ELSEVIER
The adult classificatiorn of
van Mourik M, Catsl7ian-Herrcv~~cts CE,.
Neurol 1997; I7:299-307.
Dysarthria is a motor speech deficit in neurologic
disorders. Rate, strength, and coordination of the muscles
subserving speech may be impaired to different degreec.
affecting articulation, prosody, resonance, respiration. and
phonation. Darley et al. [ I.21 analyzed speech in 2 I2 adult
patients. Deviant speech features occurred in clusters.
From the +Department of Neurology; University Hospital imd Erilsmu\
Unikcrsity: Rotterdam: ’Department of Medical Psychology:
Ziekcnhuis Walcheren Vlissingen: Vlissingen: The Netherlands:
‘Division of Applied Neurolinguistics: Department of ENT Surgery:
School of Medicine: University of Antwcrp (UlA); Antwerp: and
‘Department of Neurology; University Hospital Erasme; Free
University of Brussels (ULB); Brussels. Belgium.
0 1997 by Elsevier Science Inc. All rights reserved.
PII s0887-8994(97)0008 1-7 * 0887-8994/97/$17.00
characterizing major types of dysarthria in adults: ataxic,
flaccid, hypokinetic, hyperkinetic, and spastic. The most
distinct features of each type, as presented by Darley et al.
[2] are presented in Table 1. Darley et al. [ 1.21 concluded
that these distinctive patterns have localizing value and
can assist in identifying the underlying neuropathology, as
the deviant speech features mirrored the kind of motor
disorder in limbs and trunk.
Other studies in adults have modified the list of prom-
inent speech features. mainly for ataxic dysarthria [3-61.
Enderby et al. [7] criticized the typology of dysarthria
because pyramidal and corticospinal tracts are modulated
by extrapyramidal and cerebellar circuitry prior to excita-
tion of the final common pathways. Consequently, simi-
larities between dysarthrias with different lesions are
expected. Despite this criticism, the “adult model” ot
distinct dysarthria types [ I.21 and their correlation with
pathophysiology are widely accepted.
Acquired childhood dysarthria (AC
a rare disorder, although its overall incidence or its
prcvalencc in pediatric neurology ih not known. Despite its
frequent occurrence in the pediatric population, ACD has
refer to dysarthria in terms of underlying motor disorder
(spastic, ataxic) or neuroanatomic site of damage (c
bellar, bulbar). but provide no systematic analysis of
speech features. Thus. these studies implicitly assumt”
J:[+)r the purpose 01‘the prc\cnt stud). WC define ACD ;I\ a motor speech
diq)r&r due to t-trail1 le&ns iicqulrcd in childhood after normal onset 01
speech &velopment. WC cxciudr 4pcc’chdi\ordcr\ in d~~dopnlenta~ id
degeneriitivc disorder\.
Communications should be addressed to:
Dr. van Mourik; Department of Medical 1’sychology/Zieh~nhui~
Walcberen; Postbus 3200: 4380 DD Vlis\ingcn. The Ncthcrland\
Received October 2 I. 1996: acceptedDecember 6. 19%.
van Mourik et al: Acquired Childhood Dysarthria 399
Table 1. Comparison of features in adult dysarthria types with cording to the Mayo Clinic List [ I ,2]) soon after onset of
pediatric cases the speech problem. These speech features are indicated
by quotations in the text. These studies are summarized in
Studies Reporting
hlost Distinctive Speech Features in Features in Tables 2 to 5. To illustrate a clinical context in which ACD
Adult Dysarthria Types 121 Children may occur, we sometimes refer to cases with nonspecifi::
indications such as “slurred” or “dragged” speech. but
Ataxic dysarthria
these cases are not included in Tables 2 to 5. Fourth. cases
lrreguhlr breukdown of articulation
Excess and equal stress
were excluded when the speech disorder was a mixture of
Prolongations of phonemes and interval5 dysarthria and aphasia or verbal apraxia, as this obscures
Slow rate the very nature of the dysarthria. (For differential diagnos-
Harsh voice
tic issues, we refer to other studies [S-IO].) We classified
Monotomy
Flaccid dysarthria on the basis of neuroradiologic evidence of lesion site and
Hypemasality [Xl associated motor disorder, trlereby classifying according to
..
Nasal emission of air _:!:
the motor subsystems.
Continuous breathiness 12sj
Audible inspiration _:k

