Automatic imitation in youngsters with Gilles de la Tourette syndrome: A behavioral study.

It is widely known that humans have a tendency to imitate each other and that appropriate modulation of automatic imitative behaviors has a crucial function in social interactions. Gilles de la Tourette syndrome (GTS) is a childhood-onset neuropsychiatric disorder characterized by motor and phonic tics. Apart from tics, patients with GTS are often reported to show an abnormal tendency to automatically imitate others' behaviors (i.e., echophenomena), which may be related to a failure in top-down inhibition of imitative response tendencies. The aim of the current study is to explore the top-down inhibitory mechanisms on automatic imitative behaviors in youngsters with GTS. Error rates and reaction times from 32 participants with GTS and 32 controls were collected in response to an automatic imitation task assessing the influence of observed movements displayed in the first-person perspective on congruent and incongruent motor responses. Results showed that participants with GTS had higher error rates than controls, and their responses were faster than those of controls in incompatible stimuli. Our findings provide novel evidence of a key difference between youngsters with GTS and typically developing participants in the ability to effectively control the production of own motor responses to sensory inputs deriving from observed actions.

Osmophobia in migraine classification: a multicentre study in juvenile patients.

AIMS: This study was planned to investigate the diagnostic utility of osmophobia as criterion for migraine without aura (MO) as proposed in the Appendix (A1.1) of the International Classification of Headache Disorders (ICHD-II, 2004). METHODS: We analysed 1020 patients presenting at 10 Italian juvenile headache centres, 622 affected by migraine (M) and 328 by tension-type headache (TTH); 70 were affected by headache not elsewhere classified (NEC) in ICHD-II. By using a semi-structured questionnaire, the prevalence of osmophobia was 26.9%, significantly higher in M than TTH patients (34.6% vs 14.3%). RESULTS: Osmophobia was correlated with: (i) family history of M and osmophobia; and (ii) other accompanying symptoms of M. By applying these 'new' criteria, we found an agreement with the current criteria for the diagnosis of migraine without aura (MO) in 96.2% of cases; 54.3% of previously unclassifiable patients received a 'new' diagnosis. CONCLUSIONS: In conclusion, this study demonstrates that this new approach, proposed in the Appendix (A1.1), appears easy to apply and should improve the diagnostic standard of ICHD-II in young patients too.

Migraine with aura with onset in childhood and adolescence: long-term natural history and prognostic factors.

The long-term course of migraine with aura (MA) has been poorly explored. The present 11-year follow-up study assessed the long-term natural history and possible prognostic factors of MA with onset in childhood or adolescence. Patients were recruited from the original case records of our department, which are specifically designed to report all headache characteristics, aura symptoms and electroencephalogram (EEG) findings. A total of 77 patients (47 females; 30 males) whose records contained detailed descriptions of both headache and aura symptoms apparently meeting the International Classification of Headache Disorders (ICHD)-II criteria for MA (i.e., 1.2.1, 1.2.2, 1.2.6) underwent structured face-to-face follow-up headache interviews, all of which were conducted by the same neurologist, who has particular expertise in this field. A multivariate model (logistic regression analysis) was used to investigate the association between possible prognostic factors and the remission of both aura and headache at follow-up. The results of our study showed that 23.4% of the MA patients were headache-free at follow-up, 44.1% still had MA and 32.5% had a transformed headache diagnosis (i.e., fulfilling the criteria for ICHD-II 1.1. or 2). Patients with basilar-type migraine (1.2.6) showed the highest headache remission rate (38.5%). Our study seems to show that migraine with typical aura (1.2.1-1.2.2) is associated with a favourable evolution of aura symptoms over time (remission of aura in 54.1% of patients). Subjects experiencing only visual aura had a lower remission rate compared with those with visual +/- sensory +/- aphasic aura symptoms (36.8% vs. 61.5%, p = 0.054). A short headache duration (<12 hrs) and the presence of EEG abnormalities at baseline were the only significant predictors of aura remission at follow-up (odds ratio [OR] = 9.12, 95% confidence interval [CI]: 1.79 +/- 46.51, and OR = 4.76, 95% CI: 1.18 +/- 19.15, respectively). No significant predictors of headache remission were found. In conclusion, our results suggest that MA shows a favourable course. Further prospective studies with detailed EEG analysis both at baseline and at follow-up are needed in order to confirm the possible prognostic role of EEG abnormalities in MA. That said, it would, in our opinion, be highly premature at present to submit children with MA to EEG examinations for prognostication purposes.

