08/7,3/(6&/(526,6and DEMYELINATING DISEASES Course # 2031 &RQWDFW+RXUV Author: 3DWULFLD0DU[51%6106 ([FHUSWVSUHSDUHGE\6KHOGD/.+XGVRQ51%613+1 Course Material Valid through: 10/2014 G: Copyright © 2011 W.S. Keefer All rights reserved Published by the National Center of Continuing Education, Inc., Lakeway, Texas. Printed in the United States of America. IN UR AT FE E L S © Enhanced Learning & Skills No Exams, Just Learning! 7HVWLQJ0DQGDWRU\)RU(OHFWURORJLVWV)ORULGD2QO\ Visit www.bbb.org A NATIONAL EPIDEMIC WE ALL KNOW . . . . . . that U.S. Copyright Law grants to the copyright owner the exclusive right to duplicate copyrighted, printed and recorded materials. Piracy involves the illegal duplication of copyrighted materials. YOU MAY NOT KNOW . . . . . . that every time you use or make an illegal copy of any printed material in any form or by any method you may be subject to litigation. WKDW\RXULQVWLWXWLRQ·VGXSOLFDWLRQRUSURFHVVLQJHTXLSPHQWPD\DOVREHFRQÀVFDWHGDQGGHVWUR\HGLI involved in illegal duplication. WKDWWKHSHQDOW\IRUFULPLQDOYLRODWLRQLVXSWRÀYH\HDUVLQSULVRQDQGRUDÀQHXQGHUDWRXJK new law. (Title 17, U.S. Code, Section 506, and Title 18, U.S. Code Section 2319). . . . that civil or criminal litigation may be costly and embarrassing to any organization or individual. We UHTXHVW\RXFRQWDFWXVLPPHGLDWHO\UHJDUGLQJLOOHJDOGXSOLFDWLRQRIWKHVHFRS\ULJKWHGSULQWHGPDWHULDOV 7KH1DWLRQDO&HQWHURI&RQWLQXLQJ(GXFDWLRQZLOOSD\Dsubstantial rewardIRULQIRUPDWLRQOHDGLQJWRWKH FRQYLFWLRQRIDQ\LQGLYLGXDORULQVWLWXWLRQPDNLQJDQ\XQDXWKRUL]HGGXSOLFDWLRQRIPDWHULDOFRS\ULJKWHG E\:6.HHIHURU7KH1DWLRQDO&HQWHURI&RQWLQXLQJ(GXFDWLRQ,QF ©National Center of Continuing Education, Inc. Multiple Sclerosis Page 1 TABLE OF CONTENTS About the Author ................................................................................................................. 3 Purpose and Goals ............................................................................................................... 3 Instructional Objectives....................................................................................................... 3 The History of Multiple Sclerosis ........................................................................................ 3 An Introduction to the Demyelinating Diseases ................................................................ 3 6SHFLÀFVRQ0XOWLSOH6FOHURVLV ........................................................................................... 3 Potential Contributing Factors ........................................................................................... 4 Types of MS .......................................................................................................................... 4 Pathophysiology of MS ........................................................................................................ 4 Symptomatology of MS ........................................................................................................ 5 Figure 1: Initial Symptoms ................................................................................................. 5 Pain and MS ......................................................................................................................... 6 Figure 2: Symptom Chart .................................................................................................... 6 Cognitive Changes in MS .................................................................................................... 7 Paroxysmal Symptoms ........................................................................................................ 7 Optic Neuritis and Visual Problems ................................................................................... 7 GI Disturbances .................................................................................................................. 7 Bladder Problems ...................................................................................................... 7 Constipation and Bowel Problems ........................................................................... 8 Sexual Dysfunction .............................................................................................................. 8 Diagnosis of MS.................................................................................................................... 8 Treatment and Pharmacology ............................................................................................. 8 Complementary and Alternative Therapies ....................................................................... 9 Nutritional Approaches to MS.................................................................................. 9 9LWDPLQ''HÀFLHQF\ .............................................................................................. 