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The clinical and imaging data in 6 patients with corticobasal syndrome were retrospectively analyzed. Six patients presented asymmetric clinical symptoms, including 5 with cognitive impairment, 6 with emotional disorders, 2 with cortical sensory deficit, 5 with lalopathy, and 4 with apraxia. All patients developed limb dystonia and limb or trunk stiffness, 4 with tumble, 4 with bradykinesia, and 2 with tremor. Brain magnetic resonance imaging (MRI) showed that 4 patients had unilateral cerebral atrophy and 2 had mild atrophy of bilateral hippocampus. Localized low glucose metabolism in the unilateral cerebral lobe was seen in four patients by positron emission computed tomography (PET) examination, suggesting that PET is helpful for the diagnosis of corticobasal syndrome.
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Objective@#To explore the evaluation of joint injury by HEAD-US-C (Hemophilic Early Arthropathy Detection with UltraSound in China, HEAD-US-C) in patients with moderate or severe hemophilia A treated with prophylaxis vs on-demand.@*Methods@#The patients from June 2015 to July 2017 with moderate or severe hemophilia A were examined by ultrasound imaging of the elbows, knees and ankles; Meanwhile the HEAD-US-C ultrasound assessment scale and hemophilia joint health score scale 2.1 (HJHS2.1) were used to score the joint status. The correlation between the HEAD-US-C and HJHS score was performed in prophylaxis group and on-demand group patients, respectively.@*Results@#A total of 925 cases of joint ultrasonography were conducted in 70 patients with moderate or severe hemophilia A. Among patients with moderate hemophilia, the median (IQR) of HEAD-US-C score and HJHS score in on-demand group were significantly higher than those in the prophylaxis group[1 (0, 6) vs 0.5 (0, 3) , z=0.177, P=0.046],[2 (0, 4) vs 2 (0, 3) z=0.375, P=0.007], even though there was no significant difference of the median (IQR) number of annualized target joints bleeding episodes between on-demand and prophylaxis groups[1 (0, 7) vs 1 (0, 5) , z=1.271, P=0.137]. Unlike in moderate cases, on-demand treatment group had more annualized target joints bleeding episodes than prophylaxis group among patients with severe hemophilia[3 (0, 8) vs 2 (0, 8) , z=0.780 P=0.037]. The prophylaxis group compared favorably with on-demand therapy group in terms of HEAD-US-C score[1 (0, 6) vs 4 (0, 7) , z=2.189, P=0.008], and HJHS score[2 (0, 5) , 4 (1, 6) , z=3646, P<0.001]for the severe hemophilia patients. The positive correlation between HEAD-US-C score and HJHS score was identified (P<0.05) , whether on-demand treatment or prophylaxis groups. The correlation coefficient between HEAD-US-C score and HJHS score in on-demand treatment and prophylaxis groups were 0.739 (95% CI 0.708-0.708) , 0.865 (95% CI 0.848-0.848) respectively, and 95% CI didn’t overlap (P<0.05) , indicating that the correlation coefficient in prophylaxis group had stronger correlation than that in on-demand group.@*Conclusions@#Clinical effects of prophylaxis were significantly better than those of on-demand treatment in patients with moderate or se-vere haemophilia A. HEAD-US-C scoring system could effectively evaluate joints damage in hemophilia A patients treated with on-demand or prophylaxis, companied by significantly positive correlation with HJHS clinical evaluation system, and provided objective index for clinical effect assessment.
