Table 4 Lab and diagnostic abnormalities associated with immunotherapy responsiveness.

From: Diagnostic abnormalities, disease severity and immunotherapy responsiveness in individuals with Down syndrome regression disorder

 

Immunotherapy Responsiveness* When Feature Present

Immunotherapy Responsiveness* When Feature Absent

p Value

95% CI

Odds

Ratio

DSRD Criteria (probable v. possible)

79/109 (72%)

39/55 (71%)

0.83

0.80–1.31

1.03

History of Autoimmune Disease

63/85 (74%)

55/79 (70%)

0.53

0.79–1.58

1.12

Catatonia Present

93/123 (75%)

26/41 (63%)

0.13

0.84–3.81

1.79

C3 Abnormal

39/48 (81%)

78/116 (67%)

0.07

0.93–4.80

2.11

C4 Abnormal

31/40 (78%)

86/124 (69%)

0.32

0.66–3.51

1.52

ANA Abnormal

29/48 (60%)

89/116 (77%)

0.04

0.23–0.95

0.46

TPO Abnormal

39/56 (70%)

78/108 (72%)

0.73

0.43–1.79

0.88

Thyroglobulin Abnormal

32/46 (70%)

85/118 (72%)

0.75

0.42–1.87

0.89

CRP Abnormal

5/7 (71%)

113/157 (72%)

0.97

0.18–5.20

0.97

ESR Abnormal

24/29 (83%)

93/135 (69%)

0.14

0.77–6.07

2.17

Ferritin Abnormal

22/27 (81%)

96/137 (70%)

0.23

0.67–5.30

1.88

Cytokines Abnormal

46/54 (85%)

60/88 (68%)

0.03

1.12–6.43

2.68

EEG Abnormal

38/49 (77%)

79/115 (69%)

0.25

0.72–3.43

1.57

MRI Abnormal

47/53 (87%)

59/111 (62%)

< 0.001

3.15–20.17

7.98

LP Abnormal

30/34 (88%)

88/130 (68%)

0.02

1.18–10.82

3.58

MRI and LP Abnormal

20/21 (95%)

59/96 (61%)

0.01

1.75–105.1

13.56

EEG, MRI, and LP Abnormal

15/16 (94%)

44/76 (58%)

0.02

1.37–86.87

10.91

  1. Legend: Antinuclear antibody (ANA), complement (C), cerebrospinal fluid (CSF), C-reactive protein (CRP), electroencephalogram (EEG), immunoglobulin G (IgG), lumbar puncture (LP), magnetic resonance imaging (MRI), oligoclonal band (OCB), thyroid peroxidase. *Immunotherapy responsiveness is defined as an improvement greater than 50% on the Bush-Francis Catatonia Rating Scale or Neuropsychiatric Inventory Questionnaire within three months of starting any immunotherapy (includes steroids, IVIg, rituximab, mycophenolate mofetil, and/or tofacitinib).