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English & Irish Gothic:English & Irish Gothic:and Their Modernist and Their Modernist

TurnsTurnsYing-hsiung ChouYing-hsiung Chou

yhchou@mail.nctu.edu.twyhchou@mail.nctu.edu.tw幻奇文學跨界想像研習營 幻奇文學跨界想像研習營 (Dec. 9, 200(Dec. 9, 2007)7)

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I. Gothicism and/or ModernityI. Gothicism and/or Modernity

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In a Metro StationIn a Metro StationEzra Pound (1913)Ezra Pound (1913)

The apparition of these faces in the The apparition of these faces in the crowdcrowd

Petals on a wet, black boughPetals on a wet, black bough

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Gothicism and ModernityGothicism and Modernity

Gothicism in modern times: Gothicism in modern times:

literature, architecture, film, music, literature, architecture, film, music, fashion, etc.fashion, etc.

Modernity=Gothicism?Modernity=Gothicism?

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66

77

88

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The American Gothic, by Grant Wood (1930)The American Gothic, by Grant Wood (1930)

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Two Faces of Modernity I Two Faces of Modernity I (positive)(positive)

Modernist disenchantment (Weber)Modernist disenchantment (Weber)

Then: Then: Enchantment of the world dominated by Enchantment of the world dominated by

myth and religion in the Medieval worldmyth and religion in the Medieval world

Now: Now: Its disenchantment through science Its disenchantment through science

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Modernity characterized byModernity characterized bysecularization (in religion, e.g., Protestansecularization (in religion, e.g., Protestantism)tism)rationalization/legitimation., e.g.,rationalization/legitimation., e.g.,

capitalist rational management system capitalist rational management system bureaucracy in governance through the bureaucracy in governance through the rule of law rule of law

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Representing Modernity IRepresenting Modernity I

Perspective in visual artPerspective in visual artHarmonic principles in musicHarmonic principles in musicLiberalist realism in the novel*Liberalist realism in the novel*

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*Liberalism and the Rise of the Novel*Liberalism and the Rise of the Novel Liberalist emphasis on individuality (and developmenLiberalist emphasis on individuality (and development of its potentials) (Ian Watt)t of its potentials) (Ian Watt) Liberalist adversariality principle through which a perLiberalist adversariality principle through which a person coexists with her fellow citizensson coexists with her fellow citizens Human beings are rational and are thus capable of sorHuman beings are rational and are thus capable of sorting out their problems in their community without reting out their problems in their community without recourse to absolute powerscourse to absolute powers For example, For example, Bildungsroman Bildungsroman (novel of formative educ(novel of formative education, e.g., ation, e.g., Wilhelm Meister’s Apprenticeship, PortraiWilhelm Meister’s Apprenticeship, Portrait of the Artist as a Young Mant of the Artist as a Young Man))

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Two Faces of Modernity II: Two Faces of Modernity II: Modernist (re)enchantment? (negative)Modernist (re)enchantment? (negative) social pathology (vs. social progress), e.g.,social pathology (vs. social progress), e.g.,

anomieanomie (social instability caused by (social instability caused by the disruption of order, which is the disruption of order, which is responsible for suicide, crime and responsible for suicide, crime and mental disorder; cf. Durkheim)mental disorder; cf. Durkheim)

alienation (Marx)alienation (Marx)

the gothic/grotesque the gothic/grotesque

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Gothic ModernityGothic Modernityor, the underside of modernityor, the underside of modernity

Woolf: haunted house of the mindWoolf: haunted house of the mind

Joyce: capitalist specteralizationJoyce: capitalist specteralization

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Woolf’s experimentations onWoolf’s experimentations onThe mind and the world, and their The mind and the world, and their

interpenetrationinterpenetration

By way of probing the underside of By way of probing the underside of realityreality

A reality that is felt to be unacceptable A reality that is felt to be unacceptable (due to war, paternalism, materialism, (due to war, paternalism, materialism, etc.)etc.)

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““A Haunted House”A Haunted House”(from Virginia Woolf, (from Virginia Woolf, Monday or TuesdayMonday or Tuesday [1921]) [1921])

A classic Gothic story withA classic Gothic story with

A ghostly coupleA ghostly couple

A haunted houseA haunted house

Supernatural happeningsSupernatural happenings

Innocent subjectsInnocent subjects

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Gothicism with a Modernist TurnGothicism with a Modernist TurnCognitive HesitanciesCognitive Hesitancies

A ghostly couple revisit their old A ghostly couple revisit their old house looking for “it” (that they’ve house looking for “it” (that they’ve left behind or left unachieved before left behind or left unachieved before they died?)they died?)

They go about their search, trying as They go about their search, trying as much as possible not to disturb the much as possible not to disturb the current tenants. current tenants.