Mitral valve prolapse and abnormalities of haemostasis in children and adolescents with migraine with aura and other idiopathic headaches: a pilot study.

OBJECTIVE: To investigate the prevalence of mitral valve prolapse (MVP) and abnormalities of haemostasis in children and adolescents with migraine with aura (MA) compared with peers affected by other idiopathic headaches. MATERIALS AND METHODS: We recruited 20 MA patients (10 men and 10 women; age range 8-17 years) and 20 sex- and age-matched subjects with other idiopathic headaches. Both groups underwent colour Doppler transthoracic echocardiography to detect MVP and the following laboratory work-up: plasma prothrombin time, activated partial thromboplastin time, thrombin time, fibrinogen, protein C, protein S, homocysteine, lupus anticoagulant, von Willebrand factor (vWF) ristocetin cofactor activity, immunoglobulins (Ig) G and M anticardiolipin antibodies (aCL). Factor V Leiden, factor II and methylenetetrahydrofolate reductase were investigated (we did not test the entire genes, but screened for specific point mutations). RESULTS: The prevalence of MVP was significantly higher in the MA subjects than in the patients affected by other idiopathic headaches (40% vs 10%; P < 0.05). Moreover, the MA patients showed a higher rate of above-normal IgM aCL titres (45% vs 10%; P < 0.05). Finally, in the group of patients with MVP we found a higher prevalence of aCL in those with MA compared with those affected by other idiopathic headaches. CONCLUSIONS: A proportion, at least, of the MA patients showed a more complex phenotype characterized by MVP and/or positive aCL titres. The pathogenetic role of these associations is obscure and larger studies are needed to confirm the usefulness of echocardiographic and laboratory investigations in this area and to identify possible new treatment approaches that might be explored in this group of MA patients.

Academic performance in children with rolandic epilepsy.

The aim of this study was to investigate the frequency of reading, writing, and calculation disabilities in children with typical rolandic epilepsy (RE) and healthy control children. We also aimed to define the possible electroclinical markers of specific cognitive dysfunctions in RE. School abilities were evaluated and compared in 20 children (eight males, 12 females; mean age 10y 3mo [SD 1y 7mo]; range 7y 9mo-12y 9mo) consecutively diagnosed with typical RE, and a group of 21 healthy controls (nine males, 12 females; mean age 10y 4mo [SD 1y 8mo]; range 7y 6mo-13y 3mo). All the children received standardized neuropsychological tests. For each patient an exhaustive seizure diary was kept and all the sleep electroencephalogram (EEG) recordings were reviewed. Specific difficulties with reading, writing, and calculation (diagnosed according to the criteria of the Diagnostic and Statistical Manual of Mental Disorders, 4th edition) were found in nine out of 20 children with RE and two out of 21 healthy controls (chi2=0.01). The specific learning disabilities in the RE group were correlated with a marked increase in epileptiform discharges during sleep (chi2=0.02) and an early onset of epilepsy (chi2=0.02). Our findings suggest that seizure onset before age 8 years and epileptiform discharges (more than 50% of the sleep EEG recording) in several tracings over more than a year are relevant markers for identifying patients at risk of developing academic difficulties.

Neuropsychological profile of pre-schoolers with metaphonological difficulties: results from a non-clinical sample.

BACKGROUND: The level of language development reached in pre-school age is considered the most reliable predictor of reading acquisition. In normally developing children, learning to read is strongly related to early language skills, and in particular to phonological processing abilities. In dyslexic children, reading abilities seem to show a correlation with phonological awareness. METHODS: A group of 65 children (aged 5-6 years) were recruited and submitted to an in-depth neuropsychological assessment [i.e. metaphonological skills, intelligence, verbal short-term memory (VSTM) and other aspects of receptive and expressive language]. We were able to identify 14 children with significant metaphonological difficulties (MD): 11 children with exclusively MD, and the other three children with specific language impairment. This study compares the neuropsychological profile obtained from children with MD with that of a peer group without any language impairment (N). RESULTS: The performances of the MD were within the normal ability range in almost all the administered tests but significantly lower compared with those of their peers without language impairment (N) in some items of the intelligence scale (Wechsler Preschool and Primary Scale of Intelligence) and in the tests of VSTM and of receptive/expressive language. Nevertheless, there were not statistically significant differences between MD and N in output phonology. CONCLUSIONS: In pre-school age, in a group of non-clinical children, with a range of abilities, those with MD appear to be at the lower end of the normal range in many other verbal skills. These children could be considered at-risk for possible subsequent difficulties learning to read and thus need to be identified and to warrant prompt treatment.

