anomalies and curiosities of medicine-第201部分

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Hersman　reports　an　interesting　case　of　progressive　enlargement　of　the　hands　in　a　clergyman　of　fifty。　Since　youth　he　had　suffered　with　pains　in　the　joints。　About　three　years　before　the　time　of　report　he　noticed　enlargement　of　the　phalangeal　joint　of　the　third　finger　of　the　right　hand。　A　short　time　later　the　whole　hand　became　gradually　involved　and　the　skin　assumed　a　darker　hue。　Sensation　and　temperature　remained　normal　in　both　hands；　acromegaly　was　excluded　on　account　of　the　absence　of　similar　changes　elsewhere。　Hersman　remarks　that　the　change　was　probably　due　to　increase　in　growth　of　the　fibrous　elements　of　the　subcutaneous　lesions　about　the　tendons；　caused　by　rheumatic　poison。　Figure　283　shows　the　palmer　and　dorsal　surfaces　of　both　hands。

Chiromegaly　is　a　term　that　has　been　applied　by　Charcot　and　Brissaud　to　the　pseudoacromegaly　that　sometimes　occurs　in　syringomyelia。　Most　of　the　cases　that　have　been　reported　as　a　combination　of　these　two　diseases　are　now　thought　to　be　only　a　syringomyelia。　A　recent　case　is　reported　by　Marie。　In　this　connection　it　is　interesting　to　notice　a　case　of　what　might　be　called　acute　symptomatic　transitory　pseudoacromegaly；　reported　by　Potovski：　In　an　insane　woman；　and　without　ascertainable　cause；　there　appeared　an　enlargement　of　the　ankles；　wrists；　and　shoulders；　and　later　of　the　muscles；　with　superficial　trophic　disturbances　that　gradually　disappeared。　The　author　excludes　syphilis；　tuberculosis；　rheumatism；　gout；　hemophilia；　etc。；　and　considers　it　to　have　been　a　trophic　affection　of　cerebral　origin。　Cases　of　pneumonia　osteoarthropathy　simulating　acromegaly　have　been　reported　by　Korn　and　Murray。　

Megalocephaly；　or　as　it　was　called　by　Virchow；　leontiasis　ossea；　is　due　to　a　hypertrophic　process　in　the　bones　of　the　cranium。　The　cases　studied　by　Virchow　were　diffuse　hyperostoses　of　the　cranium。　Starr　describes　what　he　supposes　to　be　a　case　of　this　disease；　and　proposes　the　title　megalocephaly　as　preferable　to　Virchow's　term；　because　the　soft　parts　are　also　included　in　the　hypertrophic　process。　A　woman　of　fifty…two；　married　but　having　no　children；　and　of　negative　family　history；　six　years　before　the　time　of　report　showed　the　first　symptoms　of　the　affection；　which　began　with　formication　in　the　finger…tips。　This　gradually　extended　to　the　shoulders；　and　was　attended　with　some　uncertainty　of　tactile　sense　and　clumsiness　of　movement；　but　actual　anesthesia　had　never　been　demonstrated。　This　numbness　had　not　invaded　the　trunk　or　lower　extremities；　although　there　was　slight　uncertainty　in　the　gait。　There　had　been　a　slowly　progressing　enlargement　of　the　head；　face；　and　neck；　affecting　the　bone；　skin；　and　subcutaneous　tissues；　the　first　to　the　greatest　degree。　The　circumference　of　the　neck　was　16　inches；　the　horizontal　circumference　of　the　head　was　24　inches；　from　ear　to　ear；　over　the　vertex；　16　inches；　and　from　the　root　of　the　nose　to　the　occipital　protuberance；　16　inches。　The　cervical　vertebrae　were　involved；　and　the　woman　had　lost　five　inches　in　height。　It　may　be　mentioned　here　that　Brissaud　and　Meige　noticed　the　same　loss　in　height；　only　more　pronounced；　in　a　case　of　gigantism；　the　loss　being　more　than　15　inches。　In　Starr's　case　the　tongue　was　normal　and　there　was　no　swelling　of　the　thyroid。

