Vitamin C supplementation lowers advanced glycation end products (AGEs) and malondialdehyde (MDA) in patients with type 2 diabetes: A randomized, double-blind, placebo-controlled clinical trial.

This study evaluated how daily vitamin C administration impacts systemic oxidative stress and inflammation and its safety in T2D patients. This randomized, double-blinded, placebo-controlled, parallel-arm clinical trial included 70 patients with T2D. They were allocated to receive either 500 mg/day of vitamin C or a matching placebo for 8 weeks. Of the 70 subjects assigned to the trial, 57 were included in the statistical analysis (vitamin C: n = 32, placebo: n = 25). Inflammatory and oxidative markers, including advanced glycation end products (AGEs), malondialdehyde (MDA), advanced oxidation protein products (AOPP), oxidized low-density lipoprotein (ox-LDL), highly sensitive C-reactive protein (hs-CRP), tumor necrosis factor-α (TNF-α), and ferric reducing ability of plasma (FRAP) were measured at baseline and the end of the trial. In addition, vitamin C tolerance was evaluated. A nutritionist visited all participants for a standard diabetic regimen. Following vitamin C supplementation, the serum levels of MDA (p-value < .001) and AGEs (p-value = .002) demonstrated a significant decrease after controlling for multiple confounders, including age, blood pressure, waist circumference, HbA1C, TG, and LDL-C, while no significant changes were observed for AOPP (p-value = .234) and ox-LDL (p-value = .480). The FRAP showed an increasing trend as an antioxidant marker but was not statistically significant (p-value = .312). The hs-CRP and TNF-α had no significant changes (p-value: .899 and .454, respectively). Also, no major adverse events were observed. Vitamin C supplementation may be beneficial in reducing AGEs and MDA in patients with T2D.

Medical education during the COVID-19 pandemic: lessons for the orthopedic departments.

BACKGROUND: After the Coronavirus pandemic, many educational routines were stopped for the safety of medical staff. To achieve educational goals, we have implemented new policies in our hospitals. In this study, we aimed to evaluate the effect of such strategies. METHOD: This survey-based study uses questionnaires to assess newly implemented educational strategies. We surveyed 107 medical staff of the orthopedic department of Tehran University of Medical Sciences, including faculty members, residents, and students. The survey contained three series of questionnaires for these groups. RESULTS: The maximum satisfaction for all three groups was observed in the platform and facilities for using e-classes, and the cost- and time-saving capabilities (Respectively, faculty members (FM): 81.8%, residents (R): 95.2%, students/interns (S/I): 87.0%; FM: 90.9%, R: 88.1%, S/I: 81.5%). The new policies have been shown to reduce the stress level of most trainees, increase the quality of knowledge-based education, increase the opportunity for reexamining educational content, expand discussion and research opportunities, and improve work conditions. There was a broad acceptance of the virtual journal clubs and morning reports. However, there were discrepancies between residents and faculty members on issues such as the evaluation of trainees, the new educational curriculum, and flexible shift schedules. Our strategies failed to improve skill-based education and patient treatment status. Most participants indicated that e-learning should be used with face-to-face training post-pandemic (FM: 81.8%, R: 83.3%, S/I: 75.9%). CONCLUSION: Our efforts to optimize the educational system during this crisis have generally improved trainees' work conditions and educational experience. Most participants believed that e-learning and virtual methods should be used alongside traditional training as a complementary component after the pandemic.

Effect of vitamin A on recovery from the acute phase of multiple sclerosis-related optic neuritis, double-blind, randomized, placebo-controlled trial.

Background: Optic neuritis (ON) is one of the main neuro-ophthalmic presentations of multiple sclerosis (MS), and it causes optic nerve atrophy and axonal loss. However, so far, there is no effective treatment to improve long-term outcomes. Methods: In a double-blind placebo-controlled randomized clinical trial, 50 patients with MS-related ON were allocated into two arms (24 in the control group and 26 in the intervention group) receiving either 25000IU retinyl palmitate or an identical placebo for six months. Visual evoked potential (VEP), visual acuity, and the retinal nerve fiber layer (RNFL) thickness were evaluated and compared before and after the treatment. Results: RNFL thickness reduction in the affected eyes at sixth month compared to the baseline were 14.81 and 19.46 µm, in the intervention and control groups, respectively (P=0.017). However, VitA therapy did not affect visual acuity and VEP. Conclusion: Vitamin A supplementation in the patients with acute ON in MS could lessen optic nerve axonal loss.

