Sunday, 1 September 2019

Sjogren's syndrome MRI Brain


Clinically 40 yo female, a known case of Sjogren's syndrome without any neurological or psychiatric complaints.
Stable well oriented with no signs on neurological examination.

MRI brain shows confluent T2 hyperintensity in bilateral cerebral white matter, corona radiata, external capsules, bilateral dentate nuclei.
No associated cerebral cortical atrophy.
Imaging finding can be labelled as severe changes of small vessel disease, significant for age.

Sjögren syndrome and MRI brain

Sjögren syndrome is a chronic systemic autoimmune disease affecting 2–3% of adults.
Classified as primary when occurring in isolation and as secondary when associated with another autoimmune disease.
Characterized by mononuclear infiltration and destruction of the exocrine glands, mainly the lachrymal and salivary glands, resulting in xerophthalmia and xerostomia.
Apart from the glandular features, mononuclear infiltration of visceral organs and vasculitis can give rise to extraglandular manifestations like arthralgia, pulmonary involvement, renal tubular acidosis.
Neurologic involvement in primary Sjögren syndrome particularly peripheral nervous system involvement is a well-documented feature of the syndrome in about 20–25% that commonly manifests as peripheral sensory neuropathy or mononeuritis multiplex caused by small-vessel vasculitis.

MRI brain of patients with primary Sjögren syndrome has shown multiple areas of increased signal intensity in the periventricular and subcortical white matter on FLAIR and T2-weighted images.
Important to note is this have been observed in both patients with and without CNS impairment or symptoms as in our case. Brain atrophy has been reported in a limited number of studies of patients with primary Sjögren syndrome.

Reference : https://www.ajronline.org/doi/10.2214/AJR.10.5984. 

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