Assessment of children's nutritional attitudes before oral food challenges to identify patients at risk of food reintroduction failure: a prospective study.

BACKGROUND: Inappropriate dietary eliminations may impair quality of life, affect children's growth and unnecessarily impact on healthcare costs. Previous retrospective studies reported that around 25% of children continue a food-avoidance diet despite a negative oral food challenge (OFC). A definite pattern has not been found yet for patients not reintroducing the food. This study aimed to examine the role of child's nutritional attitudes and maternal anxiety in reintroducing food after a negative OFC. METHODS: A prospective study was conducted involving 81 mothers of children with IgE-mediated food allergy. They completed a survey on nutritional behaviour and attitudes and the State-Trait Anxiety Inventory on the day of OFC and 6 months later. RESULTS: In total, 11.1% of children never or rarely ate the food after a negative OFC. Consumption of the reintroduced food is positively correlated to child's interest in tasting new foods before and after OFC and to changes in child's nutritional habits after OFC. It is negatively correlated to monotony of the diet after OFC. No correlations were found with other participants' characteristics or maternal anxiety. State anxiety significantly decreased after the OFC. A correlation was found between trait and state anxiety and the degree of change in nutritional habits after OFC. CONCLUSIONS: Evaluating child's approach towards food before the OFC is a promising approach to identify patients at risk of food reintroduction failure. Furthermore, it underlined the importance of reassessing food consumption in all patients after a negative OFC and supporting patients in the reintroduction of food.

Coping strategies, alexithymia and anxiety in young patients with food allergy.

BACKGROUND: Food allergy is major public health concern affecting nearly 15 million Americans and 80 million Europeans. Risk of anaphylaxis and implications for social activities affect patients' quality of life and psychological well-being. We previously found that young patients reported higher levels of alexithymia (difficulty in recognizing and expressing emotions) compared with healthy peers and may influence affect, management style and clinical outcomes. This study aimed to explore links between coping strategies, alexithymia and anxiety among food-allergic adolescents and young adults. METHODS: Ninety-two patients with IgE-mediated food allergy (mean age 18.6 years) completed Coping Orientation to Problems Experienced Inventory, Toronto Alexithymia Scale and Trait Anxiety subscale of State-Trait Anxiety Inventory. Multivariate analyses of variance assessed differences and associations between subgroups on the scales. RESULTS: Significant differences found between alexithymia levels in coping style were explained by Avoidance strategies. 'Avoidance' had the highest contribution in explaining alexithymia, followed by trait anxiety, age, anaphylaxis and social support. Respondents with higher alexithymia use avoidance as coping strategy over and above other coping strategies such as problem-solving and positive thinking, are younger, will have experienced anaphylaxis and will have lower social support. CONCLUSIONS: Recognizing the specific role of affect regulation in health behaviours may constitute an important step in supporting patients to explore more adaptive strategies.

Perinatal stress and food allergy: a preliminary study on maternal reports.

Maternal stress in fetal and early life has been associated with the development of respiratory allergies, but no studies exist about food allergy. Stressful events and the quality of caregiving provided, as they affect the emotional and physiologic regulation of the infant, could alter the hypothalamic-pituitary-adrenal and immune system, facilitating an increased allergic response. This study aimed to investigate the influence of perinatal stress, as perceived by mothers, on developing food allergy in childhood. A survey on pregnancy and the first three months after giving birth was submitted to 59 Italian mothers of at least one child suffering from severe food allergy and one completely healthy child, for a total of 118 children examined. The presence of stressful events and the quality of perinatal period for each child were assessed retrospectively. The food allergic children's data were compared to siblings' data through inferential statistics. The results showed a significantly higher number of stressful events occurred during patients' perinatal period, compared to siblings, in particular bereavements in pregnancy and parenting difficulties in postpartum. Mothers reported harder pregnancies and more stressful, harder, and, in general, worse postpartum when referring to their food-allergic children, in comparison with their siblings (p < .05). Psychological aspects are demonstrated to be involved in the development of allergic diseases. This study constitutes the first step to examine the role of early stress and perinatal psychosocial factors in the pathogenesis of food allergy; further studies are necessary to understand individual psychological impact and its relations with genetic and biological factors.

Cognitive impairment and (CTG)n expansion in myotonic dystrophy patients.

BACKGROUND: Myotonic dystrophy (DM) is a genetic multisystemic disease with muscular, endocrine, ocular, cardiac and cognitive impairment. The molecular basis of the disease has been identified in an unstable base triplet (CTG)n repeat located in the 3' untranslated region of the miotonin protein-kinase (MT-PK) gene on the long arm of chromosome 19. Cognitive impairment could be a direct expression of this genetic alteration at the central nervous system (CNS) level rather than a consequence of the neuromuscular impairment. To explore this hypothesis, we tested a group of genetically diagnosed, adult onset DM, of their nonaffected relatives (NAR), of patients with spinal muscle atrophy (SMA), and of normal controls using the Wechsler Adult Intelligence Scale (WAIS). METHODS: Seventeen adult-onset DM patients, 9 NAR, 10 SMA patients and 20 unrelated normal controls (NC) were studied. Clinical, neuromuscular and neuropsychiatric evaluation, which included WAIS and the Schedule for Affective Disorders and Schizophrenia (SADS), were performed on the four groups. DM, NAR and NC were also assessed by a neurophysiological (P300) evaluation. A DNA analysis was performed in DM and in NAR to measure presence and magnitude of CTG expansion. RESULTS: We found a statistically significant difference between verbal (p < .0003), nonverbal (p < .0001) and total (p < .0001) IQ of DM patients compared to IQs of NAR, SMA and NC. Seven out of 11 WAIS subtests were significantly and consistently lower in DM patients compared to SMA and/or NC. In DM patients there was a statistically significant negative correlation between nonverbal (r = -.68; p < .002) and total (r = .59; p < .01) IQ and (CTG)n. Patients with DM had a significantly lower P300 amplitude compared to NAR and NC. CONCLUSIONS: Our study indicates that in DM there is a mild but significant cognitive impairment which correlates with the degree of CTG expansion and it is not dependent on the neuromuscular impairment; however further studies with larger groups of patients and controls are suggested to confirm our results, due to the small sample size and to a possible effect of educational level in our patients.

