patients) with giant dysmorphic neurones and balloon cells (all but two patients) associated with cortical laminar disruption. The patients with architectural dysplasia had lower seizure frequency than those with cytoarchitectural and Taylor-type dysplasia, and the epileptogenic zone was mainly in the temporal lobe. In patients with Taylor-type dysplasia, the epileptogenic zone was mainly extratemporal, and interictal stereo-EEG was distinctive. MRI was unrevealing in 34% of patients, but distinctive signal alterations characterized most patients with Taylor-type dysplasia, while focal hypoplasia with MRI abnormalities was found in architectural dysplasia. Patients with Taylor-type dysplasia had the best outcome, with 75% seizure-free (Engel class Ia) after at least a year of follow-up compared with 50% of cytoarchitectural dysplasia and 43% of architectural dysplasia patients seizure-free. This three-category classification is based on easily recognized histopathological characteristics and avoids complicated terminology, while the distinctive ensemble of other characteristics defines clinically homogeneous groups.

Adult↗

Cutaneous ciliated cyst: a case report with immunohistochemical evidence for dynein in ciliated cells.

Cutaneous ciliated cysts (CCCs) are rare benign lesions predominantly occurring in the lower limbs of young women. We observed such a lesion in the perineal region of a 12-year-old girl. The histogenetic interpretation of CCCs is controversial. The similarity of the epithelial lining of the cyst to the salpingeal epithelium supports the hypothesis of a Müllerian heterotopia. Strong dynein positivity observed immunohistochemically in the apical portion of CCC lining cells suggests the integrity of the ciliary apparatus. There was a marked similarity between a CCC and normal salpingeal epithelium in the mode of staining for dynein, whereas the dynein reactivity of bronchial epithelial cells showed larger cytoplasmic aggregates of positive material in proximity to the nucleus. No immunohistochemical staining for estrogen or progesterone receptors was identified in our observation, although this finding could have supported the Müllerian histogenetic hypothesis. To the best of our knowledge, this is the first time that antidynein immunohistochemistry has been applied to paraffin-embedded samples from human surgical pathology.

Child↗

Inhibitory circuits in human dysplastic tissue.

PURPOSE: Different types of epilepsies and seizures depend on the nature and location of the primary disturbance and are presumably mediated by different physiopathological mechanisms. We immunocytochemically investigated possible changes in the inhibitory-aminobutyric acid (GABA)ergic system in specimens taken from four patients who underwent surgery for intractable epilepsy and presented two different types of focal cortical dysplasia in the temporal lobe. METHODS: The patients were selected on the basis of electroclinical, imaging, and routine neuropathological data: two had Taylor focal dysplasia, and two had non-Taylor dysplasia (microdysgenesia). The study was performed using antibodies against parvalbumin (PV), glutamic acid decarboxylase (GAD), and GABA-transporter 1 (GAT1). RESULTS: In the patients with Taylor dysplasia, laminar disorganization of the cortex was associated with the presence of giant neurons and ballooned cells; there was a reduced number of PV-positive neurons and terminals, the giant neurons were surrounded by clusters of PV- and GAD-positive terminals, and there was an overall reduction in GAT1. Despite the presence of cortical laminar disorganization, no giant or ballooned cells were found in the patients with non-Taylor microdysgenesia; there was a marked decrease in PV and GAD immunoreactive elements, with a patchy distribution of GAD and GAT1 immunoreactivity but no clustering of PV and GAD terminals. CONCLUSIONS: These results suggest that the two forms of cortical dysplasia are characterized by different and selective morphofunctional alterations in the GABAergic system.

Adult↗

Clinical ictal symptomatology and anatomical lesions: their relationships in severe partial epilepsy.

High-resolution imaging techniques can demonstrate anatomic alterations in most patients identified as candidates for surgical treatment of their partial epilepsy. The demonstration of an anatomic lesion is only one step in the presurgical diagnostic procedure, which includes video-EEG and, when necessary, video-stereo-EEG recordings of seizures. A review of the literature shows that the simple removal of the magnetic resonance imaging (MRI)-evident lesion ("lesion-ectomy") reduces but does not completely suppress seizures in a large percentage of patients, especially those with neuronal migration disorders. This phenomenon could, at least in part, be explained by preliminary data (in 33 patients) showing that less than 20% of seizures correspond to a well-localized, intralesional discharge in about 40% of stereo-EEG-investigated patients with at least one intralesional electrode. The authors illustrate some anatomo-electroclinical examples of intraindividual variability of the ictal symptomatology, raising the problem of the decision about the extent of the surgical removal. Recent histologic and immunohistochemical studies have demonstrated several kinds of structural alterations in the stereo-EEG-defined epileptogenic zone, not always overlapping with the MRI-visible lesion. This aspect can further explain some failures of MRI-guided lesionectomies. That relationships between "lesions" and epileptogenic zones may be variable is also suggested by reports of patients who present with multiple lesions (i.e., cavernous angiomas, Bourneville syndrome) and are cured by removal of only one of them.

Adult↗

Presurgical strategies and epilepsy surgery in children: comparison of literature and personal experiences.

We reviewed 41 studies on epilepsy surgery in 1645 children and adolescents. The available data vary greatly from one paper to another, particularly as regards the age of the patients included, selection criteria, presurgical methodology, and the type of surgical intervention. Moreover, the surgical results are classified according to very different criteria. Our experience in Grenoble concerning 55 children (age < or = 16 years; postoperative follow-up > 18 months) is discussed in the light of data in the literature. The need is stressed for a consensus on the type of information that must be supplied by well-detailed studies on homogeneous groups of epilepsy surgery patients.

Adolescent↗

Research perspectives in cortical dysplasia and associated epilepsies.

Our understanding of cortical alterations and related epilepsies has grown enormously in the last decade thanks to the explosion of basic information from laboratory neuroscience combined with advances in diagnostic tools, therapeutic approaches and surgical techniques. In the present paper, we briefly review the most important advances in these fields from the point of view of the clinician concerned with cortical malformation-related epilepsies. We propose that a highly effective way forward, expected not only to widen knowledge of the basic mechanisms of seizure generation, but also to improved the management of patients, would be to promote interdisciplinary research programmes on resected human cortex that involve neurosurgeons, neurologists and laboratory neuroscientists.

Cerebral Cortex↗

Oncocytoma of the nasal cavity: report of a case and review of the literature.

The authors present a case of oncocytoma of the nasal cavity in a 60-year-old woman. The tumour shows the same histological and ultrastructural characteristics of oncocytomas arising in minor salivary glands of other sites and major salivary glands. The authors stress that the biological behaviour of oncocytomas of the nasal cavity seems related to their local growth rather than to the cytological characteristics of the tumour. The extreme rarity of this tumour in the nasal cavity is particularly emphasized. The few cases reported previously in literature are briefly reviewed.

Adenoma, Oxyphilic↗