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Child Neuropsychology: A Journal on

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Two Sets of Twins with Selective

Mutism: Neuropsychological Findings
Robert M. Gray , Catherine M. Jordan , Richard S. Ziegler &
Ronald B. Livingston
Published online: 09 Aug 2010.

To cite this article: Robert M. Gray , Catherine M. Jordan , Richard S. Ziegler & Ronald B.
Livingston (2002) Two Sets of Twins with Selective Mutism: Neuropsychological Findings, Child
Neuropsychology: A Journal on Normal and Abnormal Development in Childhood and Adolescence,
8:1, 41-51
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Child Neuropsychology
2002, Vol. 8, No. 1, pp. 4151

0929-7049/02/0801-041$16.00
# Swets & Zeitlinger

Two Sets of Twins with Selective Mutism:

Neuropsychological Findings
Robert M. Gray1, Catherine M. Jordan2, Richard S. Ziegler2, and Ronald B. Livingston3

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Kennedy Krieger Institute, Department of Neuropsychology, Baltimore, MD, USA, 2Department of Pediatrics,
University of Minnesota, USA, and 3Department of Psychology, University of Texas at Tyler, Tyler, TX, USA

ABSTRACT
Neuropsychological data are reviewed from two sets of dizygotic twins presenting with selective mutism
characterized by situation specific anxiety, extreme passive behavior, lack of responsivity, lack of peer
interaction, and a chronic course of selective mutism. Both sets of twins had a history of prematurity and
delayed speech development. One set of twins presented with normal intelligence and normal receptive
language skills but with expressive language and oral motor sequencing difficulties. The second set of twins
presented with Verbal IQ deficits and significant receptive and expressive language deficits. A summary of
current conceptualizations regarding etiology and treatment of selective mutism is provided.

Mutism in children can result from neurological

insult such as lesions in the posterior fossa region
(Gordon, 2001), but children may also demonstrate a persistent failure to speak in specific social
situations despite the ability to speak in other
selected situations, a rare disorder referred to as
selective or elective mutism. As early as
1877, the German physician Kussmaul described
this disorder as aphasia voluntaria, and by 1934
the Swiss child psychiatrist Moritz Tramer had
developed the term elective mutism. While
theories regarding the etiology of selective
mutism (SM) were initially predominated by
psychodynamic conceptualizations, current conceptualizations of SM suggest that multiple
factors are likely to be associated with a lack of
speaking in social situations including anxiety,
temperamental inhibition, a history of developmental delays, and speech and language deficits
(Dow, Sonies, Scheib, Moss, & Leonard, 1995;
Kristensen, 2000; Steinhausen & Juzi, 1996).
Reflecting the changes in conceptualization over

time, the term elective was changed to

selective in the 1994 edition of the Diagnostic
and Statistical Manual of Mental Disorders
(DSM-IV; American Psychiatric Association,
1994) to decrease the notion that the child might
be electing not to speak in an oppositional manner
and instead to focus on anxiety or other issues that
might be interfering with the childs ability to
speak in all but a few selected situations (Dow
et al., 1995). Diagnostic criteria in both ICD-10
(World Health Organization, 1992) and DSM-IV
(APA, 1994) include consistent failure to speak in
specific social situations despite speaking in other
situations; interference with educational or occupational achievement or with social communication; a duration of at least 1 month; and a qualifier
that the condition is not better accounted for by a
communication disorder or by a lack of knowledge of the spoken language.
While there are several excellent reviews of
children with SM (Dow et al., 1995; Dummit
et al., 1997; Gordon, 2001; Steinhausen & Juzi,

Address correspondence to: Robert M. Gray, Kennedy Krieger Institute, Department of Neuropsychology, 1750 E.
Fairmont Avenue, Baltimore, MD 21231, USA. E-mail: grayr@kennedykrieger.org
Accepted for publication: August 27, 2002.

Downloaded by [Bangor University] at 05:48 23 December 2014

42

ROBERT M. GRAY ET AL.

1996), there are few studies examining the neuropsychological functioning of children with SM,
and there is even less information available
regarding twins with SM. Below, we present a
brief summary of current conceptualizations regarding etiology and treatment of SM followed by
presentation of background and neuropsychological data on two sets of dizygotic twins with SM.

Ford et al. (1998) completed a survey of 92

children and 9 adults with SM and 43 children
and 9 adults who had previously experienced SM.
For persons, who no longer had SM, the age of
cessation of symptoms ranged from 5 to 10, but
many individuals reported ongoing difficulties
with speaking in certain places and discomfort in
social gatherings.

