LETTERS

A Case of Dandy-Walker
Variant Disorder Associated
With Multiple
Neuropsychiatric
Comorbidities Managed With
Behavioral Therapy
To the Editor: Attention-deficit hy-
peractivity disorder (ADHD) is
characterized by deficits in attention,
with impulsivity and motoric hyper-
activity, and it may have predomi-
nance of either inattention or
hyperactivity or have a mixture of
both. ADHD patients have a core
problem of being unable to delay or
inhibit a response, which is consid-
ered to be a central executive func-
tion, and one of the structures
modulating this is the cerebellum.1
Various studies have already pointed
toward cerebellar deficits in ADHD,
and vermal volume was consistently
found to be significantly less in
children with ADHD.2
The coinage Dandy-Walker (DW)
Complex comprises three entities:
Dandy-Walker malformation, Dandy
Walker variant, and the megacis-
terna magna, of which the Dandy-
Walker variant consists of cerebellar
dysgenesis without posterior fossa
enlargement, along with variable
degree of cerebellar vermis hypopla-
sia.3 Cerebellar hypoplasia has been
reported to be associated with mul-
tiple features in subjects, for exam-
ple, mental retardation, autism,
childhood-onset schizophrenia, and
so on.4 Nonetheless, the interesting
association of Dandy-Walker Com-
plex and ADHD could be a rare
clinical entity. In fact, a PubMed
search revealed only two such case
examples: In the first report, ADHD
was noted in a 13-year-old boy who
had Pascual-Castroviejo syndrome
that included Dandy-Walker Mal-
formation.5 The second case report
described ADHD in a 14-year-old
adolescent patient who had Dandy-
Walker Variant and intellectual dis-
ability.6 We present the case of
a 5-year-old child who had a constel-
lation of seizures, ADHD, and in-
tellectual disability in the backdrop
of a Dandy-Walker variant
pathology.
Clinical Presentation
A 5-year-old boy with no significant
family history; past history of
delayed birth cry; delayed speech,
social, adaptive, and intellectual
functioning, presented to our out-
patient department with attacks of
seizure since the age of 1 year. The
attacks would begin with localized
sweating in forehead, followed by
a brief period of blank stare in which
he became unresponsive to and un-
aware of his surroundings, and sub-
sequently had loss of consciousness,
uprolling of eyeballs, tonic–clonic
jerks of the limbs, aversive head
movement to right side, incontinence
of urine, and post-ictal lethargy.
Along with the above, our patient
was noted to have features of marked
hyperactivity, poor attention, and
impulsive behavior since the age of
3 years, which made it difficult for
him to attend school or play mean-
ingfully with his peer-group. He was
clinically diagnosed as a case of
Cryptogenic Localization-related ep-
ilepsy, with ADHD and mild mental
retardation.
Investigations revealed abnormal
EEG, showing generalized spike and
slow waves throughout the tracing,
once suggestive of electrical status,
and, in another record, left temporo-
frontal spike slow waves occurring
randomly, lasting about 1 second,
when EEG was repeated later on. A
CT scan of brain showed inferior
fourth ventricle communicating with
a posterior fossa cyst through wid-
ened vallecula of inferior surface of
cerebellum, and a prominent fourth
ventricle was noted, based on which
an impression of Dandy-Walker
Variant was made. Psychological
and behavioral assessment showed
a developmental age (DA) of 48
months, developmental quotient
(DQ) of 50, as per Developmental
Screening Test, and Social Age (SA)
of 54 months and Social Quotient
(SQ) of 57, as per the Vineland Social
Maturity Scale. A Conner’s ADHD
rating revealed a score of 26, which
was above the cutoff of 15 for di-
agnosis of the disorder. Blood bio-
chemistry reports were unremarkable.
He had earlier been treated with
phenobarbitone 30 mg/day, but his
hyperactivity was aggravated after
taking the medicine, so it was grad-
ually tapered off, and carbamazepine
was added and slowly built up to
a dose of 600 mg/day, along with
clobazam in a dose of 15 mg/day
and olanzapine 7.5 mg/day. The boy
became seizure-free with this com-
bination. He had already been
started on olanzapine to control
hyperactivity before a final diagnosis
of ADHD was made. He was also
subjected to a structured activity
scheduling, taught self-help skills,
and given specific tasks to improve
attention, and reinforcements were
used for tackling his hyperkinesis.
With the above combined regime
of psycho-behavioral and pharma-
cological therapy, the child
showed significant response in
his target symptoms (Conner’s
score coming down to 13 after
3 weeks) and an improved overall
quality of life as per his guardian’s
account.

J Neuropsychiatry Clin Neurosci 25:3, Summer 2013 http://neuro.psychiatryonline.org E3

LETTERS
Discussion
The case so described provided for
an absorbing study. The patient had
a multitude of clinical presentations,
including intellectual disability,
epilepsy, and ADHD.
The earlier cases reporting the
psychiatric comorbidities of DW
variant had adolescent subjects,6
whereas our patient was a child.
Also, the pattern of comorbidity
in our case included seizures,
ADHD, and intellectual disability,
whereas the earlier cases had
conduct disorder, enuresis, and
stereotypic movements.
The structural abnormality of
Dandy-Walker variant involving
the inferior cerebellum seems to be
of significance in our patient.
Researchers7 have postulated a “de-
velopmental chronometry hypothe-
sis,” in which a brain area that
develops over a protracted period of
time is more vulnerable to various
potential hypoxic, toxic, traumatic,
and other types of insults, as com-
pared with those areas that develop
over a relatively quicker period.
Both the cerebellum and prefrontal
cortex are two such areas and are
important for motor functions and
attention aspects of the subject,
which are disturbed in ADHD
patients. Both have intricate internal
connections of functional neural net-
works and thus have important bi-
ological underpinnings in our case.8
In our case example, Dandy-
Walker Variant was also associated
with seizure. It was noted that
epilepsy associated with cerebellar
malformations was usually focal,
most often with a temporal lobe
focus.9 It is to be noted that, in our
case illustration, EEG revealed left
E4 http://neuro.psychiatryonline.org
temporo-frontal epileptiform
References
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Another peculiarity was that the
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and its complications antedate the
onset of seizures in 82% of cases; per-
haps this variance could be partly ex-
plained by the additional presence of
a structural brain defect in our patient.
We successfully managed the
case with behavior therapy after
a discussion with the child’s mother.
Full control of seizures and probably
olanzapine also contributed to the
overall clinical improvement.
In conclusion, our case example
adds to the existing body of literature
documenting a psychiatric
comorbidity pattern in a child with
Dandy-Walker Variant. It also dem-
onstrated that behavioral therapy
can be successfully applied to man-
age ADHD in Dandy-Walker Variant
with comorbid epilepsy and intellec-
tual disability.
Arnab Bhattacharya, M.B.B.S.,
D.P.M.
Tata Motors Hospital
Jamshedpur, India
Pushpal Desarkar, M.D., D.P.M.
Dept. of Deaddiction Psychiatry
University of Toronto
Canada
Shamsul Haque, M.D., D.P.M.
Central Institute of Psychiatry
Ranchi, India
10. Hermann B, Jones J, Dabbs K, et al: The
Correspondence:
Dr. Bhattacharya;
e-mail: arnab2013@gmail.com
J Neuropsychiatry Clin Neurosci 25:3, Summer 2013
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