TicDisorders

inChildhood:
Physician
Awareness
is Key
Tic disorders
area common
occurrence (imitatingotherpeople's
movements)
and
in school-age
childrenandconstirure
the" copropraxia (obscene
gestures).
mostcommonmovement disorder
broughtto Similarly,
simplevocalticsconsistof
theattention
of thecareprovider
by families sniffing,snorting.
coughing,grunting,
andteachen.Thespectrum andseverity barking,throatclearing,
clicking,squeaking
rangetiom mild,transient
simplemotortics andhissing. Complexvocalticsconsistof
to thepotentially
devastating
Tourette echolalia (repeating
otherpeople'swords),
B.vThomas K. Koch,tut.D.
Prokssor, Sl"ndrome. palilalia(repeating
one'sown words).and
Pedistrics andNeurology
Director Pediatri c Neurolopv- Ticsaredefinedassudden. rapid,briei, mostdistressing, coprolalia.Thereis
repetitive
andpurposeless
involuntary actuallya thirdtypeof tic calledsensory
stere0typedmovementsor utterances.
They tics.Theseincludeanuncomfortable
tiequentlyarerefenedro as"habitspasms" sensation
suchasa tickle,initation.
or "nervoustwitches."
An appreciarionand temperature
change
or unusual feeling
understanding
of thespectrum aswellasthe causing
thepatientto produce
a voluntary
associated
disordersallowscareproviders
to movementor sound.Althoughuncommon
counsel
parentsandto treatthechild in children,
theyhavebeenreportedin up to
appropriately
whenindicated. ;10percentof adultTourette 2.
patients
In general.
ticshaveseveral
common
Table
1 characteristics
t0 assistin theirdiagnosis.
Tics
TicClassilication typicallywar andwaneandfrequently occur
lessin anofficesettingthanby history.In this
Simple Complex
situation
a videodiarysupplied
by thefamily
NIotor Eyeblinking Facialgrimacing canbehelpfulto thephysician.
Headtwitching Touching Ticsfrequently increase
with anxiety,
Headthrusting Smelling emotionalstress,anger!
excitement
and
Shouldershrugging Jumping fatigue,
Whiletheymayoccurduringsleep,
NIouthopening Echokinesis theyareusuallylessnoticeable
andthey
Copropraxia decreasewith relaxation.
Theyalso
Vocal frequentlyattenuate
duringabsorbing
Sniffing Echolalia
activities.
Ticsarebrieflysuppressible,
Snorting Palilalia
althoughattemptsto do so frequentlyresult
Coughing Coprolalia
in an"inner(emotional)tension,"
whichis
Throatclearing
relieved by "releasing"
theric.Despitethese
Grunting
facts.it is important
to recognize
thattics
Barking
areaninvoluntary
movement
andarenot
causedby stress.

ReachDr Kochat503-194-5856,
or
through
OHSUConsuh, 503-494-4567
or 1-800-245.6478.
TicClassification Oncethediagnosis
made,an attempt
of ticshasbeen
t0 classifythedisorder
Ticsaretypicailyclassified
aseither
mustbe undertaken. Thisapproach divides
motoror vocal.Bothmotorandvocaltics
tic disorders
into threegroups(Table2):
canbe either"simple"or "complex"t (Table
. "transient" (lastinglessthanoneyear)
l), Themostcommonsimplemotorticsare
. "chronic"(lastingmorerhanI year)
repetitive
eyeblinking,headtwitchingor
. "nonspecific"
thrusting,
shouldershrugging,
andmouth
Transient
tic disorder
is themildestand
opening.
Complexmotorticsconsistof
mostcommontic disorder occuning in up to
motoractionssuchasfacialgrimacing,
25 percent
of schoolchildren r. Transient
touching, jurnping,echokinesis
smelling.
ticsaresimpleticsandusually
of a motor

