[The validity of the SET 5-10 (language level test for children aged between five and ten years): first analyses]. / Die Validität des Sprachstandserhebungstests für fünf- bis zehnjährige Kinder (SET 5-10): Erste Analysen.

INTRODUCTION: No other child developmental domain is as frequently affected by disorders as language acquisition. The SET 5-10 (language level test for children aged between 5 and 10 years), was developed to assess specific language disorders. OBJECTIVE: This study examines the ability of the SET 5-10 to differentiate between the developmental increases of speech competence, present deficits resulting from previous speech deficits and the probability of assessing possible speech deficits of children with an immigrant background. METHODS: Based on data of the norm sample (n=1,052; 51.8% female) different cohorts (age-groups, children with speech impairment problems and children with immigrant background) are compared by Multivariate analysis of variance. RESULTS: In all subtests a steady increase of performance with increasing age could be found. The means of children with previous grammar deficits (n=46) and children with immigrant background (n=143) are significantly lower than those of the reference group. CONCLUSION: The results presented offer first proof of a differentiated and economic diagnosis of language achievement of children 5-10 years of age.

[Patients with fibromyalgia: gender differences]. / Geschlechtsunterschiede beim Fibromyalgiesyndrom.

BACKGROUND: The aim of this study was to investigate how sex differences affect psychological measures and coping with pain of patients with fibromyalgia. Gender differences in pain coping strategies would require different gender-specific interventions. METHODS: Men with fibromyalgia were matched to women with fibromyalgia. Data were collected using the German pain questionnaire (DSF), the Hospital Anxiety and Depression Scale (HADS-D) and the questionnaire for assessment of level of coping with pain (FESV). Multivariate variance models were used for data analysis. RESULTS AND CONCLUSIONS: No gender differences were found in pain measures. Differences were found regarding psychological measures and coping strategies. Women showed more psychological strains and used more adaptive coping strategies on the scales "cognitive restructuring", "perceived self-competence", "mental diversion" and "counterbalancing activities" than men. This implies that women need more treatment for psychological aspects and men need assistance in pain management.

[Stance toward psychosomatic rehabilitation: development of a patient-questionnaire]. / Einstellung zur psychosomatischen Rehabilitation: Entwicklung eines Patienten-Fragebogens.

BACKGROUND: The transition from home to psychosomatic rehabilitation is problematic for many patients. They show little treatment motivation because of insufficient information and fear of stigmatization. Pre-treatment seeks to reduce these problems. The patient-questionnaire for stance toward psychosomatic rehabilitation was developed and tested for performance criteria. METHODS: A German statutory pension insurance scheme sample of psychosomatic rehabilitation patients (n=317) were recruited for testing psychosometric attributes and were questioned before and after pre-treatment. To determine whether the questionnaire can show clinically relevant changes, 124 patients with pre-information were compared with 88 patients without intervention. Additionally, clinically relevant changes for patients without knowledge of psychosomatic rehabilitation (n=70) were analyzed. RESULTS AND CONCLUSIONS: Factor analysis shows a three factor solution with ten items. The discriminatory power was good. High reliability was found for two factors and lower reliability for one factor. The patient-questionnaire for stance toward psychosomatic rehabilitation contains three scales: "knowledge", "confidence", and "motivation", and can be characterized as a reliable and economic instrument. After pre-treatment a general improvement was observed regarding patients' stance toward psychosomatic rehabilitation. The analyses result in significantly higher mean for patients with pre-treatment. Patients without previous information showed less "confidence" and "motivation". Pre-treatment can have a positive impact on the stance toward psychosomatic rehabilitation.

[Psychosomatic rehabilitation: effects of pre-treatment counselling under the statutory pension insurance scheme]. / Psychosomatische Rehabilitation: Effekte einer prästationären Beratung durch die Rentenversicherung.

