185-850: Aktion T4 (German, pronounced [akˈtsi̯oːn teː fiːɐ] ) was a campaign of mass murder by involuntary euthanasia in Nazi Germany . The term was first used in post- war trials against doctors who had been involved in the killings. The name T4 is an abbreviation of Tiergartenstraße 4, a street address of the Chancellery department set up in early 1940, in the Berlin borough of Tiergarten , which recruited and paid personnel associated with Aktion T4. Certain German physicians were authorised to select patients "deemed incurably sick, after most critical medical examination" and then administer to them
370-1152: A Kripo officer who later played a prominent role in the Final Solution (extermination of Jews) as commandant of newly built death camps in occupied Poland. In addition to Brandenburg, the killing centres included Grafeneck Castle in Baden-Württemberg (10,824 dead), Schloss Hartheim near Linz in Austria (over 18,000 dead), Sonnenstein in Saxony (15,000 dead), Bernburg in Saxony-Anhalt and Hadamar in Hesse (14,494 dead). The same facilities were also used to kill mentally sound prisoners transferred from concentration camps in Germany, Austria and occupied parts of Poland. Condemned patients were transferred from their institutions to new centres in T4 Charitable Ambulance buses, called
555-457: A psychological examination , can determine whether the onset of the disease has begun. Excessive unintentional movements of any part of the body are often the reason for seeking medical consultation. If these are abrupt and have random timing and distribution, they suggest a diagnosis of HD. Cognitive or behavioral symptoms are rarely the first symptoms diagnosed; they are usually only recognized in hindsight or when they develop further. How far
740-516: A reduced display of emotions , egocentrism , aggression , and compulsive behavior and hallucination and delusion . Other common psychiatric disorders could include obsessive–compulsive disorder , mania , insomnia and bipolar disorder . Difficulties in recognizing other people's negative expressions have also been observed. The prevalence of these symptoms is highly variable between studies, with estimated rates for lifetime prevalence of psychiatric disorders between 33 and 76%. For many with
925-467: A trinucleotide repeat expansion – a short repeat , which varies in length between individuals, and may change length between generations. If the repeat is present in a healthy gene, a dynamic mutation may increase the repeat count and result in a defective gene. When the length of this repeated section reaches a certain threshold, it produces an altered form of the protein, called mutant huntingtin protein (mHtt). The differing functions of these proteins are
1110-420: A "mercy death" ( Gnadentod ). In October 1939, Adolf Hitler signed a "euthanasia note", backdated to 1 September 1939, which authorised his physician Karl Brandt and Reichsleiter Philipp Bouhler to begin the killing. The killings took place from September 1939 until the end of the war in 1945; from 275,000 to 300,000 people were killed in psychiatric hospitals in Germany and Austria, occupied Poland and
1295-466: A "trial" case in late 1938. Hitler instructed Brandt to evaluate a petition sent by two parents for the "mercy killing" of their son who was blind and had physical and developmental disabilities. The child, born near Leipzig and eventually identified as Gerhard Kretschmar , was killed in July 1939. Hitler instructed Brandt to proceed in the same manner in all similar cases. On 18 August 1939, three weeks after
1480-674: A Jewish invention". Bishop Heinrich Wienken of Berlin, a leading member of the Caritas Association , was selected by the Fulda episcopal synod to represent the views of the Catholic Church in meetings with T4 operatives. In 2008, Michael Burleigh wrote Wienken seems to have gone partially native in the sense that he gradually abandoned an absolute stance based on the Fifth Commandment in favour of winning limited concessions regarding
1665-427: A blood sample taken from the mother (via venipuncture ) between six and 12 weeks of pregnancy. It has no procedure-related risk of miscarriage. About 99% of HD diagnoses based on the typical symptoms and a family history of the disease are confirmed by genetic testing to have the expanded trinucleotide repeat that causes HD. Most of the remaining are called HD-like (HDL) syndromes . The cause of most HDL diseases
1850-492: A brief medical examination on arrival. They were induced to enter what appeared to be a shower block, where they were gassed with carbon monoxide (the ruse was also used at extermination camps). Some of the victims knew their fate and tried to defend themselves. The SS functionaries and hospital staff associated with Aktion T4 in the German Reich were paid from the central office at Tiergartenstraße 4 in Berlin from
2035-680: A child could be killed. The Ministry used deceit when dealing with parents or guardians, particularly in Catholic areas, where parents were generally uncooperative. Parents were told that their children were being sent to "Special Sections", where they would receive improved treatment. The children sent to these centres were kept for "assessment" for a few weeks and then killed by injection of toxic chemicals, typically phenol ; their deaths were recorded as " pneumonia ". Autopsies were usually performed and brain samples were taken to be used for "medical research". Post mortem examinations apparently helped to ease
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#17330862214912220-556: A concept to define certain large-scale killings of non-combatants by state actors. Some medium- and large-scale mass killings by state actors have been termed massacres , though not all such killings have been so named. The term " democide " was coined by American political scientist Rudolph Rummel to describe "murder by government" in general, which included both extrajudicial killings and widespread systematic acts of homicide. Killings committed by state actors might be called "murder" or "mass murder" in general usage, especially if seen by
2405-909: A diagnosis. The genetic test for HD consists of a blood test, which counts the numbers of CAG repeats in each of the HTT alleles. Cutoffs are given as follows: Testing before the onset of symptoms is a life-changing event and a very personal decision. The main reason given for choosing to test for HD is to aid in career and family decisions. Predictive testing for Huntington's disease has been available via linkage analysis (which requires testing multiple family members) since 1986 and via direct mutation analysis since 1993. At that time, surveys indicated that 50–70% of at-risk individuals would have been interested in receiving testing, but since predictive testing has been offered far fewer choose to be tested. Over 95% of individuals at risk of inheriting HD do not proceed with testing, mostly because it has no treatment. A key issue
2590-472: A family history of HD, excluding those who died of other causes before diagnosis. These cases can now be included in statistics; and, as the test becomes more widely available, estimates of the prevalence and incidence of the disorder are likely to increase. In centuries past, various kinds of chorea were at times called by names such as Saint Vitus' dance , with little or no understanding of their cause or type in each case. The first definite mention of HD
2775-660: A faster rate with greater cognitive decline, and chorea is exhibited briefly, if at all; the Westphal variant of slowness of movement , rigidity, and tremors is more typical in juvenile HD, as are seizures . Cognitive abilities are progressively impaired and tend to generally decline into dementia . Especially affected are executive functions , which include planning, cognitive flexibility, abstract thinking , rule acquisition, initiation of appropriate actions, and inhibition of inappropriate actions. Different cognitive impairments include difficulty focusing on tasks, lack of flexibility,
2960-551: A heavier form of tetrabenazine medication for the treatment of chorea in HD, was approved by the FDA. This is marketed as Austedo. Psychiatric symptoms can be treated with medications similar to those used in the general population. Selective serotonin reuptake inhibitors and mirtazapine have been recommended for depression, while atypical antipsychotics are recommended for psychosis and behavioral problems. Specialist neuropsychiatric input
3145-537: A house or apartment was the most common location of homicide (41%); followed by a street or highway (22%); a motor vehicle (10%); and a parking lot, public garage, or public transport (4.5%). Precipitating circumstances were identified in 69% of homicides. One-third of homicides with known circumstances were precipitated by an argument or conflict (34%), and 15% of homicides with known circumstances were related to intimate partner violence . Homicides also were commonly precipitated by another crime (23%); in 66% of those cases,
3330-498: A lack of impulse, a lack of awareness of one's own behaviors and abilities and difficulty learning or processing new information. As the disease progresses, memory deficits tend to appear. Reported impairments range from short-term memory deficits to long-term memory difficulties, including deficits in episodic (memory of one's life), procedural (memory of the body of how to perform an activity), and working memory . Reported neuropsychiatric signs are anxiety , depression ,
3515-572: A list of conditions. The conditions included schizophrenia, epilepsy, Huntington's chorea , advanced syphilis , senile dementia , paralysis , encephalitis and "terminal neurological conditions generally". Many doctors and administrators assumed that the reports were to identify inmates who were capable of being drafted for "labour service" and tended to overstate the degree of incapacity of their patients, to protect them from labour conscription. When some institutions refused to co-operate, teams of T4 doctors (or Nazi medical students) visited and compiled
3700-424: A local founder effect , a historical migration of carriers into an area of geographic isolation . Some of these carriers have been traced back hundreds of years using genealogical studies. Genetic haplotypes can also give clues for the geographic variations of prevalence. Iceland , on the contrary, has a rather low prevalence of 1 per 100,000, despite the fact that Icelanders as a people are descended from
3885-463: A mental health facility and people with simple physical disabilities. New insulin shock treatments were used by German psychiatrists to find out if patients with schizophrenia were curable. Karl Brandt , doctor to Hitler and Hans Lammers , the head of the Reich Chancellery, testified after the war that Hitler had told them as early as 1933—when the sterilisation law was passed—that he favoured