10 Supplement Safety Caveats.................................................................................... 10 Psychosocial Factors in the MS Patient ........................................................................... 11 Adrenoleukodystrophy (ALD)............................................................................................ 11 The Myelin Project ............................................................................................................. 12 Demyelinating Diseases of the Peripheral Nervous System ................................................................................................................. 12 Amyotrophic Lateral Sclerosis .......................................................................................... 12 Conclusion .......................................................................................................................... 13 Suggested Readings ........................................................................................................... 13 ( [WUDRUGLQDU\HIIRUWVKDYHEHHQPDGHE\WKHDXWKRUVWKHHGLWRUDQGWKHSXEOLVKHURIWKH1DWLRQDO&HQWHU RI&RQWLQXLQJ(GXFDWLRQ,QFFRXUVHVWRHQVXUHGRVDJHUHFRPPHQGDWLRQVDQGWUHDWPHQWVDUHSUHFLVHDQG DJUHHZLWKWKHKLJKHVWVWDQGDUGVRISUDFWLFH+RZHYHUDVDUHVXOWRIDFFXPXODWLQJFOLQLFDOH[SHULHQFHDQG FRQWLQXLQJODERUDWRU\VWXGLHVGRVDJHVFKHGXOHVDQGRUWUHDWPHQWUHFRPPHQGDWLRQVDUHRIWHQDOWHUHGRUGLV FRQWLQXHG,QDOOFDVHVWKHDGYLFHRIDSK\VLFLDQVKRXOGEHVRXJKWDQGIROORZHGFRQFHUQLQJLQLWLDWLQJRUGLVFRQWLQXLQJ DOOPHGLFDWLRQVRUWUHDWPHQWV7KHSODQQHUVDXWKRUVDQGRUHGLWRUVRIHDFKFRXUVHKDYHDWWHVWHGWRQRFRQÁLFW 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We Want You To Learn! Page 2 Multiple Sclerosis © © National Center of Continuing Education, Inc. About the Author 3DWULFLD0DU[51%6106KDVZRUNHG LQQXUVLQJVLQFHZLWKDFHUWLÀFDWLRQLQ neuroscience nursing, oncology, and hospice and palliative care. She has been involved LQPDQ\GLIIHUHQWKHDOWKFDUHVHWWLQJVIURP a busy oncology unit to hospice care. She was diagnosed with multiple sclerosis in DQGZULWHVWKLVFRXUVHIURPDSURIHV sional and personal level. She has struggled IRUPDQ\\HDUVZLWKUHODSVLQJUHPLWWLQJ06 that has now become secondary progressive. She welcomes any questions, comments, or QHWZRUNLQJSDUWQHUVLQWKHHQGHDYRURIPDN LQJOLIHHDVLHUIRUWKRVHFRXUDJHRXVSDWLHQWV with this disease. 0V 0DU[ WHDFKHV QXUVLQJ DW 3HQQ 6WDWH DQGLVD3K'VWXGHQWLQQXUVLQJ6KHKDVEHHQ DZDUGHGDJUDQWIURPWKH+DUWIRUG)RXQGD WLRQIRU([FHOOHQFHLQ*HULDWULF1XUVLQJ6KH KDVDPDVWHU·VGHJUHHLQSV\FKRORJ\IURP7KH 6WDWH8QLYHUVLW\RI1HZ<RUNDW%LQJKDPWRQ ZKHUHKHUSULPDU\IRFXVZDVRQUHVHDUFKRI neurological diseases. ([FHUSWV SUHSDUHG E\ 6KHOGD +XGVRQ 51 %61 3+1 'LUHFWRU RI +HDOWKFDUH ,QIRUPDWLRQ0V+XGVRQLVUHVSRQVLEOHIRU GLUHFWLQJ WKH DFWLYLWLHV RI WKLV GHSDUWPHQW VHOHFWLQJ TXDOLILHG FUHGHQWLDOHG DXWKRUV IRU WKH FRXUVHV RIIHUHG E\ WKH 1DWLRQDO &HQWHUDVZHOODVDGYLVLQJVWDIIRIUHTXLUHG FRXUVH GHVLJQ DQG FULWHULD 0V +XGVRQ KDVRYHU\HDUVRIH[WHQVLYHH[SHULHQFH Purpose and Goals 7KHJRDORIWKLVFRXUVHPDWHULDOLVWRHGX FDWHQXUVHVDQGRWKHUKHDOWKFDUHSURIHVVLRQDOV LQ WKH QDWXUH DQG PDQDJHPHQW RI 0XOWLSOH Sclerosis and demyelinating diseases. Top LFV LQFOXGH W\SHV RI 06 SDWKRSK\VLRORJ\ V\PSWRPDWRORJ\SDLQ06DOWHUQDWLYHWUHDW ments and more. Instructional Objectives 1. 5HFDOO WKH SDWKRSK\VLRORJ\ RI WKH demyelinating disorders. 2. 2XWOLQH WKH UROH RI P\HOLQ LQ WKH WUDQVPLVVLRQRIQHXUDOVLJQDOVZLWKLQWKH central nervous system. 3. List the demographics and peculiar JHRJUDSKLFDO GLVWULEXWLRQ RI PXOWLSOH sclerosis. 4. 1DPHWKHFKDUDFWHULVWLFW\SHVRIPXOWLSOH sclerosis. 5. 'HVFULEH WKH SULPDU\ V\PSWRPV RI multiple sclerosis. 6. ,GHQWLI\WKHIRXUW\SHVRISDLQFRPPRQWR multiple sclerosis. 7. Recognize the potential cognitive changes that may occur in individuals with multiple sclerosis. 8. ,GHQWLI\SDUR[\VPDOV\PSWRPVRIPXOWLSOH sclerosis. 9. 6WDWHWKHPDMRUGUXJVXVHGWRWUHDWPXOWLSOH sclerosis. 10. /LVW W\SHV RI FRPSOLPHQWDU\ DQG DOWHUQDWLYHWKHUDSLHVXVHGIRUSHRSOHZLWK multiple sclerosis. 0XOWLSOHVFOHURVLVLVDFKURQLFDXWRLPPXQH GLVRUGHUWKDWFDQDIIHFWPRYHPHQWVHQVDWLRQ DQGPDQ\ERGLO\IXQFWLRQV,WLVFDXVHGE\ WKHGHVWUXFWLRQRIWKHFRDWLQJRIP\HOLQWKDW VXUURXQGVWKHQHUYHVRIWKHFHQWUDOQHUYRXV system. This myelin helps the neurons to WUDQVPLW VLJQDOV IURP WKH EUDLQ DQG VSLQDO FRUGWRWKHUHVWRIWKHERG\:KHQLWLVGH stroyed, signals are transmitted erratically or not transmitted at all. Scar tissue, called plaque, is deposited over the neuron causing an even more severe barrier to communica tion. 0XOWLSOHVFOHURVLVKDVDYHU\EURDGUDQJH RIV\PSWRPVWKDWFDQDIIHFWQHDUO\HYHU\V\V tem in the body, including vision, sensation, FRRUGLQDWLRQDQGERGLO\IXQFWLRQVWKDWPRVW RIXVWDNHIRUJUDQWHG The History of Multiple Sclerosis ,W DSSHDUV WKDW WKH HDUOLHVW GHVFULSWLRQ RI PXOWLSOH VFOHURVLV FRPHV IURP WKH ODWH IRXUWHHQWKFHQWXU\$WWKHDJHRID\RXQJ ZRPDQQDPHG/XGZLQDZKROLYHGLQ+ROODQG IHOOZKLOHVNDWLQJEHFDXVHRIZHDNQHVVLQKHU legs. She recovered strength in her legs but DIWHU WKDW KDG UHFXUUHQW HSLVRGHV RI ORVV RI EDODQFHZHDNQHVVDQGYLVXDOSUREOHPV7KLV progressed over decades until she was unable WRZDON9HU\VSHFLÀFUHFRUGVZHUHNHSWE\ WKH9DWLFDQRQWKLVZRPDQEHFDXVHVKHODWHU was declared to be a saint. 