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Table 1 Age and sex distribution, CSF OCB positivity rates, CSF WCC, CSF TP and CSF glucose and lactate in patients diagnosed with MDS. §Assuming that the respective parameter was normal in samples classified as “normal” or “otherwise normal” (see the “Results” section for details). Abbreviations: CSF cerebrospinal fluid, LP lumbar puncture, MDS myelinoclastic diffuse sclerosis, OCB oligoclonal bands, TP total protein, WCC white cell count

From: Myelinoclastic diffuse sclerosis (Schilder’s disease) is immunologically distinct from multiple sclerosis: results from retrospective analysis of 92 lumbar punctures

 

Total cohort

VLCFA-tested subgroup (VLCFA normal in all)

All

Adult-onset

Childhood-onset

All

Adult-onset

Childhood-onset

Sex ratio, male:female

1:1.13

1:2.67

1:0.76

1:1.17

1:2.33

1:1

Age at onset, median (range), years

12 (2–69)

29 (18–69)

9 (2–17)

10.5 (3–50)

32.5 (20–50)

8.5 (3–17)

OCB, positive, all LPs

23.1% (9/39)

15.4% (2/13)

26.9% (7/26)

14.3% (4/28)

0% (0/10)

22.2% (4/18)

OCB, positive, all LPs, including LPs classified as “normal” (1983–)

17.6% (9/51)

0% (2/18)

0% (7/33)

10.5% (4/38)

0% (0/14)

16.6% (4/24)

OCB, positive, first LP

21.9% (7/32)

10% (1/10)

27.3% (6/22)

13.6% (3/22)

0% (0/8)

21.4% (3/14)

OCB, positive, patients

25.7% (9/35)

18.2% (2/11)

29.2% (7/24)

16.7% (4/24)

0% (0/8)

25% (4/16)

WCC, elevated, all LPs

20.7% (12/58)

32.1% (9/28)

10% (3/30)

7.7% (2/26)

20% (2/10)

0% (0/16)

WCC, elevated, all LPs, including LPs classified as “normal”

15.2% (12/79)

26.5% (9/34)

6.7% (3/45)

4.9% (2/41)

14.3% (2/14)

0% (0/27)

WCC, elevated, first LP

25% (10/40)

43.8% (7/16)

12.5% (3/24)

10.5% (2/19)

28.6% (2/7)

0% (0/12)

WCC, elevated, patients

24.4% (10/41)

43.8% (7/16)

12% (3/25)

10.5% (2/19)

28.6% (2/7)

0% (0/12)

WCC, elevated, median (range), all LPs

25 (11–277; N = 11)

24 (12–277; N = 8)

32 (11–36; N = 3)

150 (23–277; N = 2)

150 (23–277; N = 2)

N.a. (N.a.; N = 0)

WCC, elevated, median (range), first LPs

25 (11–277; N = 9)

24 (12–277; N = 6)

32 (11–36; N = 3)

150 (23–277; N = 2)

150 (23–277; N = 2)

N.a. (N.a.; N = 0)

WCC, elevated, median (range), patients

25 (11–277; N = 9)

25 (15–277; N = 6)

32 (11–36; N = 3)

150 (23–277; N = 2)

150 (23–277; N = 2)

N.a. (N.a.; N = 0)

TP, elevated, all LPs

43.8% (28/64)

46.4% (13/28)

41.7% (15/36)

37.5% (12/32)

40% (4/10)

36.4% (8/22)

TP, elevated, all LPs, including LPs classified as “normal”

32.9% (28/85)

38.2% (13/34)

29.4% (15/51)

26.1% (12/46)

28.6% (4/14)

25% (8/32)

TP, elevated, first LP

43.8% (28/64)

46.4% (13/28)

41.7% (15/36)

37.5% (12/32)

40% (4/10)

36.4% (8/22)

TP, elevated, patients

41.3% (19/46)

43.8% (7/16)

40% (12/30)

37.5% (9/24)

42.9% (3/7)

35.3% (6/17)

TP, elevated, median (range), all LPs

114 (46–220; N = 22)

135 (46–182; N = 11)

112 (56–220; N = 11)

103 (46–212; N = 7)

105.5 (46–165; N = 2)

103 (56–212; N = 5)

TP, elevated, median (range), first LPs

116 (46–220; N = 15)

97.5 (46–180; N = 6)

116 (63–220; N = 9)

134 (46–212; N = 6)

105.5 (46–165; N = 2)

134 (63–212; N = 4)

TP, elevated, median (range), patients

107.5 (46–220; N = 18)

60 (46–182; N = 7)

112 (56–220; N = 11)

103 (46–212; N = 7)

105.5 (46–165; N = 2)

103 (56–212; N = 5)

Glucose, normal, all LPs

85% (17/20)

80% (4/5)

86.7% (13/15)

100% (14/14)

100% (4/4)

100% (10/10)

Glucose, normal, all LPs, including LPs classified as “normal”

93.9% (46/49)

92.9% (13/14)

94.3% (33/35)

100% (33/33)

100% (11/11)

100% (22/22)

Lactate, normal, all LPs

100% (7/7)

100% (1/1)

100% (6/6)

100% (7/7)

100% (1/1)

100% (6/6)

Lactate, normal, all LPs, including LPs classified as “normal”

100% (35/35)

100% (10/10)

100% (25/25)

100% (24/24)

100% (8/8)

100% (16/16)