Case of primary Sjogren's syndrome preceded by dystonia

BMJ Case Rep. 2018 Jun 4:2018:bcr2017223468. doi: 10.1136/bcr-2017-223468.

Abstract

There are only six cases in literature that describe development of dystonia with Sjogren's syndrome (SS). We describe a case of a 43-year-old woman who presented with symptoms including movement disorder, sensory neurogenic bladder, sensory loss and neuropathic pain, migraine like headaches, musculoskeletal pain, Raynaud's phenomenon and dysautonomia. Symptoms started in 2000, with weakness that progressed to dystonia in 2003. Diagnostic work-up was inconclusive with negative inflammatory serologies, cerebrospinal fluid and MRI for many years. After patient developed sicca syndrome with dry eyes and mouth in 2009, her rheumatoid factor titre was elevated (550 IU/mL), erythrocyte sedimentation rate, anti-Sjogrens syndrome-related antigen A (anti-Ro/SSA) and anti-SSB/La: anti-Sjogrens syndrome-related antigen B (anti-La/SSB) became positive. Lip biopsy confirmed diagnosis of SS. She was diagnosed with primary SS with neurological involvement. Her symptoms responded well to intravenous methylprednisolone. Symptoms stabilised with trials of immune-suppressive therapy. This is a case that demonstrates the delay of diagnosing SS with preceding unique neurological association.

Keywords: Sjogren’s syndrome; movement disorders (other than Parkinsons); musculoskeletal and joint disorders.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antibodies, Antinuclear / immunology
  • Dystonia / diagnosis*
  • Dystonia / etiology
  • Dystonia / immunology
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Migraine Disorders / diagnosis
  • Migraine Disorders / etiology
  • Migraine Disorders / immunology
  • Neuralgia / diagnosis
  • Neuralgia / etiology
  • Neuralgia / immunology
  • Primary Dysautonomias / diagnosis
  • Primary Dysautonomias / etiology
  • Primary Dysautonomias / immunology
  • Raynaud Disease / diagnosis
  • Raynaud Disease / etiology
  • Raynaud Disease / immunology
  • Salivary Glands, Minor / pathology
  • Sensation Disorders / diagnosis
  • Sensation Disorders / etiology
  • Sensation Disorders / immunology
  • Sjogren's Syndrome / complications
  • Sjogren's Syndrome / diagnosis*
  • Sjogren's Syndrome / drug therapy
  • Sjogren's Syndrome / immunology
  • Syncope / diagnosis
  • Syncope / etiology
  • Syncope / immunology
  • Urinary Bladder, Neurogenic / diagnosis
  • Urinary Bladder, Neurogenic / etiology
  • Urinary Bladder, Neurogenic / immunology

Substances

  • Antibodies, Antinuclear
  • Immunosuppressive Agents
  • SS-A antibodies
  • SS-B antibodies