Posterior Reversible Encephalopathy (PRES)

Posterior Reversible Encephalopathy (PRES) Posterior Reversible Encephalopathy (PRES): A Rare Presenting Feature of Pheochromocytoma Abstract Hypertension in young is mostly due to secondary causes and one of them is pheochromocytoma. These are catecholamine secreting enterochromaffin tumors causing paroxysmal hypertension. Adrenal pheochromocytoma presenting as posterior reversible encephalopathy syndrome (PRES) is very rare and has not been described in literature so far. Here, we report a case of previously healthy adolescent boy, who presented with acute onset severe headache, blurring of vision, generalized tonic clonic motor seizure and altered sensorium. His blood pressure was 234/126 mm Hg. The magnetic resonance imaging (MRI) of brain showed hyperintense signal on T2-weighted and fluid attenuated inversion recovery (FLAIR) images in bilateral parietal and occipital regions. High blood pressure and classical MRI findings were consistent with the diagnosis of PRES. Abdominal ultrasonography (USG) revealed a right adrenal mass. A diagnosis of pheochromocytoma was confirmed by abdominal triple phase contrast- computed tomography (CT) and 24-hour urinary metanephrine assay. After the blood pressure was stabilized with alpha and beta blockade, adrenal tumor was surgically excised. Histopathologic examination of tissue confirmed the diagnosis of pheochromocytoma. The MRI brain showed complete resolution of hyperintense signals (T2-weighted and FLAIR images) on two-months follow-up. He was symptom free at six months and one year follow-up. Key words: Posterior reversible encephalopathy syndrome (PRES); Reversible posterior leukoencephalopathy (RPLE); Pheochromocytoma; Hypertensive Encephalopathy; Hypertension Abbreviations: ADC: apparent diffusion coefficient; CT: computed tomography; MRI: magnetic resonance imaging; DWI: diffusion-weighted imaging; PRES: posterior reversible encephalopathy syndrome Introduction Hypertension in young is mostly due to secondary causes which include renal diseases (chronic renal failure, renal artery stenosis, polycystic kidney disease), coarctation of the aorta, systemic lupus erythematosus (SLE) and endocrinopathies. Pheochromocytomas are rare catecholamine secreting enterochromaffin tumors. The patients of pheochromocytoma usually present with spells of headache, sweating and palpitations due to excessive catecholamines. The cerebral manifestation of pheochromocytoma are uncommon. Posterior reversible encephalopathy syndrome (PRES) as a presenting feature of pheochromocytoma is rare. Case Report A 14-year-old previously healthy boy presented in emergency department with acute onset severe holocranial headache, blurring of vision, generalized tonic clonic motor seizure followed by altered sensorium. There was no previous history of nausea, vomiting, diarrhea, flushing, excessive sweating, migraine, autoimmune and connective tissue disorders, drug abuse, toxin exposure, hypertension or diabetes mellitus. He had no similar illness in past and family history was negative. His pulse rate and blood pressure were 130 beats per minute and 234/126 mm Hg, respectively. He was confused but followed simple commands. The pupillary size and light reaction were normal on both sides. Fundus examination showed bilateral papilledema. He was moving all the four limbs equally without asymmetry on painful stimulus. Plantars were bilaterally extensor. Signs of meningeal irritation (neck rigidity and Kernigs signs) were negative. Complete hemogram, serum electrolyte, renal function tests and other biochemistry including thyroid function tests were normal. Serum anti-nuclear antibodies (ANA), anti-double-stranded DNA antibody (anti-dsDNA) and ELISA test for human immunodeficiency virus (HIV) were negative. X-ray chest was normal. Electrocardiography (ECG) showed tachycardia. Magnetic resonance imaging (MRI) of brain showed hyperintense signal changes on T2-weighted and fluid attenuated inversion recovery (FLAIR) images in bilateral occipito-parietal regions. No restriction was seen on diffusion-weighted images (DWI) [Figure 1]. CT angiography of brain vessels was normal. High blood pressure and classical MRI findings were consistent with the diagnosis of PRES. On further evaluation, abdominal ultrasonography (USG) showed right adrenal mass. An abdominal triple phase contrast-enhanced CT scan revealed heterogeneous, contrast enhancing adrenal gland mass lesion measuring 32 x 26 mm suggestive of pheochromocytoma [Figure 2]. The diagnosis was confirmed by 24-hour urinary metanephrine assay. The plasma aldosterone concentration (PAC) and plasma renin activity (PRA) ratio (PAC/PRA) was 10.4. The 24- hour urinary normetanephrine was 31,572 Â µg/24 hour (normal: 63-402 Â µg/24 hour), urinary metanephrine was 1,524 Â µg/24 hour (normal: 