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#17330862214914070-665: A near "crisis point" in Central America and the Caribbean . Of all homicides worldwide, 82 percent of the victims were men, and 18 percent were women. On a per-capita scaled level, "the homicide rate in Africa and the Americas (at 17 and 16 per 100,000 population, respectively) is more than double the global average (6.9 per 100,000), whereas in Asia, Europe and Oceania (between 3 and 4 per 100,000) it
4255-401: A new mutation. The huntingtin gene provides the genetic information for huntingtin protein (Htt). Expansion of CAG repeats of cytosine - adenine - guanine (known as a trinucleotide repeat expansion ) in the gene coding for the huntingtin protein results in an abnormal mutant protein (mHtt), which gradually damages brain cells through a number of possible mechanisms. The mutant protein
4440-534: A nurse and mother of a child with a disability, vehemently petitioned to Hermann Linden at the Reich Ministry of the Interior in Berlin to prevent her son, Alfred, from being transferred from Gugging, where he lived and which also became a euthanasia center. Wödl failed and Alfred was sent to Am Spiegelgrund , where he was killed on 22 February 1941. His brain was preserved in formaldehyde for "research" and stored in
4625-487: A secluded mountain valley in Norway , the high prevalence of dementia was associated with a pattern of jerking movement disorders that ran in families. The first thorough description of the disease was by George Huntington in 1872. Examining the combined medical history of several generations of a family exhibiting similar symptoms, he realized their conditions must be linked; he presented his detailed and accurate definition of
4810-412: A second mutated gene did not affect symptoms and progression, but it has since been found that it can affect the phenotype and the rate of progression. Huntingtin protein interacts with over 100 other proteins, and appears to have multiple functions. The behavior of the mutated protein (mHtt) is not completely understood, but it is toxic to certain cell types, particularly brain cells . Early damage
4995-518: A time. On each they marked a + (death), a - (life), or occasionally a ? meaning that they were unable to decide. Three "death" verdicts condemned the person and as with reviews of children, the process became less rigorous, the range of conditions considered "unsustainable" grew broader and zealous Nazis further down the chain of command increasingly made decisions on their own initiative. The first gassings in Germany proper took place in January 1940 at
5180-438: A transcriptional coregulator, is essential for cell function because as a coactivator at a significant number of promoters, it activates the transcription of genes for survival pathways. CBP contains an acetyltransferase domain to which HTT binds through its polyglutamine-containing domain. Autopsied brains of those who had Huntington's disease also have been found to have incredibly reduced amounts of CBP. In addition, when CBP
5365-420: A tremor. Common consequences are physical instability, abnormal facial expression, and difficulties chewing, swallowing , and speaking . Sleep disturbances and weight loss are also associated symptoms. Eating difficulties commonly cause weight loss and may lead to malnutrition. Weight loss is common in people with Huntington's disease, and it progresses with the disease. Juvenile HD generally progresses at
5550-482: A typically 4- to 8-cell embryo and then tested for the genetic abnormality, can then be used to ensure embryos affected with HD genes are not implanted, so any offspring will not inherit the disease. Some forms of preimplantation genetic diagnosis—non-disclosure or exclusion testing—allow at-risk people to have HD-free offspring without revealing their own parental genotype, giving no information about whether they themselves are destined to develop HD. In exclusion testing,
5735-528: Is McLeod syndrome . Treatments are available to reduce the severity of some HD symptoms. For many of these treatments, evidence to confirm their effectiveness in treating symptoms of HD specifically are incomplete. As the disease progresses, the ability to care for oneself declines, and carefully managed multidisciplinary caregiving becomes increasingly necessary. Although relatively few studies of exercises and therapies have shown to be helpful to rehabilitate cognitive symptoms of HD, some evidence shows
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5920-507: Is dominant , so having one parent who is a carrier of the trait is sufficient to trigger the disease in their children. Diagnosis is by genetic testing , which can be carried out at any time, regardless of whether or not symptoms are present. This fact raises several ethical debates: the age at which an individual is considered mature enough to choose testing; whether parents have the right to have their children tested; and managing confidentiality and disclosure of test results. No cure for HD
6105-415: Is expressed in all cells, with the highest concentrations found in the brain and testes , and moderate amounts in the liver , heart , and lungs . Its functions are unclear, but it does interact with proteins involved in transcription , cell signaling , and intracellular transporting . In animals genetically modified to exhibit HD, several functions of Htt have been identified. In these animals, Htt
6290-568: Is pneumonia , which causes death in one third of those with HD. As the ability to synchronize movements deteriorates, difficulty clearing the lungs, and an increased risk of aspirating food or drink both increase the risk of contracting pneumonia . The second-greatest risk is heart disease , which causes almost a quarter of fatalities of those with HD. Suicide is the third greatest cause of fatalities, with 7.3% of those with HD taking their own lives and up to 27% attempting to do so. To what extent suicidal thoughts are influenced by behavioral symptoms
6475-434: Is 5–10 cases per 100,000 persons, but varies greatly geographically as a result of ethnicity, local migration and past immigration patterns. Prevalence is similar for men and women. The rate of occurrence is highest in peoples of Western European descent, averaging around seven per 100,000 people, and is lower in the rest of the world; e.g., one per million people of Asian and African descent. A 2013 epidemiological study of
6660-419: Is Huntington's disease caused by a new mutation , where neither parent has over 36 CAG repeats. In the rare situations where both parents have an expanded HD gene, the risk increases to 75%, and when either parent has two expanded copies, the risk is 100% (all children will be affected). Individuals with both genes affected are rare. For some time, HD was thought to be the only disease for which possession of
6845-685: Is a centralized database of relevant information from death certificates , coroner and medical examiner records, and law enforcement reports, which emerged from the National Violent Injury Statistics System . This public health surveillance tool began collecting data in 2003 and is analyzed by the National Center for Injury Prevention and Control at the CDC to provide nationally representative data. In 2020, there were 18,439 cases of single homicide (28.6% of all violent deaths) in
7030-582: Is also a basal ganglia disease causing a hyperkinetic movement disorder known as chorea . As the disease advances, uncoordinated, involuntary body movements of chorea become more apparent. Physical abilities gradually worsen until coordinated movement becomes difficult and the person is unable to talk. Mental abilities generally decline into dementia , depression, apathy, and impulsivity at times. The specific symptoms vary somewhat between people. Symptoms usually begin between 30 and 50 years of age, and can start at any age but are usually seen around
7215-487: Is also possible, using fetal genetic material acquired through chorionic villus sampling . An amniocentesis can be performed if the pregnancy is further along, within 14–18 weeks. This procedure looks at the amniotic fluid surrounding the baby for indicators of the HD mutation. This, too, can be paired with exclusion testing to avoid disclosure of parental genotype. Prenatal testing can be done when parents have been diagnosed with HD, when they have had genetic testing showing
7400-497: Is an act in which a person causes the death of another person . A homicide requires only a volitional act, or an omission, that causes the death of another, and thus a homicide may result from accidental , reckless , or negligent acts even if there is no intent to cause harm. It is separate from suicide . Homicides can be divided into many overlapping legal categories, such as murder , manslaughter , justifiable homicide , assassination , killing in war (either following
7585-435: Is commonly cited as the ultimate cause of death for those with the condition. Suicide is the cause of death in about 9% of cases. Death typically occurs 15–20 years from when the disease was first detected. The earliest known description of the disease was in 1841 by American physician Charles Oscar Waters. The condition was described in further detail in 1872 by American physician George Huntington . The genetic basis
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7770-487: Is divided into two broad categories— murder and manslaughter —based upon the state of mind and intent of the person who commits the homicide. A report issued by the United Nations Office on Drugs and Crime in July 2019 documented that nearly 464,000 people around the world were killed in homicides in 2017, a number significantly in excess of the 89,000 killed in armed conflicts during the same period. Murder
7955-466: Is due to environmental factors and other genes that influence the mechanism of the disease. Life expectancy in HD is generally around 10 to 30 years following the onset of visible symptoms. Juvenile Huntington's disease has a life expectancy rate of 10 years after onset of visible symptoms. Most life-threatening complications result from muscle coordination, and to a lesser extent, behavioral changes induced by declining cognitive function. The largest risk
8140-536: Is generally suppressed, then unless we will get new measures our race must rapidly deteriorate. Within the Nazi administration, the idea of including in the programme people with physical disabilities had to be expressed carefully, because the Reich Minister of Propaganda , Joseph Goebbels , had a deformed right leg. After 1937, the acute shortage of labour in Germany arising from rearmament, meant that anyone capable of work
8325-602: Is important for embryonic development, as its absence is related to embryonic death. Caspase , an enzyme which plays a role in catalyzing apoptosis , is thought to be activated by the mutated gene through damaging the ubiquitin-protease system. It also acts as an antiapoptotic agent preventing programmed cell death and controls the production of brain-derived neurotrophic factor , a protein that protects neurons and regulates their creation during neurogenesis . Htt also facilitates synaptic vesicular transport and synaptic transmission , and controls neuronal gene transcription. If