The effectiveness of hospitalization in the treatment of paediatric idiopathic headache patients.

BACKGROUND: Headache is a disease that has a high social impact in the paediatric as well as in the adult population, often resulting in a significant reduction in the young patient's quality of life, reflected primarily in a greater number of days off school and increasingly frequent recourse to symptomatic drugs. The idea for this study came from the clinical impression that some paediatric headache patients might benefit more from inpatient than outpatient care. AIM: The aim of our study was to compare the effectiveness of hospitalization to outpatient care of patients with newly diagnosed frequent and disabling headache. METHODS: A pragmatic randomized open-label trial was conducted at the Child Neurology Clinic of the University of Pavia, Italy. Children and adolescents with a 2- to 6-month moderate-to-severe migraine or tension-type headache history were randomized to hospital admission or outpatient assessment and followed for 6 months. The efficacy of the two therapeutic strategies was measured by counting the number of responders in each arm. Other end points included the mean frequency and duration of attacks, the number of drug prescriptions taken to control pain, and the number of patients and physicians expressing satisfaction with treatment. RESULTS: The study population included 27 girls and 23 boys aged 8 through 18 years with migraine (23 cases) or tension-type headache (27 cases). Compared to outpatient assessment, hospital admission was correlated to a significant increase in the number of responders: 0 vs. 44% (1 month), 0 vs. 68% (3 months), and 12 vs. 68% (6 months). The mean frequency and duration of attacks were significantly lower in hospitalized patients (p < 0.0001). Hospitalization was correlated with a significant reduction of patients with severe headache (p < 0.005), a reduction of drug use, and a higher number of satisfied patients and physicians (p < 0.05). Logistic regression analysis confirmed the higher responder rate among hospitalized patients after adjusting for age, sex, diagnosis, and headache characteristics or admission. CONCLUSIONS: We think hospitalization reduces the emotional mechanisms that provoke stress in children and often induce or favour headache attacks. If these mechanisms can be interrupted, the management of disease may become easier and with enduring benefits.

[Brothers of children with a neurological disease: difficulties and affective needs. Study of a clinical sample]. / Les frères des enfants atteints d'une maladie neurologique chronique: difficultés et besoins émotionnels.

INTRODUCTION: Feelings of envy and jealousy among brothers belonging to families with a child affected by a chronic neuromotor impairment have rarely been studied and literature on this subject is sparce. The interest of this work arises in particular from our experience with families and children suffering from this kind of disability. AIMS OF THE STUDY: The individuation of frequency and characteristics of overt or "hidden" emotional problems in brothers of handicapped children. Needs of the handicapped child lead to the partial disinvestment of the "healthy" one. We therefore focused our attention on the defensive mechanisms involved. METHOD: The work was carried out in the Rehabilitation Unit of our Department of Child Neuropsychiatry. We selected all the families in which a son exhibited severe neuromotor impairment, and we proposed some sessions with the parents and the healthy children. Only 10 families with 11 children accepted these sessions. We asked the other families to fill-in the Achenbach checklist (CBCL). Assessment of the 11 children seen was made with talks, drawing and playing sessions, and with an interview of the parents at the beginning and at the end of the sessions. RESULTS: We present the data of 33 CBCL filled-in and returned and of 11 psychodiagnostic observations. The CBCL showed 6 pathological and 5 borderline (with high psychopathological risk) total T scores. Among the 11 clinically observed children, 4 revealed a relevant psychopathological profile, so an individual psychotherapeutic therapy was proposed. These children expressed their emotional problems by difficult peer relationships, attention and concentration deficits and failure at school. Moreover, we found a significant correlation between a relevant child psychopathology, the fragile personality structure of the parents, and problems in the parental couple. CONCLUSIONS: These data stress the importance that must be given to emotional problems of handicapped children's brothers. Very often these children aren't able to express these problems, because either the parents' mental space is full of fears for the sick child's prognosis and care, and their "free" time is filled with hospitalisations, check-ups and rehabilitation. Our experience shows that giving attention and space for thoughts encourages emotional and relational movements, with a transforming value that leads to enhanced integration of emotional experiences.

