Study on regional cerebral blood flow and correlated factors in patients with hepatolenticular degeneration / 中国医师进修杂志

ABSTRACT

Objective To study the regional cerebral blood flow (rCBF) and correlated factors in patients with hepatolenticular degeneration (HLD).Methods The rCBF of lentiform nucleus,thalamus and other sites in 14 patients with HLD of cerebral type (cerebral type group) and 10 patients with HLD of non-cerebral type (non-cerebral type group) were determined by magnetic resonance-perfusion imaging technology,meanwhile 13 healthy volunteers were selected as control group,and calculated the relative regional cerebral blood flow (rrCBF) for avoiding perfusion time lag.The correlation between the clinical symptom scores,the content of urinary copper,duration and rrCBF in HLD patients were evaluated.Results The rrCBF of cerebral type group in the left and right frontal lobe,temporal lobe,lentiform nucleus,caudate nucleus,thalamus,midbrain,pons and the left hippocampus,cerebellar cortex,dentate nucleus were lower than those of control group (1.91 ±0.35 vs.2.44 ±0.64,1.80 ±0.30 vs.2.37 ±0.65,1.37 ±0.35 vs.2.14 ±0.91,1.58 ±0.52 vs.2.39 ±0.99,1.61 ±0.38 vs.2.59 ±0.74,1.52 ±0.64 vs.2.63 ±0.73,1.88 ±0.32 vs.2.61 ±0.67,1.70 ±0.40 vs.2.35 ±0.50,1.48 ±0.13 vs.2.01 ±0.59,1.46 ±0.38 vs.2.38 ±0.99,1.47 ±0.55 vs.2.02 ±0.72,1.27 ±0.48 vs.1.91 ±0.51,1.24 ±0.38 vs.1.47 ±0.29,1.25 ±0.39 vs.1.53 ±0.37,1.74 ±0.27 vs.2.40 ±0.89,1.79 ±0.50 vs.2.22 ±0.66,2.15 ±0.41 vs.2.64 ± 0.61),and there were significant differences (P ＜ 0.05 or ＜ 0.01).There were no significant differences in the rrCBF of the parietal and occipital lobe,etc between cerebral type group and control group (P ＞ 0.05).The rrCBF of cerebral type group in the left and right lentiform nucleus were lower than those of non-cerebral type group (1.61 ± 0.38 vs.1.94 ± 0.58,1.52 ± 0.64 vs.1.99 ± 0.59),and there were significant differences (P ＜ 0.05).The clinical symptom scores were positively correlated with the rrCBF of the left and right lentiform nucleus in 24 patients with HLD (r =-0.792 and-0.764,P ＜ 0.01),the content of urinary copper and duration had no correlation with the rrCBF of the left and right lentiform nucleus(P ＞ 0.05).Conclusions The rCBF of cerebral type and non-cerebral type HLD is significantly reduced,cerebral type patients have lower rCBF than non-cerebral type patients.The rCBF is correlated with the clinical symptom scores.