Epidemiological Issues
Selective mutism is thought to be quite rare in
school-age children, with the incidence of SM in
the general population reported as approximately
1 in 1,000 (Segal, 1999). While the chances of SM
occurring in twins is likely to be quite rare, exact
incidence figures are not available and difficult to
calculate as the incidence of dizygotic twinning is
variable depending on racial group and country of
origin and is influenced by parity, age of mother,
and the increasing use of fertility medications.
Segal (1999) estimates the chance of an affected
child being a twin would be about 1 in 40,000. In
contrast to many child psychiatric disorders, SM
is generally observed more commonly in females
(Dummit et al., 1997; Steinhausen & Juzi, 1996).
The disorder is reported across all socioeconomic
status (SES) levels and a background of immigration is common in children with SM (Steinhausen
& Juzi, 1996). The typical age of onset is between
3 and 5 years, although most referrals occur
around age of 6 or 7 coinciding with entry into
kindergarten or first grade (Krohn, Weckstein, &
Wright, 1992). Professionals who most frequently
identify and confirm SM are psychologists,
followed by psychiatrists, speech and language
clinicians, social workers, and teachers (Ford,
Sladeczek, Carlson, & Kratochwill, 1998).
Children with SM demonstrate a wide range of
prognostic outcomes with many demonstrating a
decrease in symptoms over a period of months,
while others present with a more chronic course
spanning several years (APA, 1994; Kolvin &
Fundudis, 1981). In a 12-year follow-up study of
41 German children with SM, Remschmidt,
Poller, Herpertz-Dahlmann, Hennighausen, and
Gutenbunner (2001) found that 39% demonstrated
a complete remission, while the remaining individuals continued to struggle with a range of communication problems and emotional difficulties.

Etiological Issues and Associated Findings

Early theories regarding the etiology of SM
included response to family neurosis, overprotective mothers, remote fathers, early mouth trauma,
and unresolved psychodynamic conflicts (Dow
et al., 1995; Krohn et al., 1992). Reaction to major
life events or trauma has also been proposed as a
potential causative factor (Dow et al., 1995), but
major reviews of children with SM indicate that
while psychosocial stressors may be associated,
abuse and trauma are only rarely associated
factors in the development of the disorder
(Gordon, 2001; Kolvin & Fundudis, 1981;
Steinhausen & Juzi, 1996). Previous reports have
also noted associated oppositional, controlling,
negative, and manipulative behaviors (Kolvin &
Fundudis, 1981; Krohn et al. 1992).
Anxiety
Current conceptualizations of SM place less
emphasis on trauma and oppositional behavior
and instead emphasize the frequent association
with shy and withdrawn temperament and
comorbid anxiety conditions such as social
phobia, school phobia, separation anxiety, and a
family history of anxiety. Ford et al. (1998)
propose that many children with SM demonstrate
a slow-to-warm temperament characterized by
behavioral inhibition and a tendency to withdraw
in response to novel situations. Black and Uhde
(1995) found that in a sample of 30 children with
SM, 97% met diagnostic criteria for social
phobia, avoidant disorder, or both, whereas only
13% reported a history of physical or sexual
abuse, and only 10% had a diagnosis of oppositional defiant disorder. A significant family
history of anxiety problems was noted as well,
with first-degree relatives demonstrating social
phobia in 70% of families and SM noted in 37%
of the families, with 22% of the families reporting

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TWO SETS OF TWINS WITH SELECTIVE MUTISM

a parent who was selectively mute. In a study

examining 50 children with SM, Dummit et al.
(1997) reported similar findings as all 50 children
met criteria for social phobia or avoidant disorder
and 48% had additional anxiety disorders with
only one case of oppositional defiant disorder
noted. Steinhausen and Juzi (1996) found that in a
sample of 100 children with SM, the most
common personality feature was shyness, which
was noted in 85% of the children. Approximately
66% of the sample were described as anxious,
while symptoms of oppositional defiant or
aggressive behavior were noted in 20% of the
sample. As several studies cite the frequent cooccurrence of anxiety-related difficulties, some
authors have proposed that SM should be
conceptualized as a symptom of anxiety rather
than a distinct disorder (Kristensen, 2000).
Developmental Delay
In addition to the emphasis on anxiety and
temperament, various studies note that early
developmental risk factors including complicated
deliveries, and speech and language and motor
delays are common in children with SM. Kolvin
and Fundudis (1981), in a study of 24 children
diagnosed with SM, found that these children
spoke significantly later than controls and the
rates of enuresis and encopresis were significantly
greater as well. They found that 46% of the
children wet or soiled themselves either during
the day or night or both. Half of the children
demonstrated speech difficulties as identified by
speech therapists. Steinhausen and Juzis (1996)
review of 100 children with SM revealed that 43%
of the sample had complicated delivery histories,
18% demonstrated delayed motor development,
and 24% demonstrated delayed toilet training.
Speech and language disorders, primarily articulation and expressive language disorders, were
noted in 38% of the sample. Kristensen (2000)
found that in a sample of 54 children with SM,
68.5% met criteria for language and motor
developmental disorders or delays, 74.1% met
criteria for anxiety disorder, and 31.5% demonstrated elimination disorders, with 46.3% of the
children meeting criteria for both anxiety and
developmental delay. Steinhausen and Adamek
(1997) reported that disorders of speech and

