[Show abstract][Hide abstract] ABSTRACT: The aim of the study was to perform a third cognitive assessment in our pediatric-onset multiple sclerosis (MS) patient cohort and determine predictors of the individual cognitive outcome.
[Show abstract][Hide abstract] ABSTRACT: The relationship between cord arterial pH (CA-pH) >7.000 and the neonatal outcome is not clear.
To evaluate if asymptomatic infants born with unexpected cord arterial pH (CA-pH) between 7.000 and 7.100 develop clinical, biochemical, and instrumental signs of hypoxic cerebral, renal, and heart failure more frequently than symptomatic infants.
Term infants with CA-pH of 7.000-7.100 and appropriate birth weight were prospectively and consecutively enrolled and classified as asymptomatic, when they had no resuscitation, early respiratory distress or early abnormal neurologic signs, and symptomatic infants. Clinical, biochemical, and instrumental signs of hypoxic cerebral, renal, and heart failure were evaluated in the two groups.
A total of 53 infants were enrolled. Twenty-eight (53%) were asymptomatic. CA-pH was similar in both the groups, while the cTnI serum concentration in the first day of life and the occurrence of poor feeding were higher in the symptomatic than in asymptomatic infants. An arterial lactate level of ≥4.1mmol/l measured in the first hour of life was an independent risk factor for the development of a symptomatic course.
In our population the majority of infants born with a CA-pH between 7.000 and 7.100 were asymptomatic and would not have needed immediate admission to the neonatal care unit. Symptomatic infants showed a higher occurrence of subclinical heart injury and poor feeding.
Early human development 09/2013; · 2.12 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Clinical neurophysiology is both an extension of clinical examination and an integration of neuroimaging. It plays a role in diagnosis, prognosis and monitoring in the Intensive Care Unit (ICU). Electroencephalography (EEG) and somatosensory evoked potentials (SEPs) are the most informative neurophysiological tests. Both have a major prognostic role in the hypoxic-ischemic encephalopathy and traumatic brain injury (TBI). In the former the absence of bilateral cortical SEPs has an unfavorable prognostic significance of 100%, whereas bilateral normal SEPs has uncertain prognostic value. In TBI these SEP patterns have high early prognostic value for both bad and good outcome. Continuous EEG monitoring is indicated for diagnosis and treatment of non convulsive seizures and status epilepticus (NCSE), whereas SEPs are more able to indicate the occurrence of neurological deterioration. In our opinion EEG-SEP monitoring is also valuable for interpretation and management of ICP trends, contributing to optimise treatment in a single patient. The EEG seems to have the same prognostic utility in pediatric as in adult ICU. Recent reviews supported the use of SEPs in the integrated process of outcome prediction after acute brain injury in children. However differences in interpretation are needed and the issue is whether it is possible to establish an age limit over which the prediction of SEPs is similar to that in adults. There are only a few studies of seizure prevalence in pediatric ICU. The variability of frequency of NCSE in comatose children is high as in adults and, similar to the adult, remains unclear the impact on outcome.
[Show abstract][Hide abstract] ABSTRACT: OBJECTIVE: Chronic cerebrospinal venous insufficiency (CCSVI) was hypothesized to play a causative role in multiple sclerosis (MS). The assessment of pediatric-onset MS (POMS) may provide a unique window of opportunity to study hypothesized risk factors in close temporal association with the onset of the disease. METHODS: Internal jugular veins, vertebral veins and intracranial veins were evaluated with extracranial and intracranial ultrasound in 15 POMS and 16 healthy controls. Assessor's blinding was maintained during the study. We considered subjects positive to CCSVI when at least two criteria were fulfilled. RESULTS: CCSVI frequency was comparable between POMS and controls (p > 0.05). Clinical features were not significantly different between CCSVI-positive and CCSVI-negative patients. CONCLUSIONS: Our findings add to previous data pointing against a causative role of CCSVI in MS.
[Show abstract][Hide abstract] ABSTRACT: Neonatal encephalopathy is a significant cause of infant mortality and morbidity with risk of neurological sequelae in the survivors of neonates admitted to Neonatal (N) Intensive Care Unit (ICU). The EEG and Evoked Potentials (EPs) are very informative in the ICU. In particular, it is known that the SomatoSensory (SS) EPs are the best single indicator of early prognosis in adults and children patients with traumatic and/or hypoxic-ischemic coma compared to the Glasgow Coma Scale (GCS) and CTscan. Most paediatric studies excluded newborns in an attempt to eliminate the age effects, because of the structural and functional immaturity of somatosensory system. In fact, newborns differ from adults and paediatric patients for many aspects: hypoxic-ischemic aetiology, SSEPs normative data, grading and predictive values, timing and techniques recording, clinical scales of evaluation. Recently a diagnostic and predictive role of early SSEPs has been established in perinatal hypoxic-ischemic. We reported a literature review of early diagnostic/prognostic role of SSEPs and our preliminary neurophysiological data of prospective study in mild or severe perinatal hypoxic-ischemic insult.
