Publications by authors named "Filippo Greco"

54 Publications

A nationwide study on Sydenham's chorea: Clinical features, treatment and prognostic factors.

Eur J Paediatr Neurol 2022 Jan 6;36:1-6. Epub 2021 Nov 6.

Pediatric Rheumatology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Pisa, Italy.

Objectives: Sydenham's Chorea (SC) is a neuropsychiatric disorder and a major manifestation of acute rheumatic fever. The erroneous assumption that SC is a benign and self-limiting disease, has led to a lack of high-quality scientific evidence of the therapeutical and prognostic features of SC.

Study Design: We retrospectively analyzed the medical records of patients <18-years old with SC in 17 Italian pediatric centers. Recorded data included clinical, instrumental and laboratory parameters. Prognostic risk factors including treatment regimens were assessed with univariate and multivariate sub-analysis.

Results: We included 171 patients with SC. 66% had generalized chorea, and 34% hemichorea. 81% had carditis (subclinical in 65%). Additional neurological symptoms were reported in 60% of the patients, mainly dysarthria and dysgraphia. 51% had neuropsychiatric symptoms at onset, which persisted after 12 months in 10%. Among psychiatric manifestations, the most common was anxiety disorder/depression (77%). Neurological remission was reached by 93% of the patients at 6 months; 9% relapsed. Patients were treated as follows: 11% penicillin alone, 37% immunomodulatory therapy, 16% symptomatic drugs (i.e. anti-seizure medication, dopamine antagonists) and 37% both symptomatic and immunomodulatory treatment. Neurological outcome did not differ between groups. Patients receiving symptomatic drugs had a higher risk of relapse on multivariate analysis (p = 0.045).

Conclusions: Treatment of SC was largely heterogeneous. Based on our results, immunomodulatory therapy did not show higher efficacy at medium term, although it was associated to a slightly lower risk of relapse compared to symptomatic therapy. Longitudinal studies are needed to assess specific risk factors and best treatment options.
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http://dx.doi.org/10.1016/j.ejpn.2021.11.002DOI Listing
January 2022

Malformations of Cortical Development, Cognitive Involvementand Epilepsy: A Single Institution Experience in 19 Young Patients.

Children (Basel) 2021 Jul 26;8(8). Epub 2021 Jul 26.

Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Via S. Sofia 78, 95123 Catania, Italy.

Background: Malformations of cortical development (MCD) include a wide range of congenital disorders mostly causing severe cognitive dysfunction and epilepsy.

Objective: to report on clinical features including cognitive involvement, epileptic seizures with response to antiseizure medications, comorbidities in young patients affected by MCD and followed in a single tertiary hospital.

Patients And Methods: A retrospective review of the medical records and magnetic resonance images (MRI) of 19 young patients with an age ranging between eight days and fifteen years affected by MCD and admitted to Pediatrics Department University of Catania, Italy from October 2009 and October 2020 were selected. Patients were distinguished in three groups following the Barcovich et al. 2012 classification for MCD: 4 (21%) in Group I; 8 (42%) in Group II; and, and 7 (37%) in Group III. Clinical features and MRI of the patients including cognitive involvement, epilepsy type and response to drugs treatment were analyzed.

Results: In Group I, two patients showed cortical dysplasia and two dysembryoplastic neuroepithelial tumors plus focal cortical dysplasia; developmental delay/intellectual disability (DD/ID) was severe in one, moderate in one and absent in two; the type of seizures was in all the cases focal to bilateral tonic-clonic (FBTCs), and drug resistant was found in one case. In Group II, three patients showed neuronal hetero-topias and five had pachygyria-lissencephaly: DD/ID was severe in four, moderate in two, and absent in two; the type of seizure was focal (FS) in five, focal to bilateral tonic-clonic (FBTCs) in two, infantile spasms (IS) in one, and drug resistant was found in three. In Group III, six showed polymicrogyria and one schizencephaly: DD/ID was found severe in five, moderate in two, and the type of seizure was focal (FS) in five, FBTCS in two, and drug resistance was found in three.
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http://dx.doi.org/10.3390/children8080637DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8392186PMC
July 2021

Electroclinical Features of Epilepsy in Mucopolysaccharidosis III: Outcome Description in a Cohort of 15 Italian Patients.

Front Neurol 2021 19;12:705423. Epub 2021 Jul 19.

Child Neurology and Psychiatry Section, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.

Mucopolysaccharidosis III (Sanfilippo syndromes) types A-D are rare lysosomal storage disorders characterized by heparan sulfate accumulation and neurodegeneration. Patients with MPS III present with developmental stagnation and/or regression, sleep disturbance, and behavioral abnormalities usually in the first years of life. Epilepsy may occur in a proportion of patients during the disease course. However, the progression of epilepsy and EEG changes in MPS III have not been systematically investigated. We report electroclinical features in a cohort of patients with MPS III over a follow-up period ranging from 6.5 to 22 years. Participants include 15 patients (11 females; aged 7-31 years) with MPS III A ( = 7, 47%), MPS III B ( = 5, 34%), MPS III C ( = 2, 13%), and MPS III D ( = 1, 6%). At the time of this study, 8 out of 15 patients (53%) had epilepsy. Epilepsy occurred in patients with advanced disease even in the first decade of life (mean age at onset: 12.1 ± 6.7 years). However, seizure onset may also be associated with abrupt worsening of the neurobehavioral phenotype. The main epilepsy types observed were generalized (four out of eight, 50%), followed by focal (three out of eight, 37%) and combined (two out of eight, 25%) epilepsy and status epilepticus (one out of eight, 12.5%). Seizures were generally controlled by one antiepileptic drug (AED) and most patients (seven out of eight, 87%) were still on therapy after a median follow-up period of 5 years (range: 1-9 years). A total of 66 EEGs were analyzed with a median EEG follow-up duration of 7 years (range: 6 months-14 years). Slowing of the background activity occurred in 7 (46%) patients aged 4-19 years. Epileptiform EEG abnormalities were observed in 10 patients at a mean age of 9.6 ± 2.9 years. EEG epileptiform discharges were not unavoidably linked to epilepsy. Early recognition and careful monitoring of electroclinical features in MPS III is necessary for appropriate care and for the detection of disease progression.
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http://dx.doi.org/10.3389/fneur.2021.705423DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8326392PMC
July 2021

On clinical findings of Bickerstaff's brainstem encephalitis in childhood.