32-167 Â µg/24 hour) and plasma noradrenaline level was 18,635 pg/mL (normal: 0-400 pg/mL). Patient was managed intensively with nitroprusside infusion to reduce blood pressure. Injectable phenytoin was administered according to body weight to control seizures. Once patient was stabilized, he was started on oral alpha-blocker prazosin (20 mg/day) followed by beta-blocker propranolol (40 mg/day). After adequate alpha and beta blockade, patient was planned for surgery and resection of adrenal mass was done. Histopathologic examination confirmed the diagnosis of pheochromocytoma without invasion of the adrenal capsule [Figure-3]. The MRI brain showed complete resolution of hyperintense signals (T2-weighted and FLAIR images) and 24-hour urine metanephrines were normal on two-months follow-up. He was asymptomatic at six months and one year follow-up. Discussion Pheochromocytomas are neuroendocrine tumors arising from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia. The classical features like holocranial headache, palpitations, hypertension, hyperhidrosis, hyperglycemia and hypermetabolism are due to excessive catecholamines production in these tumors. The cardiac complications such as arrhythmias, myocardial infarction and sudden deaths are associated with cardiotoxic effects of high blood catecholamines levels. Pheochromocytoma is a rare cause of secondary hypertension and accounts for 0.5% to 2.0% of all causes of hypertension in children. The neurological complications (ischemic or hemorrhagic stroke) are related to increased platelet aggregation, hypertension and vasospasm due to high catecholamines levels. The various potential triggers of pheochromocytoma crisis leading to hemodynamic instability are stress, blood loss, surgery and anesthesia. The diagnosis of pheochromocytoma is confirmed by 24-hour urinary meta nephrine and normetanephrine levels. Clinical features of reversible posterior leukoencephalopathy syndrome (PRES) are acute onset headaches, vision loss, seizures and altered sensorium. It is mostly due to hypertension, however other common causes are chronic renal disease, uremic encephalopathy, ergot alkaloids, steroids, chemotherapy, vasculitis and tumors. Pheochromocytoma is one of the rare cause of PRES due to secondary hypertension. However, to the best of our knowledge, acute, life-threatening initial presentation of pheochromocytoma as PRES has not been described in literature. Magnetic resonance images (MRI) of brain typically shows hyperintense signal changes on T2-weighted and fluid attenuated inversion recovery (FLAIR) images due to vasogenic edema. It mostly involves the cortical and subcortical white matter of the parietooccipital, frontal and temporal regions. Cerebral hemorrhage, cytotoxic edema and contrast enhancement are atypical imaging findings of PRES. The parietooccipital cortex and subcortical w hite matter were affected in our case. Classically, these signal abnormalities are reversible on antihypertensive therapy. The pathophysiology of PRES is still poorly understood, however, various hypothesis have been proposed. Severe hypertension causes deranged autoregulation of sympathetically mediated cerebral arterioles. It leads to increased permeability in the blood-brain barrier and causes vasogenic edema. Sympathetic innervation of the vertebrobasilar system is not as extensive or as complete as that of the anterior circulation. Therefore, PRES predominantly affects parietooccipital cortex and subcortical white matter. Other possible mechanism may be endothelial dysfunction due to circulating toxins or chemotherapy agents. There may be cerebral infarction or hemorrhage due to compromise of the microcirculation by pressure from surrounding vasogenic edema. The exact etiology of the seizure remains unknown, but may result from effects of the pheochromocytoma on reducing seizure threshold via its actions on metabolic or hypertensive parameters. Our patient had high levels of circulating catecholamin es, produced autonomously by tumor. Once vasogenic edema subsided with antihypertensive therapy, all the abnormal MRI findings vanished. Clinical topography (acute onset headache, visual blurring, seizure and altered sensorium), high blood pressure and typical MRI findings suggested diagnosis of PRES. In our patient, other differential diagnosis such as thrombocytopenic thrombotic purpura (TTP), hemolytic uremic syndrome (HUS), encephalitis, systemic lupus erythematosus (SLE), brain mass lesions and drug toxicity were ruled out by appropriate clinical and laboratory investigations. In our patient, hypertension was detected for the first time on hospital admission and before that he never had any symptoms like headache, palpitations, perspiration or diarrhea. Our patient is of clinical interest as pheochromocytoma presented with life-threatening hypertensive