Neuroimaging Findings in Late Infantile GM1

Neuroimaging Findings in Late Infantile GM1 Gangliosidosis  Summary: Late infantile GM1 gangliosidosis is an extremely rare metabolic disorder with clinical features of seizure and progressive motor and mental retardation without facial dysmorphism or visceral organomegaly. We report the CT and MR imaging findings in one infant, which included abnormalities of the cerebral cortex, white matter, and deep nuclei. GM1 gangliosidosis is a rare lysosomal storage dis- ease characterized by a deficiency in the activity of lysosomal galactosidase, which results in increased accumulation of GM1 ganglioside and asialo-GA1 in the brain and of oligosaccharide in the visceral organs (1, 2). Patients with infantile GM1 gangliosidosis usu- ally have dysmorphic facial features, motor and men- tal retardation, seizures, and hepatosplenomegaly (2). The late infantile form of this disorder is extremely rare and has no clinical features of facial dysmor- phism or visceral organomegaly (1). Case Report An 11-month-old girl, the product of an uncomplicated full-term pregnancy, presented with complex febrile convulsion during an episode of upper respiratory infection. Physical ex- amination at first admission showed a hypotonic infant with macrocephaly (>97 percentile), normal facial features, and psychomotor retardation. Hyperactive reflexes and augmented startle response to noise were present. The abdomen was soft without hepatosplenomegaly. Examination of the eyes revealed a normal clear lens, dilatation of the pupil without light reflex in the right eye, and mild dilatation of the pupil with sluggish light reflex in the left eye. Fundoscopy showed no cherry-red  spots at the maculae. Deficient galactosidase activity and normal hexosaminidase, and glucosidase activity on lysosomal enzyme assay in cultured skin fibroblasts were documented at the patient’s second admission 3 months later for clonic-tonic seizures during another episode of acute upper respiratory infection. Radiologic investigation included chest radiography (also covering the abdomen), CT, and MR imaging, all of which were done on initial admission and again at follow-up. A bone survey was not performed owing to unremarkable somatic manifesta- tions. A CT scan of the brain at age 11 months showed in- creased attenuation of the bilateral thalami (Fig 1A). MR examinations at 11 and 14 months of age showed hyperintensity of the thalami on T1-weighted images (Fig 1B) whereas, on T2-weighted images, the signal intensity of the thalami was decreased (Fig 1C). The white matter myelination was mark- edly delayed, with only the splenium of the corpus callosum being myelinated and no interval improvement in myelination noted at the second MR study (Fig 1D). Discussion GM1 gangliosidosis is a rare inborn error of metab- olism caused by a deficiency of galactosidase activity resulting in failure of catalyzing cleavage of termi- nal link galactose from substrates, such as GM1 ganglioside, asialo-GM1, lactosylceramide, galactose- containing oligosaccharides, and mucopolysacchar- ides. The abnormally accumulated substances in the lysosomes of the affected cells in the CNS are mainly GM1 ganglioside and its asialo derivative GA1 as well as other minor glycolipids and glycopeptides. Visceral storage with oligosaccharides is variable and may cause organgomegaly. Three types of GM1 gangliosidosis, classified by age of onset, have been reported (1, 3, 4). Infantile GM1 gangliosidosis (type 1) is the most common and se- vere form, with clinical features of hypotonia, failure to thrive in the neonatal period, and clonic-tonic seizure activity. Coarse facial features, frontal boss- ing, dysostosis multiplex, hepatosplenomegaly, and hazy cornea may be present or become apparent in the first year of life (2). The late infantile or juvenile form (type 2) begins with progressive mental and motor retardation between 1 and 5 years of age. Seizures are common, and spastic tetraplegia devel- ops, with cerebellar and extrapyramidal signs. Decer- ebrate rigidity follows, and death occurs between 3 and 10 years of age, usually precipitated by recurrent bronchopneumonia. Dysmorphic facial features, hep- atosplenomegaly, corneal changes, and bony abnor- malities are usually lacking, and, when present, are due to a mild degree or absence of oligosaccharide acc umulation. Cerebral GM1 ganglioside storage is also less severe in type 2 than in type 1 disease (1, 2). A, Cranial CT scan at age 11 months shows increased attenuation of the thala- mus and decreased attenuation of the basal ganglia, which were isointense with adjacent white matter. B, T1-weighted axial MR image (600/ 20/2 [TR/TE/excitations]) obtained at the same time shows increased signal inten- sity of the thalamus. The white matter my- elination was markedly delayed. Note that only the splenium of the corpus callosum is myelinated. C, Corresponding axial T2-weighted MR image (2800/90/1) reveals hypodensity of the thalamus and normal signal intensity of the basal ganglia. The cerebrum showed almost no myelination of the white matter except the splenium of the corpus callo- sum. D, Follow-up T2-weighted MR image at 14 months of age shows persistent delayed myelination of the white matter. Note that no cortical atrophy is seen at this stage. Children or adults with chronic, or type 3, GM1 gan- gliosidoses may have a slowly progressive disorder in which dystonia, dysarthria, ataxia, myoclonus gait dis- orders, and extrapyramidal signs occur (4). Bony changes are minimal. Cherry-red spots at the macu- lae, which are found in about half the patients with type 1 GM1 gangliosidoses, are not seen in patients with type 2 and type 3 disease. The disorder can be diagnosed in several ways, including lysosomal enzyme assay of low galactosidase activity in peripheral leukocytes or cultured skin fibroblasts, detection of abnormal urinary oligosac- charide excretion, and rectal biopsy (2). Prenatal di- agnosis by measurement of enzyme activity in amni- otic fluid and cultivated amniotic fluid cells has also been established (5). Neuropathologic reports on GM1 gangliosidoses have shown diffuse neuronal storage with ballooning of neuronal cytoplasm in the cerebral and cerebellar cortex, basal ganglia, brain stem, spinal