Full text of DOI:10.1177/088307380201700321

2
41
since the mild to moderate pressure elevation
infarction in an older adult is not the only mechanismleading to
dysarthria-clumsy hand syndrome. (J Child Neurol 2002;17:
developed slowly,
nociceptivepathwayswerenotactivated. It is clear, however, that
theapparentlackof
symptoms, eitherpositive (pain, vomiting) or
241-243).
negative (lethargy, apathy)in
otherwisenormal
young
childrenwith
suspectedpapilledema, doesnotrule outraisedintracranialpres-
sure. Therefore, sedatedlumbarpunctureshould
beperformedto
accurately determine cerebrospinal fluid pressure.
Dysarthria-clumsy handsyndromewasfirst describedbyFisherl
in 1967.
Autopsyin one ofthethreereportedpatientsrevealed an
SpencerG. Weig,
MD
ischemic infarct in the basis pontis. All three patients were over
Departmentof Neurology
AlbanyMedical College
Albany, NewYork
6
5
years of
andhad chronicmedical
age
problems.
Additional cases have been described with infarction in the
and/orposteriorlimboftheinternal
ofthese cases have involved lacunar infarcts in older adults. We
Themajority
genu
capsule .2-6
Received Oct24, 2001. Acceptedfor publication Dec 14, 2001.
a
15-year-oldboywith
centralnervoussystem
vasculopathy
report
Presented in part at the Child Neurology Society Meeting in Montreal,
Quebec, October 1998.
whopresented with dysarthria-clumsy hand syndrome owing to
internal
capsule infarction.
Addresscorrespondenceto DrSpencerG.
(
5
Departmentof
Neurology
Weig,
MC70), AlbanyMedicalCollege, Albany, NY12208. Tel: 518-262-6566; fax:
18-262-9985; e-mail: weigs@mail.amc.edu.
CaseReport
A
previously well 15-year-old
right-handed
boy complained of difficulty
speaking, swallowing, andusinghisrighthandtowriteandpracticethepiano.
Hewasunable to give an exact time ofonset. Thenext dayhepresented
to the local emergency room, but was discharged home by
whofelt thathis neurologic examinationwasunremarkable.
References
a
physician
1
.
BakerRS, BaumannRJ, BuncicJR: Idiopathic intracranial hyperten-
sion (pseudotumor cerebri) in pediatric patients. Pediatr Neurol
Thefollowing dayhe presentedto his familyphysician, whoreferred
himtoourhospital. Hewasafebrile. Bloodpressurewas124/76mmHg. Skin
showedthreesmallcaf6-au-laitspotsbutnorashes. Cardiacand jointexam-
inationswereunremarkable.Thepatientwasalertandorientedandwasable
tonamesimpleobjects, buthadmilddysarthria, withdifficultyformingcom-
plex syllables.
He had slight flattening of the right nasolabial fold. Motor
strengthandsensationwereintactbilaterally. Fingertonose,rapidalternating
movement,andtandemgaitwereintact, butsequentialfingertapwasslower
1989;5:5-11.
2
3
.
.
LesselS: Pediatricpseudotumorcerebri(idiopathic intracranialhyper-
tension). Surv Ophthalmol 1992;37:155-166.
SalmanMS, KirkhamFJ,
MacGregor
DL: Idiopathic
"benign"
Child Neurol 2001;16:
intracra-
nial hypertension: Case series and review.
65-470.
WallM:Idiopathicintracranialhypertension.NeurolClin 1991;9:73-95.
review of79 cases in
J
4
4
.
.
5
Grant DN: Benign intracranial hypertension:
A
onthe rightthanontheleft, andhehaddifficultywith
Reflexes
handwriting.
werebriskbilaterally at 3+. Plantarreflexeswereequivocal.
infancyandchildhood. ArchDisChild 1971;46:651-655.
6.
7.
8.
Youroukos S, Psychou F, Fryssiras S, et al: Idiopathic intracranial
Reviewofsystemsrevealednohistory ofheadornecktrauma,fevers,
joint pains, rashes, or unexplained weight loss. He had had chicken pox
morethan 10 yearspreviously. Hedeniedrecreational druguse andsexual
hypertension in children.
JChildNeurol2000; 15:453-457.
McGrathPJ, McAlpineL: Psychologicperspectivesonpediatricpain.
J Pediatr 1993;122(Suppl):S2-S8.
FranckLS, GreenbergCS, StevensB: Painassessmentin infants and
children. PediatrClinNorthAm2000;47:487-511.
activity.
