Primary Sjogren's syndrome presenting as acute psychosis with catatonic symptoms.

BACKGROUND: Sjogren's syndrome is the second most common autoimmune rheumatic disease after rheumatoid arthritis. Central nervous system (CNS) involvement in primary Sjogren's syndrome (CNS-SS) is a rare but significant complication and can manifest with focal or diffuse neurological deficits including psychiatric disorders. CASE REPORT: We discuss the case of a 19-year-old lady, who presented with twenty days history of hallucinatory behaviour, persecutory and referential delusions, inadequate self-care, decreased oral intake, and gradual onset mutism. Intermittent low-grade fever was present at onset. Examination revealed catatonic signs of mutism, negativism, immobility, and Gegenhalten. Preliminary investigations including CT brain did not indicate an organic aetiology. A diagnosis of acute psychosis with catatonic symptoms was made and she was initiated on Quetiapine and modified ECT. Though there was initial response, symptoms worsened and Quetiapine was declared failed. She was initiated on Olanzapine on which she developed significant extrapyramidal symptoms. Five weeks into her hospital stay, she developed bilateral upper and lower limb proximal muscle weakness. A detailed neurology evaluation was sought. Clinical findings and lab investigations were confirmatory for Sjogren's syndrome. She was diagnosed to have Primary Sjogren's syndrome (PSS) with CNS involvement and initiated on immunomodulators. Her symptoms improved gradually on regular follow up. CONCLUSION: Clinicians should remember that pure psychiatric presentations of organic conditions are relatively rare. These should be looked for when there are atypical symptoms or when treatment response is unusual - treatment resistance or major side effects at relatively low doses. PSS should be in the list of differentials.