43

language were common in the families of children

with SM.
Neuropsychology
Considering the etiological perspectives reviewed
above, at least two general neuropsychological
models of SM could be hypothesized. Children
with SM and global deficits, often referred to as
developmental delay, might demonstrate a diffuse
generalized profile of neuropsychological deficits.
Children with SM and significant anxiety, behavioral inhibition, and withdrawn temperament
might be expected to demonstrate an overactive
behavioral inhibition system (BIS; Gray, 1982,
1987), a system thought to be based in the
septohippocampal system and its connections to
the prefrontal cortex featuring numerous neuroadrenergic and serotonergic pathways. Davidson
(1993) proposes that left and right anterior
cortical systems are specialized for approach
and withdrawal behaviors respectively, and it is
possible that disruption in these systems might
lead to lack of approach behaviors and increased
withdrawn behaviors. The utility of these models
in describing children with SM remains purely
speculative at this time, as no comprehensive
systematic neuropsychological or neurophysiological studies have been completed with this
population.
Previous studies of children with SM have
generally focused on intellectual testing alone as
an indicator of neurocognitive abilities. A range
of IQ performance has been noted in these children, spanning the mentally retarded to normal
range. Kolvin and Fundudis (1981) reported a
mean IQ of 84.95 in their sample based on
WISC Performance IQ scores. Krohn et al. (1992),
in a sample of 20 children who met criteria for
SM, reported an average IQ of 103 based on
scores obtained from the WISC-R Full Scale IQ,
Performance IQ, and the Leiter International Performance Scale. Kristensen (2000) reported a
mean WISC-R Performance IQ of 96.74 in a
sample of 54 children with SM.
Genetics
The frequent occurrence of anxiety and shyness,
speech and language disorders, and psychiatric
disorders in family members of children with SM

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44

ROBERT M. GRAY ET AL.

(Steinhausen & Adamek, 1997) has lead to

speculation regarding the role of genetic factors
in the etiology of SM. Grosso, Cioni, Pucci,
Morgese, and Balestri (1999) hypothesize that
deletion of the short arm of Chromosome 18 may
be associated with a subtype of SM characterized
by dysmorphic features, speech delay, and
chronic SM. This speculation is based on two
females with SM who had similarly abnormal
karyotypes and similar clinical profiles characterized by below average to borderline range Verbal
and Performance IQ, head circumference under
the tenth percentile, flattened nasal bridge, broad
philtrum, micrognathia, and short broad fingers.
Hagerman, Hills, Scharfenaker, and Lewis (1999)
describe two sisters with fragile X syndrome
(FXS) who also had histories of SM and anxiety.
The authors note that shyness and social anxiety
are frequently reported among males and females
with FXS, and a subgroup of children with FXS
demonstrate learning and attentional problems
without mental retardation. It is reported that 30%
of females with FXS express the disorder in a
milder form than boys with less physical
abnormalities, cognitive deficits ranging from
learning disabled to mild mental retardation,
unusual speech patterns, and shy and withdrawn
behaviors (Batshaw & Perret, 1992). The sisters
presented in the Hagerman et al. (1999) case
study were not mentally retarded but presented
with learning problems and classical SM behaviors including anxiety, extreme shyness, and a
lack of speaking outside the home. The authors
propose that the anxiety and social phobia
associated with FXS predisposed these children
to developing SM. In addition to the cases they
describe in their paper, the authors report that they
have clinically encountered six additional females
with FXS and SM, and they recommend further
studies regarding this association as well as more
frequent testing for FXS in children with SM.
In summary, SM appears to be a heterogeneous
condition with multiple etiological factors implicated including genetics, anxiety, temperamental
inhibition, a history of developmental delays, and
speech and language deficits. Steinhausen and
Juzi (1996) propose a vulnerability model
that implicates a genetic predisposition for development of SM that may be exacerbated by risk