Regional reductions of gray matter volume in familial dyslexia.

An in vivo anatomic study of gray matter volume was performed in a group of familial dyslexic individuals, using an optimized method of voxel-based morphometry. Focal abnormalities in gray matter volume were observed bilaterally in the planum temporale, inferior temporal cortex, and cerebellar nuclei, suggesting that the underlying anatomic abnormalities may be responsible for defective written language acquisition in these subjects.

Prevalence of tic disorders among primary school students in the city of Pavia, Italy.

BACKGROUND: The prevalence of tic disorders in children varies from 1% to 29% depending on the characteristics of the study population, the diagnostic criteria, and the study design and methods. AIMS: To calculate the prevalence of tic disorders among primary school children in Italy. METHODS: The study population comprised 2347 primary school children from the city of Pavia (pop. 80 073), Northern Italy. Using trained school teachers as the source of cases, all children with motor or vocal tics occurring intermittently and unpredictably out of a background of normal motor activity were accepted. The type, frequency, and circumstances of tic disorders were noted. School performance was correlated to the presence of tics. RESULTS: A total of 68 children (56 boys, 12 girls) aged 6-11 years were identified with tic disorders. The period prevalence was 2.9% (95% CI 2.3 to 3.7). The prevalence was 4.4% in boys and 1.1% in girls, with no detectable trends at age 6-11. Motor tics were present in 46 cases, vocal tics in 6, and motor and vocal tics in 16. Situation related tics were noted in 37 cases. A significant correlation was found between the presence of tic disorders and impaired school performance. DISCUSSIONS: Tic disorders are a fairly uncommon but disabling clinical disorder among primary school children from an urban community. The fairly low prevalence of this clinical condition, as compared to other reports, can be explained by the choice of stringent diagnostic criteria and the exclusion of patients with other movement disorders.

Are vascular disorders more prevalent in the relatives of children and adolescents with migraine?

The objective of the study was to assess whether a family history of vascular disorders is more common in children and adolescents with migraine than in the general population. Family history of stroke, arterial hypertension, myocardial infarction and diabetes was investigated by history taking in relatives of ambulatory children and young adults with migraine and in a control group. The odds ratios (ORs) with 95% confidence intervals (95% CI) were used as a risk measure. Using univariate and multivariate (logistic regression) analysis, family history was assessed in the whole sample and in subgroups by sex and age, degree of relationship (parents and grandparents vs. relatives), disease type (migraine with and without aura), and type of vascular disorder. The sample included 143 cases (migraine with aura 35, migraine without aura 108) and 164 controls aged 3-24 years (mean 12 +/- 3.8 years). Patients with migraine were at increased risk of vascular disorders in parents and grandparents but not in all relatives. Multivariate analysis indicated family history of stroke as most common only in boys. In conclusion, our study provides some clues to the assumption that migraine and vascular disorders have common pathogenic mechanisms and that genetic susceptibility plays a role in increasing the risk of migraine in the offspring of families with one or more cerebrovascular or cardiovascular conditions.

Personality traits in childhood and adolescent headache.

We evaluate personality traits, anxiety and depression in a population of paediatric and adolescent patients, correlating personality characteristics with headache and sociodemographic variables. The clinical features of headache include specific personality traits. We report a clinical study of 57 patients (age 8-18 years), divided up as follows: 12 migraine with aura, 29 migraine without aura and 16 tension-type headache. One of Cattel's tests was administered to every patient; the Children's Depression Inventory test was administered to 53 patients and the Test Anxiety Inventory test to 43 subjects. The scores obtained by every patient in each test were correlated with the characteristics of headache and with sociodemographic data. We found that patients affected by idiopathic headache share some personality traits, mainly emotional rigidity and tendency to repress anger and aggression. These traits do not seem to be correlated with sociodemographic data and the duration of headache: we considered these as characteristic of migrainous patients.

Acquired epileptic frontal syndrome as long-term outcome in two children with CSWS.

