Long-term quality of life after ETV or ETV with consecutive VP shunt placement in hydrocephalic pediatric patients.

PURPOSE: Endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunting are well-established treatments of obstructive hydrocephalus (HCP) in adult and pediatric patients. However, there is a lack of data with regard to the quality of life (QoL) of these patients during long-term follow-up METHODS: Inclusion criteria were pediatric patients with endoscopic treatment of hydrocephalus at the University Medicine Greifswald between 1993 and 2016. Patients older than 14 years at present were assessed with the Short Form-12 (SF-12) questionnaire. Patients younger than 14 years of age were assessed with the KINDL-R questionnaire that was completed by their parents. Patients' values were compared with the scores of a corresponding age-matched group of the healthy population and with patients who received later shunt treatment. Information about comorbidities, current symptoms, and educational level were gained by an additional part. Comparative analysis between patients with ETV success and failure (defined as shunt implantation after ETV) was performed. RESULTS: A total of 107 patients (53 m, 54f) were included. Fifty-seven/107 patients (53.3%) were considered as ETV success. Mean age at ETV was 6.9 ± 5.9 years. Fifty-four statements of 89 patients that are still alive were gained (response rate 63%). Of these, 49 questionnaires were complete and evaluable (23 m, 26f; mean age 19.8 ± 10.0 years with an average follow-up period of 13.7 ± 7.2 years). Twenty-six/49 patients (53.1%) are considered ETV success. No statistically significant differences could be obtained between patients with ETV success and ETV failure. Patients older 14 years show QoL within normal range, patients younger than 14 years show significantly lower result regarding their environment of peers and social contacts. Patients younger than 6 months at the time of ETV and patients with posthemorrhagic HCP show significantly lower physical QoL. Gait disturbance, fatigue, and seizures are associated with a lower QoL, and educational level is lower than in the normal population. CONCLUSIONS: Patients who underwent ETV in childhood do not have a lower health-related QoL in general. Subsequent insertions of ventriculoperitoneal (vp) shunts do not lower QoL. Certain subgroups of the patients show lower results compared to the healthy population.

Children with multiphasic disseminated encephalomyelitis and antibodies to the myelin oligodendrocyte glycoprotein (MOG): Extending the spectrum of MOG antibody positive diseases.

BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG) antibodies have been described in children with acute disseminated encephalomyelitis (ADEM), recurrent optic neuritis, neuromyelitis optica spectrum disorders and more recently in children with multiphasic disseminated encephalomyelitis (MDEM). OBJECTIVE: To delineate the clinical, cerebrospinal fluid (CSF) and radiological features of paediatric MDEM with MOG antibodies. METHODS: Clinical course, serum antibodies, CSF, magnetic resonance imaging (MRI) studies and outcome of paediatric MDEM patients were reviewed. RESULTS: A total of 8 children with two or more episodes of ADEM were identified from a cohort of 295 children with acute demyelinating events. All children had persisting MOG antibodies (median titre: 1:1280). All ADEM episodes included encephalopathy, polyfocal neurological signs and a typical MRI. Apart from ADEM episodes, three children had further clinical attacks without encephalopathy. Median age at initial presentation was 3 years (range: 1-7 years) and median follow-up 4 years (range: 1-8 years). New ADEM episodes were associated with new neurological signs and new MRI lesions. Clinical outcome did range from normal (four of the eight) to mild or moderate impairment (four of the eight). A total of four children received monthly immunoglobulin treatment during the disease course. CONCLUSION: Children with MDEM and persisting MOG antibodies constitute a distinct entity of relapsing demyelinating events and extend the spectrum of MOG antibody-associated diseases.

Hereditary orotic aciduria with epilepsy and without megaloblastic anemia.

Hereditary orotic aciduria is a rare metabolic disease that results from a defect of uridine-5-monophosphate synthase (UMPS). In affected patients, main clinical symptoms are a markedly increased urinary excretion of orotic acid combined with megaloblastic anemia. This report describes a new case of UMPS deficiency without megaloblastic anemia but with epilepsy.

Unusual mesencephalic developmental venous anomaly causing obstructive hydrocephalus due to aqueductal stenosis.

Developmental venous anomalies (DVAs) are benign vascular malformations that rarely become symptomatic. They are anatomical variations of the venous drainage system and most are incidentally discovered. Mechanical (obstruction and compression of cerebral and neural structures) and flow-related pathological mechanisms have been described in rare cases of symptomatic DVAs. The authors present the case of a 10-month-old boy with a mesencephalic DVA compressing the aqueduct and causing occlusive hydrocephalus. Endoscopic inspection confirmed the venous malformation causing aqueductal stenosis. The authors successfully performed endoscopic third ventriculostomy, resulting in decrease in the size of the ventricles. At the 6-month follow-up after surgery, the patient had significantly progressed in his psychomotor development. One year postsurgery the patient is doing fine, with no neurological or developmental deficits.

[Results of an outpatient intervention for obese children and adolescents]. / Ergebnisse einer ambulanten Interventionsstudie bei adipösen Kindern und Jugendlichen.

BACKGROUND: Aim of this study was to examine effects of an outpatient intervention program for obese children. METHOD: 45 obese children were treated with a cognitive-behavioural intervention program (IG) and were compared with 21 children of a waiting list (WG). Weight, self esteem, emotional and behaviour problems were measured at the beginning (T0) and the end (of T1) of the intervention. Additionally, predictors of therapy success were examined. RESULTS: The intervention program lead to a moderate reduction of weight, whereas there was no weight reduction in the waiting group. Furthermore, self esteem increased and emotional problems decreased in the IG. Neither a positive family history of obesity nor the weight at T0 did predict treatment outcome. CONCLUSION: Obese children profited from the cognitive-behavioral intervention program, weight reduction was, however, only moderate. Therefore, specific predictors for therapy success should be evaluated in further studies.

[Obesity in childhood and adolescence--first results of a multimodal intervention study in Mecklenburg-Vorpommern]. / Adipositas im Kindes- und Jugendalter--Erste Ergebnisse einer multimodalen Interventionsstudie in Mecklenburg-Vorpommern.

The prevalence of obesity in children and young adults in Germany has dramatically increased during the last decades. We investigated the impact of an one year outpatient intervention including physical training, psychotherapy and nutrition counselling on psychological status and course of weight of 19 adolescents who were diagnosed with obesity (intervention group, IG). The following questions were of particular interest: Is there a difference between children with obesity (n = 19, mean age 12.4 years) and controls (without obesity and overweight, control group, CG, n = 38, matched for age and sex) regarding the extent of emotional and behavioural problems, self-esteem and physical complaints? Does the intervention lead to a decrease of these problems as rated by the adolescents and mothers? Can we find a significant reduction of the BMI-SDS after one year training in the IG? The group comparisons between the IG and the CG revealed lower feelings of self-esteem, more self rated physical complaints, higher values on measures of depression/anxiety and attention problems in the CBCL for children with obesity, compared with controls. After one year training adolescents' psychological well being increased significantly while the BMI-SDS only decreased moderately. We conclude that children with obesity can benefit from receiving outpatient intervention if psychological and medical aspects are considered.