Objective Deep brain stimulation (DBS) can greatly improve the clinical symptoms of Parkinson's disease (PD),but it can poorly improve the similar clinical symptoms of multiple system atrophy P-type (MSA-P);therefore,identification is a necessity for the two diseases before DBS is carried out on these patients;surface electromyography (sEMG) was employed to analyze the surface electromyographic characteristics associated with tremor and rigidity of PD and MSA-P to explore the role of sEMG in the differential diagnosis of PD and MSA-P.Methods Twenty patients with PD and 25 patients with MSA-P,admitted to our hospital from June 2013 to January 2015,were enrolled in the study.The sEMG was performed on all patients on the 2nd d of hospitalization.Tremor frequency,tremor sEMG activity intensity and postural tremor latency were analyzed.Synchronous sEMG activity intensity during passive activities was analyzed.Root mean square (RMS) in two states was calculated,and t-test was applied to compare tremor frequency,postural tremor latency and sEMG activity intensity.Results The incidence of rest tremor in MSA-P patients was 36％ and that in PD patients was 60％,with significant difference (P＜0.05).And the incidence ofpostural tremor in MSA-P ones was 44％ and that in PD ones was 35％,with significant difference (P＜0.05).Besides,the postural tremor latency in MSA-P patients was significantly longer than that in PD patients ([9.3±3.2] s vs.[5.3±2.1] s,P＜0.05).Thepostural tremor and rest tremor frequencies of MSA-P patients ([7.3±2.1] and [6.4±3.6]-Hz) were significantly higher than those in PD patients ([5.3±2.4] and [4.9±1.2] Hz,P＜0.05).In rest tremor,RMS of flexor and extensor in MSA-P patients was significantly decreased as compared with that in PD patients (P＜0.05);in postural tremor,RMS of flexor and extensor in MSA-P patients was significantly decreased as compared with that in PD patients (P＜0.05).During passive activities,RMS of extensor in MSA-P patients was statistically higher than that in PD patients ([27.927.9± 11.4] vs.[18.318.3±6.4] μV,P＜0.05),while there was no significant difference between RMS of flexor in MSA-P and PD patients ([31.1±13.6] vs.[29.2±8.9] μV,P＞0.05).Conclusion The sEMG can be applied in the preoperative differential diagnosis for DBS of PD and MSAP.

Objective:To investigate the reasons for efficacy heterogeneity analysis of 3 patients with pantothenate kinase associated neurodegeneration (PKAN) from one family after deep brain stimulation (DBS).Methods:Three PKAN patients with the same PANK2 gene mutation from one family, admitted to and accepted bilateral subthalamic nucleus (STN)-DBS in our hospital from May to August 2021, were selected. The clinical manifestations, including general condition, medical history, symptoms, and signs, of these 3 patients were collected; Burkefahn-Marsden Dystonia Movement Rating Scale (BFMDRS) scores and surface electromyography results were analyzed and compared before DBS and 2 weeks and 6 months after DBS. Results:The clinical phenotype of these 3 patients had obvious heterogeneity: patient 1 had the latest onset age and shortest duration, and the main manifestation included abnormal body activity and forced postures, without obvious body deformation; preoperative surface electromyography suggested that the involuntary muscle contractions intensity was the smallest, and involuntary movements characterized by alternating patterns of contractile discharge activity were the predominant. The patient 2 and patient 3 had early onset age and long course of disease with gradually aggravated disease, and manifested as lower limb claudication and involuntary limb torsion; patient 3 also had marked limb deformities; preoperative surface electromyography showed high intensity of involuntary muscle contraction and torsional spastic dystonia characterized by co-contractile firing activity. The BFMDRS scores and surface electromyography results of the 3 patients after DBS were significantly improved as compared with those before surgery, with obvious heterogeneity; the improvement rates of BFMDRS scores of patient 1, 2 and 3 were 88.1%, 60.5%, and 43.2%, and the improvement rates of surface electromyography were 82.36%, 63.79% and 72.25%, respectively,at 6 months after surgery as compared with those before surgery.Conclusion:PKAN exhibits complicated clinical heterogeneity, which is one of the reasons for efficacy heterogeneity for PKAN after DBS.

Objective:To investigate the reasons for efficacy heterogeneity analysis of 3 patients with pantothenate kinase associated neurodegeneration (PKAN) from one family after deep brain stimulation (DBS).Methods:Three PKAN patients with the same PANK2 gene mutation from one family, admitted to and accepted bilateral subthalamic nucleus (STN)-DBS in our hospital from May to August 2021, were selected. The clinical manifestations, including general condition, medical history, symptoms, and signs, of these 3 patients were collected; Burkefahn-Marsden Dystonia Movement Rating Scale (BFMDRS) scores and surface electromyography results were analyzed and compared before DBS and 2 weeks and 6 months after DBS. Results:The clinical phenotype of these 3 patients had obvious heterogeneity: patient 1 had the latest onset age and shortest duration, and the main manifestation included abnormal body activity and forced postures, without obvious body deformation; preoperative surface electromyography suggested that the involuntary muscle contractions intensity was the smallest, and involuntary movements characterized by alternating patterns of contractile discharge activity were the predominant. The patient 2 and patient 3 had early onset age and long course of disease with gradually aggravated disease, and manifested as lower limb claudication and involuntary limb torsion; patient 3 also had marked limb deformities; preoperative surface electromyography showed high intensity of involuntary muscle contraction and torsional spastic dystonia characterized by co-contractile firing activity. The BFMDRS scores and surface electromyography results of the 3 patients after DBS were significantly improved as compared with those before surgery, with obvious heterogeneity; the improvement rates of BFMDRS scores of patient 1, 2 and 3 were 88.1%, 60.5%, and 43.2%, and the improvement rates of surface electromyography were 82.36%, 63.79% and 72.25%, respectively,at 6 months after surgery as compared with those before surgery.Conclusion:PKAN exhibits complicated clinical heterogeneity, which is one of the reasons for efficacy heterogeneity for PKAN after DBS.