J Integr Neurosci 2021 Jun;20(2):509-513

Rheumatology Operative Unit, University Hospital "Policlinico-San Marco", 95123 Catania, Italy.

A short review on the clinical presentation of pediatrics cases of Bickerstaff brain encephalitis emphasizing the broad clinical spectrum of the disease. Cases of pediatric Bickerstaff's brainstem encephalitis collected on three electronic medical databases (PubMed, Cochrane Library and Scopus Web of Science) are reviewed. The inclusion criteria of the cases were based on the clinical characteristics of the disorder in the pediatric age. We reviewed 20 articles on Bickerstaff's brainstem encephalitis, identifying 40 pediatric cases focused on the clinical symptoms. We saw that the prevalence was higher in male subjects, and the median age at diagnosis was 8 years. The phenotype of pediatrics patients was similar to previously published literature. We identify three cases of overlapping forms between Bickerstaff brain encephalitis and Guillain-Barré Syndrome in patients with lower limbs weakness and typical signs of Bickerstaff brain encephalitis, suggesting a combined involvement of the central and peripheral nervous system. Although there is no defined data on incidence and prevalence in the literature, Bickerstaff's brainstem encephalitis appears to be a rare disorder, especially in children. The incidence of Bickerstaff brain encephalitis and Guillain-Barré Syndrome, and Miller Fisher Syndrome has been underrated in the past, primarily due to an underestimation of the forms with a Peripheral Nervous System involvement. Bickerstaff brain encephalitis usually has a rapid and acute onset within 2-4 weeks, characterized by a typical picture of ophthalmoplegia, hyperreflexia, cerebellar symptoms as ataxia. The subsequent manifestations of hyperreflexia or consciousness disturbances as drowsiness, sleepiness, or coma, indicative of central involvement, suggest a Bickerstaff brain encephalitis clinical diagnosis.
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http://dx.doi.org/10.31083/j.jin2002054DOI Listing
June 2021

Non-Epileptic Paroxysmal Events: Clinical features and diagnostic differences with epileptic seizures. A Single Tertiary Centre Study.

Clin Neurol Neurosurg 2021 08 8;207:106739. Epub 2021 Jun 8.

Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy. Electronic address:

Background: Non-Epileptic Paroxysmal Events (NEPE) are common clinical manifestations in pediatric age presenting with dysfunction of motor and behavioral activity mimicking features of epileptic seizures.

Objective: To present and analyze number and clinical characteristic of a group of children/adolescents presenting with various types of NEPE; to compare clinical data of this group of NEPE affected children/adolescents with a group of children/adolescents affected by Epileptic Seizures (ES).

Methods: The retrospective study was conducted at the Pediatric Clinic of University of Catania, Catania, Italy, in a period ranging from January 2005 and January 2018. Two groups of children/adolescents, aged from 1 month to 15 years, were selected: 312 affected by NEPE and 192 by ES. Number and percentage of the single type of NEPE were reported. Then, demographic characteristics, clinical manifestations, duration of the events, time of diagnosis, and age of onset of each type of NEPE and ES affected children/adolescents were analyzed and compared. Results of statistical analysis of the data were carried out between ES and some type of NEPEs including Sandifer syndrome, breath-holding spells, paroxysmal tremors, vertigo, and syncope.

Results: Among the group of NEPE, vertigo, type of paroxysmal event clinically not classifiable, syncope, and Sandifer syndrome were the most common events; In the comparative analyzed samples, variability between NEPE and ES was found in the duration of the paroxysmal events, in number of episodes, in lag-time between the onset of symptoms and the diagnosis, and in age of onset. Analyzing clinical data of ES with some type of NEPE, statistical significant results were obtained in vertigo as regards the duration and average duration event, in paroxysmal tremors as number of events, in Sandifer syndrome as lag-time of diagnosis, and finally in all the types of NEPE as regards the age of onset, and loss of consciousness.

Conclusions: Analyzing the clinical features of each type of NEPE differences with ES are found. However, globally considered diagnostic differences between NEPE and ES remain difficult, questionable, and unrealizable without the support of correct parental report, direct clinical observations, and video-EEG monitoring.
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http://dx.doi.org/10.1016/j.clineuro.2021.106739DOI Listing
August 2021

The process of EUSOMA certification improves the quality of breast cancer care: the experience of an Italian EUSOMA certificated Breast Unit (BU) according to the results of a cross-sectional study.

Recenti Prog Med 2021 Jun;112(6):458-464

Unit of Medical Oncology, Brest Unit located at the Mater Salutis Hospital in Legnago, Italy.

Introduction: The adherence to recognized guidelines and the constant monitoring of performance throughout quality indicators (QIs) are strategic tools to improve the quality of care. The study is aimed to assess the effect of the EUSOMA (European Society of Breast Cancer Specialists) certification process on the quality of breast cancer care of an EUSOMA certified Breast Unit (BU) of Northern Italy.

Materials And Methods: Seventeen mandatory and recommended EUSOMA QIs, based on 594, were analysed for the years 2015-2018. Univariate logistic regression models were performed to compare QIs performance in the years before and after obtaining the EUSOMA certification (2015-6 vs. 2017-8).

Results: Compared to the years 2015-6, the second period of BU activity showed a higher number of QIs achieving both the minimum standard (15 vs. 11) and the 100% of completeness (6 vs. 1). There was a significant improvement of the two QIs evaluating the proportion of Ductal Carcinoma in situ receiving just an operation (from 76% to 95.2%; p=0.033) and the completeness of the prognostic characterisation of invasive cancers (from 94.6% to 99.5%; p=0.022). Conversely, the QI related to the endocrine-sensitive invasive carcinoma receiving adjuvant hormonal therapy dropped from 92.1% to 85.9% (p=0.042) and was significantly lower for patients over 74 compared to those aged ≤54 (73.8% vs. 94.7%; p<0.0001 Fisher's exact test).

