THE PLACE OF POPULAR CULTURE - MUSIC AND THE CITY Essay

THE PLACE OF POPULAR CULTURE - MUSIC AND THE CITY - Essay Example Even though music could be said to be part of the very traditional facets of culture, there is no denying the fact that the changing trend of music whereby it had become more versatile and dynamic has made it jumped into a new array of description, which is the popular culture description. New York is one place that has a culture of its own. Through the power and potency of music as a global communication tool, the culture of New York City has been projected by various artists and songstresses who pick various themes about the locality of New York City and amplifies them to the larger world. In this paper, the concept of cultural geography is scrutinized with a link of it to culture and how the local musical space of New York City has helped in projecting the cultural values of the city. This will be done with specific analysis of the song, A Bavarian in New York by Triumvirat. Cultural Geography and how culture is linked to place Even though cultural geography generally comes under the field of human geography, it is largely rooted in the phenomenon of culture and actually revolves around it. This is because cultural geography delves into cultural outcomes and norms that exist across spaces and places (Knox and Marston, 2012). Cultural geography also refers to the relations that exist in spaces and places differentiations as pertains to variables of culture such as religion, language, economy, and morality. Through cultural geography therefore, it should be possible to distinguish one place from another through the inputs of their cultural practices. In effect, culture is directly linked to a place because culture helps in giving a place its differential identity from other places. It is not surprising therefore through tourism, people travel across spaces and places to experience the cultural dynamism of other places. When debating the issue of cultural geography, reference is commonly made to countries, eve n though most cities have their own influential cultural geographies. Cultural geography can therefore be narrowed down to cities very easily, especially when reference is made to cities that are found in cosmopolitan countries like United States, where States and Cities try to leave independent of each other. With this said, New York City comes to mind easily as one city with so much to offer in terms of cultural geographic identity. Why music has a strong place in Geographic Orientation The discussion on cultural geography would be most inadequate if music as a variable of popular culture is not discussed. This is because music, particularly local musical spaces give music so much power and influence in the cultural geography of any given city. In reality, music has the kind of strong place it has in geographic orientation because music can be used as a very powerful communication tool beyond the mere purpose of entertainment. Discussing music as a variable of culture, aspects of music such as theme, language, rhythm, genre, and message can all be identified. Through themes of music, musicians are able to use their works of songs to highlight specific cultural themes that exist in given geographic orientations. This way, the attention of all people hearing the song is quickly drawn on the unique themes of the said geographic location, such as a city. The rhythm and genre of music have also been associated with certain geographic

Art History of 20th Century Research Paper Example | Topics and Well Written Essays - 1250 words

Art History of 20th Century - Research Paper Example Pollok made most of his painting on canvas rather than on the wall, and this was to make them more portable. Most of his works were a mixture of controllable and uncontrollable factors1. Lynda Benglis on her part is an American sculptor and visual artist majorly known for her wax painting and poured latex sculptures. She also used drip style to make her works. She was a professional photographer as she had studied artwork at a college. Given that most of her works were sensual in nature, they were ignored for a long time. Â  Given that Pollok paintings were made on canvas rather than on walls, which is common. His paintings were or portable and could easily move to the museum. This work is significant and, therefore, can be easily seen by those visiting the place. Their placement on the wall at points that it could be viewed easily by people of all heights since it’s not too high or too low. A group of viewers can as well look at it at the same as it is large and full2. Pollok believed that the size of his works had a direct relationship with how and to what extent viewers and he would bond and be part of the painting. Lynda Benglis, on the other hand, made a sculpture that could not be hanging but place on a flat surface for viewing. Her sculpture is smaller compared to the paintings of Pollok. It is only visible to a keen viewer who had the intention of looking for this particular artwork in the gallery. The location of Benglis’ sculpture Batt puts it out of sight and away from other artistic works with which they fall into the same category. The disadvantage to the painter and the lovers of their works as it takes longer to locate them thus according to them with little or no publicity. Â  

Jefferson Davis on pursuit for happiness Essay Example | Topics and Well Written Essays - 500 words

