Hand strength and dexterity in patients with Prader-Willi syndrome: a pilot intervention study.

OBJECTIVE: The study aim was to examine the hand function (hand strength and dexterity) and intervention effects of training in adults with Prader-Willi syndrome (PWS). METHODS: Six adults with PWS (two females; mean age 26.14 years) underwent hand muscle strength and dexterity training for 3 months (2 hours per week). The following hand function tests were performed pre- and post-intervention: (1) hand grip, lateral pinch, and tip pinch hand strength tests, (2) the Box and Block test (BBT) for gross manual dexterity and (3) the Purdue Pegboard test for finger dexterity. RESULTS: Before treatment, all subjects showed lower hand grip, lateral pinch, tip pinch strength, and poorer manual/finger dexterity relative to healthy adults. After training, hand function scores improved on many test items, but only the left hand tip pinch and the right hand BBT performance showed significant improvements. CONCLUSIONS: All subjects showed lower hand strength and poorer manual/finger dexterity compared with healthy adults; this should be considered during physical training programs. Owing to limitations in the intervention intensity and possible subject behavioral deficits, further research is needed to clarify the effects of this intervention on hand function in PWS patients.

Electric field simulation and analysis of modified electroconvulsive therapy and magnetic seizure therapy in a realistic human head model / 生物医学工程学杂志

Modified electroconvulsive therapy (MECT) and magnetic seizure therapy (MST) are effective treatments for severe major depression. MECT has better efficacy in the treatment than MST, but it has cognitive and memorial side effects while MST does not. To study the causes of these different outcomes, this study contrasted the electric filed strength and spatial distribution induced by MECT and MST in a realistic human head model. Electric field strength induced by MECT and MST are simulated by the finite element method, which was based on a realistic human head model obtained by magnetic resonance imaging. The electrode configuration of MECT is standard bifrontal stimulation configuration, and the coil configuration of MST is circular. Maps of the ratio of the electric field strength to neural activation threshold are obtained to evaluate the stimulation strength and stimulation focality in brain regions. The stimulation strength induced by MECT is stronger than MST, and the activated region is wider. MECT stimulation strength in gray matter is 17.817 times of that by MST, and MECT stimulation strength in white matter is 23.312 times of that by MST. As well, MECT stimulation strength in hippocampi is 35.162 times of that by MST. More than 99.999% of the brain volume is stimulated at suprathreshold by MECT. However, MST activated only 0.700% of the brain volume. The stimulation strength induced by MECT is stronger than MST, and the activated region is wider may be the reason that MECT has better effectiveness. Nevertheless, the stronger stimulation strength in hippocampi induced by MECT may be the reason that MECT is more likely to give rise to side effects. Based on the results of this study, it is expected that a more accurate clinical quantitative treatment scheme should be studied in the future.

Motor performance in Prader-Willi syndrome patients and its potential influence on caregiver's quality of life.

