Ethics, Moral Character and Authentic free essay sample

The four components of authentic transformational leadership (idealized influence, inspirational motivation, intellectual stimulation, and individualized consideration) are constrasted with their counterfeits in the dissembling pseudotransformational leadership on the basis of 1) the moral character of the leaders and their concerns for self and others; 2) the ethical values embedded in the leaders’ vision, articulation, and program, which followers can embrace or reject; and 3) the morality of the processes of social ethical choices and action in which the leaders and followers engage and collectively pursue. The literature on transformational leadership is linked to the long-standing literature on virtue and moral character, as exemplified by Socratic and Confucian typologies. It is linked, as well, to the major themes of the modern Western ethical agenda: liberty, utility and distributive justice Deception, sophistry, and pretense are examined alongside issues of transendence, agency, trust, striving for the congruence of the values, cooperative action, power, persuasion, and corporate governance to establish the strategic and moral foundations of authentic transformational leadership. We will write a custom essay sample on Ethics, Moral Character and Authentic or any similar topic specifically for you Do Not WasteYour Time HIRE WRITER Only 13.90 / page Contracts can be skewed in favor of those with more resources, contacts and bargaining power. People often appreciate leadership which points the way out of dilemmas whether it comes from others within their own collective or from external authority. Leaders as divergent in their politics as Mao Zedong and Shimon Peres agreed that the task of leadership is to sense the problems of their followers and to articulate solutions which satisfied their interests. Rost, reminiscent of Nozick and Rand, asks for leader-follower distinctions be erased to reach true participative democracy. Burns (1998) partially agrees and would substitute for leaders and followers, initiators, supporters and opponents. But the counterarguments are that if everyone in a group is responsible for its leadership, no one is responsible. Furthermore, if a group is initially leaderless, the members compete with each other for leadership. One or more leaders emerge who initiate and propose more than the other members. Followers emerge who are responsive to the leaders, and non-responsive isolated persons remain who are passive (Bass, 1954). If trying to align the values of members of an organization with the good of all stakeholders is unethical , then it is unethical to contingently reward prison inmates with time off for good behavior or for transformational teachers to move pupils to internalize the values of good citizenship for the benefit of society. Libertarians would agree that one’s life plans are paramount but they are close to espousing anarchy as are the OD extremists who charge immorality if the transformational leader intervenes in the individual follower’s life plans(Bass, 1998A, p. 79). With this line of thinking that it is immoral to align the values and behavio

The Partition of India and Creation of Pakistan

The Partition of India and Creation of Pakistan The Partition of India was the process of dividing the subcontinent along sectarian lines, which took place in 1947 as India gained its independence from the British Raj. The northern, predominantly Muslim sections of India became the nation of Pakistan, while the southern and majority Hindu section became the Republic of India. Fast Facts: the Partition of India Short Description: At the time of Indian independence from Great Britain, the subcontinent was broken into two partsKey Players/Participants: Muhammed Ali Jinnah, Jawaharlal Nehru, Mohandas Gandhi, Louis Mountbatten, Cyril RadcliffeEvent Start Date: End of World War II, the ouster of Churchill, and the ascension of the Labour Party in BritainEvent End Date: Aug. 17, 1947Other Significant Dates: The Jan. 30, 1948, the assassination of Mohandas Gandhi; Aug. 14, 1947, the creation of the Islamic Republic of Pakistan; Aug. 15, 1947, the creation of the Republic of IndiaLittle-Known Fact: In the 19th century, sectarian Muslim, Sikh, and Hindu communities shared Indias cities and countryside and cooperated to force Britain to Quit India; it was only after independence became a potential reality that religious hatred began to roil.   Background to Partition Beginning in 1757, the British commercial enterprise known as the East India Company ruled parts of the subcontinent beginning with Bengal, a period known as the Company Rule or Company Raj. In 1858, after the brutal Sepoy Rebellion, the rule of India was transferred to the English crown, with Queen Victoria proclaimed as Empress of India in 1878. By the latter half of the 19th century, England had brought the full force of the Industrial Revolution to the region, with railroads, canals, bridges, and telegraph lines providing new communication links and opportunities. Most of the jobs created went to the English; much of the land used for these advances came from the farmers and were paid for by local taxes.   Medical advances under the Company and British Raj, such as smallpox vaccinations, improved sanitation, and quarantine procedures, led to a steep rise in population. Protectionist landlords depressed agricultural innovations in the rural areas, and as a result, famines broke out. The worst was known as the Great Famine of 1876–1878, when between 6–10 million people died. Universities established in India led to a new middle class, and in turn, social reform and political action began to rise.   