7KUHHKXQGUHG\HDUVODWHULQ(QJODQGDQ LOOHJLWLPDWH JUDQGVRQ RI *HRUJH ,,, NHSW D GLDU\IRU\HDUVLQZKLFKKHUHFRUGHGWKH SURJUHVVLRQRIKLVV\PSWRPVZKLFKZHQRZ EHOLHYHWREHWKRVHRIPXOWLSOHVFOHURVLV ,Q D )UHQFK QHXURORJLVW QDPHG -HDQ0DUWLQ &KDUFRW SURYLGHG D GHÀQLWLYH GHVFULSWLRQRIZKDWZHQRZNQRZDVPXOWLSOH VFOHURVLV 06 :KHQ KH GLG DXWRSVLHV RQ LQGLYLGXDOVZLWKWKLVGLVRUGHUKHIRXQGWKH SUHVHQFHRI´SODTXHµLQWKHEUDLQDQGVSLQDO FRUG +H ZDV D PHWLFXORXV DUWLVW DQG SXE OLVKHGKLVGUDZLQJVRIWKHVHOHVLRQV )URP WKDW WLPH XQWLO QRZ VFLHQWLVWV DQG ©National Center of Continuing Education, Inc. physicians have been trying to better understand this disease in order to help SDWLHQWVZKRVXIIHUIURPWKLVGHELOLWDW LQJLOOQHVV<HWWKHUHLVVWLOOVSHFXODWLRQ DERXWWKHFDXVHRIWKHGLVHDVH1HZGL agnostic tools and new drugs have been GHYHORSHG\HWWKHFDXVHRI06UHPDLQV a mystery. An Introduction to Demyelinating Diseases 'HP\HOLQDWLQJGLVHDVHVDUHDFDWHJRU\ RISDWKRORJLHVWKDWDIIHFWWKHZKLWHPDW WHURIWKHFHQWUDOQHUYRXVV\VWHP7KLV white matter is called “myelin” and it is a protective white coating that surrounds the nerves in the brain and spinal cord. 7KLVDOORZVDVPRRWKSDVVDJHRIHOHFWUL cal discharges (action potentials) within the nervous system. Just as wires in an electrical system require an “insulator” VR WKDW HOHFWULFLW\ FDQ ÁRZ VPRRWKO\ our central nervous system requires this coating too, so that electrical signals do QRWMXPSIURPRQHSODFHWRDQRWKHUDQG DUHDEOHWRUHDFKWKHLUÀQDOGHVWLQDWLRQ 0RVWRIWKHV\PSWRPVRIGHP\HOLQDW LQJ GLVHDVHV RFFXU EHFDXVH RI WKH GH VWUXFWLRQRIWKHP\HOLQ$VWKHP\HOLQLV stripped away, scar tissue, called plaque, IRUPVZKHUHWKHP\HOLQRULJLQDOO\KDG been. The scar tissue is not conductive DQG LPSHGHV WKH VPRRWKWUDYHOOLQJ RI WKHVLJQDOVIURPWKHEUDLQWRWKHLUÀQDO destination. ,QWKLVFRXUVHZHZLOOH[DPLQHWKUHH PDMRUGHP\HOLQDWLQJGLVHDVHVPXOWLSOH sclerosis, amyotrophic lateral sclerosis DOVR FDOOHG ´/RX *HKULJ·V 'LVHDVHµ DQG DGUHQROHXNRG\VWURSK\ $/' 7KH\ KDYH PDQ\ IDFWRUV LQ FRPPRQ but we will discuss them separately in WKHFRXUVHEHFDXVHWKH\KDYHDGLIIHU HQWGLDJQRVWLFSHUVSHFWLYHDQGGLIIHUHQW GUXJVDUHXVHGIRUWUHDWPHQW 6SHFLÀFVRQ0XOWLSOH Sclerosis 0XOWLSOHVFOHURVLVLVDPDMRUFDXVHRI chronic disability in young adults with WKHRQVHWRIWKHLOOQHVVRFFXUULQJEHWZHHQ WKHDJHVRIDQG\HDUVROG2QVHW RI WKH GLVHDVH LV UDUH EHIRUH SXEHUW\ UHDFKHVDSHDNDWDJHDQGDIWHUWKH DJHRIWKHRQVHWRIPXOWLSOHVFOHURVLV is negligible. 7KHGLVHDVHDIIHFWVPRUHZRPHQWKDQ Multiple Sclerosis Page 3 PHQDWDUDWLRRI)LYHWLPHVDVPDQ\ &DXFDVLDQVKDYHWKHGLVHDVHWKDQ%ODFNV 7KHGLVHDVHDIÁLFWVSHRSOHLQWKH United States. Approximately 200 people DUHQHZO\GLDJQRVHHDFKZHHN+RZHYHU DFFRUGLQJWRDUHFHQWUHSRUWLQWKHMRXUQDO ´1HXURORJ\µ WKH LQFLGHQFH RI PXOWLSOH VFOHURVLV LV JURVVO\ XQGHUUHSRUWHG 7KH MRXUQDO FULWLFL]HV WKH &HQWHU IRU 'LVHDVH &RQWUROIRUUHSRUWLQJVXFKDORZQXPEHU Autopsy reports alone show that thousands RIFDVHVRI06JRXQGLDJQRVHG$PRUH reasonable estimate is that there are over 600,000 people in the United States with PXOWLSOHVFOHURVLV,QWKLVVDPHMRXUQDOWKH opinion is also voiced that the incidence RI PXOWLSOH VFOHURVLV KDV VN\URFNHWHG LQ WKHSDVWÀIW\\HDUVDQGPRUHDWWHQWLRQDQG UHVRXUFHVDUHQHHGHGWRÀQGWKHFDXVHRI WKLVGLVHDVH06LVDYHU\ÀQDQFLDOO\DQG emotionally draining chronic illness. Potential Contributing Factors 7KHUH LV D VWULNLQJ GLIIHUHQFH LQ WKH QXPEHURIFDVHVRIPXOWLSOHVFOHURVLVDF cording to geographic distribution. This is ZHOONQRZQDQGKDVEHHQWKRURXJKO\UH VHDUFKHG7KHLQFLGHQFHRI06LQFRXQWULHV QRUWK RI WKH GHJUHH SDUDOOHO ODWLWXGH LQFOXGLQJWKHQRUWKHUQSDUWRIWKH8QLWHG 6WDWHV &DQDGD DQG 1RUWKHUQ (XURSH is extremely high. Scientists speculate that perhaps a climatic variable, such as WHPSHUDWXUH RU WKH DPRXQW RI VXQOLJKW D SDUWLFXODUDUHDUHFHLYHVHDFKGD\LQÁXHQFHV WKH GHYHORSPHQW RI PXOWLSOH VFOHURVLV Others believe that a particular virus may DIIHFWSHRSOHOLYLQJLQWHPSHUDWHFOLPDWHV EXWWKLVYLUXVGRHVQRWÀQGWURSLFDOFOLPDWHV hospitable. *HQHV PD\ SOD\ D UROH LQ FUHDWLQJ D susceptibility to multiple sclerosis. Certain SRSXODWLRQV KDYH D KLJKHU LQFLGHQFH RI DXWRLPPXQHGLVHDVH,WLVFOHDUWKDW06LV an autoimmune disease. The body’s own GHIHQVHPHFKDQLVPVIRXQGLQWKH7FHOOV EHJLQWRDWWDFNQRWIRUHLJQLQYDGHUVOLNH bacteria and viruses), but the person’s own ERG\,QWKHFDVHRI067FHOOVDWWDFNWKH myelin and as the body attempts to heal LWVHOISODTXHLVIRUPHG,QGLYLGXDOVZLWK WKHKXPDQOHXNRF\WHDQWLJHQ+/$KDYHD PXFKKLJKHULQFLGHQWRIWKDQWKRVHZLWKRXW this antigen. Newer studies with identical WZLQV UHYHDO WKDW LI RQH RI WKH WZLQV KDV 06WKHUHLVDFKDQFHWKHRWKHUWZLQ will also develop the disease. $QRWKHUSRWHQWLDOIDFWRULQ06LVVRPH VRUWRILQIHFWLRXVDJHQW$WHDPRIUHVHDUFK Page 4 HUVIRXQGWKDWLQGLYLGXDOVZLWK06KDYHD KLJKHUWLWHUIRUWKHPHDVOHVDQWLERG\WKDQD