cord, and dorsal root ganglion in type 1 and type 2 forms, and a tendency for selective storage in the basal ganglia with neuronal loss and gliosis in type 3 disease (6, 7). The cerebral white matter is gliotic and there is loss of myelin in type 1 but not in types 2 and 3 GM1 gangli- osidoses. Neuroimaging findings in patients with type 1 GM1 gangliosidoses have been reported only in a few cases. In one case, initial thalamic hyperdensity was found on CT scans and hypointense signal of the thalami was seen on T2-weighted MR images at a later stage (3). Persistent delay in white matter myelination on serial MR studies has been described in another case (8). In type 3 GM1 gangliosidoses, the symmetrical abnormal signal intensities were more selectively seen in the caudate nucleus and putamen on T2-weighted MR images (9). To our knowledge, neuroimaging findings in type 2 GM1 gangliosidoses have not been reported previously. The CT and MR findings in our case are identical to those reported in patients with Tay-Sachs disease (GM2 gangliosidoses). This may be understood be- cause the structure of gangliosides GM1 and GM2 differs only in the terminal N-acetylgalactosamine, and they are stored together with cholesterol and phospholipid; the neuronal staining reactions and ul- trastructure are identical (7). The thalamic hyperden- sity seen on CT scans in Tay-Sachs and Krabbe lyso- somal storage disorders has been presumed to be due to calcification. Calcium deposition in the thalamus can also explain the hyperintense T1 signal and hy- pointense T2 signal on MR images, although frank calcium deposition has not been reported pathologi- cally (10). Other investigators have suggested that secondary lipofuscinosis, resulting in deposits of pigmented degradation products, occurs in the thalami and cause this appearance in GM2 gangliosidoses (11, 12). In our case, the CT study showed not only tha- lamic hyperdensity but also hypointensity of the basal ganglia, which were almost isointense with adjacent white matter (Fig 1A). The MR study verified the CT findings of abnormal thalamic attenuation but not the hypointensity of the basal ganglia. MR is the only imaging technique that is capable of demonstrating the arrested myelination of the cerebral white matter in serial examinations (Fig 1C and D). Conclusion Although the neuroimaging findings of GM1 gan- gliosidoses are unique in our case, these findings could also be identified in GM2 gangliosidoses and, to some extent, in late-stage Canavan disease. Patients with late-stage Canavan disease may have dense thal- ami on T1-weighted images, as well as white matter necrosis, resulting in cavitation, and brain stem and cerebellar atrophy, which are not observed in GM1. Other neurometabolic diseases that often manifest with macrocephaly include mucopolysaccharidosis and Alexander disease. These disorders have distinct neuroimaging features and they should be distin- guished from GM1. A definite diagnosis of this rare disorder can only be made by obtaining lysosomal enzyme assay results of deficient galactosidase and normal hexosaminidase. References 1. Gascon GG, Ozand PT, Erwin RE. GM1 gangliosidosis type 2 in two siblings. J Child Neurol 1992;7:S41–S50 2. Suzuke Y, Sakuraba H, Oshima A. Beta-galactosidase deficiency (beta-galactosidosis): GM1 gangliosidosis and Morquio B disease. In: Scriver CR, Beaudet AL, Sly WS, et al, eds. The Metabolic and Molecular Bases of Inherited Disease. New York: McGraw-Hill; 1995:2785–2823 3. Kobayashi 0, Takashima S. Thalamic hyperdensity on CT in infan- tile GM1-gangliosidosis. Brain Dev 1994;16:472– 474 4. Tanaka R, Momoi T, Yoshida A, et al. Type 3 GM1 gangliosidosis: clinical and neuroradiological findings in an 11-year-old girl. J Neurol 1995;242:299 –303 5. Tasso MJ, Martinez-Gutierrez A, Carrascosa C, et al. GM1-gangli- osidosis presenting as nonimmune hydrops fetalis: a case report. J Perinatal Med 1996;24:445– 449 6. Bieber FR, Mortimer G, Kolodny EH, et al. Pathologic findings in fetal GM1 gangliosidosis. Arch Neurol 1986;43:736 –738 7. Lake B. Lysosomal and peroxisomal disorders. In: Graham DI, Lantos PL, eds. Greenfield’s Neuropathology. 6th ed. London: Ar- nold; 1997 8. Kaye EM, Alroy J, Raghavan SS, et al. Dysmyelinogenesis in an animal model of GM1 gangliosidosis. Pediatr Neurol 1992;8:255–261 9. Uyama E, Terasaki T, Watanabe S, et al. Type 3 GM1 gangliosido- sis: characteristic MRI findings correlated with dystonia. Acta Neurol Scand 1992;86:609 – 615 10. Brismar J, Brismar G, Coates R, et al. Increased density of the thalamus on CT scans in patients with GM2 gangliosidoses. AJNR Am J Neuroradiol 1990;11:125–130 11. Lowden JA, Callahan JW, Gravel RA, et al. Type 2 GM gangli- osidosis with neuronal ceroid lipofusinosis. Neurology  1981;31:719 –724 12. Stalker HP, Jan BK. Thalamic hyperdensity: a previously unre- ported sign of Sandhoff disease. AJNR Am J Neuroradiol 1989;10: S82

Fool Chapter 14

FOURTEEN ON TENDER HORNS â€Å"I shagged a ghost,† said Drool, wet, naked, and forlorn, sitting in the laundry cauldron under Castle Gloucester. â€Å"There's always a bloody ghost,† said the laundress, who was scrubbing the lout's clothes, which had been most befouled in the moat. It had taken four of Lear's men, along with me, to pull the great git from the stinking soup. â€Å"No excuse for it, really,† said I. â€Å"You've the lake on three sides of the castle, you could open the moat to the lake and the offal and stink would be carried away with the current. I'll wager that one day they find that stagnant water leads to disease. Breeds hostile water sprites, I'll wager.† â€Å"Blimey, you're long-winded for such a wee fellow,† said the laundress. â€Å"Gifted,† I explained, gesturing grandly with Jones. I, too, was naked, but for my hat and puppet stick, my own apparel having taken a glazing of oozy moat mess during the rescue as well. â€Å"Sound the alarm!† Kent came storming down the steps into the laundry, sword unsheathed and followed closely by the two young squires he'd trounced not an hour before. â€Å"Bolt the door! To arms, fool!† â€Å"Hello,† said I. â€Å"You're naked,† said Kent, once again feeling the need to voice the obvious. â€Å"Aye,† said I. â€Å"Find the fool's kit, lads, and get him into it. Wolves are loosed on the fold and we must defend.