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Locked in Syndrome (In preparation)


Learning objectives

  • What is locked in syndrome
  • What are the causes
  • Diagnosis of locked in syndrome
  • Management of locked in syndrome

Introduction

  • Damage to the brainstem especially the ventral pons can result in a locked in syndrome
  • Cases vary in their degree and there is a spectrum
  • First descrbed by Plum and posner in 1966

Anatomy

  • Bilateral damage to the pons can result in quadriparesis
  • Facial and Bulbar paralysis
  • Preservation of Midbrain IIIrd/IVth eye movements but loss of lateral gaze
  • Awareness and consciousness
  • Whatever deficits causes by other strokes beyond pons e.g. Blindness, ataxia etc.

Aetiology

  • Pontine stroke Infarct or haemorrhage affecting ventral pons usually due to damage to ventral perforators from Basilary artery
  • Central pontine myelinosis
  • Demyelination
  • Late stage Motor neurone disease
  • Tumour affecting ventral pons
  • Guillain Barre synbdrome
  • Myasthenia
  • Trauma

Clinical

  • May wake up after a period of coma. Horizontal gaze palsy
  • Appears awake - can open eyes to command and move eyes depending on nuclei damaged
  • Usually mute and unable to speak due to bulbar weakness but understands and can produce non verbal language
  • Quadriparesis which depends on extend of damage to corticospinal tracts in ventral pons
  • Vigilance is fluctuating and eye movements may be inconsistent, very small, and easily exhausted
  • Maybe some cognitive deficits but not usually severe

Investigations

  • CT scan may show bleed. MRI is best to show anatomy of any lesion
  • EEG: will show wakefulness

Management

  • Acute stroke management depending on cases e.g. Thrombectomy for basilar artery occlusion
  • Once medically stable, and given appropriate medical care, life expectancy increases to several decades.
  • Long term will need PEG tube for feeding
  • May need respiratory support and will be at high risk for aspiration
  • Chronic LIS patients typically self-report meaningful quality of life and their demand for euthanasia is surprisingly infrequent.
  • Patients suffering from LIS should not be denied the right to die - and to die with dignity
  • They should not be denied the right to live - with dignity and the best possible revalidation, and pain and symptom management

References and further reading


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