The Rise of Sectarian Separation   In 1885, the Hindu-dominated Indian National Congress (INC) met for the first time. When the British made an attempt to divide the state of Bengal along religious lines in 1905, the INC led huge protests against the plan. This sparked the formation of the Muslim League, which sought to guarantee the rights of Muslims in any future independence negotiations.  Although the Muslim League formed in opposition to the INC, and the British colonial government attempted to play the INC and Muslim League off one another, the two political parties generally cooperated in their mutual goal of getting Britain to Quit India. As British historian Yasmin Khan (born 1977) has described,  political events were to destroy the long-term future of that uneasy alliance.   In 1909, the British gave separate electorates to different religious communities, which had the outcome of hardening of boundaries among the different sects. The colonial government emphasized these differences, by such activities as providing separate restroom and water facilities for Muslims and Hindus at the railway terminals. By the 1920s, a heightened sense of religious ethnicity became apparent. Riots broke out at such times as during Holi festival, when sacred cows were slaughtered, or when Hindu religious music was played in front of mosques at prayer time.   World War I and Afterward Despite the growing unrest, both the INC and the Muslim League supported sending Indian volunteer troops to fight on Britains behalf in World War I. In exchange for the service of more than one million Indian soldiers, the people of India expected political concessions up to and including independence. However, after the war, Britain offered no such concessions. In April 1919, a unit of the British Army went to Amritsar, in Punjab, to silence pro-independence unrest. The units commander ordered his men to open fire on the unarmed crowd, killing more than 1,000 protesters. When word of the Amritsar Massacre spread around India, hundreds of thousands of formerly apolitical people became supporters of the INC and the Muslim League. In the 1930s, Mohandas Gandhi (1869–1948) became the leading figure in the INC. Although he advocated a unified Hindu and Muslim India, with equal rights for all, other INC members were less inclined to join with Muslims against the British. As a result, the Muslim League began to make plans for a separate Muslim state. World War II World War II sparked a crisis in relations between the British, the INC, and the Muslim League. The British government expected India once again to provide much-needed soldiers and material for the war effort, but the INC opposed sending Indians to fight and die in Britains war. After the betrayal following World War I, the INC saw no benefit for India in such a sacrifice. The Muslim League, however, decided to back Britains call for volunteers, in an effort to curry British favor in support of a Muslim nation in post-independence northern India. Before the war had even ended, public opinion in Britain had swung against the distraction and expense of empire: the cost of the war had severely depleted Britains coffers. The party of British prime minister Winston Churchill (1874–1965) was voted out of office, and the pro-independence Labour Party was voted in during 1945. Labour called for almost immediate independence for India, as well as more gradual freedom for Britains other colonial holdings. A Separate Muslim State The Muslim Leagues leader, Muhammed Ali Jinnah (1876–1948), began a public campaign in favor of a separate Muslim state, while Jawaharlal Nehru (1889–1964) of the INC called for a unified India. The INC leaders such as Nehru were in favor of a united India since Hindus would have formed the vast majority of the Indian population and would have been in control of any democratic form of government.   As independence neared, the country began to descend toward a sectarian civil war. Although Gandhi implored the Indian people to unite in peaceful opposition to British rule, the Muslim League sponsored a Direct Action Day on August 16, 1946, which resulted in the deaths of more than 4,000 Hindus and Sikhs in Calcutta (Kolkata). This touched off the Week of the Long Knives, an orgy of sectarian violence that resulted in hundreds of deaths on both sides in various cities across the country. Indian Independence Act of 1947 In February 1947, the British government announced that India would be granted independence by June 1948. Viceroy for India Louis Mountbatten (1900–1979) pleaded with the Hindu and Muslim leaders to agree to form a united country, but they could not. Only Gandhi supported Mountbattens position. With the country descending further into chaos, Mountbatten reluctantly agreed to the formation of two separate states.   Mountbatten proposed that the new state of Pakistan would be created from the Muslim-majority provinces of Baluchistan and Sindh, and the two contested provinces of Punjab and Bengal would be halved, creating a Hindu Bengal and Punjab, and Muslim Bengal and Punjab. The plan gained agreement from the Muslim League and the INC, and it was announced on June 3, 1947. The date for independence was moved up to Aug. 15, 1947, and all that was left was fine-tuning, determining the physical border separating the two new states. Difficulties of Separation With the decision in favor of partition made, the parties next faced this nearly impossible task of fixing a border between the new states. The Muslims occupied two main regions in the north on opposite sides of the country, separated by a majority-Hindu section. In addition, throughout most of northern India, members of the two religions were mixed together- not to mention populations of Sikhs, Christians, and other minority faiths. The Sikhs campaigned for a nation of their own, but their appeal was denied. In the wealthy and fertile region of Punjab, the problem was extreme, with a nearly even mixture of Hindus and Muslims. Neither side wanted to relinquish this valuable land, and sectarian hatred ran high.   