Background/Purpose Primary Sjögren’s syndrome (PSS) is an autoimmune exocrinopathy with multiple clinical manifestations. 

We aimed to evaluate the frequency and type of cognitive dysfunction and its association with anti-ganglioside antibodies in patients with PSS.

Methods

Twenty-eight female cases  with PSS fulfilling the American-European consensus criteria  and 20 female control subjects matched in terms of age and education level, examined between June,  2011 and August, 2013 were enrolled into the study. The mean age was 45,7±10,6 in PSS, and 42,1±10,3 (p=0,27) in the control group.

 Neuropsychological tests including attention, information processing speed, short term memory, long term memory, visual memory and visual-spatial perception were examined in both group. Verbal frequency  functions were examined and measured by COWAT, naming concentration by BNT, verbal learning by SDLT, immediate, short term and long term verbal memory by AVLT, visual spatial perception with BJLOT, and immediate, short term and long term visual memory with RCFT. Cognitive dysfuntion was defined as ”mild” if there was a deterioration in 1 or 2 test performances , and as ”severe” if an impairment in 3 or 4 test performances was observed.

A standard western blot test (Euroimmun, Germany)  was used to investigate IgM and IgG anti-ganglioside antibodies (GM1, GM2, GM3, GD1a, GD1b, GT1b, GQ1b)  in the  patients and healthy controls.

Results

Primary Sjögren’s syndrome patients had lower performance in tests evaluating verbal learning, verbal memory and visual-spatial perception  in SDLT and BJLOT compared to healthy controls (p<0,01) in PSS group (Table 1). Lower performance was also observed in the patient group  in clock drawing, COWAT, PASAT, and AVLT  tests, however the difference did not reach a significant level (p>0,05).

	Patient (n=28)	Healthy control (n=20)	P
Clock Drawing	6,71 ± 0,71	7,00 ± 0,00	0,07
COWAT	25,71 ± 10,69	28,15 ± 8,43	0,38
PASAT	46,85 ± 8,65	51,00 ± 7,07	0,10
SDLT	8,0 ± 7,34	16,50 ± 4,34	<0,01
AVLT
Immediate verbal memory	6,32 ± 1,92	7,40 ± 1,84	0,78
Long term verbal memory	10,32 ± 2,34	10,75 ± 2,02	0,63
BNT	33,50 ± 1,87	32,60 ± 2,58	0,21
BJLOT	21,07 ± 3,75	24,45 ± 2,46	<0,01

 

 

IgM anti-ganglioside antibodies were positive in 9  patients with PSS, and 2  in healthy controls. Three PSS patient had two IgM anti-ganglioside antibody positivity  (GM1-GM2, GM3-GD1b, GT1b-GQ1b). IgG anti-ganglioside antibodies were positive in 3  patients with PSS, and 1  in healthy controls.

Conclusion

We found  impairment in attention, information processing speed, long term memory and short term memory in PSS patients .   Anti-ganglioside IgM antibodies may play a role in cognitive dysfunction in PSS by autoimmune neuroinflammation.

 Implementing detailed neuropsychological tests is  helpful in assessing subclinical cognitive dysfunction in PSS and early treatment.

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