Unusual presentation of Sjögren's syndrome

• Published in: BMJ case reports Vol. 2014; p. bcr2014206489
• Main Authors: Bouraoui, Aicha, Bari, Syed Farhan, Nash, Julian, Dawson, Kenneth
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 20-11-2014; BMJ Publishing Group
• Series: Case Report
• Subjects: Aged; Antibodies; Antibodies, Antinuclear - analysis; Ataxia; Connective tissue diseases; Diagnosis, Differential; Evoked Potentials, Somatosensory; Female; Follow-Up Studies; Glucocorticoids - administration & dosage; HIV; Human immunodeficiency virus; Humans; Injections, Intravenous; Magnetic Resonance Imaging; Methylprednisolone - administration & dosage; Patients; Peripheral Nervous System Diseases - diagnosis; Peripheral Nervous System Diseases - drug therapy; Peripheral Nervous System Diseases - etiology; Rheumatology; Sjogren's Syndrome - complications; Sjogren's Syndrome - diagnosis; Sjogren's Syndrome - drug therapy; Tomography, X-Ray Computed; United Kingdom; Unusual Presentation of More Common Disease/Injury; White
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2014-206489

We present a case of a 67-year-old woman admitted from the neurology clinic for further investigations of progressive ataxia and sensory symptoms. Neurological examination showed reduced pinprick and absent vibration sensations in the lower limbs. Motor system examination was normal. Her antinuclear antibodies titre was 1:100 with positive Ro antibodies. Her initial nerve conduction studies were normal. However, the lower limb somatosensory-evoked potentials (SSEP) demonstrated impairment of central sensory conduction pathway. Rheumatology review revealed a history of fatigue and Sicca symptoms and her Schirmer's test was strongly positive. This lead to the diagnosis of ganglionopathy associated to Sjögren's syndrome. She had an excellent response to intravenous methylprednisolone followed by oral prednisolone and intravenous cyclophosphamide infusions. This case highlights that dorsal column involvement can precede the diagnosis of primary Sjögren’s syndrome.