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DystoniaMedical
ResearchFoundation
CONTRIBUTORS
Guy Bouvier, MD
Notre Dame HosPital
DeparEnentofSurgery
St. Larnbert,Quebec,Canada
Writtenby JessicaFeeley,Editor/SpecialProjectsCoordinator
ADystonta Medical Research Foundation' Prinled 12/03 ' 500
Suncrcer- ron Dvsront.r
INrrnvENTloNs
TlsI,n oF CoNTENTS
I.Introduction.. ...2
I I . P e r i p h e r aSl u r g e r i e s . . . .....3
CervicalDystonia./SpasmodicTorticollis ....3
The BertrandProcedure:SelectivePeripheralDenervation
Rhizotomy
M icrovascularDecompression
SpasmodicDysphonia/Larytgeal Dystonia. . . . . . . . .1
SelectiveLaryngealDenervationand Reinnervation
Thyroplasty
Blepharospasm.... ........10
Myectomy Surgery
GeneralizedDystonia&Hemidystonia.. ....12
Intrathecal Baclofen
IV.Conclusion.. ........25
V.Appendix.... ........26
Dystonia
The Nervous System
Anatomy
Eyes
Neck
Larynx
I.IxrnooucrloN
As researchaboutdystoniaprogresses, greatattentionis being paid to the
role of surgicalinterventionsfor alleviatingsymptoms.Surgicaltreatments
for dystoniamay be an option for casesthat do not respondto oral
medications or botulinumtoxin injections.Researchers areactivelv
refining currenttechniquesandcollectinginformationaboutwhich
patientsmay benefitthe most from surgicaltreatments.
posteriorrami (branch)of
one or more spinalnerves
along the cervical vertebrae. I n f e r i o r A n t e c o l l i s Inferior Retrocollis
(This elementof the proce- .)
dureis calledoosterior /"-
ramiseclonn'.) Spinalnerves
\/<4
i /-/.,\!,..
are arrangedin pairs along s1- \
the lengthof the spinalcord
and supplymusclesand
Y
organs.Some research The six elementalforms of spasmodictorticollis.
Sr nctc.qr-Ilrcnr' rr:rroNsron Dysrosl,r
To dateover2.000ceruicaldystoniapatientshaveundergonethis proce-
dure.Somecentersrepofi significantimprovementin as manyas 887'oof
cases.Althoughthe proceduremay benefitindividualswith a rangeof
symptoms,the categoriesof patientswho may havethe bestresultsfrom
the Berlrandprocedureareindividualsin which:
Rhizotomy
Thyroplasty
'fiwroplas\,
surgeriesincludea group of surgicaltechniquesto modif-v
'fhese
thecartilagesurroundingthe larynx. adjustableandreversible
procedures involvemanipulating thecartilageby implantingwedeesor
to hold the tissuein place.A numberof variationsof this procedure
:-;hims
are currentlyusedand are effectivefor restorationof the voice after
paralysisor in changingthe pitchof the voice.
'lype
I thyroplastyhasbeenusedfor the abductorvarietyof spasmodic
dysphonia.In this procedure,the vocalcordsare broughtclosertogether
in hopesof decreasingthe effectof the abductorspasms.Resultsare
mixed,with somepatientsgettinggoodreliefandothershavingminimal
effect.
-lype
lI thyroplastyis a procedurefor adductorspasmodicdysphoniathat
involvesspreadingthe vocalcordsapartby insertinga shimthatprevents
them from contactingeachother during the spasmsthat occur with this
disorder.Although somepatientshavereportedgood relief of vocal strain,
othersfeel the trade offto a breathvand weak voice is excessive.
