[Transition from neuropediatrics to neurology in neuromuscular diseases]. / Transition von der Neuropädiatrie zur Neurologie bei neuromuskulären Erkrankungen.

BACKGROUND: Neuromuscular diseases in childhood, adolescence and adulthood are rare or very rare diseases and for many of them the prevalence and incidence are unknown. Causal therapies are currently used for individual disease entities only. Nevertheless, new genetic methods, a better understanding of the pathophysiology and multidisciplinary treatment concepts help to improve patient life expectancy and quality of life. As a result, more and more patients with an early disease onset reach adulthood and further care in adult medicine is necessary. This imposes new challenges particularly on neurology and the requirements for interdisciplinary cooperation in adult medicine are increased. OBJECTIVE: How can transition be made meaningful? Where do structural and content problems stand out? MATERIAL AND METHOD: Using the example of Duchenne muscular dystrophy, the content and structural requirements for transition are presented and important aspects and possible problems are pointed out. CONCLUSION: The transition process is complex and requires time and personnel resources. If carried out sensibly, it can lead to a better and more efficient care of patients in the long term and thus can also become economically more effective.

Cognitive training in Parkinson's disease reduces cognitive decline in the long term.

BACKGROUND AND PURPOSE: Patients with Parkinson's disease (PD) are at high risk for cognitive dysfunction. Non-pharmacological interventions have attracted increasing interest for enhancing PD patients' cognitive functions. METHODS: One-year follow-up data (T2 ) of a randomized controlled trial evaluating two 6-week cognitive trainings - a structured (NEUROvitalis, NV) and an unstructured (mentally fit, MF) program - compared with a waiting list control group (CG) in non-demented PD patients (Hoehn and Yahr I-III) are presented. Forty-seven PD patients were examined at T2 . Effects on overall cognitive functions (Mini-Mental State Examination and DemTect) were compared between all groups with repeated measurement analyses of variance. A combined score of the percentage change value from baseline (T0 ) to T2 was calculated to identify patients who retained or improved their cognitive state (responders). The risk of developing mild cognitive impairment (MCI) was analyzed. RESULTS: Significant time × treatment effects on overall cognitive functions were found for both training groups, each compared separately to the CG (DemTect, P < 0.05). Nine patients (56.3%) of the NV group, seven (41.2%) of the MF group and three (21.4%) of the CG were responders. Comparing NV to CG the odds ratio was 4.7 [95% confidence interval (0.8; 33.3)], and comparing MF to CG it was 2.6 [95% confidence interval (0.4; 17.4)]. MCI risk for patients without prior MCI was 40.0% in CG, 18.2% in MF and 18.2% in NV. The odds ratio was 3 comparing NV to CG, MF to CG. DISCUSSION: This study gives evidence that cognitive training may be effective to prevent cognitive decline and onset of MCI in PD patients.

[How to diagnose and treat limb apraxia]. / Diagnostik und Therapie der Gliedmabenapraxie.

Apraxia is a disorder of higher motor cognition. Deficits in imitating abstract and symbolic gestures as well as deficits in appropriate tool use are common apraxic symptoms which, importantly, cannot be explained by primary sensorimotor deficits alone. In spite of the relevance of apraxia for neurorehabilitation and the individual stroke patient's prognosis, apraxia is to date still too rarely diagnosed and treated. In this review the currently published assessments for the diagnosis of apraxia are evaluated. Based on this, an apraxia screening instrument as well as a diagnostic test for clinical use are recommended. In addition, different published approaches to the therapy for apraxia are described. Although current evidence is scarce, the gesture training suggested by Smania and co-workers can be recommended as a therapy for apraxia, because its effects were shown to extend to activities of daily living and to persist for at least two months after completion of the training. This review aims at directing the clinician's attention to the importance of apraxia. Moreover, it provides the interested reader with instruments for a reliable diagnosis and effective treatment of apraxia. These are also important prerequisites for further research into the neurobiological mechanisms underlying apraxia and the development of new therapy strategies leading to an evidence-based effective treatment of apraxia.