Comparison Between Distal and Proximal Approaches for Local Corticosteroid Injection in Carpal Tunnel Syndrome Management: A Randomized Controlled Trial.

BACKGROUND: The present, open-labeled study aimed to compare the distal approach (DA) for local corticosteroid injection (LCI) with the conventional proximal approach (PA) in alleviating the symptom and improving the electrodiagnostic parameters of the patients with carpal tunnel syndrome (CTS). METHODS: A total of 60 participants with nonsevere CTS were included in the present randomized controlled trial (RCT), of which 29 and 31 were assigned to the DA and PA groups, respectively. Each group received a single, landmark-guided injection of local methylprednisolone. The participants were assessed preintervention and 3 months later using the measures of visual analog scale (VAS), Boston Carpal Tunnel Questionnaire (BCTQ), hand grip strength, and nerve conduction study (NCS). RESULTS: Following a 3-month follow-up, both groups had significant improvements in VAS, both functional and severity subscales of BCTQ, hand grip strength, and some electrodiagnostic parameters (all P-values < .05). Moreover, the DA group had a significantly lower procedure duration than the PA group (9.80 ± 1.12 vs. 27.61 ± 1.77; P < .001). CONCLUSIONS: LCI using the DA should be considered a feasible, safe, and effective therapeutic method in patients with mild to moderate CTS. It had a shorter procedure duration than conventional PA, while their clinical and electrophysiological results were similar.

Effects of Nonconsecutive Sessions of Transcranial Direct Current Stimulation and Stationary Cycling on Walking Capacity in Individuals With Multiple Sclerosis.

BACKGROUND: Exercise has been demonstrated to be safe and well-tolerated in individuals with multiple sclerosis (MS). Physical activity has been shown to enhance the therapeutic effects of transcranial direct current stimulation (tDCS). This study aimed to determine the efficacy of intermittent tDCS combined with riding a stationary bicycle to improve walking capacity in individuals with MS. METHODS: This double-blind randomized controlled trial enrolled 50 eligible participants. Thirty-nine participants completed the study: 21 in the active group and 18 in the control group. Participants were assigned randomly to exercise on a stationary bike in conjunction with anodal tDCS or to exercise combined with a sham tDCS protocol. Walking capacity tests (2-Minute Walk Test, 5-Meter Walk Test, Timed Up and Go test), manual muscle testing, the Fatigue Severity Scale, and the Multiple Sclerosis Quality of Life-54 were used to determine outcomes. RESULTS: In terms of observed changes in 2-Minute Walk Test and 5-Meter Walk Test values, the exercise + tDCS group achieved significantly higher posttreatment values than the exercise + sham tDCS group. After the intervention and 1 month later, the intervention group's mean Timed Up and Go test value decreased significantly (P = .002) compared with that of the control group. There was no difference in Fatigue Severity Scale score, Multiple Sclerosis Quality of Life-54 score, or manual muscle testing improvement between the 2 groups. CONCLUSIONS: Nonconsecutive sessions of anodal tDCS combined with stationary cycling may have a greater effect on the walking capacity of individuals with MS than exercise alone.

The safety and efficacy of umbilical cord blood mononuclear cells in individuals with spastic cerebral palsy: a randomized double-blind sham-controlled clinical trial.

