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Spalice A.,University of Rome La Sapienza | Ruggieri M.,University of Catania | Grosso S.,University of Siena | Verrotti A.,University of Chieti Pescara | And 7 more authors.
Pediatric Neurology | Year: 2010

Dysembryoplastic neuroepithelial tumors (DNETs) are benign intracortical masses that are typically observed in children and young adults and are classified as glioneuronal tumors (WHO grade I). Large and retrospective series of patients with DNETs have been reported, but prospective studies on pediatric cohorts of patients with DNETs have been lacking. In the present study, 13 children (8 boys, 5 girls; age 8-18 years) who had simple (n = 2) or complex (n = 11) partial seizures (seizure duration range, 2-4 years; mean, 1.5 years; mode, 1.2 years) were prospectively enrolled and monitored over 13 years. The DNETs were located in the frontal (n = 2), temporal (n = 9), or occipital (n = 2) cortex. In 11/13 cases, the seizures were resistant to drug therapy, and all the children had surgery consisting of extended lesionectomy coupled with neuronavigation. Pathology examination revealed cortical dysplasia (n = 8), glial nodules (n = 11), calcification (n = 4), cellular atypia (n = 3), endothelial proliferation (n = 1), perivascular inflammation (n = 3), and meningeal involvement (n = 6). All children were seizure free throughout postsurgical follow-up of 2-11 years. This first prospective study with follow-up monitoring of a childhood population with DNETs confirms, on a long-term basis, that the coupled strategy of extended lesionectomy and neuronavigation has good outcome for long-term seizure control. © 2010 by Elsevier Inc. All rights reserved.


Nicita F.,University of Rome La Sapienza | Ruggieri M.,University of Catania | Polizzi A.,Superior Institute of Health | Mauceri L.,Health Care Center for Obstetrics and Gynecology Lucina | And 11 more authors.
Epilepsia | Year: 2012

Sotos syndrome (SS) is an overgrowth syndrome characterized by typical facial appearance, learning disability, and macrocephaly as cardinal diagnostic features. Febrile (FS) and afebrile seizures are reported in 9-50% of cases. There is no evidence that patients with SS and FS later develop epilepsy, and no studies have investigated the electroclinical features and the long-term outcome in epileptic SS patients. The authors report a series of 19 SS patients with FS and/or epilepsy during childhood and a long-term follow-up. More than half of FS evolved to epilepsy. Temporal lobe seizures were recorded in 40% of patients with SS. Seizures were easy to control with common antiepileptic drugs in almost all patients. A careful neurologic evaluation is useful for SS patients, since seizures are an important finding among people with this overgrowth syndrome. © Wiley Periodicals, Inc.


Salpietro V.,Messina University | Mankad K.,University College London | Polizzi A.,Superior Institute of Health | Sugawara Y.,Tokyo Medical and Dental University | And 6 more authors.
Pediatrics International | Year: 2014

Hashimoto encephalopathy is a syndrome of encephalopathy associated with elevated concentration of circulating serum anti-thyroid antibodies usually responsive to steroid therapy. We report a 13-year-old girl with Hashimoto encephalopathy and peripheral nervous system involvement. The child had experienced high-grade pyrexia, global headache and sleeplessness. After admission she had an ileus with a distended urinary bladder, hallucinations and cognitive impairment. She had reduced deep tendon reflexes and distal sensory deficiency. Anti-thyroglobulin antibodies were raised at 2121 IU/mL (normal, 0-40) and the anti-thyroperoxidase was high at 886 IU/mL (normal, 0-50). Progressive neurological and psychiatric remission was noted after i.v. methylprednisolone. Follow-up magnetic resonance imaging showed complete resolution of the foci of signal abnormality previously yielded. This case report is the first, to the best of our knowledge, to describe peripheral nervous system involvement in a child with a diagnosis of Hashimoto's encephalopathy. © 2014 The Authors. Pediatrics International © 2014 Japan Pediatric Society.


Salpietro V.,Messina University | Polizzi A.,Superior Institute of Health | Berte L.F.,Messina University | Chimenz R.,Messina University | And 6 more authors.
Neuroendocrinology Letters | Year: 2012

The clinical syndrome idiopathic intracranial hypertension (IIH), also termed pseudotumor cerebri, consists of symptoms of headache, nausea, vomiting and visual field defects in combination with findings of papilledema. IIH is more commonly seen in overweight women where the rise in intracranial pressure is putatively a consequence of an endocrine-based disturbance of electrolytes. Less frequently, it can also occur in men and in the pediatric age group. Associated risk factors include primary and secondary aldosteronism, pregnancy, recombinant growth hormone (r-GH) therapy, oral contraceptives, obesity, vitamin A intoxication or deficiency, Addison disease, corticosteroid therapy or acute withdrawal of steroid therapy and Cushing disease. Herein, we review the association between these conditions and IIH working toward its having a unifying neuroendocrine hypothesis. © 2012 Neuroendocrinology Letters.