Laboratory results were remarkable only for
throcyte sedimentation rate of 15 mnvhr, an elevated triglyceride level of
.81 mmol/Lwithnormaltotal cholesterollevel, andanelevated eosiniphil
level of60%, whichnormalizedonrepeat testing dayslater. Theremain-
derofthecompletebloodcell count, serumelectrolytes, liverfunctiontests,
creatine phosphokinase, C-reactive protein, C-3C complement, C4
a
mildly elevated ery-
3
2
level,
serum immunoglobulins, protein C, protein S, antithrombin, fibrinogen,
partialthromboplastintime, andinternationalnormalizedratio wereunre-
markable. Screeningforanti-double-strandedDNA,anticardiolipinantibody,
andlupus anticoagulantwasnegative. Lumbarpunctureshowednored or
whitebloodcells, glucoseandproteinwerewithinnormallevels, andbac-
terial cerebrospinalfluid cultures hadnogrowth. Echocardiograminclud-
ing injection of agitated saline to screen for patent foramen ovale was
unremarkable.
A
15-Year-Old
BoyWithCentral
Nervous
SystemVasculopathyPresentingWith
Dysarthria-Clumsy
Hand Syndrome
ABSTRACT
Computedtomographicscandemonstrated
a
hypodenseareainvolv-
ing the genu and posterior limb of the left internal capsule. Diffusion-
weightedmagneticresonanceimagingdemonstratedhyperintensityinthe
sameregion. Magneticresonanceangiographyandconventionalangiography
revealedfocalstenosis ofthesupraclinoidportionoftheleft internal carotid
Dysarthria-clumsyhandstroke syndromehasbeendescribedfre-
butnotin children. Wereport
15-year-old
handedboywithsuddenonsetofdysarthria, dysphagia, rightfacial
weakness, andmildright-handclumsiness. Computedtomographic
scanandmagnetic resonanceimagingdemonstratedinfarctionin
quentlyin adults
a
right-
artery
andthe origin ofthe left ophthalmic artery (Figure 1). Angiography
ofthe renal arteries wasunremarkable.
The patient wastreated initially with low-molecular-weight heparin
the
andposteriorlimb ofthe left internal capsule. Magnetic
genu
andwasswitchedto aspirin (80mgdaily) andhigh-doseprednisone(60
mg
resonance angiography and conventional angiography demon-
strated stenosis of the supraclinoid portion of the left internal
carotidarteryandthe origin oftheleft ophthalmicartery. Lacunar
daily) afterthe angiography
onhospitalday6. Hisneurologic examination
normalized over several weeks. Prednisone wastapered, butaspirin was
continued. At 10 weeksafter he remainedsymptomfree.
discharge,
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2
42
Discussion
appeartorespondwelltoprednisone,
althoughit is unclearifpred-
Dysarthria-clumsyhandsyndromehasbeenwelldescribedinthe
adultliterature&dquo; butnotinthepediatricliterature. InFisher’s3 clas-
nisone
Calabrese and colleagues9uo have suggested therapy with
courseofhigh-doseprednisoneforseveralweeksfollowedbyrapid
Moremalignantformsof vasculitis, suchasprimaryangiitis
is actuallyrequired; fewuntreatedcaseshavebeenreported.
a
brief
.
sic definition,
taper.
ofthe central nervous system, mayrequire
immunosuppressivetherapywithprednisone
a
prolonged course of
andcyclophosphamide
.
..facial
weakness, severe
binedwith weaknessandclumsinessofthehand
dysarthria, and
dysphagiaare
com-
slight
(notice-
to prevent disease progression.9,13--15 It canbe verydifficult to dif-
ferentiate betweenbenignandmalignantformsof vasculopathyin
theearly stages,s-1° andthesepatientsrequirecloseclinicalfollow-
up. Thispatient’s presentation
able, for example, in writing), brisk tendon reflexes, and
a
’
Babinski
onthesameside. The
responsiblelesionis either
sign
in the basis pontis...or...at the
ofthe internal
genu
capsule...
wasfelt to bemostconsistentwith
There is no
deficit.
sensory
benign cerebral
vasculopathy. Prednisone wasdiscontinued after
briefcourse. Aspirinwascontinued. Inbothbenignandmalignant
forms of cerebral vasculopathy, delayed diagnosis and delayed
onset oftherapymayleadto pooroutcome.8-16
Presentationwithnew-onsetdysarthriaandmildhandclum-
siness warrants further investigation with
magnetic resonance
theneedtoconsiderstrokeinthe dif-
a
Ourpatient’s
andthe location ofhis infarct were
symptoms
consistent with Fisher’s description. The rarity and subtlety of
presentation in
children mayhave contributed to the missed
diagnosis at
this
the outside
hospital.