encephalopathy. Management of pheochromocytoma requires aggressive approach including fluid resuscitation and antihypertensive therapy (both alpha and beta blockade) followed by surgical resection of tumor. Conclusion Pheochromocytomas are catecholamine secreting enterochromaffin tumors causing paroxysmal hypertension. Our patient is of clinical interest as an acute, life-threatening hypertensive encephalopathy (PRES) as a presenting feature of adrenal pheochromocytoma has not been described in literature so far. Pheochromocytoma should be ruled out in every young patient with acute hypertensive encephalopathy. Figure Legends Figure 1. Magnetic resonance imaging (MRI) of brain showing hyperintense signals on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images in parieto-occipital region. No diffusion restriction is seen. [ T1-weighted axial (A), T2-weighted axial and sagittal (B,C), FLAIR (D), diffusion weighted (DWI) (E) and corresponding apparent diffusion coefficient (ADC) (F) images]. These abnormal signals completely vanished on follow-up MRI after six weeks. Figure 2. Triple phase contrast-enhanced computed tomography (CT) of abdomen showing heterogenous enhancing mass lesion measuring 32 x 26 mm in right adrenal gland. [Axial CT: Arterial phase (A,B), Venous phase (C) and Delayed phase (D)]. Figure 3. Hematoxylin and eosin (HE) stained microphotographs showing large pleomorphic nuclei, abundant basophilic cytoplasm and cell-nesting pattern (zellballen pattern). [HE stain 40x view (A), 100x view (B,C)]. 1

Revisiting the NASL

It is said that we are able to see the correctness, validity and impact of our actions only after the moment we act had passed. This article looks back into the experience of the North American Soccer League. This is an attempt to revisit the rise and demise of the league.A Brief HistoryThe North American Soccer League (NASL) was established as a result of the merger of the United Soccer Association and the National Professional Soccer League. It was the culmination of the first modern attempts to create a truly national soccer league in the United States. The NASL even had franchise teams from Canada. Prior to its establishment, soccer competition in the United States was primarily on the semi-professional and amateur level.The United Soccer Association (USA) was sanctioned by the Federation of International Footbal Association (FIFA) and established itself in twelve major cities across the United States. The National Professional Soccer League (NPSL), on the other hand, was not san ctioned by FIFA. It also did not abide by FIFA player transfer rules and had ten franchise teams. Fan interest for the two leagues was initially high and attendance was not bad for the first year.The merger of the two leagues into the NASL, however, was an act of desperation. Fan interest had quickly faded, television ratings were terrible and the teams of the USA and NPSL had experienced massive financial losses. The disastrous start was such that only five teams of the original seventeen teams that started the NASL survived the first two seasons (Holroyd, 2005).Teams folded but new franchises took their place in the league. The turning points in league history took place in the 1971 to 1975 period. The New York Cosmos joined the NASL in 1971 and won the league in 1972. The entry of the Cosmos established the league’s presence in a major market and brought greater media attention to the league. The following year saw the Philadelphia Atoms joining the league. The Atoms also won the league title in their inaugural season. The team was bannered by Kyle Rote Jr., son of a former star of the National Football League’s (NFL) New York Giants. Rote also won the league’s Rookie of the Year and Most Valuable Player awards. By 1974, the NASL had grown and have fifteen teams competing (Holroyd, 2005).Before the start of the 1975 season, the New York Cosmos got Edson Arantes do Nascimento (more popularly known as Pelà ©) to play for the team. This move placed the league in the spotlight and significantly increased public interest in soccer in the United States. Pelà ©, of course, is considered possibly the greatest soccer player ever. He gained the moniker â€Å"King of Football† or simply King Pelà © during his career. The attention snowballed through 1977 and culminated with 77,691 spectators for the 1977 playoff game between the Cosmos and the Ft. Lauderdale Strikers at Giants stadium. Pelà © retired from the sport, and the league, in 1 977 after an exhibition game between the two professional football clubs he had played for — New York Cosmos and Santos FC from Brazil (Holroyd, 2005).The NASL followed its success by undergoing a major expansion, adding six teams in 1978. League competition was organized into a NFL-style divisional lineup with a 30-game season. Teams also went