FRQWUROJURXS2WKHUVLPSOLFDWHWKH(SVWHLQ %DUUYLUXVWKHKHUSHVYLUXV++9DQG D UHWURYLUXV +RZHYHU WKHUH LV QR FOHDU HYLGHQFHIRUWKLVYLUDOEDFWHULDOWKHRU\ 6SHFXODWLRQDERXWGLHWLVRQHRIWKHP\V WHULHV WKDW VXUURXQGV 06 7KH FRXQWULHV ZKHUH WKHUH LV WKH KLJKHVW SUHYDOHQFH RI 06DUHVRPHRIWKHZHDOWKLHVWQDWLRQVLQ WKHZRUOG$FKDUDFWHULVWLFRIWKHVHQDWLRQV is that the people in them consume much PRUHDQLPDOIDWVDQGGDLU\SURGXFWVWKDQLQ poorer nations. A diet containing no meat RUPLONSURGXFWVFDOOHG´7KH6ZDQN'LHWµ KDVEHHQFLUFXODWLQJIRUDERXW\HDUV1R research has been done to understand the LPSDFWRIWKLVGLHWRQWKHSURJUHVVLRQRI06 $VFDQEHVHHQIURPWKLVGLVFXVVLRQWKHUH DUHPDQ\WKHRULHVDERXWWKHFDXVHRI06 EXWWKHGLVHDVHVWLOOP\VWLÀHVUHVHDUFKHUV 1RHWLRORJ\IRU06KDVEHHQIRXQG,WGRHV QRWÀWLQWRDQHDWOLWWOHER[VRWU\LQJWRÀQG DFXUHIRUWKHGLVHDVHUHPDLQVGLIÀFXOW$ FRPELQDWLRQRIIDFWRUVJHRJUDSKLFDODUHD JHQHWLFSUHGLVSRVLWLRQDVORZDFWLQJYLUXV may be responsible. Types of Multiple Sclerosis 7KHUH DUH VHYHUDO W\SHV RI PXOWLSOH VFOHURVLV7KHVHGLIIHUHQWW\SHVPDQGDWHD YDULRXVGUXJWUHDWPHQWDQGPRVWOLNHO\D GLIIHUHQWSURJQRVLV ,W LV FXVWRPDU\ WR FODVVLI\ 06 DFFRUG ing to the diseases progression such as: UHPLWWLQJUHODSVLQJ FKURQLF SURJUHVVLYH secondary progressive, or progressive and DJJUHVVLYHIURPRQVHW 7KH PRVW FRPPRQ IRUP RI 06 LV WKH UHODSVLQJUHPLWWLQJYDULHW\,WLVFKDUDFWHU L]HG E\ DQ LQLWLDO DWWDFN D UHPLVVLRQ WKDW gives the person an almost complete return RIDOOIXQFWLRQDOORVVHVDQGWKHQSHULRGLF UHODSVHV WKDW PD\ RFFXU D IHZ PRQWKV RU HYHQ GHFDGHV ODWHU$IWHU WKH UHODSVH WKH SHUVRQ FDQ KDYH VLJQLÀFDQW UHFRYHU\ RI IXQFWLRQ ,I WKH LQLWLDO V\PSWRP ZDV ORVV RI YLVLRQ RSWLF QHXULWLV WKH YLVLRQ PD\ return completely. As a person experiences more relapses over the years, there may be a much poorer chance that they would regain FRPSOHWHIXQFWLRQDODELOLW\DQGWKH\DUHOHIW ZLWKD´UHVLGXDOµRIWKHV\PSWRPV $V WKH SHUVRQ DJHV WKH FRXUVH RI WKH disease may evolve into “secondary pro gressive.” The person then experiences PRUHSHUPDQHQWV\PSWRPVZLWKRXWIXUWKHU remissions. Multiple Sclerosis ,QWKHFKURQLFSURJUHVVLYHW\SHRI06WKH SHUVRQZLOOKDYHDQLQLWLDODWWDFNIROORZHGE\D JUDGXDOZRUVHQLQJRIV\PSWRPVRYHUDSHULRG RI\HDUVZLWKRXWDQ\VLJQRIDUHPLVVLRQ7KLV W\SHRI06LVXVXDOO\IRXQGLQWKRVHLQGLYLGXDOV ZKRZHUHGLDJQRVHGDIWHUWKHDJHRI $UDUHPDOLJQDQWIRUPRI06LQYROYHVGHDWK ZLWKLQ ILYH \HDUV RI GLDJQRVLV 6HYHUH DQG intense demyelination and the development RIGHP\HOLQDWLRQLQWKHSDUWVRIWKHEUDLQUH VSRQVLEOHIRUYLWDOERGLO\IXQFWLRQVSDUWLFXODUO\ WKHEUDLQVWHPPD\UHVXOWLQGHDWKEHFDXVHRI WKHLQDELOLW\RIWKHEUDLQWRFRQWUROUHVSLUDWLRQ and heart rate. 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The heart beats because RI D FRPSOH[ VLJQDOLQJ V\VWHP WKDW EHJLQV in the brain and is transmitted via neurons WRWKHKHDUW:KHQZHEHFRPHIULJKWHQHGRU DQ[LRXV D FRPSOH[ QHWZRUN RI KRUPRQHV DIIHFWV WKHVH QHXURQV DQG WKH\ WHOO WKH KHDUW WRVSHHGXS(YHU\PRYHPHQWLVLQLWLDWHGE\ WKH ZRUNLQJ RI QHXURQV ZKLFK FDQ EH ÀQHO\ tuned in an Olympic athlete or damaged as in a child with cerebral palsy who struggles with ZDONLQJWDONLQJDQGHYHQFRQWUROOLQJERGLO\ movements. :KHQDQ\GDPDJHRFFXUVWRQHXURQVVSHFLÀF IXQFWLRQVDUHLPSDLUHG6RXVLQJ D FRPPRQ example, when a person has a cerebrovascular DFFLGHQW&9$LIWKHQHXURQVLQWKHVSHHFK center are damaged, the person will have prob OHPVZLWKWDONLQJ 1HXURQV LQ WKH FHQWUDO QHUYRXV V\VWHP RI DGXOWVDUHWKRXJKWWREHLQFDSDEOHRIUHJHQHUD WLRQ,QYHU\\RXQJFKLOGUHQLWKDVEHHQVKRZQ that neurons will actually regenerate and a child PD\ UHJDLQ IXQFWLRQ PXFK PRUH HDVLO\ WKDQ DQDGXOW7KLVGHJUHHRIUHJHQHUDWLRQLVFDOOHG ´QHXUDOSODVWLFLW\µ9HU\\RXQJFKLOGUHQKDYH DKLJKGHJUHHRISODVWLFLW\ ,Q PXOWLSOH VFOHURVLV WKH GDPDJH GRHV QRW LQLWLDOO\RFFXUWRWKHDFWXDOERG\RIWKHQHXURQ © National Center of Continuing Education, Inc. but the covering surrounding that neuron, called the “myelin.” So, to review once DJDLQWKLVSURFHVVWKHP\HOLQLVDWWDFNHGE\ the body’s own immune system. Scar tissue IRUPVSODTXHDQGWKLVVFDUWLVVXHLVWHUPHG “lesions”. These lesions impair the conduc WLYLW\RIWKHQHUYRXVV\VWHP Symptomatology of Multiple Sclerosis 0XOWLSOHVFOHURVLVFDQSUHVHQWZLWKDOHJLRQ RIV\PSWRPVZKLFKDUHRIWHQSX]]OLQJIRU the physician who must attempt to diagnose the problem a person is having. These symp WRPVDWÀUVWPD\´FRPHDQGJRµPDNLQJLW VHHPDWWLPHVDVWKRXJKWKHGRFWRULVORRNLQJ DW D ´PHQWDO FRQGLWLRQµ 0DQ\ LQGLYLGXDOV ZLWK06EHFRPHDQJU\DQGIUXVWUDWHG7KH\ UHWXUQ DJDLQDQGDJDLQ WR D GRFWRU ZLWK vague symptoms such as transient visual ORVVSHULRGVRIYHUWLJRDQGQXPEQHVVWKDW FDQ RFFXU IRU D SHULRG RI D IHZ KRXUV DQG WKHQ GLVDSSHDU %HFDXVH WKH OHVLRQV RI 06 can occur anywhere in the brain or spinal cord, one individual may have the presenting V\PSWRPRIIDFLDOSDLQZKLOHDQRWKHUPD\ KDYHWURXEOHOLIWLQJDIRRWRURGGVHQVDWLRQV RIEXUQLQJRUWLQJOLQJLQDOHJ There are six characteristic initial symp WRPVIRXQGLQPRVWLQGLYLGXDOVZLWKPXOWLSOH sclerosis )LJXUH. 