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Objective@#To assess the feasibility of HEAD-US scale in the clinical application of hemophilic arthropathy (HA) and propose an optimized ultrasound scoring system.@*Methods@#From July 2015 to August 2017, 1 035 joints ultrasonographic examinations were performed in 91 patients. Melchiorre, HEAD-US (Hemophilic Early Arthropathy Detection with UltraSound) and HEAD-US-C (HEAD-US in China) scale scores were used respectively to analyze the results. The correlations between three ultrasound scales and Hemophilia Joint Health Scores (HJHS) were evaluated. The sensitivity differences of the above Ultrasonic scoring systems in evaluation of HA were compared.@*Results@#All the 91 patients were male, with median age of 16 (4-55) years old, including 86 cases of hemophilia A and 5 cases hemophilia B. The median (P25, P75) of Melchiorre, HEAD-US and HEAD-US-C scores of 1 035 joints were 2(0,6), 1(0,5) and 2(0,6), respectively, and the correlation coefficients compared with HJHS was 0.747, 0.762 and 0.765 respectively, with statistical significance (P<0.001). The positive rates of Melchiorre, HEAD-US-C and HEAD-US scale score were 63.0% (95%CI 59.7%-65.9%), 59.5% (95%CI 56.5%-62.4%) and 56.6% (95%CI 53.6%-59.6%) respectively, and the difference was statistically significant (P<0.001). Even for 336 cases of asymptomatic joints, the positive rates of Melchiorre, HEAD-US-C and HEAD-US scale score were 25.0% (95%CI 20.6%-29.6%), 17.0% (95%CI 12.6%-21.1%) and 11.9% (95%CI 8.4%-15.7%) respectively, and the difference was statistically significant (P<0.001). There were significant changes (P<0.05) in the ultrasonographic score of HA before and after onset of hemorrhage in 107 joints of 40 patients. The difference in variation amplitude of HEAD-US-C scores and HEAD-US scores before and after joint bleeding was statistically significant (P<0.001).@*Conclusion@#Compared with Melchiorre, there were similar good correlations between HEAD-US, HEAD-US-C and HJHS. HEAD-US ultrasound scoring system is quick, convenient and simple to use. The optimized HEAD-US-C scale score is more sensitive than HEAD-US, especially for patients with HA who have subclinical state, which make up for insufficiency of sensitivity in HEAD-US scoring system
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To explore clinical characteristics,electrophysiological findings and treatment response in diabetic patients with chronic inflammatory demyelinating polyradiculoneuropathy (DM-CIDP). Thirty-six CIDP patients were identified, 10 were DM-CIDP and 26 were non-DM-CIDP. Clinical medical records, electrophysiological data and treatment were retrospectively reviewed.DM-CIDP patients were significantly older than non-DM-CIDP patients [(56.7 ± 10.0) years old vs. (40.4 ± 16.9) years old, P=0.001]. However, clinical characteristics and abnormalities of electrophysiological tests in both groups were comparable. DM-CIDP subjects responded to corticosteroids or intravenous immunoglobulin, thus with better prognosis.
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Objective To investigate the clinical and radiological features of medullary infarction (MI), and to compare the clinical characteristics of lateral medullary infarction (LMI) and medial medullary infarction (MMI).Methods Patients diagnosed as acute MI who were treated from January 2009 to December 2014 in Department of Neurology, Peking University Third Hospital, were retrospectively enrolled in the study and data were analyzed including risk factors, clinical features, laboratory findings, radiological characteristics, etiology and outcomes.Results A total of 62 cases of MI were enrolled with 48 cases of LMI and 14 cases of MMI , including 2 cases of bilateral MMI.The mean onset age of LMI and MMI was 60.2 ± 12.3 and 56.9 ± 14.2, respectively.The frequently affected location of LMI was the middle and upper part of medulla [40 cases (83.3％)].The common symptoms and signs of LMI were dizzy (38 cases ,79.2％), sensory disturbance (33 cases ,68.8％), dysarthria (32 cases ,66.7％), dysphagia (30 cases ,62.5％), diminished pharyngeal reflex (30 cases, 62.5％), Homer's sign (29 cases, 60.4％), ataxia (26 cases, 54.2％) and nausea or vomiting (25 cases, 52.1％).The frequently affected location of MMI was the upper part of medulla (13 cases, 92.9％).The common symptoms and signs of MMI were motor dysfunction (12 cases, 85.7％), sensory disturbances (11 cases, 78.6％), dizzy (10 cases, 71.4％) and dysarthria (10 cases, 71.4％).Infarctions caused by atherosclerosis were found in 35 cases of LMI (72.9％) and 12 cases of MMI (85.7％).Five cases (10.4％) of LMI died in hospital, while 1 case (7.1％) of MMI died in hospital.No lesion was found in 16 cases (25.8％) by MRI-DWI within the first 24 hours of onset.Conclusions Our study showes that the mean onset age of LMI is older than that of MMI.The lesion of LMI is frequently located in the upper and middle medulla, whereas the lesion of MMI is mostly in the upper medulla.The prognosis of LMI is worse than that of MMI.Atherosclerosis of the vertebral arteries is the predominant vascular pathology in MI.