Cretinism　is　an　endemic　disease　among　mountainous　people　who　drink　largely　of　lime　water；　and　is　characterized　by　a　condition　of　physical；　physiologic；　and　mental　degeneracy　and　nondevelopment；　and　possibly　goiter。　The　subjects　of　this　disease　seldom　reach　five　feet　in　height；　and　usually　not　more　than　four。　The　word　cretin　is　derived　from　the　Latin　creatura。　They　are　found　all　over　the　world。　In　Switzerland　it　is　estimated　that　in　some　cantons　there　is　one　cretin　to　every　25　inhabitants。　In　Styria；　the　Tyrol；　and　along　the　Rhine　cretins　are　quite　common；　and　not　long　since　cases　existed　in　Derbyshire。　These　creatures　have　been　allowed　to　marry　and　generate；　and　thus　extend　their　species。　In　〃Le　Medicin　de　Campagne；〃　Balzac　has　given　a　vivid　picture　of　the　awe　and　respect　in　which　they　were　held　and　the　way　in　which　they　were　allowed　to　propagate。　Speaking　of　the　endemic　cretins；　Beaupre　says：　〃I　see　a　head　of　unusual　form　and　size；　a　squat　and　bloated　figure；　a　stupid　look；　bleared；　hollow；　and　heavy　eyes；　thick；　projecting　eyelids；　and　a　flat　nose。　His　face　is　of　a　leaden　hue；　his　skin　dirty；　flabby；　covered　with　tetters；　and　his　thick　tongue　hangs　down　over　his　moist；　livid　lips；　his　mouth；　always　open　and　full　of　saliva；　shows　teeth　going　to　decay。　His　chest　is　narrow；　his　back　curved；　his　breath　asthmatic；　his　limbs　short；　misshapen；　without　power。　The　knees　are　thick　and　inclined　inward；　the　feet　flat。　The　large　head　droops　listlessly　on　the　breast；　the　abdomen　is　like　a　bag。〃　The　cretin　is　generally　deaf　and　dumb；　or　only　able　to　give　a　hoarse　cry。　He　is　indifferent　to　heat　and　cold；　and　even　to　the　most　revolting　odors。　The　general　opinion　has　always　been　that　the　sexual　desire　and　genital　organs　are　fully　developed。

A　quotation　under　our　observation　credits　Colonel　Sykes　with　the　following　statistics　of　cretinism；　which　show　how　in　some　locations　it　may　be　a　decided　factor　of　population。　In　December；　1845；　in　a　population　of　2；558；349　souls　（the　locality　not　mentioned）；　there　were　18；462　people　with　simple　goiter。　Of　the　cretins　without　goiter　there　were　2089。　Of　cretins　with　goiter　there　were　3909；　and　cretins　in　which　goiter　was　not　stated　962；　making　a　total　of　6960。　Of　these　2185　had　mere　animal　instincts；　3531　possessed　very　small　intellectual　faculties；　196　were　almost　without　any；　1048　not　classified。　Of　this　number　2483　were　born　of　healthy　and　sane　fathers；　2285　from　healthy　mothers；　961　from　goitrous　fathers；　1267　from　goitrous　mothers；　49　from　cretin　fathers；　41　from　cretin　mothers；　106　from　cretin　fathers　with　goiter；　66　from　cretin　mothers　with　goiter；　438　fathers　and　405　mothers　were　not　specified。

Sporadic　cretinism；　or　congenital　myzedema；　is　characterized　by　a　congenital　absence　of　the　thyroid；　diminutiveness　of　size；　thickness　of　neck；　shortness　of　arms　and　legs；　prominence　of　the　abdomen；　large　size　of　the　face；　thickness　of　the　lips；　large　and　protruding　tongue；　and　imbecility　or　idiocy。　It　is　popularly　believed　that　coitus　during　intoxication　is　the　cause　of　this　condition。　Osler　was　able　to　collect　11　or　12　cases　in　this　country。　The　diagnosis　is　all…important；　as　the　treatment　by　the　thyroid　extract　produces　the　most　noteworthy　results。　There　are　several　remarkable　recoveries　on　record；　but　possibly　the　most　wonderful　is　the　case　of　J。　P。　West　of　Bellaire；　Ohio；　the　portraits　of　which　are　reproduced　in　Plate　11。　At　seventeen　months　the　child　presented　the　typical　appearance　of　a　sporadic　cretin。　The　astonishing　results　of　six　months'　treatment　with　thyroid　extract　are　shown　in　the　second　figure。　After　a　year's　treatment　the　child　presents　the　appearance　of　a　healthy　and　well…nourished　little　girl。