Ravi C. The Radcliffe Line To identify the final or real border, Mountbatten established a Boundary Commission under the chairmanship of Cyril Radcliffe (1899–1977), a British judge and rank outsider. Radcliffe arrived in India on July 8 and published the demarcation line a mere six weeks later on August 17. Punjabi and Bengali legislators were to have a chance to vote on a potential split of the provinces, and a plebiscite for or against joining Pakistan would be necessary for the North-West Frontier Province.   Radcliffe was given five weeks to complete the demarcation. He had no background in Indian affairs, nor did he have any prior experience in adjudicating such disputes. He was a confident amateur, in the words of Indian historian Joya Chatterji, chosen because Radcliffe was supposedly a nonpartisan and thus apolitical actor.   Jinnah had proposed a single commission made up of three impartial persons; but Nehru suggested two commissions, one for Bengal and one for Punjab. They would each be made up of an independent chairman, and two people nominated by the Muslim League and two by the INC. Radcliffe served as both chairs: his job was to put together a rough-and-ready plan for dividing each province as soon as possible, with the fine details to be resolved later.   On Aug. 14, 1947, the Islamic Republic of Pakistan was founded. The following day, the Republic of India was established to the south. On Aug. 17, 1947, Radcliffes award was published.   The Award The Radcliffe line drew the border right down the middle of Punjab province, between Lahore and Amritsar. The award gave West Bengal an area of some 28,000 square miles, containing a population of 21 million people, of whom about 29 percent were Muslims. East Bengal got 49,000 square miles with a population of 39 million, of whom 29 percent were Hindu. In essence, the award created two states in which the ratio of the minority population was almost identical. When the reality of the Partition hit home, residents who found themselves on the wrong side of the Radcliffe line felt extreme confusion and dismay. Worse still, most people did not have access to the printed document, and they simply did not know their immediate future. For more than a year after the award was made, rumors spread through the border communities that they would wake up to find the borders had changed again.   Post-Partition Violence On both sides, people scrambled to get onto the right side of the border or were driven from their homes by their erstwhile neighbors. At least 10 million people fled north or south, depending on their faith, and more than 500,000 were killed in the melee. Trains full of refugees were set upon by militants from both sides, and the passengers massacred. On Dec. 14, 1948, Nehru and the Pakistan Prime Minister Liaquat Ali Khan (1895–1951) signed the Inter-Dominion Agreement in a desperate attempt to calm the waters. The tribunal was ordered to resolve the boundary disputes growing out of the Radcliffe Line Award, to be headed by Swedish judge Algot Bagge and two high-court judges, C. Aiyar of India and M. Shahabuddin of Pakistan. That tribunal announced its findings in February 1950, clearing up some of the doubts and misinformation, but leaving difficulties in the definition and administration of the border.   Aftermath of Partition According to historian Chatterji, the new border ruptured agricultural communities and divided towns from the hinterlands that they had habitually relied on to supply their needs. Markets were lost and had to be reintegrated or reinvented; supply railheads were separated, as were families. The result was messy, with cross-border smuggling emerging as a thriving enterprise and an increased military presence on both sides.   On Jan. 30, 1948, Mohandas Gandhi was assassinated by a young Hindu radical for his support of a multi-religious state. Separately from Indias partition, Burma (now Myanmar) and Ceylon (Sri Lanka) gained independence in 1948; Bangladesh gained independence from Pakistan in 1971. Since August 1947, India and Pakistan have fought three major wars and one minor war over territorial disputes. The boundary line in Jammu and Kashmir is particularly troubled. These regions were not formally part of the British Raj in India, but were quasi-independent princely states; the ruler of Kashmir agreed to join India despite having a Muslim majority in his territory, resulting in tension and warfare to this day. In 1974, India tested its first nuclear weapon. Pakistan followed in 1998. Thus, any exacerbation of post-Partition tensions today- such as Indias August 2019 crackdown on Kashmiri independence- could be catastrophic. Sources Ahmad, Nafis. The Indo-Pakistan Boundary Disputes Tribunal, 1949–1950. Geographical Review 43.3 (1953): 329–37. Print.Brass, Paul R. The Partition of India and Retributive Genocide in the Punjab, 1946–47: Means, Methods, and Purposes 1. Journal of Genocide Research 5.1 (2003): 71–101. Print.Chatterji, Joya. The Fashioning of a Frontier: The Radcliffe Line and Bengals Border Landscape, 1947–52. Modern Asian Studies 33.1 (1999): 185–242. Print.Khan, Yasmin. The Great Partition: The Making of India and Pakistan. New Haven: Yale University Press, 2017. Print.Wilcox, Wayne. The Economic Consequences of Partition: India and Pakistan. Journal of International Affairs 18.2 (1964): 188–97. Print.