21
 malesthanfemales (3:l).Themeanageof beennotedin 50percent
of childrenwith
Table
2 6 to 7 yearsof agewith
onsetis between andmayprecede
Tourette's theonsetof the
TicSyndrome
Classification mostpatientspresenting
beforeagel3 yeus. tics.
I. Transient
TicDisorder Eyeblinking,facialgrimacing
or head
(duration
< I year) twitchesarethemosrcommon
initialtics. Determining
impact
AlthoughTourette
syndrome
hasbeen Determining
themajorsourceof
IL Chronic
TicDisorder
thoughtto bea life-iongdisorder,
in 30 to difficultyis essential
beforeanyspecific
(duration
> I year)
40 percentof childrenwithTS all tic recommendations
andtreatrnent
canbe
A) ChronicSingle
Tics
will disappear
symptoms by lateadoles- instituted.
Ticsrarelycausesignificant
B) Chronic
MultipleMotoror
cenceandanadditional will
30 percent physicaldiscomfort
or damage,however
VocalTics
experience The
a markedimprovement. theiradverse
effecton psychosocial
C)TouretteSyndrome
remaining patients
will havesymptoms development
andinterpersonal
relationships
III.Nonspecific
TicDisorder s. At present
persistintoadulthood thereare canbe immense.
no reliableprognostic that
characteristics It is essential
for thecareproviderto
permitidentification
of thosepatients
who inquirenotonlyaboutthenumber, intensity
variety,although
transient
vocalticscan
will havespontaneousimprovementin their andfrequency
of thetics.butalsoto
occur.Dueto theirmild andbenignnature,
symptoms.
Remission
appears
to be question
thedegreeof impairment and
treatment
is notnecessary
or recommended. independent
of theuseof medications. impacttheyhaveon thechild'sactivities
Chronictic disorders
includethree
Nonspecific
tic disorder
is a category
for andsocialinteractions.
It mustalsobe
subtypes:
chronicsingletic disorder,
childrenwhodo not meetthecriteriafor a determinedwhetherthereareanyassociated
chronicmultiplemotoror vocaltic disorder
specifictic disorder.
lncludedin thisgroup problems suchasattention-deficit
hyperac-
andTourettesyndrome.
Ckonic singletic arechildrenwhodevelop
ticsin response
to tivity disorder,
obsessive-compulsive
disorderrequirestheoccunenceof onlya medicationssuchasstimulants
or neurolep- disorder
or behavioral
difficulties.
singlemotor,or lesscommonly,vocaltic
tic withdrawal
andthosewhohaveonsetof Aftera complete
assessmentthecare
for morethanoneyear.Chronicmultiple
theirticsfollowingan acuteinsultsuchas providershouldeducate
thepatienrand
(CMTD)consists
tic disorder of multiple herpesencephalitis.
Therecently
described familyaboutthedisorder
anddehnethe
tics,eitherall motoror lessfrequently,
all (Pediatric
PANDAS AutoimmuneNeuro- targetsymptoms
thatneedto be addressed.
vocal,butnotboth.occuningoveroneyear,
logicDisorders with Streptococ-
Associated Justbecause
a symptom
is present
doesnot
Several
studies
haveshownthatCMTD 6.
calinfection)mayalsobein thiscategory meanit requirespharmacotherapy.
tendsto be a mild form of Tourette
syndromeandthatbotharetransmitted
inherited a.
traitsin thesamefamilies
as problems
Gomorbid
A varietyof comorbidbehavioral
Tourettesyndrome(TS)is a chronic,
problemsareidentified
in childrenwith
Table
3
complex,
fluctuating
tic disorder
of ?.Thetwomost
Tourette
Syndrome
Criteria
Tourette
syndrome
variableseveritycharacterized
by both
prevalentandoftenproblematic
are l. Onsetpriorto 2l yearsof age
motorandvocalticsof bothsimpleand
obsessive-compulsive
disorder
and 2. Presence
of multipletypesof
complextypes.Cunentessential diagnostic
attention-defi
cit hyperactivity
disorder. motortics
criteriafor thediagnosisof Tourette
Additionally, speech
learningdifficulties,
syndromeinclude(Table3): 3. Presence
of oneor moretypeof
andlanguage andsleepproblems
disorders
. onsetbeforeage2l years, vocaltic
havecommonly
beenobserved.
. multiplemotortics.
Commoncompulsive
symptoms
include 4. A waxingandwaning
course
. oneor morevocaltics.
orderingandananging
habits,checking
. a waxingandwaningcourse, 5. Gradualreplacement
of old tics
rituals,frequent
counting.andritualsto
. thegradualreplacement
of old tics with with newones
decontaminate or bodypartssuchas
objects
newones,
handwashing.
Obsessir,e-compulsive 6. Absence
of othermedical
. theabsence
of othermedicalexDlana.
behavior
hasbeenreported in morethan40 explanation
for tics
tionsfor tics.and t.
of childrenwithTourette's
percent
. durationfor morethanoneyear, 7. Durationfor morethan1 year
Attention-deficit disorder
hyperactivity has
Tourette
syndrome
is morecommonin