Myxedema　proper　is　a　constitutional　condition　due　to　the　loss　of　the　function　of　the　thyroid　gland。　The　disease　was　first　described　by　Sir　William　Gull　as　a　cretinoid　change；　and　later　by　William　Ord　of　London；　who　suggested　the　name。　It　is　characterized　clinically　by　a　myxedematous　condition　of　the　subcutaneous　tissues　and　mental　failure；　and　anatomically　by　atrophy　of　the　thyroid　gland。　The　symptoms　of　myxedema；　as　given　by　Ord；　are　marked　increase　in　the　general　bulk　of　the　body；　a　firm；　inelastic　swelling　of　the　skin；　which　does　not　pit　on　pressure；　dryness　and　roughness　which　tend；　with　swelling；　to　obliterate　the　lines　of　expression　in　the　face；　imperfect　nutrition　of　the　hair；　local　tumefaction　of　the　skin　and　subcutaneous　tissues；　particularly　in　the　supraclavicular　region。　The　physiognomy　is　remarkably　altered；　the　features　are　coarse　and　broad；　the　lips　thick；　the　nostrils　broad　and　thick；　and　the　mouth　enlarged。　There　is　a　striking　slowness　of　thought　and　of　movement；　the　memory　fails；　and　conditions　leading　to　incipient　dementia　intervene。　The　functions　of　the　thoracic　and　abdominal　organs　seem　to　be　normal；　and　death　is　generally　due　to　some　intercurrent　disease；　possibly　tuberculosis。　A　condition　akin　to　myxedema　occurs　after　operative　removal　of　the　thyroid　gland。

In　a　most　interesting　lecture　Brissaud　shows　the　intimate　relation　between　myxedema；　endemic　cretinism；　sporadic　cretinism；　or　myxedematous　idiocy；　and　infantilism。　He　considers　that　they　are　all　dependent　upon　an　inherited　or　acquired　deficiency　or　disease　of　the　thyroid　gland；　and　he　presents　cases　illustrating　each　affection。　Figure　285　shows　a　case　of　myxedema；　one　of　myxedema　in　a　case　of　arrested　developmenta　transition　case　between　myxedema　of　the　adult　and　sporadic　cretinismand　a　typical　case　of　sporadic　cretinism。

Cagots　are　an　outcast　race　or　clan　of　dwarfs　in　the　region　of　the　Pyrenees；　and　formerly　in　Brittany；　whose　existence　has　been　a　scientific　problem　since　the　sixteenth　century；　at　which　period　they　were　known　as　Cagots；　Gahets；　Gafets；　Agotacs；　in　France；　Agotes　or　Gafos；　in　Spain；　and　Cacous；　in　Lower　Brittany。　Cagot　meant　the　dog　of　a　Goth；　they　were　of　supposed　Gothic　origin　by　some；　and　of　Tartar　origin　by　others。　These　people　were　formerly　supposed　to　have　been　the　descendants　of　lepers；　or　to　have　been　the　victims　of　leprosy　themselves。　From　the　descriptions　there　is　a　decided　difference　between　the　Cagots　and　the　cretins。　In　a　recent　issue　of　Cosmos　a　writer　describes　Cagots　as　follows：

〃They　inhabit　the　valley　of　the　Ribas　in　the　northwestern　part　of　the　Spanish　province　now　called　Gerona。　They　never　exceed　51　1/2　inches　in　height；　and　have　short；　ill…formed　legs；　great　bellies；　small　eyes；　flat　noses；　and　pale；　unwholesome　complexions。　They　are　usually　stupid；　often　to　the　verge　of　idiocy；　and　much　subject　to　goiter　and　scrofulous　affections。　The　chief　town　of　the　Ribas　Valley　is　Ribas；　a　place　of　1500　inhabitants。　about　800　feet　above　sea…level。　The　mountains　rise　about　the　town　to　a　height　of　from　6000　to　8000　feet；　and　command　an　amazingly　beautiful　panorama　of　mountain；　plain；　and　river；　with　Spanish　cities　visible　upon　the　one　side　and　French　upon　the　other。　The　region　is　rich；　both　agriculturally　and　minerally；　and　is　famous　for　its　medicinal　springs。　In　this　paradise　dwell　the　d