Evaluation of autonomic disorders in familial amyloidotic polyneuropathy (FAP)

Abstract

Most serious complaint of familial amyloid0tic polyneuropathy (FAP) patients, beside polyneurolmthy, is disorder of the autonomic nervous system such as alternating diarrhea and constipation, orthostatic hypotension, urinary incontinence, dry mouth and dry eyes. To evaluate various autonomic dysfunctions found in FAP patients systematically, we performed various upto-date autonomic function examinations, such as reflex blood flow in the palm by laser Doppler flowmetry (LDF), power spectrum of cardiographic R-R interval, day profile and oral glucose tolerance test, modified gam test, tear secretion test, pupillary function test by iris corder, and detection of abnormal vessels in the conjunctiva. Especially, analysis of LDF and R-R interval revealed that the autonomic nervous system was first affected in asymptomatic carriers of FAP and FAP patients at the very early stage of FAP. Abnormalities of day profile of glucose, oral glucose tolerance test and modified gain test were seen even at the early stages of FAP. However, ophthalmological autonomic examinations revealed that severity of autonomic dysfunction did not correlate with the progressive course of FAP. Since FAP patients with severe autonomic disorders show the undesirable prognosis, precise examinations and therapy for the autonomic dysfunctions may be indispensable for controlling the clinical symptoms of FAP patients.