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But the latter (the narrator being one of But the latter (the narrator being one of them) are already aware of their visitthem) are already aware of their visit

While the house is haunted by the visitors, While the house is haunted by the visitors, the ghostly couple are also being stalked the ghostly couple are also being stalked by the narratorby the narrator

And in the “melee” of knowing and being And in the “melee” of knowing and being known, the house also takes on a life of its known, the house also takes on a life of its own and pronounces, “Safe, safe, safe”own and pronounces, “Safe, safe, safe”

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To add to the confusion, nature--as an To add to the confusion, nature--as an extension of the house, including what’s extension of the house, including what’s inside and outside --also joins in, making inside and outside --also joins in, making “sense” further inaccessible “sense” further inaccessible

The outside scene is seen darkly through The outside scene is seen darkly through the glass (cf. 1 Corinthians 12: “Now we the glass (cf. 1 Corinthians 12: “Now we see through a glass, darkly)see through a glass, darkly)

And as the natural world is projected unto And as the natural world is projected unto the domestic space, it becomes free-the domestic space, it becomes free-floating and intangiblefloating and intangible

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As the narrator pushes for an explanatioAs the narrator pushes for an explanation for the visitation, death is introduced an for the visitation, death is introduced as it has already taken the ghostly couple s it has already taken the ghostly couple away from each other hundreds of years away from each other hundreds of years agoago””Death was the glass; death was betweDeath was the glass; death was between us”en us”

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The story ends with “I” (the narrator) The story ends with “I” (the narrator) waking up (cf. “human voices wake waking up (cf. “human voices wake us, and we drown,”) crying, us, and we drown,”) crying,

““Oh, is this Oh, is this youryour buried treasure? The buried treasure? The light in the heart” light in the heart”

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Six ImplicationsSix Implications Am I the one you’ve been looking for? Or, am Am I the one you’ve been looking for? Or, am I the light in the heart? (The perceiver turned iI the light in the heart? (The perceiver turned into the perceived?)nto the perceived?) And, like narrator, like reader:And, like narrator, like reader:

Am I, the reader, the haunted, somehow suffering frAm I, the reader, the haunted, somehow suffering from my inability to know the worldom my inability to know the world And yet am paradoxically implicated in narration (i.And yet am paradoxically implicated in narration (i.e., the focus being no longer on the hard-nosed wore., the focus being no longer on the hard-nosed world out there, but the mind itself, asking: “What do I ld out there, but the mind itself, asking: “What do I know” [cf. Montaign] )?know” [cf. Montaign] )?

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Strategies of a Modern Gothic Strategies of a Modern Gothic ReadingReading

1. Floating pronouns (in the absence of fi1. Floating pronouns (in the absence of fixed antecedents): they (the ghostly coupxed antecedents): they (the ghostly couple?), one (the resident in the house, I?), it le?), one (the resident in the house, I?), it (the buried treasure, the past, death?)(the buried treasure, the past, death?)2. countrapuntality of self and other2. countrapuntality of self and other

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3. Seeing and being seen as two-3. Seeing and being seen as two-way, reciprocal cognitionway, reciprocal cognition

I, the narrator, am left undisturbed andI, the narrator, am left undisturbed andI stalk them even though they could not I stalk them even though they could not

been seen, because,been seen, because,we are separated by the glass, which is we are separated by the glass, which is

deathdeath

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4. The interpenetration of the real and 4. The interpenetration of the real and the ghostly the ghostly

The world is seen darkly in/through the glassThe world is seen darkly in/through the glassThe world casts its shadows in the drawing The world casts its shadows in the drawing

room—”spread about the floor, hung upon room—”spread about the floor, hung upon the walls, pendant from the ceiling—what?”the walls, pendant from the ceiling—what?”

But the world cannot be grasped empiricallyBut the world cannot be grasped empirically—”. . .my hands are empty”—”. . .my hands are empty”

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5. The interpenetration of the 5. The interpenetration of the animate and the inanimateanimate and the inanimate

The house seems to be energized by the The house seems to be energized by the visitation and takes on a life of its own, visitation and takes on a life of its own, providing a shelter to the visitants providing a shelter to the visitants (“Safe, safe, safe”)(“Safe, safe, safe”)

Above all, the house is intelligent and Above all, the house is intelligent and knowledgeable about the buried treasureknowledgeable about the buried treasure

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6. The interpenetration of life and death6. The interpenetration of life and death

Our eyes darken; we hear no steps beside Our eyes darken; we hear no steps beside us; we see no lady spread her ghostly cloakus; we see no lady spread her ghostly cloak

Waking, I cried “Oh, is this Waking, I cried “Oh, is this your your buried buried treasure? The light in the heart.”treasure? The light in the heart.”

(Existential and epistemological death-in-(Existential and epistemological death-in-life?)life?)

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Two Modes of Death in Modern Two Modes of Death in Modern LifeLife

Woolf: the ambivalences of human intellWoolf: the ambivalences of human intelligence (i.e., its/her inability to know vis-igence (i.e., its/her inability to know vis-à-vis the privileging of reverie) renders pà-vis the privileging of reverie) renders people less than human (epistemological eople less than human (epistemological aporia)aporia)Joyce: the dehumanized condition of citJoyce: the dehumanized condition of city life reduces people to a stage of paralyy life reduces people to a stage of paralyzed automatons and hence deathzed automatons and hence death

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““The Dead”The Dead”(from James Joyce, (from James Joyce, Dubliners Dubliners [1907,1914])[1907,1914])

Biblical Gabriel: An archangel in the Bible, he Biblical Gabriel: An archangel in the Bible, he was employed to announce the birth of John was employed to announce the birth of John the Baptist to Zechariah and to announce the the Baptist to Zechariah and to announce the birth of Jesus to the Virgin Marybirth of Jesus to the Virgin Mary