INTRODUCTION: The current multi-center, randomized, double-blind study was conducted among children with cerebral palsy (CP) to assess the safety and efficacy of umbilical cord blood mononuclear cell (UCB-MNC). We performed the diffusion tensor imaging to assess the changes in the white matter structure. METHODS: Males and females aged 4 to 14 years old with spastic CP were included. Eligible participants were allocated in 4:1 ratio to be in the experimental or control groups; respectively. Individuals who were assigned in UCB-MNC group were tested for human leukocyte antigen (HLA) and fully-matched individuals were treated with UCB-MNCs. A single dose (5 × 106 /kg) UCB-MNCs were administered via intrathecal route in experimental group. The changes in gross motor function measure (GMFM)-66 from baseline to one year after treatment were the primary endpoints. The mean changes in modified Ashworth scale (MAS), pediatric evaluation of disability inventory (PEDI), and CP quality of life (CP-QoL) were also evaluated and compared between groups. The mean changes in fractional anisotropy (FA) and mean diffusivity (MD) of corticospinal tract (CST) and posterior thalamic radiation (PTR) were the secondary endpoints. Adverse events were safety endpoint. RESULTS: There were 72 included individuals (36 cases in each group). The mean GMFM-66 scores increased in experimental group; compared to baseline (+ 9.62; 95%CI: 6.75, 12.49) and control arm (ß: 7.10; 95%CI: 2.08, 12.76; Cohen's d: 0.62) and mean MAS reduced in individuals treated with UCB-MNCs compared to the baseline (-0.87; 95%CI: -1.2, -0.54) and control group (ß: -0.58; 95%CI: -1.18, -0.11; Cohen's d: 0.36). The mean PEDI scores and mean CP-QoL scores in two domains were higher in the experimental group compared to the control. The imaging data indicated that mean FA increased and MD decreased in participants of UCB-MNC group indicating improvements in white matter structure. Lower back pain, headaches, and irritability were the most common adverse events within 24 h of treatment that were related to lumbar puncture. No side effects were observed during follow-up. CONCLUSIONS: This trial showed that intrathecal injection of UCB-MNCs were safe and effective in children with CP. TRIAL REGISTRATION: The study was registered with ClinicalTrials.gov ( NCT03795974 ).

Osteoarthritis-Related Knee Pain Treated With Genicular Artery Embolization: A Systematic Review and Meta-analysis.

BACKGROUND: Genicular artery embolization (GAE) is an innovative technique that has been investigated as a supplementary treatment method for chronic pain secondary to knee osteoarthritis (OA). PURPOSE: To evaluate the current evidence on the effectiveness and safety of GAE for OA-related knee pain. STUDY DESIGN: Systematic review; Level of evidence, 4. METHODS: A systematic literature search was conducted in the PubMed, Web of Science, EMBASE, and Scopus databases to identify studies related to knee OA treated with GAE. Treatment agents were categorized as Embozene, imipenem/cilastatin, resorbable microspheres, and polyvinyl alcohol. The main outcomes were the mean difference (MD) in pre- and postembolization pain based on the visual analog scale (VAS) or the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) scores as well as changes in the need for pain medication. Random- and fixed-effects models were applied for data analysis. RESULTS: Of 379 initially inspected publications, 11 (N = 225 patients; 268 knees) were included in the final review. The quality of the studies was fair in 8 and poor in 3-categorized according to the National Institutes of Health quality assessment tool. Overall, 119, 72, 13, and 21 patients were treated with imipenem/cilastatin, Embozene, resorbable microspheres, and polyvinyl alcohol, respectively. Symptomatic improvement was reported in all studies. The pooled effect size, characterized by MD, showed a significant improvement in the VAS and WOMAC pain scores, with better functional status after GAE. Pre- versus postembolization MDs in VAS scores ranged from 32 within the first week to 58 after a 2-year follow-up (equivalent to 54% and 80% improvement, respectively). There was a similar trend in the overall WOMAC scores, with MDs ranging from 28.4 to 36.8 (about 58% and 85% improvement, respectively). GAE resulted in a decreased need for pain medication for knee OA, with a 27%, 65%, and 73% decline in the number of patients who used opioids, nonsteroidal anti-inflammatory drugs, and intra-articular hyaluronic acid injection, respectively (P < .00001 for all). No significant difference between embolic agents was seen with regard to post-GAE pain reduction. No severe or life-threatening complications were reported. CONCLUSION: OA treated by GAE using different embolic particles can be considered generally safe, with good efficacy and no reported serious complications.

Efficacy of single high-molecular-weight versus triple low-molecular-weight hyaluronic acid intra-articular injection among knee osteoarthritis patients.