7KHVH V\PSWRPV FDQ RFFXU IRU D SHULRG RI VHYHUDO KRXUV RU GD\V DQG WKHQ VHHP WR GLVDSSHDU0DQ\SHRSOHGLDJQRVHGZLWK06 PD\KDYHKDGWKHGLVHDVHIRUPDQ\\HDUVDQG MXVWLJQRUHGWKHV\PSWRPVXQWLOWKH\EHFDPH more alarming. At this point, the person may KDYHÀUVWYLVLWHGDSK\VLFLDQDERXWWKHP %HFDXVH WKH\ WHQG WR ´FRPH DQG JRµ LW LV GLIÀFXOW WR GLDJQRVH 06 DIWHU RQO\ RQH HSLVRGH7KHGLDJQRVWLFFULWHULDIRU06PDQ dates that at least two episodes must occur EHIRUH WKHUH FDQ EH DEVROXWH FHUWDLQW\ WKDW WKHSHUVRQKDV06 As an individual experiences recurrent relapses (or in the chronic progressive IRUPKDVQRUHPLVVLRQEXWRQO\ZRUVHQLQJ V\PSWRPVVSHFLÀFV\PSWRPVEHFRPHPRUH SURPLQHQW LQFOXGLQJ LQFUHDVLQJ ZHDNQHVV spasticity, ataxia, sensory loss, and the loss RIEODGGHUFRQWURO :HDNQHVVLQRQHRUPRUHH[WUHPLWLHVLVD SUHGRPLQDWH IHDWXUH RI PXOWLSOH VFOHURVLV ,WXVXDOO\EHJLQVZLWKRQHOHJ,QRQHFDVH study, a woman reported that she was driv LQJ KHU FDU DQG HDFK GD\ LW VHHPHG DV LI VRPHWKLQJZDVZURQJZLWKWKHEUDNHSHGDO EHFDXVHLWEHFDPHPRUHGLIÀFXOWWRSUHVV+HU KXVEDQGUHDOL]HGLWZDVQRWWKHEUDNHSHGDO and the woman was subsequently diagnosed with multiple sclerosis. 3DWLHQWVPD\FRPSODLQRIDKHDY\IHHOLQJ in a leg or the inability to move a leg prop HUO\DIWHUDSHULRGRIH[HUWLRQ$VWKHGLVHDVH SURJUHVVHVWKHZHDNQHVVPD\UHVXOWLQDWRWDO inability to use that leg. The arms may also be DIIHFWHGDVWKHGLVHDVHSURJUHVVHV7KLVPD\ ÀUVWEHQRWLFHGE\DWUHPRUWKDWRFFXUVZKHQ WKHSHUVRQJRHVWRUHDFKIRUDFRIIHHFXS ,QUDUHFDVHVZHDNQHVVRIWKHPXVFOHVRI WKHGLDSKUDJPOHDGWRWKHLQDELOLW\WRÀOOWKH lungs completely with each breath. As a re sult patients may develop chronic respiratory LQIHFWLRQV 3QHXPRQLD FDQ EH TXLWH GHDGO\ IRU06SDWLHQWV$KHDOWKFDUHSURIHVVLRQDO PXVWEHRQDOHUWIRUVKDOORZEUHDWKLQJDQGWKH UHVWULFWLRQRIWKHPXVFOHVXVHGIRUUHVSLUDWLRQ 6SDVWLFLW\ LV DQRWKHU FDUGLQDO IHDWXUH RI multiple sclerosis and is most commonly seen Initial Symptoms Percent Extremities Weakness Optic neuritis Paresthesia Diplopia Fatigue Vertigo Urinary problems 40% 22% 21% 21% 19% 5% 5% Figure 1 in the lower extremities. The examiner may see increased extensor tone and hyperactive UHÁH[HVLQDOHJ7KLVPD\ÀUVWEHQRWLFHG E\WKHSDWLHQWDVWKH\OD\GRZQLQEHGIRU the night. Their legs become very rigid and, DW WLPHV SDLQIXO$V WKH GLVHDVH EHFRPHV FKURQLFÁH[RUWRQHEHJLQVWRWDNHFRQWURORI the leg. 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The Social Security Administration has recently DGGHG IDWLJXH WR WKHLU OLVW RI V\PSWRPV IRUPXOWLSOHVFOHURVLV )DWLJXHFDQEHRQHRIWKHPRVWIUXVWUDW LQJV\PSWRPVIRUWKH06SDWLHQWEHFDXVH LW PD\ EH ´LQYLVLEOHµ WR RWKHUV 3HRSOH PD\UHPDUNWRDSHUVRQZLWKWKHIDWLJXH RI06´2K\RXORRNVRJRRG,ZRXOGQRW HYHQNQRZ\RXKDYH06µ2IFRXUVH WKLV UHPDUN LV WR HQFRXUDJH EXW FDQ LQVWHDGEHIUXVWUDWLQJ7KHRWKHUSHUVRQ cannot experience what the individual with the illness experiences. 7KLVIDWLJXHLVEHOLHYHGWREHSULPDULO\ FDXVH E\ WKH SRRU FRQGXFWLRQ RI QHUYH VLJQDOVIURPWKHEUDLQWRSDUWVRIWKHERG\ +RZHYHU LW PD\ DOVR EH FDXVHG E\ WKH LQÁDPPDWRU\SURFHVVWKDWRFFXUVDVWKH FHQWUDOQHUYRXVV\VWHPLVDWWDFNHGE\WKH SHUVRQ·VLPPXQHFHOOV,WLVZHOONQRZQ WKDWSHRSOHVXIIHULQJIURPPRUHFRPPRQ DLOPHQWVVXFKDVDFROGRUWKHÁXH[SHUL HQFHIDWLJXHEHFDXVHWKHERG\UHVSRQGV WR DQ LPPXQH DWWDFN ZLWK D IHHOLQJ RI exhaustion. 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Within the past three \HDUVDQHZPHGLFDWLRQFDOOHG´3URYLJLOµ has been developed which seems to help VRPH LQGLYLGXDOV ZLWK WKLV IDWLJXH ,Q other cases, Ritalin has been prescribed in order to increase the person’s energy output. 0DQ\ SHRSOH ZLWK 06 XQNQRZLQJO\ LQFUHDVHWKHDPRXQWRIFDIIHLQDWHGEHYHU DJHVWKH\GULQNLQRUGHUWR´JHWWKURXJK the day.” ,Q DGGLWLRQ WR WKH V\PSWRPV OLVWHG DERYHWKHUHLVDQHQWLUHDUUD\RIVHQVRU\ V\PSWRPVWKDWPD\DFFRPSDQ\06,Q WKHHDUO\VWDJHVRIWKHGLVHDVHPDQ\SHR ple only have sensory symptoms that are GLIÀFXOWWRGHVFULEHWRDSK\VLFLDQ7KRVH SK\VLFLDQVXQIDPLOLDUZLWK06PD\DWWUL bute these symptoms to “hypochondria” ZKLFK IUXVWUDWHV WKH SDWLHQW HYHQ PRUH They may begin to wonder “Are these V\PSWRPV DOO LQ P\ KHDG"µ 3K\VLFLDQV ZKRKDYHOLWWOHH[SHUWLVHLQ06PD\WKHQ Multiple Sclerosis Page 5 SUHVFULEHDQDQWLGHSUHVVDQWRUDQWLDQ[LHW\ GUXJ7KLVIXUWKHULQFUHDVHVWKHSDWLHQW·V OHYHO RI IDWLJXH DV WKH\ VWUXJJOH QRZ ZLWKWKHV\PSWRPV$1'WKHVLGHHIIHFWV RIWKHVHXQQHFHVVDU\GUXJV2QHSDWLHQW ZLWK06VWDWHG “The third time I went to my doctor and told him I felt like bees were stinging my leg, he put me on Valium. Then I still felt the pains in my leg and began to walk into walls because the Valium made me so ‘loopy’.” 7KLV ´IHHOLQJ RI EHH VWLQJVµ LV \HW DQRWKHU V\PSWRP RI 06 FDOOHG SDUHV thesia (numbness and cold sensations) DQGK\SHUHVWKHVLDVDIHHOLQJRIEXUQLQJ HOHFWULFVKRFNVRQWKHH[WUHPLWLHV6RPH people describe a strange vibratory sensa tion either in the extremities or abdomen. 