Jefferson Davis on pursuit for happiness - Essay Example Thomas Jefferson described life, liberty, and the pursuit of happiness as â€Å"inherent† and â€Å"inalienable†, or in other words, natural and undeniable rights (Jefferson and Peterson, 7). Jefferson wanted the Thirteen Colonies to form a government that protected the right of life. At the time this meant protecting citizens of the Colonies from war and Native Americans, but has come to mean much more. Americans have the right to procreate or not. Individuals are not limited to how many children they have like in China. Life is not terminated for lack of funding, like in Nazi Germany. The United States protects American lives worldwide from Jefferson’s time up until today. The second natural or undeniable right was liberty. Liberty is sometimes confused with freedom. True freedom is anarchy, because everyone does what they want. Liberty is the freedom to have the same opportunities as everyone else, obey natural laws. An example would be the freedom to worship God, Allah, or Jehovah anyway a person feels the need to. Jefferson spoke â€Å"of liberty to worship our Creator in the way we think is most agreeable to his will† (Jefferson and Peterson, 19). Liberty means equality for everyone. At the time Jefferson wrote the Declaration of Independence, he owned slaves. This contradicted his position on equal opportunity for everyone. When he spoke of liberty, Jefferson meant white men. Jefferson knew that the democracy he was fighting for was far from perfect, but could become better by always changing. Jefferson stated, â€Å"’The price of freedom,’ as Thomas Jefferson observed, ‘is eternal vigilance’† (Washburne). Through eternal vigilance, the United States has more liberty today than in Jefferson’s day. Natural laws are laws that come natural to humans. Most individuals think thievery, murder, rape, and torture is wrong. On the other hand, most individuals believe taking care of children, labor,

THE PLACE OF POPULAR CULTURE - MUSIC AND THE CITY Essay

THE PLACE OF POPULAR CULTURE - MUSIC AND THE CITY - Essay Example Even though music could be said to be part of the very traditional facets of culture, there is no denying the fact that the changing trend of music whereby it had become more versatile and dynamic has made it jumped into a new array of description, which is the popular culture description. New York is one place that has a culture of its own. Through the power and potency of music as a global communication tool, the culture of New York City has been projected by various artists and songstresses who pick various themes about the locality of New York City and amplifies them to the larger world. In this paper, the concept of cultural geography is scrutinized with a link of it to culture and how the local musical space of New York City has helped in projecting the cultural values of the city. This will be done with specific analysis of the song, A Bavarian in New York by Triumvirat. Cultural Geography and how culture is linked to place Even though cultural geography generally comes under the field of human geography, it is largely rooted in the phenomenon of culture and actually revolves around it. This is because cultural geography delves into cultural outcomes and norms that exist across spaces and places (Knox and Marston, 2012). Cultural geography also refers to the relations that exist in spaces and places differentiations as pertains to variables of culture such as religion, language, economy, and morality. Through cultural geography therefore, it should be possible to distinguish one place from another through the inputs of their cultural practices. In effect, culture is directly linked to a place because culture helps in giving a place its differential identity from other places. It is not surprising therefore through tourism, people travel across spaces and places to experience the cultural dynamism of other places. When debating the issue of cultural geography, reference is commonly made to countries, eve n though most cities have their own influential cultural geographies. Cultural geography can therefore be narrowed down to cities very easily, especially when reference is made to cities that are found in cosmopolitan countries like United States, where States and Cities try to leave independent of each other. With this said, New York City comes to mind easily as one city with so much to offer in terms of cultural geographic identity. Why music has a strong place in Geographic Orientation The discussion on cultural geography would be most inadequate if music as a variable of popular culture is not discussed. This is because music, particularly local musical spaces give music so much power and influence in the cultural geography of any given city. In reality, music has the kind of strong place it has in geographic orientation because music can be used as a very powerful communication tool beyond the mere purpose of entertainment. Discussing music as a variable of culture, aspects of music such as theme, language, rhythm, genre, and message can all be identified. Through themes of music, musicians are able to use their works of songs to highlight specific cultural themes that exist in given geographic orientations. This way, the attention of all people hearing the song is quickly drawn on the unique themes of the said geographic location, such as a city. The rhythm and genre of music have also been associated with certain geographic

Jefferson Davis on pursuit for happiness Essay Example | Topics and Well Written Essays - 500 words