Hypokinetic dysarthria AGD in Relation to Site of

Monopitch
_:/:
_:,: Impairment
Mono-kudness
Hypophonia
_ :.:
Imprecise articulation [333.361 Cereheihr Lesions. Speech features of cerebellar dys-
Variable rate 133,351 arthria in adults are described in Table I. In children.
Short bursts of speech (stuttering) 133.351 cerebellar tumor resection may cause a syndrome of
Quick hyperkinetic dysarthriu (chorea)
mutism with subsequent dysarthria: MSD. This clinical
Hypernasality
Harshness _:j picture has been described in a greater number of children
Breathiness -1:
than has ACD associated with any other lesion site. The
Loudness variations primary site of lesion is the cerebellar vermis. but the
Slow hyperkinetic dysarthriu (dystonia)
Imprecise articulation
tumor and the effects of surgery may affect adjacent
Hypophonia structures.
Variable rate Most studies refer to type and severity of dysarthria in
Harshness plus strained strangled voice
_ :i: the acute stage after recovery from mutism. In 20 cases,
Transient breathiness plus audible
Inspiration
speech was judged according to the Mayo Clinic List of
Spustic dysnrthrin speech features by experienced professionals or speech
Iniprcckc ilrlicllliltion _:!:
pathologists. These studies are presented in Table 2. All
I
SlOW rilk!
children with MSD 1I 1-231 had severe limb and trunk
Low pitch _ 4:

tlilPit1 voice _I ataxia. “Slow articulation,” “monotony,” and “hoarse

Strnincd SWungled voice -_
Pitch breaks _’
Reduced \tre\\ _.!
I’Not reported in children.
appropriateness of the adult model of dysarthria for
children.
Therefore. we (a) surveyed the literature on speech
features in ACD in relation to lesion site and motor
dysfunction in order to discuss the validity of the adult
model, i.e., the existence of distinct dysarthria types in
childhood: and (b) reviewed studies in which the analysis
of ACD is of importance for clinicai management.
Inclusion and exclusion criteria were as follows. First,
we searched Medline databases from 1980 to 1996 for
publications available in English under the search strate-
gies “dysarthria” or “speech disorders.” We selected,
reviewed, and analyzed studies pertaining to acquired
dysarthria. Second. we included only descriptions of
children aged 16 years or less. Third, we included studies
mentioning perceptual judgment of speech features (ac-
1. soft voice” were the most frequent speech abnormalities.
Irregular articulatory breakdown and excess and equal
stress resulting in scanning speech. which are most prom-
inent in ataxic speech in adults (Table I), were certainly
not the hallmark of ACD in these children. Nagatani et al.
I IS] and Al Jarallah et al. [ 191 explicitly mentioned that
speech was “not explosive nor scanning.” van Dongen et
al. [ 181 reported that the observed speech features did not
fit into any specific cluster of deviant speech dimensions.
In the course of improvement, there was marked dissoci-
ation between speech impairment and motor disability:
The initially severe dysarthria recovered (almost) com-
pletely while ataxic motor impairment persisted in most
children.
We could find only two reports on speech characteris-
tics of ACD after cerebellar damage resulting from a
different etiology (Table 3). Echenne et al. 1241 reported
swallowing difficulties, velar paralysis, and “dysphonic.
hoarse speech” of short duration after two episodes of
vomiting, headache, and dizziness (Table 3). Computed
tomography scan demonstrated an area of decreased den-
sity in the lateral part of the left cerebellar hemisphere,
caused by a filling defect of the distal portion of the basilal

300 PEDIATRIC NEUROLOGY Vol. 17 No. 4

 Patient Lower
SexlAge Cranial
Reference (y) I,csi011 Site Motor Disorder Nerves

Rekate et al. 1I II F/8 Medial Limb ataxia. R paresis N Slow. monotonous

Volciln et al. 1I z ] FIX Medial Trunk aturiit. R paresi\ L VI p;Lre4is Monotonou5. monos~llahic
Dictlc and Mickle 1I.31 M/7 RH and LH Trunk ;tt:rxi:: N Sparse. slow. monotonous
Ferrnnte et al. I I-II F/5 Medial Ataxic fait N Scannino
Nagatani et al.1 IS] F/-l Medial Trunh nta\ia N Slow. m~motonous. not e%plosivc
or scanning
Catsman-Berrevoet\ et al. II61 M/O Medial Trunk and L/R XII Audible respiration, incoordination
paresis paresis of respiration and speech. hoarse.
nasal. consonant distorsion
Herb and Thyen I I7 1 M/9 Media? R ataxia. R parcsis L/R VII Aphonia. slow articulation. no
paresis synchronization of articulation and
phonation
van Dongen et al. I I Xl FIX Meditil Trunh und limb titaxirl L VII Alternating loudness. flat vowels.
paresis. L consonant cluster reduction
XII paresis
van Dongen et al. I I Ki MIX Medial Trunh and limb ataxia. R L Xii p”resi4 Hoane. soft voice. omission on ot
paresis final sounds
van Dongen et al. I I Xl hi/S Medial Trunk Ltnd limb ittaxia Xl I weahness Slow rate. strained-strangled voice.
syllabification
!I Jlrrallah et al. II91 Medial N N Slow. monotonous. r10f explosive
C utchfield et al. j20) Medial Upper limb and trunk ataxia N Whispering
Ku oni;l et al. 12lI Medial L hemtpltresis L VII pares’, Slovv,. weak. monosyllabic
Kingna et al. 121 I Fourth v cnlriclc R :ttn\ia NR Slow speech rate
Pollacb et al. 1221 Medial L paresi N High-pitched nasal voice
Pollack $1 al. 1221 Medial plus L atrtxia L VI paresis Hi_rh-pitched voice
fourth v~entriclr
Pollrtck et .4. 1231 F:/h Medial plt.14 R Lttaxia N Whispering. monosyllabic. slow
fourth ventricle
Asamoto et al. I3 I FIX MeGial Dysmetria N Monotonou\. monosyllabic.
‘ t
sc3nnin~~

