Person and ethics of a psychiatrist during National Socialism: Friedrich Meggendorfer (1880-1953).

Evaluation of sources not previously considered makes it possible to describe Friedrich Meggendorfer's role as a National Socialist university psychiatrist. Relevant archive material and literature were both assessed. The gene-hygiene affinity promulgated by Meggendorfer was based on his own scientific interests, early academic influences, and also positive reinforcement from his career choices. His application of scientific knowledge in the legitimization of National Socialist jurisdiction reflects a dark facet in Meggendorfer's life. One can also criticize his ethics in failing to use his eugenics expertise to stop 'euthanasia'. Future studies into the history of the ethical aspects of Nazi psychiatry should benefit from the setting up of criteria for the collection of biographical data. This would render comparisons and contrasts fairer and more stable.

Huntington disease: A quarter century of progress since the gene discovery.

Huntington disease (HD) is an autosomal dominant neurodegenerative disorder characterized by motor, behavioral, and cognitive manifestations. It is caused by an expansion of a trinucleotide repeat in the huntingtin gene (HTT) on chromosome 4. Although disease onset is currently clinically defined by motor signs, the presence of non-motor symptoms prior to motor diagnosis is increasingly recognized. Complex multimodal symptoms adversely affect quality of life and longevity of patients. Thoughtful interdisciplinary symptomatic care can make a major positive impact for patients and families. A variety of symptomatic treatments are currently available, and new symptomatic and potentially disease modifying therapies are being actively developed. Functional and quality of life outcome measures can be used to assess efficacy of clinical interventions. These outcomes along with clinical data and novel longitudinal biomarkers are increasingly utilized in clinical trials, particularly those testing disease-modifying therapeutics. Recent advances in novel therapeutic strategies, including targeting mutant huntingtin (HTT) and the HTT gene, promise another wave of disease-modifying trials in the near future. Better appreciation of heterogeneous clinical phenomenology and immediate tractable treatment goals coupled with advances in new therapeutics heralds a golden age of HD treatment that will positively impact quality of life and longevity of HD patients and inform advances in other inherited and neurodegenerative neurological disorders.

The Neuropathology of Huntington´s disease: classical findings, recent developments and correlation to functional neuroanatomy.

Huntington's disease (HD) is a severe, autosomal dominantly inherited, gradually worsening neurological disorder, the clinical features of which were first described in 1863 by Irving W. Lyon and with additional details, in 1872, by George Huntington. Progress in molecular biological research has shown that HD is caused by meiotically unstable CAG-repeats in the mutated HD gene (the so-called IT 15 gene) on chromosome 4p16.3, which encodes the mutated protein huntingtin (Htt). This monograph provides a survey of the stepwise progress in neuropathological HD research made during a time period of more than hundred years, the currently known neuropathological hallmarks of HD, as well as their pathogenic and clinical relevance. Starting with the initial descriptions of the progressive degeneration of the neostriatum (i.e., caudate nucleus and putamen) as one of the key events in HD, the worldwide practiced Vonsattel HD grading system of striatal neurodegeneration will be outlined. Correlating qualitative and quantitative neuropathological data with characteristics pertaining to the functional neuroanatomy of the human brain, subsequent chapters will highlight the latest neuropathological HD findings: the area- and layer-specifi c neuronal loss in the cerebral neo- and allocortex, the neurodegeneration of select thalamic nuclei, the affection of the cerebellar cortex and the deep cerebellar nuclei, the involvement of distinct brainstem nuclei, and the pathophysiological relevance of these pathologies for the clinical phenotype of HD. Finally, the potential pathophysiological role of axonal transport deficit

Witchcraft and Huntington's disease: a salutary history of societal and medical stigmatisation.

OBJECTIVE: This paper examines the notions that psychiatry can be greatly influenced by what society considers as 'normal', and that psychiatric thoughts and beliefs ebb and flow according to history and the social and cultural values of the time. CONCLUSIONS: As part of the medical profession, psychiatrists have much power in determining treatment and outcomes for patients. Unfortunately, this also means psychiatry has also been involved with the darker aspects of humanity, such as during the Nazi regime, and the abuse of patients' human rights. Huntington's disease (HD) is a neuropsychiatric illness from which observation and little knowledge reported by the medical profession spanned decades of incorrect and sensationalised documentation, that was also influenced by the values of the time. Such was the atmosphere of society during this period that the ideas and notions regarding HD disseminated by the respected medical profession were believed and accepted as fact by the general population and other professions, who would have been ignorant of any other contrary information. We need to be aware of social and cultural values as these can influence our understanding of diagnoses and treatments of our patients.

Huntington's disease: advocacy driving science.

My mother, Leonore, was diagnosed with Huntington's disease (HD) in 1968 at age 53. I was 23, my sister Alice 26, and our father, Milton Wexler, 60 years old. The same year, our father created the Hereditary Disease Foundation (HDF), dedicated to finding treatments and cures for HD. HD is an autosomal dominant, neurodegenerative disorder. Alice and I each have a 50% chance of inheriting and dying from the disorder. Over the past 43 years, we have been proud to change the face of science. Through Milton Wexler Interdisciplinary Workshops, judicious funding, and focusing on innovation and creativity, the HDF is an integral partner in key discoveries. The HDF recruited and supported >100 scientists worldwide who worked together as the Huntington's Disease Collaborative Research Group in a successful ten-year search for the HD gene. We found a DNA marker for the HD gene in 1983-the first marker to be found when the chromosomal location was unknown. We isolated the HD gene itself a decade later. These breakthroughs helped launch the Human Genome Project. We supported creating the first mouse model of HD and many other model systems. Currently, we focus on gene silencing, among other approaches, to create new treatments and cures.

Huntington's Disease - A Brief Historical Perspective.

Although the disease today known as Huntington's was described as early as the mid-19th century, knowledgeable physicians despaired of finding successful therapies and affected families largely kept it hidden. Starting in the late 1960 s, the confluence of grass-roots advocacy by HD family members, advances in Parkinson's treatment, and the development of molecular genetics and neuroscience helped turn HD into a focus of growing biomedical research. While therapies lag behind laboratory discoveries, disease altering interventions are now moving closer to the clinic. The Journal of Huntington's Disease is a welcome new resource in this effort.

Productivity and Impact of the Top 100 Cited Huntington's Disease Investigators.

Huntington's disease (HD), research has grown dramatically over the last 25 years of research to the point where an analysis of productivity is warranted. We have compiled a list of the 100 most-cited researchers in HD together with H-Indices as a means to assess productivity and impact over the last 25 years. We also present a table of the most cited researchers of the last decade for comparison.