factors including birth complications, motor

delays, and a history of immigration. No largescale neuropsychological studies exist and no
specific neuropsychological profile or pattern of
performance has been identified for children
with SM as a whole or subtypes of children with
SM.
Treatment Issues
Considering information regarding etiology of
SM, effective treatment approaches would likely
emphasize decreasing anxiety and encouraging
the use of speech across settings. Large-scale
treatment studies of children with SM have not
been conducted, and the majority of information
regarding treatment comes from case studies. SM
is a disorder that is considered by some to be
chronic and resistant to change and the disorder
has even been described as intractable (Kolvin
& Fundudis, 1981). Psychodynamic approaches
were at one time considered the treatment of
choice, and therapists continue to utilize play and
art therapeutic techniques to treat SM, but
currently, behavioral approaches are being utilized most often and considered more effective
(Dow et al., 1995; Krohn, 1992). Numerous
behavioral interventions including reinforcement
for speaking, shaping with successive approximations (reinforce mouth movements to whispers to
speech), self-modeling (child watches videotaped
segments of him/herself speaking), and schoolbased behavioral programs (teacher and peer
reinforcement of verbal behavior) are described
as effective treatments for SM (Dow et al., 1995;
Kehle, Madaus, Baratta, & Bray, 1998). Punitive
measures are not recommended, as they are likely
to increase the childs anxiety (Dow et al., 1995).
Speech and language therapy services are often
provided in conjunction with behavioral modification techniques, and it is suggested that children
with SM, with and without speech and language
disorders, will benefit from the structured and
supportive language practice offered in individual
speech and language therapy (Dow et al., 1995).
Kristensen (2000) emphasizes that due to the
frequent occurrence of developmental delays in
children with SM, interventions should also focus
on adapting environmental and therapeutic
demands to the childs level of functioning.

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TWO SETS OF TWINS WITH SELECTIVE MUTISM

In terms of school programming, it is clearly

important that children with SM receive a comprehensive evaluation as they may present with a
broad pattern of abilities and symptoms, ranging
from relatively normal cognitive abilities with
concurrent anxiety to significant developmental,
cognitive, language, and behavioral deficits.
Dummit et al. (1997) found in their sample of
50 children with SM, most children were placed
in the regular classroom, 12% had documented
speech and language needs requiring intervention,
and approximately 10% were inappropriately
placed in special education speech and language
programs as they were mistakenly conceptualized
as having a primary speech and language disorder
rather than an anxiety-related problem.
Pharmacological treatment of children with
SM using antidepressants or antianxiety agents
has also been investigated. Dummit, Klein,
Tancer, Asche, and Martin (1996) investigated
the efficacy of fluoxetine (Prozac) in 21 children
with SM and anxiety disorder, aged 514 years.
No control group was included. After 9 weeks of
treatment, 76% were reported to demonstrate
diminished anxiety and increased speech in public
settings including school. Black and Uhde (1994)
completed a 12-week placebo-controlled study of
fluoxetine for children with SM. The six children
taking fluoxetine demonstrated significant improvement on some parent ratings of mutism
and anxiety in comparison to controls but subjects
in both groups remained symptomatic at the
conclusion of the study. Carlson, Kratochwill,
and Johnston (1999) examined the efficacy of
sertraline (Zoloft) in five children diagnosed
with SM, aged 517 years, using a double-blind
placebo-controlled study design. After 16 weeks,
improvements were noted in some individuals,
but no significant group changes were noted.
Phenelzine, an irreversible, nonselective monoamine inhibitor, has also been utilized in pharmacological treatment studies of SM because of its
reported success in the treatment of social phobia
(Golwyn & Sevlie, 1999). Golwyn and Sevlie
present four case studies of children with SM,
aged 57 years who demonstrated improvements
over the course of 2460 weeks, which were
attributed to medication benefits. Due to the
possibility of serious food and drug interactions,