Pavone P.,University of Catania | Pettoello-Mantovano M.,University of Foggia | Le Pira A.,University of Catania | Giardino I.,University of Foggia | And 8 more authors.
Neuropediatrics | Year: 2010

Background: There are only a few series in the literature on acute disseminated encephalomyelitis (ADEM) in children. Objectives and Methods: the aims of this study were to perform (i) a prospective clinical/imaging study (1992-2009) on ADEM in children consecutively referred to our institution in Catania, Italy, and (ii) to undertake a systematic review and meta-analysis of published ADEM pediatric cohorts (>10 cases). Results: We identified 17 patients with ADEM (incidence <10 years of age=1.1 per 100 000 person-years). 15 previously published cohorts were compared with our cohort: (i) systematically reviewed (750 cases); and (ii) meta-analyzed (492/750 cases). The 17 patients had the following characteristics: (a) male-to-female ratio, 1.4 (vs. 1.2-1.3 in previous cohorts); (b) mean age at presentation, 3.6 years (vs. 7.1 years in previous cohorts); (c) specific preceding triggering factor, 88% (vs. 69-79% in previous cohorts); (d) the most common initial signs were ataxia, seizures, headache, and thalamic syndrome; (e) brain imaging revealed >3 lesions in 100% (vs. 92% in previous cohorts); (f) the outcome was good in 94% (vs. 70-75% in previous cohorts); and (g) 12% relapsed once (vs. 18% in previous cohorts). Conclusions: ADEM is generally a benign condition that mosly affects boys more than girls and rarely recurs. © Georg Thieme Verlag KG Stuttgart · New York.


Salpietro V.,Messina University | Mankad K.,Great Ormond Street Childrens Hospital | Kinali M.,Chelsea and Westminster Hospital | Adams A.,Great Ormond Street Childrens Hospital | And 15 more authors.
Journal of Pediatric Endocrinology and Metabolism | Year: 2014

Aim: To unravel the potential idiopathic intracranial hypertension (IIH) endocrine-metabolic comorbidities by studying the natural (and targeted drug-modified) history of disease in children. IIH is a disorder of unclear pathophysiology, characterized by raised intracranial pressure without hydrocephalus or space-occupying lesion coupled with normal cerebrospinal fluid (CSF) composition. Methods: Retrospective study (years 2001-2010) of clinical records and images and prospective follow-up (years 2010-2013) in 15 children (11 girls, 4 boys; aged 5-16 years) diagnosed previously as "IIH", according to the criteria for pediatric IIH proposed by Rangwala, at four university pediatric centers in northern, central, and southern Italy. Results: We identified six potential endocrine-metabolic comorbidities including, weight gain and obesity (n=5), recombinant growth hormone therapy (n=3), obesity and metabolic syndrome (n=1), secondary hyperaldosteronism (n=1), hypervitaminosis A (n=1), and corticosteroid therapy (n=1). Response to etiologically targeted treatments (e.g., spironolactone, octreotide) was documented. Conclusions: IIH is a protean syndrome caused by various potential (risk and) associative factors. Several conditions could influence the pressure regulation of CSF. An endocrine-metabolic altered homeostasis could be suggested in some IIH patients, and in this context, etiologically targeted therapies (spironolactone) should be considered.


PubMed | University of Milan Bicocca, University of Ferrara and Superior Institute of Health
Type: Journal Article | Journal: Journal of Indian Society of Periodontology | Year: 2015

So far, definitive implant abutments have been performed with high elastic modulus materials, which prevented any type of shock absorption of the chewing loads and as a consequence, the protection of the bone-fixture interface. This is particularly the case when the esthetic restorative material chosen is ceramic rather than composite resin. The adoption of an anisotropic abutment, characterized by an elastic deformability, could allow decreasing the impulse of chewing forces transmitted to the crestal bone.According to research protocol, the mechanical resistance to cyclical load was evaluated in a tooth-colored fiber-reinforced abutment (TCFRA) prototype and compared to that of a titanium abutment (TA), thus eight TCFRAs and eight TAs were adhesively cemented on as many titanium implants. The swinging that the two types of abutments showed during the application of sinusoidal load was also analyzed.In the TA group, both fracture and deformation occurred in 12.5% of samples while debonding 62.5%. In the TCFRA group, only debonding was present in 37.5% of samples. In comparison to the TAs, the TCFRAs exhibited a greater swinging during the application of sinusoidal load. In the TA group, the extrusion prevailed, whereas in the TCFRA group, the intrusion was more frequent.The greater elasticity of TCFRA to the flexural load allows absorbing part of the transversal load applied on the fixture during the chewing function, thus reducing the stress on the bone-implant interface.


