clinical features

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All the clinical features of AIP relate to the autonomic nervous system. There are acute attacks characterised by:

  • autonomic neuropathy:
    • abdominal pain: in 80-90% of cases; diffuse or localised; often intermittent and spastic (1)
    • constipation
    • vomiting
    • hypertension
    • postural hypotension
    • peripheral neuropathy: localised pain to a complete generalised flaccid paralysis

  • respiratory failure - bulbar paresis

  • cerebellar signs may develop

  • hypothalamic dysfunction - inappropriate ADH secretion

  • hyponatremia - from inappropriate ADH, or GI or renal sodium loss

  • patients with AIP often have psychiatric disturbances, particularly with features of delerium - confusion, agitation, disorientation and hallucinations

  • severity and frequency of attacks varies widely between individuals

Reference:

  • Stanbury JB et al (1983). The Metabolic Basis of Inherited Disease, 5th edn. McGraw-Hill.

Last reviewed 01/2018

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