† â€Å"Stop!† said I. The squires stopped thrashing wildly around the laundry and stood at attention. â€Å"Excellent. Now, Caius, what are you on about?† â€Å"I shagged a ghost,† said Drool to the young squires. They pretended they couldn't hear him. Kent shuffled forward, held back some by the alabaster grandeur of my nakedness. â€Å"Edmund was found with a dagger through his ear, pinned to a high-backed chair.† â€Å"Bloody careless eater he is, then.† â€Å"‘Twas you who put him there, Pocket. And you know it.† â€Å"Moi? Look at me? I am small, weak, and common, I could never – â€Å" â€Å"He's called for your head. He hunts the castle for you even now,† said Kent. â€Å"I swear I saw steam coming out his nostrils.† â€Å"Not going to spoil the Yule celebration, is he?† â€Å"Yule! Yule! Yule!† chanted Drool. â€Å"Pocket, can we go see Phyllis? Can we?† â€Å"Aye, lad, if there's a pawnbroker in Gloucester, I'll take you soon as your kit is dry.† Kent raised a startled porcupine of an eyebrow. â€Å"What is he on about?† â€Å"Every Yule I take Drool down to Phyllis Stein's Pawnshop in London and let him sing ‘Happy Birthday' to Jesus, then blow the candles out on the menorah.† â€Å"But the Yule's a pagan holiday,† said one of the squires. â€Å"Shut up, you twat. Do you want to ruin the twit's fun? Why are you here, anyway? Aren't you Edmund's men? Shouldn't you be trying to put my head on a pike or something?† â€Å"They've changed allegiance to me,† said Kent. â€Å"After the thrashing I gave them.† â€Å"Aye,† said squire one. â€Å"We've more to learn from this good knight.† â€Å"Aye,† said squire two. â€Å"And we were Edgar's men, anyway. Lord Edmund is a scoundrel, if you don't mind me saying, sir.† â€Å"And, dear Caius,† said I. â€Å"Do they know that you are a penniless commoner and can't really maintain a fighting force as if you were, say – oh, I don't know – the Earl of Kent?† â€Å"Excellent point, Pocket,† said Kent. â€Å"Good sirs, I must release you from your service.† â€Å"So we won't be paid, then?† â€Å"My regrets, no.† â€Å"Oh, then we'll take our leave.† â€Å"Fare thee well, keep your guard up, lads,† said Kent. â€Å"Fighting's done with the whole body, not only the sword.† The two squires left the laundry with a bow. â€Å"Will they tell Edmund where we're hiding?† I asked. â€Å"I think not, but you better get your kit on just the same.† â€Å"Laundress, how progresses my motley?† â€Å"Steamin' by the fire, sir. Dry enough to wear indoors, I reckon. Did I hear it right that you put a dagger through Lord Edmund's ear?† â€Å"What, a mere fool? No, silly girl. I'm harmless. A jab from the wit, a poke to the pride are the only injuries a fool inflicts.† â€Å"Shame,† said the laundress. â€Å"He deserves that and worse for how he treats your dim friend – † She looked away. † – and others.† â€Å"Why didn't you just kill the scoundrel outright, Pocket?† asked Kent, kicking subtlety senseless and rolling it up in a rug. â€Å"Well, just shout it out, will you, you great lummox.† â€Å"Aye, like you'd never do such a thing, ‘Top of the morning; grim weather we're having; I've started a bloody war!'† â€Å"Edmund has his own war.† â€Å"See, you did it again.† â€Å"I was coming to tell you when I found the girl ghost having a go at Drool. Then the lout leapt out the window and the rescue was on. The ghost implied that the bastard might be rescued by France. Maybe he's allied with bloody King Jeff to invade.† â€Å"Ghosts are notoriously unreliable,† said Kent. â€Å"Did you ever consider that you might be mad and hallucinating the whole thing? Drool, did you see this ghost?† â€Å"Aye, I had a half a laugh wif her before I got frightened,† said Drool, sadly, contemplating his tackle through the steamy water. â€Å"I fink I gots deaf on me willie.† â€Å"Laundress, help the lad wash the death off his willie, would you?† â€Å"Not bloody likely,† said she. I held the tip of my coxcomb to stay any jingling and bowed my head to show my sincerity. â€Å"Really, love, ask yourself, What would Jesus do?† â€Å"If he had smashing knockers,† added Drool. â€Å"Don't help.† â€Å"Sor-ry.† â€Å"War? Murder? Treachery?† reminded Kent. â€Å"Our plan?† â€Å"Aye, right,† said I. â€Å"If Edmund has his own war it will completely bollocks up our plans for civil war between Albany and Cornwall.† â€Å"All well and good, but you didn't answer my question. Why didn't you just slay the bastard?† â€Å"He moved.† â€Å"So you meant to kill him?† â€Å"Well, I hadn't thought it through completely, but when I sent his dagger at his eye socket I believed that there might be a fatal outcome. And I must say, although I didn't stay to revel in the moment, it was very satisfying. Lear says that killing takes the place of bonking in the ancient. You've killed a multitude of chaps, Kent. Do you find that to be the case?† â€Å"No, that's a disgusting thought.† â€Å"And yet, with Lear lies your loyalty.† â€Å"I'm beginning to wonder,† said Kent, sitting down now on an overturned wooden tub. â€Å"Who do I serve? Why am I here?† â€Å"You are here, because, in the expanding ethical ambiguity of our situation, you are steadfast in your righteousness. It is to you, my banished friend, that we all turn – a light amid the dark dealings of family and politics. You are the moral backbone on which the rest of us hang our bloody bits. Without you we are merely wiggly masses of desire writhing in our own devious bile.† â€Å"Really?† asked the old knight. â€Å"Aye,† said I. â€Å"I'm not sure I want to keep company with you lot, then.† â€Å"Not like anyone else will have you, is it? I need to see Regan before my bastard ear piercing poisons our cause. Will you take her a message, Kent – er, Caius?† â€Å"Will you put on your trousers, or at least your codpiece?† â€Å"Oh, I suppose. That had always been part of the plan.† â€Å"Then I will bear your message to the duchess.† â€Å"Tell her – no, ask her – if she still holds the candle she promised for Pocket. Then ask her if I may meet her somewhere private.† â€Å"I'm off, then. But try to manage not to get murdered while I'm gone, fool.† â€Å"Kitten!† said I. â€Å"You poxy little vermin,† said Regan, in glorious red. â€Å"What do you want?† Kent had led me to a chamber far in the bowels of the castle. I couldn't believe that Gloucester would house royal guests in an abandoned dungeon. Regan must have somehow found her own way here. She had an affinity for such places. â€Å"You received the letter from Goneril, then?