[Familial amyloidotic polyneuropathy --Portuguese type-- clinical and neurophysiologic course].
Sixteen (16) patients with Familial Amyloidotic Polyneuropathy-Portuguese Type (FAP) were evaluated longitudinally, using the EMG and Clinical protocols with the aim of establishing the clinical and neurophysiological evolution rate of FAP. We conclude that spontaneous evolution of FAP patients shows a linear progression. Clinical and neurophysiological evolution times are parallel, with the EMG scores being higher than Clinical ones. We propose that Clinical and EMG scores should be used in the evaluation of future therapeutic trials, namely immunodepuration and liver transplantation.
Serviço de Neurologia, Centro de Estudos Egas Moniz, Hospital de Santa Maria, Lisboa.
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D000328:Adult; D000686:Amyloidosis / Q000235:genetics / Q000503:physiopathology*; D004576:Electromyography; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000235:genetics / Q000503:physiopathology*; D011174:Portugal