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2015 ; 10
(ä): 66
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Selective cognitive and psychiatric manifestations in Wolfram Syndrome
#MMPMID26025012
Bischoff AN
; Reiersen AM
; Buttlaire A
; Al-Lozi A
; Doty T
; Marshall BA
; Hershey T
Orphanet J Rare Dis
2015[May]; 10
(ä): 66
PMID26025012
show ga
BACKGROUND: Wolfram Syndrome (WFS) is known to involve diabetes mellitus,
diabetes insipidus, optic nerve atrophy, vision loss, hearing impairment, motor
abnormalities, and neurodegeneration, but has been less clearly linked to
cognitive, sleep, and psychiatric abnormalities. We sought to determine whether
these abnormalities are present in children, adolescents, and young adults with
WFS compared to age- and gender-matched individuals with and without type 1
diabetes using standardized measures. METHODS: Individuals with
genetically-confirmed WFS (n?=?19, ages 7-27) were compared to age- and gender-
equivalent groups of individuals with type 1 diabetes (T1DM; n?=?25), and
non-diabetic healthy controls (HC: n?=?25). Cognitive performance across multiple
domains (verbal intelligence, spatial reasoning, memory, attention, smell
identification) was assessed using standardized tests. Standardized self- and
parent-report questionnaires on psychiatric symptoms and sleep disturbances were
acquired from all groups and an unstructured psychiatric interview was performed
within only the WFS group. RESULTS: The three groups were similar demographically
(age, gender, ethnicity, parental IQ). WFS and T1DM had similar duration of
diabetes but T1DM had higher HbA1C levels than WFS and as expected both groups
had higher levels than HC. The WFS group was impaired on smell identification and
reported sleep quality, but was not impaired in any other cognitive or
self-reported psychiatric domain. In fact, the WFS group performed better than
the other two groups on selected memory and attention tasks. However, based upon
a clinical evaluation of only WFS patients, we found that psychiatric and
behavioral problems were present and consisted primarily of anxiety and
hypersomnolence. CONCLUSIONS: This study found that cognitive performance and
psychological health were relatively preserved WFS patients, while smell and
sleep abnormalities manifested in many of the WFS patients. These findings
contradict past case and retrospective reports indicating significant cognitive
and psychiatric impairment in WFS. While many of these patients were diagnosed
with anxiety and hypersomnolence, self-reported measures of psychiatric symptoms
indicated that the symptoms were not of grave concern to the patients. It may be
that cognitive and psychiatric issues become more prominent later in life and/or
in later stages of the disease, but this requires standardized assessment and
larger samples to determine. In the relatively early stages of WFS, smell and
sleep-related symptoms may be useful biomarkers of disease and should be
monitored longitudinally to determine if they are good markers of progression as
well. TRIAL REGISTRATION: Current Clinicaltrials.gov Trial NCT02455414 .
|Adolescent
[MESH]
|Adult
[MESH]
|Child
[MESH]
|Cognition/*physiology
[MESH]
|Diabetes Mellitus, Type 1/physiopathology/psychology
[MESH]
free
free
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