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Rehabilitation Practice and Science

Translated Title

陣發性夜間血紅素尿症併發動脈性腦梗塞：病例報告

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder caused by a somatic mutation of the hematopoietic stem cells, which results in a loss of the complement regulatory proteins usually present on the membrane of blood cells. The outcome is an increased tendency for complement to damage the blood cells and, after a series of reactions, hemolytic and thrombotic episodes may occur. On reviewarticleing past reports, PNH-related thrombosis usually occurs in the venous system and only rarely happens in the arterial system.In our case, a male patient was found to have suffered from dark urine and anemia after each of a series of febrile episode and these had occurred since senior high school. The patient had no past history of hypertension, diabetes mellitus, hyperlipidemia and/or smoking; in addition, a family history of stroke was also denied. PNH was diagnosed and long term medication control was applied; however, compliance with the medication was not good due to side effects. Unfortunately, at the age of 52, an arterial cerebral infarction occurred and there was thrombosis of the right middle cerebral artery over a length of about 10 cm. Based on this, primary intra-arterial thrombolysis was not indicated. An emergency decompression operation was carried out to decrease the intracranial pressure. After he was in a stable condition, a rehabilitation program was initiated and there has been partial improvement in the patient's situation. The rehabilitation program for this patient was similar to those of other stroke patients, but because an infectious event in a PNH patient may induce a hemolytic episode, it was very important to prevent the occurrence of any infection, including urinary tract infection and aspiration pneumonia. Notwithstanding this, the patient actually suffered from dark urine and anemia after an episode of urinary tract infection during the rehabilitation period. Using this case, we reviewarticle and discuss the pathophysiology of PNH and what we consider to be the appropriate treatment regimen and rehabilitation for stroke patients suffering from PNH.

Language

Traditional Chinese

First Page

127

Last Page

133

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