2QH SDWLHQW VWDWHG ´, IHOW OLNH P\ FHOO SKRQHZDVLQP\SRFNHWYLEUDWLQJXQWLO, UHDOL]HG,GLGQRWKDYHP\FHOOSKRQHLW ZDVWKH06µ 1HXURORJLVWVRIWHQXVHSLQSULFNWHVWLQJ to determine how much sensory loss has occurred. This test would be quite pain IXO WR D KHDOWK\ SHUVRQ )RU PDQ\ ZLWK 06WKHUHLVQRIHHOLQJDVWKHSK\VLFLDQ GLJVDQHHGOHLQWRDVHQVLWLYHDUHDRIWKH OHJ6RPHSHRSOHUHSRUWIHHOLQJDVOLJKW SUHVVXUHEXWQRWSDLQ2WKHUVZKRVXIIHU IURP K\SHUHVWKHVLD H[WUHPH VHQVLWLYLW\ to even a bed sheet being placed over WKHPUHSRUWH[FUXFLDWLQJSDLQIURPWKH pin. These sensory symptoms worsen as more demyelination occurs. 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This V\PSWRPPD\LQWHUIHUHZLWKÀQHPRWRU VNLOOVDQGLVREYLRXVLQWKHKDQGZULWLQJRI 06SDWLHQWVZLWKWKLVSUREOHP7KRVHZKR KDGEHDXWLIXOSHQPDQVKLSPD\JUDGXDOO\ GULIWLQWRKDQGZULWLQJWKDWLVVORSS\DQG at times illegible. 7KH SDWLHQW·V IDPLO\ PD\ QRWLFH WKLV V\PSWRPÀUVWDQGPD\FRQVLGHULWWREH ´FOXPVLQHVVµ$ SHUVRQ PD\ JR WR SLFN up a glass, and not realize that it is not completely in the hand and drop it on the ÁRRU3DWLHQWVPD\EHJLQWRWULSJRLQJXS RU GRZQ VWHSV DQG HYHQ ZKHQ ZDONLQJ RQDÁDWDQGHYHQVXUIDFHPD\ORVHWKHLU IRRWLQJHDVLO\ A very disturbing sensation, described E\ WKH WDONVKRZ KRVW 0RQWHO :LOOLDPV LQ KLV UHFHQW ERRNV DERXW KLV VWUXJJOHV ZLWK06LVZKDWKDVEHHQGHVFULEHGDV ´WKH 06 KXJµ 8VLQJ WKH ZRUG ´KXJµ Page 6 FHUWDLQO\LVDQXQGHUVWDWHPHQWRIZKDWDFWX ally happens. 7KH ´06 KXJµ V\PSWRP LV WHFKQLFDOO\ UHIHUUHGWRDV´JLUGOHSDLQµ,WDFWXDOO\IHHOV OLNH D ERD FRQVWULFWRU KDV EHJXQ WR ZUDS LWVHOIDURXQGWKHWKRUD[DQGDEGRPHQRIWKH person, becoming tighter and tighter. Some IHHO DV WKRXJK WKH\ FDQQRW WDNH D EUHDWK EHFDXVH RI WKLV WLJKWQHVV 2WKHUV GHVFULEH MXVW WU\LQJ WR ÀQG D FRPIRUWDEOH SRVLWLRQ XQWLOWKHVHQVDWLRQWHPSRUDULO\JRHVDZD\,W FDQEHWULJJHUHGE\GULQNLQJKRWEHYHUDJHV exercise, or an increase in environmental temperature. ,WLVEHOLHYHGWKLVV\PSWRPPD\EHFDXVHG E\ WKH GHP\HOLQDWLRQ RI WKH VSLQDO FRUG but clearly, no one is absolutely sure why WKLV YHU\ XQFRPIRUWDEOH V\PSWRP RFFXUV ,W FDQ EH VR SDLQIXO WKDW VRPH LQGLYLGXDOV ZLWK 06 PXVW WDNH SDLQ PHGLFDWLRQ DQG antispasmotics. Symptoms Chart Fatigue unsteady gait muscle rigidity muscle spasms loss of sense of position girdle pain Figure 2 An old saying concerning multiple scle URVLVZDVWKDW´,IWKHUHLVSDLQWKHQLWLVQRW 06µ 8QIRUWXQDWHO\ VRPH PHGLFDO SUDFWL tioners continue to believe this, even with FRXQWOHVVUHSRUWVRI06UHODWHGSDLQ 7KHDVVHVVPHQWDQGWUHDWPHQWRISDLQLQ DQ\ GLVHDVH KDV EHFRPH WKH IRFXV RI WKH ,QVWLWXWHRI0HGLFLQH,20WKH-RLQW&RP PLVVLRQ IRU WKH$FFUHGLWDWLRQ RI +RVSLWDOV -&$+2 DQG WKH 1DWLRQDO ,QVWLWXWHV RI +HDOWK1,+%HFDXVHLWKDVEHFRPHVRLP SRUWDQWDVDIDFWRULQWKHTXDOLW\RIOLIHLQ06 patients, the entire segment below is devoted WRWKHWUHDWPHQWRISDLQLQ06 Pain and MS 3DLQZDVWKRXJKWWREHDUDUHDQGPLQRU V\PSWRP LQ SDWLHQWV ZLWK 06 EXW VWXGLHV FRQGXFWHGIURPWRWKHSUHVHQWVKRZWKDW RISDWLHQWVZLWK06FRPSODLQRISDLQ 3DWLHQWVZLWKPXOWLSOHVFOHURVLVPD\H[SHUL Multiple Sclerosis HQFHPRUHWKDQRQHW\SHRISDLQV\QGURPH 7KLVSDLQLVFODVVLÀHGLQWRIRXUFDWHJRULHV neuropathic pain (2) pain indirectly related WR0606WUHDWPHQWUHODWHGSDLQSDLQ XQUHODWHGWR06 7KHPRVWFRPPRQSDLQRI06LVKHDGDFKH QHXURSDWKLF OLPE SDLQ EDFN SDLQDQGSDLQIXOVSDVPV'LV VDWLVIDFWLRQZLWKWKHOLVWHQLQJVNLOOVDQGFDUH by the physician in regard to this pain was UHSRUWHGLQRIWKHSDWLHQWV &KURQLF SDLQ KDV D GUDVWLF LQÁXHQFH RQ TXDOLW\RIOLIH,WDIIHFWVUHFUHDWLRQDODFWLYL WLHV H[HUFLVH DQG ZRUN &KURQLF SDLQ FDQ EHFRPH WKH VRXUFH RI FOLQLFDO GHSUHVVLRQ DQG LW GUDPDWLFDOO\ LQFUHDVHV WKH UDWH RI VXLFLGH7KHUHDGHUPD\UHFDOOWKHLQIDPRXV 'U.HYRUNLDQDQGWKRVHZKRZHQWWRKLP IRUSK\VLFLDQDVVLVWHGVXLFLGHIRUW\SHUFHQW were individuals with multiple sclerosis. 2QHVRXUFHRISDLQLQ06LVQHXURSDWKLF pain, which is described as any pain that has LWVVRXUFHZLWKLQWKHQHUYRXVV\VWHP+HDW SUHVVXUHRYHUZRUNDQGODFNRIVOHHSPD\DJ gravate this symptom. The pain is described as extremely intense and intolerable during the night.. The drug gabapentin (Neurontin) has been XVHGIRUWKLVSDLQZLWKDGRVHUDQJHIURP PJGD\XSWRPJGD\0RUHUHFHQWO\D GUXJIURPWKHVDPHFODVVDVJDEDSHQWLQKDV been developed called pregabalin (Lyrica) XVLQJPJGD\ 7KHVH GUXJV PD\ RIWHQ EH XVHG ZLWK DQ opioid analgesic, although this continues to EHDVRXUFHRIFRQWURYHUV\6RPHSK\VLFLDQV DUHRSSRVHGWRWKHXVHRIRSLRLGVIRUDQ\W\SH RI FKURQLF SDLQ 7UDPDGRO PRUSKLQH DQG R[\FRGRQHDVZHOODVIHQWDQ\OWUDQVGHUPDO DSSOLFDWLRQ KDYH EHHQ XVHG VXFFHVVIXOO\ RIWHQ LQ FRPELQDWLRQ ZLWK JDEDSHQWLQ RU pregabalin. ,QSDWLHQWVZKRVXIIHUIURPSDLQIXOQHXURS DWKLHVLQWUDWKHFDOEDFORIHQDQDQWLVSDVPRGLF DJHQWLVXVHGIRUWUHDWPHQW7KLVLQYROYHVWKH LPSODQWDWLRQRIDSXPSRULQMHFWLRQLQWRWKH spinal cord via a lumbar puncture. /DWHO\WKHXVHRIFDQQDELQRLGV7+&0D ULQROKDVEHHQWULHGIRUQHXURSDWKLFSDLQRI 06EXWVWXGLHVDUHLQFRQFOXVLYHDWWKLVSRLQW 3DLQ DVVRFLDWHG ZLWK PXVFOH VSDVWLFLW\ DIIHFWVRIDOOSDWLHQWVZLWK067KH GUXJV RI FKRLFH WR FRQWURO WKLV LV EDFORIHQ DQG 9DOLXP +RZHYHU ERWK DUH DVVRFLDWHG ZLWKLQFUHDVHGZHDNQHVVLQWKHDIIHFWHGOLPE While trying to relax a very tight spastic PXVFOH WKH GUXJV KDYH WKH QHJDWLYH HIIHFW RIFDXVLQJDÁDFFLGPXVFOHWKDWLVZHDNHU © National Center of Continuing Education, Inc. +HDGDFKHVDUHVHHQLQRISHRSOHUHFHLY