Jefferson Davis on pursuit for happiness - Essay Example Thomas Jefferson described life, liberty, and the pursuit of happiness as â€Å"inherent† and â€Å"inalienable†, or in other words, natural and undeniable rights (Jefferson and Peterson, 7). Jefferson wanted the Thirteen Colonies to form a government that protected the right of life. At the time this meant protecting citizens of the Colonies from war and Native Americans, but has come to mean much more. Americans have the right to procreate or not. Individuals are not limited to how many children they have like in China. Life is not terminated for lack of funding, like in Nazi Germany. The United States protects American lives worldwide from Jefferson’s time up until today. The second natural or undeniable right was liberty. Liberty is sometimes confused with freedom. True freedom is anarchy, because everyone does what they want. Liberty is the freedom to have the same opportunities as everyone else, obey natural laws. An example would be the freedom to worship God, Allah, or Jehovah anyway a person feels the need to. Jefferson spoke â€Å"of liberty to worship our Creator in the way we think is most agreeable to his will† (Jefferson and Peterson, 19). Liberty means equality for everyone. At the time Jefferson wrote the Declaration of Independence, he owned slaves. This contradicted his position on equal opportunity for everyone. When he spoke of liberty, Jefferson meant white men. Jefferson knew that the democracy he was fighting for was far from perfect, but could become better by always changing. Jefferson stated, â€Å"’The price of freedom,’ as Thomas Jefferson observed, ‘is eternal vigilance’† (Washburne). Through eternal vigilance, the United States has more liberty today than in Jefferson’s day. Natural laws are laws that come natural to humans. Most individuals think thievery, murder, rape, and torture is wrong. On the other hand, most individuals believe taking care of children, labor,