8510-489: Is information concerning family planning choices, care management, and other considerations. The length of the trinucleotide repeat accounts for 60% of the variation of the age of symptoms onset and their rate of progress. A longer repeat results in an earlier age of onset and a faster progression of symptoms. Individuals with more than sixty repeats often develop the disease before age 20, while those with fewer than 40 repeats may remain asymptomatic. The remaining variation
8695-622: Is known, and full-time care is required in the later stages. Treatments can relieve some symptoms and in some, improve quality of life . The best evidence for treatment of the movement problems is with tetrabenazine . HD affects about 4 to 15 in 100,000 people of European descent. It is rare among the Finnish and Japanese, while the occurrence rate in Africa is unknown. The disease affects males and females equally. Complications such as pneumonia , heart disease , and physical injury from falls reduce life expectancy; although fatal aspiration pneumonia
8880-418: Is most evident in the subcortical basal ganglia , initially in the striatum , but as the disease progresses, other areas of the brain are also affected, including regions of the cerebral cortex . Early symptoms are attributable to functions of the striatum and its cortical connections—namely control over movement, mood, and higher cognitive function. DNA methylation also appears to be changed in HD. Htt
9065-511: Is normally less than the penalty for murder. The two broad categories of manslaughter are: Another form of manslaughter in some jurisdictions is constructive manslaughter , which may be charged if a person causes a death without intention but as the result of violating an important safety law or regulation. Not all homicides are crimes, or subject to criminal prosecution. Some are legally privileged, meaning that they are not criminal acts at all. Others may occur under circumstances that provide
9250-477: Is not found in HD, but the interactions of the altered huntingtin protein with numerous proteins in neurons lead to an increased vulnerability to glutamine. The increased vulnerability is thought to result in excitotoxic effects from normal glutamine levels. Initially, damage to the brain is regionally specific with the dorsal striatum in the subcortical basal ganglia being primarily affected, followed later by cortical involvement in all areas. Other areas of
9435-439: Is one of several trinucleotide repeat disorders that are caused by the length of a repeated section of a gene exceeding a normal range. The HTT gene is located on the short arm of chromosome 4 at 4p16.3. HTT contains a sequence of three DNA bases —cytosine-adenine-guanine (CAG)—repeated multiple times (i.e. ... CAGCAGCAG ...), known as a trinucleotide repeat. CAG is the three-letter genetic code ( codon ) for
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#17330862214919620-549: Is overexpressed, polyglutamine-induced death is diminished, further demonstrating that CBP plays an important role in Huntington's disease and neurons in general. Diagnosis of the onset of HD can be made following the appearance of physical symptoms specific to the disease. Genetic testing can be used to confirm a physical diagnosis if no family history of HD exists. Even before the onset of symptoms, genetic testing can confirm if an individual or embryo carries an expanded copy of
9805-399: Is recommended since people may require long-term treatment with multiple medications in combination. There has been a number of alternative therapies experimented in ayurvedic medicine with plant-based products, although none have provided good evidence of efficacy. A recent study showed that the stromal processing peptidase (SPP), a synthetic enzyme found in plant chloroplasts , prevented
9990-444: Is recommended. People with Huntington's disease may see a physical therapist for noninvasive and nonmedication-based ways of managing the physical symptoms. Physical therapists may implement fall risk assessment and prevention, as well as strengthening, stretching, and cardiovascular exercises. Walking aids may be prescribed as appropriate. Physical therapists also prescribe breathing exercises and airway clearance techniques with
10175-517: Is roughly half". In its 2013 global report, UNODC estimated the total number of homicides worldwide had dropped to 437,000 in 2012. The Americas accounted for 36 percent of all homicides globally, Africa 21 percent, Asia 38 percent, Europe five percent and Oceania 0.3%. The world's average homicide rate stood at 6.2 per 100,000 population in 2012, but the Southern Africa region and Central America had intentional homicide rates four times higher than
10360-486: Is the anxiety an individual experiences about not knowing whether they will eventually develop HD, compared to the impact of a positive result. Irrespective of the result, stress levels are lower two years after being tested, but the risk of suicide is increased after a positive test result. Individuals found to have not inherited the disorder may experience survivor guilt about family members who are affected. Other factors taken into account when considering testing include
10545-414: Is the most serious crime that can be charged following a homicide. In many jurisdictions, murder may be punished by life in prison or even capital punishment . Although categories of murder can vary by jurisdiction, murder charges fall under two broad categories , or degrees : In some jurisdictions, a homicide that occurs during the commission of a dangerous crime may constitute murder, regardless of
10730-464: Is unclear, as they signify a desire to avoid the later stages of the disease. Suicide is the greatest risk of this disease before the diagnosis is made and in the middle stages of development throughout the disease. Other associated risks include choking; due to the inability to swallow, physical injury from falls, and malnutrition. The late onset of Huntington's disease means it does not usually affect reproduction. The worldwide prevalence of HD
10915-697: Is unknown, but those with known causes are due to mutations in the prion protein gene (HDL1), the junctophilin 3 gene (HDL2), a recessively inherited unknown gene (HDL3—only found in two families and poorly understood), and the gene encoding the TATA box-binding protein ( SCA17, sometimes called HDL4 ). Other autosomal dominant diseases that can be misdiagnosed as HD are dentatorubral-pallidoluysian atrophy and neuroferritinopathy . Also, some autosomal recessive disorders resemble sporadic cases of HD. These include chorea acanthocytosis and pantothenate kinase-associated neurodegeneration . One X-linked disorder of this type
11100-544: The Algerian War and Soviet–Afghan War , terrorist mass killings such as strategic bombing during World War II and the blockade of Biafra , and German and Japanese imperialism during World War II as examples of imperialist mass killings. A 2011 study by the United Nations Office on Drugs and Crime brought together a wide variety of data sources to create a worldwide picture of trends and developments. Sources included multiple agencies and field offices of
11285-717: The Danzig (now Gdańsk ) area, some 7,000 Polish patients of various institutions were shot and 10,000 were killed in the Gdynia area. Similar measures were taken in other areas of Poland destined for incorporation into Germany. The first experiments with the gassing of patients were conducted in October 1939 at Fort VII in Posen (occupied Poznań), where hundreds of prisoners were killed by means of carbon monoxide poisoning, in an improvised gas chamber developed by Albert Widmann , chief chemist of
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#173308622149111470-693: The Holodomor , Great Leap Forward and Cambodian genocide for communist mass killings, the White Terror , the Holocaust and Dirty War for fascist mass killings, the Armenian and Rwandan genocides for ethnic mass killings and the American Indian Wars and Herero and Nama genocide for territorial mass killings. He also included examples of coercive mass killings, including counterinsurgency killings during
11655-604: The Protectorate of Bohemia and Moravia (now the Czech Republic). The number of victims was originally recorded as 70,273 but this number has been increased by the discovery of victims listed in the archives of the former East Germany . About half of those killed were taken from church-run asylums, often with the approval of the Protestant or Catholic authorities of the institutions. The Holy See announced on 2 December 1940 that
11840-611: The Royal Air Force over German troops. In 2009, Richard J. Evans wrote that "This was the strongest, most explicit and most widespread protest movement against any policy since the beginning of the Third Reich". Local Nazis asked for Galen to be arrested but Goebbels told Hitler that such action would provoke a revolt in Westphalia and Hitler decided to wait until after the war to take revenge. In 1986, Lifton wrote, "Nazi leaders faced
12025-868: The United Nations , the World Health Organization , and national and international sources from 207 countries. The report estimated that in 2010, the total number of homicides globally was 468,000. More than a third (36%) occurred in Africa , 31 percent in the Americas , 27 percent in Asia , five percent in Europe and one percent in Oceania . Since 1995, the homicide rate has been falling in Europe, North America, and Asia, but has risen to
12210-470: The University of Paderborn , Joseph Mayer, on the likely reactions of the churches in the event of a state euthanasia programme being instituted. Mayer – a longstanding euthanasia advocate – reported that the churches would not oppose such a programme if it was seen to be in the national interest. Brack showed this paper to Hitler in July and it may have increased his confidence that
12395-416: The amino acid glutamine , so a series of them results in the production of a chain of glutamine known as a polyglutamine tract (or polyQ tract), and the repeated part of the gene, the polyQ region . Generally, people have fewer than 36 repeated glutamines in the polyQ region, which results in the production of the cytoplasmic protein huntingtin. However, a sequence of 36 or more glutamines results in
12580-451: The basal ganglia affected include the substantia nigra ; cortical involvement includes cortical layers 3, 5, and 6 ; also evident is involvement of the hippocampus , Purkinje cells in the cerebellum , lateral tuberal nuclei of the hypothalamus and parts of the thalamus . These areas are affected according to their structure and the types of neurons they contain, reducing in size as they lose cells. Striatal medium spiny neurons are