45

phenelzine was described as a medication treatment that should be reserved for those children
who do not respond to behavior therapy or SSRI/
fluoxetine therapy (Golwyn & Sevlie, 1999).
In general, pharmacotherapy is not recommended as the treatment of first choice for children with SM, but medications are considered
potentially useful for those children who do not
respond to behavioral intervention, and may
also be more effective when used in combination with behavioral methods (Gordon, 2001;
Kumpulainen, 2002).
Twins with Selective Mutism
While very little systematic research exists, SM
has been reported among siblings and twins.
Wallaces (1986) popular novel, The Silent Twins,
relays the story of June and Jennifer Gibbons,
identical twins with SM who had delayed speech
noted at 3 years of age, apparently spoke only to
each other, developed a secret language, and went
on to suffer multiple social, occupational, and
legal hardships as adults. Segal (1999) notes that
twins often demonstrate language difficulties, and
that many twins, with and without SM, will
demonstrate secret signs or private words and
gestures, and such behavior should not be
considered distinctive to twins with SM. Segals
(1999) interview with a parent-based organization, the Selective Mutism Foundation, revealed
20 families with affected twins in an organization
with 350 subscribers, and SM was thought to be
more prevalent in both twins when they were
identical twins rather than fraternal. Ford et al.s
(1998) survey revealed 14 twins with SM or SMlike behaviors, but their zygosity was not
indicated.
The possibility that twins present with SM
more frequently than singletons is unclear but
Dummit et al. (1997) identified three affected
identical twin pairs representing 12% of the
individuals in that study which far exceeds the
estimate of 0.8% identical twins in the general
population. Segal (1999) notes that the majority
of twin research indicates that most behavioral
disorders do not occur more often in twins than in
singletons, but it has been speculated that twins
with SM may be more likely to reinforce each
others lack of speech leading to a potentially

46

ROBERT M. GRAY ET AL.

higher rate of SM in twins than in the general

population as well as a more chronic presentation
of SM.

METHOD

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Materials and Procedure

The current study describes the neuropsychological
profiles of two sets of dizygotic twins. Both were
referred for comprehensive neuropsychological evaluation due primarily to SM although each presented with
additional emotional, academic, or cognitive concerns
for which the neuropsychological evaluation was
tailored.
Following clinical interviews with parents, each
twin was evaluated over the course of three mornings
for approximately 23 hr, each session. Evaluation
sessions were conducted by an experienced psychometrist or one of three post-doctoral fellows in pediatric
neuropsychology.
Neuropsychological measures completed by Twins
A1 and A2 included: Wechsler Intelligence Scale for
Children Third Edition (WISC-III; Wechsler, 1991),
Kaufman Assessment Battery for Children (KABC;
Kaufman & Kaufman, 1983), Oromotor Sequences
from the NEPSY (Korkman, Kirk, & Kemp, 1998),
Clinical Evaluation of Language Fundamentals Third
Edition (CELF-III; Semel, Wiig, & Secord, 1995),
Peabody Picture Vocabulary Test Third Edition
(PPVT-III; Dunn & Dunn, 1997), Picture Vocabulary
subtest from the WoodcockJohnson Tests of Cognitive
Ability Revised (WJ-R; Woodcock & Mather, 1989),
Test of Variables of Attention (TOVA; Greenberg &
Kindschi, 1996), Purdue Pegboard (Tiffan, 1968),
Vineland Adaptive Behavior Scales (VABS; Sparrow,
Balla, & Cicchetti, 1984), and the Behavior Assessment
System for Children Parent Rating Scale (BASC-PRS)
(Reynolds & Kamphaus, 1998).
The second set of twins completed a somewhat
different battery of tests because of their particular
presentation and areas of concern. In addition to the
measures completed by Twins A1 and A2, Twins B1
and B2 completed: Wechsler Individual Achievement
Test (WIAT; Wechsler, 1992) and the BeeryBuktenica
Developmental Test of Visual-Motor Integration (VMI;
Beery, 1997).

Background Information
Twins A1 and A2
Twins A1 and A2 were 6-year-old Caucasian females
referred for a pediatric neuropsychological evaluation
due to a lack of speech and poor school performance.

The twins demonstrated significant social anxiety and a

fear of speaking in the home and school setting. The
twins were reported to prefer dressing alike although
their parents discouraged this behavior. It was reported
that separation of the twins in the school setting led to
some improvements in their attempts to communicate
with others. The twins were delivered at 35-weeks
gestation at a weight of 5 lb, 10 oz. They required 4.5
days of ventilation and were hospitalized for a total of 9
days in the NICU before being discharged. They were
reported to have walked at 15 months of age. It was
reported that they were 2 years of age before they began
talking, and as early as 2 and 3 years of age, they
demonstrated severe social anxiety. Family history was
significant for a paternal grandmother with features of
social anxiety and obsessivecompulsive behaviors.
Neurological evaluation of both twins was normal.

