Case of primary Sjogren’s syndrome preceded by dystonia

Abstract

There are only six cases in literature that describe development of dystonia with Sjogren's syndrome (SS). We describe a case of a 43-year-old woman who presented with symptoms including movement disorder, sensory neurogenic bladder, sensory loss and neuropathic pain, migraine like headaches, musculoskeletal pain, Raynaud's phenomenon and dysautonomia. Symptoms started in 2000, with weakness that progressed to dystonia in 2003. Diagnostic work-up was inconclusive with negative inflammatory serologies, cerebrospinal fluid and MRI for many years. After patient developed sicca syndrome with dry eyes and mouth in 2009, her rheumatoid factor titre was elevated (550 IU/mL), erythrocyte sedimentation rate, anti-Sjogrens syndrome-related antigen A (anti-Ro/SSA) and anti-SSB/La: anti-Sjogrens syndrome-related antigen B (anti-La/SSB) became positive. Lip biopsy confirmed diagnosis of SS. She was diagnosed with primary SS with neurological involvement. Her symptoms responded well to intravenous methylprednisolone. Symptoms stabilised with trials of immune-suppressive therapy. This is a case that demonstrates the delay of diagnosing SS with preceding unique neurological association.

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

“…Oher MRI brain findings described include cranial nerve involvement (e.g. 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 14,15,18–24 . Central pontine myelinolysis, hemorrhage, lacunar infarcts, and leptomeningeal enhancement, have also been reported in some case series 14,15,18–24 …”

“…17 Hyperintense, non-enhancing, bilateral basal ganglia, thalamic, and brainstem lesions have also been reported. 18,19 Oher MRI brain findings described include cranial nerve involvement (e.g. 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.…”

“…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.…”

“…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 18,19 …”