Gabriel Conroy attends a Christmas party Gabriel Conroy attends a Christmas party and despite his self-claimed voice of and despite his self-claimed voice of enlightenment finds himself increasingly enlightenment finds himself increasingly alienated, even accused of being a stranger, alienated, even accused of being a stranger, a West Briton (149)a West Briton (149)

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As the party winds down, Gabriel sees a womaAs the party winds down, Gabriel sees a woman standing at the top of the staircase listening n standing at the top of the staircase listening to some indistinct music. The woman turns outo some indistinct music. The woman turns out to be his wife, t to be his wife, but there is something about her that he cannbut there is something about her that he cannot quite see throughot quite see through The distant music turns out to be a tune GrettaThe distant music turns out to be a tune Gretta’s teenage lover once sang to her before he di’s teenage lover once sang to her before he died soon after serenading her in the rained soon after serenading her in the rain

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Gabriel’s alienation multiplies. He is Gabriel’s alienation multiplies. He is physically denied by his wife.physically denied by his wife.

The story ends with Gabriel feeling the The story ends with Gabriel feeling the onset of death—onset of death—

””one by one they were all becoming shades one by one they were all becoming shades (176). He imagines he sees “the form of a (176). He imagines he sees “the form of a young man standing under a dripping tree” young man standing under a dripping tree”

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““The solid world itself which these The solid world itself which these dead had one time reared and lived dead had one time reared and lived in was dissolving and dwindling” (All in was dissolving and dwindling” (All That’s Solid Melts into Air.)That’s Solid Melts into Air.)

““The time had come for him to set The time had come for him to set out on his journey westward”out on his journey westward”

Voices from/of the other (world)Voices from/of the other (world)

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II. English GothicII. English Gothic

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Psychological grounds for GothicismPsychological grounds for Gothicism

Progress or regression/degeneration Progress or regression/degeneration Reason or un-reason (the unconscious)Reason or un-reason (the unconscious)

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Historical grounds for Gothicism?Historical grounds for Gothicism?

French RevolutionFrench RevolutionIndustrial Revolution(s)Industrial Revolution(s)

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All That is Solid Melts into AirAll That is Solid Melts into AirWhat seems is not what isWhat seems is not what isSpectralization of modern lifeSpectralization of modern life

““The Adoration of the Magi” (1897) by YeatsThe Adoration of the Magi” (1897) by Yeats““Midsummer Night Madness” (1932) by O’Midsummer Night Madness” (1932) by O’FaolainFaolain““A Haunted House” (1921) by WoolfA Haunted House” (1921) by Woolf

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Figuration between the Marvellous Figuration between the Marvellous and the Uncannyand the UncannyAccepting the supernatural as it isAccepting the supernatural as it is

Realizing the potential inadequacy of Realizing the potential inadequacy of a rational epistemological (i.e., how a rational epistemological (i.e., how we know what we know) outlookwe know what we know) outlook

Cognitive hesitanciesCognitive hesitancies

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Hesitancies 1Hesitancies 1Past and Present/FuturePast and Present/Future

Past coming back to haunt present, e.g., Past coming back to haunt present, e.g., CarCarmilla, “milla, “The Words on the Window Pane.”The Words on the Window Pane.”Self and OtherSelf and Other

Self being possessed by Other, e.g., “The ASelf being possessed by Other, e.g., “The Adoration of the Magi”doration of the Magi”

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Hesitancies 2Hesitancies 2Real and Un-realReal and Un-real

Historical settings and characters transformHistorical settings and characters transformed into their uncanny other, e.g., “Midsumed into their uncanny other, e.g., “Midsummer Night Madness”mer Night Madness”Textual/Visual and historicalTextual/Visual and historical

Textually constructed world superimposed Textually constructed world superimposed upon the real world, e.g., upon the real world, e.g., DraculaDracula

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English and/or Irish Gothic 1English and/or Irish Gothic 1(Cf. English Gothic slide 45)(Cf. English Gothic slide 45)

Minerva Press (London) publishes a large Minerva Press (London) publishes a large portion of Gothic novels written during the portion of Gothic novels written during the 1790s by women on Irish themes1790s by women on Irish themes

English Gothic: Contra-realist outlook*English Gothic: Contra-realist outlook*

things are not what they seem, esp. in modern things are not what they seem, esp. in modern times dominated by reason, technology and times dominated by reason, technology and bureaucracybureaucracy

representation of the uncanny as an act of representation of the uncanny as an act of empowermentempowerment

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*Realism*RealismIan Watt, Ian Watt, The Rise of the NovelThe Rise of the Novel (1957) (1957)

Decline of classical idealism Decline of classical idealism (universal Idea)(universal Idea)

In favor of a modern outlook with In favor of a modern outlook with emphasis on the individual:emphasis on the individual:

Possessive individualismPossessive individualismLiberal individualismLiberal individualism

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English and/or Irish Gothic 2English and/or Irish Gothic 2 Irish Gothic:Irish Gothic:

A branch of English GothicA branch of English Gothic A uniquely Irish/Anglo-Irish or Anglo-A uniquely Irish/Anglo-Irish or Anglo-

Anglican modeAnglican mode

Attachment to Irish history and politics Attachment to Irish history and politics (McCormack 833), or(McCormack 833), or