Twins B1 and B2
Twins B1 and B2 were 9-year-old Hmong females
referred due to a lack of speech outside the home.
Although they spoke with each other in both English
and Hmong at home, they were not responsive to
teachers at school. They were reported to speak and
play with their cousins but not with other children. In
the home setting, they required frequent repetition of
parental requests. Both at home and at school, they
were described as extremely passive and as
demonstrating a lack of initiation, to the point that
they would not request to go the bathroom at school.
This family had been in the United States for over 20
years, primarily spoke Hmong in the home, and their
acculturation status was considered to be moderate.
There was no family history of emotional or learning
problems reported, and the twins had an older sibling
with no behavioral or learning concerns noted. Due to a
kidney condition, the twins mother was on immunosuppresant drugs during her pregnancy. The twins were
delivered at 29-weeks gestation. Birth weight was not
available for Twin B1 but Twin B2 weighed 2 lb, 11 oz.
They required brief ventilation in the NICU, were
treated for apnea and were hospitalized for 2 months.
They were noted to walk at around 16 months and
speech was reportedly delayed with single words
noted around age 3. Their parents noted that they
would occasionally speak with each other and with
family members in the home setting, but historically
did not speak outside of the home or family environment. The twins did not attend preschool, but once they
began kindergarten, they were described as slow
learners and began receiving special education services
under the speech and language impaired classification.
School personnel decided to separate them and they
demonstrated minor improvements in speech therapy.
The twins spent much of their time together, rarely

47

TWO SETS OF TWINS WITH SELECTIVE MUTISM

interacted with other children outside of the home, and

they were often dressed alike by the parents. Neurological evaluation of both twins was normal overall, with
both twins noted to have a slight asymmetry of the
palpebral fissures.

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RESULTS
Evaluation of the twins was initially problematic
due to their extreme social anxiety and limited
communication. Therefore, prior to standardized
administration of neuropsychological measures,
an informal play or unstructured session was
utilized to help the twins relax and build rapport
with the examiners. The twins were initially
silent, but became more verbal as testing progressed. Twins A1 and A2 demonstrated notable
articulation difficulties. They made limited eye
contact with the examiners and would only do so
for brief moments before looking away. Twins B1
and B2 would not respond when the examiner was
looking at them, but would give minimal responses when the examiner looked away. Many
one-word responses were provided by Twins B1
and B2 in the form of a question, with an upward
inflection at the end as if they were asking for
approval to their response. Interestingly, once
Twin B2 became comfortable with the examiner,
she began to ask many short questions regarding
pictures that represented children involved in
everyday scenarios. Her questions often revealed
a lack of basic knowledge regarding common childhood activities and age appropriate functioning.
Neuropsychological results are presented for
both sets of twins in Table 1. Parent behavioral
rating data is presented in Table 2. Twins A1 and
A2 demonstrated a strikingly similar pattern of an
average KABC Mental Processing Composite
(MPC) score, average WISC-III Performance
IQ, and average to low average WISC-III Verbal
IQ. Their average to above average receptive
language skills were significantly better than
their low average to below average expressive
language skills. Assessment of the twins oromotor skills was successfully completed and both
twins demonstrated deficits in oromotor coordination. Both girls demonstrated a similar
pattern of slightly lower right (dominant) handed
performance than left (nondominant) handed

Table 1. Summary of Neuropsychological Test Results

in Standard Scores.
Measure
WISC-III VIQ
WISC-III PIQ
KABC MPC
CELF-REC
CELF-EXP
PPVT-III
PIC VOC
TOVA-OM
TOVA-CO
TOVA-RT
TOVA-VR
VMI
PEGS R
PEGS L
PEGS B
READ
SPELL
MATH
V COM
V SOC
V DLLV

Twin A1 Twin A2 Twin B1 Twin B2

90
103
106
118
86
91
92
54
118
72
89

95
108
105

80

87
108
105
112
75
93
92
90
111
87
93

93
107
97

84

50
83
91
50
50
71
65
73
104
71
76
89
66
72
76
82
95
73
71
53
59

63
84
80
57
57
73
60
40
108
74
88
86
86
90
58
90
83
70
71
53
56

Note. WISC-III VIQ/PIQ Wechsler Intelligence

Scale for Children Third Edition Verbal and
Performance IQ; KABC MPC Kaufman
Assessment Battery for Children Mental Processing Composite; CELF REC/EXP Clinical
Evaluation of Language Fundamentals Third
Edition Receptive and Expressive Language
Composites; PPVT-III Peabody Picture Vocabulary Test Third Edition; TOVA OM/CO/RT/
VR Test of Variables of Attention Omission,
Commission, Response Time, Variability; PIC
VOC WoodcockJohnson Picture Vocabulary
subtest; VMI Beery Developmental Test of
Visual-Motor Integration; PEGS R/L/B
Purdue Pegboard Right, Left, and Bilateral;
READ/SPELL/MATH Wechsler
Individual
Achievement Test Basic Reading, Spelling, and
Mathematics Reasoning; V COM/SOC/
DLLV Vineland Adaptive Behavior Scales
Communication, Socialization, Daily Living.

performance, although fine motor speed and coordination scores all fell within the average range.
On a computerized measure of attentional testing,
the twins performance was more discrepant with
Twin A2 performing within the average range
with a low average reaction time while Twin A1
demonstrated a high number of omissions and

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ROBERT M. GRAY ET AL.

