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- Primary Sjögren syndrome
- neuromyelitis optica
- longitudinally extensive transverse myelitis (LETM)
Sjögren syndrome (SS) is a chronic autoimmune disorder, commonly seen in woman in their 4th and 5th decade of life. It is characterised by dry mouth (xerostomia), dry eyes (xerophthalmia) and lymphocytic infiltration of the exocrine glands (Sicca complex). This clinical diagnose is supported by serological (positive anti-La and anti-Ro antibodies) and histological tests. Neurological manifestations occur in 20% to 25% of diagnosed cases of SS,1 but it is unusual for neurological manifestations to be the initial presenting feature of primary SS.2
A 23-year-old woman presents with 3-week history of being nauseous, intractable hiccough with tingling sensation in both lower limbs with bowel and bladder incontinence.
Neurological examination showed bitemporal hemianopsia (confirmed by perimetry) and bilateral lower-limb allodynia with no sensory level.
↵i Longitudinally extensive transverse myelitis, defined by a lesion extending three vertebral segments or longer on T2-weighted MRIs of the spine during an acute episode of myelitis.
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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