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English and/or Irish Gothic 3English and/or Irish Gothic 3Anglo-Irish double bind (choosing Anglo-Irish double bind (choosing

between two unsatisfactory decisions)between two unsatisfactory decisions)

demonization of the indigenous demonization of the indigenous Catholic tradition (for its corrupt Catholic tradition (for its corrupt practices)practices)

realization of their own identity with realization of their own identity with that which they demonize (cf. more that which they demonize (cf. more Irish than the Irish)Irish than the Irish)

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Recap: Irish Gothic 1Recap: Irish Gothic 1A branch of English Gothic, with specific A branch of English Gothic, with specific local colors, e.g., local colors, e.g.,

Catholic/Irish ChurchCatholic/Irish ChurchNationalism/sectarianismNationalism/sectarianismnational character(s), Protestant magic/occnational character(s), Protestant magic/occultism (Freemasons)ultism (Freemasons)faminefamineetc.etc.

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Recap: Irish Gothic 2Recap: Irish Gothic 2

A unique mode of A unique mode of representing/reading the Irish representing/reading the Irish experience;experience;

But what precisely is unique about But what precisely is unique about the Irish Gothic?the Irish Gothic?

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SpeculationsSpeculations

Ireland as a Gothic space, which is paradoxical, Ireland as a Gothic space, which is paradoxical, anomalousanomalous

Demonization of Irish and Identifying with the Demonization of Irish and Identifying with the IrishIrish

Anglo-Irish writing in a Gothic mode to make up Anglo-Irish writing in a Gothic mode to make up for their declining influence (Roy Foster, in for their declining influence (Roy Foster, in Killeen 2)Killeen 2)

Gothic writing as exorcismGothic writing as exorcism

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English Gothic 1English Gothic 1(Periodization)(Periodization) Classic/Literary Gothic (1764-1820s), often deaClassic/Literary Gothic (1764-1820s), often dealing with dynastic disorders in an alien setting ling with dynastic disorders in an alien setting involving victimizations (of a hapless maiden)involving victimizations (of a hapless maiden)

The Castle of Otronto The Castle of Otronto (1764): recovered text with d(1764): recovered text with dubious authenticity and dynastic disorders (cf. Macubious authenticity and dynastic disorders (cf. MacPherson, Pherson, Ossian, 1765Ossian, 1765) ) The Mysteries of Udolpho The Mysteries of Udolpho (1794): the heroine’s gr(1794): the heroine’s gradual realization of her identity as a illegitimate chiadual realization of her identity as a illegitimate child (Miles 46-47)ld (Miles 46-47)

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Strawberry HillStrawberry Hill

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English Gothic 2English Gothic 2Romantic (1820s-1840s)Romantic (1820s-1840s)

FrankensteinFrankenstein (1818, 1831): (1818, 1831):

Instinctual self (vs. rational self)Instinctual self (vs. rational self)Forbidden knowledge, overreaching (the Forbidden knowledge, overreaching (the

Faustian) (vs. Grace)Faustian) (vs. Grace)Science and reason (vs. religion)Science and reason (vs. religion)Monstrosity (vs. humanity)Monstrosity (vs. humanity)

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Schloss-FrankensteinSchloss-Frankenstein

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Benjamin Franklin, 1752Benjamin Franklin, 1752

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Luigi Galvani, 1771Luigi Galvani, 1771

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BioelectricityBioelectricity

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English Gothic 3English Gothic 3Victorian (1840s*-1890s)Victorian (1840s*-1890s)

Strange Case of Dr. Jekyll and Mr. Hyde (188Strange Case of Dr. Jekyll and Mr. Hyde (1886):6): self and other; good and evil; outward re self and other; good and evil; outward respectability and inner lustspectability and inner lustThe Picture of Dorian Gray (1890): fin-de-sieThe Picture of Dorian Gray (1890): fin-de-siecle cle quest for authenticity and spontaneityquest for authenticity and spontaneity

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*Gothic Revival*Gothic Revival1840s-1840s-

A new movement in architecture A new movement in architecture favoring the medieval over the favoring the medieval over the classicalclassical

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Royal Scottish Academy, EdinburghRoyal Scottish Academy, Edinburgh

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Victoria Tower, WestminsterVictoria Tower, Westminster

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Cathedral of Learning, University of PittsburghCathedral of Learning, University of Pittsburgh

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Richard Mansfield, 1887 Richard Mansfield, 1887 (Strange Case of Dr. Jekyll and Mr. Hyde)(Strange Case of Dr. Jekyll and Mr. Hyde)

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Deacon William Brodie Deacon William Brodie (1741-1788, councillor, craftsman, thief)(1741-1788, councillor, craftsman, thief)

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English Gothic 4English Gothic 4Modern (1890s-)Modern (1890s-)

Dracula, 1897Dracula, 1897Occultism against scienceOccultism against scienceDesire against MannersDesire against Manners

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Castle DraculaCastle Dracula

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Dracula (1931)Dracula (1931)

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Marfan SyndromeMarfan Syndrome 馬凡氏症 馬凡氏症 Marfan Syndrome (MFS)Marfan Syndrome (MFS)