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Table 2. Summary of Behavioral Assessment Scale for

ChildrenParent Rating Scale (BASC-P) data
in T-Scores.
BASC
Scale

Twin
A1

Twin
A2

Twin
B1

Twin
B2

HYP
AGG
CON
ANX
DEP
SOM
ATY
WTH
ATT
ADP
SOC
LED

45
46
45
47
46
39
36
91
43
39
42
41

45
48
49
51
43
41
36
85
43
41
45
41

49
32
41
40
46
47
55
63
58
33
27
30

55
32
37
36
43
50
55
54
63
33
25
19

Note. HYP Hyperactivity;

AGG Aggression;
CON Conduct Problems; ANX Anxiety;
DEP Depression;
SOM Somatization;
ATY Atypical; WTH Withdrawal; ATT
Attention; ADP Adaptability; SOC Social
Skills; LED Leadership.

below average reaction time. Both twins were

rated as below average in terms of their adaptive
communication skills.
Twins B1 and B2 demonstrated greater neuropsychological deficits than Twins A1 and A2. Like
Twins A1 and A2, their WISC-III Verbal IQ was
lower than their WISC-III Performance IQ score or
KABC MPC score, but their overall level of
performance across these indices was much
lower than Twins A1 and A2, generally falling
below the average range. Their receptive and expressive language performance was much more
uniformly depressed than Twins A1 and A2, ranging from the impaired to borderline range.
Qualitative analysis of both sets of twins
WISC-III verbal responses across subtests revealed a general tendency to respond in oneword sentences or short utterances. Twin B1
chose to respond to the Arithmetic subtest of the
WISC-III in writing rather than verbally. She
made attempts to point and gesture when responding to the Vocabulary subtest of the WISC-III and
several of the twins refused to respond to the
Comprehension subtest. Twin B2 offered occasional 23 word responses but responded at a very

slow rate. Her occasional sentence length

responses were characterized by poor grammar
and sentence construction (bicycle is you ride
in it).
The sisters demonstrated a discrepant fine
motor performance with Twin B1 performing
below the average range across all trials and
Twin B2 performing within the low average to
average range across right- and left-handed trials
but within the impaired range, when using both
hands. While dominant and nondominant trials
were not markedly discrepant, both sets of twins
demonstrated a pattern of generally better nondominant than dominant handed performance.
Twins B1 and B2 performed within the low end
of the average range on a measure of visual motor
integration. On a computerized measure of attentional testing, twins B1 and B2s performance was
similar to that of Twin A1, characterized by a high
number of omissions and below average reaction
time. This type of pattern characterized by slow
reaction time, frequent omissions, and a resultant
spuriously normal commission score is often
considered indicative of individuals with anxiety-related difficulties. On measures of academic
achievement, Twins B1 and B2 demonstrated a
generally similar pattern of results with reading
and spelling higher than math performance. This
may be related to language difficulties as the
subtest administered was a Mathematics Reasoning subtest, which features more receptive language demands than the basic reading and
spelling tasks which feature straightforward
directions and tasks that are typically overlearned
in school. The B twins adaptive behavior rating
performances were remarkably similar with
below average ratings across all measures, but
interestingly communication was noted as a relative strength while socialization and daily living
skills were rated as impaired.
Examination of parental ratings of behavioral
and emotional functioning (see Table 2) revealed
similar profiles within twin sets, and a pattern of
generally greater internalizing than externalizing
difficulties across the twin pairs. Parental ratings
of Twins A1 and A2 revealed few behavioral
problems or concerns overall, with the notable
exception of both twins significant elevations on
the Withdrawal scale, reflecting extremely shy,

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TWO SETS OF TWINS WITH SELECTIVE MUTISM

avoidant, and isolated behaviors. Parental ratings

of Twins B1 and B2 revealed mild concerns with
B1s withdrawn behaviors, while concerns with
B2s attention and concentration were noted.
Significant adaptive behavioral deficits were
noted for both Twin B1 and B2, characterized
by difficulties adapting to changes in their environment, and poor interpersonal and social interaction skills. While ratings indicating withdrawn
and socially avoidant behaviors were expected, it
is interesting that neither set of parents noted
concerns related to anxious behaviors. This
may suggest that parents of children with SM
tend to view their childs behavior as part of their
shy temperament, rather than as anxious behavior.
This finding may also be due to the wording of the
items on the Anxiety scale of the parent BASC,
which focus less on social anxiety and more on
worries and fears, which might rarely be
expressed by children with SM.
Recommendations for both sets of twins
included speech and language services with a
spoken language behavioral reinforcement program and a program to help teachers and parents
learn and utilize a variety of strategies for increasing responses. Additional recommendations for
Twins B1 and B2 included in home therapy to
assist the parents in separating the children more
often and exposing them to more diverse age
appropriate activities and experiences.