BACKGROUND: To compare intra-articular (IA) knee injections of a cross-linked high-molecular-weight hyaluronic acid (HMW-HA) with a linear low-molecular weight HA (LMW-HA) in terms of pain and functional improvement among knee osteoarthritis (OA) patients. METHODS: In this single-blinded RCT, the patients were randomly divided into two groups for HA injections. The first group received an HMW-HA (Arthromac) injection, while the other received three weekly LMW-HA (Hyalgan) injections. Pain and function were assessed using the outcome measures including WOMAC, Lequesne and VAS indices, once prior to injection, as well as 2 and 6 months after injections. RESULTS: A total of 90 patients were included. There was no significant difference in baseline characteristics including age and sex between the two groups. Our analysis showed that total WOMAC, Lequesne and VAS mean scores remarkably improved at both follow-up time-points compared to the baseline measurements (p < 0.001). There was no significant superiority between the two therapeutic protocols according to our outcome measures at any time-point of follow-up. The only except was about the improvement in WOMAC stiffness subscale that was significantly higher in LMW-HA group compared to HMW-HA (p = 0.021). Moreover, no significant difference was observed in minor complications and injection-induced pain scores between the two groups. CONCLUSION: This study proved that a single HMW-HA injection is as effective as multiple injections of LMW-HA counterparts in periods of 2 and 6 months follow-up. This study protocol was registered in Iranian database of RCTs (IRCT; www.irct.ir ) with the trial registration number IRCT20130523013442N24 and registration date 2018-07-13.

Ultrasound-Guided Corticosteroid Injection in Carpal Tunnel Syndrome: Comparison Between Radial and Ulnar Approaches.

PURPOSE: To compare two common approaches for ultrasonography (US)-guided injection. PATIENTS AND METHODS: Sixty patients with mild-to-moderate CTS were included in this double-blind randomized controlled trial (RCT). They received a single shot of corticosteroid injection through either the US-guided in-plane approach: radial or ulnar side. Participants were evaluated using Boston Carpal Tunnel Questionnaire (BCTQ) and visual analogue scale (VAS) for pain, as well as electrodiagnosis (EDX) and US parameters before the intervention, and within 12 weeks of follow-up. RESULTS: In both groups, all outcomes, except for the electrodiagnostic measures, significantly improved within the follow-up. Pain-VAS and both subscales of BCTQ questionnaire, as our main subjective outcomes, revealed dramatic improvement, with the largest amount of changes in VAS (70%; comparing to baseline value), and about 37% for both of BQSS and BQFS scales, all indicating superiority of radial to ulnar in-plane approach. During the first follow-up, we did not detect any remarkable preference between the groups in either subjective or electrodiagnostic variables. However, there was a significant difference at next follow-up time-points in terms of VAS for pain and BQFS favoring radial approach (Table 3). Furthermore, US-measured parameters including nerve-circumference and CSA improved only in the radial in-plane group. CONCLUSION: The current data proved that radial in-plane approach for CTS injection could be at least as effective as the more common ulnar in-plane method. Even the pain-relief effect was longer for the radial in-plane approach. Also, patients' functional status and objective variables all revealed better outcomes via the new approach.

Clinical Characteristics and Electrodiagnostic Features of Guillain-Barré Syndrome Among the Pediatric Population.

INTRODUCTION: Guillain-Barré syndrome is an immune-mediated peripheral neuropathy characterized by different clinical manifestations. We aimed to describe the clinical features, seasonal distribution, subtypes, and electrodiagnostic characteristics of Iranian children with Guillain-Barré syndrome. METHODS: In this prospective study, a total of 30 children with Guillain-Barré syndrome were evaluated. All demographic features were collected and electrodiagnostic study was assessed. RESULTS: Twelve participants were diagnosed with acute inflammatory demyelinating polyradiculoneuropathy and 18 patients were identified with acute motor axonal neuropathy. The initial findings showed that a significant number of patients (23 cases, P = .003) resided in rural areas. Our results showed a higher incidence of Guillain-Barré syndrome in summer and autumn months. No significant difference was observed between the seasonal distribution of acute inflammatory demyelinating polyradiculoneuropathy and acute motor axonal neuropathy subtypes. Antecedent history of pulmonary infections was recorded in 14 children with Guillain-Barré syndrome. Electrophysiological findings revealed a pattern of prolonged F wave latency with reduced persistency, absence of sensory nerve action potential, reduced compound muscle action potential amplitude, prolonged distal motor latency, reduced nerve conduction velocity, and abnormal temporal dispersion or conduction block in most patients with acute inflammatory demyelinating polyradiculoneuropathy. However, reduced compound muscle action potential amplitude, F wave with normal latency and reduced persistency, normal sensory nerve action potential amplitude, normal distal latency, normal sensory nerve conduction velocity, and conduction block or temporal dispersion were observed in most acute motor axonal neuropathy patients. CONCLUSION: The data support a correlation between Guillain-Barré syndrome incidence with seasonal variation and living area. Further studies should assess the Guillain-Barré syndrome features in pediatric population.