簡介、病因簡介、病因　　馬凡氏症是一種系統性的結締組織病變，古時候的劉備（傳言其雙手過膝），及高瘦的美國總統林肯均被懷疑為馬凡氏症候群之病患。最早於　　馬凡氏症是一種系統性的結締組織病變，古時候的劉備（傳言其雙手過膝），及高瘦的美國總統林肯均被懷疑為馬凡氏症候群之病患。最早於 18961896 年由法國小兒科醫師 年由法國小兒科醫師 Marfan Marfan 提出臨床報告。馬凡氏症患者在臨床上具有骨骼系統、眼睛系統、心血管系統等三大方面臨床特徵，目前已知約提出臨床報告。馬凡氏症患者在臨床上具有骨骼系統、眼睛系統、心血管系統等三大方面臨床特徵，目前已知約 70%~93%70%~93% 馬凡氏患者是因為位於第馬凡氏患者是因為位於第 1515 對染色體上的對染色體上的 fibrillin-1 (FBN1) fibrillin-1 (FBN1) 基因發生突變所造成，帶有此基因突變者均會發病，但是依據基因突變點的不同，臨床症狀也有相當大差別，甚至同一家族中也可基因發生突變所造成，帶有此基因突變者均會發病，但是依據基因突變點的不同，臨床症狀也有相當大差別，甚至同一家族中也可能有不同程度的臨床症狀，嚴重者可能出生即有生命危險，輕微者早期幾乎不具任何症狀。能有不同程度的臨床症狀，嚴重者可能出生即有生命危險，輕微者早期幾乎不具任何症狀。發生率、遺傳模式發生率、遺傳模式　　馬凡氏症的發生率依國外統計約為　　馬凡氏症的發生率依國外統計約為 1/100001/10000 ，而且不具有人種或性別差異性，國內並無相關統計資料，但依照台北榮總患者人數估計，國內發生率約與國外相當。馬凡氏症為體染色體顯性遺傳疾病，因此如果雙親中有一人患有此症，其子代不分性別，每胎皆有，而且不具有人種或性別差異性，國內並無相關統計資料，但依照台北榮總患者人數估計，國內發生率約與國外相當。馬凡氏症為體染色體顯性遺傳疾病，因此如果雙親中有一人患有此症，其子代不分性別，每胎皆有 50%50% 的機會患有此症。的機會患有此症。臨床症狀及診斷臨床症狀及診斷　　臨床診斷標準根據 　　臨床診斷標準根據 1996 1996 年 年 DePaepe DePaepe 等人所提出的主要及次要特徵作為診斷的參考：等人所提出的主要及次要特徵作為診斷的參考：一、 骨骼系統一、 骨骼系統1. 1. 主要特徵主要特徵

胸腔骨骼異常：胸骨突出或凹陷（雞胸或漏斗胸）胸腔骨骼異常：胸骨突出或凹陷（雞胸或漏斗胸） 上下半身比例縮減（ 上下半身比例縮減（ < 0.85< 0.85／成年及年長之兒童）、展臂長與身高之比值過高（ ／成年及年長之兒童）、展臂長與身高之比值過高（ > 1.05> 1.05 ）） 因為四肢骨骼過度生長，因此具有蜘蛛狀手指，使得手腕有因為四肢骨骼過度生長，因此具有蜘蛛狀手指，使得手腕有Walker-MurdochWalker-Murdoch 之特徵（當一邊手掌握住對側的手腕時，其姆指與小指能夠重疊在一起）；姆指有 之特徵（當一邊手掌握住對側的手腕時，其姆指與小指能夠重疊在一起）；姆指有 SteinbergSteinberg 特徵（指在沒外力的協助下，當手姆指可以向尺骨側彎曲時，可以超出掌面）特徵（指在沒外力的協助下，當手姆指可以向尺骨側彎曲時，可以超出掌面） 脊柱側彎（脊柱側彎（scoliosisscoliosis ）、脊柱後凸（）、脊柱後凸（kyphosiskyphosis）或脊柱脫位（）或脊柱脫位（spondylolithesisspondylolithesis ）） 肘關節伸展程度減小（ 肘關節伸展程度減小（ < 170 < 170 度）度） 扁平足扁平足 髖骨突出（髖骨突出（ protrusio acetabuliprotrusio acetabuli ））

2. 2. 次要特徵次要特徵 漏斗胸嚴重程度中度漏斗胸嚴重程度中度 關節過動（關節過動（hypermobilityhypermobility ）） 高而呈拱形的上顎及發育不全的下顎、牙齒排列擁擠高而呈拱形的上顎及發育不全的下顎、牙齒排列擁擠 臉部特徵：頭骨細長（臉部特徵：頭骨細長（dolichocephalydolichocephaly ）、）、Malar hypoplasiaMalar hypoplasia 、眼球內陷（、眼球內陷（ enophthalmosenophthalmos ）、下顎後縮（）、下顎後縮（retrognathiaretrognathia ）、眼瞼下垂、眼裂。）、眼瞼下垂、眼裂。

二、 眼睛系統二、 眼睛系統1. 1. 主要特徵主要特徵

水晶體異位（水晶體異位（ ectopia lentisectopia lentis ）：容易引發高度近視、視網膜剥離、青光眼、白內障）：容易引發高度近視、視網膜剥離、青光眼、白內障2. 2. 次要特徵次要特徵