Conclusions: The EUSOMA certification process enhanced the clinical practice, promoting a tailored-patient primary systemic or adjuvant therapy and avoiding unnecessary invasive surgical and local-regional treatments.
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http://dx.doi.org/10.1701/3620.36029DOI Listing
June 2021

Characteristics and outcome of BRCA mutated epithelial ovarian cancer patients in Italy: A retrospective multicenter study (MITO 21).

Gynecol Oncol 2021 06 20;161(3):755-761. Epub 2021 Apr 20.

U.O.C. Oncologia, AULSS3 Mirano Hospital, via Don Giacobbe Sartor 4, 30035 Mirano, Venice, Italy. Electronic address:

Objective: Around 15% of epithelial ovarian cancer (EOC) patients (pts) harbor a germline BRCA1 or 2 mutation, showing different features than BRCA wild-type pts. The clinical and pathological features of an Italian BRCA mutated EOC cohort were described.

Methods: We retrospectively analyzed clinical, pathological and mutational data from a cohort of Italian BRCA mutated EOC pts. treated in 15 MITO centers between 1995 and 2017.

Results: Three-hundred thirty-one pts. were recorded. Two-hundred forty (72%) and 91 (27.5%) pts. harbored a BRCA1 and BRCA2 mutation, respectively. Median age at diagnosis was 52 years. The most frequent diagnosis was a high grade serous FIGO III or IV EOC and platinum doublet in first-line was administered to almost all pts. Fifty-three % of them had no residual disease (R = 0) at surgery. Median progression-free-survival (mPFS) after first-line chemotherapy was 29 months. Expected percentage of pts. alive at 5 years was 72.5% (CI 60.2-80.8%) and R = 0 predicted a significantly longer overall survival (OS). Sixty-six pts. (19,9%) had both an EOC and a breast cancer (BC) diagnosis. The first diagnosis was BC in 81,8% of cases with a mean interval between the two diagnoses (IBTDs) of 132.4 months. Mutational data show that the founder mutation c.5266dupC in BRCA1 was the most frequently recorded.

Conclusions: This is the largest Italian BRCA mutEOC cohort. The only predictor of longer OS was R = 0. EOC pts. that developed subsequently a BC are long-term survivors.
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http://dx.doi.org/10.1016/j.ygyno.2021.04.014DOI Listing
June 2021

Second-line Eribulin in Triple Negative Metastatic Breast Cancer patients. Multicentre Retrospective Study: The TETRIS Trial.

Int J Med Sci 2021 27;18(10):2245-2250. Epub 2021 Mar 27.

Bio-Statistics Unit, IRCCS Regina Elena National Cancer Institute, Rome, Italy.

Large and consistent evidence supports the use of eribulin mesylate in clinical practice in third or later line treatment of metastatic triple negative breast cancer (mTNBC). Conversely, there is paucity of data on eribulin efficacy in second line treatment. We investigated outcomes of 44 mTNBC patients treated from 2013 through 2019 with second line eribulin mesylate in a multicentre retrospective study involving 14 Italian oncologic centres. Median age was 51 years, with 11.4% of these patients being metastatic at diagnosis. Median overall survival (OS) and progression free survival (PFS) from eribulin starting were 11.9 (95%CI: 8.4-15.5) and 3.5 months (95%CI: 1.7-5.3), respectively. We observed 8 (18.2%) partial responses and 10 (22.7%) patients had stable disease as best response. A longer PFS on previous first line treatment predicted a better OS (HR=0.87, 95%CI: 0.77-0.99, p= 0.038) and a longer PFS on eribulin treatment (HR=0.92, 95%CI: 0.85-0.98, p=0.018). Progression free survival to eribulin was also favorably influenced by prior adjuvant chemotherapy (HR=0.44, 95%CI: 0.22-0.88, p=0.02). Eribulin was generally well tolerated, with grade 3-4 adverse events being recorded in 15.9% of patients. The outcomes described for our cohort are consistent with those reported in the pivotal Study301 and subsequent observational studies. Further data from adequately-sized, ad hoc trials on eribulin use in second line for mTNBC are warranted to confirm our findings.
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http://dx.doi.org/10.7150/ijms.54996DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8040412PMC
November 2021

Psychological impact of Covid-19 pandemic on oncological patients: A survey in Northern Italy.

PLoS One 2021 16;16(3):e0248714. Epub 2021 Mar 16.

Department of Medical Oncology, AULSS 9 Scaligera, Verona, Italy.

The psychological impact of the Covid 19 pandemic on cancer patients, a population at higher risk of fatal consequences if infected, has been only rarely evaluated. This study was conducted at the Departments of Oncology of four hospitals located in the Verona area in Italy to investigate the psychological consequences of the pandemic on cancer patients under active anticancer treatments. A 13-item ad hoc questionnaire to evaluate the psychological status of patients before and during the pandemic was administered to 474 consecutive subjects in the time frame between April 27th and June 7th 2020. Among the 13 questions, 7 were considered appropriate to elaborate an Emotional Vulnerability Index (EVI) that allows to separate the population in two groups (low versus high emotional vulnerability) according to observed median values. During the emergency period, the feeling of high vulnerability was found in 246 patients (53%) and was significantly associated with the following clinical variables: female gender, being under chemotherapy treatment, age ≤ 65 years. Compared to the pre-pandemic phase, the feeling of vulnerability was increased in 41 patients (9%), remained stably high in 196 (42%) and, surprisingly, was reduced in 10 patients (2%). Overall, in a population characterized by an high level of emotional vulnerability the pandemic had a marginal impact and only a small proportion of patients reported an increase of their emotional vulnerability.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0248714PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7963060PMC
March 2021

Vitamin B12 Deficiency and West Syndrome: An Uncommon but Preventable Cause of Neurological Disorder. Report on Three Cases, One of Them with Late Onset during Vitamin B12 Treatment.

Neuropediatrics 2021 08 3;52(4):333-336. Epub 2021 Mar 3.

Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P Garrahan" Buenos Aires, Buenos Aires, Argentina.