into an unprecedented spending spree to sign up international players in an attempt to match the Cosmos’ success or at least remain competitive in the league (Litterer & Holroyd, 2003).Seven years after Pelà ©'s farewell, the league started 1984 (its 17th season) smaller than it had been in over a decade with only nine teams. Also, four of these teams were on shaky financial ground. The league eventually closed down after the Chicago-Toronto best-of-three finals series in Toronto, Canada (Holroyd, 2006).Addressing the MistakesPolicy 1: Develop Homegrown Talents to Generate AwarenessWhat could have the league done to sustain its elf? What could have been avoided? Among the team sports played in the United States, soccer had not yet reached the level of fan interest and media attention that is given to professional baseball, basketball and football. As a result, the franchise teams that made the NASL were in shaky financial standing except for that brief golden period that spanned 1971 to 1977.In this regard, the first issue to address is generating awareness and increasing the interest of fans. Soccer was not, and is not yet, dear to the hearts of American sports spectators. It has not reached the manic-obsessive levels of fan interest outside of the United States. According to Paglia (2007), the nature of the game runs counter to the fundamental element required of a US spectator sport.A hallmark of the most popular outdoor spectator sports in America, for example baseball and football, is that fans try to anticipate the next plays, possible game outcomes and share these speculations with one another. Socc er, by its nature, is an extemporaneous sports with many improvisational plays. It is so fluid that speculating on outcomes is close to impossible.Fan interest could, however, be impacted by creating awareness. In 1975, fan interest and awareness of soccer went up when Pelà © played in the league. At the time of his entry, Pelà © was famous even in the United States. This tack is being duplicated again by the Major League Soccer when the LA Galaxy contracted David Beckham to play for the team. Paglia (2007) sees that Beckham will create an impact but this alone will not â€Å"convert the curious to fans†. Furthermore, the impact may not be at the same level as when Pelà © played in the NASL. The two players are not the same whether in stature or even in playing style.Giorgio Chinaglia, the former Italian international who played alongside Pelà ©, does not think Beckham alone can promote the sport and league. Other big European names have to follow him to produce sustained success. Chinaglia even estimates that around 50 international soccer stars may be needed. (Adderley, 2007). But the NASL have already gone through this route before and it was with disastrous results.Some, even in 1978, had criticized the move to bring in more foreign players as shortsighted. They said that it was more important to develop American talent. But those voices were lonely cries in the wilderness.A lesson could be learned from 1973 when the Philadelphia Atoms won the NASL championship. Fan interest was sparked by the emergence of Kyle Rote Jr. He was considered the first â€Å"All-American† soccer star. The American spectator wants a star to relate to and follow. And it would be better if the star was homegrown. The NASL should link up with amateur, collegiate and semi-professional soccer leagues. It would even be better if it had its own developmental league.Policy 2: Cooperation and Fiscal DisciplineLooking back into the 1978 NASL spending spree, despite the i ncreased audience base and interest, more money was being spent than what was being earned. Operating a sports league is an awkward venture. The owners have to co-operate and make many business decisions collegially despite the fierce team competition. Taking a page from the National Football League’s business model, the NASL could have developed an internal incentive system that would stabilize team expenditures and â€Å"equalize† revenue generation.Two sets of incentives used by the NFL are (1) the teams' owners share roughly 70% of their revenues with each other; and (2) they stick to a strict salary cap that limits the amount each team can spend on players' salaries. As a result of these incentives, all thirty-two teams in the NFL have an even chance of being financially viable and athletically competitive despite having different market base (The Economist, 2006). Admittedly, the NASL attempted to institute a salary cap in 1984 but by then it was to little to lat e.Policy 3: Align Game to International StandardsSoccer’s World Cup is the biggest sporting event in world. The third policy that NASL could have done was to align its tournament format to that of the World Cup and international competition.By establishing a tournament format aligned