Persian Adaptation of Actionable Bladder Symptom Screening Tool Among Patients with Multiple Sclerosis.

PURPOSE: Multiple sclerosis (MS) is the most neurologic disease among individuals of 20-45 years. About 75% of MS patients report bladder problems that have a moderate-high impact on their life. The present study aimed to translate and determine the validity and reliability of the Persian version of ABSST questionnaire. METHODS: The standard validation process for preparing the Persian version of ABSST was performed by means of an expert committee. After a pilot study and confirming the harmonized translated form, we tested the final version of questionnaire on 40 patients with a definite diagnosis of MS symptoms, once at the baseline and another after two weeks in order to prevent recall-induced agreement. Test-retest reliability was calculated as Spearman's correlation coefficient. Also, content validity indices (CVIs), as well as internal consistency were analyzed in STATA. RESULTS: Forty participants with a mean age of 38.8 years were included in this study. Only 20% of them were male. The Persian version achieved a good internal consistency with a Cronbach-α value of 0.91, relatively similar to the original version. The coefficients for measuring the correlation between each item score with the total score of our questionnaire were between 0.58-0.89. This value confirmed an appropriate validity for the Persian version of ABSST. Regarding test-retest reliability assessment, total Spearman`s coefficient was 0.85; with 0.84 for the severity of symptoms and 0.87 for the impact of urologic disorders on patients` social life. CONCLUSION: The current findings proved that this accurate questionnaire could be used to investigate urinary symptoms among Persian-speaking MS patients.

Is Dextrose Prolotherapy Superior To Corticosteroid Injection In Patients With Chronic Lateral Epicondylitis?: A Randomized Clinical Trial.

PURPOSE: To compare the efficacy of dextrose prolotherapy versus steroid injection in the treatment of patients with chronic lateral epicondylitis. METHODS: Thirty subjects with chronic lateral epicondylitis were randomly assigned into two groups of hypertonic dextrose or methylprednisolone injection. Participants were assessed through Quick DASH and VAS scores, once before injection, and then after 1- and 3-months follow-up. Two patients were excluded due to not completing the follow-up timepoints. RESULTS: In both groups VAS scores revealed significant improvement during the first month follow-up [mean difference (MD) = 1.9±3.3, versus 1.5±1.9 for the prolotherapy and steroid groups, respectively]. This declining trajectory continued at the third month visit in the prolotherapy group and MD reached 4.4±2.9, while it did not change remarkably in the steroid group (MD=1.9±3.4). In fact, comparing VAS scores between the 1st- and 3rd-month time points did not reveal a significant improvement in the steroid group (p=0.6). Also, the Quick DASH index showed a similar pattern and improved remarkably in both groups during the first visit. However, only the efficacy in the prolotherapy group persisted after 3-month follow-up (MD = 9.5±21.6, p=0.044). One month after injections no preference between the two interventions was observed (p=0.74 for VAS and 0.14 for Quick DASH score). However, the 3rd-month follow-up revealed a meaningful superiority (p=0.03 for VAS and p=0.01 for Quick DASH score) favoring the prolotherapy method. CONCLUSION: Both methods were proven to be effective in the short-term treatment of chronic lateral epicondylitis, but dextrose prolotherapy seems to be slightly more efficacious than steroid injection over a longer period.Clinical trial registration: Iranian Registry of Clinical Trials Database: IRCT20170311033000N3.

Comparing The Efficacy Of Local Triamcinolone Injection In Carpal Tunnel Syndrome Using Three Different Approaches with or without Ultrasound Guidance.