Women language Essay Example for Free

Women language Essay One of the debates which is of long standing within the study of linguistics is concerned with whether women speak in a language and perceive situations in a manner significantly different from that of men. Many theorists both feminists and anti-feminists have attempted to prove that women do indeed speak in a different way from men; and that there is such a thing as the â€Å"women language†. The debate attempts to place in context matters such as sex differences in linguistic behavior at the level of phonology and to a lesser extent grammar. The concepts upon which the debate has been built however reflect, to a greater or lesser extent, the influence of feminism over the past two decades. Feminism has fore grounded issues of gender differences and male domination in society; it has prompted a concern with putting women ‘on the map’ and a critical reappraisal of feminist and non-feminist research. The discussions on gender linguistics therefore focus on the feminist projects of ‘redressing the balance’, as manifested in the academic field of languages. Phonology Cognitive research on gender differences has drawn interest from linguistic experts and language scholars. Holmes and Meyeroff (2003, p. 144), the research more often than not directs itself to the hypothesis that women posses much better verbal skills than men. Most of the studies have drawn the conclusion that there are in fact differences based on gender, in verbal performance and verbal cognitive processing. It is often believed that females are far better in reading and posses much stronger language acquisition skills than males. Many scholars equate this probability to the phonological superiority possessed by women, (Christie 2002, p. 102). The difference in phonological ability is often attributed by some researchers and scholars to the structure of the brain which in this case favors the female. Studies suggest that the left hemisphere of the brain which is much more developed in males is superior in mathematical and scientific processing and analysis (direct speech). On the other hand the right hemisphere (much highly developed) in females is much more superior in word structure, grammar recognition and verbalization (indirect speech), (Christie 2002, p. 104). However the evidence provided to support this theory has often drawn inconsistent results making it much difficult to prove. Another school of researchers in linguistics suggests that culture is the main foundation for any form of language processing and development. For this school of scholars the role of women as caregivers in society makes them much more sensitive in their speech structure and verbalization. They know that they must be more knowledgeable in the different aspects of language and speech, (Holmes and Meyerhoff 2003, p. 142). The women are the first teachers for the child and therefore they take much more time to understand the speech techniques and pronunciation so they can be able to direct the children much easily. The men’s social role as providers on the other hand, pre-disposes them to poor performance in reading, sounds and sentence structure. This means that women easily develop direct and indirect speech much easily than men. Men are much better at direct speech. Use of Slang In historical society and the study of linguistics, women have been given the conservative linguistic role while men are often considered much more creative, and innovative in their language use. In traditional societies, subordination of women denied them the chance to use creative, innovative language that violated the speech protocol. It is from this perspective that many scholars develop the notion that women are less likely to develop â€Å"slang† or terms considered as â€Å"slang† in their speech. Medieval society put in place taboos and regulations that denied women the chance to distort conventional language regulations. Women had to use respectable language, refrain from swearing and insults; and use properly structured words and sentences. Grammatical errors and poor pronunciations were frowned upon, a woman’s speech was considered a reflection of her character and conduct, (Christie, 2000, p. 154). Surprisingly this practice is still present in today’s modernized society. We all have a tendency to judge and draw conclusions regarding an individual’s character from their command of conventional language, moreso women. Although there is no evidence that the use of ‘slang’ is more predominant in a specific gender than the other; it is found that men revert to the use of ‘slang’ terminologies much more easily than women. Even when women are influenced by the use of ‘slang’ and they incorporate ‘slang’ terminologies in their speech, the difference can be seen from the choice of terminologies. Women tend to use more euphemistic terminologies, while men apply complete use of ‘slang’ vocabulary. Use of Polite Speech In any form of communication, the speaker often hopes to get respect from his audience or the people he is speaking to. The difference in language and speech comes from how the women and men perceive respect. While the men view fear and admirations as forms of respect, women view agreement, generosity and sympathy as the measures of respect. Socio-linguists use this reason to explain why women are much more pre-disposed to the use of polite language than men. The mutual agreement is that women find polite language to be much more productive than men who prefer aggressive terms. Men are socialized by society to use rougher and more commanding language than women. Women on the hand are required to â€Å"act like ladies†, be respectable and meek. Coates (1998, p. 87) women are much more likely to use polite words such as please, excuse me and thank you than men. Women identify with the lesser classes and the abused or misused, and find it natural to appreciate others. Men on the other hand are socialized to demand action, appreciation and praise especially from women. Though being male does not necessarily mean that one is rude and arrogant, it does mean that the person is less likely to say please or thank you. In written literature, feminist scholars have often insinuated that female writers have developed what is known as the â€Å"female sentence†, which is structured to appeal to the polite nature of the female reader as well as the writer. Women are socialized to be much more submissive, as young girls for example sharing use of polite language and general respect to others is applauded. On the other hand boys are encouraged to be much more aggressive and demanding. It is this form of socialization rather than the biological composition of gender that linguists believe conditions women to be much more polite than men. Conversation Dominance A study conducted in 1975 by psychologist and linguist Zimmerman and West, based on a turn taking conversation model revealed that men are much more likely to dominate a conversation through interruption than females, (as quoted in Coates 1998,p. 161). Men feel the need to be dominant in all areas of their life, always commanding the full attention of others. They employ strategies such as interruption, questions in the middle of sentences and change of topics to maintain the dominance of the conversation. Men are well versed in strategies such as ‘no response’, interrupting, poor and inadequate responses and silence employed to change the path of the conversation to what suits them best. Eckert and McConnell- Ginnet (2003, p. 121) state that women may resort to arguments and shouting much easily when ignored, but they also tend to calm and compromise easily. However men are much more adamant, they can become completely uncooperative when ignored. While women have a tendency to imply what they would like to address or their wants and needs, men just tend to come out and directly demand what they want. Men are more confident with voicing their own needs even when they are infringing on some one else’s needs and time. As children parents and the society in general socializes the young girl to be more compromising allowing the male to get the attention they seem to thrive on. The male on the other hand is socialized to fight for and demand this attention. Conclusion The analysis of language and speech in gender provides a reflection of the cultural roles and status in society. The biological composition of each sex is less important in the development of language as compared to the socialization patterns utilized by different cultures. In general women are much more likely to develop subjective language and speech, easily influenced by the people they are speaking to. Men however are much more technical and object oriented in their language and speech, incorporating as little emotion as they can. To understand the difference in speech and language between men and women, one must first understand the gender roles and status defined by specific cultures. Works Cited 1. Christie Christine. Gender and Language: Towards Feminist Pragmatics. Edinburgh. Edinburgh University Press . 2000. 2. Coates Jennifer. Language and Gender: A Reader. Massachusetts. Blackwell Publishing 1998. 3. Eckert Penelope and McConnell -Ginet Sally. Language and Gender. United Kingdom. Cambridge University Press. 2003 4. Holmes Janet and Meyerhoff Miriam. Handbook of Language and Gender. Massachusetts. Blackwell Publishers. 2003.

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). 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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)