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Cognitive function and 99mTc-ECD brain SPECT are significantly correlated in patients with primary Sjögren syndrome: a case–control study
  1. V Le Guern1,
  2. C Belin2,
  3. C Henegar1,
  4. C Moroni2,
  5. D Maillet2,
  6. C Lacau2,
  7. J L Dumas3,
  8. N Vigneron4,
  9. L Guillevin1
  1. 1
    Department of Internal Medicine, Hôpital Cochin, Paris, France
  2. 2
    Department of Neurology, Hôpital Avicenne, Bobigny, France
  3. 3
    Department of Radiology, Hôpital Avicenne, Bobigny, France
  4. 4
    Department of Nuclear Medicine, Hôpital Avicenne, Bobigny, France
  1. Correspondence to Dr V Le Guern, Department of Internal Medicine, Hôpital Cochin, 27, rue du faubourg Saint-Jacques, 75679 Paris Cedex 14, France; veronique.le-guern{at}


Objectives: To assess subclinical central nervous system (CNS) involvement in primary Sjögren syndrome (pSS), by comparing standard brain MRI, in-depth neuropsychological testing and 99mTc-ECD brain single-photon emission computed tomography (SPECT) of patients with pSS with matched controls.

Methods: 10 women (<55 years old), with pSS defined using European–American criteria, presence of anti-SSA and/or anti-SSB antibodies and no history of neurological involvement were prospectively investigated, and compared with 10 age- and sex-matched controls. All subjects underwent, within 1 month, brain MRI, neuropsychological testing, including overall evaluation and focal cognitive function assessment, and 99mTc-ECD brain SPECT.

Results: 99mTc-ECD brain SPECT abnormalities were significantly more common in patients with pSS (10/10) than controls (2/10; p<0.05). Cognitive dysfunctions, mainly expressed as executive and visuospatial disorders, were also significantly more common in patients with pSS (8/10) than controls (0/10; p<0.01). Notably, between-group comparisons enabled a significant correlation to be established between neuropsychological assessment and 99mTc-ECD brain SPECT abnormalities in patients with pSS (rs = 0.49, p<0.01). MRI abnormalities in patients and controls did not differ significantly.

Conclusions: Neuropsychological testing and 99mTc-ECD brain SPECT seem to be the most sensitive tools to detect subclinical CNS dysfunction in pSS. The strong correlation between cortical hypoperfusion in 99mTc-ECD brain SPECT and cognitive dysfunction suggests an organic aetiology of CNS dysfunction in pSS. These data should be confirmed in a larger study.

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  • Competing interests None.

  • Ethics approval Ethics committee approval from the institution revision board/Comité Consultatif des Personnes Participant à la Recherche Biomédicale.

  • Patient consent Patient consent received.