PURPOSE: The present article has investigated the added value of ultrasound (US) guidance on improving the efficacy of local triamcinolone injection via comparing two US-guided methods versus a conventional landmark-guided approach. METHODS: Eighty-one subjects with mild or moderate CTS were included and randomly assigned into three categories including landmark-guided, conventional US-guided midline approach and US-guided ulnar in-plane method. Primarily, participants in the three groups were relatively similar in terms of demographics and their clinical variables comprising visual analog scale (VAS) for pain, pain-free grip strength (PFGS), Boston CTS questionnaire (BCTQ), EDX parameters, and cross-sectional area (CSA) of median nerve measured by ultrasonography. Ten weeks after injection, the changes of clinical and para-clinical outcomes were reassessed for 76 patients who finished the study. RESULTS: Our findings showed that all three injection methods were associated with a significant and relatively similar improvement in clinical and electrodiagnostic parameters. The post-injection evaluation showed a statistically significant change in all variables except for symptom severity score (SSS) of BCTQ. The best effect-size values were observed for VAS [56%] and functional severity scale (FSS) of BCTQ [42%], both reported in the US-guided midline group. However, no significant difference was found between the groups regarding their improvement in any of the outcome variables (P value >0.05). CONCLUSION: Based on the current data, all three injection methods were effective in improving electrodiagnostic findings and clinical symptoms of CTS. Although all approaches were relatively similar, US-guided midline approach was associated with slightly better outcomes.

Humeral shaft fracture: a randomized controlled trial of nonoperative versus operative management (plate fixation).

PURPOSE: This randomized controlled trial was conducted to investigate the outcomes of humeral shaft-fracture management with the functional Sarmiento brace (nonoperative) versus open reduction internal fixation (ORIF). METHODS: Sixty humeral shaft-fracture patients with a minimum age of 18 years were randomly assigned into two groups: operative treatment with open reduction-internal fixation (ORIF) or functional brace (Sarmiento). A similar postoperative rehabilitation program was applied for all subjects for the next 12 months. The outcomes of each method were measured in terms of nonunion rate, union time, "quick" Disabilities of Arm, Shoulder, and Hand (DASH) questionnaire scores, and rate of complications, such as malunion, infection, and radial nerve injury. RESULTS: The two groups had similar baseline characteristics, including age, sex, smoking status, and type and mechanism of fracture. The mean union time was about 4.8 weeks shorter in the ORIF group (13.9 weeks in operative group versus 18.7 weeks in nonoperative group), indicating a definite significant superiority (p=0.001) of ORIF management to functional Sarmiento bracing. However, a comparison of quick DASH scores revealed a borderline-significant difference between the groups (p=0.065). Additionally, we found that treatment of humeral shaft fractures using functional bracing was associated with slightly higher risk of nonunion; however this was not significant (p=0.492). CONCLUSION: According to the present findings, there is remarkable superiority of ORIF over functional Sarmiento bracing in the management of patients with humeral shaft fracture.

Local ozone (O2-O3) versus corticosteroid injection efficacy in plantar fasciitis treatment: a double-blinded RCT.

PURPOSE: To compare the efficacy of local ozone injection versus corticosteroid in plantar fasciopathy treatment. PATIENTS AND METHODS: This double-blinded randomized trial was performed on 44 adult patients with plantar fasciopathy. One group (23 patients) received local injection of 40 mg methylprednisolone, while a local injection of 3 cc oxygen-ozone solution was performed for the other group (21 subjects). Severity of pain, functional level, and pressure-pain threshold (PPT) were measured before treatment and 1, 4, and 12 weeks after injection using VAS, Foot and Ankle Ability Measure (FAAM) questionnaire, and algometer for PPT, respectively. RESULTS: The majority (65.9%) of the total 44 patients analyzed, were women. Both treatments efficiently relieved patients' pain and improved their functions at 1 and 3 months follow-up time-points. But one week after injection, the improvement in VAS (p<0.001) and FAAM (p<0.001) was significant only in the corticosteroid group. During the first month, VAS (p=0.35) and PPT (p=0.003) were still better in the corticosteroid group. However, FAAM revealed no remarkable difference between the two groups (p=0.083). Eventually, at the third month of follow-up, there was no remarkable preference between the treatments regarding any of the outcome measures (p>0.05). Nevertheless, both methods efficiently improved patients' symptoms, ie, over the 50% of pain reduction and 30% improvement in functional status. CONCLUSION: The present results showed no remarkable superiority between the two groups. In other words, although ozone injection showed a slower efficacy than methylprednisolone, it could be used in plantar fasciitis management as an appropriate alternative.

