Correlation between clinical, electromyographic and dysautonomic evolution of familial amyloidotic polyneuropathy of the Portuguese type

Acta Neurol Scand. 1994 Oct;90(4):266-9. doi: 10.1111/j.1600-0404.1994.tb02719.x.


To assess the correlation between the severity of clinical symptoms and sensorymotor and autonomic function in familial amyloidotic polyneuropathy of the Portuguese type (FAP-PT), clinical grade (CG), electromyographic score (ES), sympathetic (SS) and parasympathetic score (PS) were compared in 47 patients. SS and PS were both abnormal in 72% of patients with ES = 0% and in 73% of patients with CG = 0. When SS and PS were compared with either CG and ES, PS progressed more rapidly and stabilized earlier than SS. As a whole, SS and PS were greater as CG and ES were higher. This pattern of autonomic involvement should be taken into account when assessing the course of the disease and evaluating new forms of therapy.

MeSH terms

  • Adult
  • Aged
  • Amyloid Neuropathies / diagnosis
  • Amyloid Neuropathies / genetics*
  • Amyloid Neuropathies / physiopathology
  • Cardiovascular System / innervation
  • Dysautonomia, Familial / diagnosis
  • Dysautonomia, Familial / genetics*
  • Dysautonomia, Familial / physiopathology
  • Electromyography*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Motor Neurons / physiology
  • Muscles / innervation
  • Neurologic Examination*
  • Parasympathetic Nervous System / physiopathology
  • Reflex / physiology
  • Sympathetic Nervous System / physiopathology