† I asked. â€Å"Yes. What is it to you, fool?† â€Å"The lady confided in me,† said I, bouncing my eyebrows and displaying a charming grin. â€Å"What is your thought?† â€Å"Why would I want to dismiss father's knights, let alone take them into my service? We have a small army at Cornwall.† â€Å"Well, you're not at Cornwall, are you, love?† â€Å"What are you saying, fool?† â€Å"I'm saying that your sister bade you come to Gloucester to intercept Lear and his retinue, and thus stop him from going to Cornwall.† â€Å"And my lord and I came with great haste.† â€Å"And with a very small force, correct?† â€Å"Yes, the message said it was urgent. We needed to move quickly.† â€Å"So, when Goneril and Albany arrive, you will be away from your castle and nearly defenseless.† â€Å"She wouldn't dare.† â€Å"Let me ask you, lady, where do you think the Earl of Gloucester's allegiance lies?† â€Å"He is our ally. He has opened his castle to us.† â€Å"Gloucester, who was nearly usurped by his eldest son – you think he sides with you?† â€Å"Well, with Father, then, which is the same thing.† â€Å"Unless Lear is aligned with Goneril against you.† â€Å"But she relieved him of his knights. He ranted about it for an hour after his arrival, called Goneril every foul name under the sun, and praised me for my sweetness and loyalty, even overlooking my throwing his messenger into the stocks.† I said nothing. I removed my coxcomb, scratched my head, and sat on some dusty instrument of torture to observe the lady by torchlight and watch her eyes as the rust ground off the twisted gears of her mind. She was simply lovely. I thought about what the anchoress had said about a wise man only expecting so much perfection in something as its nature allows. I thought that I might, indeed, be witnessing the perfect machine. Her eyes went wide when the realization hit. â€Å"That bitch!† â€Å"Aye,† said I. â€Å"They'll have it all, she and Father?† â€Å"Aye,† said I. I could tell her anger didn't arise from the betrayal, but from not having thought of it first. â€Å"You need an ally, lady, and one with more influence than this humble fool can provide. Tell me, what do you think of Edmund the bastard?† â€Å"He's fit enough, I suppose.† She chewed a fingernail and concentrated. â€Å"I'd shag him if my lord wouldn't murder him – or come to think of it, maybe because he would.† â€Å"Perfect!† said I. Oh Regan, patron saint of Priapus,[38] the most slippery of the sisters: in disposition preciously oily, in discourse, deliciously dry. My venomous virago, my sensuous charmer of serpents – thou art truly perfection. Did I love her? Of course. For even though I have been accused of being an egregious horn-beast, my horns are tender, like the snail's – and never have I hoisted the horns of lust without I've taken a prod from Cupid's barb as well. I have loved them all, with all my heart, and have learned many of their names. Regan. Perfect. Regan. Oh yes, I loved her. She was a beauty to be sure – there was none in the kingdom more fair; a face that could inspire poetry and a body that inspired lust, longing, larceny, treachery, perhaps even war. (I am not without hope.) Men had murdered each other in competition for her favors – it was a hobby with her husband, Cornwall. And to her credit, while she could smile as a bloke bled to death with her name on his lips, she was not tight-fisted with her charms. It only added to the tension around her that someone was going to be shagged silly in the near future, and how much more thrilling if his life hung by a thread as he did the deed. In fact, the promise of violent death might be to the princess Regan like the nectar of Aphrodite herself, now that I think of it. Why else would she have called for my death all those years ago, when I had so diligently served her, after Goneril had left the White Tower to wed Albany. It had begun, it seems, with a bit of jealousy. â€Å"Pocket,† said Regan. She was perhaps eighteen or nineteen at the time, but unlike Goneril, had been exploring her womanly powers for years on various lads about the castle. â€Å"I find it offensive that you gave personal counsel to my sister, yet when I call you to my chambers I get nothing but tumbling and singing.† â€Å"Aye, but a song and a tumble seem all that's needed to lift the lady's spirits, if I may say so.† â€Å"You may not. Am I not fair?† â€Å"Extremely so, lady. Shall I compose a rhyme to your beauty? A ravishing tart from Nantucket – â€Å" â€Å"Am I not as fair as Goneril?† â€Å"Next to you, she is less than invisible, just a shimmering envious vacuum, is she.† â€Å"But do you, Pocket, find me attractive – in a carnal way – the way you did my sister? Do you want me?† â€Å"Ah, of course, lady, from the morning I wake, I have but one thought, one vision: of your deliciousness, under this humble and unworthy fool, writhing naked and making monkey noises.† â€Å"Really, that's all you think about?† â€Å"Aye, and occasionally breakfast, but it's only seconds before I'm back to Regan, writhing, and monkey noises. Wouldn't you like to have a monkey? We should have one around the castle, don't you think?† â€Å"So all you think of is this?† And with that, she shrugged off her gown, red as always, and there she stood, raven-haired and violet-eyed, snowy fair and finely fit, as if carved by the gods from a solid block of desire. She stepped out of the pool of bloodred velvet and said, â€Å"Drop your puppet stick, fool, and come here.† And I, ever the obedient fool, did. And oh it led to many months of clandestine monkey noises: howling, grunting, screeching, yipping, squishing, slapping, laughing, and no little bit of barking. (But there was no flinging of poo, as monkeys are wont to do. Only the most decent, forthright monkey sounds as are made from proper bonking.) I put my heart into it, too; but the romance was soon crushed beneath her cruel and delicate heel. I suppose I shall never learn. It seems a fool is not so often taken as a medicine for melancholy, as for ennui, incurable and recurring among the privileged. â€Å"You've been spending a lot of time with Cordelia of late,† said Regan, basking glorious in the gentle glow of the afterbonk (your narrator in a sweaty puddle on the bedside floor, having been summarily ejected after rendering noble service). â€Å"I am jealous.