Composition titled there is nothing that we as individuals can do to Essay

Composition titled there is nothing that we as individuals can do to prevent climate change - Essay Example They suggest that efforts to reduce  Ã‚  emissions are unnecessary and dangerous to economic growth and development.† (University Corporation for Atmospheric Research) The emission of Carbon Dioxide from burning of Fossil fuels is primarily cited as the cause of greenhouse effect, which has caused climate changes like global warming. Our lives are heavily dependent on energy obtained from burning of fossil fuels to keep our industries, homes and transport systems running; therefore, it is impossible not to use it. Scientists are not sure what impact will minimizing greenhouse gas emissions have on climate change. Therefore, even if we as individuals try to limit the green house gases, it would have no effect on the overall climate change because after all it is a global phenomenon and it has already been triggered. The rate at which the climate is changing is so fast that we as individuals can do nothing to prevent

Culinary Arts Research Paper Example | Topics and Well Written Essays - 250 words

Culinary Arts - Research Paper Example The experience is great because class sizes are small, technical training, and allows students to work. Culinary groups are split into two shifts, morning and afternoons. Students get a real-chance of creating these dishes as it applied to their major. Students also encouraged to think freely and question the preparation meals in order to encompass it in their food making skills. The students prepared a tofu dish for a vegetarian customer, separated duck meat, and other appetizers. The dining team also prepares a wine base to ensure that the soups are being made. The baking and pastry team cut the dough and prepared the dish along with the tiramisu. A narrative of a student along with other chefs is captured in which every meal is elaborated. Meanwhile, the service team is preparing dining tables for the guest to ensure that they are ready for serving their guests. The wine team also prepares the hierarchy of wines, which provides a comprehensive accumulation of the model restaurant. Overall, all the students did a wonderful job to provide a comprehensive dining

Scientic psychology cannot live with folk psychology and cannot live Essay

Scientic psychology cannot live with folk psychology and cannot live without it - Essay Example Folk psychology explains people actions in terms of their goals however scientific psychology explains actions of individuals based on what leads them to undertake these actions. Despite the distinction scientific psychology still has some normative aspects and for this reason cannot live without folk psychology. Folk psychology has been challenged by scientific psychology and this is based on how folk psychology results are achieved, in most cases folk psychology results will conflict with results of scientific psychology. For this reason therefore it is very difficult to choose which results best explain human behavior, due to conflicting results of the two disciplines scientific psychology tend to criticize the results of folk psychology and for this reason scientific psychology cannot live with folk psychology. Folk psychology assumes that people already know why they tend to believe in their actions and why they take these actions, according to scientific psychology people are not aware of why they take certain actions, this is a conflict of assumption and therefore it is evident that scientific psychology cannot live with folk psychology. According to Stephen Stitch it is possible to undertake scientific psychology without folk psychology where he gave an example using beliefs. ... gave an example of a day that had rained and the two characters X and Y are asked to narrate on what had happened, X said that the day had rained very heavily and Y said that it did not rain very heavily. He posed the question who of the two individuals was telling the truth, however Stephen Stitch said that these two persons may telling the truth but it depends on their understanding and observations on the way they have learned from the groups, that is family or friends on how they belief in a particular aspect. According to J.B Watson it is possible to identify someone's behavior at his or her current age as long as he or she is not in the childhood age but it depends on how the person behaves and socializes. For this reason therefore Watson verifies that it is only necessary to study the persons behavior without taking into consideration believes of the individuals, in his statement he states that it is possible for scientific psychology to live without folk. Folk Psychology assumes that an individual can directly explain the different feelings they experience, this is to say that individuals are well aware of what they feel and why they feel it, on the other hand scientific psychology agues that individuals feeling are based on the inferences about changes in what they are undergoing. For this reason therefore there is a conflict of assumptions that people are ware of their feelings and for this reason both disciplines will yield different results, for this reason therefore scientific psychology cannot live with folk psychology. Why scientific psychology cannot live without folk psychology: Despite the many reasons why scientific psychology cannot live with folk psychology it is evident that scientific psychology cannot live without folk psychology, folk