Fisherl3
suggested that dysarthria-clumsy
hand
syndrome
Thiscase
imaging.
highlights
wasthe result of
a
lesion ofthe corticospinal tract at the ponsor
probably a variant of ataxic hemi-
paresis. Spertell and Ransom2pointed outthat morerecentwork
ferential diagnosisofchildrenpresentingwithacute onsetofneu-
the internal capsule and was
rologic symptoms.
has
suggestedthat
the
corticospinalfibers
runinthe
posteriorthird
ofthe internal
so
a
lesion at the
wouldbe
capsule,
genu
with
unlikely
Acknowledgments
TheauthorsthankDrDerek
to affectthem.
Theydescribed patient
a
thissyndromeowing
Armstrongfordiscussionof radiogaphicissuesand
to aninfarct ofthe
and
postulatedthat
the
symptoms
resulted
MsSelina Hune, NP, for heraid in the care ofthis patient.
genu
frominjury to the anteriorthalamic radiation andthe
frontopon-
tine tract. The exact mechanism leading to these symptoms was
notclear.
Meredith R. Golomb, MD
Shelly K. Weiss, MD
ShahnazH. Ibrahim, MD
Gabrielle A. deVeber, MD
Division of Neurology
InFisher’s’,3 originalarticles, theinfarctsleadingto dysarthria-
clumsyhandsyndromewereusuallycausedbysmallpenetrating
brancharterydiseasein olderadultswith
a
historyofhypertension.
However, in some patients, the artery supplying the infarcted
region waspatent, suggesting that the infarct wasthe result of
Department of Pediatrics
The
a
HospitalforSick Children
smallembolusfromtheheartorfrom
a
thrombusin
a
largerparent
Ontario
Toronto,
artery. Browerandcolleagues’ described 36 children withinfarc-
tion ofthe internal capsule, basal ganglia, orthalamus andfound
that the most common lesion demonstrated on
Received Nov20, 2001. Receivedrevised Jan24, 2002. Acceptedforpubli-
cation Feb 7, 2002.
magnetic reso-
Thisworkwasdoneat TheHospital for Sick Childrenin Toronto, Ontario,
Canada. Thefirst authoris supportedbyagrantfromtheOntarioFederation
for Cerebral Palsy.
nance
was
a
large vessel occlusion with secondary
occlusionofdownstreamsmallpenetratingvessels. Ourpatientwas
anadolescentwithnohistory ofhypertension, normalheart on
andstenosis ofthe ipsilateral internal carotid
angiography
a
Address correspondenceto: DrMeredith R. Golomb, Section ofPediatric
Neurology, Riley Hospital for Children, Room 1757, 702 Barnhill Drive,
Indianapolis, IN 46202-5200. Tel: 317-274-8757; fax: 317-274-3622; e-mail:
echocardiography,
artery. Themostlikelymechanismleadingtostrokeinthispatient
wasembolization from
small thrombusat the internal carotid.
Thecombinationoffocalstenosis
the supraclinoidportion
of the internal carotid artery together with other stenotic cere-
mgolomb@earthlink. net.
a
m
References
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1
.
Fisher CM:Alacunarstroke: The dysarthria-clumsyhandsyndrome.
Neurology 1967;17:614-617.
RansomBR:
brovascularlesionsmaybe
a
signof
a
relativelybenigncerebralarte-
2.
SpertellRB,
Dysarthria-clumsy
hand
syndromeproduced
or of
a
more malignant cerebral vasculitis.8-1O,13-15
bycapsularinfarct. AnnNeurol 1979;6:263-265.
Fisher CM: Lacunarstrokes and infarcts:
review.
2:871-876.
riopathy$w2
3
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A
Neurology
1982;
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3
opathyinyoungchildren
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4
Chamorro
Sacco
Mohr
JP,
etal:
A,
RL,
Clinical-computedtomographic
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and
alonemay
beenoughtopreventrecurrent stroke. Cal-
1991;22:175-181.
aspirin
5
.
.
Schonewille
Tuhrim
S, Singer MB, Atlas SW: Diffusion-weighted
correlation
WJ,
abrese and colleagues9,1° and others&dquo;,&dquo; have described benign
angiopathyofthecentralnervoussystemthatoccursinpatientsrang-
ingfromadolescenceto middleadulthood. Thesepatientspresent
MRIin acute lacunar
A
syndromes.
clinical-radiological
study. Stroke
1
999;30:2066-2069.
6
Urban
Wicht
Vukurevic
G, et
al:
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acute ischemic
PP,
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S,
clinicoradiologic correlation, and etiol-
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ogy. Neurology 2001; 56:1021-1027.
cerebrospinal fluid cell
ofother systemic disorders
counts and
protein
levels, and have no
7.