Vitamin B12 is a water-soluble vitamin that plays a fundamental role as an essential cofactor for two enzymes responsible for the production of succinyl-CoA and methionine. Vitamin B12 deficiency can occur in infants and may be related to the breastfeeding mother's adherence to a vegan diet or somatic diseases in the mother. It should be differentiated from inborn errors of vitamin B12 metabolism. Herein, we report the cases of three infants with West syndrome; all three were breastfed by mothers who followed a strict vegan diet. In one of the three infants, West syndrome developed during treatment with vitamin B12 and normalization of the vitamin B12 level. Early treatment and replacement therapy are worthwhile to prevent serious neurological problems and to improve the patient's clinical course.
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http://dx.doi.org/10.1055/s-0041-1725013DOI Listing
August 2021

Secondary Scoliosis as a Complication of Acute Transverse Myelitis in a Child.

J Funct Morphol Kinesiol 2020 Jun 9;5(2). Epub 2020 Jun 9.

Department of Orthopaedics and Traumatologic Surgery, AOU Policlinico-Vittorio Emanuele, University of Catania, Via Santa Sofia, 78, 95123 Catania, Italy.

Acute transverse myelitis (ATM) is a rare neurological condition that affects the spinal cord. Several events, including infections, autoimmune conditions, inflammatory, and drug-induced factors, may cause this disorder. Correct and rapid etiological diagnosis is necessary in order to start appropriate treatment that mainly consists of immunomodulating therapy, high dose intravenous corticosteroids, and in plasma exchange in noninfectious cases. The outcome is varied and depends on several factors. In children, the prognosis is usually good. We report a case of an 11-year-old boy who presented with interscapular pain, right leg steppage, homolateral hyposthenia of the upper limb, and signs of autonomic dysfunction. After performing specific and instrumental exams, a diagnosis of transverse myelitis was reached, and appropriate therapy was performed. A few days post-treatment, the child developed a secondary scoliosis, involving a thoracolumbar curve with loss of cervical and lumbar lordosis. After rehabilitative treatment was undertaken for 12 months, a complete recovery and normal restoration of spinal physiological curves was obtained. The pediatric cases of ATM have a good response to steroid therapy combined with physiotherapy. Collaboration among the various specialists is worthwhile, in order to lead to a correct and rapid diagnosis.
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http://dx.doi.org/10.3390/jfmk5020039DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7739325PMC
June 2020

Cerebral Palsy and Epilepsy in Children: Clinical Perspectives on a Common Comorbidity.

Children (Basel) 2020 Dec 31;8(1). Epub 2020 Dec 31.

Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, 95123 Catania, Italy.

Cerebral palsy (CP) is a frequent cause of childhood disability often associated with a complex group of disorders, including epilepsy, which is reported to impact approximately 40% of affected individuals. This retrospective study involved a group of children affected by CP, some of whom also had comorbid epilepsy. The aim of this study was to report our experience of analyzing, in particular, (a) some of the clinical aspects of the different type of CP, and (b) the relationship between the clinical data of children affected by CP plus epilepsy and each type of CP.

Methods: This retrospective single-center study was performed with 93 children admitted to the Pediatric Department of the University of Catania, Italy, affected by CP and distinguished according to the type of motor clinical presentation, with 46 showing epileptic seizures, compared to a control group of 136 children affected by epilepsy without other neurologic disorders.

Results: Among the 93 CP children, 25 (27%) had spastic quadriplegia (plus one patient with dystonic quadriplegia), 39 (42%) had spastic hemiplegia, 11 (12%) had spastic diplegia (plus two with ataxia and one with dyskinetic CP), and 14 (15%) did not have a well-defined type of CP. The frequency of epilepsy was higher in affected CP children who showed major motor dysfunction (GMFCS IV-V types). As regards the 46 children with CP plus epilepsy, compared to the group of the control, the age of epilepsy onset was found to be statistically significant: 21 ± 35.1 months vs. 67 ± 39.7.

Conclusions: Epilepsy represents one of the most frequent comorbidities of cerebral palsy. In children with CP, particular attention should be paid to the early identification and treatment of comorbid epilepsy.
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http://dx.doi.org/10.3390/children8010016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7823826PMC
December 2020

Inflammatory indexes as predictive factors for platinum sensitivity and as prognostic factors in recurrent epithelial ovarian cancer patients: a MITO24 retrospective study.

Sci Rep 2020 10 23;10(1):18190. Epub 2020 Oct 23.

Medical Oncology Department, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy.

Neutrophil-to-lymphocyte ratio (NLR) and systemic inflammatory index (SII) are prognostic factors in epithelial ovarian cancer (EOC). Their predictive value for platinum-sensitivity and their role in recurrent EOC are unknown. A total of 375 EOC patients were retrospectively analyzed. The correlation between baseline NLR and SII, and platinum-free interval (PFI) according to first line bevacizumab treatment were analyzed using logistic regression analyses adjusted for baseline patient characteristics. Subsequently NLR and SII calculated before second line treatment initiation were evaluated to identify a potential correlation with progression-free survival (PFS) and overall survival (OS) in platinum-sensitive and in platinum-resistant population. In multivariate analysis, NLR ≥ 3 is an independent predictive factor for PFI at 6 months in the chemotherapy group (OR = 2.77, 95% CI 1.38-5.56, p = 0.004), not in bevacizumab treated patients. After having adjusted for ECOG performance status, histology, ascites, bevacizumab treatment at second line and BRCA status, NLR ≥ 3 and SII ≥ 730 are significantly associated with worse OS in platinum-sensitive (HR = 2.69, 95% CI 1.60-4.53, p = 0.002; HR = 2.11, 95% CI 1.29-3.43, p = 0.003, respectively), not in platinum-resistant EOC patients. Low NLR is an independent predictive factor for platinum-sensitivity in patients treated without bevacizumab. NLR and SII are prognostic factors in recurrent platinum-sensitive EOC patients.
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http://dx.doi.org/10.1038/s41598-020-75316-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7585431PMC
October 2020

Poland Syndrome with Atypical Malformations Associated to a de novo 1.5 Mb Xp22.31 Duplication.

Neuropediatrics 2020 10 3;51(5):359-363. Epub 2020 Feb 3.

Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, Catania, CT, Italy.

