Anaesthetic management of a patient with familial amyloid polyneuropathy of the Portuguese type.

Although familial amyloid polyneuropathy of the Portuguese type (FAP-PT) was first described in 1952, there is little in the medical literature detailing the anaesthetic management of such patients. FAP-PT is a disease with multiple clinical manifestations which include disturbances of sensibility, progressive paresis starting in the lower extremities, autonomic dysfunction, cardiac conduction disturbances, gastro-intestinal disorders, nephrotic syndrome, sexual and sphincter disorders, extreme emotionalism and apprehension. Several intermingling problems have to be considered in the anaesthetic management of each individual case. In our patient a sinus dysrhythmia resolved after isoflurane and this seems to be a good choice for general anaesthesia in patients with FAP-PT, if they are in an early stage of heart involvement.