Ahhrcviation~:
F = Female
H = Hemisphere
L = Left
M = M~tle
MSD = Mutism with sub* xquent dysarthria
N = Normul
NR 7 Not reported
R = Right
-- -- --

artery. Dietze and Mickle ( 13t observed ACD after the We conclude that dysarthria subsequent to a mutt phase
resection of a cerebellar arteriovt.nous malformation, but after cerebellar tumor resection is frequently c
the features did not mble those ctf adult dysarthria after by slow speech rate. monotony. and hoarse soft voice.
ccrebellar lesions ( e 3). Excess and equal stress. distinctive for ataxic dysarthria in

Table 3. CPinical data of children with cerebektar lesions

:
ent
Age .ower Crania
il Gology Motor er Nerves S ~‘catures
Reference @I

Inftirction L c,rcbcllar H L hemichorelt and IX/X purcsis Dysphoniir.

Echenne et al. 1241 M/C)
ata\ia. R paresis ho;trscncs\
Dllflw l~yp”tonl~. N M~motc~llou\.
DietLe and Mickle [ 131 f-115 Kew.xlon ot arle! ~o\Lww

malformation R ;1ncrL bilateral and labored and

cerebellJr H truncal atilxiil bmdyhtnetic

pbbreviations:
Same ;1s in Table 7.

van Mourik et ;~l: Acquired Childhood Dysarthria 301

Table 4. Clinical data of children with brainstem lesions

Patient
Sex/Age Lower Cranial
Reference (Y) Etiology Motor Disorder Nerves Speech Features

Bak et al. 1251 Mlti Infarct L posterior thalamus L paresis L VII. L IX/X. Imprecise consonants, distorted
L XII pareses vowels, hypernasality. breathy
voice. harsh voice, monopitch.
mono-loudness
van Dongen et al. M/IO Fourteentla ventricle tumor Mild limb ataxia R IX. X. and Very soft voice

1181 XII paresis

van Dongen et al.
I181
Frim and Ogilvy (261
F/4 Posterior fossa tumor Flaccid tetraparalysis N Whispering a limited number
anterior to the basilar artery of words
F/8 Surgical resection of L paresis L VII weakness Dysarthria of speech prosody
pontine cavernous and rhythm
hemangioma

Abbreviations:
Same as in Table 2.

adults, were evident in only two cases (Table 1). In MSD,
dysarthria usually disappears, whereas ataxia persists in
most children for a considerable time. The results of the
cited studies do not sufficiently support the current prac-
tice of labeling dysarthria in MSD ataxic. Neither did
cerebellar lesions of a different etiology cause speech
features resembling ataxic dysarthria in adults.
Bmins~enz I,esions. Damage to the nuclei of the lower
cranial ilervcs in the bulbar region of the brainstem may
result in flaccid bulbar dysarthria. Prominent speech fea-
tures of adult bulbar dysarthria [ I,21 are described in
Table 1.
For the purpose of the present study, we excluded
diseases affecting the (peripheral) neurohmuscular junction
or the muscles such as in myasthenia gravis, muscular
dystrophy, or Guillain-Barr6 syndrome.
We reviewed studies presenting neuroradiologic evidence
of brainstem lesions associated with ACD (Table 4). Strokes
due to vertebral artery occlusion, a well-known entity in
adults, are rare in the pediatric population [27,28]. Older

