Ataxia telangiectasia, Menkes kinky hair disease and neurocutaneous melanosis.

This article explores three neurocutaneous syndromes (NCSs), i.e. genetic disorders producing developmental abnormalities of the skin and an increased risk of neurological complications. In this review, different aspects of ataxia telangiectasia, Menkes kinky hair disease and neurocutaneous melanosis are examined: clinical features, genetic defect, mutation spectrum, pathogenesis, and neurobiological basis; indications for clinical practice are also provided to the readers. The aim of this review is to stress the importance of cooperation among dermatologists, neurologists and psychiatrists, in order to provide patients suffering from these diseases with timely diagnosis and targeted treatments.

Adolescence: psychopathological discomfort, distress or psychiatric disorder? Clinical experience, description and analysis of a sample.

BACKGROUND: The aim of this study was to highlight how many psychopathological disorders emerging during adolescence are not easily detectable and interpretable. METHODS: We investigated the social, emotional and demographic factors related to psychopathological distress, measuring the overall functioning of the child/adolescent and the consequent impact on academic and social functioning, along with its implications on the emergence of feelings of discomfort and anxiety. RESULTS: Many psychopathological disorders are easily detectable and diagnosable in this particular stage of life, and although they are rooted in adolescent distress, they can generate real psychiatric disorders. CONCLUSIONS: The data emerging from clinical practice stress the need for a comprehensive in order to identify possible subclinical symptoms for onset of mental illness, so as to be able to implement more targeted and less invasive therapeutic strategies.

The Rorschach Test Evaluation in Chronic Childhood Migraine: A Preliminary Multicenter Case-Control Study.

OBJECT: About 1.2-3.2% of children at 7 years of age with increasing age up to 4-19% in adolescents are suffering from migraine without aura (MwA). The aim of the present study is investigating the personality style associated with children and adolescents affected by MwA, administrating the Rorschach test, and comparing with typical developing healthy controls (TD). METHODS: 137 patients (74 males), aged 7.3-17.4 years (mean age 11.4, SD 3.02 years), affected by MwA according to the IHs-3 criteria. The Rorschach variables were treated as numerical variables and statistically tested with t-Student's analysis. RESULTS: No statistical differences were found between the MwA and TD for age (p = 0.55), and gender (p = 0.804). From the comparison between the two samples, MwA group shows lower W responses (p < 0.001), good quality W responses (p < 0.001), high frequency of detailed responses (p < 0.001), the presence of even minor form of good quality responses (p < 0.001), increased presence of animals answers (A%) (p < 0.001), more frequent trivial answers (Ban%) (p < 0.001). DISCUSSION: Rorschach interpretation pinpointed many interesting and, perhaps, peculiar aspects in our MwA population such as a trend predisposition for: analytical reasoning rather than synthetic, ease/practicality rather than creativity, oppositionality rather than external adaptation to the environment that may be interpreted as effect of general maladaptivity.

Adolescence, imperceptible boundary between normality and pathology: a literature review.

A reorganization of the brain occurs in adolescence and it is documented by neuroimaging, neurophysiology and pathology of development. The high levels of neural plasticity allow the intellectual and emotional development during adolescence, a transition period of life full of physical and psychological changes. However, potentially dangerous agents could be the genesis of psychopathology of the adult and mental illness or distress. Adolescence is a crucial phase for the maturation of the brain. Therefore, future research should study how the environment affects and influences the function and the organization of the brain. Teenagers look for new experiences and strong emotions, sometimes endangering their health. Neuroscientific explanations have been proposed for the typical teenagers' behavior. In the postnatal period the highest density of gray matter can be found in the primary sensorimotor cortex, while prefrontal cortex matures later. Subcortical areas of the brain, especially the limbic system and the reward system, develop earlier; therefore, there is an imbalance between the more mature subcortical areas and the less mature prefrontal areas during adolescence. This could explain the typical behavior patterns of this period of life.

Skin and brain: itch and psychiatric disorders.

Skin diseases (atopic eczema, psoriasis, idiopathic urticaria), systemic diseases (chronic hepatic or renal failure, morbus Hodgkin, diabetes mellitus) and psychiatric disorders (obsessive compulsive disorders, depression, delusions of parasitosis) can occur with itching. The aim of this review is to clarify the link between pruritus and psychiatric morbidity and emphasize the importance of a psychiatric consultation for patients with a chronic itching, without a skin disease. In the last years, there is a growing awareness regarding psychogenic itch, although these types of itch are significantly less studied in comparison to other types of pruritus. Psychogenic pruritus is usually a diagnosis of exclusion. There are not controlled studies about treatment of psychogenic itch, but the same drugs prescribed for neuropathic pain, depression, and anxiety are used. There is a strong association between pruritus and psyche; so, it is important that the dermatologist evaluates psychosomatic dimension. According to the analysis of scientific literature and our clinical experience, pruritus seems to be a rather common phenomenon in patients suffering from depression. Future works should explain the basis of psychopathology of chronic itching thanks to studies of selected groups of patients with a particular type of chronic itching, highlighting the clinical features to establish appropriate and individual targeted care, based on the several types of pruritus. Some questions still unanswered could be clarified in this way. It is really important to decrease the symptoms "itching", because the quality of life of the patient will be improved, but the goal is to identify the underlying mechanisms of itch and establish a targeted therapy, depending on the biological changes and the underlying disease.

