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Neurological Rarities
A Faint in the Emergency Department (Due to Primary Systemic Amyloidosis Neuropathy)
  1. Michele T. M. Hu*,
  2. Carolyn M. Gabriel*,
  3. Helen J. Lachmann†,
  4. Rosalind King‡,
  5. Philip N. Hawkins†,
  6. Lionel Ginsberg‡
  1. *National Hospital for Neurology and Neurosurgery, Queen Square,
  2. †National Amyloidosis Centre, Royal Free and University College Medical School, Rowland Hill Street, and
  3. ‡Department of Neurology, Royal Free Hospital, Pond Street, London, UK; Email: micheleh{at}foresthill32.fsnet.co.uk

Abstract

THE STORY

A 46-year old man was admitted as an emergency after a collapse suggestive of syncope. In fact he had been unwell for a year with malaise and fatigue, significant weight loss, progressive breathlessness and night sweats. Indeed, he had seen his family doctor 9 months previously and was found to be polycythaemic, and had had a total of seven units of blood venesected without clinical improvement. Subsequent detailed questioning elicited a history of postural dizziness and presyncope – particularly after venesection, urinary frequency, impotence, gustatory sweating, and more recently, ascending numbness and dysaesthesia of the hands and feet, poor grip strength and bilateral foot drop.

THE EXAMINATION AND INVESTIGATIONS

On examination he looked thin and unwell. An ejection systolic cardiac murmur was noted and there was a marked postural drop in systolic blood pressure (by 40–70 mmHg at various times). There was distal weakness, generalized areflexia and glove

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