BACKGROUND: Currently, little is known about the influence of pre-treatment in rehabilitation outcomes of patients with psychosomatic symptoms. This study examines whether there are differences in therapy motivation and impairment between patients with and those without pre-treatment measures. Additionally, the outcome for patients with prior knowledge of psychosomatic rehabilitation is analyzed. METHODS: As part of a randomized controlled pre-post design the Patients' questionnaire of therapy motivation (PAREMO) and the Brief Symptom Inventory (BSI) were distributed to 166 patients in psychosomatic rehabilitation. The selection of patients with prior knowledge of psychosomatic rehabilitation was made according to the scale "knowledge" of the patients' questionnaire. Changes were tested by analysis of variance at two treatment points. RESULTS: Although there were positive effects of rehabilitation, no interaction was found between measurement points and groups. The analyses of patients without prior knowledge of psychosomatic rehabilitation showed different outcomes for males and females. Higher rehabilitation outcomes for females than for males were found on the BSI scales "somatisation", "phobic anxiety", and "psychoticism". CONCLUSIONS: The findings show that pre-treatment facilitates the patients' approach to psychosomatic rehabilitation. Apart from receiving information patients should be motivated more strongly and their confidence in psychosomatic rehabilitation strengthened.

[Fibromyalgia: influence of depressive symptoms in coping with pain]. / Patienten mit Fibromyalgiesyndrom: Der Einfluss von Depressivität auf die Einstellung zur Schmerzbewältigung.

BACKGROUND: The aim of this study was to investigate how depressiveness affects coping of patients with fibromyalgia. It was assumed that depressiveness has an influence on coping, self-efficacy und motivation for therapy. METHODS: A total of 251 patients with fibromyalgia. The investigation started at the outset of their stay in the clinic, and both patients with and without depressiveness (HADS-D cut-off >or=11) were examined. To investigate the differences, multivariate variance models were used for cognitive and behavioural coping, self-efficacy and motivation for therapy. Data were collected using the Hospital Anxiety and Depression Scale (HADS-D), German pain questionnaire (DSF), Questionnaire for assessment of level of coping with pain (FESV) and the German version of the Pain stages of change questionnaires (FF-STABS). RESULTS AND CONCLUSIONS: Comparison of the groups shows significantly lower means for the fibromyalgia patients with depressive symptoms concerning "perceived self-competence", "cognitive restructuring", and "countertraded activity". The same results can be observed for "self-efficacy". Analysis of the motivation for therapy in the "carefreeness" scale shows significantly higher means in the fibromyalgia patients with depressive symptoms. In the "maintenance" scale, however, lower means are observed in this patient group. As fibromyalgia patients with depressive symptoms show greater strain on account of their psychosomatic symptoms, depressive symptoms should be dealt with during treatment.

[Self management in chronically ill adolescents: development of a short questionnaire]. / Entwicklung eines Kurz-Fragebogens zum Krankheitsmanagement chronisch kranker Jugendlicher.

PURPOSE: Behavioural interventions in chronically ill adolescents aim to improve disease-related self-management. The study presents a short questionnaire for examining the self-management strategies in paediatric patients with obesity, asthma, type I diabetes or atopic eczema. METHODS: For each indication, six favourable disease management strategies were identified: adherence to the subjective body perception, avoidance of trigger factors, adequate control of one's own behaviour, appropriate emergency treatment, and regular prolonged treatment including, depending on the disease at hand, adherence to alimentation. For each indication a "disease management" scale with six items was constructed. Psychometric properties (item statistics, reliability) were examined among 416 adolescents. Participants were diagnosed at least with one chronic illness and took part in an inpatient rehabilitation programme in a German rehabilitation clinic. RESULTS: For the scale obesity (KM-KJ-Adi) (n=192) difficulty of the items was 0.30-0.43, discrimination was 0.32-0.57 and Cronbach's alpha was 0.72. For the scale asthma (KM KJ AB) (n=164) difficulty of the items was 0.43-0.65, discrimination was 0.29-0.52 and Cronbach's alpha was 0.70. For the scale diabetes (KM-KJ-DM1) (n=78) difficulty of the items was of 0.42-0.74, discrimination was of 0.25-0.87 and Cronbach's alpha was 0.68. For the scale atopic eczema (KM-KJ-AE) (n=125) difficulty of the items was 0.31-0.71, discrimination was of 0.24-0.51 and Cronbach's alpha was 0.64. DISCUSSION: Up to now, no economic, indication-specific and reliable questionnaire exists for assessing the competences of adolescents in their disease self-management. The presented questionnaire fills this gap, the indication-specific scales with only a few items show satisfactory psychometric results. CONCLUSIONS: This questionnaire can be used as an instrument for self-management strategies in chronically ill adolescents. The responsiveness (change score) and validity have to be further investigated.