Episodic Dystonia and Hallucinations Due to DLAT Genes

Episodic Dystonia and Hallucinations Due to DLAT Genes Title: Carbamazepine responsive Episodic Dystonia and Hallucination due to Pyruvate Dehydrogenase E2 (DLAT) gene mutation Fatema J Serajee1, Salman Rashid2, and AHM M Huq1 ABSTRACT: BACKGROUND: PDH E2 deficiency due to DLAT mutations is a very rare condition with only 4 reported cases to date. METHODS: We describe a 15-year-old girl with mild intellectual disability, paroxysmal dystonia and bilateral basal ganglia signal abnormalities on brain MRI. Additional neurophysiological, imaging, metabolic and exome sequencing studies were performed. RESULTS: Routine metabolite testing, and GLUT1 and PRRT2 mutation analysis were negative. A repeat brain MRI revealed Eye-of-the-tiger-sign. Exome sequencing identified homozygous valine to glycine alteration at amino acid position 157 in the DLAT gene. Bioinformatic and family analyses indicated that the alteration was likely pathogenic. Patients s dystonia was responsive to low dose carbamazepine. On weaning carbamazepine, patient developed hallucinations which resolved after carbamazepine was restarted. CONCLUSIONS: PDH E2 deficiency due to DLAT mutation has a more benign course compared to common forms of PDH E1 deficiency due to X-linked PDHA1 mutations. All known cases of PDH E2 deficiency due to DLAT mutations share the features of episodic dystonia and intellectual disability. Our patients dystonia and hallucinations responded well to low dose carbamazepine. Introduction: Pyruvate Dehydrogenase (PDH) E2 deficiency is a rare pediatric neurometabolic disease due to mutation in DLAT gene (Head et al., 2005; McWilliam et al., 2010). Only 4 cases with DLAT gene mutations have previously been reported (Head et al., 2005; McWilliam et al., 2010). All share the features of dystonia and some degree of developmental delay and characteristic globus pallidus signal abnormalities on brain MRI. This disease tends to have more benign course as compared to PDH E1 deficiency (Head et al., 2005; Huq et al., 1991; McWilliam et al., 2010; Patel et al., 2012). We report an additional case with DLAT mutation with new phenotype and treatment information. Case Report A 15-year-old girl presented with paroxysmal episodes of left lower extremity weakness and stiffening for the last 8 years. These episodes were triggered by exercise but no exacerbating or relieving factors were noted. There was no associated aura, alteration of consciousness, incontinence or other associated neurological symptoms. Her parents were second cousins, but family history was negative for known genetic disorders. Birth and past medical histories were also unremarkable. Patient had a speech delay but met her other childhood milestones appropriately. Later, she developed academic difficulties and at 15 years of age she was performing at a 4th grade level. At presentation, the patient had a normal examination except for some cognitive and reading difficulties. At the time of initial presentation to a pediatric neurologist at 7 years of age, an MRI of the brain revealed bilateral T2 hyperintensities in the basal ganglia. In addition, she was found to have decreased NAA peak an d the suggestion of a lactate peak on MR spectroscopy. EEG, EMG and nerve conductions studies were unremarkable. Over the years the patient was considered to have paroxysmal kinesiogenic dyskinesia and was treated with carbamazepine (100 mg daily). The patient was initially evaluated by us at age of 14 years. Metabolic work up for serum lactate, serum amino acids, acyl carnitine profile, serum copper and ceruluplasmin and GLUT1 or PRRT2 mutation analysis were unremarkable. Repeat MRI revealed basal ganglia signal changes including Eye of the tiger sign (Figure 1). MR spectroscopy studies were suboptimal. Exome sequencing was performed through Ambry laboratory as previously described (Serajee and Huq, 2015). The patient had homozygous c.470T>G (p.V157G) alteration in the DLAT (Dihydrolipoamide acetyltransferase (PDHC E2) gene suggesting the diagnosis of pyruvate dehydrogenase E2 deficiency, a rare cause of pyruvate dehydrogenase deficiency. Both parents and one brother were heterozygous carriers and another brother was homozygous normal. The p.V157G alteration (c.470T>G), is in coding exon 3 of the DLAT gene, results from a T to G substitution at nucleotide position 470. The valine at codon 157 is replaced by