Psychological status of patients with mucopolysaccharidosis type II and their parents.
BACKGROUND
The aim of the present study was to delineate the psychological status of 10 patients with the attenuated phenotype of mucopolysaccharidosis type II (MPS-II) and their parents (six fathers and five mothers) for the improvement of clinical management.


METHODS
Intellectual ability was evaluated using the Wechsler Intelligence Scale. Activities of daily living (ADL) was assessed using the Functional Independence Measure. The personality and psychiatric aspects were analyzed using the Yatabe-Guilford Personality test (Y-G test) and the Tree-Drawing Test. Mental health was assessed using the General Health Questionnaire 60 (GHQ-60) and State-Trait Anxiety Inventory (STAI).


RESULTS
Intellectual background, measured with full-scale, verbal and performance IQ, were 72.8, 76.1 and 79.3, respectively. Nine of 10 patients were not judged as having neurosis and a psychotic tendency with the Y-G test. In the tree-drawing test, many patients drew a tree without ground, suggesting that they have difficulties in making relationships with surrounding people and the community. The child patient with a psychosis pattern on the Y-G test, drew a bizarre tree, suggesting psychological problems. GHQ-60 and STAI survey indicated that the patients and their parents had higher levels of anxiety. A significant negative correlation between GHQ-60 score and ADL (R = -0.77) was identified, suggesting that the psychological status may worsen as ADL decreases.


CONCLUSIONS
Patients with MPS-II and their parents had higher risks for mental problems. Understanding psychological status is essential when providing genetic counseling or therapeutic intervention.
Kuratsubo
Izumi
I
Department of Pediatrics, Gifu University Graduate School of Medicine, Gifu, Japan. ikuratsubo@yahoo.co.jp
Suzuki
Yasuyuki
Y
Orii
Koji O
KO
Kato
Tomomi
T
Orii
Tadao
T
Kondo
Naomi
N
D000293:Adolescent; D000328:Adult; D005260:Female; D006801:Humans; D007360:Intelligence; D008297:Male; D008875:Middle Aged; D016532:Mucopolysaccharidosis II / Q000523:psychology*; D010290:Parents / Q000523:psychology*; D010551:Personality