Kaohsiung Journal of Medical Sciences (2016) 32, 52e54
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LETTER TO THE EDITOR
Cerebellar cognitive affective syndrome:
Attention deficitehyperactivity disorder
episode of adolescent with cerebellar atrophy
in a psychiatric ward
Dear Editor,
Since Dr Jeremy Schmahmann proposed the existence of
cerebellar cognitive affective syndrome (CCAS) [1,2], we
have rethought the function of the cerebellum. Besides its
ability to modulate action, the cerebellum affects cognition, emotion, behavior, personality, and even psychosis
[3e5]. However, cerebellar disease remains unfamiliar
territory for mental health professionals. This is a case
report of a female adolescent who was diagnosed with
cerebellar atrophy only when she was admitted into the
psychiatry ward because of an episode of CCAS/attention
deficitehyperactivity disorder (ADHD). The patient is a 16year-old girl who was brought to our psychiatry outpatient
department (OPD) for a secondary opinion because she
had been diagnosed by a local doctor as having “bipolar I
disorder, manic episodes.” She was talkative, hyperactive, distractible, changeable, emotional, and lacking in
adequate interpersonal skills. The physician examined her
but found no typical signs of mania, such as euphoria or
exaggeration. Therefore, she was admitted to our psychiatric ward for further evaluation and intervention. The
nurse in the ward also observed that the patient had an
unsteady gait. The patient’s motor imbalance and slurred
speech since childhood were reported by her parents at
the end of the evaluation. Magnetic resonance imaging
was performed and finally indicated mild bilateral atrophy
of the cerebellum. The patient was referred to a clinical
psychologist for further evaluation. Based on the psychological assessment result, the clinical psychologist suggested it would be prudent to treat the patient’s acute
mental symptoms as ADHD in CCAS, instead of mania.
After treatment with a low dose of methylphenidate, the
ADHD symptoms significantly improved on the 1st day and
were controlled for 1 week. Then the patient was discharged, and stopped methylphenidate treatment
because of nausea. Nevertheless, her ADHD symptoms did
not relapse. We reassessed the patient’s cognitive function in the OPD 3 months later. Her intelligence development was delayed as manifested in many functions,
especially in her performance [Wechsler Adult Intelligence Scale, third edition: verbal intelligence quotient
(IQ) Z 91; performance IQ Z 72; full-scale IQ Z 81]. Her
longitudinal development profile was constructed using
both motor symptoms (ataxia) and nonmotor symptoms
(CCAS) in Table 1.
In this case, we can see that some mental symptoms of
CCAS may appear in the patient’s early age and remain
until later, and that some others may have sudden onset
and can be treated at the acute stage. We want to indicate
the importance for psychiatric staff to have a clear
concept of cerebellar disease and work as a team for early
detection and intervention for patients to reduce the
permanent impairment of their cognitive and affective
functions.
Conflicts of interest: All authors declare no conflicts of interest.
http://dx.doi.org/10.1016/j.kjms.2015.10.006
1607-551X/Copyright ª 2015, Kaohsiung Medical University. Published by Elsevier Taiwan LLC. This is an open access article under the
CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Letter to the Editor
Table 1
Development profile of a 16-year-old girl with cerebellar atrophy.
Age
Education
0y
Kindergarten
Motor
dysfunction
(ataxia)a
1.Poor motor balancede.g., could not stand on one leg
2.Limb weaknessde.g., unsteady gait, like penguin pace
3.Poor fine-motor coordinationde.g., could not tie shoelaces
4.Slurred speech, dysarthria
Trait: very shy and
1.Trait: mostly interacted only
dependent
with her mother and older sister
2.Cognition: her first-grade teacher
noted her inattention and poor
learning, but nothing similar to
the symptoms of pure mental
retardation.
Nonmotor
dysfunction
(CCAS)
6y
Primary school
13 y
Junior high school
1.Trait: childish behavior,
poor social skills, and lack
of empathy. A teacher visited
her family because of her
isolation from peer groups.
2.Cognition: her academics still
ranked behind her classmates’.
16 y
Vocational school, music class
Her parents arranged for her to play musical
instruments to train her fine-motor control,
but her technical performance was far
behind her classmates.
1.D/Dx: she was initially diagnosed by an LMD
as having a bipolar disease manic episode and
finally diagnosed in our psychiatric ward as
having a cerebellar atrophy ADHD episode.
2.Tx: her ADHD was treated with
methylphenidate, and she was released
immediately.
3.Cognition: intellectual function was assessed
in the OPD (WAIS-III: VIQ Z 91; PIQ Z 72;
FIQ Z 81).
4.Trait: she still showed childish behavior,
distractibility, inadequate emotion, and
poor social skills.
ADHD Z attention deficitehyperactivity disorder; CCAS Z cerebellar cognitive affective syndrome; D/Dx Z differential diagnosis; FIQ Z full-scale intelligence quotient (IQ); LMD Z local
medical doctor; OPD Z outpatient department; PIQ Z performance IQ; Tx Z treatment; VIQ Z verbal IQ; WAIS-III Z Wechsler Adult Intelligence Scale, third edition.
a
The patient’s parents were used to denying or rationalizing her motor and nonmotor development delay until she was diagnosed and treated as a CCAS patient.
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References
[1] Schmahmann JD, Sherman JC. The cerebellar cognitive affective syndrome. Brain 1998;121:561e79.
[2] Schmahmann JD. Disorders of cerebellum: ataxia, dysmetria of
thought, and the cerebellar cognitive affective syndrome. J
Neuropsychiatry Clin Neurosci 2004;16:367e77.
[3] Karatekin C, Lazareff JA, Asarnow RF. Relevance of the cerebellar hemispheres for executive function. Pediatr Neurol
2000;22:106e12.
[4] Schnahmann JD. The role of the cerebellum in cognition
and emotion: personal reflections since 1982 on the dysmetria of thought hypothesis, and its historical evolution
from theory to therapy. Neuropsychol Rev 2010;20:
236e60.
Letter to the Editor
[5] Levisohn L, Cronin-Golomb A, Schmahmann JD. Neuropsychological consequences of cerebellar tumor resection in children.
Brain 2000;123:1041e50.
Chi Chang*
Shun-Wun Siao
Department of Psychiatry,
Kaohsiung Veterans General Hospital,
Kaohsiung,
Taiwan, ROC
*Corresponding author. Department of Psychiatry,
Kaohsiung Veterans General Hospital, 386 Dazhong First
Road, Zuoying District, Kaohsiung City 813, Taiwan, ROC.
E-mail address: [email protected] (C. Chang)