Objective:

N/A

Background:

Sjogren’s syndrome (SS) is a known systemic disease that can have neurologic manifestations including central nervous system lesions and sensory neuronopathy or ganglionopathy. Symptoms include paresthesias, ataxia, difficulty with fine motor movements, reduced reflexes, and proprioceptive loss. Muscular strength is usually preserved, and EMG shows widespread reduction of sensory potential amplitudes. We present a rare case of SS with absent sicca symptoms, and notable EMG and MRI findings.

Design/Methods:

N/A

Results:

A 72-year-old woman presented with two weeks of lower extremity weakness. Exam revealed distal weakness, areflexia, and impaired proprioception. Autoimmune serum and cerebrospinal fluid testing and malignancy screenings were all unrevealing. MRI was notable for multiple foci of periventricular T2 hyperintensity, along with non-enhancing lesions at the T3 and T6 levels. EMG was consistent with primarily axonal polyneuropathy. Given rapid progression of her symptoms, she received 5 days of IVIg for possible GBS with improvement. A few months later, she presented with a “sandpaper” sensation in her arms. Exam showed sensory changes from bilateral fingertips to elbows. NCS showed axonal polyneuropathy, primarily affecting sensory nerves consistent with sensory neuronopathy or ganglionopathy. Serum SSA antibody was positive. Patient underwent a salivary gland biopsy which met histologic criteria for SS.  

Conclusions:

It is important not to anchor on a diagnosis (in this case, GBS). Sjogren’s neuronopathy/ganglionopathy should be considered even in the absence of sicca symptoms. In 25-60% of cases of primary SS (pSS), neurological symptoms precede the diagnosis by an average of two years and can manifest as peripheral neuropathy or central nervous system lesions.