Background Central nervous system (CNS) involvement in primary Sjogren’s syndrome (pSS) is controversial with regard to incidence, aetiology and pathogenesis. In the literature, the incidence of CNS-SS varies widely, ranging from rare to incidence rates of 20% to 25%. The clinical picture is diverse, ranging from mild cognitive symptoms to fatal cerebrovascular accidents.
Objectives We describe a patient with pSS presenting with dysphagia and progressive respiratory failure resulting from anterior horn cell disorder. The disease responded to corticosteroids, hydroychloroquine and azathioprine treatment. To our knowledge this is the first case reported in the medical literature.
Methods A 58-year-old woman had a one year history of dry eyes and dry mouth. She experienced progressive dysphagia and dyspnea ten days prior to admission. She developed progressive respiratory muscle weakness which resulted in respiratory failure and received mechanical ventilation during admission. Examination revealed symmetrically decreased muscle power without sensory impairment. Schirmer test was positive. Blood investigations revealed positive ANA, anti-SS-A and elevated IgG level up to 2820 mg/dl. Full blood cell count and creatine kinase level were normal. Sjogren’s syndrome was proved by minor salivary gland biopsy. Electromyography, nerve conduction velocity and evoked potentials studies showed anterior horn cell disorder. MRI of brain revealed negative finding. The disease improved with administration of corticosteroids, hydroxychloroquine and azathioprine.
Conclusion Sjogren’s syndrome should be considered in the differential diagnosis of patients with motor neuron diseases. Prompt diagnosis and treatment prevent further morbidity and mortality.
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