[Achievement motivation in children with migraine and their healthy siblings]. / Leistungsmotivation bei Kindern mit Migräne und ihren gesunden Geschwistern.

QUESTIONS: Psychosocial variables like stress or a high achievement motivation are discussed as a possible contributory factor to an attack in migraine. The aim of our study was to examine, whether children with migraine may be at risk for impaired achievement motivation. PATIENTS/METHODS: 37 children with migraine and their 17 non-affected siblings, were examined by means of an especially developed test for achievement motivation and by the Kaufman-Assessment-Battery for Children (K-ABC). RESULTS: There were no significant differences in achievement motivation and cognition between both groups. Children with migraine and their siblings demonstrated a neurocognitive performance within the average normal test range. There was a slight positive correlation between an atypical achievement motivation and the duration of migraine attacks. CONCLUSION: In general there are no signs of a pathologically elevated achievement motivation in children with migraine, but we found evidence that there might be a subgroup of patients, within whom an atypical achievement motivation might serve as a trigger variable, and who might profit from relaxation techniques and psychological-educational counselling.

Intraocular pressure, safety and quality of life in glaucoma patients switching to latanoprost from adjunctive and monotherapy treatments.

PURPOSE: To evaluate efficacy, safety and quality of life in ocular hypertensive or open-angle glaucoma patients changed to latanoprost from previous therapy. METHODS: A prospective, multicenter, active-controlled design in which qualified patients had their previous therapy substituted for latanoprost and were followed for at least three months. RESULTS: In 1068 patients, latanoprost was continued 92% throughout the 36-month observation period. Latanoprost treatment reduced the intraocular pressure (1OP)(p < 0.001) when compared to previous monotherapies including: beta-blockers (-4.0 +/- 3.7 mmHg, 42%), alpha-antagonists (-3.9 +/- 3.0 mmHg, 14%), miotics (-3.8 +/- 3.5 mmHg, 2%), or carbonic anhydrase inhibitors (CAI) (-3.8 +/- 3.6 mmHg, n = 16%), and adjunctive therapy including: beta-blocker and CAI (-3.7 +/- 3.1 mmHg, n = 12%), alpha-agonist (-3.7 +/- 3.4 mmHg, n = 5%), or pilocarpine (-3.4 +/- 3.7 mmHg, n = 6%), or CAI and alpha-agonist (-4.6 +/- 6.4 mm Hg, n = 2%)(p < 0.0017). The most common adverse event with latanoprost was ocular allergy (1.5% incidence). Patients showed a preference for latanoprost for many systemic and ocular quality of life measures on a non-validated questionnaire (p < 0.05). CONCLUSIONS: In a clinical setting, patients who have their mono- and adjunctive therapy treatment substituted for latanoprost may on average experience reduced IOP, decreased side effects and increased quality of life measures.

[Methodological perspectives for the assessment of adaptation in chronic diseases, exemplified by asthma bronchiale in childhood]. / Methodologische Perspektiven zur Erfassung der Adaptation bei chronischen Erkrankungen am Beispiel des Asthma bronchiale im Kindesalter. Psychopathologie, Adaptation und gesundheitsbezogene Lebensqualität bei Asthma bronchiale.

BACKGROUND: Regarding the measurement of psychosocial adaptation due to chronic diseases in childhood and adolescence, there is a shift from a more reductionist and biomedical oriented disease-model towards a more integrated, biopsychosocial view of chronic diseases. The three paradigms in measuring psychosocial adaptation (psychopathology, coping, health related quality of life) will be discussed at the example of corresponding empirical studies in children with asthma bronchiale. The psychopathology-oriented research emphasizes the risk of the induced psychopathological comorbidity, whereas the more coping-oriented paradigm primarily includes the dynamic process of the perceived stress and the corresponding coping efforts due to a chronic disease. The third paradigm, the quality of life paradigm, sets its main focus on the subjective view of the chronically ill subject. CONCLUSIONS: In future studies, all three paradigms--each measuring different aspects of psychosocial adaptation--should be simultaneously included to come to a more complex view of adaptation in chronic diseases in general and asthma bronchiale in particular.

Neurodevelopmental outcome after intrauterine red cell transfusion for parvovirus B19-induced fetal hydrops.