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<p><b>OBJECTIVE</b>To investigate the characteristics of ultrasonography in haemophilic arthropathy in knee joints.</p><p><b>METHODS</b>The knee joints (n=135) of 68 patients with haemophilia A (n=59) or haemophilia B (n=9) were consecutively evaluated with ultrasonography and scored according to MELCHIORRE ultrasound score. The correlations between ultrasound score and the number of knee joint bleeding episodes, age, and World Federation Haemophilic orthopedic (WFHO) score were studied.</p><p><b>RESULTS</b>68 male patients with median age 20 (3-48) years old were enrolled in this study. The prevalences of hydrops articuli, fibrotic septa, synovial hypertrophy, synovial hypertrophy with angiogenesis, haemosiderin deposition were 39.2% (53/135), 3.0% (4/135), 77.0% (104/135), 49.6% (67/135), 37.8% (51/135), respectively; and the prevalences of cartilage damage, bone erosion, bone remodeling, osteophytes were 63.7% (86/135), 48.1% (65/135), 3.0% (4/135), 13.3% (18/135) respectively. The average ultrasound score was 5.21[95% CI (4.50-5.93)]. The ultrasound score of knees joints according to age 0-6, 7-18 and > 18 years old groups were 0.63[95% CI (0.15-1.10)], 2.88[95% CI (1.90-3.85)], 7.83[95% CI (7.07-8.60)], respectively; and the difference among groups was statistically significant (P<0.01). The significant difference of ultrasound score was not found among moderate, mild and severe haemophilia (F=0.90, P=0.914). A significant positive correlation between ultrasound and WFHO scores was demonstrated (r=0.745, P<0.01). The ultrasound score increased significantly with the increase of number of knee bleed Episodes (F=49.926, P<0.01).</p><p><b>CONCLUSION</b>The main characteristics of ultrasonography of haemophilic arthropathy in knee joints were identified as followings: synovial hypertrophy, cartilage damage, bone erosion, hydrops articuli, and haemosiderin deposition. Ultrasonography was a fast, effective, safe, and available inspection method in assessment of haemophilic arthropathy.</p>
Subject(s)
Adolescent , Adult , Child , Child, Preschool , Humans , Male , Middle Aged , Young Adult , Hemophilia A , Hemophilia B , Joint Diseases , Diagnostic Imaging , Knee Joint , Diagnostic Imaging , UltrasonographyABSTRACT
Objective To explore the significance of motor unit number estimation (MUNE) by using multiple point stimulating technique to evaluate patients with Hirayama disease (HD).Methods Multiple point stimulating technique was used to estimate the motor unit number of abductor pollicis brevis and abductor digiti minimi in 35 normal subjects [14-33 years old,mean (20.9 ±4.0) years old,33 men and 2 women]without nerve and muscle disease and 69 patients definitely diagnosed as HD [16-35 years old,mean (21.46 ±6.61 ) years old,67 men and 2 women].The differences between the two groups were examined by Fisher's exact test and t test.Results There were 42 patients with atrophy and 27 patients with normal clinical manifestation of left hand.For right hand there were 54 patients with atrophy and 15 normal.For controls,the MUNE value of left abductor pollicis brevis was 226.97 ± 30.59,while that of right side was 22g.31 ± 25.35.The MUNE value of left abductor digiti minimi was 237.43 ± 30.78,while that of right side was 240.20 ± 37.73.For HD patients,the MUNE of left abductor pollicis brevis and abductor digiti minimi was 145.66 ± 126.10 ( t =5.07,P ＜ 0.01 ) and 102.20 ± 112.67 ( t =9.31,P ＜ 0.01 )respectively,while those of right hand was 149.72 ± 117.80 ( t =5.31,P ＜ 0.01 ) and 64.23 ± 69.27 ( t =16.76,P ＜0.01 ) respectively.MUNE of left abductor digiti minimi in 17 patients that was below 200 among 27 patients with normal clinical manifestation (x2 =9.57,P =0.002).MUNE of right abductor digiti minimi in 12 patients that was below 200 among 15 patients with normal clinical manifestation ( x2 =4.64,P =0.03).Conclusions The differences of MUNE values by multiple point stimulating technique between the normal subjects and the HD patients is significant,which suggests this method is very useful to evaluate HD in the early state.