A New Kind of and Reversible Brainstem Involvement in Primary Sjögren's Syndrome as an Initial Manifestation

Abstract

The prevalence of abnormalities on brain magnetic resonance imaging (MRI) varies in primary Sjögren's syndrome (pSS) patients and they are generally multiple hyperintense areas in the subcortical and periventricular white matter on T2-weighted and fluid-attenuated inversion recovery sequences. Here, we report brainstem involvement in a patient with pSS that was extensive on MRI, but reversible. The patient's outcome was positive. To our knowledge, a similar case has not previously been described.

The expanding phenotype of CLIPPERS: is it a disease or a syndrome?

“…MRI features of brainstem involvement in primary Sjögren's syndrome occasionally presents punctate gadolinium-enhancing foci peppering the pons, middle cerebellar peduncles, cerebellar hemispheres and vermis, and mesencephalon, which are quite similar to those of CLIPPERS 15. The subclinical involvement of exocrine glands found in some CLIPPERS cases6 suggests a possibility that pontocerebellar involvement of CLIPPERS could be the first manifestation of certain systemic diseases, such as Sjögren's syndrome.…”

Neuroimaging Pearls from the MDS Congress Video Challenge. Part 2: Acquired Disorders

“…FLAIR sequences are the most frequent MRI brain abnormalities seen in Sjogren syndrome. [14][15][16] Punctate contrast-enhancing foci may be present in the pons, mesencephalon, middle cerebellar peduncles, and cerebellum. 17 Hyperintense, non-enhancing, bilateral basal ganglia, thalamic, and brainstem lesions have also been reported.…”

“…optic nerve T1-weighted hyperintensity with the enhancement of the optic chiasm), bilateral temporal lobar and hippocampal T2/FLAIR hyperintensities seen in the setting of limbic encephalitis, tumefactive lesions, and marked cerebellar atrophy. 14,15,[18][19][20][21][22][23][24] Central pontine myelinolysis, hemorrhage, lacunar infarcts, and leptomeningeal enhancement, have also been reported in some case series. 14,15,[18][19][20][21][22][23][24] In Sjogren-related myelopathy, spinal cord involvement typically appears as longitudinally extensive T2 hyperintensities in the posterior columns without T1 gadolinium enhancement (although enhancement has also been described).…”

“…14,15,[18][19][20][21][22][23][24] Central pontine myelinolysis, hemorrhage, lacunar infarcts, and leptomeningeal enhancement, have also been reported in some case series. 14,15,[18][19][20][21][22][23][24] In Sjogren-related myelopathy, spinal cord involvement typically appears as longitudinally extensive T2 hyperintensities in the posterior columns without T1 gadolinium enhancement (although enhancement has also been described). 16,22 Extensive lesions involving the entire spinal cord have also been rarely reported.…”

“…Behçet's disease. 14,20 Tuberculosis and brain metastases are important differential diagnoses for other contrastenhancing posterior fossa lesions. 14 LETM involving multiple spinal segments in Sjogren syndrome may mimic neuromyelitis optica spectrum disorder (NMOSD).…”