glycine, an amino acid with dissimilar properties. The V157 amino acid position is highly conserved in all available vertebrate species. The p.V157G alteration is predicted to be probably damaging by Polyphen and deleterious by SIFT in silico analyses. The V157 amino acid is located within the biotin/lipoyl attachment domain of the DHAT protein. The DLAT c.470T>G alteration was n ot observed in healthy cohort databases such as NHLBI Exome Sequencing Project (ESP) or the 1000 Genomes Project or the Database of Single Nucleotide Polymorphisms (dbSNP). Based on data from the HGMD, only the four alterations reported by Head et al. (2005) and McWilliam et al. (2010) have been observed within the DLAT gene to date (Head et al., 2005;McWilliam et al., 2010). These include one missense alteration, two splice alterations, and one small in-frame deletion. Based on the above evidence, the homozygous c.470T>G (p.V157G) alteration was considered pathogenic. Her parents refused treatment with the ketogenic diet. When carbamazepine was weaned off due to parental concerns of side effects, within few weeks, patient developed hallucinations. Parents reported resolution of symptoms after carbamazepine was restarted. Discussion: The Pyruvate Dehydrogenase Complex functions in the oxidative decarboxylation of pyruvate to acetyl coenzyme A. The complex contains three subunits: E1, E2 and E3 (Patel and Roche, 1990). The most common form of pyruvate dehydrogenase deficiency is due to mutations affecting the E1 subunit, and results in a variety of clinical manifestations depending upon the residual function of the enzyme (Huq et al., 1991;Patel et al., 2012). E1 subunit is encoded by PDHA1 gene of X chromosome. Most patients present in infancy with lactic acidosis, ataxia and hypotonia, either chronically or episodically (Huq et al., 1991;Patel et al., 2012). The mutation in our patient is in the E2 subunit (dihydrolipoamide acetyltransferase), which forms the structural core of the enzyme and functions in accepting the acetyl groups and transferring them to coenzyme A, an essential step preceding the entrance of glucose into the TCA cycle (Head et al., 2005;Patel and Roche, 1990). E2 subunit is encoded by DLAT g ene located on chromosome 11q23.1. To date, however, there are only four reported cases of pyruvate dehydrogenase deficiency caused by alterations in the DLAT gene, making it a very rare cause of the condition (Head et al., 2005;McWilliam et al., 2010). In addition, Robinson et al reported an additional patient with reduced E2 dihydrolipoyl transacetylase enzyme activity (32% of the control and undetectable E2 immunoreactive protein (Robinson et al., 1990). For this patient, no gene mutation data is available (Robinson et al., 1990). The patient reported by Robinson et al. had a different phenotype compared to our patient and four other genetically confirmed DLAT mutation cases and had profound retardation and microcephaly (Robinson et al., 1990). Head et al. (2005) first described two unrelated individuals with PDH deficiency caused by homozygous non-protein truncating mutations in the DLAT gene (Head et al., 2005). One patient demonstrated a deletion of glutamic acid in the outer lipoyl domain of the protein, whereas the second expressed a missense mutation in the catalytic site, leading to a substitution of leucine for phenylalanine. Both patients were male children born of first-cousin parents. These patients presented with a less severe phenotype compared to individuals with the more common type of PDH caused by alterations in the PDHA1 gene encoding the E1 subunit, and their common features included episodic dystonia, hypotonia, ataxia, and developmental delay(Head et al., 2005). Episodes of dystonia were often triggered by stress or fever, and developmental progress appeared to slow after the episodes as well. Additional reported features included inconsolable crying, nystagmus and abnormal eye movements, ptosis, drooli ng, jerky head movements, arching of the body, bottom shuffling, stiffening of the limbs, episodic clenching of the hands, head lag and hypotonia. Brain MRI findings in each patient included focal signal abnormality in the basal ganglia with high T2 signal and low T1 signal in the globus pallidus which was compatible with an abnormality of energy metabolism (Head et al., 2005). The authors concluded that mutations in the DLAT gene are an extremely rare cause of PDH deficiency and that patients with this type of PDH may be more likely to respond to a ketogenic diet (Head et al., 2005). McWilliam et al. (2010) also described two sisters born of non-consanguineous parents affected with pyruvate dehydrogenase E2 deficiency caused by compound heterozygous splice mutations in the DLAT gene (McWilliam et al., 2010). Clinical features were like those described in Head et al. (2005), including progressive episodic dystonia, cognitive impairment, and globus pallidus hyperintensity on brain MR I. Both patients were treated with a modified