Continuous spikes and waves during slow sleep (CSWS) are a well-known EEG pattern that can be associated with cognitive and behavioural deterioration. We present the long-term clinical, neuropsychological and EEG follow-up of two patients who developed CSWS during childhood. In both the CSWS onset was followed immediately by rapid cognitive and behavioural deterioration. Later the CSWS fragmented or fluctuated and the spike-wave discharges diminished and this was associated with progressive clinical improvement. At the same time bilateral frontal EEG abnormalities appeared awake and in sleep. After the initial period of rapid cognitive and linguistic improvement both patients stabilised. The latest neuropsychological assessment showed a frontal syndrome. The presence of frontal EEG abnormalities superimposed on CSWS, their persistence after CSWS resolution and, in addition, the finding of subtle frontal-type neuropsychological alterations early in recovery may indicate poor long-term outcome.

Vigabatrin use in psychotic epileptic patients: report of a prospective pilot study.

OBJECTIVES: Evaluation of any possible behavioural reactions in epileptic patients during vigabatrin treatment. MATERIAL AND METHODS: Ten patients with refractory partial epilepsy, previous mental retardation and psychosis or other significant psychiatric morbidity treated with vigabatrin were submitted periodically to specific tests (to quantify any possible change in behavioural parameters) and also to EEG recordings. RESULTS: After 1 year of treatment 5/10 patients became seizure-free, 3/10 of them presented reduction of seizures by over 75%. None of the subjects presented episodes that could be interpreted as psychotic reactions and, moreover, some patients showed a reduction in stereotypies, instability and aggressiveness. In 66% of these patients an improvement in cognitive functions was observed. CONCLUSION: The onset of side effects can be prevented by a gradual introduction of vigabatrin and by the use of the drug in moderate doses. These data do not confirm the contraindication to treat with vigabatrin patients with a history of psychiatric disturbances.

Dolicho-vertebrobasilar abnormality and migraine-like attacks.

This is a study of two children with neuroradiologically diagnosed dolichobasilar anomaly: in both cases, the diagnosis was based on magnetic resonance imaging and magnetic resonance angiography findings. We examined the first child (R., aged 6 years and 8 months) following four episodes of intense pain in the left orbit, lasting between 10 and 30 min and accompanied by photophobia and vomiting. The second child (S., aged 9 years and 2 months), suffering from Marfan's syndrome, was examined following recurrent episodes of intense pain that started above the left orbit, moving to the homolateral orbit after a few minutes. These episodes, which lasted between 20 and 30 min, took place mainly at night and were accompanied by phono-photophobia and vomiting. In both cases, the pain attacks can be interpreted as a result of compression of the root of the trigeminal nerve caused by an arterial malformation.

Continuous spikes and waves during sleep in children with shunted hydrocephalus.

Focal epileptiform abnormalities in awake children submitted to ventricular shunting are well described in the literature, but there are few reports about EEG patterns during sleep. We studied 20 children affected by hydrocephalus of various aetiology and submitted to shunting during the first year of life. We found focal abnormalities in all the children and in 95% of cases they were on the same side as the shunt; in 65% of cases they had an amplitude of 300 mV or more. During sleep there was activation of abnormalities in all subjects, and in 33% we found continuous spikes and waves during slow sleep (CSWS). We discuss the aetiology of CSWS and its possible role in causing the neuropsychological disturbances of our patients.

Periodic syndrome and migraine in children and adolescents.

Many reports in the literature seem to confirm the hypothesis that the symptoms of periodic syndrome are precursors or the equivalent of migraine: the aim of this study was to assess the prevalence of periodic syndrome in a group of children and adolescents suffering from migraine in comparison with that observed in various control groups. We considered seven symptoms: recurrent vomiting and abdominal pain, migrating limb pain, vertigo, recurrent hyperthermia with no visible cause, sleep disturbances and eating disorders. The study involved 171 children divided into four groups; 42 migraineurs; 37 subjects with chronic nervous pathologies but no psychosomatic symptoms; 46 subjects with a known psychosomatic disease, and 46 healthy subjects. The prevalence of the symptoms in the different control groups was different, although the pattern was more similar in the migraineurs and psychosomatic patients than in the other control groups. The development continuum of the syndrome may support the view that periodic syndrome is predictive of the subsequent development of a psychosomatic pathology.