OBJECTIVE: To assess long term neurodevelopmental outcome of children after intrauterine intravascular red cell transfusion (JUT) for Parvovirus B19-induced fetal hydrops. DESIGN: Data of study children were investigated retrospectively. Neurodevelopmental evaluation was performed by appropriate standard tests (Griffiths, Snijders-Oomen, Kaufmann Assessment Battery for Children tests). SETTING: Tertiary care university teaching hospital. SAMPLE: Twenty children who had Parvovirus-induced fetal hydrops and intrauterine transfusion of packed red blood cells (IUT). METHODS: Retrospective chart analysis and standard neurodevelopmental testing. MAIN OUTCOME MEASURES: Developmental quotient (DQ) and intelligence quotient (IQ) according to the age at testing. RESULTS: Twenty survivors of Parvovirus B19-induced fetal hydrops successfully treated by IUT were followed until 13 months to nine years of age. On clinical follow up, no neurologic sequelae were evident. Neurodevelopmental scores of all children ranged within two standard deviations of a normal population (median 101, range 86-116) and exceeded one standard deviation in three children. There was no significant neurodevelopmental delay. CONCLUSION: Children having survived successful IUT for Parvovirus B19-induced fetal anaemia and hydrops have a good neurodevelopmental prognosis. Our results support the use of IUT for correction of Parvovirus B19-induced fetal anaemia and subsequent hydrops.

Cognitive development in children with migraine and their healthy unaffected siblings.

The discussion about neuropsychological sequalae from migraine occurring in adults is controversial. The purpose of our study was to examine whether children with migraine may be at risk for impaired intellectual development compared with their siblings free of migraine.

Growth hormone deficiency in pituitary disease: relationship to depression, apathy and somatic complaints.

OBJECTIVE: Adults with GH deficiency (GHD) have been reported to suffer from increased levels of depression and apathy, which are thought to contribute to the reduced quality of life observed in these patients when compared with healthy controls. Recent studies, however, cast doubt on the attribution of these impairments to GHD as opposed to an unspecific stress response to the chronic medical condition. DESIGN: To further clarify this relationship, we used psychometric tests to quantify depression, apathy and typical psychosomatic complaints in patients with different types of pituitary disease and compared the results with measurements of the patients' widely varying GH status. SUBJECTS AND METHODS: In 98 patients, serum IGF-I was measured and at least one provocative test of the somatotrope pituitary axis was performed (GH-releasing hormone test (GHRHT) and/or insulin tolerance test (ITT)). All patients completed a set of well-established psychometric instruments (Beck Depression Inventory (BDI), Apathy Evaluation Scale (AS) and List of Somatic Complaints (LSC)). In addition, AS was administered in an informant report version for completion by a close relative or friend to verify the validity of the patient's self assessment. RESULTS: No relationship between measures of GHD (IGF-I, GHRHT and ITT) and psychometrically measured depression, apathy or psychosomatic well-being was found. A highly significant linear correlation between scores of all psychometric instruments (BDI, AS and LSC) was found. CONCLUSIONS: The absence of any relationship between the severity of GHD and the level of depression/apathy/psychosomatic complaints suggests that these impairments are not specific symptoms of GHD. The reported improvement of these symptoms under GH substitution therapy might thus be interpreted as a secondary effect of somatic effects of GH substitution. Consequently, indication for GH substitution therapy should not be based on psychological impairments alone without the presence of somatic symptoms of GHD.

The voltage-gated sodium channel gene SCN2A and idiopathic generalized epilepsy.

We tested the hypothesis that genetic variation in the human sodium channel gene SCN2A confers liability to idiopathic generalized epilepsy (IGE). We performed a systematic search for mutations in 46 familial IGE cases and detected three novel polymorphisms, however, allele frequencies did not differ significantly between patients and controls. A rare mutation (R1918H) was identified in one patient but was absent in one further affected family member. Thus, our results do not suggest a major role of SCN2A in the etiology of IGE.

Successful cognitive-behavioral habituation training toward photophobia in photogenic partial seizures.

We report on the cognitive-behavioral treatment of a 12-year-old boy with photogenic partial seizures with secondary generalization who had developed phobic avoidant behavior toward all kinds of situations with potential photostimulation, leading to serious impairments of life quality. Based on a behavioral analysis of seizure and anxiety reaction, a habituation training (systematic desensitization procedure) was developed and performed, while maintaining protection against seizures with antiepileptic pharmacotherapy. The treatment was to gradually increase exposure to computer monitor and television screen photostimulation, closely adjusted to the level of subjective distress and tolerance. In addition to exercising control of photogenic input by regulating the monitor control button, rapid relaxation and imagery techniques were taught and applied as countermeasures at the onset of seizure precipitants. The treatment resulted in complete remission of phobic anxieties and responses and may also have facilitated ongoing seizure control after termination of antiepileptic medication.

