血卟啉病的神经系统表现分析

为了探讨血卟啉病神经系统表现规律,增加对本病的认识,并给临床医生提供一个实用的临床诊断和治疗方法。笔者报道了2例,加上国内近10年报道的13例,本文对此15例进行了全面的分析,对该病的发病机制、分类、临床表现、诊断、治疗及预后进行了系统的讨论。结果发现血卟啉病神经系统表现为周围感觉运动神经病4例、内脏自主神经病9例、抽搐发作4例及精神症状6例。我们报道的例2中有枕叶梗塞所致的皮层盲,国外少见,国内未见报道。血、尿中尿卟啉、粪卟啉及前体的测定是诊断有关键,避免诱因、静脉滴注血红素及葡萄糖是主要的治疗手段。

Neurologic manifestions of Porphyria ang clinical analysis of 15 cases.

In order to study the regularity in the neurologic manifestations of Dorphyria, raise the rate of diagnosis and provide clinicians with a practical doagnostic and management approach. 2 patients were described and 13 cases were reported in recent 10 years in our country. We make a systematic summary ahout this 15 cases and review the oertinent literatures. Its mechanism, classification, clinical manifestations, dignosis and treatment are discussed. The results show that the most common neurologic manifestations of Porphyria are peripheral motor and/or sensory neuropathy, autonomic neuropathy, mental change and seizures. 1 patient with cortical blindness caused by bioccopotal infarctions is reported. It is rare abroas and has not been reported in our country. Measurement of porphyrins or porphyrin precursors in blood and urine are key for the clinical diagnosis. The precondition of diagnosis is enhancing the Inowledge about Porphyia. Avodance of acute attacks and intravenous therapy with heme and glucose are important.