異常平坦角膜異常平坦角膜 眼球軸長過長眼球軸長過長 虹膜或睫狀肌發育不全虹膜或睫狀肌發育不全

三、 心血管系統三、 心血管系統1. 1. 主要特徵主要特徵

升主動脈擴張升主動脈擴張 主動脈管壁有裂縫、剥離主動脈管壁有裂縫、剥離

2. 2. 次要特徵次要特徵 二尖瓣脫垂、閉鎖不全二尖瓣脫垂、閉鎖不全 肺動脈擴張（肺動脈擴張（4040歲以前且無其他影響因素）歲以前且無其他影響因素） 二尖瓣鈣化（二尖瓣鈣化（4040歲以前）歲以前） 其他動脈擴張或破裂（其他動脈擴張或破裂（5050歲以前）歲以前）

四、 其他四、 其他除了主要三大系統的臨床表現外，其他的系統也有一些臨床症狀除了主要三大系統的臨床表現外，其他的系統也有一些臨床症狀1. 1. 肺臟：自發性的氣胸、肺尖的水泡肺臟：自發性的氣胸、肺尖的水泡2. 2. 皮膚包膜：皺縮的紋路（非壓迫、懷孕或體重減少所引起）、週期性或割口性的疝氣皮膚包膜：皺縮的紋路（非壓迫、懷孕或體重減少所引起）、週期性或割口性的疝氣3. 3. 腰椎部位的硬腦膜膨大（腰椎部位的硬腦膜膨大（ lumbosacral dural ectasialumbosacral dural ectasia ），這是三大器官系統以外的主要標準，可以利用），這是三大器官系統以外的主要標準，可以利用CTCT或或MRIMRI來檢查。來檢查。　　馬凡氏症臨床特徵中，心血管的病變是造成患者早期死亡的主要原因，最常見的是主動脈瘤、主動脈管壁剥離、二尖瓣脫垂及閉鎖不全所造成的回流。目前已有藥物可以延緩心血管症狀的發生，因此早期診斷早期治療對馬凡氏症患者有相當大的幫助。最常用的藥物是　　馬凡氏症臨床特徵中，心血管的病變是造成患者早期死亡的主要原因，最常見的是主動脈瘤、主動脈管壁剥離、二尖瓣脫垂及閉鎖不全所造成的回流。目前已有藥物可以延緩心血管症狀的發生，因此早期診斷早期治療對馬凡氏症患者有相當大的幫助。最常用的藥物是proprannololproprannolol ，這是一種已經被使用很久的藥，主要可以減緩心跳的速度，並減少心臟的收縮力，藉此減少血流對升主動脈的衝擊，進而避免主動脈瘤的破裂，長期使用後需注意不可貿然停藥，否則將有心律不整的危險。，這是一種已經被使用很久的藥，主要可以減緩心跳的速度，並減少心臟的收縮力，藉此減少血流對升主動脈的衝擊，進而避免主動脈瘤的破裂，長期使用後需注意不可貿然停藥，否則將有心律不整的危險。治療與照護治療與照護　　目前仍無有效治療方法，早期診斷後藉由藥物治療可改善預後情形，延長患者生命及生活品質，若能早期診斷，避免劇烈運動，將可延緩併發症發作時間，許多患者往往因為體型高大因而成為球類校隊，嚴格的訓練更提早心臟病的發生。　　目前仍無有效治療方法，早期診斷後藉由藥物治療可改善預後情形，延長患者生命及生活品質，若能早期診斷，避免劇烈運動，將可延緩併發症發作時間，許多患者往往因為體型高大因而成為球類校隊，嚴格的訓練更提早心臟病的發生。　　馬凡氏症患者應定期進行心臟超音波以及主動脈血管的檢查和監測，同時須定期追蹤眼睛及水晶體功能及異常情形。由於骨骼發展嚴重程度會隨年齡而增加，尤其青春期時惡化速度會加快，因此孩童及青少年時期應細心觀察骨骼系統發展，如脊柱側彎情形嚴重或胸骨有嚴重異常壓迫心肺正常功能時，都必須適時進行手術矯治。　　馬凡氏症患者應定期進行心臟超音波以及主動脈血管的檢查和監測，同時須定期追蹤眼睛及水晶體功能及異常情形。由於骨骼發展嚴重程度會隨年齡而增加，尤其青春期時惡化速度會加快，因此孩童及青少年時期應細心觀察骨骼系統發展，如脊柱側彎情形嚴重或胸骨有嚴重異常壓迫心肺正常功能時，都必須適時進行手術矯治。　　眼睛部份水晶體異位幾乎雙眼都會發生，其中以往上方的異位最多見，通常水晶體異位本身並不會嚴重影響視力，但其可能引發的高度近視、視網膜剥離、白內障或青光眼，造成視力的損傷，甚至有失明的可能。關於水晶體異位的治療，通常只有針對其造成的視力不良以眼鏡矯正，除非異位情況嚴重才需要進行手術摘除。　　眼睛部份水晶體異位幾乎雙眼都會發生，其中以往上方的異位最多見，通常水晶體異位本身並不會嚴重影響視力，但其可能引發的高度近視、視網膜剥離、白內障或青光眼，造成視力的損傷，甚至有失明的可能。關於水晶體異位的治療，通常只有針對其造成的視力不良以眼鏡矯正，除非異位情況嚴重才需要進行手術摘除。　　正常的主動脈直徑決定於身高與體重，因此用體表面積可以評估理想的主動脈大小。當患者主動脈直徑大於正常值的　　正常的主動脈直徑決定於身高與體重，因此用體表面積可以評估理想的主動脈大小。當患者主動脈直徑大於正常值的 1.51.5到到22倍時，可進行預防性的手術修復，因為隨著管壁增大，發生管壁剥離的機率也增加，最後可能造成主動脈瘤而危及生命。倍時，可進行預防性的手術修復，因為隨著管壁增大，發生管壁剥離的機率也增加，最後可能造成主動脈瘤而危及生命。基因檢測及產前檢查基因檢測及產前檢查　　目前已知約　　目前已知約 70%~93%70%~93% 馬凡氏症患者是因為第馬凡氏症患者是因為第 1515 對染色體上位於對染色體上位於q12-q21.1q12-q21.1 的的FBN1FBN1基因發生突變所導致。由於基因發生突變所導致。由於FBN1FBN1 基因的組成多達基因的組成多達6565個外顯子（個外顯子（ exonsexons）區域，大小約）區域，大小約 110Kb110Kb，實為相當大一段區域，而且突變位置分散，各個家族間有獨特的突變點及臨床表現，家族中成員進行連鎖分析以早期診斷，適時給予預防及控制。，實為相當大一段區域，而且突變位置分散，各個家族間有獨特的突變點及臨床表現，家族中成員進行連鎖分析以早期診斷，適時給予預防及控制。參考資料：參考資料：1. OMIM#154700 1. OMIM#154700 － － Marfan SyndromeMarfan Syndrome2. Genetest 2. Genetest － － http://http://www.genetests.orgwww.genetests.org3. 3. 罕見疾病基金會－罕見疾病基金會－Marfan SyndromeMarfan Syndrome ，馬凡氏症，馬凡氏症4. 4. 遺傳諮詢服務窗口－遺傳諮詢服務窗口－Marfan SyndromeMarfan Syndrome ，馬凡氏症，馬凡氏症字型大小：大 中 小字型大小：大 中 小 永久地址永久地址 http://http://www.marfan.org.twwww.marfan.org.tw/....../......cleidcleid=a_20061102_005118=a_20061102_005118