LQJRQHRIWKHFODVVLFWUHDWPHQWVXVHGIRU06 ,QWHUIHURQEHWDWREHGLVFXVVHGODWHULQWKLV DUWLFOHDQGFDQEHFRQWUROOHGZLWK16$,'6 QRQVWHURLGDODQWLLQÁDPPDWRU\GUXJV0L JUDLQHKHDGDFKHVZHUHUHSRUWHGWREHWKHÀUVW V\PSWRPRI06LQRISDWLHQWV %DFNSDLQLVVHYHUHLQRISDWLHQWVZLWK 06 EHFDXVH RI VSDVWLFLW\ WKDW RFFXUV LQ WKH OHJVDQGXQHYHQDQGDZNZDUGDPEXODWLRQ 7UHDWPHQW IRU WKLV PD\ FRQVLVW RI SK\VLFDO WKHUDS\ DQGRU 7(16 WUDQVGHUPDO HOHFWUL cal nerve stimulation, to be discussed under “Complimentary Therapies”) $YHU\RGGW\SHRISDLQH[SHULHQFHGDWOHDVW RQFHE\PRVW06SDWLHQWVLVWKH/KHUPLWWH·V Sign. This can be elicited in a person by hav ing them stand up straight and then lower their FKLQ WR WKH FKHVW 7KLV PD\ HOLFLW D IHHOLQJ RIHOHFWULFVKRFNWKDWEHJLQVLQWKHKHDGDQG VKRRWVGRZQWRWKHEDVHRIWKHVSLQDOFRUG,W LVGHVFULEHGDV´YHU\XQSOHDVDQWµE\PRVW06 SDWLHQWV7KH\WU\WRDYRLGWKLVPRWLRQIRUIHDU it will elicit this pain. 3HUVLVWHQWSDLQLVRQHRIWKHPRVWGLIÀFXOW problems encountered in pain management FOLQLFV EHFDXVH RI WKH GLYHUVH DQG GLIIXVH QDWXUH RI WKH SDLQ 6RPH LQGLYLGXDOV PD\ KDYH VSDVWLFLW\ ZLWK D SDLQIXO QHXURSDWK\ DQG PLJUDLQH KHDGDFKHV )LQGLQJ WKH ULJKW FRPELQDWLRQRIGUXJVDQGDOWHUQDWLYHWKHUDSLHV LVYLWDOWRDJRRGTXDOLW\RIOLIHZLWKPLQLPDO depression. 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The patient may smile and laugh inappropriately and GLVPLVVDQ\FRQFHUQVDERXWHYHQOLIHWKUHDW ening health problems. Paroxysmal Symptoms of Multiple Sclerosis ,QDGGLWLRQWRWKHFODVVLFV\PSWRPVRI06 WKHUHLVDFDWHJRU\RIV\PSWRPVUHIHUUHGWRDV “paroxysmal symptoms”. These may occur GXULQJDUHODSVHRUHYHQIRUYHU\EULHISHULRGV RIWLPHZKLOHDSHUVRQLVLQUHPLVVLRQ7KH\ PD\ ODVW IURP VHYHUDO PLQXWHV WR VHYHUDO ZHHNVDQGWKHQGLVDSSHDU Trigeminal neuralgia is a symptom involv LQJ WKH GHP\HOLQDWLRQ RI WKH ÀIWK FUDQLDO nerve and/or its nucleus in the pons. Tri geminal neuralgia can cause severe pain on RQHVLGHRIWKHIDFHVRWKDWHYHQDEUHH]HFDQ cause severe pain. 06 SDWLHQWV ZLWK WULJHPLQDO QHXUDOJLD RIWHQ JR WR D GHQWLVW EHOLHYLQJ WKH\ PD\ KDYHVHYHUHGHQWDOSUREOHPVDQGRIWHQÀQG themselves in the emergency room because WKHSDLQLVVRVHYHUH7KHWUHDWPHQWIRUWKLV disorder is carbamazepine. Other paroxysmal symptoms include the RFFXUUHQFHRIVHL]XUHVLQRI06SDWLHQWV uncontrollable itching, and double vision. ©National Center of Continuing Education, Inc. Optic Neuritis and Visual Problems ,QPRVWSDUWVRIWKHZRUOGGHP\HOLQ ating optic neuritis is the most common FDXVHRISDLQIXOXQLODWHUDOYLVXDOORVVDQG RIWHQ RQH RI WKH ÀUVW VLJQV RI PXOWLSOH VFOHURVLV,WFDQEHFRPHZRUVHRYHUDSH ULRGRIDIHZGD\VWRWZRZHHNV7KHUHLV SHULRFXODUH\HSDLQSDUWLDOORVVRIYLVLRQ LQRQHH\HDUHFXUUHQFHRIV\PSWRPVZLWK LQFUHDVHG ERG\ WHPSHUDWXUH 8KWKRII·V SKHQRPHQRQDQGSDOORURIWKHRSWLFGLVF $SSUR[LPDWHO\ RI WKH FDVHV JR RQ WR GHYHORS RWKHU V\PSWRPV RI PXOWLSOH sclerosis within 15 years. :LWKLQZHHNVDQUHFRYHU\RI visual loss occurs but with subsequent DWWDFNVWKHYLVXDOORVVLQFUHDVHV 7KHWUHDWPHQWIRURSWLFQHXULWLVLVRIWHQ WKHVDPHDVIRUPXOWLSOHVFOHURVLVWKHXVH RI FRUWLFRVWHURLGV DV DQWLLQÁDPPDWRU\ agents. 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Actually, patients are UHOLHYHGZKHQWKH\UHDOL]HWKH\FDQVSHDN ZLWKWKHLUSK\VLFLDQDERXWWKHLUIUXVWUDWLRQ with the disease. ALD $GUHQROHXNRG\VWURSK\ $/' ZDV mentioned previously in regard to The 0\HOLQ )RXQGDWLRQ ,W LV D GHP\HOLQDW ing disease with onset in childhood and V\PSWRPV EHJLQQLQJ WR VKRZ IURP WKH DJHVRI\HDUVRIDJH,WLVDVH[OLQNHG JHQHWLF GLVHDVH DQG VR LV RQO\ IRXQG LQ males, yet the trait is carried only on the ;FKURPRVRPH This is a very rare disease that came to light with the movie “Lorenzo’s Oil.” /RUHQ]RKDGWKLVGLVHDVHDQGZHQWIURP being a very bright and active child to a paralytic within 18 months. The parents were told even though many young boys had this disease throughout the world, it was considered to be an “orphan disease” and no drug companies would invest in re VHDUFKDQGGHYHORSPHQWIRUDGLVHDVHWKDW KDGRQO\DIHZKXQGUHGWKRXVDQGYLFWLPV Through endless library research (even EHIRUHWKHGD\VRIWKH,QWHUQHWDQGYLWDO communication with primary research FKHPLVWV WKLV IDPLO\ ZDV DEOH WR GH velop “Lorenzo’s Oil” (ericic acid), which EORFNHG WKH IRUPDWLRQ RI WKH YHU\ ORQJ FKDLQIDWW\DFLGVDW&DQG&FDUERQ chains) that cause the disease. $/' EHJLQV ZLWK VXGGHQ EHKDYLRU FKDQJHVVXFKDVK\SHUDFWLYLW\GLIÀFXOW\ in concentration, and changes in person Multiple Sclerosis Page 11 ality, such as inappropriate screaming and FU\LQJ7KHVHV\PSWRPVDUHIROORZHGE\ QHXURORJLFDO GHÀFLWV VXFK DV SUREOHPV with gait and a gross tremor. The disease SURJUHVVHVWRFRUWLFDOEOLQGQHVVGHDIQHVV DQGLPSDLUPHQWRIDOOKLJKHULQWHOOHFWXDO IXQFWLRQV These symptoms are caused by the rapid DQG PDVVLYH GHP\HOLQDWLRQ RI WKH EUDLQ The adrenal glands are also destroyed by these abnormal lipid deposits and Ad GLVRQ·V 'LVHDVH GHYHORSV FRPSOHWH ODFN RIWKHSURGXFWLRQRIWKHDGUHQDOKRUPRQH cortisol). 