PubMed | Superior Institute of Health
Type: Journal Article | Journal: International journal of immunopathology and pharmacology | Year: 2010

This study reports the immunomodulatory activity on human monocyte derived dendritic cells (MDDCs) of a vaccine preparation shown to be effective against an HPV16-related tumour in an animal model. The vaccine is composed of extract from Nicotiana benthamiana leaves containing HPV16 E7 protein expressed by a potato virus X-derived vector (NbPVX-E7). The effect of the extract was evaluated on MDDC differentiation and maturation by monitoring the phenotypic expression of specific markers. The results show that NbPVX-E7 does not induce monocyte differentiation to dendritic cells, but does induce MDDC maturation. Plant extract does not influence MDDC-uptake of E7-FITC while it significantly improves the Ovalbumin-FITC uptake, considered as a model antigen. Importantly, NbPVX-E7-pulsed MDDCs/PBMCs are able to prime human blood-derived lymphocytes from healthy individuals to induce HPV16 E7-specific cytotoxic activity. This is a propaedeutic study for a possible use of E7-containing plant extract in human immunotherapy of HPV-related lesions.


PubMed | Superior Institute of Health
Type: Journal Article | Journal: Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery | Year: 2012

Mutation analysis of the SCN1B, SCN1A and GABRG2 genes in children affected by Genetic (Generalised) Epilepsy with Febrile Seizures plus (GEFS(+)) and their affected and some unaffected family members, coming from a restricted geographic area, was performed.Eight GEFS(+) families (58 members) diagnosed according to current GEFS(+) criteria were studied.A heterozygous point mutation A2336G was detected in exon 13 of the SCNA1 gene in three affected members of one family but not in their unaffected relatives; a novel Ile1944Thr mutation was located within the intracellular C-terminal region of the SCNA1 gene in the proband and his healthy father in a second family. In the former family, the proband had dysmorphic features including large forehead, large nasal bridge, pointed nasal tip, triangular nostrils, deep nasolabial folds, thin upper lips with large mouth, congenital gingival hyperplasia with wide gingiva and mental retardation, abnormalities not previously listed in the clinical spectrum of GEFS(+).Our study confirms that just a few GEFS(+) families have mutations in the five genes classically known and reinforces the genetic and also the phenotypic variability of GEFS(+) featuring clinical manifestations. Question rises whether the cognitive problems seen in two siblings and dysmorphic features in one of them may be related to the channelopathy as it occurs in other well-known ion channel disorders.


PubMed | University of Rome La Sapienza and Superior Institute of Health
Type: | Journal: International journal of surgery (London, England) | Year: 2016

Authors analyze their experience of parathyroid autotransplantation during total thyroidectomy, with the purpose of seeing whether this practice influenced the rate of postoperative hypocalcemia and/or hypoparathyroidism. We identified three groups of patients: group A, consisting of 57 patients, underwent parathyroid autotransplantation during total thyroidectomy; group B consisting of 87 patients not submitted to intraoperative autotransplantation in whom, as an incidental finding, a parathyroid gland was detected in the surgical specimen; group C consisted of 100 patients who did not undergo autotransplantation and whose surgical specimens were not found to contain parathyroid glands. The three groups were compared for sex and age as well as for a series of clinical and laboratory parameters on the first three postoperative days and at six months after surgery. The rate of permanent hypoparathyroidism was 3.5% in Group A, 3.45% in Group B, and 1% in Group C. Multivariate analysis revealed that all three groups showed postoperative recovery of calcium levels, although the rate and extent of this recovery differed between them. The control group showed a more rapid and more complete recovery of serum calcium values compared with Groups A and B. Calcium recovery in Groups A and B was comparable, in terms of both rate and extent. The same pattern of results emerged for the iPTH values. The analysis of the data showed that there were no significant differences in the analyzed parameters between Groups A and B. This suggests that parathyroid autotransplantation does not influence the rate of postoperative hypocalcemia and/or hypoparathyroidism.

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