Neurodevelopmental risks in twin-to-twin transfusion syndrome: preliminary findings.

The purpose of this study was to determine the neurodevelopmental risks in patients with twin-to-twin transfusion syndrome, a rare but serious complication of monochorionic twin gestations. From a total sample of 94 twins with twin-to-twin transfusion syndrome, admitted during 1989 and 1993, 49 patients survived and 40 patients were followed to a mean age of 24 months. Neurological status and psychomotor development (Denver and Griffiths Developmental Tests) were determined. Parameters of the neonatal period were evaluated for their potential prediction. Of the 40 tested patients 18 showed a normal psychomotor development. Thirteen patients exibited a specific delay in language development and/or showed minor neurological dysfunctions. Nine twins had severe psychomotor retardation in combination with cerebral palsy. Major neurological sequelae were found, more common in recipients than in donors (6/19 vs 3/21). Correspondingly, neonatal ultrasound showed more pathological results (especially periventricular leucomalacia) in recipients. Neither anaemia nor polycythaemia at birth can predict developmental outcome. Apart from a high prenatal mortality rate, both twins, donators as well as recipients, are highly at risk for brain damage of different aetiology, associated with abnormal neonatal cerebral ultrasound.

[Significance of spontaneous motor activity and visual information processing for developmental diagnosis in infancy]. / Bedeutung von Spontanmotorik und visueller Informationsverarbeitung für die Entwicklungsdiagnostik im Säuglingsalter.

The neurodevelopmental examination during the first years of life is very often confronted with the problem of differentiating between normal variability and pathology of development. Recent research provides evidence about the clinical relevance of the systematic observation and examination of spontaneous movements, of postural control as well as of visual information processing in toddlers.

[Psychosocial adaptation and compliance in chronic diseases in childhood]. / Psychosoziale Adaptation und Compliance bei chronischen Erkrankungen im Kindesalter.

A chronic disease in childhood often entails multidimensional, medical, psychosocial and financial risks which empede efficient adaptation to the disease for the whole family. Aim of this review is to describe possible strategies for effective coping with the chronic disease with the main focus on self-help-competence for the affected child and his family. Furthermore, there are proposals regarding practical handling of therapeutical noncompliance in the chronically ill infant. The family of a child with a chronic disease often contacts nurses for further, non-medical information. Therefore the aim of the review is to inform nurses of the biopsychosocial risks and consequences for every day life as well as to provide them with supportive self-help-strategies of the affected family.

Evidence of a specific vulnerability for deficient sequential cognitive information processing in epilepsy.

The aim was to study sequential versus simultaneous cognitive information processing and their relation to acquired intellectual abilities in children with epilepsy. A total of 175 children with epilepsy (mean age 8.7 years; SD 2.9) were neuropsychologically examined on the basis of the Kaufman Assessment Battery for Children (K-ABC). To avoid confoundation with factors other than epilepsy, patients with underlying metabolic disorders or patients who were incapable of neuropsychologic testing because of severe mental retardation were excluded. We found significantly reduced cognitive processing, especially in sequential information processing compared with simultaneous information processing (mean score 85.9 versus 91.0). This selective impairment in sequential cognitive processing turned out to be a good statistical predictor for acquired abilities in the group of idiopathic epilepsy (beta = 0.53; P < .01), indicating a specific epileptic neuropsychologic association. Our findings provide evidence of a specific vulnerability for deficient sequential information processing presumably impairing academic achievement in childhood epilepsy.

[Processes of body perception and their therapeutic use in pediatrics. From nonspecific relaxation therapy to training to recognize disease-specific symptoms]. / Prozesse der Körperwahrnehmung und deren therapeutische Nutzung in der Pädiatrie: Die Weiterentwicklung unspezifischer Entspannungsverfahren zum Training krankheitsspezifischer Beschwerde- und Symptomwahrnehmung.

