Kriegstein A R, Shungu D C, Millar W S, Armitage B A, Brust J C, Chillrud S, Goldman J, Lynch T
Department of Neurology, Columbia University, College of Physicians and Surgeons, New York, NY, USA.
Neurology. 1999 Nov 10;53(8):1765-73. doi: 10.1212/wnl.53.8.1765.
Inhalation of heated heroin vapor ("chasing the dragon"), which is gaining popularity among drug users seeking to avoid the risks of parenteral drug administration, can produce progressive spongiform leukoencephalopathy.
We studied the clinical phenotype and course, MRI, MRS, and brain pathology in the first American patients described with this syndrome.
Two of the three heroin users studied inhaled heroin pyrolysate together daily over the course of 2 weeks. They developed ataxia, dysmetria, and dysarthria. Patient 1 progressed to an akinetic mute state with decorticate posture and subsequent spastic quadriparesis. Patient 2 developed a mild spastic quadriparesis and gait freezing. Patient 3 was asymptomatic following less heroin exposure. Brain MRI showed diffuse, symmetrical white matter hyperintensities in the cerebellum, posterior cerebrum, posterior limbs of the internal capsule, splenium of the corpus callosum, medial lemniscus, and lateral brainstem. MRS showed elevated lactate. Brain biopsy (Patient 1) showed white matter spongiform degeneration with relative sparing of U-fibers; electron microscopy revealed intramyelinic vacuolation with splitting of intraperiod lines. Progressive deterioration occurred in Patients 1 and 2 over 4 weeks. Both were treated with antioxidants including oral coenzyme Q, and clinical improvement occurred. Patient 1 recovered nearly completely over 24 months. Patient 2 improved, but developed a delayed-onset cerebellar hand tremor. Both still have white matter abnormalities on MRI and MRS.
Elevated lactate in white matter and the possible response to antioxidants suggests mitochondrial dysfunction in progressive spongiform leukoencephalopathy following inhalation of heated heroin vapor.
吸入加热后的海洛因蒸汽(“追龙”)在试图避免注射吸毒风险的吸毒者中越来越流行,可导致进行性海绵状白质脑病。
我们研究了首例被描述为此综合征的美国患者的临床表型、病程、磁共振成像(MRI)、磁共振波谱(MRS)及脑病理学。
三名海洛因使用者中的两名在两周时间里每天共同吸入海洛因热解产物。他们出现共济失调、辨距不良和构音障碍。患者1进展为运动不能性缄默状态,伴有去皮层姿势,随后发展为痉挛性四肢瘫。患者2出现轻度痉挛性四肢瘫和步态冻结。患者3因接触海洛因较少而无症状。脑部MRI显示小脑、大脑后部、内囊后肢、胼胝体压部、内侧丘系和脑桥外侧弥漫性、对称性白质高信号。MRS显示乳酸升高。脑活检(患者1)显示白质海绵状变性,U形纤维相对保留;电子显微镜显示髓鞘内空泡形成,伴周期线分裂。患者1和患者2在4周内病情逐渐恶化。两人均接受了包括口服辅酶Q在内的抗氧化剂治疗,临床症状有所改善。患者1在24个月内几乎完全康复。患者2有所改善,但出现了迟发性小脑性手部震颤。两人的MRI和MRS检查仍显示有白质异常。
白质中乳酸升高以及对抗氧化剂可能的反应提示吸入加热海洛因蒸汽后发生的进行性海绵状白质脑病存在线粒体功能障碍。