Characteristics of children with hasal ganglia lesions

Patient ll,ower
Sex/Age Motor c('GlIIiill

Heference (y) Irtiology Disorder Nerves Speech I:eiltlllTS

Murdoch ct al. M/l3 t’OStillK~,XiC bililtcrill ClilliCill fcillllrCS L/R VII At 7 wk. impairment 01
(33] Striillcl-cupslll;u of parhinsonism paresis hilabial consonants. slow
lesions rate, and labored speech; at
IO wk. difficulty controlling
speech rate and impaired
initiation of speech
Al-Matecn et al. F/c) Inflammatory Dystonia. NR Hypophonic
1341 lesions of caudate choreiform incomprehensible utterances
nucleus. putamen, movements ol
and globus pallidus the extremities
Pran/ntelli ct ill. F/l? Anaphylactic shock Dystonia. NR After mutism. hypophonic
(351 bradykinesia. speech
rigidity
Pran/.atelli et ill. M/I-l Head trauma Dystonia. NR Stuttering speech
[351 hemiballismus,.
bradykincsia.
rigidit)
Aram ct al. (361 FIX VilSClllilr Icsion4 1. R paresi\ N Consonant cluster reduction.
globu~ pallidus. consonant omissions
putameft. posteriol
pill? of internal
capsule, caudate

Abbreviations:
Same as in Table 7.