6LQFHWKHDGGLWLRQRIHUXFLFDFLG WRWKHGLHWRIDIIHFWHGFKLOGUHQKDVKHOSHG them to be normal. The discovery was too ODWHIRU/RUHQ]R+HKDGDOUHDG\VXIIHUHG irreversible brain damage. 1RZLQDWHDPDWWKH8QLYHUVLW\RI 3DULVKDVIRXQGDZD\WRUHPRYHWKHFDXV DWLYH JHQH IURP WKH ;FKURPRVRPH DQG UHSODFHLWZLWKDQRUPDOJHQH7KH0\HOLQ )RXQGDWLRQJLYHVJUDQWVWRVFLHQWLVWVZKR ZLVKWRZRUNRQWKHFXWWLQJHGJHLQÀQGLQJ WKHFXUHIRUGHP\HOLQDWLQJGLVHDVHV The Myelin Project ,Q$XJXVWRDQG0LFKDHOD2GRQH FUHDWHGWKH0\HOLQ3URMHFWZKHQWKHLUVRQ Lorenzo, was diagnosed with adrenoleu NRG\VWURSK\DQGWKH\ZHUHWROGWKHUHZDV QRWKLQJWKDWFRXOGEHGRQHIRUKLP7KH\ ZHUHWROGWRWDNHKLPKRPHDWWKHDJHRI DQGMXVWZDLWIRUKLPWRGLH7KH\ZHUH unwilling to accept that prognosis and cre DWHG7KH0\HOLQ3URMHFW 7KHSURMHFWEHJDQDVDQDWWHPSWWRJHW VFLHQWLVWV WR ZRUN WRJHWKHU WR VKDUH FRO laborate and critique their research on demyelinating diseases. They also wanted WRXQLWHWKHVHVFLHQWLVWVZLWKWKHIDPLOLHV WKDWVXIIHUHGIURPWKHVHGLVHDVHV7KHJRDO ZDV QRW ´VFLHQFH IRU VFLHQFH·V VDNHµEXW VFLHQFHWRSUHVHUYHKXPDQOLIH ,QWKHPRYLH´/RUHQ]R·V2LOµZDV SURGXFHGZLWKPDMRU+ROO\ZRRGDFWRUVDQG was seen by millions across the United 6WDWHV7KLVEURXJKWDQDZDUHQHVVRIGH P\HOLQDWLQJGLVHDVHVWRFRPPRQSHRSOH,Q 2009, their website www.myelin.org had RYHUYLVLWRUVIURPFRXQWULHV 7KH\ XVH D YHU\ GLIIHUHQW DSSURDFK WR research in actually trying to regenerate P\HOLQRPHJDDQGRPHJDIDWW\DFLGV DQGWKHSURJUHVVLRQRIPXOWLSOHVFOHURVLV (IIRUWV WR WUDQVSODQW JOLDO FHOOV DQG WKH XVHRIXPELOLFDOFRUGRUVWHPFHOOVRIWKDW person to regenerate myelin are now being GRQHWKURXJKWKLVIRXQGDWLRQ Page 12 Demyelinating Diseases of the Peripheral Nervous System The peripheral nervous system includes DOOQHUYRXVVWUXFWXUHVO\LQJRXWVLGHRIWKH SLDOPHPEUDQHRIWKHVSLQDOFRUGDQGEUDLQ 7KHVHQHUYHVHPHUJHIURPWKHVSLQDOFRUGDW YDULRXVOHYHOVRIWKHVSLQDOQHUYHURRWV,WLV SRVVLEOHWKDWWKHP\HOLQRIWKHVHVWUXFWXUHV can degenerate in a disease process. These QHUYHVXQOLNHWKRVHLQWKHFHQWUDOQHUYRXV V\VWHPDUHFDSDEOHRIUHJHQHUDWLRQ The most predominant disease in this category is acute idiopathic polyneuritis, DOVRNQRZDV*XLOODLQ%DUUH6\QGURPH,W LVIRXQGWKURXJKRXWWKHZRUOGDQGDIIHFWV FKLOGUHQDQGDGXOWV,QRIWKHFDVHVWKH RQVHWRIWKHGLVHDVHLVSUHFHGHGE\DYLUDO LQIHFWLRQWKDWRFFXUUHGZHHNVSULRUWR WKHRQVHWRIWKHGLVHDVH$PDMRURXWEUHDN RIWKH*XLOODLQ%DUUH6\QGURPHRFFXUUHGLQ DIWHUWKHDGPLQLVWUDWLRQRIWKH6ZLQH )OXYDFFLQH 7KHÀUVWPDMRUFOLQLFDOV\PSWRPLVZHDN QHVVWKDWHYROYHVRYHUDSHULRGRIVHYHUDO hours to several days and includes all ex WUHPLWLHVDQGIUHTXHQWO\WKHPXVFOHVXVHG LQUHVSLUDWRU\IXQFWLRQ7KLVZHDNQHVVFDQ progress to total motor paralysis and death IURPUHVSLUDWRU\IDLOXUH :HDNQHVV LV IROORZHG E\ D ORVV RI WKH GHHS WHQGRQ UHÁH[HV 7KH FHUHEURVSLQDO ÁXLGLVQRUPDOIRUWKHÀUVWZHHNEXWWKHQ VKRZVKLJKSURWHLQOHYHOVIRUWKHQH[WVL[ ZHHNVLQGLFDWLYHRIWKHEUHDNGRZQRIP\ elin within the peripheral nervous system. 0RVWUHVHDUFKVKRZVWKDW*XLOODLQ%DUUH LVDUHVXOWRIWKHFHOOPHGLDWHGLPPXQRORJL cal reaction directed against the peripheral QHUYRXVV\VWHPDQGVSHFLÀFDOO\WKHP\HOLQ ,WVHHPVWKDWKDYLQJKDGDYLUDOLQIHFWLRQLQ WKHZHHNVSUHFHGLQJWKHRQVHWRIWKHGLVHDVH somehow activates the immune system to DFWXDOO\DWWDFN´VHOIWLVVXHµPXFKOLNHZKDW happens in multiple sclerosis. *XLOODLQ%DUUH 6\QGURPH JRHV LQWR UH mission naturally and results in complete UHFRYHU\ LQ WKH PDMRULW\ RI FDVHV 7KH GDQJHURXV SHULRG LV LQ WKH ÀUVW IHZ GD\V when the respiratory system can actually VKXWGRZQIURPWKHORVVRIPRYHPHQWRI the diaphragm. Within hours, a person can GHWHULRUDWHZLWKQRFRPSHQVDWLRQIRUSXOVH R[\JHQOHYHOVWKDWGLYHLQWRWKHUDQJH RUORZHU$WWKHÀUVWVLJQRIG\VSQHDLQWXED tion and ventilation should begin. $ERXWRISDWLHQWVGRQRWVXUYLYHWKH Multiple Sclerosis illness and death may occur without a rational H[SODQDWLRQ,QRIWKHSDWLHQWVUHVLGXDO disability can be severe, requiring ongoing mechanical ventilation. Recovery can occur LQDIHZZHHNVDOWKRXJKVRPHSHRSOHWDNH months to achieve recovery. A controlled study using plasmapheresis GHPRQVWUDWHG D PXFK EHWWHU SURJQRVLV IRU WKRVHVXIIHULQJIURP*XLOODLQ%DUUH6\QGURPH 6WUDQJHO\FRUWLFRVWHURLGVVHHPWREHLQHIIHF WLYHLQWKHWUHDWPHQWRIWKLVGLVHDVH $ FODVVLF DFFRXQW RI D ZRPDQ ZKR ZHQW WKURXJKWKHHQWLUHFRXUVHRIWKLVGLVHDVHDQG her very bad experience with uncaring medi FDOSURIHVVLRQDOVLVUHFRUGHGLQWKHERRN´%HG 1XPEHU7HQµZKLFKLVUHTXLUHGUHDGLQJIRU all my nursing students). Amyotrophic Lateral Sclerosis (ALS) aka Lou Gehrig’s Disease Although not technically a “demyelinating GLVHDVHµLWLVFRYHUHGZLWKLQWKHVFRSHRIWKLV UHYLHZSDSHUEHFDXVHRIVLPLODULWLHVLWKDVZLWK the demyelinating process. 7KLV GLVHDVH WDNHV LWV QRQPHGLFDO QDPH IURPWKH<DQNHHÀUVWEDVHPDQZKRDFTXLUHG LWLQDQGGLHGLQ0DQ\SHRSOHDUH IDPLOLDUZLWKLWIRUWKDWUHDVRQ $/6LVDPRWRUQHXURQGLVHDVHRIXQNQRZQ etiology that causes a progressive degeneration RIWKHFRUWLFRVSLQDOWUDFWVDQGFHOOVLQWKHDQ WHULRUKRUQRIWKHVSLQDOFRUG7KHDYHUDJHDJH RIRQVHWLV\HDUVRIDJH,WLVPRUHFRPPRQ in males. The patient may begin to have slight neurological impairments, such as speech that sounds slurred. 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