The journal of maternal-fetal & neonatal medicine: the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians 08/2011; 24 Suppl 1:69-71. · 1.36 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: There is limited information on fatigue and its clinical and psychosocial correlates in children and adolescents with multiple sclerosis (MS).
To assess the relationships between fatigue, cognitive functioning and depression in paediatric MS.
The study cohort consisted of patients with MS recruited for an Italian collaborative study on cognitive and psychosocial functioning in paediatric MS. The present assessment included evaluation of fatigue on the Pediatric Quality of Life Inventory-Multidimensional Fatigue Scale, cognitive functioning on an extensive neuropsychological battery and depression on the Children's Depression Inventory (CDI). A psychiatric interview through the Kiddie-SADS-Present and Lifetime Version was also administered.
In total, 57 patients with relapsing-remitting MS were compared with 70 healthy controls. Percentages of fatigued patients ranged from 9% to 14% according to self-reports, and from 23% to 39% according to parent reports. Fatigue was significantly related with higher scores on the CDI (p < 0.03). Higher levels of self-reported cognitive fatigue were associated with impaired performance on a problem-solving test, whereas higher levels of parent-reported cognitive fatigue were associated with impairment on tests of verbal learning, processing speed, complex attention and verbal comprehension.
Our data show that fatigue can affect a sizeable proportion of paediatric MS patients, and confirm the association between fatigue and depressive symptoms in MS. They also highlight the difficulties of fatigue assessment in the paediatric population and provide a few clues to further research in the field.
[Show abstract][Hide abstract] ABSTRACT: Approximately, 3-10% of patients with multiple sclerosis (MS) present a disease onset before the age of 18 years. Although growing attention is dedicated to cognitive impairment and its functional consequences in paediatric MS, so far no study has explored possible neurophysiologic correlates. The study's aim was to describe event-related potentials in relationship with cognitive performance in children and adolescents with MS compared with demographically matched healthy controls (HC), providing two-year follow-up data. Six MS subjects aged between 9 and 17 years were assessed through an extensive neuropsychological battery at two time points. Event-related potentials with an odd-ball acoustic paradigm were also recorded in the patients and in nine HC. At baseline, four out of six patients failed three or more cognitive tasks and were classified as cognitively impaired. In all the cognitively impaired patients, we found abnormal latencies and amplitudes of the P300. After 2 years, five patients exhibited a deteriorating cognitive performance and a corresponding deterioration of the P300 parameters. In our group of children and adolescents with MS, changes in P300 parameters proceeded in parallel with deteriorating cognitive performance. P300 might represent an objective parameter to monitor cognitive changes in paediatric MS.
[Show abstract][Hide abstract] ABSTRACT: Antibodies to MOG in serum have a dubious prognostic value in multiple sclerosis. The MOG recombinant protein conformational properties relevant to the antigenic activity are unknown. We employed a solid-phase ELISA based on a product (rMOG(ED)(His)(6)) expressed in E. coli after subcloning the cDNA of the extracellular domain of rat MOG, performing a refolding procedure on column and affinity purification. The far-UV Circular Dichroism (CD) spectra of rMOG(ED)(His)(6) showed a β-sheet, a characteristic feature of the Ig-fold. However, in MS sera and controls we failed to detected IgM or IgG antibodies.
Journal of neuroimmunology 01/2011; 233(1-2):216-20. · 2.84 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To assess the evolution of cognitive and psychosocial functioning in a cohort of childhood and juvenile multiple sclerosis (MS) cases after a mean period of 2 years had elapsed since baseline evaluation.
In this cohort study, we used the same extensive neuropsychological battery with alternative versions of the tests assessing memory, attention/concentration, executive functions, and language. Fatigue and depression were also measured. An interview on school and daily living activities was obtained from the parents. The cognitive performance of the patients was compared with that of demographically matched healthy controls (HC).
Fifty-six patients and 50 HC were assessed. At follow-up, criteria for cognitive impairment (failure on at least 3 tests) were fulfilled in 39 patients (70%) and 75% of the cases were classified as having a deteriorating cognitive performance. Changes were prominent in tests of verbal memory, complex attention, verbal fluency, and receptive language. In the regression analysis, the only significant predictor of cognitive deterioration was older age of the subject (odds ratio 1.9, 95% confidence interval 1.2-2.9, p = 0.003). Psychiatric disorders, most frequently depression, were diagnosed in 12 patients (30.5%). Fatigue was reported by 21% of the patients. MS negatively affected school and everyday activities in 30% to 40% of the subjects.
Our findings confirm the importance of systematic assessment of cognitive and psychosocial issues in children and teens with MS. The progressive nature of the cognitive difficulties emphasizes the need for developing effective treatment strategies.