Poland's syndrome (PS; OMIM 173800) is a rare congenital syndrome which consists of absence or hypoplasia of the pectoralis muscle. Other features can be variably associated, including rib defects. On the affected side other features (such as of breast and nipple anomalies, lack of subcutaneous tissue and skin annexes, hand anomalies, visceral, and vertebral malformation) have been variably documented. To date, association of PS with central nervous system malformation has been rarely reported remaining poorly understood and characterized. We report a left-sided PS patient carrying a de novo 1.5 Mb Xp22.31 duplication diagnosed in addiction to strabismus, optic nerves and chiasm hypoplasia, corpus callosum abnormalities, ectopic neurohypophysis, pyelic ectasia, and neurodevelopmental delay. Since, to our knowledge, this features' association has not been previously reported, we argue that this case may contribute to further widening of the variability of PS phenotype.
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http://dx.doi.org/10.1055/s-0039-3402009DOI Listing
October 2020

Grisel syndrome, sigmoid sinus thrombosis and rheumatic carditis: Case report of a rare association.

Int J Pediatr Otorhinolaryngol 2020 Feb 9;129:109774. Epub 2019 Nov 9.

Pediatric Clinic, Departmente of Clinical and Experimental Medicine, University of Catania, Via Santa Sofia 78, 95123, Catania, Italy.

Grisel's syndrome (GS) is a non traumatic atlanto-axial rotatory subluxation of C1-C2 joint. A six year old girl, 20 days after an episode of fever, developed a torticollis and a 3/6 heart murmur. The echocardiography showed a Rheumatic Carditis. The Brain and cervical spine Magnetic resonance imaging (MRI) and the Computerized Tomography (CT) showed rotary dislocation of C1-C2 vertebrae, compatible with GS, and cerebral venous thrombosis (CVT). An antibiotic therapy, Prednisone and a low molecular weight heparin for 7 days was prescribedfollowed by an oral anticoagulant for 6 months. After a month the MRI showed a reduction of the dislocation and cerebral venous recanalization.
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http://dx.doi.org/10.1016/j.ijporl.2019.109774DOI Listing
February 2020

Neuroaspergillosis as the Presenting Sign of a Chronic Granulomatous Disease.

Pediatr Neurol 2020 01 24;102:79-80. Epub 2019 Aug 24.

Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.08.008DOI Listing
January 2020

Focal Neuropathy Mimicking Focal Dystonia in a Child: Diagnostic and Rehabilitative Tools.

J Funct Morphol Kinesiol 2019 Aug 5;4(3). Epub 2019 Aug 5.

Department of Biomedical and Biotechnological Sciences, University of Catania-U.O. Rehabilitation A.O.U. "Policlinico-Vittorio Emanuele", 95123 Catania, Italy.

Object: Focal neuropathy results from an injury of any etiology that occurs in a peripheral nerve. The lesion may be followed by alteration of the sensory sphere (either dysesthesia or paresthesia with or without neuropathic pain), or by compensatory attitudes that are attributable to the altered contraction in muscles that are innervated by the injured nerve.

Methods: We describe the case of a 13-year-old boy who attended our hospital for a focal neuropathy of the radial nerve.

Conclusion: This neuropathy was revealed after the removal of a plaster Zimmer splint that was applied following a post-traumatic subluxation of the metacarpal-trapezoid joint.
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http://dx.doi.org/10.3390/jfmk4030054DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7739221PMC
August 2019

Optic Neuropathy, Secondary to Ethmoiditis, and Onodi Cell Inflammation during Childhood: A Case Report and Review of the Literature.

Neuropediatrics 2019 12 22;50(6):341-345. Epub 2019 Jul 22.

ENT Unit, Department of Otorhinolaryngology, University of Catania, Italy.

Optic neuropathy consists of several etiological events. The primary etiologies of its acute form include optic neuritis, ischemic optic neuropathy, inflammatory (nondemyelinating) disorders, and trauma. Its subacute and chronic forms are most often linked to compressive, toxic, nutritional, or hereditary-genetic causes. Visual loss, dyschromatopsia, and visual field defects are the presenting symptoms. The Onodi cell (sphenoethmoidal air cell) is an anatomic variant located laterally and superior to the sphenoid sinus; it is closely related to the optic nerve. Onodi cell disorders are rare and may be unnoticed in differential diagnoses of patients with ocular and neurological manifestations. Here, we present the case of a 12-year-old boy with headache and acute loss of sight characterized by hemianopsia in the left eye and retrobulbar optic neuropathy caused by left sphenoethmoidal sinusitis with the presence of Onodi cell inflammation. The diagnosis was confirmed by multilayered paranasal computed tomography and cerebral magnetic resonance imaging. Therapeutic treatment resulted in gradual improvement: at the 2-week follow-up, the patient no longer had headaches and his visual acuity returned to normal. Inflammation of Onodi cells should be considered in children with headache and abnormal vision.
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http://dx.doi.org/10.1055/s-0039-1693156DOI Listing
December 2019

Distinct HR expression patterns significantly affect the clinical behavior of metastatic HER2+ breast cancer and degree of benefit from novel anti-HER2 agents in the real world setting.

Int J Cancer 2020 04 7;146(7):1917-1929. Epub 2019 Aug 7.

Department of Medical Oncology, Policlinico Universitario "A. Gemelli", Rome, Italy.