12765-413: The laws of war or as a war crime ), euthanasia , and capital punishment , depending on the circumstances of the death. These different types of homicides are often treated very differently in human societies ; some are considered crimes , while others are permitted or even ordered by the legal system . Criminal homicide takes many forms, including accidental killing and murder. Criminal homicide
12950-597: The " Law for the Prevention of Hereditarily Diseased Offspring " prescribed compulsory sterilisation for people with conditions thought to be hereditary, such as schizophrenia, epilepsy , Huntington's chorea and "imbecility". Sterilisation was also legalised for chronic alcoholism and other forms of social deviance. The law was administered by the Interior Ministry under Wilhelm Frick through special Hereditary Health Courts ( Erbgesundheitsgerichte ), which examined
13135-412: The "euthanasia" decree, in a 1939 conference with Leonardo Conti , Reich Health Leader and State Secretary for Health in the Interior Ministry, and Hans Lammers, Chief of the Reich Chancellery, Hitler gave as examples the mentally ill who he said could only be "bedded on sawdust or sand" because they "perpetually dirtied themselves" and "put their own excrement into their mouths". This issue, according to
13320-553: The "euthanasia" programme would be acceptable to German public opinion. Notably, when Sereny interviewed Mayer shortly before his death in 1967, he denied that he formally condoned the killing of people with disabilities but no copies of this paper are known to survive. Some bureaucrats opposed the T4 programme; Lothar Kreyssig , a district judge and member of the Confessing Church , wrote to Justice Minister Franz Gürtner protesting that
13505-453: The 20th century, the homicide rate fell to 1.4 per 100,000. Police forces seldom existed outside the cities; prisons only became common after 1800. Before then, harsh penalties were imposed for homicide (severe whipping or execution) but they proved ineffective at controlling or reducing the insults to honor that precipitated most of the violence. The decline does not correlate with economics or measures of state control. Most historians attribute
13690-513: The 48 states and DC, a rate of 6.7 per 100,000 inhabitants. There were 695 cases of multiple homicide (1%) and 571 cases (<1%) of homicide followed by suicide with an overall homicide rate of 7.5 per 100,000 population. The weapons most commonly used in homicides were firearms, used in 76.7% of homicides overall; followed by a sharp instrument (9%); a blunt instrument (3%); personal weapons (e.g., hands, feet, or fists; 2.5%); and hanging, strangulation, or suffocation (1.5%). Among all homicide victims,
13875-506: The Brandenburg Euthanasia Centre. The operation was headed by Brack, who said "the needle belongs in the hand of the doctor". Bottled pure carbon monoxide gas was used. At trials, Brandt described the process as a "major advance in medical history". Once the efficacy of the method was confirmed, it became standard and was instituted at a number of centres in Germany under the supervision of Widmann, Becker and Christian Wirth –
14060-549: The Community Patients Transports Service. They were run by teams of SS men wearing white coats, to give it an air of medical care. To prevent the families and doctors of the patients from tracing them, the patients were often first sent to transit centres in major hospitals, where they were supposedly assessed. They were moved again to special treatment ( Sonderbehandlung ) centres. Families were sent letters explaining that owing to wartime regulations, it
14245-584: The Gaubschat GmbH in Berlin and before February 1942, killed 3,830 Polish Jews and around 4,000 Romani , under the guise of "resettlement". After the Wannsee conference , implementation of gassing technology was accelerated by Heydrich. Beginning in the spring of 1942, three killing factories were built secretly in east-central Poland. The SS officers responsible for the earlier Aktion T4 , including Wirth, Stangl and Irmfried Eberl , had important roles in
14430-547: The German Criminal Police (Kripo). In December 1939, Reichsführer-SS Heinrich Himmler witnessed one of these gassings, ensuring that this invention would later be put to much wider uses. The idea of killing adult mental patients soon spread from occupied Poland to adjoining areas of Germany, probably because Nazi Party and SS officers in these areas were most familiar with what was happening in Poland. These were also
14615-463: The Gestapo. It is a terrible, unjust and catastrophic thing when man opposes his will to the will of God ... We are talking about men and women, our compatriots, our brothers and sisters. Poor unproductive people if you wish, but does this mean that they have lost their right to live? Galen's sermons were not reported in the German press but were circulated illegally in leaflets. The text was dropped by
14800-452: The HD pathology through multiple cellular changes. In its mutant (polyglutamine expanded) form, the protein is more prone to cleavage that creates shorter fragments containing the polyglutamine expansion. These protein fragments have a propensity to undergo misfolding and aggregation, yielding fibrillar aggregates in which non-native polyglutamine β-strands from multiple proteins are bonded together by hydrogen bonds. These aggregates share
14985-484: The Holocaust. As a related aspect of the "medical" and scientific basis of this programme, the Nazi doctors took thousands of brains from 'euthanasia' victims for research. From August 1939, the Interior Ministry registered children with disabilities, requiring doctors and midwives to report all cases of newborns with severe disabilities; the 'guardian' consent element soon disappeared. Those to be killed were identified as "all children under three years of age in whom any of
15170-561: The Nazi appropriation of Church property in Münster to accommodate people made homeless by an air raid, in July and August 1941, the bishop of Münster , Clemens August Graf von Galen , gave four sermons criticising the Nazis for arresting Jesuits , confiscating church property and for the euthanasia program. Galen sent the text to Hitler by telegram, calling on ... the Führer to defend the people against
15355-623: The Nazi regime with enthusiasm. Many were appointed to positions in the Health Ministry and German research institutes. Their ideas were gradually adopted by the majority of the German medical profession, from which Jewish and communist doctors were soon purged. During the 1930s, the Nazi Party had carried out a campaign of propaganda in favour of euthanasia. The National Socialist Racial and Political Office (NSRPA) produced leaflets, posters and short films to be shown in cinemas, pointing out to Germans
15540-469: The Nazi regime, assumed a new urgency in wartime. After the invasion of Poland , Hermann Pfannmüller (Head of the State Hospital near Munich ) said It is unbearable to me that the flower of our youth must lose their lives at the front, so that that feeble-minded and asocial element can have a secure existence in the asylum. Pfannmüller advocated killing by a gradual decrease of food, which he believed
15725-510: The SS men of Einsatzkommando 16, Selbstschutz and EK-Einmann under the command of SS- Sturmbannführer Rudolf Tröger, overseen by Reinhard Heydrich , during Operation Tannenberg . All hospitals and mental asylums of the Wartheland were emptied. The region was incorporated into Germany and earmarked for resettlement by Volksdeutsche following the German conquest of Poland. In
15910-491: The T4 programme. Hitler wrote a confidential letter in October 1939 to overcome opposition within the German state bureaucracy. Hitler told Bouhler that, "the Führer's Chancellery must under no circumstances be seen to be active in this matter". Gürtner had to be shown Hitler's letter in August 1940 to gain his co-operation. In the towns where the killing centres were located, some people saw the inmates arrive in buses, saw smoke from
16095-442: The action was illegal since no law or formal decree from Hitler had authorised it. Gürtner replied, "If you cannot recognise the will of the Führer as a source of law, then you cannot remain a judge" and had Kreyssig dismissed. Hitler had a policy of not issuing written instructions for matters which could later be condemned by the international community but made an exception when he provided Bouhler and Brack with written authority for
16280-406: The actions of a co-defendant or third party who is reacting to the crime. The preterintentional killing occurs when a person, with actions aimed at hitting or harming, unintentionally causes the death of a person: the agent will be liable for objective responsibility (or fault, for the laws that require it) for the death event. Manslaughter is a form of homicide in which the person who commits
16465-471: The actor's intent to commit homicide. In the United States , this is known as the felony murder rule . In simple terms, under the felony murder rule a person who commits a felony may be guilty of murder if someone dies as a result of the commission of the crime, including the victim of the felony, a bystander or a co-felon, regardless their intent—or lack thereof—to kill, and even when the death results from
16650-457: The advanced stages of the disease. Functional neuroimaging techniques, such as functional magnetic resonance imaging (fMRI) and positron emission tomography (PET), can show changes in brain activity before the onset of physical symptoms, but they are experimental tools and are not used clinically. Because HD follows an autosomal dominant pattern of inheritance, a strong motivation exists for individuals who are at risk of inheriting it to seek
16835-429: The age of 20, known as juvenile HD. Juvenile HD is typically of the Westphal variant that is characterized by slowness of movement, rigidity, and tremors. This accounts for about 7% of HD carriers. Huntington's disease has autosomal dominant inheritance, meaning that an affected individual typically inherits one copy of the gene with an expanded trinucleotide repeat (the mutant allele ) from an affected parent. Since
17020-447: The age of 40. The disease may develop earlier in each successive generation . About eight percent of cases start before the age of 20 years, and are known as juvenile HD , which typically present with the slow movement symptoms of Parkinson's disease rather than those of chorea. HD is typically inherited from an affected parent , who carries a mutation in the huntingtin gene ( HTT ). However, up to 10% of cases are due to
17205-420: The age of the onset of symptoms. The remaining variation is attributed to the environment and other genes that modify the mechanism of HD. About 36 to 39 repeats result in a reduced-penetrance form of the disease, with a much later onset and slower progression of symptoms. In some cases, the onset may be so late that symptoms are never noticed. With very large repeat counts (more than 60), HD onset can occur below
