The Cognitive Impairment Characteristics And TCM Syndrome Differentiation Of CADASIL Patients In Guangdong Area

ObjectiveTo understand the distribution of TCM syndrome in CADASIL patients with cognitive impairments in Guangdong area and investigate the correlation of cognitive impairment caused by different TCM syndrome types.MethodsWe collected the clinical and imaging data of 50 CADASIL patients with cognitive dysfunction from Guangdong Provincial Hospital of Traditional Chinese Medicine from January 2012 to December 2020.These patients were measured with the Scale for the Differentiation of the Syndromes of Vascular Dementia(SDSVD).According to the results of syndrome differentiation,they are divided into the kidney essence deficiency group and the non-kidney essence deficiency group.Then two groups were measured with the Mini-Mental State Examination(MMSE),Clock draw test(cloxl,clox2),Hamilton Depression Scale(HAMD),and the Vascular Dementia Assessment Scale cognitive subscale(VaDAS-cog),Ability of Daily Living(ADL),the Chinese version of the executive interview(C-EXIT25)to evaluate and compare the correlation of cognitive impairment between the two groups.ResultsMutations in 50 CADASIL patients diagnosed by Notch3 gene testing mainly occurred in exon 11(62%)and exon 4(20%).In terms of clinical symptoms,the most common first symptom was cerebral infarction(58%),followed by cognitive impairment(26%),and migraine accounted for only 2%.In imaging,cranial white matter hyperintensity(WMH)mainly involved the lateral ventricle(100%),external capsule(14%),corona radiata(12%),centrum semiovale(10%);The lacunar infarcts mainly involved the corona radiata(76%),centrum semiovale(74%),basal ganglia(68%),and thalamus(64%);Among 15 patients undergoing SWI sequence examination,cerebral microbleeds were detected in 10 patients(50%),mainly located in the thalamus(100%),followed by cerebellum(90%),brain lobes(cortex/subcortex,70%),brain stem(70%),and basal ganglia(60%).The main TCM syndromes of CADASIL patients are mainly kidney essence deficiency syndrome(60%),blood stasis blocking collateral syndrome(20%),phlegm turbidity blocking orifice syndrome(12%),followed by Qi and blood deficiency syndrome(4%),Liver-yang hyperactivity(2%),fiery internal hyperactivity(2%).Two or three forms of TCM combination are more common,and a single syndrome combination accounts for only 1 case.A total of 15 syndrome-type combinations appeared in this group of patients,and 2 of the single syndrome-type combinations were all syndromes of deficiency of kidney essence;Of the two syndrome combinations,the main syndromes were deficiency of kidney essence and blood stasis blocking the collaterals(17%),kidney Essence deficiency and phlegm turbidity to prevent orifice(11%)mainly.The combination of three types of syndromes is mainly composed of deficiency of kidney essence and blood stasis blocking collaterals and phlegm turbidity blocking orifice(4%),deficiency of kidney essence and phlegm turbidity blocking orifice and deficiency of qi and blood(3%).The total scores of kidney essence deficiency syndrome MMSE and the correct reduction test scores in VaDAS-cog were lower than those of the non-kidney essence deficiency group.The difference was statistically significant,P<0.05;No statistically significant(P>0.05)in the scores of cloxl,clox2,ADL,C-EXIT25 among two groups.Conclusion1.CADASIL is a hereditary disease.The deficiency of kidney essence accounts for the highest proportion of TCM syndromes of all patients,indicating that the kidney is closely related to biological genetic inheritance.The deficiency syndrome of kidney essence can be used as an entry point.Explore the pathogenesis of CADASIL.2.From the results of the MMSE and VaDAS-cog scales,the degree of cognitive impairment in the kidney essence deficiency group is higher than that of the non-renal essence deficiency group,indicating that the kidney essence deficiency may play an important role in the disease process of cognitive impairment effect.