Department of Human Pathology, Gunma University Graduate School of Medicine, Maebashi, Japan.
Department of Global Infectious Diseases, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Japan.
Neuropathology. 2022 Jun;42(3):190-196. doi: 10.1111/neup.12798. Epub 2022 Feb 14.
An 82-year-old man, who was healthy and had worked as a farmer, experienced worsening neurological symptoms over a seven-month period, which eventually caused his death. Multiple fluctuating brain lesions were detected radiographically. Clinically, sarcoidosis was ranked high among the differential diagnoses because of the presence of skin lesions showing granulomatous inflammation, confirmed by biopsy. The patient's cerebrospinal fluid was also examined, but no definitive diagnosis was made while he was alive. An autopsy revealed multiple granulomatous amebic encephalitis lesions in the brain. Genetic and immunohistochemical analyses identified Balamuthia (B.) mandrillaris, a free-living ameba, which resides in soil and fresh water, as the causative organism. A retrospective examination revealed B. mandrillaris in the biopsied skin as well as cerebrospinal fluid, strongly suggesting that the ameba had spread into the brain percutaneously. Few studies have detailed the cutaneous pathology of B. mandrillaris infections. In general, granulomatous amebic encephalitis is extremely difficult to diagnose without autopsy, but the present case provides a clue that could allow similar cases to be diagnosed earlier; that is, the presence of skin lesions.
一位 82 岁的男性,身体健康,曾是一名农民,在七个月的时间里出现了逐渐恶化的神经症状,最终导致其死亡。影像学检查发现多处多发波动性脑病变。由于存在皮肤病变,且活检证实存在肉芽肿性炎症,临床上将结节病列为鉴别诊断的重点之一。还对患者的脑脊液进行了检查,但在其生前并未做出明确诊断。尸检显示大脑中有多处肉状阿米巴脑炎病变。基因和免疫组化分析确定了自由生活的阿米巴原虫——曼氏巴贝西虫(Balamuthia mandrillaris)为致病病原体,该原虫存在于土壤和淡水中。回顾性检查发现在活检的皮肤和脑脊液中也存在曼氏巴贝西虫,强烈提示该阿米巴原虫经皮穿透进入大脑。很少有研究详细描述曼氏巴贝西虫感染的皮肤病理学。一般来说,如果没有尸检,很难诊断出肉芽肿性阿米巴脑炎,但本病例提供了一个线索,可能使类似病例更早得到诊断;即存在皮肤病变。