Remembering and knowing the past: a case study of isolated retrograde amnesia.

We describe a patient, RM, who suddenly became amnesic for premorbid autobiographic events in the absence of any known precipitating event. Learning abilities as well as semantic knowledge were normal. Knowledge of famous facts and persons was good, although not perfect. Whether RM suffered from organic or psychogenic isolated retrograde amnesia (IRA) could not be established on the basis of available clinical and neuropsychological elements. Regardless of its aetiology, RM's case respects the boundaries between semantic and episodic memory and so gives further support to the distinction between these two memory systems.

Reversible aphasia in adolescence: A late-onset form of Landau Kleffner syndrome? Report of a single case.

An adolescent boy developed a long-lasting pattern of global aphasia, concomitant to focal (left temporal) EEG abnormalities; this was followed by complete recovery. Laboratory and neuroimaging studies were within normal limits. The possibility of a late-onset Landau Kleffner syndrome is discussed.

Depressive equivalents: a clarification using the Rorschach test.

The authors present a study on 20 patients of uncertain diagnostic placement admitted to a neurological department. The patients are characterized by an atypical clinical neurological symptomatology and the absence of organic pathology. Through psychiatric examination and the Rorschach tests, which show some characteristics and constant aspects, the authors arrive at the diagnosis of depressive equivalents. It is emphasized that the Rorschach test offers a useful contribution to the often difficult diagnosis of these clinical pictures.

Depression and neuroticism in multiple sclerosis.

88 subjects (36 males and 52 females) affected by multiple sclerosis (MS), were studied with the CES-D and SRT tests for the evaluation of depressive reactions and neuroticism. Comparing the results with those of the control group, we found a significant score for depression and somatization in the MS patients, whereas the scores for anxiety and inadequacy were normal.

Divergence of central nervous system involvement in 2 Italian sisters with congenital muscular dystrophy: a clinical and neuroradiological follow-up.

We report the clinical and neuroradiological follow-up of 2 Italian sisters, 10 and 6 years of age, affected by congenital muscular dystrophy (CMD) with divergent CNS involvement. In both, CMD was diagnosed by finding dystrophic alterations in muscle biopsy and muscular deficit at birth. The elder sister suffered also from marked intellectual deficit and epilepsy, as usually reported in children with Fukuyama CMD. In the same patient, at 2 years of age, CT scan showed severe hypodensity of cerebral white matter and severe ventricular dilatation of occipital horns. At 8 years of age, MRI also showed clearcut pachygyria mainly in the parietal and occipital lobes. MRI and CT scan at the same age showed improvement of the leukoencephalopathy and unchanged ventricular dilatation, as reported for patients with Fukuyama CMD. Unlike Japanese cases, however, she showed no progression in her muscular deficit and her muscle immunostaining of laminin M chain (merosin) was normal. The younger sister had normal mental development, never experienced epileptic fits and had always normal EEG. However, as often seen in classical CMD, her CT scan showed moderate hypomyelination of cerebral white matter and mild dilatation of lateral ventricles. MRI did not show any other brain abnormalities. Sequential CT scan at 2, 4 and 6 years of age showed improvement of the leukoencephalopathy. Her muscular deficit had a stationary clinical course. Her immunostaining of muscle merosin was moderately reduced. The finding of Fukuyama-like and classical CMD in 2 sisters indicates the possibility that different forms of CMD may be different expressions of the same genetic disease.

Brain alterations in the classical form of congenital muscular dystrophy. Clinical and neuroimaging follow-up of 12 cases and correlation with the expression of merosin in muscle.

In the classical form of congenital muscular dystrophy (CMD), subclinical brain involvement is frequent. In order to establish the natural evolution of CNS alterations in this type of CMD, the cerebral functions of 12 cases were examined longitudinally for a mean period of 8 years. There were 7 boys and 5 girls, with a mean age of 5 years at first evaluation and 13 at the last one. Merosin expression in muscle fiber basement membrane, evaluated in 10 of them, was normal in 6 and deficient in 4. CNS conditions were followed up by repeated neuropsychiatric examinations, intelligence tests, EEG and brain CT scan and/or MRI. Eight of the 12 patients (including the 4 with merosin-deficiency) had normal intelligence, while 4 had mild to moderate mental retardation: in all the intellectual ability was unchanged during the follow-up study. CT scan detected minor brain alterations in 9 patients: 6 of these, the 4 with merosin deficiency and 2 others in whom merosin was not evaluated, presented leukoencephalopathy: on neuroimaging reappraisal it was unchanged in 3, improved in 2 and worse in 1 (a merosin-deficient case). Cerebellar alterations or mild ventricular dilatation were detected in 8 cases, including 3 merosin-non-deficient ones: these abnormalities were unchanged at the last study by CT and MRI, as were the normal neuroimaging findings observed in 3 other cases. Overall, during our study the brain alterations found in classical CMD showed a stationary or an improving course; progressive worsening was observed only in 1 of 4 merosin-deficient cases with leukoencephalopathy.