BACKGROUND: Prader-Willi syndrome (PWS) is a complex, multisystem genetic disorder characterized by a variety of physical, cognitive, and behavioral impairments. PWS is a unique sarcopenia model characterized by an abnormal increase in body fat mass and a decrease in muscle mass that predisposes patients to reduced physical activity, functional limitations, and disability. These manifestations may require both symptomatic and supportive management, thus negatively influencing their lifelong family caregiver's quality of life. The aim of this study was to examine the functional motor performance of adults with PWS in Taiwan and to measure the quality of life of their primary family caregivers. METHODS: The functional motor tests consisted of the following: (1) 30-s sit-to-stand test, (2) timed up-and-go test, (3) hand grip and lateral pinch strength tests, and (4) Berg Balance Scale. The World Health Organization Quality of Life-short form (WHOQOL-BREF) and the Short-Form 36 Health Survey Questionnaire (SF-36) were used to evaluate health-related quality of life, and the parenting stress index was used to assess the magnitude of stress within the parent-child system. RESULTS: The participants included seven adults (two females and five males) with genetically confirmed PWS and their respective main caregivers. The mean age of the adults with PWS was 25.28 years; range 18-31 years, SD 5.10; the mean BMI was 29.2 kg/m2, SD 6.43. All adults with PWS showed lower hand grip and lateral pinch strengths, fewer sit-to-stand cycles during the 30-s chair stand test, and greater average time during the timed up-and-go test when compared to the normative data on healthy adults. Balance was negatively correlated with the caregiver's health concepts of social functioning (rs -0.879, P = 0.009) and with role limitations due to physical problems (rs -0.899, P = 0.006) and emotional problems (rs -0.794, P = 0.033); hand grip strength was negatively correlated with bodily pain (rs -0.800, P = 0.031), as assessed using the SF-36 questionnaire. The timed up-and-go test was positively correlated with the social relationship domain (rs 0.831, P = 0.021), as assessed using the WHOQOL-BREF questionnaire. The parenting stress index showed no association with the PWS patient's physical activities. CONCLUSIONS: All adults with PWS showed decreased upper and lower limb strength and functional mobility when compared to healthy adults. Some of their motor performance might have negative effects on their primary family members in terms of social participation and physical and emotional role limitations. Future research should explore the relationship between physical performances, psychological difficulties of PWS and caregiver's QOL.

Precision pinch performance in patients with sensory deficits of the median nerve at the carpal tunnel.

To investigate how sensory symptoms impact the motor control of hands, in this study we examined the differences in conventional sensibility assessments and pinch force control in the pinch-holding-up activity (PHUA) test between carpal tunnel syndrome (CTS) patients and healthy controls. CTS patients (n = 82) with 122 affected hands and an equal number of control subjects were recruited to participate in the threshold, discrimination, and PHUA tests. The patients showed significantly poorer hand sensibility and lower efficiency of force adjustment in the PHUA test as compared with the control subjects. Baseline pinch strength and the percentage of maximal pinch strength for the PHUA were significantly higher for the subgroup of sensory nerve action potential (SNAP) of <16 µV than for the subgroup of SNAP of 16 µV. Using a PHUA perspective to analyze the efficiency of force-adjustment could assist the clinical detection of sensory nerve dysfunction.

Detection of early cognitive impairment using AD8 in a young patient with stroke with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy syndrome: a case report.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) syndrome is a hereditary disease resulting from NOTCH3 gene mutation. The clinical presentations include migraine, recurrent stroke, and cognitive impairment. The severity of cognitive impairment varies in different stages, and early recognition poses a challenge. A 47-year-old lady presented with chronic migraine and sudden onset of hemiparesis. Magnetic resonance imaging revealed compatible findings of CADASIL, which was confirmed by mutation analysis of NOTCH3 gene. Early cognitive impairment was detected by her score of 3 in Ascertain Dementia 8 (AD8) questionnaire and confirmed by detailed neuropsychological assessments. After 21 months of follow-up, deterioration in her cognition and ability to perform instrumental activities of daily living were significant with a follow-up AD8 score of 7. Ascertain Dementia 8 questionnaire is an easy and valid screening tool for early cognitive impairment in patients with CADASIL syndrome.

Effect of acetazolamide for long-lasting paroxysmal dystonia in a patient with multiple sclerosis: a case report and review of literature.

Dystonia is a rare manifestation of multiple sclerosis (MS), but it always interferes with the functional performance and quality of life. We report a rare case of long-lasting paroxysmal dystonia associated with MS. The patient was a 40-year-old woman with relapsing- remitting MS for 6 years. During the latest attack of MS, she suffered from long-lasting paroxysmal dystonia in her left hand. Despite treatment with pulse high-dose intravenous methylprednisolone, interferon, and baclofen, along with occupational therapy, the dystonia persisted and significantly bothered her daily activities. Finally, she was treated with oral acetazolamide (250 mg, three times a day for 4 days), which was very effective for the control of her dystonia. The dystonic movement subsided without recurrence in a follow-up of 17 months. We advocate this effective and safe treatment for patients with paroxysmal dystonia associated with MS.