BLEPHAROSPASM
Myectomy
tseforetheavailabilityof'botulinumtoxin,mvectomvwasessentiall;-
'[-he the
only treatmentoptionfor blepharospasm. introductionot'botulinunr
toxin injectionsin r989benefitedmanypersonswith brepharospasm
tf erebychangingthepopulationof individualseligiblefo. myeciorn,r-..
candidatesfbr mv'ectomv becamethosefor whom botulinumtorin rsnot
sufficient.
l0
Srrncrclr-INrenvrNrroNsponDvstotue
ll
Sunctcel In-rrrveMnoNsron DysroMA
but may last a year or more. Loss of tissuevolume in the eyelid areamay
occurwith the muscleremoval,but the improvedbrow, lid position,and
decreased eyelid wrinkling generallygivesan improvedcosmeticappear-
ance.Decreasedeyelid closureoccursas a result of eyelid muscle
removaland may requirethe needfor additionalartificial tearsand
lubricatingointment.As the eyelidswellingresolves,the eyelidclosure
improvesand the dry eye symptomsgenerallyimprove.Chronic lid
swellingwhich may last six monthsor longerin somepatientscan be a
chronicandtroublesomecomplication.Chroniclid swellingis much less
severeand persistentin the modernmyectomypracticesin which upper
and lower lid myectomiesare performedseparately.lnfection,hematoma,
brow hair loss,and abnormalpositioningof the lower lid canoccasionally
occurbut are uncommon.
Intrathecalbaclofentherapyis Figure 2
a non-destructive,adjustable,
and reversibletreatment.
l
Severalhundred dystonia a
*
patientshavebeentreated 7
,{r
u ith intrathecal
baclofenover rt
the courseofabout l0 years. :\{t
It hasbeenusedfor children
lfi
*s
andadultswith generalized
dystonia(bothprimaryand ;a
secondary t andhemidystonia
u,horespondto baclofen.
Many personstreatedwith
intrathecalbaclofenhavea
combinationof dystoniaand
cerebralpalsy.lntrathecal
baclofenmay be usedto treat
dystoniaaffectingthe upper
andlowerlimbs.
In orderto determineif an
f'
individualis eligiblefor intrath-
I tt tt'q tlt ec'qI bcrc ktf'ert
ecalbaclofen,he/shewill undergoa
hutdvare in body.
screeningtestto obseruethe body's
responseto baclofen.A responseto the oral drug rnaynecessitate
a screeningtestto observethe body'sresponse to a small doseinjected
directlyinto thespinalfluid. Thernedicationis injectedusinga standard
lumbarpunctureor spinaltap.The screeningtestprocedureinvolves
injectionof the medicationfollowedby severalhor.rrs of observation.
Relaxationof themusclesindicatesthatan irnplantedbaclofenpurnpwill
likely be eff-ective.The effectsof the screeningtest are telnporaryand
may lastseveralhoursafterthe injection.If a patientdoesnot responda1
all to the screeningtest,a secondtestusingthe sameproceduremay be
tried the next day or at a later date.
IJ
Suncrcer-INrenveunoNsror. DysroNn
t4
Suncrcer-Ilrrnmmons nonDvsroNIA
Different partsof the brain work togetherto help the body accomplisha
specifictask,suchas tappingthe foot. 1-hepartsof the brain communi-
catevia pathwaysof individualbraincellsthattransmitchemicalmes-
sagesfrom one to the other.In an indrvidualwith dystonia,the pathways
that facilitatethe movementof the foot are disruptedby abnormal
activity.The goal of brain surgeryis to freeup the pathwaysso that the
brain andbody may accomplishthe intendedfunction-in this case,
movingthe foot.
l6
Suncrcel hrsnvENTroNS
FoRDysroNrA
'l'he
practiceof lesioningpartsof the brain in dystoniapatientswasverv
comnlonin the 1950sand 1960s,sinceat thattime it wasessentially the
only availabletreatmentfor severecases.Theseprocedures. as practiced
50 'vearsago,had mixed results.In sonrecasesthc inrprovement u'as
spectacular;in othercasescomplications developed;and in still other
casesrepeatedprocedureswere necessary.By the 1980s,brain surgery
for dystoniahad fallen out of favor and was not widely practiced. How-
ever,the increased understanding of the basisof movementdisorders
suchas Parkinson'sdiseaseand the successin treatingit with surgical
approaches, plus the developmentof brain imagingtechnology,led to a re-
evaluationof surgeryas an option for patientswith dystonia.