[COPD: Burden, risk factors and prevalence]. / COPD: Belastung, Risikofaktoren und Prävalenz.

Previous COPD prevalence rates are highly variable. This high variability probably depends on different criteria and methods used. The actual impact of COPD is probably underestimated. Measures of the burden of COPD, such as morbidity, mortality, and costs are based on these prevalence rates, and thus underly the same challenges. The GOLD strategies, published in 2001, established objectifiable physiological criteria to define prevalence and severity of COPD. These guidelines will enhance the quality of prevalence rates and the burden of COPD in the near future. Until now only a few epidemiological studies considered these criteria. The present review outlines current findings on COPD risk factors, COPD burden and recent results of the international Burden of Obstructive Lung Disease study (BOLD).

[Can indicator parameters for patient estimation of rehabilitation success be derived from the SGRO (Saint George's Respiratory Questionnaire)?]. / Kann man aus dem SGRQ (Saint George's Respiratory Questionnaire) Indikatorwerte zur Patienteneinschätzung des Reha-Erfolgs ableiten?

OBJECTIVES AND METHODS: Quality of life assessed by the Saint George's Respiratory Questionnaire (SGRQ) is an important outcome-parameter for pneumological rehabilitation programmes. However, criteria for qualitative assessment of the change are lacking. To evaluate clinically significant changes, the approach of a global patient's judgement is used to assess a patient-experienced clinical outcome as external criterion. The changes in quality of life of 166 asthma and COPD patients who estimated the treatment efficacy differently were compared by analysis of variance. Group means with distinct 95% confidence boundaries were used to define indicators to estimate the clinically significant change in HRQOL for the patients. RESULTS: Whereas no distinct confidence boundaries are found in the subscales, the SGRQ total score could be used to identify indicators for clinically significant changes in quality of life. CONCLUSION: The assessment of an empirically-based, patient judgement as criteria for changes in quality of life by rehabilitation programmes increases the evidence-based evaluation by a patient-oriented perspective. At the same time it reduces the lack of significant criteria for estimating clinically significant changes.

[Medication behavior of children and adolescents with asthma before and after in-patient rehabilitation--results of a multicenter study]. / Medikationsverhalten asthmakranker Kinder und Jugendlicher vor und nach stationärer Rehabilitation - Ergebnisse einer multizentrischen Studie.

The article presented is based on data of a multicenter study examining the effectiveness of an inpatient rehabilitation program for children and adolescents with asthma. The study took place from August 1996 to November 2001. It shows more noticeable long-term improvement of disease management and lung function parameters after in-patient rehabilitation (n = 226) than after out-patient rehabilitation programs (n = 92). The re-analysis of the data examines the influence of medication on these results. A decisive effect on treatment results for both in- and outpatients can be achieved by complying with the guidelines recommended by the medical societies. However, at the same time the results demonstrate that prescribing a correct treatment plan is not always sufficient. The significant improvement of outcome parameters (functional restriction, lung function, days absent from school, visits to a physician) of the rehabilitation group shows the importance of rehabilitation for teaching strategies for disease management and increase in compliance.