17390-796: The aggregation of proteins associated with Huntington's disease. However, repeat studies and clinical validation are needed to confirm its true therapeutic potential. The families of individuals, and society at large, who have inherited or are at risk of inheriting HD have generations of experience of HD but may be unaware of recent breakthroughs in understanding the disease, and of the availability of genetic testing. Genetic counseling benefits these individuals by updating their knowledge, seeking to dispel any unfounded beliefs that they may have, and helping them consider their future options and plans. The Patient Education Program for Huntington's Disease has been created to help educate family members, caretakers, and those diagnosed with Huntington's disease. Also covered
17575-537: The areas where Germans wounded from the Polish campaign were expected to be accommodated, which created a demand for hospital space. The Gauleiter of Pomerania , Franz Schwede-Coburg , sent 1,400 patients from five Pomeranian hospitals to undisclosed locations in occupied Poland, where they were shot. The Gauleiter of East Prussia , Erich Koch , had 1,600 patients killed out of sight. More than 8,000 Germans were killed in this initial wave of killings carried out on
17760-438: The beginning of the Third Reich", according to Richard J. Evans . Several reasons have been suggested for the killings, including eugenics , racial hygiene , and saving money. Physicians in German and Austrian asylums continued many of the practices of Aktion T4 until the defeat of Germany in 1945, in spite of its official cessation in August 1941. The informal continuation of the policy led to 93,521 "beds emptied" by
17945-568: The best young men died in war, causing a loss to the Volk of the best genes. The genes of those who did not fight (the worst genes) then proliferated freely, accelerating biological and cultural degeneration". The advocacy of eugenics in Germany gained ground after 1930, when the Depression was used to excuse cuts in funding to state mental hospitals, creating squalor and overcrowding. Many German eugenicists were nationalists and antisemites , who embraced
18130-450: The brain. These abnormalities include muscle atrophy , cardiac failure , impaired glucose tolerance , weight loss , osteoporosis , and testicular atrophy . Everyone has two copies of the huntingtin gene ( HTT ), which codes for the huntingtin protein (Htt). HTT is also called the HD gene, and the IT15 gene, (interesting transcript 15). Part of this gene is a repeated section called
18315-402: The cause of pathological changes, which in turn cause the disease symptoms. The Huntington's disease mutation is genetically dominant and almost fully penetrant ; mutation of either of a person's HTT alleles causes the disease. It is not inherited according to sex, but by the length of the repeated section of the gene; hence its severity can be influenced by the sex of the affected parent. HD
18500-495: The cell, but other experiments have shown that they may form as part of the body's defense mechanism and help protect cells. Several pathways by which mHtt may cause cell death have been identified. These include effects on chaperone proteins , which help fold proteins and remove misfolded ones; interactions with caspases , which play a role in the process of removing cells ; the toxic effects of glutamine on nerve cells ; impairment of energy production within cells; and effects on
18685-519: The centres continued to be used to kill concentration camp inmates: eventually some 20,000 people in this category were killed. In 1971, Gitta Sereny conducted interviews with Stangl, who was in prison in Düsseldorf , having been convicted of co-responsibility for killing 900,000 people, while commandant of the Sobibor and Treblinka extermination camps in Poland. Stangl gave Sereny a detailed account of
18870-697: The child-killing programme, such as Unger, Heinze and Hermann Pfannmüller. The recruits were mostly psychiatrists, notably Professor Carl Schneider of Heidelberg, Professor Max de Crinis of Berlin and Professor Paul Nitsche from the Sonnenstein state institution. Heyde became the operational leader of the programme, succeeded later by Nitsche. In early October, all hospitals, nursing homes, old-age homes and sanatoria were required to report all patients who had been institutionalised for five years or more, who had been committed as "criminally insane", who were of "non- Aryan race" or who had been diagnosed with any on
19055-539: The clinic for sixty years. The Lutheran theologian Friedrich von Bodelschwingh (director of the Bethel Institution for Epilepsy at Bielefeld ) and Pastor Paul-Gerhard Braune (director of the Hoffnungstal Institution near Berlin) protested. Bodelschwingh negotiated directly with Brandt and indirectly with Hermann Göring , whose cousin was a prominent psychiatrist. Braune had meetings with Gürtner, who
19240-444: The cognitive executive system and the motor circuit. The basal ganglia ordinarily inhibit a large number of circuits that generate specific movements. To initiate a particular movement, the cerebral cortex sends a signal to the basal ganglia that causes the inhibition to be released. Damage to the basal ganglia can cause the release or reinstatement of the inhibitions to be erratic and uncontrolled, which results in an awkward start to
19425-687: The commentator as unethical, but the domestic legal definitions of murder, manslaughter, etc., usually exclude killings carried out by lawful government action. Dartmouth College professor Benjamin Valentino outlines two major categories of mass killings: dispossessive mass killing and coercive mass killing. The first category includes four subcategories: communist, fascist, ethnic and territorial mass killings, while second category includes counterinsurgency , terrorist and imperialist mass killings. Valentino included several prominent examples of dispossessive mass killing to include his arguments, including
19610-407: The consciences of many of those involved, giving them the feeling that there was a genuine medical purpose to the killings. The most notorious of these institutions in Austria was Am Spiegelgrund, where from 1940 to 1945, 789 children were killed by lethal injection, gas poisoning and physical abuse. Children's brains were preserved in jars of formaldehyde and stored in the basement of the clinic and in
19795-480: The cost of maintaining asylums for the incurably ill and insane. These films included The Inheritance ( Das Erbe , 1935), The Victim of the Past ( Opfer der Vergangenheit , 1937), which was given a major première in Berlin and was shown in all German cinemas, and I Accuse ( Ich klage an , 1941) which was based on a novel by Hellmuth Unger, a consultant for "child euthanasia". In mid-1939, Hitler authorised
19980-674: The creation of the Reich Committee for the Scientific Registering of Serious Hereditary and Congenital Illnesses ( Reichsausschuss zur wissenschaftlichen Erfassung erb- und anlagebedingter schwerer Leiden ) led by his physician, Karl Brandt, administered by Herbert Linden of the Interior Ministry, leader of German Red Cross Reichsarzt SS und Polizei Ernst-Robert Grawitz and SS - Oberführer Viktor Brack . Brandt and Bouhler were authorised to approve applications to kill children in relevant circumstances, though Bouhler left
20165-484: The crematoria chimneys and noticed that the buses were returning empty. In Hadamar, ashes containing human hair rained down on the town and despite the strictest orders, some of the staff at the killing centres talked about what was going on. In some cases families could tell that the causes of death in certificates were false, e.g. when a patient was claimed to have died of appendicitis , even though his appendix had been removed some years earlier. In other cases, families in
20350-764: The crime of intentional homicide in 2012 was 43 percent. The 2011 Global Study on Homicide reported that "[W]here homicide rates are high and firearms and organized crime in the form of drug trafficking play a substantial role, 1 in 50 men aged 20 will be murdered before they reach the age of 31. At the other, the probability of such an occurrence is up to 400 times lower. [H]omicide is much more common in countries with low levels of human development, high levels of income inequality and weak rule of law than in more equitable societies , where socioeconomic stability seems to be something of an antidote to homicide. In cases of intimate partner and family-related homicide cases, women murdered by their past or present male partner make up
20535-753: The crime was in progress at the time of the incident like assault or homicide (38.9%), robbery (32.9%), drug trade (14.5%), burglary (11%), motor vehicle theft (5%), rape or sexual assault (2%). A larger proportion of homicides of females than males resulted from caregiver abuse or neglect (9.0% versus 2.7%) or were perpetrated by a suspect with a mental health problem (e.g., schizophrenia or other psychotic conditions, depression, or posttraumatic stress disorder ) (6.3% versus 1.7%). Homicide rates are known to be higher in males and in communities with concentrated poverty, stressed economies, residential instability, neighborhood disorganization, low community cohesion, and informal controls. The overall firearm homicide rate in 2020
20720-604: The defendant with a full or partial defense to criminal prosecution. Common defenses include: The availability of defenses to a criminal charge following a homicide may affect the homicide rate. For example, it has been suggested that the availability of " stand your ground " defense has resulted in an increase in the homicide rate in U.S. jurisdictions that recognize the defense, including Florida. Homicides committed by state actors may be considered lawful or unlawful according to: Types of state killings include: The term mass killing has been proposed by genocide scholars as
20905-420: The details to subordinates such as Brack and SA- Oberführer Werner Blankenburg . Extermination centres were established at six existing psychiatric hospitals: Bernburg , Brandenburg , Grafeneck , Hadamar , Hartheim , and Sonnenstein . One thousand children under the age of 17 were killed at the institutions Am Spiegelgrund and Gugging in Austria. They played a crucial role in developments leading to
21090-506: The development of respiratory problems. Consensus guidelines on physiotherapy in Huntington's disease have been produced by the European HD Network. Goals of early rehabilitation interventions are prevention of loss of function. Participation in rehabilitation programs during the early to middle stage of the disease may be beneficial as it translates into long-term maintenance of motor and functional performance. Rehabilitation during
21275-513: The disease and their families, these symptoms are among the most distressing aspects of the disease, often affecting daily functioning and constituting reason for institutionalization . Early behavioral changes in HD result in an increased risk of suicide. Often, individuals have reduced awareness of chorea, cognitive, and emotional impairments. Mutant huntingtin is expressed throughout the body and associated with abnormalities in peripheral tissues that are directly caused by such expression outside