DISCUSSION
As described previously, there is little information
regarding SM in twins or the neuropsychological
performance of SM in general. To the authors
knowledge, this is the first published case
presentation of the neuropsychological performance of twins with SM and most likely the only
presentation of neuropsychological performance
in two sets of twins with SM. A review of the
literature indicates that the occurrence of SM in
twins is rare but does occur frequently enough
that larger sample sizes could be acquired.
Both sets of twins demonstrated similar behavioral presentations characterized by situation
specific anxiety, extreme passive behavior, lack
of responsivity, lack of peer interaction, and a

49

chronic course of SM spanning several years.

Both sets of twins had a history of prematurity,
and delayed speech and language development
but with no significant abnormal findings upon
neurological examination. Both sets of twins
demonstrated similar socialization patterns,
often dressing similarly and spending a great
deal of time playing and communicating with
each other, but rarely interacting with other
peers or adults outside the home. It is of interest
to note that these marked similarities were seen
across families of different cultural backgrounds.
While a history of anxiety was noted in family
members of Twins A1 and A2, the parents of
Twins B1 and B2 reported no significant family
history of anxiety or learning problems. Parent
ratings by both sets of parents revealed no concerns related to anxious behaviors, possibly
related to a lack of instrument sensitivity or
perhaps due to parents viewing their childrens
mute and withdrawn behaviors as part of their shy
temperament, rather than as anxious behavior.
Marked differences were noted in level of
neuropsychological functioning between the two
sets of twins, exemplifying two etiological factors
thought to be strongly associated with SM, namely
developmental delay and primary anxiety. Twins
A1 and A2 demonstrated a pattern of generally
intact neuropsychological functioning, but with
articulation difficulties, expressive language deficits, and significant anxiety. In contrast, Twins B1
and B2 demonstrated a neuropsychological pattern
that might be considered more consistent with
developmental delay related to prematurity, low
birth weight, or intrauterine exposure to medication, but it is important to note that their lack of
exposure to early preschool and socialization with
other children may have also contributed to their
poor neuropsychological performance.
While there are marked differences in the level
of neuropsychological performance between the
two sets of twins, similarities in the patterns of
neuropsychological performance were noted. Both
sets of twins demonstrated a lower Verbal than
Performance IQ score, and three of the four twins
demonstrated a similar performance pattern on a
computerized test of attentional functioning commonly noted in anxious individuals, characterized
by infrequent responding to stimuli leading to a

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50

ROBERT M. GRAY ET AL.

spuriously elevated Commission score as well as

slowed response speed, perhaps reflecting an
overly cautious approach to the task. Comparison
of each set of twins profile indicates striking
similarities and frequently parallel profiles,
although both sets of twins appeared to feature
one twin who was performing slightly better than
her twin sister.
Few twins with SM have been reported in the
literature, and it is difficult to determine the effect
that the twins had on each other in terms of
exacerbating or reinforcing maladaptive behaviors. It may be that twins with SM interact in a
manner that increases the severity and duration of
anxious and mute behaviors in comparison to
individual children presenting with SM. Both
sets of twins in the current study demonstrated a
tendency to isolate themselves and mimic each
others dress and behavior, which may have contributed to their lack of communication and interaction with others, and it was noted that both sets
of twins demonstrated minor improvements in
communication when separated. It would be of
interest to examine historical, behavioral, and
neuropsychological differences among dizygotic
and monozygotic twins with SM, although opportunities for such investigations are likely to be
quite limited.
In conclusion, the two sets of twins with SM
presented in this study exemplify the multiple
factors that can be associated with a lack of
speech in children including prematurity, speech
and general developmental delay, and anxiety.
Neuropsychological testing can assist the clinician in determining the profile of abilities of
children with SM, leading to intervention plans
targeting primary language and anxiety-related
difficulties or more significant developmental delay. Further investigations are necessary to determine the degree to which twins with SM interact
with each other in a manner that might exacerbate
the severity or duration of maladaptive behavior.
ACKNOWLEDGEMENT
This article is based on a poster presented at the
National Academy of Neuropsychology 2001 annual
meeting.

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