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Clinical features

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

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.

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