† â€Å"She's a little girl,† said I. â€Å"But when she has you, I cannot. She's my junior. It's not acceptable.† â€Å"But, lady, it's my duty to keep the little princess smiling, your father has commanded it. Besides, if I am otherwise engaged you can have that sturdy fellow you fancy from the stable, or that young yeoman with the pointy beard, or that Spanish duke or whatever he is that's been about the castle for a month. Does that bloke speak a word of English? I think he may be lost.† â€Å"They are not the same.† I felt my heart warm at her words. Could it be real affection? â€Å"Well, yes, what we share is – â€Å" â€Å"They rut like goats – there's no art to it, and I weary of shouting instructions to them, especially the Spaniard – I don't think he speaks a word of English.† â€Å"I'm sorry, milady,† said I. â€Å"But that said, I must away.† I stood and gathered my jerkin from under the wardrobe, my leggings from the hearth, my codpiece from the chandelier. â€Å"I've promised to teach Cordelia about griffins and elves over tea with her dolls.† â€Å"You'll not,† said Regan. â€Å"I must,† said I. â€Å"I want you to stay.† â€Å"Alas, parting is such sweet sorrow,† said I. And I kissed the downy dimple at the small of her back. â€Å"Guard!† called Regan. â€Å"Pardon?† I inquired. â€Å"Guard!† The door to her solar opened and an alarmed yeoman looked in. â€Å"Seize this scoundrel. He hath ravaged your princess.† She had conjured tears, in that short span of time. A bit of a wonder, she was. â€Å"Fuckstockings,† said I, as two stout yeomen took me by the arms and dragged me down to the great hall in Regan's wake, her dressing gown open and flowing out behind her as she wailed. It seemed a familiar motif, yet I did not feel the confidence that comes with rehearsal. Perhaps it was that Lear was actually holding court before the people when we entered the great hall. A line of peasants, merchants, and minor noblemen waited as the king heard their cases and made judgments. Still in his Christian phase, he had been reading about the wisdom of Solomon, and had been experimenting with the rule of law, thinking it quaint. â€Å"Father, I insist you hang this fool immediately!† Lear was taken aback, not only by the shrillness of his daughter's demand, but by the fact that she stood frontally bare to all the petitioners and made no effort to close her red gown. (Tales would be told of that day, of how many a plaintiff, having seen the snowy-skinned princess in all her glory, did hold his grievance pitiful, indeed, his life worthless, and went home to beat his wife or drown himself in the mill pond.) â€Å"Father, your fool hath violated me.† â€Å"That's a fluttering bottle of bat wank, sire,† said I. â€Å"Begging your pardon.† â€Å"You speak rashly, daughter, and you appear frothing-dog mad. Calm yourself and state your grievance. How hath my fool offended?† â€Å"He hath shagged me roughly, against my will, and finished too soon.† â€Å"By force? Pocket? He isn't eight stone on a feast day – he couldn't shag a cat by force.† â€Å"That's not true, sire,† said I. â€Å"If the cat is distracted with a trout, then – well, uh, nevermind – â€Å" â€Å"He violated my virtue and spoiled my virginity,† said Regan. â€Å"I insist you hang him – hang him twice, the second time before he's finished choking from the first – that'll be fitting justice.† I said: â€Å"What has put vengeance in your blood, princess? I was just going to tea with Cordelia.† Since the little one wasn't present, I hoped invoking her name might awaken the king to my cause, but it only seemed to incense Regan. â€Å"Forced me down and used me like a common tart,† said Regan, adding rather more pantomime than the petitioners in the hall could bear. Several began to beat their fists to their heads, others grabbed at their groins and sank to their knees. â€Å"No!† said I. â€Å"I've had many a wench by stealth, a few by guile, a number by charm, a brace by mistake, the odd harlot for coin, and, when all else has failed, I've made do by begging, but by God's blood, none by force!† â€Å"Enough!† said Lear. â€Å"I'll hear no more. Regan, close your robe. As I have decreed, we are a kingdom of laws. There shall be a trial, and if the rascal is found guilty, then I'll see him hanged twice myself. Make way for a trial.† â€Å"Now?† asked the scribe. â€Å"Yes, now,† said Lear. â€Å"What do we need? A couple of chaps to do the prosecuting and defending, grab a few of those peasants for witnesses, and with due process, habeas corpus, fair weather and whatnot, we'll have the fool dangling black-tongued before tea. Will that suit you, daughter?† Regan closed her robe and turned away coyly. â€Å"I suppose.† â€Å"And you, fool?† Lear winked at me, none too subtly. â€Å"Aye, majesty. A jury, perhaps, chosen from that same group as the witnesses.† Well, one has to make an effort. From their reaction I would be acquitted, on a â€Å"who could blame† him basis: justifiable shaggicide, they'd call it. But no. â€Å"No,† said the king. â€Å"Bailiff read the charges.† The bailiff obviously hadn't written up charges, so he unrolled a scroll on which was written something entirely unconnected to my case, and faked it: â€Å"The Crown states that on this day, October fourteenth, year of Our Lord, one thousand, two hundred, and eighty-eight, the fool known as Pocket, did with forethought and malice, shag the virgin princess Regan.† There was cheering from the gallery, a little scoffing from the court. â€Å"There was no malice,† said I. â€Å"Without malice, then,† said the bailiff. At this point, the magistrate, who normally functioned as a castle steward, whispered to the bailiff, who normally was the chamberlain. â€Å"The magistrate wishes to know how was that?† â€Å"‘Twas sweet, yet nasty, your honor.† â€Å"Note that the accused hath stated that it was [sweet and nasty], thereby admitting his guilt.† More cheering. â€Å"Wait, I wasn't ready.† â€Å"Smell him,† said Regan. â€Å"He reeks of sex, like fish and mushroom and sweat, doesn't he?† One of the peasant witnesses ran forth and sniffed my bits mercilessly, then looked to the king, nodding. â€Å"Aye, your honor,† said I. â€Å"I'm sure I have an odor about me. I must confess, I was sans trou today in the kitchen, while awaiting my laundry, and Bubble had left a casserole out on the floor to cool, and it did trip me and I fell prick-deep in gravy and goo – but I was on my way to chapel at the time.