Central Impairment And Functional Remodeling Of Sensory Conduction In Charcot-Marie-Tooth Disease

Partâ… Central impairment of somatosensory conductive pathway in Charcot-Marie-Tooth DiseaseObjective:Identify the central impairment of deep sensation pathway in Charcot-Marie-Tooth disease.Materials and methods:We recruited 30 Charcot-Marie-Tooth Disease (CMT) patients in Hua Shan Hospital and 26 age,sex,height matched healthy people from November,2009 to March, 2010. We conducted CMT patients'clinical evaluation, including medical history inquiry:family history,course of disease; Neuropathy Symptom Score(NSS),Neuropathy Disability Socre(NDS) of Low limb and Nerve Conduction Velocity(NCV).Peak latency of N9,Peak latency of N13 and Peak latency of N9 were tested to to calculate the subjects' central conduction time(CCT). To compare the CCT of CMT group with the CCT of control group,we could judge if the CCT of CMT group were prolonged.So we could know CMT patients'central segment impairment of deep sensation conduction.Results:1. Gender:There is no statistically significant difference about central conduction time(CCT) between male and female healthy subjects. This means CCT has no relationship to gender.2. Height:Peak latencies (PL) of N9,N13 and CCT showed significantly positive correction in relation to height in control group.It showed that we should standardize the peak latency by height or the lenth of Limbs.3. Age:Peak latencies of N9,N13 showed no significant correction in relation to age in control group. Peak latencies of N20 and CCT showed significant positive correction in relation to age in control group.There is aging changes in somatosensory conduction pathway.4.PL of N9 and NCV:PL of N9 showed significantly negative correction in relation to NC V of median nerve in CMT group. PL of N9 could show the impairment of peripheral nerver.5.CCT and NCV:CCT showed no significant correction in relation to NCV of median nerve in CMT group.It showed that peripheral nerve conduction velocity has little influence to CCT.6. Interpeak latency (IPL)of N9-N13 was significantly later in CMT group than in control group which showed that CMT patients had the impairment of juxtamedullary brachial plexus.CCT was significantly later in CMT group than in control group which showed that CMT patients might have the impairment of dorsal column,medial lemniscus,thalamus,fibers from thalamus to cortex.Conclusion:1.As a stable component,CCT can be used to identify the central impairment of somatosensory pathway in Charcot-Marie-Tooth Disease.2. There is the impairment of peripheral nerve and central somatosensory pathway in CMT disease.Partâ…¡Functional imaging of central remodeling of sensory in Charcot-Marie-Tooth DiseaseObjective:Explore the central remodeling of sensory in Charcot-Marie-Tooth disease. Materials and methods:5 CMT1A patients,2 CIDP patients and 2 healthy people experienced fMRI scans. fMRI was performed with a blood oxygenation level-dependent (BOLD) sensitive T2-weighted multislice gradient echo EPI sequence. Using block design, stimulus was evoked by a soft brush. Background tasks included Attend-to-stimulus Task and Distraction Task. Group analysis of functional data was performed with statistical parametric mapping(SPM). Results:1. Healthy subjects:In attend-to-stimulus task, stimulating one side of lower limb, another side of postcentral gyrus activation was observed in two healthy subjects. In distraction task, the activation was decreased slightly but still existed obviously.2.CIDP subjects:In attend-to-stimulus task and in distraction task, no activation was observed.3.CMT patients with sensory symptoms:In attend-to-stimulus task, stimulating one side of lower limb, both sides of postcentral gyrus activations were observed in two CMT subjects(subjectl,subject2). In distraction task, both sides of activations disappeared completely.4.CMT patients without sensory symptoms:1) subject3:In attend-to-stimulus task, stimulating one side of lower limb, another side of postcentral gyrus activation was observed. In distraction task, another side of activation disappeared completely.2)subject4 and subject5:In attend-to-stimulus task, stimulating one side of lower limb, another side of postcentral gyrus activation was observed in two healthy subjects. In distraction task, the activation was decreased slightly but still existed obviously. Conclusion:There are different activation mode in CMT patients with different symptoms and course of disease.The patients with sensory symptoms and long course of disease might develop some compensation and amplification mechanism of "up-down". In the patients without sensory symptoms and long course of disease,the mechanism was not clear.