[Show abstract][Hide abstract] ABSTRACT: To review the predictive powers of SEPs in comatose children after acute brain injury.
MEDLINE, EMBASE, OVID, ISI Web of Knowledge, BIOMED Central and the Cochrane Library (1981-2007) were searched. First, predictive values were calculated for each primary study. Second, we analysed effects of different factors on the SEP diagnostic odds ratio by meta-regression. Third, we compared SEP predictive values in children and in adults.
We selected 14 studies covering 732 patients; analysis was conducted in 11, while the other 3 were used for simple qualitative examination. In individual papers, the presence of SEP predicted favourable outcomes as shown by the area under both sROC curves being 0.958. The same value was shown by SEP absence for predicting unfavourable outcomes. All covariates showed no significant effects on diagnostic accuracy, but only a slight non-significant trend. For SEP grading, a simple sub-group analysis showed a high predictive value for non-awakening for absence of SEPs (PPV 97.0%) and a high prognostic power to predict awakening for normal SEPs (PPV 92.2%). Pathological SEPs did not show reliable predictivity. In children, the presence of SEPs showed a high prognostic power similar to that in adults.
This study supports the use of SEPs in the integrated process of outcome prediction after acute brain injury in children. Caution is recommended in predicting unfavourable outcomes in patients with an absence of SEPs in both TBI and HIE comas. Future studies are needed to resolve the issue of the effect of aetiology and age on SEP's predictive power.
European Journal of Intensive Care Medicine 07/2010; 36(7):1112-26. · 5.17 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: In adult-onset multiple sclerosis (MS) cases, major depression, fatigue and psychological distress are common, whereas there is little information on these issues in children with the disease. The aim of this study was to assess psychosocial disorders in an Italian cohort of children and adolescent with MS. We evaluated 56 patients through self-assessment scales of depression (Children Depression Inventory) and fatigue (Fatigue Severity Scale), a psychiatric interview [Kiddie-SADS-Present and Lifetime Version (K-SADS-PL)] and an interview on school and everyday activities. Significant fatigue was found in 11 patients (20%). Twelve of the 39 patients who underwent the K-SADS-PL received a formal diagnosis of an affective disorder. Moreover, MS affected school activities in 28% of cases, daily living activities in 41% and social relationships in 28%. Our study confirms the critical role of psychosocial difficulties in children and adolescents with MS and provides a few cues to clinical management.
[Show abstract][Hide abstract] ABSTRACT: Neuromyelitis optica spectrum disorder (NMOsd) is a group of demyelinating disorders recently redefined and associated with
NMO-IgG/anti-aquaporin 4 antibodies. Because NMOsd is of unknown prevalence worldwide, we conducted a retrospective, cross-sectional
study of 850 patients with demyelinating disorders hospitalized in North East Tuscany from 1998 to 2006 to examine the prevalence
of NMO and related disorders among unselected consecutive neurological patients with inflammatory CNS diseases and to evaluate
the clinical phenotype spectrum of identified cases. Clinical data were updated after at least 2years of follow-up. An immunofluorescence
technique was used to detect NMO-IgG on rat brain tissue. Sera from other 828 neurological patients, 65 non-neurological patients
and 50 healthy donors served as controls. The prevalence of NMOsd was 1.5%, with a MS:NMOsd ratio of 42.7. Among 13 NMOsd
patients, 77% had long spinal cord lesions, 38% had severe optic neuritis and 23% had brain or brainstem lesions. Only 56%
had clinically definite NMO at follow-up. The final EDSS score ranged from 1 to 10, mainly depending on brainstem involvement
occurrence. Our findings confirm a low prevalence of NMO and related disorders among demyelinating inflammatory diseases in
a Caucasian population. Moreover, this study demonstrates an unexpectedly high prevalence of limited and atypical variants
of this disease, not previously documented.
KeywordsNeuromyelitis optica-NMO-IgG-Prevalence-Anti-aquaporin 4 antibodies
Journal of Neurology 11/2009; 256(11):1891-1898. · 3.58 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: A critical problem with neuropsychological assessment in children and adolescents with multiple sclerosis (MS) is the absence of a standardized, well-validated neuropsychological battery specifically tailored for detecting disease-related cognitive problems in this age range.
To develop a Brief Neuropsychological Battery for Children (BNBC) with MS.
We assessed cognitive functions in 61 patients with childhood and juvenile MS and 58 demographically matched healthy controls through an extensive neuropsychological battery.
In MS patients, we found a proportion of cognitive impairment of 41%. In the BNBC, we included the tests with higher discriminating ability (the Selective Reminding Test, the Symbol Digit Modalities Test, the Trail Making Test, and the Vocabulary test from the Wechsler Intelligence Scale for children). The BNBC yielded a sensitivity of 96% and a specificity of 76%.
Our findings provide preliminary evidence of the usefulness of the BNBC as a screening tool for detecting cognitive impairment in childhood and juvenile MS cases.