† â€Å"You put your dick in my lunch?† said Lear. Then to the bailiff, â€Å"The fool put his dick in my lunch?† â€Å"No, in your beloved daughter,† said Regan. â€Å"Quiet, girl!† barked the king. â€Å"Captain Curan, send a guard to watch the bread and cheese before the fool has his way with it.† It went on like that, with things looking rather grim for me as the evidence mounted against me, peasants taking the opportunity to describe the most lecherous acts they could imagine a wicked fool might perpetrate on an unsuspecting princess. I thought testimony of the sturdy stable boy particularly damning at first, but eventually it led to my acquittal. â€Å"Read that back, so the king may hear the true heinous nature of the crime,† said my prosecutor, who I believe butchered cattle for the castle as his normal vocation. The scribe read the stable boy's words: â€Å"Yes, yes, yes, ride me, you crashing tree-cocked stallion.† â€Å"That's not what she said,† said I. â€Å"Yes, it is. It's what she always says,† said the scribe. â€Å"Aye,† said the steward. â€Å"Aye, it is,† said the priest. â€Å"S,† said the Spaniard. â€Å"Well, she never says that to me,† said I. â€Å"Oh,† said the stable boy. â€Å"Then it's ‘Prance, you twig-dicked little pony,' is it?† â€Å"Possibly,† said I. â€Å"She never says that to me,† said the yeoman with the pointy beard. Then there was a moment of silence, while all who had spoken looked around at one another, then furiously avoided eye contact and found spots on the floor of great interest. â€Å"Well,† said Regan, chewing a fingernail as she spoke, â€Å"there is a chance that, uh, I was having a dream.† â€Å"Then the fool did not take your virtue?† asked Lear. â€Å"Sorry,† said Regan sheepishly. â€Å"It was but a dream. No more wine at lunch for me.† â€Å"Release the fool!† said Lear. The crowd booed. I walked out of the hall side by side with Regan. â€Å"He might have hung me,† I whispered. â€Å"I'd have shed a tear,† said she with a smile. â€Å"Really.† â€Å"Woe to you, lady, should you leave that rosebud asterisk of a bum-hole unguarded on our next meeting. When a fool's surprise comes unbuttered, a Pocket's pleasure will a princess punish.† â€Å"Oooo, do tease, fool, shall I put a candle in it so you can find your way.† â€Å"Harpy!† â€Å"Rascal!† â€Å"Pocket, where have you been?† said Cordelia, who was coming down the corridor. â€Å"Your tea has gone cold.† â€Å"Defending big sister's honor, sweetness,† said I. â€Å"Oh bollocks,† said Regan. â€Å"Pocket dresses the fool, but he is ever our hero, isn't he, Regan?† said Cordelia. â€Å"I think I'm going to be ill,† said the elder princess. â€Å"So, love,† said I, rising from my perch on the torture machine and reaching into my jerkin. â€Å"I'm pleased you feel that way about Lord Edmund, for he has sent me with this letter.† I handed her the letter. The seal was dodgy, but she wasn't looking at the stationery. â€Å"He's smitten with you, Regan. In fact, so smitten he tried to cut off his own ear to deliver with this missive, to show you the depth of his affection.† â€Å"Really? His ear.† â€Å"Say nothing at the Yule feast, tonight, lady, but you'll see the bandage. Mark it as a tribute of his love.† â€Å"You saw him cut his ear?† â€Å"Yes, and stopped him before the deed was done.† â€Å"Was it painful, do you think?† â€Å"Oh yes, lady. He has already suffered more than have others in months of knowing you.† â€Å"That's so sweet. Do you know what the letter says?† â€Å"I was sworn not to look upon pain of death, but come close – â€Å" She leaned close to me and I squeezed the witch's puffball under her nose. â€Å"I believe it speaks of a midnight rendezvous with Edmund of Gloucester.†

Online vs Traditional Learning Essay - 1723 Words

Online degrees are becoming an ever more trendy method to receive a college education and many students are switching to online education due to the quality of material, ease, and the level of flexibility in distance education programs. Recently, the internet has developed into a reliable capital of information for college seekers. Everything from applying for financial assistance to taking a course can be done online. Now, a student can even obtain online degrees from one of many schools offering online education as a practical choice to a traditional classroom education. There’s a load of information regarding online education, and distance education is becoming increasingly popular for students everywhere. Primarily, there are some†¦show more content†¦He alludes to an argument that states students learn far too little when the teacher’s personal presence is not available because the student has more to learn from the teacher than the texts. Many advocates of distance education are ardent about their venue and very critical of traditional education. These online education devotees view traditional classes as being unchangeable, inflexible, teacher-centered, and static (Angiello, 2010). However, proponents argue that many simply would not be able to get a degree without distance education—the full-time police officer, the mother of four, or the individual living in a rural area approximately 100-200 miles away from any educational institution. Many individuals desperately need distance education courses because they have jobs, families, civic responsibilities. They are thirsting. But some want us to say, Sorry you don’t want to drink the water there, but we can’t bottle our fresh spring water, so you’ll have to come here or drink nothing (Baker, 1986). Proponents contend that distance education is as good as traditional education. In other words, learning occurs as much in distance education as it does in traditional education. However, is this really so? Does distance education work better for some studentsShow MoreRelatedTraditional Vs. Online Learning1510 Words   |  7 PagesAmong schools with 12th-grade students during the 2010–11 school year, the average percentage of 12th-grade students who graduated with a high school diploma was 89 percent for traditional public schools, 91 percent for public charter schools, and 92 percent for private schools (Institute of Educational Sciences). Traditional