CASE REPORT 
Annals of Nuclear Medicine Vol, 11, No. 2, 139-141, 1997 
Scan findings of various myocardial SPECT agents in a case of amyloid polyneuropathy with suspected myocardial involvement 
Ali Syed ARBAB, Kiyoshi KOIZUMI, Keiji TOYAMA, Takao ARAI, Tatsuya YOSHITOMI and Tsutomu ARAKI 
Deparlment of Rediology, Yamanashi Medical University 
A 31-year-old male having familial amyloid polyneuropathy underwent a Tc-99m(V)-DMSA study to evaluate the myocardial involvement. The patient also underwent Tl-201, I-123-BMIPP and I-123-MIBG myocardial SPECT studies to evaluate blood perfusion, fatty acid metabolism and sympathetic function of the heart, respectively. Tc-99m(V)-DMSA SPECT showed uptake to the myocardium indicating myocardial involvement of amyloidosis. Both Tl-201 and I-123-BMIPP studies showed normal uptake indicating normal blood perfusion and fatty acid metabolism but I-123-MIBG SPECT showed no uptake to the heart, indicating severe impairment of sympathetic function. 
Key words: amyloidosis, Tc-99m(V)-DMSA, Tl-201 , I-123-BMIPP, I-123-MIBG 
INTRODUCTION 
AMYLOIDOSIS is a group of diseases that are characterized by the extracellular deposition of the fibrous protein amyloid in one or more sites in the body. The disease may involve virtually any organ of the body. The specific diagnosis of amyloidosis depends on the demonstration of amyloid with appropriate staining in a biopsy specimen.1 Recently Tc-99m(V)-dimercaptosuccinic acid [Tc-99m(V)-DMSA] has shown potentiality in the detection of amyloidosis in various organs.2.3 Manni et al. have reported a case of cardiac amyloidosis detected by Tc-99m(V)-DMSA.4 In this case report, scan findings of various cardiac SPECT agents are described in a patient with amyloid polyneuropathy with suspected involve-ment of the myocardium which was diagnosed by Tc-99m(V)-DMSA scan. 
CASE PRESENTATION 
A 31-year-old man was admitted with numbness, disability in his lower extremities and alternating constipation and diarrhea. He had no past history of cerebrovascular or cardiac accident, drug abuse, or endocrine, metabolic or other systemic disorders. On examination there were impairment of sensory and motor functions in lower extremities, loss of tendon reflexes, hyperplasia at the sole of the foot including part of the lower extremities, and atrophy of the pretibial muscles. Laboratory tests showed no remarkable changes expect an increased level of creatinine phosphokinase (CPK) and alpha2 fraction of albumin. Biopsy from the rectum, peripheral nerves and skin showed amyloid protein deposits, confirming a case of amyloidosis. His mother died at the age of 34 due to sudden cardiac arrest following development of arrhythmia and gradual loss of eyesight which started at the age of 30. With this family history he was diagnosed finally as a case of familial amyloid polyneuropathy. On admission he had normal sinus rhythm with a rate of 88/min and a blood pressure of 124/82. Echocardiography showed marked hypertrophy of the myocardium. A radionuclide gated study showed borderline normal ejection fraction. ECG showed incomplete right bundle branch block with flat ST