A novel case report of spinal muscular atrophy with progressive myoclonic epilepsy from Iran.

Spinal muscular atrophy (SMA) is a disorder characterized by decreased motor function due to the muscle atrophy in the background of degenerated anterior horn cells and motor cells of lower cranial nerves nuclei. The most frequent form is inherited as an autosomal recessive trait resulting from mutations in the survival motor neuron gene (SMN-1). On the other hand, a rare variant of this condition, named progressive myoclonic epilepsy subtype (SMA-PME) occurs in the result of a mutation in N-acylsphingosine amidohydrolase-1 gene (ASAH-1). The latter gene is responsible for lysosomal acid-ceramidase production. SMA-PME has been characterized by a progressive muscle weakness from ages 3-7 years, accompanied by epilepsy, an intractable seizure, and sometimes sensorineural hearing loss. In this report, we have presented a 15-year old female patient with SMA-PME that was attended to neurology clinic for a new onset tremor, seizure and proximal weakness in all limbs. We identified a homozygous mutation in exon II on her ASAH-1 gene [c.173C>T (p. Thr58Met)]. Also, a modest reduction was found in ceramidase-activity. As was expected patient`s seizures did not respond to conventional therapies.

Interesting effectiveness of ozone injection for carpal tunnel syndrome treatment: a randomized controlled trial.

Purpose: Carpal tunnel syndrome (CTS) is the most common entrapment neuropathy in the upper limb. Local injection of different substances has been increasingly used as an acceptable conservative treatment in non-severe cases. This study aimed to evaluate the efficacy and safety of local ozone injection in the management of non-severe CTS. Methods: In the current randomized controlled trial (RCT), 40 patients with mild or moderate CTS were included in two parallel groups. Both of them used the resting volar wrist splint for 8 weeks; while the intervention group received a single dose of local ozone injection, except for the same splinting protocol. The main outcome measures including visual analog scale (VAS) for pain; symptom severity or functional status, based on Boston questionnaire (BQ); and median nerve conduction study, were reassessed 10 weeks after the treatment. Results: All of the measures including VAS, symptom severity, functional status and EDX improved significantly in both groups with the maximal changes in VAS. The VAS reduction was more remarkable in the ozone group than the control group [64% versus 45.3%, respectively]. Moreover, both of the BQ subscales showed significantly higher improvement in the ozone group compared to the control group (P=0.01 and 0.02, respectively). Although the improvement of EDX parameters was slightly better in the ozone group, the difference was not significant. Neither minor nor major side effects were reported. Conclusion: Ozone therapy as a safe and low-cost method, could provide promising results among women with mild to moderate CTS, at least for short-term treatment. Clinical trial registration: IRCT2016040913442N9.

Diagnostic value of ultrasonography versus electrodiagnosis in ulnar neuropathy.

PURPOSE: Ulnar neuropathy at elbow is the second-most common compression neuropathy. The main aim of this study was to assess the diagnostic value of ultrasonography (US) as an alternative method to electrodiagnosis (EDX), which had traditionally been used as the method of choice. METHODS: This diagnostic study was conducted on 66 participants (32 patients` elbows and 34 normal elbows) referred for EDX. Both groups were reassessed by US to evaluate the consistency of the two tests. The quantitative parameters of US, such as cross-sectional area (CSA) of the ulnar nerve at three different levels around the medial epicondyle (ME) were compared between groups. RESULTS: Our findings demonstrated that CSA at the ME and 2 cm distal to the ME were significantly larger in the patient group than normal participants. This higher nerve size was more prominent among those who had predominant axonal loss rather than demyelinating lesions (P<0.01). Finally, we evaluated US diagnostic value with the best singular feature (2 cm distal to ME) at a cutoff of 9 mm2, which revealed specificity of 80% and sensitivity 84%. CONCLUSION: Based on these results we can conclude that US is a sensitive and specific method in diagnosing ulnar neuropathy at the elbow and can be used as an acceptable complementary method, in particular when EDX is not available.