to international (or globally accepted/recognized) format helps the league in two ways. One is that the American audience is educated on the mechanics of the game, and consequently increases the level of appreciation for the sport. Another is that the level of play of American players also gets aligned to that of foreign national teams.European football leagues hold inter-league competitions to determine the â€Å"continental† champion. For example, the Italian League champion plays against the French League champion. The NASL had already previously done this through friendly tours. The Ft. Lauderdale Strikers made a tour of England in February 1978. Foreign teams also toured the United St ates.Becoming competitive against foreign soccer teams could have helped in sustaining fan interest and support. Case in point is the impact of US National Team’s achievement in the 1994 World Cup. The team surpassed all expectations and reached the quarterfinals. This accomplishment sparked greater interest in the sport.ReferencesAdderley, Nigel. 2007. Beckham’s US Test. Accessed October 25, 2007 from http://news.bbc.co.uk/sport1/hi/football/6294762.stmHolroyd, Steve. 2005. The Year in American Soccer – 1984. Accessed October 25, 2007 from http://www.sover.net/~spectrum/year/1984.html2006. The Year in American Soccer – 1968. Accessed October 25, 2007 from http://www.sover.net/~spectrum/year/1968.htmlLitterer, David and Steve Holroyd. 2003. The Year in American Soccer – 1978. Accessed October 25, 2007 from http://www.sover.net/~spectrum/year/1978.htmlPaglia, Jim. 2007. A Naysayer View of Pro Soccer. Accessed October 25, 2007 from http://www.soccera merica.com/index.cfm?fuseaction=Articles.showArticleHomePage;art_aid=22406The Economist. 2006. In a league of its own. [Electronic version] Accessed October 25, 2007 from http://www.economist.com/business/displaystory.cfm?story_id=6859210

The Importance of Science to Civilization

The importance of science to civilization It is said that modern civilization is a scientific civilization. For an individual, science has rendered us great comfort nowadays. We no longer have to travel on foot; instead, we can just sit comfortably without any fatigue in cars, air-conditioned trains, buses, etc. We are free from hard labor which our uncivilized forefathers suffered from. Neither do we follow barbarous practice of having slaves, since science has provided us with slaves of a new kind, which undertake most of the work, that is, machine.On top of that, we are also free from diseases and various pains since drugs and operation techniques have been developed dramatically. Maslow's theory of hierarchy of needs suggests that the physiological needs must be met before the individual will strongly desire (or focus motivation upon) the secondary or higher level needs, which means the human that lacks food, love, esteem, or safety would consider the greatest of his/her needs to be food. And as mentioned above, it is science that satisfies our basic demands; thus, we have opportunities to pursue something higher, such as art and literature.Science also promote the development of spiritual civilization. With the advent of the internet, and extraordinary growth in information and communication technologies in recent years, more information than ever before is made freely available and easily shared. Almost all the masterpieces of artist, musicians, writers, philosophers, scientists, etc can be enjoyed at fingertips. We are not only consumers, but also producers. When Gangnam Style was released, tens of thousands people made their own versions and uploaded on the internet, which could be appreciated by millions of netizens.For a nation, science is of critical significance for the progress of its civilization. China's long history has seen some extremely important inventions emerge, most noticeably gunpowder, paper making, printing and the compass, which, in t he words of Roger Bacon, changed the whole appearance and status of things in the world and also made China among the four great ancient civilizations. However, in the nineteenth century, Europe entered into world history with immense developments in technology, which was later identified as the Industrial Revolution.From then on, China lost its advantages over the the rest of world in various aspects, and it was invaded by the great powers into a series of Unequal Treaties. Chinese civilization were, during that period, regarded inferior and the Chinese nation was referred to as â€Å"Sick Man of East Asia†. Even the Chinese people abandoned their own cultural identity and turned to the Western civilization. Therefore, science is the foundation as well as the major force behind the progress of civilization. 0101102027

Competition, Stability, And Civil Society - 958 Words

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