on campus classes are those in which a student earns a diploma or a degree in a classroom environment taught by a professor. Students are provided with high amounts of socialRead MoreOnline Vs. Traditional Learning915 Words   |  4 PagesOnline Versus Traditional Learning Today s generation relies so much on technology that it has now been incorporated into the schools curriculum. What we once knew and have learned by using school books, is now being taught strictly electronic communication. Although the online environment learning can be utilized to enhance classroom lessons, doesn t mean it should come a main source of teaching. Schools around the nation has remove the use of textbooks and worksheet from the classroom it isRead MoreOnline Learning Vs. Traditional Classroom Learning1850 Words   |  8 Pages Online Learning vs. Traditional Classroom Learning Crystal I. Grimes Grimes9892@mail.philau.edu IT-201 Professor Jim Greeneâ€Æ' Abstract: Technology has had and imperative influence in almost every aspect of life. It has also made an impact on how we learn today. Some question how effective learning could be via the internet, while most stick with the familiarity of traditional classroom learning. Either option you choose still substantiates that technology has changed the way we learn and willRead MoreOnline Learning vs. Traditional Classroom Learning618 Words   |  3 Pagesthrough online learning, rather than in a traditional classroom. As, a student is making that choice it is critical that they choose the best learning environment that allows them to meets their individual needs. When students are selecting the best learning environment there are various consideration that should be made before a decision is made. Education ourselves about the benefits and disadvantages allows us to narrow our selection between online learning and traditional classroom learning. RegardlessRead MoreOnline Learning Vs. Traditional Classroom843 Words   |  4 Pagescan also broaden your horizons in the job market, getting the best education is important whether it is online learning or in a traditional classroom. The key is finding the one that will be most efficient for your learning. I would say online learning is more hard because I think there is a lot of unnecessary 1busy work assigned in my personal opinion than a classroom setting. Online learning has advantages as well such as being in the convenience of one’s own home and having the flexibility toRead MoreOnline Learning Vs. Traditional Classroom1586 Words   |  7 Pagesstudent has decided to pursue. The traditional learning experience is sold as we tour campuses. This is the way higher education has been completed since its inception. Society has evolved and so has the ways to obtain higher learning. Students now have the option to complete their learning online, as well as, the traditional route. Online learning has been looked at as a lower level of learning, compared to the traditional classroom setting. I believe that online learning has now evolved to the levelRead MoreOnline Learning Vs. Traditional Classroom891 Words   |  4 Pagesalso those who have chosen to get their degree in an onl ine setting. Online learning gives people the opportunity to get their education in a way that is more flexible and can fit in with their daily lives. Though both are reputable forms of higher educations, there are distinct differences between learning in a tradition classroom and learning in an online setting. The most obvious difference in online learning versus traditional classroom learning is the setting. Traditionally, students would gatherRead MoreOnline Learning Vs. Traditional Classroom959 Words   |  4 PagesOnline learning is much different from the traditional classroom settings. Aside from the obvious location differences, there are pros and cons to online learning that are very different from a classroom. A student must be willing to embrace these differences to successfully move forward and excel in the online environment. One of the things in online learning that is important is establishing your classroom. With a traditional class, the classroom and times are designated to the student. Read MoreOnline Classes vs. Traditional Classroom Learning 623 Words   |  2 PagesOnline Classes vs. Traditional Classroom Learning When deciding to take college courses there are many choices you have to make. One of the more important choices is where you plan to take your classes. Online classes are becoming increasingly popular because they can be easier to fit into a busy schedule, but some people still argue that nothing beats a traditional classroom setting. Having the option of online classes makes continuing an education more appealing to people that have to balanceRead MoreTraditional Classroom Learning Vs. Online Education2355 Words   |  10 PagesVs. Online Education Pauline Nguyen English 240 Professor Jawad Ali Traditional Classroom Learning Vs. Online Education Education is becoming one of the most essential things in a person’s life. Nowadays, many individuals are looking to go the extra mile and further their education. This is because numerous employers require a degree and a significant amount of experience in order to become eligible for certain occupations. The next question to consider is how to go about this. Younger

Womens Fight For Women - 1361 Words