302 PEDIATRIC NEUROLOGY Vol. I7 No. 4

studies reported “slow”, “slurred”, or “dragged” speech
127,291. One study reports speech features after occlusion of
the basilar artery in a 6-year-old boy [25]. ACD in this child
was associated with bulbar palsy. The speech pattern resem-
bled bulbar dysarthria in adults. Initially. computed tomog-
raphy scan showed no abnormalities, but some weeks later. a
small infarct in the left thalamus was demonstrated.
The clinical picture of mutism and subsequent dysar-
thria, frequently labeled cerebellar, has also been reported
after surgical resection of a cavernous hemangioma of th,e
right pons at the level of the middle cerebellar peduncle
1261 (Table 4). “Impairment of prosody and rhythm of
speech” was labeled as “cerebellar” dysarthria.
Two children of a series of children with posterior fossa
tumor did not manifest mutism but did have mild speech
problems after surgery [ 18] (Table 4). A 1O-year-old boy
spoke with a very “soft voice” 2 days after extubation
after undergoing resection of a tumor in the fourth ventri-
cle invading the right cerebellar hemisphere and the right
cerebellar peduncle. A 4-year-old girl answered questions
by silently moving her lips and whispered a limited
number of words after undergoing resection of a menin-
gioma situated anterior to the basilar artery.
In adults, speech deficits resulting from bilateral tha-
lamic lesions have been documented by Ackermann et al.
1303. In children, little is known about speech deficits
resulting from thalamic lesions. Garg and DeMyer [3 1 ]
presented the clinical features of 6 children with thalamic
strokes, of whom one child had “slurred speech”. Parker
et al. (321 also reported “slurred speech” after infarction
in the left thalamus associated with mycoplasma infecti:jn
in an &year-old girl, but no further details on the speech
features were described.
In one case study 1251. a cluster of deviant speech
features after brainstem IcGon. with clinical signs ot
bulbar palsy resembling flaccid bulbar dy\arthria in adults.
was reported. lmppairmcnt ot’ prosody was reported at’tet
resection of a cavernous hemangioma of the pons 1261.
Bmcrl Gmglitr Lc~sims. In adults. movement disorders
associated with basal ganglia lesions fall into two catego-
ries with opposite signs: hypokinesia and muscle rigidity,
respectively, hyperkinesia with choreatic or dystonic
movements. These two clinical entities are well recog-
nized in speech as hypokinetic and hyperkinetic dysarthria
[ I.21 (Table 1).
In children, pure hypokinetic or hyperkinetic movement
disorders seldom occur. More often, there is a mixed
extrapyramidal syndrome. Characteristics of children with
basal ganglia lesions and associated speech features are
listed in Table 5.
Murdoch et al. 1331 reported a I Syear-old boy with
posthypoxic hypohinetic movement disorder. etailed
speech analysis during a long follow-up period after a
mute phase revealed the following speech features: “dif-
ficulty in controllin, ~7 speech rate within a phrase.” and
“increase of speech rate within a sentence” (Table 5).
These features were labelled hypokinetic dysarthria be-
cause they are highly specific for adult patients with
parkinsonism and do not occur in any other dysarthria
tYPe.
We found one case of ACD associated with hyperki-
netic dystonic and choreiform movement disorder 1341
(Table 5). Al-Mateen et al. [34] described a g-year-old girl
with encephalitis due to mycoplasma infection. -Magnetic
resonance imaging showed areas of low-signal intensity
invohing the caudate., putamen, and globus pallidus. One
month after disease onset, she had dystonic posturing of
the right foot, dyskinetic movements of the face, and
choreiform movements of the extremities. Speech was
“hypophonic with incomprehensible vocal utterances,” In
the course of improvement, a marked discrepancy between
severity of speech deficit and movement disorder was
observed: 18 months after onset of the illness, “she was
still unable to initiate speech without cuing but she could
run, skip, and climb” [34].
Mixed hyper- and hypokinetic movement disorder as-
sociated with ACD has been described in detail in two
cases 1351 (Table 5). Pranzatelli et al. 1351 examined 6
patients with extrapyramidal movement disorders due to
various etiologies. Abnormal speech was present in all
children, of whom 3 were initially mute. Case 1. a
12-year-old girl, virtually unable to move or speak, im-
proved with medication and produced “hypophonic
speech” (Table 5). Case 2, a I4-year-old boy. with
bradykinesia and rigidity, also exhibited writhing hemi-
ballistic movements of the left hand. This mixed extrapy-
ramidal movement disorder was associated with “stutter-
ing speech” (Table 5).
Pn one case, basal ganglia lesions were associated with
ACD but not with extrapyramidal movement disorder.
After sustaining a left-sided infarction. involving the
crlobus pallidus, the putamen, the body of the caudate, and
c
the posterior limb of the internal capsule. Case 2 of’Aram
et al. 1361 (Table 5) exhibited dysarthria. The patient
manifested right-sided hemiparesis, but no signs of’ extra-
pyramidal movement disorder. Speech was mildly af-
fected, mainly in articulation. Silverstein and
1371 reported mild dysarthria with rapid resolution in 2
children with postvaricella basal ganglia infarction. 0~
child presented with hemiparesis and hcmichorea, associ-
ated with “slurred speech”.
The review shows some resemblance of such disorders
to that in the adult model. In children with extrapyramidal
movement disorders. dysarthria may be characterid by
hypophonia. stutterin,. u and difficulty in controlling speech
rate. which are high1 pecific for hypokinetic dystlrthria
in adults (Table 1). oreover. the degree of movement
disorder appears to correlate with the speech impairment
except for one case 1341. Both may subside with m~dica-
tion. which substantiates the correlation of the extIrapyr:i-
midal movement disorder with the severity of the rjysar-
thria.
