Susac Syndrome

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Learning objectives

  • What is Susac syndrome
  • Recognition and treatment

Introduction

  • Unusual an acute autoimmune neurological syndrome
  • Encephalopathy, seizures, migrainous headache
  • Microangiopathy of brain, retina, cochlea affecting mainly young women
  • small infarctions of cochlear, retinal and encephalic tissue
  • Clinical course is usually self-limited, fluctuating, and monophasic.
  • Lasts 2–4 years but can be longer or shorter
  • Bilateral sensorineural hearing loss which is asymmetric and is thought to reflect sequelae of microinfarction in the apical cochlea


Aetiology

  • Anti-endothelial cell antibodies

Clinical

  • Headache, Memory loss, personality change, delirium, confusion
  • Ataxia, Acute hearing loss
  • Acute visual loss - branch retinal artery occlusions
  • may worsen during pregnancy

Investigations

  • MRI changes involving central corpus callosum is classic. The lesions are typically small, multifocal, and frequently enhance during the acute stage (70%). Leptomeningeal enhancement was present in 33% and deep grey matter involvement (basal ganglia and thalamus) in 70%. Another article describes changes as Central callosal ‘icicle’ and ‘spoke’ lesions in contact with the roof of the callosum and a characteristic ‘string of pearls’ appearance of microinfarcts in the internal capsule have also been recognised
  • CSF protein can be elevated
  • Fluorescein angiography as BRAOs may be diagnostic

Differentials


Management

  • Non proven but consider - Steroids, Azathioprine, IVIG, Plasmapharesis
  • Intratympanic injection of dexamethasone in the acute phase of hearing loss and tinnitus may provide transient benefit
  • Most make a recovery

References and further reading