ketogenic diet and the parents reported improvements in concentration, fine motor control, and decreased fatigue (McWilliam et al., 2010). Previous reports noted the phenotypic overlap to patients with PKAN, and suggested investigation for PDH E2 deficiency in patients suspected to have atypical PKAN with negative genetic testing (Head et al., 2005;McWilliam et al., 2010). PKAN is one of several diseases classified under the umbrella of neurodegeneration with brain iron accumulation (NBIA). It is caused by a mutation in the pentothenate kinase 2 gene, an abnormality of coenzyme A metabolism (Zhou et al., 2001). CoPAN (Coenzyme A synthetase protein-associated neurodegeneration) is another NBIA that affects the synthesis of coenzyme A. It is caused by a mutation in coenzyme A synthetase (COASY) gene (Schneider, 2016;Tonekaboni and Mollamohammadi, 2014). Clinical features of PKAN and CoPAN also include ataxia, dystonia, chorea and Parkinsonism, cognitive decline and psychiatric manifestations (Schneider, 2016;Tonekaboni and Mollamohammadi, 2014). In NBIA, whether iron accumulation is a cause or an effect of the disease pro cess is still not known (Schneider, 2016;Tonekaboni and Mollamohammadi, 2014). In our patient, the pattern of MRI changes in the bilateral globus pallidus is remarkably like that seen in PKAN and CoPAN, revealing the eye-of-the-tiger sign . On brain MRIs of patients with PKAN and CoPAN, the central hyperintensity of the eye-of-the-tiger sign is thought to be due to the tissue necrosis, while the surrounding hypointensity is attributed to the iron accumulation (Dusi et al., 2014;Kumar et al., 2006). Other diseases including cortical basal degeneration, multisystem atrophy, multiple sclerosis and neurofibromatosis may have similar neuro-radiological findings. However, these diseases differ from PKAN in their clinical behavior and pattern of MR abnormalities (Kruer et al., 2012). PKAN and CoPAN also affect the substantia nigra (Kruer et al., 2012); however, the involvement of the substantia nigra has not yet been reported in cases of PDH E2 deficiency due to DLAT mutations. Out of the four previously reported cases of PDH E2 deficiency due to DLAT mutation, only t wo patients had serial MRI scans. In one patient, the brain MRI was normal at one year of age but follow up at 6 years-old showed an abnormal hyperintense T2 signal in the bilateral globus pallidus. In the other patient, similar lesions were noticed at 15 months-old that remained unchanged on follow up at 2 and 6 years of age (Head et al., 2005). As opposed to the eye-of-the-tiger sign seen in our patient, all the previously reported cases showed homogenous basal ganglia hyperintensities (Head et al., 2005;McWilliam et al., 2010). As discussed above, PKAN and CoPAN result from a defect in coenzyme A synthesis (Schneider, 2016;Tonekaboni and Mollamohammadi, 2014). PDH E2 deficiency due to DLAT mutations, on the other hand, affects the transfer of acetyl group formed by decarboxylation of pyruvate to coenzyme A (Kumar et al., 2006;McWilliam et al., 2010;Patel and Roche, 1990). It is possible that the clinical and radiological similarities of PKAN, CoPAN and PDH E2 deficiency are due t o shared abnormalities in the acetyl-CoA metabolism. Like our patient, the 4 previously reported DLAT mutation cases presented with dystonia and intellectual disability, with a more benign course than those affected with the PDH E1 subunit deficiency (Head et al., 2005;McWilliam et al., 2010) . In this regard, PDH E2 deficiency due to DLAT mutation is like PDH deficiency due E3 binding protein deficiency (Head et al., 2005). Serum and CSF lactate were elevated in only one patient, but all demonstrated characteristic hyperintense T2 and hypointense T1 signal in the bilateral globus pallidi on brain MRI. In 3 out of the 4 patients, ketogenic diet was helpful in alleviation of the disease symptomology(Head et al., 2005;McWilliam et al., 2010). Our patients had hallucination, which was not described in other 4 reported patients. Her dystonia and hallucinations responded to low dose carbamazepine. Our case thus expands upon the phenotype for PDH E2 deficiency associated with the DLAT gene mutation. Reference List Dusi, S., Valletta, L., Haack, T.B., Tsuchiya, Y., Venco, P., Pasqualato, S., Goffrini, P., Tigano, M., Demchenko, N., Wieland, T., Schwarzmayr, T., Strom, T.M., Invernizzi, F., Garavaglia, B., Gregory, A., Sanford, L., Hamada, J., Bettencourt, C., Houlden, H., Chiapparini, L., Zorzi, G., Kurian, M.A., Nardocci, N., Prokisch, H., Hayflick, S., Gout, I., and Tiranti, V. (2014). Exome sequence reveals mutations in CoA synthase as a cause of neurodegeneration with brain iron accumulation. Am. J. Hum. Genet. 94, 11-22. Head, R.A., Brown, R.M., Zolkipli, Z., Shahdadpuri, R., King, M.D., Clayton, P.T., and Brown, G.K. (2005). Clinical and genetic spectrum of pyruvate dehydrogenase deficiency: dihydrolipoamide acetyltransferase (E2) deficiency. Ann. Neurol. 