Semisynthesis and myocardial activity of thaliporphine N-homologues.

The N-homologues and optical isomers of thaliporphine (5a), a potent antiarrhythmic agent, were prepared starting from laurolitsine (1), an abundant aporphine present in Phoebe formosana. Treating N-propylnorglaucine with 90% H2SO4 yielded one additional product, an 11-sulfonyl-1,11-anhydroaporphine. Reaction of N-formylnorglaucine (3a) with 90% H2SO4, however, yielded the 9-sulfonyl-seco product as a major product. Treatment of 3a with 98% H2SO4 yielded pancordine (10), which, upon catalytic hydrogenation, yielded (±)-wilsonirine. (1)H NMR spectroscopic analysis was applied successfully to monitor the optical purity of the crystalline salt while undertaking optical resolution. Thaliporphine (5a) was demonstrated to possess better positive inotropic and less negative chronotropic effects than the left-hand optical isomer and showed the best activity on rat cardiac tissue among the N-homologues prepared.

Therapeutic effects of lidocaine patch on myofascial pain syndrome of the upper trapezius: a randomized, double-blind, placebo-controlled study.

OBJECTIVE: The aim of this study was to compare topical 5% lidocaine patch with placebo patch in the treatment of myofascial pain syndrome of the upper trapezius. DESIGN: In this prospective, randomized, double-blind, placebo-controlled study, 60 participants were randomly assigned, placing 31 subjects in the 5% lidocaine patch group and 29 subjects in the placebo patch group. We used the Verbal Rating Scale (VRS), the Pressure Pain Threshold, the ranges of motion of the neck, and the Neck Disability Index to evaluate the subjective pain intensity, objective pain intensity, ranges of motion, and disability of the neck, respectively. Outcome measures were performed before (day 0) the treatment course, 12 hrs after removal of the final patch on the seventh day (day 7), and 1 wk (day 14) and 3 wks (day 28) after the completion of treatment course. RESULTS: The characteristics of the participants did not differ at baseline. Pain intensity assessed by the VRS decreased at day 7 in both the lidocaine patch and placebo patch groups. There was no significant difference between the two groups in the VRS, the Pressure Pain Threshold, the ranges of motion, and the Neck Disability Index. At day 14, the experimental group continued to improve in the VRS (1.06), but the pain of the placebo group aggravated (VRS, 1.5). The difference is significant (P = 0.03). In addition, the Neck Disability Index in the lidocaine patch group decreased significantly as compared to that in the placebo group. The pain-relieving effect of the lidocaine patch attenuated, and it was not significantly different between the two groups at day 28 in the VRS and the Neck Disability Index. Neither the Pressure Pain Threshold nor the ranges of motion were significantly different through the periods of this study. CONCLUSIONS: The application of the 5% lidocaine patch is probably superior to the placebo patch in relieving pain and in reducing associated neck disability for a period of longer than 1 wk for treating patients with myofascial pain syndrome of the upper trapezius.

Empirical study on application of insulin in treating traumatic spinal cord injury in rat models / 重庆医科大学学报

Objective:To observe neuroprotective effects of insulin on traumatic spinal cord injury in rat models and discuss its potential mechanisms. Methods:60 SD rats were randomly divided into A group(control group) and B group(insulin group), with 30 rats in each group,The animal SCI model was established according to method Allen's,A group and B group were respectively infused with isotonic Na chloride or 1Iu/kg insulin through abdominal cavity,three times a day,for 7 days. apoptosis neurons were observed on 8 hours,3 days,7 days,14 days,and 28 days after SCI,and tigroid body,neurons,GAP-43 positive cells,and BBB score were measured on 1 day,3 days,7 days,14 days,and 28 days after SCI. Results:Neuron number,GAP-43 positive cells,and BBB score in multitude time points were significantly higher in B group than in A group(P