| Background and Purpose Heroin Spongiform Leukoencephalopathy(HSLE) is a neurological disorder mainly affected brain white matter,which is related with inhalation of the pyrolysate of heroin.HSLE was firstly discovered in Netherlands in 1982. Since then, individual cases were reported in German, Italy, Belgium, the United States and Taiwan,except for the report of mass occurrence in the Netherlands( 1982) by Wolters and in the Chinese mainland by us(2000).The etiology and pathogenesis of HSLE are unknown.Clinical features of HSLE were different from acute or chronic heroin toxication absolutely. The main symptom of HSLE is cerebellar ataxia.The main pathological feature of lISLE was extensive vacuolation in white matter,which was like that of transmissible spongiform encephalopathy(T SE). The golden diagnostic standard of TSE is positive PrP immunohistochemistry reaction iii brain tissue.Recently, 14-3-3 protein in CSF was found to have high sensibility and specificity in CJD patients,and was regarded as a reliable premortem diagnostic standard of CJD.To make clear the relationship between HSLE and TSE,laboratory examination of blood and CSF, neurological imaging,brain tissue pathological and immunohistochemistry research were carried out in all the 29 cases of HSLE we collected and inoculation of homogenate of dead patientsrain to ratsrain to observed the transmisibility of HSLE were conducted. Materials and Methods The sum of cases of HSLE is 29.EEG,CT,MRI were carried out for each patient.Brain biopsy was conducted in 11 cases ,and necropsy was conducted in 3 dead cases.HE.,Congo red,myelin stains and GEAP,PrP immunohistochemistry stains were carried out.Blood and CSF of patients were collected for syphilis,toxoplasma gondii and routine,cytological, glycose,protein,chloride, virology examination and 14-3-3 protein Western Blot of CSF were were carried out too.Homogenate of 2 dead patients抌rain was inoculated in Wistar ratsrain,and the observation period was 6 months. Results I-LIVHSV,MCV,EB,toxoplasma gondii,syphilis examination were all negative.Routine and biochemistry examination of CSF were normal without infection.Immunoassay detection of 14-3-3 protein in CSF were negative. CT showed Symmetric low-density lesions with distinct boundaries in white matters,without effect of edema or mass.MRI showed extensive abnormal signals in white matters in lesion location with long TI and long T2.FLAJR images showed higher signals in white matters of lesion locations and their ranges mainly according with T2 images. The pathology character was spongiform vacuolar degeneration of white matter and neurons in grey matter did not decreased.There were no vacuole,edema and lymphoid infiltration in grey matter and small vessel periphery.There were no apparent reactive astroglial proliferation. o ligodendroglia relatively decreased. Positive reactivities of prion protein by immunohistochemistry were seen in neurons.Foci od ischemic necrosis were not found.Electron microscopy revealed a multivacuolar degeneration of the oligodendroglias. Swollen mitochondria and distended endoplasmic reticulum were found in the remaining cytoplasm.Ln many myelin sheaths vacuoles formed between the myelin lamellae by splitting of the intraperiod lines were observed.In some places continuity of the myelin sheath was interrupted by a ball of whorled myelin layer. The inoculated rats did not onset in 6 months,and the... |
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