22
Treatment
0ptions antagonists
havebeenusedwithvariable tic disorders.
Thespectrum
is wide.ranging
Thepharmacologic treatmentof tic success.Clonidine
is etfective
in treating frombenigntransient
simplemotorticsto
disorders
is stricrlysymptomatic
andnot ticsin approximately
50 percentro a Tourette
syndrome. Recognition of this
curative.
Treatment shouldbereserved tbr somewhat overlyoprimistic
70 percent
of spectrumandfamiiiariiywith tic classifica-
thosechildrenwith signiticant patients e.
withTourette's
ticscausing tionandtic syndrome
classification allows
psychosocial
or functionallydisabling Whileitsefficacyis lessthanthe thecareprovider
to properlyeducate
the
problems.Thegoalis to reducetheticsto a (80percent),
neuroleptics it is frequently familyandultimarely determine whether
tolerable
level,realizingthatinadication theinitialmedication
prescribed
dueto a themajorsource of distress
is relaterJ
maynotbepossible.
Whilealternative relatively
benignside-ettect
profrle.It can directlyt0 ticsor associatedbehaviors.
nonpharmacologic
treatmentshavebeen be administered
asa tabletor trans-dermal Oncethishasbeendone,thenan
used,including
conditioning
techniques, patch.An alternative
alpha-adrenergic individualized
multimodal
treatmentplan
relaxation.
biofeedback
andhypnosis, agonists,
guanfacine
(Tenex),
maybe tried canbedeveloped.A valuable
resource
for
pharmacotherapy
is mostfrequently if behavioral
problemsareprominent. additional
informationfor careproviders,
employed. Neuroleptics
arethemosteffective
tic- parentsandpatients
is thenonprofit
At present
therearetwo mainclasses
oi suppressing
agenrsalthough
theirside- voluntary
TouretteSyndromeAssociation.
medicationsto suppress
tics:alpha- effectprofilemaylimit theiruse.Themost Newyork,
42-40BellBlvd.,Bayside.
adrenergicagonistsandneuroleptics
Oable commonly usedagentsinclude:haloperidol, (718)22+-2999:
I l36l-2871; andon the
4). Otheragentsincludingbenzodiaz- pimozide andtluphenazine.
Theyall appear Internetat t0urotte@
ix.netcom.com.
epines,calciumchannelblockers, to be approximarely
equipotenr
in suppress-
catecholamine-deplering
agentsandopiate ing thetics. Selected
references
In children,
generally
pimozide
and l. Singer HS,Walkup JT.Tourerte syndrome and
fluphenazine
areusedbeforehaloperidol otherticdisorders:Diagnosis, pathophysiol-
Table
4 becausetheyhavea slightlylower
ogy,andtrearmenr. illedicinel99t:70:15-12.
Treatment
ofTicsand incidence
of side-effects
andaretolerated
2.Kurlan R,LichterD,HewittD.Sensory tics
Comorbid
Problems in Tourette's
svndrome. Neurology
somewhat
better. 1989;19:73l-34.
Tics Treatment of anycomorbid 3.Shapiro AK,Shapiro ES,Young JG,Feinberg
problems
Alpha-Adrenergic
agonists TE.Gillesdela Tourette Syndrome. 2d ed.
needsto beundertakenwith careful NewYork:Raven Press. i988.
Clonidine consideration
to thetotalpatientcare.The 4.Pauls DL,Cohen DJ,Heimburch R,etal.
Guanfacine
{Tenex) approach
to theattention-deficit
hyperactive Familialpatternandransrnission of Gilles
delaTourene syndrome andmultiple tics.
Neuroleptics disorder
is somewhat
controversial
because
ArchGenPsychiatn 198I ;38:109I -93.
Pimozide(Orap) psychostimulant
medications
mayprovoke 5.Erenberg G,Cruse Rl Rorhner AD.The
(Prolixin)
Fluphenazine or intensifytics.If behavioral
andeduca- naturalhistory
of Tourette syndrome: A
tionalapproaches fail.desipramine and follow-upsyndrome. AnnNeurol
(Haldol)
Haloperidol
1987:22:383-85
alpha-adrenergicagonists maybetried.If
Attention.DeficitHyperactivityDisorder 6.Swedo SE,Leonard HL,Garvey M, etal.
necessarya brieftrialof centralstimulants Pediatric
Autoimmune Neuropsychiatric
Behavioral
andeducationalinterventions DisordersAssociated withSrreptococcal
canbe undertaken withcarefulattentionto
tic exacerbation. Infections:
Clinicaldescriprion of thefirsr
Pharmacologic
treatment
50cases.Aml Prychiatn 1998;155:264-
Desipramine In Tourette
patienrs
wirhdisabling 2'tr.
Alpha-Adrenergic
agonists obsessive-compulsive
disorder,
additional 7. StefllvlE.Illentalhealthneedsassociated
with
Clonidine psychiatric
intervention
andbehavioral Tourettesyndrome. Am J PublicHeulth
1984;74:1310-13.
(Tenex)
Guanfacine modification
techniques
shouldbe
8. SingerHS,Rosenberg LA. Thedevelopment
Centralstimulants considered.
Twopsychotropic
drugs, of behavioral andemotional problemsin
Ritalin clomipramine andfluoxetine,
havebeen Tourettesyndrome. PediatrNeurol
helpiulin treatingsevere
obsessive- 1989;5:41-44.
Dexedrine
9. GoetzCG,Tanner CM, WilsonRS,et al.
(Cylert)
Pemoline compulsive
manifestations.
Clonidine andGillesdela Tourerre
syndrome: Double-blindstudyusing
Obsessive.Compulsive
Disorder
(Prozac)
Fluoxetine
Conclusion objectiveratingmethods.AnnNeurol
1987;ll:307-10.
Physicianawareness
is thekeyto
(Anafranil)
Clomipramine
properlydiagnosing
andtreatingpediatric

23