| background Hepatolenticular degeneration(HLD) or Wilson(WD) disease, is one of the few genetic disorders of the nervous system with current clinical can be treated with medication. the main performance of HLD is liver symptoms and movement disorder. Blood determination of copper metabolism and cornea K-F ring are reliable diagnostic method at present. the gold standard for diagnosis is the detection of liver copper.Hepatolenticular degeneration of traditional Chinese medicine thinks is copper toxin evil cohesion and disease,the major clinical according to the Chinese traditional medicine dialectics of eight aspects and viscera theory,comprehensive analysis of data gained by four diagnostic methods,dialectical theory of governance. At present, Chinese scholars of the disease dialectical type views are inconsistent.Due to the diversity of the incidence situation,the complex of clinical symptom,the strange disease to medical staff,are be misdiagnose and missed diagnosis,is delaied treatment.In order to improve the diagnosis rate, reduce disability rate and death rate, except to continue to explore the field of molecular biology of HLD on modern,the detection of cranial magnetic resonance imaging(CMRI) played a supporting role about diagnosis and the survey of symptoms of HLD.To observe the effect of MRI on scan and cerebral damage of HLD while at home and abroad have been reported, but are based on small sample reports and analysis for many, large prospective studies is lack so far.Retrospective analysis the patients MRI to this research is mainly based on 330 cases that arediagnosed by the Anhui University of traditional Chinese Medicine Research Institute affiliated hospital at first without the system drive off copper to the patients of HLD before are treatment, to investigate the MRI features of HLD in the brain of,patient and its correlation with clinical signs, symptoms and so on, so as to provide reference to the clinical diagnosis and treatment.purpose 1 To Analysis the characteristics of HLD to the different clinical types characteristics about the damage in the brain of patients,establish the value of clinical application of MRI on HLD. 2 To study on HLD in patients with cranial magnetic resonance imaging and patient age, disease duration, Kayser Fleischer ring, the clinical symptoms, clinical type, the relationship between liver etc., provide guidance and basis for clinical diagnosis and observation of disease condition and prognosis 3 To study whether there are abnormalities of cranial magnetic resonance imagingin in patients with subclinical to HLD, to provide an objective basis of inspection found brain lesions in the patients with HLD for early.Method In 330 cases of the first hospitalization and confirmed HLD patients underwent CMRI routine scaned and recorded primarily in patients with clinical data(name, age, sex, course of disease, western medicine and traditional Chinese medicine typing, clinical symptoms, K-F ring, liver B ultrasound type etc.).By using the SPSSl7.0 statistical software package. The analysis of Correlation between qualitative variables using chi square contingency table test R*C,correlation between clinical symptoms and MRI brain lesion by using correspondence analysis(correspondance analysis) method. p<0.01 is a very significant difference, p<0.05 had significant difference, p >0.05 had no significant difference.Result 1 In 330 patients, only 15 CMRI patients without abnormal, 315 cases were found abnormal signal that all kinds of parts and the different of degree on cranial magnetic resonance. Among 275 cases of abnormal nucleus(83.3%), brain stem in 189 cases(57.3%), caudate nucleusin 126 cases(38.2%),124 cases of thalamus(37.6%), 112 cases of brain atrophy(33.9%,), the globus pallidus in 45 cases(13.6%), softening ofthe brain in 11 cases(3.3%), 7 cases of the cerebellum(2.1%),other 44 cases were abnormal(13.3%). Mesencephalic brainstem abnormal signal in 145 cases(76.7%), 89 cases(47.1%) the pons, medulla oblongata in 2 cases(1.1%).2 In 315 cases of HLD patients with skull in 291 cases showed abnormal signal of long T1, long T2 which symmetry or approximately symmetrical, 24 cases of mixed high and low signal.3 The lesions in honeycomb, capsule shaped, speckled, cords.4 The Western sub type of each type in patients with brain lesions mainly in the putamen, thalamus, atrophy of the caudate nucleus, brainstem, encephalatrophy,brain stem damage mainly concentrated in the midbrain. Statistical analysis of the Western sub type and the putamen, caudate nucleus, thalamus, cerebellum have significant correlation(p<0.05).5 The Chinese medicine dialectical typing endoretention of damp heat type incidence was the highest, followed by Qi blood deficiency and liver kidney yin deficiency, phlegm fire disturbing heart and stagnation of phlegm type low incidence.Statistical analysis of patients with thalamic and brain HLD softening have obvious correlation between TCM syndrome type(p<0.05).6 age of onset with HLD mainly at the age of 11 to 20 years old, the putamen accounted for 84.4%,40 years after onset of the rare. Statistical analysis of the thalamus, cerebellum have obvious correlation with age(p<0.05).7 There was no correlation between the course of disease and brain damage(p>0.05).8 There are obvious extrapyramidal symptoms of patients, MRI can appear normal. statistical analysis of clinical symptoms have obvious correlation with putamen, Brain atrophy,encephalomalacia(p<0.05). Dystonia and tremor are related to the putamen, thalamus, caudate lesions, dysarthria is related to putamen, thalamus, caudate nucleus, brainstem lesions.9 There is a significant correlation between K-F ring and putamen, globus pallidus, thalamus(p<0.05).10 The types of Liver have no correlation with CMRI(p>0.05).Conclusion 1 patients of HLD with skull lesions are widely distributed, the lesions are mainly concentrated in the basal ganglia, brainstem, thalamus, brain atrophy and encephalomalacia are the common pathological characteristics.2 Characteristics of HLD about head MRI the most significant for the long T1, long T2 signal symmetry in basal ganglia or approximate symmetry, will display the level mixed signal.3 Classification and the putamen, caudate nucleus, thalamus, cerebellum have significant correlation(p<0.05);The thalamus and cerebral softening have obvious correlation betwee type of syndrome to TCM(p<0.05);The thalamus, cerebellum have obvious correlation with age(p<0.05).There was no correlation between the course of disease and brain damage(p>0.05);clinical symptoms and putamen, cerebral atrophy, encephalomalacia have obvious correlation(p<0.05); there is a significant correlation between K-F ring and putamen, globus pallidus, thalamus(p<0.05); The type of liver has no correlation with CMRI(p>0.05). |
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