BrowerMC, Rollins
RoachES: Basal
andthalamic infarc-
N,
tion in children. ArchNeurol
ganglia
signs
predisposingto
vasculitis. They
1996;53:1252-1256.
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8
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Ferro JM: Vasculitis of the central nervous system.
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CalabreseLH, FurlanAJ, GraggLA, RoposTJ: Primaryangiitis of the
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Cleve Clin JMed1992;59:293-306.
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Lanthier S, Lortie A, MichaudJ, et al: Isolated angiitis ofthe CNSin
children. Neurology2001;56:837-842.
1
1
0.
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CalabreseLH, GraggLA, FurlanAJ: Benignangiopathy:
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Adistinctsub-
1
1
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Lopez-YunezAM, GargBP: Noninfectious cerebral vasculitis in chil-
dren. Semin CerebrovascDis Stroke2001;1:249-263.
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Neurol 1978;35:612-614.
andfuture directions. SeminPediatrNeurol
Recognition, treatment,
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Correspondence
Late-Onset orPersistent Febrile Seizures
clonic seizures in childhood with preceding febrile seizures. It
be
may
equallytrue offebrile seizurepluswithotherseizuretypes.
To the Editor: The article byVerrotti et all
describing children in
whomfebrile seizurespersistedbeyondthe age of years orwho
hadtheir first febrile seizure after the age of years makesinter-
Late-onsetfebrile seizuresnotonlyleave the
It is obvious that there is
a lot ofresemblance between the
5
patients described by Verrotti et all and those with generalized
epilepsy with febrile seizures plus,2,3 particularly with regard to
seizure onset, persistence ofseizuresbeyondthe age ofexpected
remission, typeof afebrile seizure, andfindingsonneurologicand
psychomotorevaluation. Therefore, the observationsof Verrotti et
all that children with late-onset or persistent febrile seizures
5
estingreading.
diag-
nosisoffebrile seizuresin doubt, theyalsocauseparentalanxiety.
The studyhas established that such children probablyrepresent
a
of patients at greater risk of developing unprovoked
group
seizures. It alsofoundgrandmalseizurestobethemostfrequent,
beyond the age of remission represent
a
distinct group with
withabsenceandcomplexpartialseizuresaccountingfortherest.
enhancedriskofdevelopingafebrile seizurescomparedwiththose
with classic febrile seizures provide further credence to the con-
ceptofgeneralizedepilepsywithfebrile seizuresplus. Morestud-
ies willhelpto strengthenordisprovethis conceptandto delineate
the true spectrumofthe disorder.
All ofthe children had
a
normal
examinationandnor-
neurologic
malpsychomotordevelopment.
Thepatients reportedbyVerrotti etal’ areverysimilartothose
epilepsy with febrile seizures plus.2,3 This term,
with
generalized
first usedbyScheffer and Berkovic2 andlater expandedby
Singh
a wide
et al,3 described
a
new genetic epilepsy syndrome with
Jatinder S. Goraya, MD
DepartmentofPediatrics
GovernmentMedicalCollegeHospital
Chandigarh, India
spectrum of clinical epilepsy phenotypes. These phenotypes
included classic febrile seizures, febrile seizures plus, febrile
seizuresplus withother seizures (absence, myoclonic,
or
atonic,
complexpartial), andmyoclonic-astaticepilepsy. Thefebrileseizure
plus, the
mostcommonphenotype, defined the group ofchildren
fromfebrileseizuresextendingbeyondthe
of6
withorwithoutassociatedafebrile generalizedtonic-clonic seizures.
References
suffering
age
years,
1.
Verrotti A, Giuva T, Cutarella R, et al: Febrile convulsions after
of Neurol
2000;15:811-813.
5
years
Scheffer
age: long-term follow-up. Child
J
Nearly all
of the patients with generalized
epilepsy with febrile
normalintellect andnormal
2.
Berkovic SF: Generalized
withfebrile seizures
clinical phenotypes.
IE,
epilepsy
plus.
Brain 1997;120:479-490.
A
genetic disorder with
heterogeneous
seizuresplus
had
a
exam-
neurologic
phenotype of febrile
seizureplusprobablyaccountedformanychildrenwithout
cific epilepsysyndromediagnosiswhohadhadgeneralizedtonic-
ination. The authors even opined that the
3
.
SinghR, SchefferIE, CrosslandK, BerkovicSF. Generalizedepilepsy
with febrile seizures plus: commonchildhood-onset genetic syn-
drome.AnnNeurol 1999;45:75-81.
a
spe-
A
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