21460-455: The disease has progressed can be measured using the unified Huntington's disease rating scale, which provides an overall rating system based on motor, behavioral, cognitive, and functional assessments. Medical imaging , such as a CT scan or MRI scan , can show atrophy of the caudate nuclei early in the disease, as seen in the illustration to the right, but these changes are not, by themselves, diagnostic of HD. Cerebral atrophy can be seen in
21645-466: The disorder progresses. These are signs that the system in the brain that is responsible for movement has been affected. Psychomotor functions become increasingly impaired, such that any action that requires muscle control is affected. When muscle control is affected such as rigidity or muscle contracture this is known as dystonia . Dystonia is a neurological hyperkinetic movement disorder that results in twisting or repetitive movements, that may resemble
21830-471: The early Germanic tribes of Scandinavia which also gave rise to the Swedes ; all cases with the exception of one going back nearly two centuries having derived from the offspring of a couple living early in the 19th century. Finland , as well, has a low incidence of only 2.2 per 100,000 people. Until the discovery of a genetic test, statistics could only include clinical diagnosis based on physical symptoms and
22015-404: The embryo's DNA is compared with that of the parents and grandparents to avoid inheritance of the chromosomal region containing the HD gene from the affected grandparent. In nondisclosure testing, only disease-free embryos are replaced in the uterus while the parental genotype and hence parental risk for HD are never disclosed. Obtaining a prenatal diagnosis for an embryo or fetus in the womb
22200-501: The end of 1941. Technology developed under Aktion T4 , particularly the use of lethal gas on large numbers of people, was taken over by the medical division of the Reich Interior Ministry, along with the personnel of Aktion T4 , who participated in mass murder of Jewish people . The programme was authorised by Hitler but the killings have since come to be viewed as murders in Germany. The number of people killed
22385-433: The end of the year, partly because the areas they served had been cleared and partly because of public opposition. In 1941, however, the centres at Bernburg and Sonnenstein increased their operations, while Hartheim (where Wirth and Franz Stangl were successively commandants) continued as before. Another 35,000 people were killed before August 1941, when the T4 programme was officially shut down by Hitler. Even after that date
22570-399: The expansion of the HTT gene, or when they have a 50% chance of inheriting the disease. The parents can be counseled on their options, which include termination of pregnancy , and on the difficulties of a child with the identified gene. In addition, in at-risk pregnancies due to an affected male partner, noninvasive prenatal diagnosis can be performed by analyzing cell-free fetal DNA in
22755-423: The expression of Htt is increased, brain cell survival is improved and the effects of mHtt are reduced, whereas when the expression of Htt is reduced, the resulting characteristics are more as seen in the presence of mHtt. Accordingly, the disease is thought not to be caused by inadequate production of Htt, but by a toxic gain-of-function of mHtt in the body. The toxic action of mHtt may manifest and produce
22940-416: The expression of genes. Mutant huntingtin protein has been found to play a key role in mitochondrial dysfunction . The impairment of mitochondrial electron transport can result in higher levels of oxidative stress and release of reactive oxygen species . Glutamine is known to be excitotoxic when present in large amounts, that can cause damage to numerous cellular structures. Excessive glutamine
23125-410: The families could afford it, transferred them to private clinics beyond the reach of T4. Other doctors "re-diagnosed" patients so that they no longer met the T4 criteria, which risked exposure when Nazi zealots from Berlin conducted inspections. In Kiel , Professor Hans Gerhard Creutzfeldt managed to save nearly all of his patients. Lifton listed a handful of psychiatrists and administrators who opposed
23310-417: The following 'serious hereditary diseases' were 'suspected': idiocy and Down syndrome (especially when associated with blindness and deafness); microcephaly ; hydrocephaly ; malformations of all kinds, especially of limbs, head, and spinal column; and paralysis, including spastic conditions". The reports were assessed by a panel of medical experts, of whom three were required to give their approval before
23495-503: The front and was supervised by Bouhler and Brandt. The officials in charge included Herbert Linden, who had been involved in the child killing programme; Ernst-Robert Grawitz, chief physician of the SS and August Becker , an SS chemist. The officials selected the doctors who were to carry out the operational part of the programme; based on political reliability as long-term Nazis, professional reputation and sympathy for radical eugenics. The list included physicians who had proved their worth in
23680-432: The homicide either does not intend to kill the victim, or kills the victim as the result of circumstances that would cause a reasonable person to become emotionally or mentally disturbed to the point of potentially losing control of their actions. The distinction between murder and manslaughter is sometimes said to have first been made by the ancient Athenian lawmaker Draco in the 7th century BC. The penalty for manslaughter
23865-560: The implementation of the "Final Solution" for the next two years. The first killing centre, equipped with stationary gas chambers, modelled on technology developed under Aktion T4 , was established at Bełżec in the General Government territory of occupied Poland; the decision preceded the Wannsee Conference of January 1942 by three months. In January 1939, Brack commissioned a paper from Professor of Moral Theology at
24050-631: The incident at Absberg noted that "the removal of residents from the Ottilien Home has caused a great deal of unpleasantness" and described large crowds of Catholic townspeople, among them Party members, protesting against the action. Similar petitions and protests occurred throughout Austria as rumours spread of mass killings at the Hartheim Euthanasia Centre and of mysterious deaths at the children's clinic, Am Spiegelgrund in Vienna. Anna Wödl,
24235-407: The inmates of nursing homes, asylums, prisons, aged-care homes and special schools, to select those to be sterilised. It is estimated that 360,000 people were sterilised under this law between 1933 and 1939. The policy and research agenda of racial hygiene and eugenics were promoted by Emil Kraepelin . The eugenic sterilisation of persons diagnosed with (and viewed as predisposed to) schizophrenia
24420-601: The jurisdiction of the national medical division of the Reich Interior Ministry. Further gassing experiments with the use of mobile gas chambers ( Einsatzwagen ) were conducted at Soldau concentration camp by Herbert Lange following Operation Barbarossa . Lange was appointed commander of the Chełmno extermination camp in December 1941. He was given three gas vans by the Reich Security Main Office (RSHA), converted by
24605-517: The killing of the boy, the Reich Committee for the Scientific Registering of Hereditary and Congenital Illnesses was established to register sick children or newborns identified as defective. The secret killing of infants began in 1939 and increased after the war started; by 1941, more than 5,000 children had been killed. Hitler was in favour of killing those whom he judged to be lebensunwertes Leben (' Life unworthy of life '). A few months before
24790-462: The killing of the incurably ill but recognised that public opinion would not accept this. In 1935, Hitler told the Leader of Reich Doctors, Gerhard Wagner , that the question could not be taken up in peacetime; "Such a problem could be more smoothly and easily carried out in war". He wrote that he intended to "radically solve" the problem of the mental asylums in such an event. Aktion T4 began with
24975-621: The killing of those designated as juvenile delinquents. Jewish children could be placed in the net primarily because they were Jewish; and at one of the institutions, a special department was set up for 'minor Jewish-Aryan half-breeds'. More pressure was placed on parents to agree to their children being sent away. Many parents suspected what was happening and refused consent, especially when it became apparent that institutions for children with disabilities were being systematically cleared of their charges. The parents were warned that they could lose custody of all their children and if that did not suffice,
25160-685: The killing programme must be implemented with stealth "...to avoid a probable backlash of public opinion during the war". On 4 December 1940, Reinhold Sautter, the Supreme Church Councillor of the Württemberg State Church, complained to the Nazi Ministerial Councillor Eugen Stähle against the murders in Grafeneck Castle. Stähle said "The fifth commandment Thou shalt not kill, is no commandment of God but
25345-554: The killings; many doctors collaborated, either through ignorance, agreement with Nazi eugenicist policies or fear of the regime. Protest letters were sent to the Reich Chancellery and the Ministry of Justice, some from Nazi Party members. The first open protest against the removal of people from asylums took place at Absberg in Franconia in February 1941 and others followed. The SD report on
25530-432: The late stage aims to compensate for motor and functional losses. For long-term independent management, the therapist may develop home exercise programs for appropriate people. Additionally, an increasing number of people with HD are turning to palliative care , which aims to improve quality of life through the treatment of the symptoms and stress of serious illness, in addition to their other treatments. Tetrabenazine
25715-470: The lists, sometimes in a haphazard and ideologically motivated way. During 1940, all Jewish patients were removed from institutions and killed. As with child inmates, adults were assessed by a panel of experts, working at the Tiergartenstraße offices. The experts were required to make their judgements on the reports, not medical histories or examinations. Sometimes they dealt with hundreds of reports at
25900-468: The most vulnerable, particularly ones with projections towards the external globus pallidus , with interneurons and spiny cells projecting to the internal globus pallidus being less affected. HD also causes an abnormal increase in astrocytes and activation of the brain's immune cells, microglia . The basal ganglia play a key role in movement and behavior control. Their functions are not fully understood, but theories propose that they are part of
