Sphincter abnormality in polytransfused patient due to paroxysmal nocturnal hemoglobinuria (PNH): initial manifestation of tropical spastic paraparesis/HTLV-1 associated myelopathy (TSP/HAM)

Laboratory of Experimental Neurology and Neurophysiology/DFF/UFC and Institute of Biomedicine of the Brazilian Semiarid/ INCT/CNPq: MD, MSc; MD, PhD, Full Professor of Neurology and Neurophysiology/DFF/UFC; MD, Medical Doctor of the Brasilia Base Hospital; MSc, PhD, Associate Professor of the Medicine Course of the Christus Faculty; DVM; DVM, PhD, Professor of the Superior School of Agriculture of Mossoro; DVM, PhD, Associate Professor of Histology/DM/UFC. Paroxysmal nocturnal hemoglobinuria (PNH) is a clinical entity resulting from specific change to pluripotent hematopoietic cells and subsequent clonal proliferation, with the capacity to affect the entire line. The erythrocytic membrane has greater sensitivity to lithic action of the complement, for CD59 deficiency. Allogenic bone marrow transplantation is the only support treatment available. According to the clinical manifestations of the disease, folic acid and iron, corticosteroids in low doses, androgens, immunosuppressants and blood component transfusion may be administered. In 1993, HTLV investigation in blood donor banks was officially regulated in Brazil. It is believed that there are around 750,000 HTLV carriers in the country. However, only 5% of them will become symptomatic after years, or even decades. The immunobiological characteristics of the disease that are responsible for this remain unclear. Individuals who develop symptoms will present insidious and gradually increasing muscle weakness in the lower limbs with spasticity, and this is associated with bladder and sensory dysfunction to varying degrees. The case presented here draws physicians’ attention to patients with similar antecedents and unclassified neurological disease, in order to search for a retrovirus as the causal agent. We describe the association between PNH and tropical spastic paraparesis/HTLV-1 associated myelopathy (TSP/HAM), with regard to its diagnosis, management and rehabilitation.