Noninvasive evaluation of adult onset myopathy from carnitine palmitoyl transferase II deficiency using proton magnetic resonance spectroscopy.

OBJECTIVE: The adult onset metabolic myopathy of carnitine palmitoyl transferase II (CPT II) deficiency is under-recognized, in part due to variable degrees of enzyme deficiency and symptomatology, as well as limitations in means for noninvasive evaluation. We describe a proton magnetic resonance spectroscopy (MRS) technique, using a standard clinical magnetic resonance imaging scanner, to diagnose and help monitor the response to therapy in adult CPT II deficiency. METHODS: A 53-year-old woman presented with a long standing history of diffuse aching and fatigue provoked by high fat intake, fasting, or prolonged exertion. Muscle biopsy revealed myopathic features and a deficiency (33% of control) of CPT II activity with elevated palmitoyl carnitine. Proton MRS of the soleus muscle was performed using a 1.5 Tesla scanner before and during dietary therapy. RESULTS: Proton MRS revealed shortening of the transverse relaxation time (T2), consistent with increased acetylation of the carnitine pool. The symptoms resolved completely by treatment with frequent feedings of a high carbohydrate diet low in long chain fatty acids supplemented with medium chain triglycerides and L-carnitine. Recovery of normal muscle MRS and carnitine T2 relaxation was documented by the third month of therapy. CONCLUSION: Proton MRS is a novel, potentially useful, and readily available adjunct in the diagnosis and therapeutic monitoring of muscle CPT II deficiency.

[The efficacy of 'Training with aggressive children' (TaK) from the wiewpoint of therapists]. / Die Effizienz des Trainings mit aggressiven Kindern (TaK) aus der Sicht der Therapeuten.

In all German speaking-countries the "Training mit aggressiven Kindern" is one of the most common intervention programs for the treatment of aggressive behavior children and adolescents (conduct disorder). The following analysis examines how the users of this training program judge its efficacy. The study outlines preferentially used methods and shows which factors (e.g. practical experience, setting, gender) influence choice and judgement.

Case of the month: March 1999--A 26 year old HIV positive male with dura based masses.

A 26-year-old male with AIDS presented with a chief complaint of headaches and neck pain. An MRI revealed two enhancing extra-axial dura based masses, one in the area of the left sphenoid wing and one at the level of C2-3. In both cases, microscopic sections showed actin positive spindle cell neoplasms with long slender nuclei, consistent with leiomyomas. Both tumors were positive for Epstein Barr virus by in situ hybridization. This case report serves to emphasize the importance of considering soft tissue tumors such as leiomyoma in the differential diagnosis of mass lesions that occur in the central nervous system in AIDS and discusses the role of EBV in tumorigenesis.

Abnormalities in central nervous system development in osteogenesis imperfecta type II.

Osteogenesis imperfecta (OI) type II is a perinatally lethal condition resulting from mutations in type I collagen genes. In addition to characteristic skeletal anomalies, OI type II has recently been shown to be associated with neuropathological alterations, specifically perivenous microcalcifications, and impaired neuroblast migration. In light of these findings, and because type I collagen promotes neuritic maturation both in vitro and in vivo, we sought to determine if additional central nervous system (CNS) developmental anomalies could be found in previously autopsied OI type II cases, and if specific abnormalities correlate with OI subtypes. We retrospectively studied brains of nine patients diagnosed with OI. Of these, seven were OI type II: five were OI type IIA, one was type IIB, and one was type IIC. One OI type I specimen and one OI type III brain were included for comparison, as well as five controls. The IIC brain showed hippocampal malrotation, agyria, abnormal neuronal lamination, diffuse hemorrhage, and periventricular leukomalacia (PVL). The IIB brain had white matter gliosis, PVL, and perivascular calcifications, but was normally developed. Of the five type IIA brains, two showed migrational defects with coexisting PVL and gliosis, two were normally developed with similar white matter injuries, and one was grossly normal. These findings support the contention that collagen mutations might negatively impact CNS development.