van Mourik et al: Acquired Childhood Dysarthria 303
 Cerebrlll C(jrtiwl Lesions: ht~~i(~l’ O/WIYW~U~ S_W- drome after infectious disease was also poor in 2 other
iltWW. Functional impairment or structural damage to children 1451. At follow-up after 2 years, Case 1 was able
the anterior or part of the insula. the operculum, leads to to speak with “impaired articulation” and “nasal
the well-studied opercular syndrome with uniform find- speech.” At onset, Case 2, a 24-month-old girl, had
ings. Its most significant hallmark is LOSSof voluntary complete absence of speech and of all voluntary move-
control of orofacial musculature with drooling, anar- ments of jaw, lips, and tongue. Two years later, she had
thria (i.e., speechlessness as the most severe form of not regained any speech except for sounds and “a few and
dysarthria), and aphonia. Mental status is preserved and poorly articulated words” [ 451.
language comprehension is intact. There is no emo- On an ictal or postictal basis, functional impairment of
tional lability. Patients cannot produce voluntary ore- the anterior opercula interferes with orofacial musculature
facial movements on request, whereas patients may and speech. The mode of onset, relapses, and evolution
yawn, laugh, or be able to perform complex movements show various patterns [39]. Outcome is favorable once the
spontaneously, such as blowing out a match [38]. In epilepsy is adequately treated.
adults, the opercular syndrome is most often attribut- After structural lesions, the clinical picture is similar to
able to cerebral ischemia. The major deficits, dysphagia that in adults. The prognosis is poor, as is the case in
and anarthria, are unlikely to be reversible since no full adults: Patients remain mute for a long time and rarely
recovery has been documented [38J. regain speech. Speech features have therefore been incom-
In children, various etiologies may cause the (rare) pletely analyzed.
opercular syndrome. Because of its uniform clinical pic- Other Crrebr-crlCar-ticcrlcud htmud Cqmrle Lesiorls.
ture, data are not summarized in Table 1 to 6. Deonna et Deviant speech features in adults with spastic dysarthria,
al. [391 reported oromotor and speech disturbances as a detected after cerebral cortical and internal capsule le-
focal epileptic manifestation. They examined 3 children sions, are presented in Table 1. Bilateral damage may
diagnosed with benign partial epilepsy with rolandic result in persistent speech impairment in adults. Accom-
spikes. As a result of recurring seizures around the sylvian panying neurologic deficits consist of a pyramidal syn-
area, “speech arrest” of short duration, “slow speech drome on one or both sides.
rate.’ ’ ’‘poor prosody,’ ’ and “imprecise articulation” In children, dysarthria without aphasia or verbal
occurred as prolonged or postictal deficits, sometimes apraxia [S- 101 resulting from bilateral cerebral hemi-
resembling an incomplete anterior opercular syndrome. sphere lesions not located in the opercula have rarely
Shafrir and Prensky [40] observed opercular syndrome been documented. Data are not summarized in Tables I
of epileptic origin in a child with recurrent prolonged to 6 because we could find only one case that fits WI
episodes of severe oral apraxia, dysarthria, and drooling. inclusion criteria [46]. “Stuttering-like speech” was
During these episodes, there was an increase in right noted in this 27-month-old child with multiple infarc-
temporal spikes and appearance of generalized spikes. tions in the periventricular white matter, 14 months
polyspikes, and slow wave discharges. Remissions were after a subcortical infarction in the left basal ganglia
associated with improvement evident in the electroenceph- that cxtendcd into the cortex 1461. et’ stutter. ti I,ich
alogram. which either showed right temporal focus or was lasted 7 weeks, affected mainly the initial phonemes.
1’rec of cpilcptiform activity [40]. and speech was “hypophonic.”
Continuous partial seizures in the rolandic areas for a Unilateral hemisphere lesions, on the other hand,
prolonged time caused extreme drooling, impairment of have been reported to cause ACD, but descriptions of
tongue function, and “slurred speech” in 2 children with speech features have been less specific. After varicella
preexistent benign partial epilepsy with rolandic spikes. infection, a 9-year-old boy sustained a left cerebral
Speech recovered fully after adequate treatment of the infarction involving the internal capsule and the caudate
seizures [4 11. nucleus. He became dysarthric and developed right-
Other etiologies of opercular syndrome have been sided central facial palsy and right-sided hemiplegia
described in children. A 13-year-old boy 1421 sustained affecting the upper limb. All neurologic features re-
biopercular infarctions after vasculitis resulting from a solved completely within hours [ 471.
scorpion bite. The mutism lasted for months, after which “Slurred speech” and hemiparesis of the right arm and
he was able to laugh and cough and produce a few isolated leg were the signs in a 6-year-old boy who sustained a
vowels. small left middle cerebral artery ischemic infarction ex-
Massive infarctions in both opercula after tuberculous tending into the subcortical white matter. His clinical
meningitis also caused opercular syndrome in a 3-year-old status demonstrated rapid improvement with complete
boy, who became verbally mute and aphonic, with his recovery after 3 months [481.
mouth constantly open and dribbling saliva [43]. One yeal Reports of ACD subsequent to circumscribed cerebral
later, he had regained the ability to articulate simple hemisphere lesions are scarce. Because unilateral lesions
words. The patient of Prats et al. (441 who manifested may lead to transient ACD, studies have not focused on its
(jpercular syndrome after herpes simplex encephalitis re- specific features. Bilateral nonopercular hemisphere les-
mained completely mute. Recovery from opercular syn- ions associated with dysarthria, frequent in adults, were