26085-481: The motion or motions to be unintentionally initiated or in a motion to be halted before or beyond its intended completion. The accumulating damage to this area causes the characteristic erratic movements associated with HD known as chorea, a dyskinesia . Because of the basal ganglia's inability to inhibit movements, individuals affected by it inevitably experience a reduced ability to produce speech and swallow foods and liquids (dysphagia). CREB-binding protein (CBP),
26270-438: The mouth may also be of use to prevent choking. If eating becomes too hazardous or uncomfortable, the option of using a percutaneous endoscopic gastrostomy is available. This feeding tube, permanently attached through the abdomen into the stomach , reduces the risk of aspirating food and provides better nutritional management. Assessment and management by speech-language pathologists with experience in Huntington's disease
26455-413: The number of repeats present. This usually leads to new expansions as generations pass ( dynamic mutations ) instead of reproducing an exact copy of the trinucleotide repeat. This causes the number of repeats to change in successive generations, such that an unaffected parent with an "intermediate" number of repeats (28–35), or "reduced penetrance" (36–40), may pass on a copy of the gene with an increase in
26640-441: The number of repeats that produces fully penetrant HD. The earlier age of onset and greater severity of disease in successive generations due to increases in the number of repeats is known as genetic anticipation . Instability is greater in spermatogenesis than oogenesis ; maternally inherited alleles are usually of a similar repeat length, whereas paternally inherited ones have a higher chance of increasing in length. Rarely
26825-701: The onset, progression, and extent of cognitive and behavioral symptoms vary significantly between individuals. The most characteristic initial physical symptoms are jerky, random, and uncontrollable movements called chorea . Many people are not aware of their involuntary movements, or impeded by them. Chorea may be initially exhibited as general restlessness, small unintentionally initiated or uncompleted motions, lack of coordination, or slowed saccadic eye movements . These minor motor abnormalities usually precede more obvious signs of motor dysfunction by at least three years. The clear appearance of symptoms such as rigidity, writhing motions, or abnormal posturing appear as
27010-513: The operations of the T4 programme based on his time as commandant of the killing facility at the Hartheim institute. He described how the inmates of various asylums were removed and transported by bus to Hartheim. Some were in no mental state to know what was happening to them but many were perfectly sane and for them various forms of deception were used. They were told they were at a special clinic where they would receive improved treatment and were given
27195-491: The orders of local officials, although Himmler certainly knew and approved of them. The legal basis for the programme was a 1939 letter from Hitler, not a formal "Führer's decree" with the force of law. Hitler bypassed Conti, the Health Minister and his department, who might have raised questions about the legality of the programme and entrusted it to Bouhler and Brandt. Reich Leader Bouhler and Dr. Brandt are entrusted with
27380-505: The parents could be threatened with call-up for 'labour duty'. By 1941, more than 5,000 children had been killed. The last child to be killed under Aktion T4 was Richard Jenne on 29 May 1945, in the children's ward of the Kaufbeuren - Irsee state hospital in Bavaria , Germany, more than three weeks after US Army troops had occupied the town. Brandt and Bouhler developed plans to expand
27565-528: The penetrance of the mutation is very high, those who have a mutated copy of the gene will have the disease. In this type of inheritance pattern, each offspring of an affected individual has a 50% risk of inheriting the mutant allele, so are affected with the disorder (see figure). This probability is sex-independent. Sex-dependent or sex-linked genes are traits that are found on the X or Y chromosomes. Trinucleotide CAG repeats numbering over 28 are unstable during replication , and this instability increases with
27750-434: The policy was contrary to divine law and that "the direct killing of an innocent person because of mental or physical defects is not allowed" but the declaration was not upheld by all Catholic authorities in Germany. In the summer of 1941, protests were led in Germany by the bishop of Münster, Clemens von Galen , whose intervention led to "the strongest, most explicit and most widespread protest movement against any policy since
27935-551: The possibility of discrimination and the implications of a positive result, which usually means a parent has an affected gene and that the individual's siblings will be at risk of inheriting it. In one study, genetic discrimination was found in 46% of individuals at risk for Huntington's disease. It occurred at higher rates within personal relationships than health insurance or employment relations. Genetic counseling in HD can provide information, advice and support for initial decision-making, and then, if chosen, throughout all stages of
28120-714: The prevalence of Huntington's disease in the UK between 1990 and 2010 found that the average prevalence for the UK was 12.3 per 100,000. Additionally, some localized areas have a much higher prevalence than their regional average. One of the highest incidences is in the isolated populations of the Lake Maracaibo region of Venezuela , where HD affects up to 700 per 100,000 persons. Other areas of high localization have been found in Tasmania and specific regions of Scotland , Wales and Sweden . Increased prevalence in some cases occurs due to
28305-520: The private collection of Heinrich Gross , one of the institution's directors, until 2001. When the Second World War began in September 1939, less rigorous standards of assessment and a quicker approval process were adopted. Older children and adolescents were included and the conditions covered came to include ... various borderline or limited impairments in children of different ages, culminating in
28490-415: The production of a protein with different characteristics. This altered form, called mutant huntingtin (mHtt), increases the decay rate of certain types of neurons . Regions of the brain have differing amounts and reliance on these types of neurons and are affected accordingly. Generally, the number of CAG repeats is related to how much this process is affected, and accounts for about 60% of the variation of
28675-451: The programme of euthanasia to adults. In July 1939 they held a meeting attended by Conti and Professor Werner Heyde , head of the SS medical department. This meeting agreed to arrange a national register of all institutionalised people with mental illnesses or physical disabilities. The first adults with disabilities to be killed en masse by the Nazi regime were Poles. After the invasion on 1 September 1939, adults with disabilities were shot by
28860-454: The progression of the disease, and studying procedures such as stem-cell therapy with the goal of replacing damaged or lost neurons. Signs and symptoms of Huntington's disease most commonly become noticeable between the ages of 30 and 50 years, but they can begin at any age and present as a triad of motor, cognitive, and psychiatric symptoms. When developed in an early stage, it is known as juvenile Huntington's disease. In 50% of cases,
29045-445: The prospect of either having to imprison prominent, highly admired clergymen and other protesters – a course with consequences in terms of adverse public reaction they greatly feared – or else end the programme". Evans considered it "at least possible, even indeed probable" that the T4 programme would have continued beyond Hitler's initial quota of 70,000 deaths but for the public reaction to Galen's sermon. Burleigh called assumptions that
29230-448: The psychiatric symptoms appear first. Their progression is often described in early stages, middle stages, and late stages with an earlier prodromal phase. In the early stages, subtle personality changes, problems in cognition and physical skills, irritability , and mood swings occur, all of which may go unnoticed, and these usually precede the motor symptoms. Almost everyone with HD eventually exhibits similar physical symptoms, but
29415-456: The responsibility of extending the authority of physicians, to be designated by name, so that patients who, after a most critical diagnosis, on the basis of human judgment [ menschlichem Ermessen ], are considered incurable, can be granted mercy death [ Gnadentod ]. The killings were administered by Viktor Brack and his staff from Tiergartenstraße 4, disguised as the "Charitable Foundation for Cure and Institutional Care" offices which served as
29600-518: The restriction of killing to 'complete idiots', access to the sacraments and the exclusion of ill Roman Catholic priests from these policies. Despite a decree issued by the Vatican on 2 December 1940 stating that the T4 policy was "against natural and positive Divine law" and that "The direct killing of an innocent person because of mental or physical defects is not allowed", the Catholic Church hierarchy in Germany decided to take no further action. Incensed by
29785-452: The same fundamental cross-beta amyloid architecture seen in other protein deposition diseases . Over time, the aggregates accumulate to form inclusion bodies within cells, ultimately interfering with neuronal function. Inclusion bodies have been found in both the cell nucleus and cytoplasm . Inclusion bodies in cells of the brain are one of the earliest pathological changes, and some experiments have found that they can be toxic for
29970-652: The same town would receive death certificates on the same day. In May 1941, the Frankfurt County Court wrote to Gürtner describing scenes in Hadamar, where children shouted in the streets that people were being taken away in buses to be gassed. During 1940, rumours of what was taking place spread and many Germans withdrew their relatives from asylums and sanatoria to care for them at home, often with great expense and difficulty. In some places doctors and psychiatrists co-operated with families to have patients discharged or if
30155-590: The sermon affected Hitler's decision to suspend the T4 programme "wishful thinking" and noted that the various Church hierarchies did not complain after the transfer of T4 personnel to Aktion Reinhard . Henry Friedlander wrote that it was not the criticism from the Church but rather the loss of secrecy and "general popular disquiet about the way euthanasia was implemented" that caused the killings to be suspended. Homicide#By state actors Note: Varies by jurisdiction Note: Varies by jurisdiction Homicide