58, 234-241. Huq, A.H., Ito, M., Naito, E., Saijo, T., Takeda, E., and Kuroda, Y. (1991). Demonstration of an unstable variant of pyruvate dehydrogenase protein (E1) in cultured fibroblasts from a patient with congenital lactic acidemia. Pediatr. Res. 30, 11-14. Kruer, M.C., Boddaert, N., Schneider, S.A., Houlden, H., Bhatia, K.P., Gregory, A., Anderson, J.C., Rooney, W.D., Hogarth, P., and Hayflick, S.J. (2012). Neuroimaging features of neurodegeneration with brain iron accumulation. AJNR Am. J. Neuroradiol. 33, 407-414. Kumar, N., Boes, C.J., Babovic-Vuksanovic, D., and Boeve, B.F. (2006). The eye-of-the-tiger sign is not pathognomonic of the PANK2 mutation. Arch. Neurol. 63, 292-293. McWilliam, C.A., Ridout, C.K., Brown, R.M., McWilliam, R.C., Tolmie, J., and Brown, G.K. (2010). Pyruvate dehydrogenase E2 deficiency: a potentially treatable cause of episodic dystonia. Eur. J. Paediatr. Neurol. 14, 349-353. Patel, K.P., OBrien, T.W., Subramony, S.H., Shuster, J., and Stacpoole, P.W. (2012). The spectrum of pyruvate dehydrogenase complex deficiency: clinical, biochemical and genetic features in 371 patients. Mol. Genet. Metab 105, 34-43. Patel, M.S., and Roche, T.E. (1990). Molecular biology and biochemistry of pyruvate dehydrogenase complexes. FASEB J. 4, 3224-3233. Robinson, B.H., MacKay, N., Petrova-Benedict, R., Ozalp, I., Coskun, T., and Stacpoole, P.W. (1990). Defects in the E2 lipoyl transacetylase and the X-lipoyl containing component of the pyruvate dehydrogenase complex in patients with lactic acidemia. J. Clin. Invest 85, 1821-1824. Schneider, S.A. (2016). Neurodegeneration with Brain Iron Accumulation. Curr. Neurol. Neurosci. Rep. 16, 9. Serajee, F.J., and Huq, A.M. (2015). Homozygous Mutation in Synaptic Vesicle Glycoprotein 2A Gene Results in Intractable Epilepsy, Involuntary Movements, Microcephaly, and Developmental and Growth Retardation. Pediatr. Neurol. 52, 642-646. Tonekaboni, S.H., and Mollamohammadi, M. (2014). Neurodegeneration with brain iron accumulation: an overview. Iran J. Child Neurol. 8, 1-8. Zhou, B., Westaway, S.K., Levinson, B., Johnson, M.A., Gitschier, J., and Hayflick, S.J. (2001). A novel pantothenate kinase gene (PANK2) is defective in Hallervorden-Spatz syndrome. Nat. Genet. 28, 345-349. Figure 1 Legend:   Ã‚   MRI of the brain: A. Axial T2 image showing hyperintensity in bilateral globus pallidus, which is surrounded by a hypointense signal B. Axial T1 image showing hypointensity in bilateral globus pallidus C. Coronal T2 FLAIR image showing hyperintensity in bilateral globus pallidus, which is surrounded by a ring of hypointense signal (eye-of-the-tiger sign)

Inner Truths in The House of the Seven Gables :: House of the Seven Gables Essays

Inner Truths in The House of the Seven Gables It was Hawthorne’s belief that romances deal with inner truths, while novels are based on "mere fact." Because he held himself to be a romance writer, inner truths were elemental themes in The House of the Seven Gables. The truths that he conceived, and expressed, in the story range from the concept that death and suffering do not discriminate based on one’s position in society to the karmic effects one generation may have on those of future generations. Hawthorne saw these themes as important concepts that went beyond simple didactic commentaries. As a romance writer he wanted his reader to understand his conceptions on a complete level, and to achieve this he realized that he must delve into an unusual space in the reader's mind. The supernatural plays an important role in this goal in The House of the Seven Gables. The Supernatural challenges the reader to use her imagination and step out of her usual stereotypes and beliefs so that she may observe the story as Hawt horne wrote it. This challenge is meant to help the reader grasp Hawthorne’s conceptions. Maule’s curse at the gallows is the beginning of the development for one of Hawthorne’s central themes: guilt will stay for generations. In regards to this "karmic" theme, Maule’s curse, a supernatural power, foreshadows the future of the Pyncheon family. Maule insists, "God will give him blood to drink!" and as we read on it appears that this portion of the curse does indeed come to pass. But the effects of the curse do not end there. As men began to build the Pyncheon home on Maule’s land, the famous spring water on the property "entirely lost the deliciousness of its pristine quality." The land that Colonel Pyncheon intended to have for his family immediately started losing its value as the "pristine" well became foul. As the story goes on it, becomes clear that the curse will similarly effect the Pyncheon family, making what once was rich very poor. Maule’s supernatural power is further developed with the use of ghosts. The use of these spirits implies that all inhabitants of the house are in a state of unrest. Although Colonel Pyncheon was the one to commit the sin against Maule, all his relatives will pay for the deed. Alice Pyncheon was said "to haunt the House of the Seven Gables and.