303 PEDIATRICNEUROLOGY Vol.17No.3

documented in only I child, who developed stuttering-like thria to intelligible speech preceded improvement in
speech impairment [46]. To our knowledge, dysarthria other motor functions.
without aphasia or verbal apraxia, resulting from acquired Dysarthria may occur as an initial feature of rare
nonopercular cortical lesions and resembling spastic, structural lesions during MTX treatment. It may signal
pseudobulbar dysarthria in adults has not been described structural brain damage as a result of ischemia. As such.
in children. its sudden onset as a focal neurologic feature differs from
the neurotoxic reactions in MTX encephalopathy. Recog-
The Presence of AC in Clinical Management nition of the sudden onset of dysarthric speech is therefore
of diagnostic significance and contributes to clinical man-
We studied the relationship between ACD on the one agement [5 11. In thalamotomy performed to alleviate
hand, and motor disorder on the other, according to the posttraumatic tremor, the dysarthria may worsen despite
clinical features or the neuroradiologic evidence of lesion improvement in other motor function. The presence of
site. The second aim of our study was to review studies dysarthria constitutes a major contraindication for thalam-
demonstrating that the occurrence of dysarthria may be a otomy [52,53]. Recovery from dysarthria may precede
marker of clinical deterioration or that recovery from neurologic improvement [ 541.
dysarthria may herald clinical improvement. Analysis of
dysarthria may therefore be of importance for clinical iscussion
management.
Reitman et al. [49] analyzed speech deficits in survivors Our main aim in the present study was to provide a
of Reye’s syndrome. Twenty-six of the 43 examined survey of speech features in ACD and to compare them
children were dysarthric, aphonia being the most common with the adult types of dysarthria [ 1,2j. In children.
features. In 4 children, the dysarthria was the predominant dysarthria associated with cerebellar tumor resection (Ta-
handicap at 12 months follow-up. The investigators con- ble 2) has received more attention than dysarthria associ-
cluded that in rehabilitation of Reye’s syndrome. one ated with any other type of lesion. In this group. speech
should anticipate the occurrence of speech problems. was frequently characterized by slow speech rate and
Intrathecal methotrexate (MTX) treatment is an essen- monotony. These feature5 are not distinctive of ataxic
tial part of central nervous system prophylaxis for leuke- dysarthria because they may occur in other dysarthria
mia in children. Neurologic complications due to neuro- types. Excess and equal stress-scanning speech-con-
toxic reactions may follow such therapy [50,5 1j. Yim et al. sidered the hallmark of ataxic dysarthria in adults and not
[St ] observed severe hemiparesis and brief but profound occurring in any other dysarthria type [ 1,2], was rarely
dysarthria of sudden onset in 2 children after they received observed even in children with severe dysarthria. We
intrathecal MTX treatment. Initially, these signs were found one other case of ACD resulting after cerebella1
considered part of an MTX-induced encephalopathy. stroke 1241 that did net show typical ataxic speech features
Later, computed tomography and magnetic resonance either. Therefore in children. cercbcllar Icsions, particu-
imaging scans showed ischemic structural lesions in the larly tumor resection, may cause speech deficits not
cerebral hemisphere in both children. This clinical picture. resembling ataxic dysarthria in adults. As yet. the validity
in which the profound dysarthria was prominent. m;ry bc of the adult model for children with ccrcbcllar Icsions ha\
an indication of ischemic brain lesions and differs from the not been sufficiently proven.
clinical picture of MTX encephalopathy. In other patient groups with lesions in different areas of
The presence of dysarthria appears to have a prog- the brain, grossly classified on the basis of ncuroradiologic
nostic value in cases of thalamotomy performed to or neurophysiologic similarities, clusters of deviant spccc~
alleviate the disabling tremor that may occur in post- features that would be specific for each group have not
traumatic midbrain syndrome. In two patient groups emerged either. An exception appears to he the SpWCh
(aged 10 to 29 years) 152.531, thalamotomy alleviated features associated with basal ganglia lesions [ 33-371.
the tremor. However, the preexistent posttraumatic Features that are not reported in other dysarthria types.
dysarthria tended to worsen postoperatively. In both such as short rushes of speech, difficulty in controlling
studies 152,531, worsening of the dysas :hria uas con- speech rate (fcstination), and stuttering were also recorded
sidered a major limitation to the surg\cal intervention in children (Table 1). which suggests that ksion~ invdc-
and the existence of a preoperative :Ilysarthria was ing the basal ganglia may cause speech deviancies that
considered a contraindication for thalamotomy. resemble dysarthria in adults with similar movement
van Dongen et al. [54] analyzed dysarthria speech disorders.
features in children with supranuclear or peripheral We had two reasons to take the adult model of clusters
facial palsy. (Because the lesions were diffuse, we did of speech features mirroring the motor impairment as a
not include this study in the previous text.) In the starting point [ 1,I?]. First, cited studies comprise the largest
present context, we note that severity of dysarthria was groups of dysarthric adults ever investigated. The typology
analyzed in relation to neurologic deficits. In patients of dysarthrias [ 1.21 has been applied universally to label
with supranuclear palsy, recovery from severe dysar- deviant speech. in adults as well as in children. Second, an

van Mourik et al: Acquired Childhood Dysarthria 305

equivalent model of motor speech disorders for children is speech disorders in progressive supranuclear palsy. Neurology 1993;43:
563-6.
lacking. Therefore, Murdoch et al. [55] claim that although
[7] Enderby P. Relationships between dysarthric groups. Br J
it is difficult to apply models and theories developed for Commun Dis l986;2 I : 189-97.
adults to children it appeLars to be appropriate to use the [8] Paquier PF, Van Dongen HR. Review of research on the clinical
classification system of Darley et al. [I] to describe the presentation of acquired childhood aphasia. Acta Neurol Stand 1996;93:

equivalent speech disorders in children until more infor- 428-36.

mation becomes available to refute its appropriateness.
In light of this, it is of importance to stress that the
quality of nonspeech movements, articulation, velopha-
ryngeal function, and orofacial musculature change during
[9] Square PA, Aronson AE. Hyman E. Case study of the redevel-
opment of motor speech control following acquired brain damage in early
childhood, Am J Speech Lang Pathol 1994;3:67-80.
[lo] Deonna T, Chevrie C. Hornung E. Childhood epileptic speech
disorder: prolonged, isolated deficir of prosodic features. Dev Med Child

development [56,57]. Moreover, normal speech in chil- Neurol I987;29:96- 109.

