Clinical features

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.