| Background and Objective:We have found a group patients since 2000.Their symptoms were similar, mainly including cerebellum symptoms , such as progressive bradykinesia, ataxia, and slurred speech,paralysis and even coma. The results of serologic tests for the human immunodeficiency virus (HIV), routine analyses of serum chemistry, hematologic tests, and cerebrospinal fluid studies were normal. A computed tomographic scan and magnetic resonance imaging (MRI) showed diffuse, symmetric change of the cerebellar and cerebral white matter. A brain biopsy revealed spongiform degeneration of the white matter, with relative sparing of subcortical fibers (U fibers). Because all patients had the history of inhaling heroin vapor, Heroin spongiform leucoencephalopathy was diagnosed. Heroin spongiform leucoencephalopathy was firstly reported by Wolters E [1]. of holand at Amsterstan in 1982. Within the next 20 years, there is no more than 20 cases reported in Japan, German, Belgium, America and Taiwan district of China. Beside Holand, there is no cases in batches be reported by now and no datas from epidemiology available. Due to scarce knowledge about this disease, this disease can not be studied systemically and few of the reported cases include detailed information concerning long-term follow-up. The etiopathogenesis about this disease is unknown. Now we have the biggist cases of HSLE in the world, so it is a good chance for us to study and follow-up this special encephalopathy.-7-We have studied 36 cases of HSLE, patients (33 males and 3 females) aged 23-45 . All the cases had a history of inhalation of heated heroin vapor with the duration ranging from 1 to 10 years. 18 cases administered heroin by intravenous at the same time. 32 cases were attacked after abstinence or the symptoms aggravated after abstinence. A series of detail examinations, for example virology, including human immunodeficiency virus (HIV) etc, tubercle bacilli (TB), treponema pallidum, Toxoplasma, blood chemical and cerebrospinal fluid (CSF) was carried out for each patient. Head CT and MRI examination were carried out in all cases including one case of MRI reinforcement. SPECT of 11 cases and PET of 4 cases were finished. Brain autopsies of 3 cases and brain biopsies of 11 cases were taken. Methods(1) Clinical datas obtained had been imported into computer where Statistics analysis was made by SPSS software.(2) Controlled study of a clinical cases26 cases was randomly divided into the controlled group and the treating group. In the the controlled group, the treatment was consisted of oral administration of vitamin B and coenzyme Q10 , 20mg 3 times daily, and injection of the Citicolin and Radix Salviae Milfiorrhizae,! time daily. In the treating group , glucocortioid 20mgd for 10 days were given besides vitamin B and coenzyme Q10, then amount of the glucocortioid was gradually decreased. The neurological function default scale were recorded before treatment and one month, 6 months ,12 months and 24 months after treatment.The curative effect was evaluated(3) Following-upInvestigators are consisted of experienced neurologists in our hospital.The neurological examination and questionnaire was taken to all of them in6,12,24 and 36 months after the onset of symptoms .Result1. symptoms and signsThe most patients were acute onsets . The first symptoms were cerebellar ataxia including dysarthria and liability of gait and clumsiness of arm movements. The clinical manifestation and progression of the illness had a similar course. So HSLE should be divided into three phases according to the state of awareness, presence of cerebellar signs or damages of pyramidal tract. Phase I: Simple cerebellar signs; Phase II: Cerebellar signs complicated with the damages of pyramidal tract; Phase HI: Disturbance of consciousness (coma, decorticate status, locked-in syndrome and akinetic mutism) complicated with the damages of pyramidal tract.4 cases died . Abstinence Was a familiar inducement.There is no relativity between the severity of t...
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