The comparison of the effectiveness between different doses of local methylprednisolone injection versus triamcinolone in Carpal Tunnel Syndrome: a double-blind clinical trial.

PURPOSE: Local corticosteroid injection is one of the most prevalent methods in treating carpal tunnel syndrome (CTS). However, the most efficient substance and its appropriate dosage remain controversial. In the present double-blind randomized controlled trial, the efficacy and safety of local injection of two corticosteroids (triamcinolone and methylprednisolone) were compared at two different dosages, 20 and 40 mg. PATIENTS AND METHODS: We consecutively included 80 patients with mild or moderate CTS and randomly assigned them to four groups: 20 or 40 mg triamcinolone (T20 or T40) and 20 or 40 mg methylprednisolone (M20 or M40) groups; each patient received a single injection of steroid using conventional approach. The four groups were relatively comparable and did not show any significant difference initially in their baseline measurements including pain intensity measured using VAS, pain-free grip strength (PFGS), nerve conduction study (NCS), and two parts of Boston Carpal Tunnel Syndrome Questionnaire: symptom severity scale (SSS) and functional status scale (FSS); the latter was our primary outcome measure. Three months after injection, they were reassessed to evaluate the clinical and electrodiagnostic changes. RESULTS: Almost all NCS parameters, VAS, and PFGS significantly improved after treatment in all the groups (P<0.05). Compound motor action potential amplitude significantly improved only in T40 group (P=0.032), while there was no significant improvement in other groups. Furthermore, SSS remarkably decreased in all the four groups, without any significant difference between the groups (P=0.87). A similar significant decrease was found in FSS, with a higher improvement in T40 group (P=0.009). There was no significant difference between the four groups in other variables after treatment. CONCLUSION: Based on the current data, the efficacy and safety of local injection of triamcinolone and methylprednisolone at doses of 20 and 40 mg were associated with a significant improvement in pain, functional status, and strength. Although, there was no remarkable superiority, 40 mg injection, especially for triamcinolone, yielded better NCS results and functional status.

An investigation into the efficacy of intra-articular ozone (O2-O3) injection in patients with knee osteoarthritis: a systematic review and meta-analysis.

PURPOSE: This study aimed to review and pool the current literature on intra-articular ozone injection in knee osteoarthritis (OA) patients. METHODS: A systematic review of three big databases was performed to identify all English-language randomized clinical trials (RCTs) that evaluated the efficacy of intra-articular ozone injection vs a control injection for knee OA sufferers, using the following two measuring tools: pain VAS and Western Ontario and McMaster Universities Arthritis Index (WOMAC). RESULTS: A total of 428 patients in five RCTs were included, from which 53% (n=225) were in the ozone group and 47% in the control (hyaluronic acid [HA], dextrose, and air injection) group (n=203). The mean age of the patients in both groups was 64 years. Females were the majority. All studies had at least 2 months of follow-up (F/U). Mean difference (MD) between the groups for VAS in the first month was -0.23 with a P-value of 0.71 (negative value was in favor of ozone), whereas this difference in the third and sixth months reached 1.04 and 1.31, respectively, favoring the control group. These data demonstrated that control injection had a more prolonged pain relief period. A similar trend was seen regarding WOMAC scores; pooled results showed that ozone was slightly better than the control injections during the first month (MD =-7.84 [P=0.15]), but it declined to MD=2.55 and 8.23 at 2- to 3- and 4- to 6-month F/U, respectively, again in favor of control injections. Also, adverse events occurred homogeneously in both ozone (6/150 cases, 4%) and control groups (7/129 cases, 5.4%; P-value=0.31). CONCLUSION: Based on the current meta-analysis, intra-articular ozone injection efficacy was significantly superior to placebo and slightly lower to other control injections with non-significant difference. Therefore, ozone could be recommended as an efficient non-surgical treatment, durable for at least 3-6 months, in mild or moderate knee OA management.