Focussing on processes of body perception is a major pathway of relaxation therapies (progressive relaxation, autogenic training, guided imagery, hypnotherapy, biofeedback). Traditionally its application has been related to psychosomatic and psychotherapeutic indications. Beyond this classical approach, recent behavioral medicine has emphasized the relevance of interoception processes and adequate attribution patterns concerning bodily sensations as a major source of adequate coping and self-management with somatic illness. Clinical application may refer to an improved cognitive-behavioral pain management in disease and treatment related conditions. Especially children and adolescents suffering from chronic conditions that may exacerbate rapidly may benefit from an education approach that teaches them to perceive their disease-related complaints and symptoms accurately and to attribute them correctly. A precise, panic-free and immediate symptom recognition of sudden airway obstruction is an important precondition of adequate coping with acute asthma crisis and starting risk orientated antiasthmatic treatment. In a similar way, the child with diabetes mellitus may identify early signs of hypoglycemia by self-observation, recognition and discrimination of physical, vegetative and psychological indicators of blood glucose decline that enable the child to take appropriate countermeasures. Other childhood disorders that offer chances for symptomatic self-monitoring and self-control comprise atopic dermatitis or epileptic seizures. Training young patients in precise symptom recognition may not only empower them in handling acute crisis but also strengthen global development of autonomy, control beliefs, self-responsibility and self-esteem.

Interstitial deletion in Xp22.3 is associated with X linked ichthyosis, mental retardation, and epilepsy.

We describe monozygotic male twins with an interstitial deletion of Xp22.3 including the steroid sulphatase gene (STS). The twins had X linked ichthyosis, X linked mental retardation, and epilepsy. A locus for X linked mental retardation has been assigned to a region between STS and DXS31 spanning approximately 3 Mb. Recently the locus was further refined to an approximately 1 Mb region between DXS1060 and GS1. By PCR analysis of flanking STS gene markers in our patients we succeeded in narrowing down the locus to between DXS6837 and GS1.

Symptomatic versus substitution growth hormone therapy in short children: from auxology towards a comprehensive multidimensional assessment of short stature and related interventions.

There is an ongoing debate as to whether symptomatic growth hormone treatment (GHT) in short children without growth hormone deficiency (GHD) is justified, since there is no substitutional indication. The increasing evidence that final height cannot be normalized in these patients (e.g. in Ullrich-Turner syndrome) reinforces this controversy. We have focused on the empirical evidence on the psychosocial and physical meaning of being short in childhood as well as on the underlying assumptions of the different GHT indications. The indication for GHT in patients with non-GHD may be seen as a pharmacotherapeutic intervention in order to prevent the developmental, physical and psychosocial risks associated with short stature. This requires a qualitative shift in methodological assessment with respect to the psychosocial and physical impact of being short as well as of the potential benefit of new treatment indications in terms of a more comprehensive evaluation including health-related quality of life for these patients.

Good prognosis for psychomotor development in survivors with nonimmune hydrops fetalis.

We examined the psychomotor development of 33 of 61 surviving children, from a series of 107 consecutive live-born cases with nonimmune hydrops fetalis. The majority had a normal outcome. Three had a (simultaneous) serious underlying disease (e.g. fetal herpes infection) and had either severe psychomotor retardation or blindness. Two showed clumsiness and were considered to have minor neurological dysfunction. We conclude that survivors, especially those with transient benign intrauterine conditions, such as lymphatic aetiology have no additional risk to their psychomotor development.

Adjustment in conditions with short stature: a conceptual framework.

A major justification of extended indications for GH therapy in conditions with short stature is based on the objective of preventing or alleviating suspected psychosocial maladjustment. Despite more sophisticated research, results are still controversial concerning the actual seriousness of adjustment difficulties in various conditions with short stature, thus making it difficult to determine a patient's need for treatment. This paper discusses different concepts and assessment strategies of "adjustment" as a major source of the apparent heterogeneity among study findings, conclusions and treatment recommendations. A concise framework is developed that identifies and differentiates three hierarchical levels of research on adjustment in conditions with growth retardation: (1) stress exposure due to short stature, (2) quality of coping responses, and (3) occurrence of psychopathology. Choosing a particular research level and its corresponding measures may imply whether a high, medium or very low impact of short stature on adjustment is found, and the need for treatment that is inferred. The integration of these different research approaches within an integrative model may help to resolve apparent inconsistencies among empirical studies and reveal sources of confoundation due to other, short stature independent risk factors associated with the particular underlying growth disorder. The key terms of the suggested model are transformed into a clinical guideline for psychological assessment in patients with growth retardation and into three key criteria for the decision making process on extended indications for GH treatment with respect to the improvement of psychosocial adjustment.