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Baroreflex failure secondary to paraneoplastic encephalomyelitis in a 17 year old woman with neuroblastoma
  1. D L Jardine1,
  2. C P T Krediet2,
  3. B A Robinson3
  1. 1Department of General Medicine, Christchurch Hospital, Christchurch, New Zealand
  2. 2Academic Medical Center, Amsterdam, The Netherlands
  3. 3Department of Oncology, Christchurch Hospital, Christchurch, New Zealand
  1. Correspondence to:
 D L Jardine
 General Medicine Department, Private Bag 4710, Christchurch Hospital, Christchurch, New Zealand; david.jardinecdhb.govt.nz

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Baroreflex failure is a rare cause of postural hypotension but should be considered in any patient with a diffuse central nervous system disease involving the brainstem. Paraneoplastic encephalomyelitis (PEM) is such a disease and, although rare, is becoming more frequently diagnosed because of improved imaging and specific antibody testing. We present the first case of baroreflex failure secondary to PEM. In January 2002, a 17 year old woman presented with a 3 week history of pain in the right shoulder which spread down the forearm to the radial border of her hand. She had also become anorexic and lost 18 kg over 3 months. A mild resting tachycardia was noted but there were no objective neurological signs, and routine blood tests, including inflammatory markers, were normal. In March, both pupils became dilated, non-reactive to light or near stimuli, and myotonic (that is, Holmes-Adie pupils). Limb reflexes were decreased and nerve conduction studies demonstrated a mild motor-sensory polyneuropathy. The aetiology of her anorexia was thought to be psychological and over the next 3 months she was treated with sedatives, antidepressants, and motility agents. She regained 12 kg but her neurological abnormalities persisted, and she developed psychomotor retardation and symptomatic postural hypotension. In July she underwent tilt testing and autonomic studies.

The patient was positioned horizontally on the tilt table and continuous blood pressure (BP) was monitored using digital plethysmography, stroke volume was derived from the arterial pulse …

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