clinical features
Last reviewed 01/2018
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.
motor neuropathy in acute intermittent porphyria