6969

Vlad Tepes Dracula (1431-1476)Vlad Tepes Dracula (1431-1476)

7070

III. Irish GothicIII. Irish Gothic

7171

7272

Anglo-Irish Ascendancy: Four Phases Anglo-Irish Ascendancy: Four Phases (1)(1)

First Settlement (1603)First Settlement (1603)

Old (Gaelic) IrishOld (Gaelic) IrishOld EnglishOld English

vs.vs.

Anglo-Irish/Anglican (vs. Catholics, Anglo-Irish/Anglican (vs. Catholics, Presbyterians and Dissenters)Presbyterians and Dissenters)

7373

Irish Rebellion and Re-conquests (1652-Irish Rebellion and Re-conquests (1652-60)60)

Execution of rebels and Catholics after 1641 Execution of rebels and Catholics after 1641 Irish uprising and settlement to the Irish uprising and settlement to the CaribbeanCaribbean

Proposed dispossessing of land originally Proposed dispossessing of land originally owned by the Catholics to make way for the owned by the Catholics to make way for the adventurers/settlers (notably from Scotland)adventurers/settlers (notably from Scotland)

7474

Anglo-Irish Ascendancy: Four Phases Anglo-Irish Ascendancy: Four Phases (2)(2)

Ascendancy (Anglo-Irish/Anglican)Ascendancy (Anglo-Irish/Anglican)Irish rising and the Williamite War (1689-169Irish rising and the Williamite War (1689-1691)1)Penal Laws against the Catholics and the PrePenal Laws against the Catholics and the PresbyterianssbyteriansBy mid-18th century, 95% of land in the ProtBy mid-18th century, 95% of land in the Protestants’ ownershipestants’ ownership

7575

1798 rising (Emmet)1798 rising (Emmet)Act of Union 1801Act of Union 1801

7676

Anglo-Irish Ascendancy: Four Phases Anglo-Irish Ascendancy: Four Phases (3)(3)

ContractionContraction

Absentee landlordsAbsentee landlords

Catholic Emancipations (1829)Catholic Emancipations (1829)

Potato Famine (1845-52)Potato Famine (1845-52)

Food shipped to EnglandFood shipped to EnglandRestrictions on relief fundsRestrictions on relief funds

7777

Anglo-Irish Ascendancy: Four Phases Anglo-Irish Ascendancy: Four Phases (4)(4)

SiegeSiege

Home Rule (to grant autonomy to the territory)Home Rule (to grant autonomy to the territory)

legislation aimed at ending exploiting the Irish since legislation aimed at ending exploiting the Irish since the Act of Unionthe Act of Union

Gladstone’s unsuccessful attempts to pass a Home Gladstone’s unsuccessful attempts to pass a Home Rule bill in 1886, 1893Rule bill in 1886, 1893

revived in 1912 but interrupted by WW1revived in 1912 but interrupted by WW1

Irish War of Independence (1919-1922)Irish War of Independence (1919-1922)

7878

Irish Gothic 1Irish Gothic 1Late 18th century:Late 18th century:

Regina Maria Roche, Regina Maria Roche, Children of the AbbeyChildren of the Abbey (1 (1796)796)Mrs. Kelly, Mrs. Kelly, Ruins of Avondale Priory Ruins of Avondale Priory (1796)(1796)Mrs. F.C. Patrick, Mrs. F.C. Patrick, The Irish HeiressThe Irish Heiress (1797) (1797)‘‘the Wife of an Officer,” the Wife of an Officer,” Most Ghosts Most Ghosts (1798)(1798)Mrs. Colpoy, Mrs. Colpoy, The Irish Excursion The Irish Excursion (1801)(1801)

7979

Irish Gothic 2Irish Gothic 2Charles Robert Maturin* (1780-1824): Charles Robert Maturin* (1780-1824):

Melmoth the Wanderer Melmoth the Wanderer (1820) (1820)insanity as a symptom/trope of divided loyainsanity as a symptom/trope of divided loyalty during the civil warlty during the civil war

8080

*Huguenots*HuguenotsFrench Protestants emigrating to EnglanFrench Protestants emigrating to England and Ireland in the late 17th centuryd and Ireland in the late 17th century

1598: Edict of Nantes1598: Edict of Nantes1572: Massacre of St. Barthelomew1572: Massacre of St. Barthelomew1629: Revocation of Edict1629: Revocation of Edict1685: 400,000 emigrated to England, Nether1685: 400,000 emigrated to England, Netherlands, Germanylands, Germany

8181

Irish Gothic 3Irish Gothic 3Joseph Sheridan LeFanu (1814-1873): Joseph Sheridan LeFanu (1814-1873): CaCarmilla rmilla (1872)(1872)

Vampire Mircalla comes back after 2 hundreVampire Mircalla comes back after 2 hundred years and disguise herself as Carmilla (Laud years and disguise herself as Carmilla (Laura’s roommate) and Millarca (the Generalra’s roommate) and Millarca (the General’s niece) (anagrams)’s niece) (anagrams)A past that refuses to be put to rest returns tA past that refuses to be put to rest returns to haunt modern peopleo haunt modern people

8282

Illustration of Carmilla from Illustration of Carmilla from The Dark BlueThe Dark Blue by D. H. Friston, 1872 by D. H. Friston, 1872 http://www.lacrypte.net/images/carmilla1.jhttp://www.lacrypte.net/images/carmilla1.jpgpg

8383

Irish Gothic 4Irish Gothic 4Bram Stoker (1847-1912)Bram Stoker (1847-1912)

Dracula Dracula (1897)(1897)

8484

Irish Gothic 5Irish Gothic 5W.B. Yeats, John Millington Synge, ElizaW.B. Yeats, John Millington Synge, Elizabeth Bowenbeth Bowen

8585

ReferencesReferences Killeen, Jarlath. “Irish Gothic: A Theoretical IKilleen, Jarlath. “Irish Gothic: A Theoretical Introduction.” ntroduction.” The Irish Journal of Gothic and The Irish Journal of Gothic and Horror Studies.Horror Studies. http://irishgothichorrorjournal.homestead.cohttp://irishgothichorrorjournal.homestead.com/jarlathprinter.htmlm/jarlathprinter.html McCormack, W.J. “Irish Gothic and After.” McCormack, W.J. “Irish Gothic and After.” TThe Field Day Anthology of Irish Writing. he Field Day Anthology of Irish Writing. Eds. SeEds. Seamus Deane (General Editor), Andrew Carpentamus Deane (General Editor), Andrew Carpenter and Jonathan Williams. Derry: Field Day Puer and Jonathan Williams. Derry: Field Day Publications, 1991. Vol. II, 831-854.blications, 1991. Vol. II, 831-854.

8686

Miles, Robert. “Ann Radcliffe and MatthMiles, Robert. “Ann Radcliffe and Matthew Lewis.” ew Lewis.” A Companion to the Gothic. A Companion to the Gothic. Ed. David Punter. Oxford: Blackwell, 200Ed. David Punter. Oxford: Blackwell, 2001. 41-57.1. 41-57.Sage, Victor. “Irish Gothic: C.R. Maturin Sage, Victor. “Irish Gothic: C.R. Maturin and J.S. LeFanu.” and J.S. LeFanu.” A Companion to the A Companion to the Gothic. Gothic. Ed. David Punter. Oxford: Black Ed. David Punter. Oxford: Blackwell. 81-93.well. 81-93.

8787

IV. Appendix: IV. Appendix: Nationalist GothicismNationalist Gothicism

8888

The Terrific Register; or, Record of The Terrific Register; or, Record of Crimes, Judgments, Providences and Crimes, Judgments, Providences and CalamitiesCalamities, 2 vols. (London: , 2 vols. (London: Sherwood, Jones and Co., 1825).Sherwood, Jones and Co., 1825).

8989

The Terrific Register 2:17 The Terrific Register 2:17 (The Midnight Assassination)(The Midnight Assassination)

9090

Robert Emmet (1778-1803)Robert Emmet (1778-1803)

9191

Unconscious act as a result of some supUnconscious act as a result of some supernatural visitations connected with natiernatural visitations connected with nationalismonalismCf. “Cathleen Ni Houlihan” (1902) by YCf. “Cathleen Ni Houlihan” (1902) by Yeats about 1798 Irish Rebellioneats about 1798 Irish Rebellion

9292

9393

Maud Gonne (1866-1953)Maud Gonne (1866-1953)

The EndThe End