Table 1.
Demographics and clinical features
| DSRD (n= 72) | DS (n= 1217) | p value | 95%CI | |
|---|---|---|---|---|
| Sex | ||||
|
Male Female |
35 (49%) 37 (51%) |
578 (47.5%) 638 (52.5%) |
0.85 | 0.80–1.31 |
| Race | ||||
|
Caucasian Asian Black |
55 (76%) 10 (14%) 7 (10%) |
1058 (87%) 109 (9%) 49 (4%) |
0.06 0.15 0.22 |
0.77–1.01 0.85–2.83 0.13–5.14 |
| Hispanic ethnicity | 51 (71%) | 370 (35%) | < 0.001 | 1.96–2.76 |
| Medical history | ||||
|
Obstructive sleep apnea (n= 70) Congenital heart disease Autoimmune disease (any) Thyroid disease |
35 (50%) 38 (53%) 33 (46%) 25 (34%) |
523 (43%) 548 (45%) 426 (35%) 182 (15%) |
0.22 0.12 0.02 < 0.001 |
0.91–1.48 0.96–1.51 1.04–1.75 1.64–3.27 |
| Age at onset (median, IQR) | 14 (12–17) | 11 (7–15)a | < 0.001 | 1.05–3.55 |
|
Preceding trigger Infectionb Change in school/work/home environment Loss of family/caregiver/friend Change in residence Abuse Death in immediate social network Medical change |
37 (51%) 16 (43%) 10 (27%) 4 (10%) 2 (5%) 2 (5%) 2 (5%) 1 (3%) |
|||
| Months to symptom peak (median, IQR) | 3 (1–6) | |||
| Clinical symptoms | ||||
|
Social withdrawal Loss of acquired skills New autistic features Diminished eye contact Apathy (n = 70) Impaired attention Anxiety Mutism/expressive language regression Insomnia Anorexia Whispered speech Confusion/disorganization Memory impairment Catatonia Freezing/bradykinesia Abulia/avolition (n = 71) Emotional lability (n = 61) Stereotypy Urinary retention Incontinence (urine/feces) Inappropriate/mirthless laughter (n = 65) Circadian rhythm alteration New obsessive-compulsive tendencies Tics (n = 69) Aggression/agitation (n = 71) Hyperactivity Autonomic dysfunction (n = 58) Persistent focal neurologic deficits Seizure (n = 71) Transient neurologic deficits/TIA |
72 (100%) 72 (100%) 72 (100%) 69 (96%) 66 (94%) 64 (89%) 62 (86%) 59 (82%) 58 (81%) 56 (78%) 56 (78%) 55 (76%) 55 (76%) 55 (76%) 52 (72%) 46 (65%) 37 (61%) 38 (53%) 38 (53%) 36 (50%) 34 (52%) 26 (36%) 24 (33%) 19 (28%) 19 (27%) 11 (15%) 8 (13%) 3 (4%) 1 (1%) 1 (1%) |
|||
CI confidence interval, DS Down syndrome, DSRD Down syndrome regression disorder, IQR interquartile range, TIA transient ischemic attack
aAge at data collection
bAny non-life-threatening bacterial or viral infection (e.g., upper respiratory tract infection, urinary tract infection, or pneumonia)