l8
proceduremay be done in a single surgeryor in two separatesurgeries.
lf a secondtarget is to be lesioned,the mappingprocedureis repeatedfor
that specifictarget.Most patientsare in the hospitalfor two or three
days.IVledications may be temporarilyresumed,and after a short time the
patientreturnsto the neurologistfor a follow-up exam.
'l'here
is a smallbut realrisk of complications
associatedwith lesioning.
'l'he
mostseriousrisk is a 1-2Yoincidenceof strokeor hemorrhage during
the mappingphaseof the surgery.Also, the targetof the pallidotomy,the
internalsegmentof the globuspallidus,is locatedright abovethe optic
tract which may be damagedif the electrodeis not targetedprecisely.
-l'here
alsoexiststherisk thatthe pallidotomywill not improvethe symp-
toms.Howeveqthe procedurehasbeenshownto dramaticallyimprove
dystoniain somepatients.
Deepbrainstimulation(DBS) involvesimplantingstimulatingelectrodes
into selectedtargetsin the brain in orderto mimic the effectsof lesioning.
SurgeonsbeganusingDBS in placeof lesioningfor Parkinson's disease
patientsin the mid-1990s.DBS alsohasapplications to tremorand pain.
WhereasDBS has beenusedto treat thousandsof personswith
Parkinson's disease,the procedurebeganbeingappliedto dystoniaonly in
the late 1990s.The resultsof more than 200 dystoniapatientshave been
publishedascasestudiesin medicaljournals.
l9
The complete Figure 3
DBS apparatus
includesthe
DBS electrode.
a connecting
wire, and a
pulsegenerator
(a.k.a."brain
pacemaker"or
stimulator)that
contains
a battery.The
initialprocedure
to implantDBS
is identicalto that Deep brain stimulation horchrore in body.
Lesioningprocedures
andDBS havemanyelementsin commonincluding:
. Patientselectioncriteria
. Area of brain targeted
. Basicsurgicalprocedure
. Potentialfor profoundbenefitto eligiblepatients
. Risk ofcomplicationsincludinghemorrhageduringsurgeryhemiple-
gia or hemiparesis,sensoryimpairments,speech/language
impairment
2.
Llecause lesioningcreatesa permanentchangein the brain tissue,thereis
a slightl;-higherrisk of permanent complications duringthe surgerysuch
asswallowingdifficult1,, speechdifflrcultv.andcerebralhemorrhage.
BecauseDllS involvesthe implantationof hardware,complications
':ssociated * ith theapparatusarepossible. includinginfection. erosion
'l-he
the skin.hardwarebreakage.
ilrrr-rugh and stimulatorfailure. risk of
irardu'arecomplications existsfor as longas the hardwareis implanted.
Lesioning
Controlleddestruction Non-destructive
No cosmetic
issues Hardwaremay be slightly
visible beneathskin in
some people
B
Children & Brain Surgery
z+
IV. Coxcr,usroN
Having surgeryis a very significantstepfor an individual to take in the
treatrnentofdystonia. Ifyou are consideringsurgeryor ifsurgery has
beenrecommendedto you by a movementdisorderspecialist,the follow-
ing questionsmay helpyou initiatediscussionswith your doctors:
Dystonia
25
Tlrc Nervous System & Brain
Figure 4
The nervoussystemis
dividedinto two parts:the CentralNen'ous Systenl
centralnervoussystem Brain and Spinal Cord
nervesextendingfrom the
Thoracic
spinalcord. region
rangingfrom
activities, Sacral
heartrateand muscle region
rnovement to emotionsand
leaming.
Basal ganglia
sncl related stnt(tttres of
ilrc brain--pt'ofi I e vi ew.