[ll] Rekate HL, Grubb RL, Aram DM, Hahn JF, Ratcheson RA.
dren may reveal features such as strained voice, breathi-
Muteness of cerebellar origin. Arch Neurol 1985;42:697-8.
ness, and hyponasality that are considered pathologic in 1121 Volcan I, Cole GP, Johnston K. A case of muteness of
adult speech [58]. Developmental norms are well known cerebellar origin. Arch Neurol 1986:43:3 13-4.
to speech pathologists working with children developmen- [13] Dietze D. Mickle JP. Cerebellar mutism after posterior fossa
surgery. Pediatr Neurol 1990;4:228-30.
tally impaired in speech/language but may be poorly
[14] Ferrante L, Mastronardi L, Acqui M, Fortuna A. Mutism after
recognized within clinical circles. We could find only one
posterior fossa surgery. J Neurosurg I990:72:959-63.
study addressing the discrimination of developmental and [IS] Nagatani K. Waga S, Nakagawa Y. Mutism after removal of a
acquired dysarthric aspects of articulation in acquired vermian medulloblastoma: Cerebellar mutism. Surg Neurol I99 136:
cerebellar lesions [59], but this study did not fit our 307-C).
1161 Catsman-Berrevoets CE. Van Dongen HR. Zwetsloot CP.
inclusion criteria. As to the second aim of our review, the
Transient loss of speech followed by dysarthria after removal of posterior
available literature shows that analysis of dysarthria may
fossa tumour. Dev Med Child Neurol 1992;34: I IO2- I?.
play a crucial role in the recognition of clinical changes. 1171 Herb E. Thyen U. Mutism after cerebellar medulioblastoma
All observations reviewed in the present study are based surgery. Neuropediatrics l992;23: 144-6.
on single case studies, which illustrates the fact that [IS) van DcsngenHR, Catsman-Berrevoets CE, van Mourik M. The
syndrome of “cerebellar” mutism and subsequent dysarthria. Neurology
dysarthria has been treated as a Cinderella [60], in children
I994;44:2040-6.
even more so than in adults. As yet, the study of ACD is
[19] Al-Jarallah A. Cook JD, Gascon G. Kanaan I, Sigueira E.
still in its infancy, as was true of the study of acquired Transient mutism following posterior fossa surgery in children. J Surg
childhood aphasia in the late 1970s. Until then, studies of Oncol I994:SS: I 26-3 I.
aphasia in children had focused mainly on the fundamental [20] Crutchfield JS, Sa~~aya R. Meyers CA, Moore BD. Postopcr-
ative mutism in neurosurgery. J Neurosurg 1994:8 I : I I S-2 I.
differences between the neurology of language in children
1211 Kingma A, Mooij JJA. Metzemaekcrs JDM. Leeuw JA. Tran-
and adults 1611. More recently, systematic studies of
sient mutism and speech disorders after posterior tbssa surgery in
recovery from acquired childhood aphasia and of lesion children with brain tumors. AcIa Ncurochir (Wien) 1994; I3 I :73-O.
~malyses 18 1demonstrated that correl,ltions between apha- 1221 il’ollack IF:. Polinko I’. Albright AL. Towbin R. Fill C. Mutism
sia type and Icsion localization parallel those in adults :md pscudobulbar syn~l~tomx al’lcr rcscction of po\tcrior fossa tumors in
children: Incidence and pi~Ihl~l~hysil~ll~gy.Neurosurgery I995:37:885-033.
[ 8,61 1. As to ACD, it is not yet possible to predict what
1231 Asamoto M. Ito H. Suzuki N. Oiwa Y. Saito K. Haraoha J.
systematic analyses of speech features and anatomoclini-
Transient mulism after posterior fhssa surgery. Child Nerv Syst 1994:
cal correlation studies will teach us about the similarity 10:27!i-8.
and differences between dysarthria in children and in 124) Echenne B. Gras M. Astruc J. Castan P. Bnrnel D. Vertcbro-
adults. The current knowledge of ACD. reviewed herein, basilar arterial occlusion in childhood-report of a case and review of the
literature. Brain Dev 1983;5:577-8 I.
indicates that ACD requires its own classification, devel-
[251 Bak E. van Dongen HR. Arts WFM. The analysis of acquired
oped through detailed analysis of speech features in
dysarthria in childhood. Dev Med Child Neural 1983;25:81-94.
dysarthric children. 1261 Frim DM. Ogilvy CS. Mutism and cerebellar dysarthria after
brainstem surgery: Case report. Neurosurgery I995:36:854-7.
1271 Pascual-Castroviejo I. Pascual-Pascunl JI. Mulas F, Roche
MC. Tendero A. Bilateral obstruction of the vertebral arteries in a
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