30340-405: The spring of 1940. The SS and police from SS-Sonderkommando Lange responsible for murdering the majority of patients in the annexed territories of Poland since October 1939, took their salaries from the normal police fund, supervised by the administration of the newly formed Wartheland district; the programme in Germany and occupied Poland was overseen by Heinrich Himmler. Before 2013, it
30525-1002: The testing process. Because of the implications of this test, patients who wish to undergo testing must complete three counseling sessions which provide information about Huntington's. Counseling and guidelines on the use of genetic testing for HD have become models for other genetic disorders, such as autosomal dominant cerebellar ataxia . Presymptomatic testing for HD has also influenced testing for other illnesses with genetic variants such as polycystic kidney disease, familial Alzheimer's disease and breast cancer . The European Molecular Genetics Quality Network have published yearly external quality assessment scheme for molecular genetic testing for this disease and have developed best practice guidelines for genetic testing for HD to assist in testing and reporting of results. Embryos produced using in vitro fertilization may be genetically tested for HD using preimplantation genetic diagnosis . This technique, where one or two cells are extracted from
30710-545: The trend in homicides to a steady increase in self-control of the sort promoted by Protestantism , and necessitated by schools and factories. Eisner argues that macro-level indicators for societal efforts to promote civility , self-discipline, and long-sightedness are strongly associated with fluctuations in homicide rates over the past six centuries. In the US, the National Violent Death Reporting System
30895-520: The trinucleotide repeat (CAG) in the HTT gene that causes the disease. Genetic counseling is available to provide advice and guidance throughout the testing procedure and on the implications of a confirmed diagnosis. These implications include the impact on an individual's psychology, career, family-planning decisions, relatives, and relationships. Despite the availability of pre-symptomatic testing, only 5% of those at risk of inheriting HD choose to do so. A physical examination , sometimes combined with
31080-443: The usefulness of physical therapy , occupational therapy , and speech therapy . Weight loss and problems in eating due to dysphagia and other muscle discoordination are common, making nutrition management increasingly important as the disease advances. Thickening agents can be added to liquids, as thicker fluids are easier and safer to swallow. Reminding the affected person to eat slowly and to take smaller pieces of food into
31265-493: The vast majority of homicide victims worldwide." In the mid-second millennium, local levels of violence in Europe were extremely high by the standards of modern developed countries. Typically, small groups of people would battle their neighbors using the farm tools at hand, such as knives, sickles, hammers, and axes. Mayhem and death were deliberate. The vast majority of Europeans lived in rural areas till 1800. Cities were few, and small in size, but their concentration of population
31450-448: The victims were cremated en masse ). The preparation of thousands of falsified death certificates took up most of the working day of the doctors who operated the centres. During 1940, the centres at Brandenburg, Grafeneck and Hartheim killed nearly 10,000 people each, while another 6,000 were killed at Sonnenstein. In all, about 35,000 people were killed in T4 operations that year. Operations at Brandenburg and Grafeneck were wound up at
31635-505: The world average. They were the most violent regions globally, outside of regions experiencing wars and religious or sociopolitical terrorism. Asia exclusive of West Asia and Central Asia, Western Europe, Northern Europe, as well as Oceania had the lowest homicide rates in the world. About 41 percent of the homicides worldwide occurred in 2012 with the use of guns, 24 percent by stabbing with sharp objects such as knife, and 35 percent by other means such as poison. The global conviction rate for
31820-538: Was a respectable field of medicine. Canada , Denmark , Switzerland and the US had passed laws enabling coerced sterilisation . Studies conducted in the 1920s ranked Germany as a country that was unusually reluctant to introduce sterilisation legislation. In his book Mein Kampf (1924), Hitler wrote that one day racial hygiene "will appear as a deed greater than the most victorious wars of our present bourgeois era". In July 1933,
32005-433: Was about 200,000 in Germany and Austria, with about 100,000 victims in other European countries. Following the war, a number of the perpetrators were tried and convicted for murder and crimes against humanity. At the beginning of the twentieth century, the sterilisation of people carrying what were considered to be hereditary defects and in some cases those exhibiting what was thought to be hereditary "antisocial" behaviour,
32190-445: Was advocated by Eugen Bleuler , who presumed racial deterioration because of "mental and physical cripples" in his Textbook of Psychiatry , The more severely burdened should not propagate themselves... If we do nothing but make mental and physical cripples capable of propagating themselves, and the healthy stocks have to limit the number of their children because so much has to be done for the maintenance of others, if natural selection
32375-653: Was always dubious about the legality of the programme. Gürtner later wrote a strongly worded letter to Hitler protesting against it; Hitler did not read it but was told about it by Lammers. Bishop Theophil Wurm , presiding over the Evangelical-Lutheran Church in Württemberg , wrote to Interior Minister Frick in March 1940 and that month a confidential report from the Sicherheitsdienst (SD) in Austria, warned that
32560-824: Was approved in 2000 for treatment of chorea in Huntington's disease in the EU, and in 2008 in the US. Although other drugs had been used " off label ", tetrabenazine was the first approved treatment for Huntington's disease in the U.S. The compound has been known since the 1950s. An alternative to tetrabenazine is amantadine but there is limited evidence for its safety and efficacy. Other drugs that help to reduce chorea include antipsychotics and benzodiazepines . Hypokinesia and rigidity, especially in juvenile cases, can be treated with antiparkinsonian drugs, and myoclonic hyperkinesia can be treated with valproic acid . Tentative evidence has found ethyl eicosapentaenoic acid to improve motor symptoms at one year. In 2017, deutetrabenazine ,
32745-616: Was believed that 70,000 persons were murdered in the euthanasia programme, but the German Federal Archives reported that research in the archives of former East Germany indicated that the number of victims in Germany and Austria from 1939 to 1945 was about 200,000 persons and that another 100,000 persons were victims in other European countries. In the German T4 centres there was at least the semblance of legality in keeping records and writing letters. In Polish psychiatric hospitals no one
32930-437: Was conducive to violence and their trends resembled those in rural areas. Across Europe, homicide trends show a steady long-term decline. Regional differences were small, except that Italy's decline was later and slower. From about 1200 AD through 1800 AD, homicide rates from violent local episodes, not including military actions, declined by a factor of ten, from approximately 32 deaths per 100,000 people to 3.2 per 100,000. In
33115-423: Was deemed to be "useful", exempted from the law and the rate of sterilisation declined. The term Aktion T4 is a post-war coining; contemporary German terms included Euthanasie ( euthanasia ) and Gnadentod (merciful death). The T4 programme stemmed from the Nazi Party policy of "racial hygiene", a belief that the German people needed to be cleansed of racial enemies, which included anyone confined to
33300-548: Was discovered in 1993 by an international collaborative effort led by the Hereditary Disease Foundation . Research and support organizations began forming in the late 1960s to increase public awareness, provide support for individuals and their families and promote research. Research directions include determining the exact mechanism of the disease, improving animal models to aid with research, testing of medications and their delivery to treat symptoms or slow
33485-612: Was higher than in the last 20 years, disproportionately borne by Native Americans and Black persons. It is thought that the COVID-19 pandemic increased social and economic stress. [REDACTED] Law portal Huntington%27s chorea Huntington's disease ( HD ), also known as Huntington's chorea , is an incurable neurodegenerative disease that is mostly inherited . The earliest symptoms are often subtle problems with mood or mental/psychiatric abilities. A general lack of coordination and an unsteady gait often follow. It
33670-838: Was in a letter by Charles Oscar Waters (1816–1892), published in the first edition of Robley Dunglison 's Practice of Medicine in 1842. Waters described "a form of chorea, vulgarly called magrums", including accurate descriptions of the chorea, its progression, and the strong heredity of the disease. In 1846 Charles Rollin Gorman (1817–1879) observed how higher prevalence seemed to occur in localized regions. Independently of Gorman and Waters, both students of Dunglison at Jefferson Medical College in Philadelphia, Johan Christian Lund [ no ] (1830–1906) also produced an early description in 1860. He specifically noted that in Setesdalen ,
33855-407: Was left behind. Killings were inflicted using gas-vans, sealed army bunkers and machine guns; families were not informed about the murdered relatives and the empty wards were handed over to the SS. After the official end of the euthanasia programme in 1941, most of the personnel and high-ranking officials, as well as gassing technology and the techniques used to deceive victims, were transferred under
34040-445: Was more merciful than poison injections. The German eugenics movement had an extreme wing even before the Nazis came to power. As early as 1920, Alfred Hoche and Karl Binding advocated killing people whose lives were "unworthy of life" ( lebensunwertes Leben ). Darwinism was interpreted by them as justification of the demand for "beneficial" genes and eradication of the "harmful" ones. Robert Lifton wrote, "The argument went that
34225-441: Was not possible for them to visit relatives in these centres. Most of these patients were killed within 24 hours of arriving at the centres and their bodies cremated. Some bodies were dissected for medical research whilst others had their gold teeth extracted. For every person killed, a death certificate was prepared, giving a false but plausible cause of death. This was sent to the family along with an urn of ashes (random ashes, since
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