Mitochondrial DNA polymerase gamma deficiency and mtDNA depletion in a child with Alpers' syndrome.

Deficiency of mitochondrial DNA polymerase gamma activity was found in a patient with mtDNA depletion and Alpers' syndrome. Metabolic evaluation revealed fasting hypoglycemia, dicarboxylic aciduria, and reduced activity of the electron transport chain in skeletal muscle. The patient died in early childhood of fulminant hepatic failure, refractory epilepsy, lactic acidemia, and coma. mtDNA content was 30% of normal in skeletal muscle and 25% in the liver. The activity of mtDNA polymerase gamma was undetectable.

Clinical implications of clubfoot histopathology.

Ipsilateral peroneus brevis muscle histopathology was studied in 64 children with idiopathic rigid equinovarus at the setting of initial posteromedial-lateral release. Fifty percent of biopsies demonstrated abnormal muscle fiber morphology, classified as congenital fiber-type disproportion or fiber-size variation. Forty-one infants (59 feet) underwent initial surgical intervention within the first year of life with a minimum 2-year follow-up. Feet with abnormal muscle histology had a significantly greater incidence of recurrent equinovarus deformity requiring reoperation; the relative risk of clubfoot recurrence in children with fiber abnormalities was 5.6. Male patients with bilateral deformity and abnormal peroneus brevis histology had a particularly high incidence of recurrent equinovarus. Developmental internal tibial torsion requiring surgical intervention was also greater in the abnormal-fiber histology group. The incidence of postoperative metatarsus adductus/varus necessitating surgery was comparable despite histologic findings. Thus muscle-fiber abnormalities are prevalent in idiopathic equinovarus. Such fiber-type anomalies may predict recurrent limb deformities.

Case of the month: January 1998--43 year old male with radicular pain.

A 43-year-old male presented with progressively worsening right lower extremity pain. MRI of the spine showed a discrete intradural, extramedullary, homogeneously enhancing, sausage-shaped mass at L1-L2, noted intraoperatively to expand the filum terminale. Gross, histological, and electron microscopic findings were those of a paraganglioma. The case is used to discuss the differential diagnosis for sausage-shaped tumors of the filum terminale.

Subpial vacuolar myelopathy after intrathecal ketamine: report of a case.

Previous studies of intrathecal ketamine use in humans have documented the clinical effects of its administration without reference to local central nervous system (CNS) toxicity (Reich and Silvay, 1989). Although several post-mortem studies in small groups of rabbits, monkeys, and baboons have largely shown no significant CNS damage after intrathecal ketamine use, a study in rats reported vacuolation of dorsal root ganglia (Ahuja, 1983). We report post-mortem CNS histopathological changes of subpial spinal cord vacuolation in a terminally ill cancer patient who received continuous infusion intrathecal ketamine at a rate of 5 mg/day for a duration of 3 weeks.

Amebic meningoencephalitis caused by Balamuthia mandrillaris: case report and review.

Balamuthia mandrillaris, formerly referred to as a leptomyxid ameba, is a free-living ameba that has recently been identified as a cause of meningoencephalitis. Previously, only two genera, Naegleria and Acanthamoeba, were recognized as causes of central nervous system (CNS) infections in humans. In contrast to Naegleria, Balamuthia causes a subacute-to-chronic infection of the CNS. Distinct from Acanthamoeba, which appears to favor the immunocompromised host, Balamuthia is capable of infecting both healthy and immunosuppressed hosts. Retrospective analyses as well as an accumulation of newly identified cases have demonstrated that this ameba is an increasingly important pathogen to recognize. We report the isolation, histopathologic features, and confirmation by indirect immunofluorescence of B. mandrillaris in a case of fatal amebic meningoencephalitis.