J. Duclasoares et al., CORRELATION BETWEEN CLINICAL, ELECTROMYOGRAPHIC AND DYSAUTONOMIC EVOLUTION OF FAMILIAL AMYLOIDOTIC POLYNEUROPATHY OF THE PORTUGUESE TYPE, Acta neurologica Scandinavica, 90(4), 1994, pp. 266-269
To assess the correlation between the severity of clinical symptoms an
d sensorymotor and autonomic function in familial amyloidotic polyneur
opathy of the Portuguese type (FAP-PT), clinical grade (CG), electromy
ographic score(ES), sympathetic (SS) and parasympathetic score (PS) we
re compared in 47 patients. SS and PS were both abnormal in 72% of pat
ients 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 st
abilized 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.