We analyzed data from 738 HER2-positive metastatic breast cancer (mbc) patients treated with pertuzumab-based regimens and/or T-DM1 at 45 Italian centers. Outcomes were explored in relation to tumor subtype assessed by immunohistochemistry (IHC). The median progression-free survival at first-line (mPFS1) was 12 months. Pertuzumab as first-line conferred longer mPFS1 compared to other first-line treatments (16 vs. 9 months, p = 0.0001), regardless of IHC subtype. Median PFS in second-line (mPFS2) was 7 months, with no difference by IHC subtype, but it was more favorable with T-DM1 compared to other agents (7 vs. 6 months, p = 0.03). There was no PFS2 gain in patients with tumors expressing both hormonal receptors (HRs; p = 0.17), while a trend emerged for tumors with one HR (p = 0.05). Conversely, PFS2 gain was significant in HRs-negative tumors (p = 0.04). Median overall survival (mOS) was 74 months, with no significant differences by IHC subtypes. Survival rates at 2 and 3 years in patients treated with T-DM1 in second-line after pertuzumab were significantly lower compared to pertuzumab-naïve patients (p = 0.01). When analyzed by IHC subtype, the outcome was confirmed if both HRs or no HRs were expressed (p = 0.02 and p = 0.006, respectively). Our results confirm that HRs expression impacts the clinical behavior and novel treatment-related outcomes of HER2-positive tumors when treatment sequences are considered. Moreover, multivariate analysis showed that HRs expression had no effect on PFS and OS. Further studies are warranted to confirm our findings and clarify the interplay between HER2 and estrogen receptor pathways in HER2-positive (mbc) patients.
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http://dx.doi.org/10.1002/ijc.32583DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7027476PMC
April 2020

Multiseptate Gallbladder in a Child: A Possible Cause of Poor Growth?

J Pediatr Gastroenterol Nutr 2019 Jan;68(1):e13

Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania.

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http://dx.doi.org/10.1097/MPG.0000000000001664DOI Listing
January 2019

Diagnostic management of occult nodal lymphangioleiomyomatosis detected during pelvic cancer staging. Localized finding or systemic disease?

Sarcoidosis Vasc Diffuse Lung Dis 2019 1;36(1):33-38. Epub 2019 May 1.

Pneumology Unit, Hospital "Mater Salutis", ULSS9 Scaligera, Legnago (VR), Italy.

Background: Lymphangioleiomyomatosis (LAM) is a neoplastic disease that generally arises in the lung (pLAM) and may be associated with "Tuberous sclerosis complex" (TSC). Occasionally, LAM can arise at the extrapulmonary sites (eLAM), such as the mediastinum, the retroperitoneum or the lymph nodes. 25-30% of the patients affected by pLAM develop eLAM. In asymptomatic patients, the presence of mediastinal and retroperitoneal eLAM preceded that of pLAM by usually 1-2 years. Nevertheless, some authors reported that the nodal eLAM, detected during pelvic cancer staging, arise in patents without pLAM and/or TSC. In this paper we review the Literature of this rare condition suggesting its diagnostic management.

Results: To date, it has been reported 30 cases. The mean age at diagnosis is 55 years and around 30% of patients are postmenopausal. In only 2 cases was diagnosed a following p-LAM. One patient with endometrioid carcinoma and pelvic nodal eLAM reported germiline mutation. None case was associated with both p-LAM and TSC.

Conclusions: The retrospective probability to have p-LAM in patients with staging pelvic nodal e-LAM is 6,6% (4/30) lower than the probability to have e-LAM in patients affected by p-LAM (25-30%). In both this association is more probable sporadically than associated with TSC. The association between cancer staging pelvic nodal e-LAM and TSC is low (3%; 1/30). The p-LAM developed are asymptomatic with a behavior, regardless of hormonal status, similar to lesions diagnosed in postmenopausal although further studies are mandatory to confirm it.
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http://dx.doi.org/10.36141/svdld.v36i1.7110DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7247108PMC
June 2020

Colonic Ewing Sarcoma/PNET associated with liver metastases: A systematic review and case report.

Pathol Res Pract 2019 Feb 29;215(2):387-391. Epub 2018 Nov 29.

Mater Salutis" Hospital, ULSS9, 37045 Legnago, Verona, Italy. Electronic address:

Ewing Sarcoma is a highly lethal undifferentiated tumor of bone. ES is a small round cell tumor with etiological and characteristic chromosomal translocations between TET/FET (TLS/FUS, EWSR1, and TAF15) and ETS (E26 transformation-specific) family genes. Generally, therapeutic approach for metastatic Ewing Sarcoma includes both local (surgery and radiotherapy) and systemic (chemotherapy) disease control with an overall cure rate of 20%. For extra-osseous tumors, the most common primary sites of disease are trunk, extremities, head and neck, retroperitoneum. Among other sites, Ewing Sarcoma/PNET may also rarely arise in colon and rectum. Even if colonic Ewing Sarcoma/PNET have been previously reported in 5 cases, none of those reports came from right side of the colon. In this article, we report the first case of right-sided Ewing Sarcoma with synchronous liver metastases completely responding to first line chemotherapy. Furthermore, we provide a systematic qualitative review of the current literature on adult colorectal Ewing Sarcoma using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA).
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http://dx.doi.org/10.1016/j.prp.2018.11.021DOI Listing
February 2019

Arterial ischemic stroke (AIS) in childhood: clinical report from a single control center.

Childs Nerv Syst 2019 02 12;35(2):283-293. Epub 2018 Dec 12.

Department of Pediatrics, AOU Policlinico-Vittorio Emanuele, University of Catania, via S. Sofia 78, 95123, Catania, Italy.

Introduction: Stroke is the clinical designation for a rapidly developing loss of brain function due to an interruption in the blood supply to all or part of the brain. It is the third cause of death in adults and one of the top 10 causes in pediatric age. The perinatal period of onset is the second only to adult age group in the incidence of stroke. Arterial ischemic stroke during childhood occurs most frequently in the perinatal period with an incidence of 1 out 2300-5000 live infant births.

Materials And Methods: This is a retrospective study that includes 28 patients affected by perinatal arterial ischemic stroke. Family and gestational history, risk factors of perinatal stroke, gender and clinical data of affected children and outcome are reported.

Results: A stroke family history was registered in three unrelated families. Gestational history disclosed cases of threats of abortion, preterm delivery, hyperthermia, gestosis, and placental disorders. In the children, onset of seizures were reported within 3 days of life and diagnosis of stroke was confirmed by brain MRI which disclosed involvement of the middle cerebral artery in all the cases. Hemilateral cerebral palsy, epileptic seizures, and intellectual disability from mild to severe were the most frequent complications.

Conclusion: Stroke is still a common and dreadful events in perinatal period as this disorder is often unpredictable and cause of severe neurological impairment.
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http://dx.doi.org/10.1007/s00381-018-4017-1DOI Listing
February 2019

Palbociclib plus endocrine therapy in HER2 negative, hormonal receptor-positive, advanced breast cancer: A real-world experience.