Women in Combat For a long time now women have been an important part of the U.S. military, having performed admirably in Operations Iraqi Freedom and Enduring Freedom. The debate on whether women should play a role in combat has been a long standing debate that has not yet reached a consensus. According to Daniel Davis in his article â€Å"The Truth About Women in Ground Combat Roles† President Obama commended the December 3 decision by Secretary of Defense Ash Carter to open all combat jobs to women. Many arguments have been raised in defense of military policy that bans women from combat roles. According to Megan Mackenzie in â€Å"Let Women Fight† nearly 214,098 women serve in the U.S. military, representing 14.6 percent of total service members. The main question or concern is not whether women are capable of conducting combat operations, but instead is can women endure the physical and physiological rigors of sustained combat operations. Many people who are against the involvement of women on the front line make the argument that with women entering combat the standards of the military will be lowered. Women have already proven t heir selves to be able to do just about as much as men can in a combat role. However, there are quite a few concerns when it comes to women serving in combat. For example, the physical ability of women. Majority of jobs in the armed forces are open equally to men and women, but there are some jobs to which women are not physically able to pursue. ThereShow MoreRelatedEssay about The History of the Women’s Suffrage Movement977 Words   |  4 PagesWomen’s suffrage, or the crusade to achieve the equal right for women to vote and run for political office, was a difficult fight that took activists in the United States almost 100 years to win. On August 26, 1920 the 19th Amendment to the Constitution of the United States was ratified, declaring all women be empowered with the same rights and responsibilities of citizenship as men, and on Election Day, 1920 millions of women exercised their right to vote for the very first time. The women’sRead MoreWomen s Rights During The 19th Amendment1414 Words   |  6 Pages4 states gave women the right to vote. The western states gave women the right to vote so that more people could come to their states. The women suffrage was run by strong women. Two of the women were Susan B. Anthony and Elizabeth Cady. The women got the right to vote because they have been fighting for their rights since 1775, women were striking for their rights at their job to so that they can be equal as men, the women’s right convention that opened the new possibility for women, and the 4 statesRead MoreWomen s Suffrage Movement : Women1440 Words   |  6 Pageshour April 24, 2016 Women’s Suffrage Movement In the late 1800’s through the early 1900’s, women were not given the rights they have today and were being mistreated, but because of a few brave women who gave up their lives to fight for what they knew was right, this all changed. Many of these women were educated and brave, but were still denied their rights. Women have suffered through this long battle to get what they knew they deserved and took time out of their lives to fight for what they believedRead MoreAnalysis Of The Poem Diving Into The Wreck 1365 Words   |  6 Pages â€Å"Diving into the Wreck†: An Analysis of Women’s Status Change in the Current Society At the beginning of Adrienne Rich’s poem- â€Å"Diving into the Wreck†, the poet uses â€Å"book of myths, camera, knife, body armor, and grave and awkward mask† to start the poem. Through those words, it is hard to tell if she wants to fight for women’s rights, or just â€Å"dive into the wreck.† However, in general, people usually use their cameras to take pictures or record videos to memorialize things that happened to themRead MoreWomen s Suffrage Movement : Women1440 Words   |  6 Pageshour April 24, 2016 Women’s Suffrage Movement In the late 1800’s through the early 1900’s, women were not given the rights they have today and were being mistreated, but because of a few brave women who gave up their lives to fight for what they knew was right, this all changed. Many of these women were educated and brave, but were still denied their rights. Women have suffered through this long battle to get what they knew they deserved and took time out of their lives to fight for what they believedRead MoreThe Equal Rights Of The Sexes Movement1430 Words   |  6 PagesThe Equal Rights of the Sexes Movement Women have been fighting the fight for women’s rights for a long time. In fact, evidence can be traced back centuries of women’s desire for equality. Specifically, women have fought for their rights in the eighteenth and nineteenth centuries. The eighteenth century had its share of freedom supporters. The eighteenth century is a time of the included focus points of the daughters of liberty, Mary Wollstonecraft, and Judith Sargent Murray. Right after the revolutionRead MoreThe Fight to Vote Essay1312 Words   |  6 PagesMany women and African American men had long dreamed to have the right to vote. In many states, they could only vote if their state allowed them the privilege. The dedicated men and women fought for their right to vote in the Civil Rights Movement in the early and mid 1900s. Congress passed the Fifteenth Amendment and the Voting Rights Act to give African Americans the rights to vote. It would have not occurred if the Civil Rights Movement had not taken place. The Nineteenth Amendment would not haveRead MoreThe Equal Rights Of The Sexes Movement1196 Words   |  5 PagesThe Equal Rights of the Sexes Movement Women have been fighting the fight for women’s rights for a long time. In fact, evidence can be traced back centuries of women’s desire for equality. Specifically, women have fought for their rights in the eighteenth and nineteenth centuries. The eighteenth century had its share of freedom supporters. The eighteenth century is a time of the included focus points of the daughters of liberty, Mary Wollstonecraft, and Judith Sargent Murray. Right after the revolutionRead MoreWomen s Degradation By Elizabeth Cady Stanton928 Words   |  4 Pagesâ€Å"Women’s degradation is in man’s idea of his sexual rights. Our religion, laws and customs are all founded on the belief that woman was made for man† (Stanton) Believed to be one of the greatest and most influential feminists of not only her generation, but of all time, Elizabeth Cady Stanton paved the way for women and their rights in a time when they had none. Elizabeth, was one of the first feminist theorists in America and through her beliefs that women deserve equality and equal rights, sheRead MoreWomen s Rights Is The Fight For Equality1083 Words   |  5 PagesWomen’s rights is the fight for the idea that women should have equal rights with me n. Over history, this has taken the form of gaining property rights, the women’s suffrage, or the right of women to vote, reproductive rights, and the right to work for for equal pay. Women were conditioned to be treated like second class citizens solely because of their gender until a crucial movement in American history during the late nineteenth century that would inspire a long fight for equality for generations