Journal: Der Nervenarzt
Spinocerebellar ataxias are genetically heterogeneous autosomal dominant ataxia disorders. To date more than 30 different subtypes are known. In Germany particularly SCA1, SCA2, SCA3 and SCA6 are prevalent, as well as the less frequent subtypes SCA5, SCA14, SCA15, SCA17 and SCA28. Genetic causes range from coding repeat expansions (polyglutamine diseases), to non-coding expansions as well as conventional mutations. In some subtypes the genetic background is currently unknown. Age of onset, typical clinical findings and geographic distribution may help to reach a correct diagnosis; however a definitive diagnosis requires molecular genetic testing.
Psychosocial interventions improve cognitive abilities (cognitive stimulation, cognitive training), enhance emotional well-being (activity planning, reminiscence), reduce behavioral symptoms (aromatherapy, music therapy) and promote everyday functioning (occupational therapy). Through these effects they reinforce and augment pharmacological treatments for dementia. In addition, psychosocial interventions complement the treatment of patients by supporting family caregivers (educational groups, support programs). The potential of psychosocial interventions in dementia needs to be explored further in studies using improved methodology to determine effective components, clinical relevance and duration of effects, predictors of individual treatment response and health-economic implications.
Depressive disorders are common and lead to both individual suffering and high socioeconomic costs. Despite the fact that several effective therapies are available, remission is often not achieved. Electroconvulsive therapy (ECT) can be a therapeutic option in these cases.
The Forensic Therapeutic Outpatient Clinic (FTA) in Berlin targets the professional aftercare treatment of classified high-risk violent and sexual offenders released from prison or forensic psychiatric hospitals.
When the National Socialists (NS) came to power in 1933, the German health care system was reorganized according to the principles of eugenics. Neuropsychiatric patients were victims of compulsory sterilisation and “euthanasia”. As the Saar territory did not become part of the German Reich until the 1 March 1935, it is of special interest how quickly and completely NS health care policies were implemented.
Current international projections suggest that reducing the prevalence of seven well-established risk factors, midlife hypertension and obesity, diabetes mellitus, depression, physical inactivity, smoking and low educational attainment, may also substantially reduce the prevalence of Alzheimer’s dementia (AD).
Amyotrophic lateral sclerosis (ALS) is associated with an increased mortality. Knowledge of possible causes of death could lead to an individualization of the palliative treatment concept and result in a differentiated palliative treatment pathway. Currently, only few systematic data are available on the heterogeneity of causes of death associated with ALS.
This article reviews the results of longitudinal studies on frontal brain volume reduction in patients with schizophrenia spectrum disorders and focuses on the relationship with antipsychotic treatment. Based on a systematic literature search all studies were included in which results on changes of brain volumes over a longer period of time were correlated with antipsychotic treatment dose and disease severity. The findings indicate that there is evidence for grey and white matter volume changes of the frontal brain, which cannot be explained by the severity of the disease alone but are also very likely a manifestation of long-term effects of antipsychotics. Whether second generation antipsychotics have an advantage compared to first generation antipsychotics is currently unclear. Considering the contribution of antipsychotics to the changes in brain structure, which seem to depend on cumulative dosage and can exert adverse effects on neurocognition, negative and positive symptoms and psychosocial functioning, the guidelines for antipsychotic long-term drug treatment should be reconsidered. This is the reason why we and others recommend prescribing the lowest dose necessary to control symptoms. In non-schizophrenic psychiatric disorders, antipsychotics should be used only with great caution after a careful risk-benefit assessment. Moreover, treatment approaches which can help to minimize antipsychotic medication or even administer them only selectively are of increasing importance.
More than half of the patients diagnosed with schizophrenia and a subgroup of patients diagnosed with bipolar disorder show impairment in neurocognitive and social cognitive performance. The degree of impairment varies from person to person. An improvement of cognitive impairment results in increased subjective quality of life and increased psychosocial functioning, to a much greater extent than successful treatment of other symptoms. Therefore, it is reasonable to not only recognize and bear decreased cognitive functioning in mind but also to offer specific treatment of impairments. The systematic review article in hand provides an overview of practice relevant treatment options in this respect. At present, cognitive remediation therapy shows the best treatment results, although only with moderate effect sizes. Physical activity can also be considered beneficial, even with the smaller number of studies available. Specific psychopharmacotherapy and non-invasive neurostimulation are still being assessed in clinical trials but show a promising perspective. Even though there are various beneficial treatment options to improve cognitive impairment potentially apparent in schizophrenia and bipolar disorder, future research will hopefully bring about even more effective interventions.
The paraneoplastic and autoimmune encephalitides are now well-established entities. Detection of neural autoantibodies enables specific diagnoses, provides information on the underlying disease pathophysiology, immunological treatability and the likelihood of a tumor being the underlying cause. This is true for the “high ranking” neural antibodies that have been established in the context of circumscribed clinical images and in consideration of large control groups, have been found in the same way by other laboratories and they respond to immunotherapy. The immune reaction can be triggered by tumors and virus encephalitides, e.g. N‑methyl-D-aspartate (NMDA) receptor antibodies. In some cases a genetic predisposition has been shown. Some antibodies are formed peripherally, others intrathecally. The route of the antibodies into the brain can be via the blood-brain barrier or cerebrospinal fluid (CSF). In the brain itself, the antibodies lead to an internalization of antigenic receptors, such as NMDA and α‑amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptors, or to nerve-destroying activation of the classical complement cascade. In other conditions, cytotoxic T cells are at the core of the pathophysiology. For diagnostic purposes, the testing of CSF-serum pairs with broad spectrum antigen panels is recommended. Therapeutically, the aim is to suppress the production of pathogenic antibodies or even to eliminate them directly. A sequence of first-line treatment (steroids, intravenous immunoglobulins and/or apheresis) and second-line treatment (rituximab and/or cyclophosphamide) has been established.