J Cell Physiol 2019 06 10;234(6):7708-7717. Epub 2018 Dec 10.

Division of Medical Oncology, Ospedale S. Maria Goretti, Latina, Italy.

Data from 423 human epidermal growth factor receptor 2-negative (HER2-), hormone receptor-positive (HR+) advanced breast cancer (aBC) patients treated with palbociclib and endocrine therapy (ET) were provided by 35 Italian cancer centers and analyzed for treatment outcomes. Overall, 158 patients were treated in first line and 265 in second/later lines. We observed 19 complete responses and 112 partial responses. The overall response rate (ORR) was 31% (95% confidence interval [CI], 26.6-35.4) and clinical benefit was 52.7% (95% CI, 48-57.5). ORR was negatively affected by prior exposure to everolimus/exemestane ( p = 0.002) and favorably influenced by early line-treatment ( p < 0.0001). At 6 months, median progression-free survival was 12 months (95% CI, 8-16) and median overall survival was 24 months (95% CI, 17-30). More favorable outcomes were associated with palbociclib in early lines, no visceral metastases and no prior everolimus/exemestane. The main toxicity reported was neutropenia. Our results provide further support to the use of palbociclib with ET in HER2-, HR+ aBC. Differences in outcomes across patients subsets remain largely unexplained.
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http://dx.doi.org/10.1002/jcp.27832DOI Listing
June 2019

Pott Disease in a 14-year-old Girl Affected by Congenital Lamellar Ichthyosis Type 3 and Diabetes Mellitus.

J Glob Infect Dis 2018 Jul-Sep;10(3):166-168

Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.

Extrapulmonary manifestations of tuberculosis (TB) are particularly frequent during childhood, and usually involve the lymph nodes and the skull. They are related to predisposing immunosuppression conditions. A patient affected by diabetes mellitus type 1 (DMT1) and congenital lamellar ichthyosis type 3 came at our attention with a 4-year history of recurrent parotitis and severe back pain and inferior limb hypomobility, which had lasted for 6 months. A diagnosis of chronic TB parotitis combined with Pott disease was performed after a suggestive spinal magnetic resonance imaging, and positive culture and polymerase-chain reaction examination. Surgical aspiration of the fluid collection and a 12-month antitubercular treatment resulted in complete resolution of the symptomatology. This is the first report of a Pott disease in a patient affected by the two co-occurrences of two immunosuppression diseases such as DMT1 and congenital lamellar ichthyosis type 3.
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http://dx.doi.org/10.4103/jgid.jgid_82_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6100339PMC
September 2018

Inflammatory Indexes as Prognostic and Predictive Factors in Ovarian Cancer Treated with Chemotherapy Alone or Together with Bevacizumab. A Multicenter, Retrospective Analysis by the MITO Group (MITO 24).

Target Oncol 2018 08;13(4):469-479

Department of Medical Oncology, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy.

Background: The variability in progression-free survival (PFS) and overall survival (OS) among patients with epithelial ovarian cancer (EOC) makes it difficult to reliably predict outcomes. A predictive biomarker of bevacizumab efficacy as first-line therapy in EOC is still lacking.

Objective: The MITO group conducted a multicenter, retrospective study (MITO 24) to investigate the role of inflammatory indexes as prognostic factors and predictors of treatment efficacy in FIGO stage III-IV EOC patients treated with first-line chemotherapy alone or in combination with bevacizumab.

Patients And Methods: Of the 375 patients recruited, 301 received chemotherapy alone and 74 received chemotherapy with bevacizumab. The pre-treatment neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and systemic immune inflammation index (SII) were evaluated to identify a potential correlation with PFS and OS in both the overall population and the two treatment arms.

Results: In the overall population, the PFS and OS were significantly longer in patients with low inflammatory indexes (p < 0.0001). In multivariate analyses, the NLR was significantly associated with OS (p = 0.016), and the PLR was significantly associated with PFS (p = 0.024). Inflammatory indexes were significantly correlated with patient prognosis in the chemotherapy-alone group (p < 0.0001). Patients in the chemotherapy with bevacizumab group with a high NLR had a higher PFS and OS (p = 0.026 and p = 0.029, respectively) than those in the chemotherapy-alone group. Conversely, PFS and OS were significantly poorer in patients with a high SII (p = 0.024 and p = 0.017, respectively).

Conclusion: Our results suggest that bevacizumab improves clinical outcome in patients with a high NLR but may be detrimental in those with a high SII.
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http://dx.doi.org/10.1007/s11523-018-0574-1DOI Listing
August 2018

Hypoglossal nerve paralysis in a child after a dental procedure.

Neurol Neurochir Pol 2018 May - Jun;52(3):406-409. Epub 2018 Feb 6.

Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy; Maurice Wohl Clinical Neuroscience Institute, King's College London, London, UK. Electronic address:

Unilateral palsy of the hypoglossal nerve is a rare complication of orthodontic procedures. The main reported causes of HNP are: orthopedic and otorhinolaryngology surgical interventions, and in particular maneuvers involving compression or overstretching of the hypoglossal nerve, dental procedures and traumas, and also infections, motoneuron disorders, tumors, vascular diseases. Diagnosis is usually performed by electrophysiology studies (EMG-VCN), and brain magnetic resonance imaging (MRI) is useful to exclude other causes. The prognosis depends on the location and extension of the damage. Currently there is not a standardized treatment approach except the speech therapy, although, in some cases, the high-dose steroid treatment could be useful. We describe the case of a ten-year-old female, who was admitted in our Unit after a deviation of the tongue associated with dysarthria and dysphagia, occurred after the application of a mobile orthodontic device.
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http://dx.doi.org/10.1016/j.pjnns.2018.01.006DOI Listing
May 2019

A retrospective multicentric observational study of trastuzumab emtansine in HER2 positive metastatic breast cancer: a real-world experience.

Oncotarget 2017 08 25;8(34):56921-56931. Epub 2017 May 25.

Department of Medico-Surgical Sciences and Biotechnologies, "Sapienza" University of Rome, Oncology Unit, Istituto Chirurgico Ortopedico Traumatologico, Latina, Italy.