Anxiety and depression levels in prepubertal obese children: a case-control study.

INTRODUCTION: Childhood obesity has become a worldwide epidemic in Western and in developing countries and has been accompanied by many serious and severe comorbidities, such as diabetes, hypertension, sleep apnea syndrome, depression, dyslipidemia, impaired glucose homeostasis, steatohepatitis, and intracranial hypertension, as well as medical concerns unique to youth, such as accelerated pubertal and skeletal development and orthopedic disorders. To date, no specific studies about the psychological assessment in pediatric obesity are present. Therefore, the aim of this study was to evaluate the putative relationship between psychological troubles and obesity in a sample of school-aged children. MATERIALS AND METHODS: The study population consists of 148 obese subjects (body mass index [BMI] >95th percentile) (69 males, mean age 8.9±1.23 years) consecutively referred from clinical pediatricians to the Child and Adolescent Neuropsychiatry department at the Second University of Naples. In all subjects, weight, height, and BMI z-score were evaluated. In order to assess the anxiety levels and the presence of depressive symptoms, the Children Depression Inventory (CDI) and the Italian Self-Administered Psychiatric Scales for Children and Adolescents (SAFA) were administered. The control group consisted of 273 healthy children (129 males and 144 females) (mean age 9.1±1.8 years), enrolled in schools within the Campania region of Italy. RESULTS: No significant differences between the two study groups were found for age (8.9±1.23 years in the obese sample and 9.1±1.8 years in the control group) (P=0.228) or sex (ratio male/female: 69/79 in the obese group versus 129/144 in the control group) (P=0.983). Obviously, significant difference was found for the BMI z-score (2.46±0.31 in the obese group vs 0.73±0.51 in the control group) (P<0.001). The obese subjects showed significant higher level of depressive symptoms (CDI total score) (16.82±7.73 vs 8.2±2.9) (P<0.001) and anxiety (SAFA - Anxiety [SAFA-A]) scale score (58.71±11.84 vs 27.75±11.5) (P<0.001) compared with the control group. Moreover, the Pearson's correlation analysis showed a significantly positive relationship between the BMI z-score and both the CDI (r=0.677; P<0.001) and SAFA-A scores (r=0.591; P<0.001). CONCLUSION: Our findings highlighted the importance of assessing the presence of internalizing problems, such as anxiety and depression, in the common management of childhood obesity.

Segmental immune disorders resulting from neurologic injuries.

The immunocompromised cutaneous district (ICD) is a novel pathogenic concept that refers to the occurrence of opportunistic skin disorders (such as infections, malignancies, and immune diseases) at a cutaneous site previously marked by a damaging event, usually involving the local lymph network or peripheral sensory nerves. In addition to herpetic infections, which are notoriously harmful for sensory nerve fibers and therefore already included among the potential causes of ICD, there are a large and variegate group of further neurologic diseases, both peripheral (carpal tunnel syndrome, facial nerve palsy, and trigeminal trophic syndrome) and central (poliomyelitis and brain stroke), which may be added to the wide and expanding spectrum of injuring events resulting in an ICD. The neural compartment of skin immunity plays a key role in immune homeostasis, and this assertion is confirmed by the fact that any neurologic injury, whatever the origin (peripheral or central) or the cause (infection, trauma, ischemia), can give rise to immune destabilization of the innervated area, which becomes a site prone to the occurrence of opportunistic skin disorders. A neural-driven process may be responsible for the cutaneous immune dysregulation ensuing from some neurologic diseases.

Stereotyped movements in a group of autistic children.

The authors studied the stereotyped movements presented by a group of 20 autistic children, evaluating the patient observation protocols according to a psychodynamic model of autism. The stereotyped movements were analysed considering ten different parameters: type, site, morphology, frequency, association, complexity, active sensory channel, trigger event, affect during stereotyped movement, and function. The results indicate that each autistic child possesses highly idiosyncratic "stereotypical behavioural equipment", which includes behaviours ranging from simple, reflex-like actions, to much more complex movement patterns. Stereotyped movements were found to appear, in response to different stimuli, at moments when the subject's psychophysical state needed "resetting". Eight possible functions of stereotyped movements were identified. These correspond to two main purposes: reinforcement or weakening of the autistic barrier. The need for rehabilitation is questioned, since not all stereotyped behaviours interfere with explorative and cognitive activities.