B a s a lG a n g l i a
G l o b u sP a l l i d u s
Thalamus
S u b s t a n t i aN i g r a
Cerebellum
Figure 6
Artatomy Orbicularis
oculi
>\
Eyes L o 1 1 1 1 u1 1 . 1 1 ,'
Theorbicularisoculimuscle Pr,,..-rur-;P
encirclesthe openingof theeye
socketand actsto closethe Y..i
,j*:{'
eyelids.The corrugatorr.nuscle
draw'sthe eyebrollstogetherand
wrinklesthe brorv.The procerus J
.t
muscleis a facialrnusclebetrveen
the eyebrowsand down the nose. &
Ey'entttsclesin pro/ile
28
Neck Figure 7
The stemocleidomastoid and
trapeziusmusclesare major
musclesin the neck.The two -ilt*r...-
/
stenrocleidomastoid muscles -: ,,/
are thick muscleson eachside S t e r n o cl e i d o mas t o i d
ofthe neckthat act to bend,
rotate,flex, and extendthe
head.The trapeziusmuscle
movesthe shoulderblades Trapezius
upward in a shrug.
Larynx
The larynx or "voice box" is an organ in the neck that plays a crucial role
in speakingand breathing.The framework of the larynx is madeup of the
thyroid cartilage.The front portion of the thyroid cartilageis visible in
somepeopleas the "Adam's apple." The vocal cords are locatedin the
centerof the larynx. The thyroarytenoidmuscleis responsiblefor closing
the vocal cords,and the posteriorcricothyroidmuscleis responsiblefor
openingthe vocal cords.
Posterior
Figure8 cr i co a r y t e n o d
i
Lateral
Thyroarytenoid c r ic o a r y t e n o d
i
muscle muscle
D
Larynx (continued)
Figure 9
Figure l0
;;&,
Surfaceofthe neck.
VI. Souncps:
''Surgery
Panel" presentations
at 7d Worldwide DystoniaPatientSymposiurn& 2"d
I-'amilySymposium. November8-10.2002.
3l
SM Kitgore,H Bronte-Stewart,Outcomeof palladialsurgeryin primary vs, secondary
dystoni4Neurology,2002(suppl7); 58, P05.l5l; ,4.395
Images
Figure l-l'arsy, D., Vitek, J., Lozano, A., Surgical Treatmentof Parkinsonb Diseaseand
Other MovementDisorders,Totow4 NJ: HumanaPress,2003.
Figure 2-Image provided courtesyof Medtronic.
Figure 3-Image provided courtesyof Medtronic.
Figure 4-Dystonia Medical ResearchFoundation,8-18 Guidebook,1994.
Figure S-Henkel,J, "Parkinson'sDisease:New TreatmentsSlow Onslaughtof Symp-
toms," -FDl Corxumer: The lvlagazineof the U.S. Food & Drug Administration,Yol.32,
No. 4. July-August1988.
Figure 6-Gray, H., Anatomyof theHuman Body, Philadelphia:Lea & Febiger,l9l8;
Bartleby.com,2000. www.bartleby.com/ I 07l. | | I 1212003.
Figure 74ray,H., Anatomltof theHyman Bod,, Philadelphia'.Lea&Febiger,l9l8;
Bartleby.com,2000. wwwbartlebv.com/I 07/. | | I 1212003.
Figure 8-Gray,H., Anatomyof the Human Body,Philadelphia:Lea & Febiger,l9l8;
Bartleby.com,2000. www.bartteby.com/ I 07/. | | I 1212003.
Figure9-Gray.H.,AnatomltoftheHumanBody,Philadelphia: Lea&Febigeq l9l8;
Bartlebv.com.2000. www.bartleby.com/I 07l. | | I 1212003 .
Figure lO-Stark.F., Gray s Anatomy: A FacrFilled Coloring BooN
Philadelphia:RunningPressBooks, I 991.
JZ