We addressed trastuzumab emtansine (T-DM1) efficacy in HER2+ metastatic breast cancer patients treated in real-world practice, and its activity in pertuzumab-pretreated patients. We conducted a retrospective, observational study involving 23 cancer centres, and 250 patients. Survival data were analyzed by Kaplan Meier curves and log rank test. Factors testing significant in univariate analysis were tested in multivariate models. Median follow-up was 15 months and median T-DM1 treatment-length 4 months. Response rate was 41.6%, clinical benefit 60.9%. Median progression-free and median overall survival were 6 and 20 months, respectively. Overall, no differences emerged by pertuzumab pretreatment, with median progression-free and median overall survival of 4 and 17 months in pertuzumab-pretreated (p=0.13), and 6 and 22 months in pertuzumab-naïve patients (p=0.27). Patients who received second-line T-DM1 had median progression-free and median overall survival of 3 and 12 months (p=0.0001) if pertuzumab-pretreated, and 8 and 26 months if pertuzumab-naïve (p=0.06). In contrast, in third-line and beyond, median progression-free and median overall survival were 16 and 18 months in pertuzumab-pretreated (p=0.05) and 6 and 17 months in pertuzumab-naïve patients (p=0.30). In multivariate analysis, lower ECOG performance status was associated with progression-free survival benefit (p<0.0001), while overall survival was positively affected by lower ECOG PS (p<0.0001), absence of brain metastases (p 0.05), and clinical benefit (p<0.0001). Our results are comparable with those from randomized trials. Further studies are warranted to confirm and interpret our data on apparently lower T-DM1 efficacy when given as second-line treatment after pertuzumab, and on the optimal sequence order.
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http://dx.doi.org/10.18632/oncotarget.18176DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5593613PMC
August 2017

Acute diplopia in the pediatric Emergency Department. A cohort multicenter Italian study.

Eur J Paediatr Neurol 2017 Sep 3;21(5):722-729. Epub 2017 Jun 3.

Pediatric Emergency Department, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Background: Acute diplopia (AD) is an uncommon and distressing symptom of numerous ocular and neurological conditions, with potentially serious sequelaes. No data are present in pediatrics on the presentation and management of AD.

Aim: This study investigated characteristics, etiology and health care utilization of the pediatric population with AD accessed to pediatric Emergency Departments (ED), trying to identify "red flags" associated with potentially life-threatening (LT) conditions.

Methods: We conducted a cohort multicenter study on children with AD in ten Italian hospitals. Patients were classified into diagnostic categories, comparing children with and without LT disease.

Results: 621 children presented AD at a rate of 3.6 per 10.000. The most frequent diagnosis among no-LT conditions (81.2%) were headache, ocular disorders and minor post-traumatic disease, while LT conditions (18.8%) were represented by brain tumors, demyelinating conditions, idiopathic intracranial hypertension and major post-traumatic diseases. The LT group showed a significantly higher age, with the odds increased by 1% for each month of age. Monocular diplopia occurred in 16.1%, but unlike adult one-fifth presented LT conditions. Binocular diplopia, associated ocular manifestations or extraocular neurological signs were significantly more common in the LT group. At regression logistic analysis strabismus and ptosis were associated with LT conditions.

Conclusion: The majority of children presented no-LT conditions and more than one-fourth of patients had headache. Monocular diplopia in the LT group was never isolated but associated with other signs or symptoms. Our study was able to identify some specific ocular disturbances or neurologic signs potentially useful for ED physician to recognize patients with serious pathologies.
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http://dx.doi.org/10.1016/j.ejpn.2017.05.010DOI Listing
September 2017

Acute cerebellitis in children: an eleven year retrospective multicentric study in Italy.

Ital J Pediatr 2017 Jun 12;43(1):54. Epub 2017 Jun 12.

Pediatric and Infectious Diseases Unit, IRCCS Bambino Gesù Children Hospital, Rome, Italy.

Background: Acute cerebellitis (AC) and acute cerebellar ataxia (ACA) are the principal causes of acute cerebellar dysfunction in childhood. Nevertheless. there is no accepted consensus regarding the best management of children with AC/ACA: the aim of the study is both to assess clinical, neuroimaging and electrophysiologic features of children with AC/ACA and to evaluate the correlation between clinical parameters, therapy and outcome.

Methods: A multicentric retrospective study was conducted on children ≤ 18 years old admitted to 12 Italian paediatric hospitals for AC/ACA from 01/01/2003 to 31/12/2013. A score based on both cerebellar and extracerebellar signs/symptoms was computed for each patient. One point was given for each sign/symptom reported. Severity was divided in three classes: low, moderate, severe.

Results: A total of 124 children were included in the study. Of these, 118 children received a final diagnosis of ACA and 6 of AC. The most characteristic finding of AC/ACA was a broad-based gait disturbance. Other common symptoms included balance disturbances, slurred speech, vomiting, headache and fever. Neurological sequelae were reported in 6 cases (5%) There was no correlation among symptoms, cerebrospinal fluid findings, clinical outcome. There was no correlation between clinical manifestations and clinical score on admission and length of hospital stay, sex, age and EEG findings with sequelae (P > 0.05). Children with pathological magnetic resonance imaging (MRI) or computed tomography (CT) had a higher probability of having clinical sequelae. Treatment was decided independently case by case. Patients with a higher clinical score on admission had a higher probability of receiving intravenous steroids.

Conclusions: We confirmed the literature data about the benign course of AC/ACA in most cases but we also highlighted a considerable rate of patients with neurological sequelae (5%). Pathological MRI or CT findings at admission correlate to neurological sequelae. These findings suggest the indication to perform an instrumental evaluation in all patients with AC/ACA at admission to identify those at higher risk of neurological outcome. These patients may benefit from a more aggressive therapeutic strategy and should have a closer follow-up. Randomized controlled trials are needed to confirm these observations. The ultimate goal of these studies could be to develop a standardized protocol on AC/ACA. The MRI/CT data, associated with the clinical manifestations, may allow us to define the class risk of patients for a neurological outcome.
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http://dx.doi.org/10.1186/s13052-017-0370-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5469162PMC
June 2017
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