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Figure 1

Lifetime Psychiatric Severity Score Distribution. Score Interpretation: 0 = Few or no symptoms; 1 = Mild symptoms, little or no impairment; 2= Mild symptoms, some impairment; 3= Moderate symptoms, clear impairment; 4= Severe symptoms, clear impairment (see methods for more detailed description). Numbers above each bar are the percentage of Wolfram Syndrome participants with the indicated lifetime severity level as of the most recent clinic visit.
Lifetime Psychiatric Severity Score Distribution. Score Interpretation: 0 = Few or no symptoms; 1 = Mild symptoms, little or no impairment; 2= Mild symptoms, some impairment; 3= Moderate symptoms, clear impairment; 4= Severe symptoms, clear impairment (see methods for more detailed description). Numbers above each bar are the percentage of Wolfram Syndrome participants with the indicated lifetime severity level as of the most recent clinic visit.

Best-Estimate Lifetime Psychiatric Diagnoses in Wolfram Syndrome Participants.

Best Estimate Diagnoses Children Age 6-12 yrs (N=12) Adolescents Age 13-17 yrs (N=11) Adults 18-30 yrs (N=16) Total 6-30 yrs (N=39)
Any Neurodevelopmental or Disruptive Behavior Disorder, n (%) 6 (50) 3 (27) 3 (19) 12 (31)
ADHD-full criteria, n (%) 3 (25) 1 (9) 1 (6) 5 (13)
ADHD-unspecified, n (%) 1 (8) 0 (0) 2 (13) 3 (8)
ADHD-any, n (%) 4 (33) 1 (9) 3 (19) 8 (21)
Oppositional-Defiant Disorder, n (%) 4 (33) 2 (18) 1 (6) 7 (18)
Conduct Disorder, n (%) 0 (0) 1 (9) 0 (0) 1 (3)
Autism Spectrum Disorder, n (%) 1 (8) 1 (9) 0 (0) 2 (5)
Tourette Syndrome, n (%) 1 (8) 0 (0) 0 (0) 1 (3)
NDD-Unspecified, n (%) 0 (0) 1 (9) 0 (0) 1 (3)
Any Anxiety Disorder, n (%) 11 (92) 9 (82) 10 (63) 30 (77)
Generalized Anxiety Disorder, n (%) 1 (8) 1 (9) 3 (19) 5 (13)
Social Anxiety Disorder, n (%) 0 (0) 0 (0) 1 (6) 1 (3)
Separation Anxiety Disorder, n (%) 1 (8) 2 (18) 0 (0) 3 (8)
Specific Phobia, n (%) 1 (8) 1 (9) 1 (6) 3 (8)
Unspecified Anxiety Disorder, n (%) 9 (75) 5 (45) 6 (38) 20 (51)
Any Obsessive-Compulsive Spectrum Disorder, n (%) 4 (33) 4 (36) 4 (25) 12 (31)
Obsessive-Compulsive Disorder, n (%) 0 (0) 0 (0) 2 (13) 2 (5)
Excoriation Disorder, n (%) 4 (33) 3 (27) 2 (13) 9 (23)
Unspecified Obsessive-Compulsive Disorder, n (%) 0 (0) 1 (9) 0 (0) 1 (3)
Any Mood Disorder, n (%) 3 (25) 4 (36) 6 (38) 13 (33)
Major Depressive Disorder, n (%) 0 (0) 0 (0) 3 (19) 3 (8)
Persistent Depressive Disorder, n (%) 0 (0) 1 (9) 0 (0) (3)
Disruptive Mood Dysregulation Disorder, n (%) 1 (8) 1 (9) 0 (0) (5)
Unspecified Depressive Disorder, n (%) 2 (17) 2 (18) 2 (13) 6 (15)
Unspecified Bipolar Disorder, n (%) 0 (0) 0 (0) 1 (6) 1 (3)
Any Sleep-Wake Disorder, n (%) 2 (17) 3 (27) 7 (44) 12 (31)
Hypersomnolence Disorder, n (%) 1 (8) 3 (27) 6 (38) 10 (26)
Sleep Apnea, reported diagnosis based on past sleep study, n (%) 1 (8) 0 (0) 1 (6) 2 (5)
Restless Legs Syndrome/Periodic Limb Movements of Sleep, based on reported symptoms and past sleep study, n (%) 1 (8) 0 (0) 0 (0) 1 (3)
Unspecified Sleep-Wake Disorder, n (%) 0 (0) 0 (0) 1 (6) 1 (3)
Hallucination-like Experiences, n (%) 6 (50) 3 (27) 6 (38) 15 (38)
Unspecified Psychotic Disorder, n (%) 1 (8) 0 (0) 0 (0) 1 (3)
Unspecified Eating Disorder, n (%) 1 (8) 0 (0) 0 (0) 1 (3)
Any Trauma and Stressor Related Disorder, n (%) 1 (8) 1 (9) 3 (19) 5 (13)

Characteristics of Wolfram syndrome participants with at least one psychiatric interview in the 2013-2017 timeframe (Total N=39).

1 clinic visit, n (%) 4 (10)
2 clinic visits, n (%) 8 (21)
3 clinic visits, n (%) 8 (21)
4 clinic visits, n (%) 19 (49)
Age at first visit, mean (range) 14 (5-27)
Age at last visit, mean (range) 16 (6-30)
Male sex at birth, n (%) 16 (41)
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Medicine, Basic Medical Science, other