Steroid-resistant peripheral neuropathy in a child: a rare finding in immunoglobulin a vasculitis.

BACKGROUND: Immunoglobulin A vasculitis (IgAV; Henoch-Schönlein purpura) is the most common vasculitis of childhood, affecting the small vessels with systemic involvement, especially the skin, joints, gastrointestinal system and kidneys. Peripheral neuropathy is very rare. Herein, we present a patient who was diagnosed as IgAV and developed refractory peripheral neuropathy in the course of disease. CASE: An 11-year-old boy was admitted to our clinic with pain and swelling in both ankles and symmetric palpable purpura extending from the knees to the dorsum of his feet. IgAV diagnosis was established and outpatient follow-up was started. On the 18th day of follow-up, he was admitted with widespread palpable purpura, myalgia and edema in the lower extremity, abdominal pain and left scrotal swelling. Intravenous prednisolone 2 mg/kg/day was started, all his symptoms improved and edema was resolved, but on the third day of the prednisolone therapy, the patient suffered from numbness in the left foot. Electromyoneurography showed moderate to severe axonal degeneration of the left tibial nerve. The symptoms of patient didn`t improve with bolus methylprednisolone and intravenous immunoglobulin therapy. All of the patient`s neurological complaints and signs regressed significantly within one week after bolus cyclophosphamide therapy. His oral prednisolone was gradually tapered and stopped at the end of the third month. After a follow-up period of six months, the patient had no complaints. CONCLUSION: Peripheral neuropathy is a rare complication of IgAV and occasionally it could be severe. Cyclophosphamide therapy should be kept in mind in patients with refractory neuropathy due to IgAV.

Effectiveness of Functional Electrical Stimulation - Cycling Treatment in Children with Cerebral Palsy.

AIM: The purpose of this study was to evaluate the effects of functional electrical stimulation (FES) bicycle therapy system on motor function, gait pattern, spasticity, daily living activities, and aerobic capacity in children with cerebral palsy (CP) and to compare the results with sham stimulation and standard treatment. METHODS: Patients with cerebral palsy who received botulinum toxin type-A injections to lower extremities and those with Gross Motor Function Measure Classification System (GMFCS) levels I - III, were included in the study. Twenty-five patients were randomly assigned into three treatment groups for 4-weeks: Group 1, FES-cycling and standard treatment; Group 2, Sham stimulus FES-cycling and standard treatment; Group 3, Standard treatment. Clinical assessment tools included the Modified Ashworth Scale (MAS), Modified Tardieu Scale (MTS), Pediatric Functional Independence Measure (WeeFIM), GMFCS, Gross Motor Function Measure-88 (GMFM-88), selective motor control tests, 6-minute walk test, and Visual Gait Analysis (VGA). RESULTS: In all groups, there were significant improvements in MAS, MTS, WeeFIM, GMFM-88, 6-minute walk test, and VGA scores. No changes in GMFCS levels were observed in any group. At the end of the study, there was no significant difference among the groups in terms of any clinical assessment parameter. CONCLUSIONS: All groups showed statistically significant improvements in motor function, walking pattern, spasticity, daily living activities, and aerobic capacity in patients with CP following the rehabilitation period. Although FES-cycling demonstrated no superiority over the other approaches and provided no additional benefit to the results, FES appears to be safe and well-tolerated in children with CP, at least as much as standard exercise treatment.

Comparing the effectiveness of ultrasound guided versus blind genicular nerve block on pain, muscle strength with isokinetic device, physical function and quality of life in chronic knee osteoarthritis: a prospective randomized controlled study.

BACKGROUND: The genicular nerve block (GNB) is demonstrated from several reports to alleviate pain and improve knee functionality in patients with chronic knee osteoarthritis (OA). Ultrasound (US)-guided GNB has been the most used imaging method. This study aimed to compare the effectiveness of US-guided versus blind GNB in the treatment of knee OA. METHODS: This prospective, randomized clinical trial included patients with knee OA based on American College of Rheumatology diagnostic criteria. The patients were evaluated for clinical and dynamometer parameters at the baseline, 4 weeks after treatment, and 12 weeks after treatment. The patients underwent blind injection or US-guided injection. RESULTS: When compared with the baseline, both groups showed significant improvement in pain, physical function, and quality of life parameters. Significant differences were observed between the groups for clinical parameters (30-second chair stand test, 6-minute walk test) in favor of the US-guided group. On the other hand, blind injection was more significantly effective on some parameters of the Nottingham Health Profile. There wasn't any significant improvement in isokinetic muscle strength for either group. CONCLUSIONS: This study demonstrated that both US-guided and blind GNB, in the treatment of knee OA, were effective in reducing symptoms and improving physical function. GNB wasn't an effective treatment for isokinetic muscle function. USguided injections may yield more effective clinical results than blind injections.

The effect of lateral pectoral nerve sparing technique and radiotherapy on the pectoralis major muscle applied with modified radical mastectomy.

BACKGROUND/OBJECTIVE: The aim of this study was to evaluate with electromyography (EMG) the effect of lateral pectoral nerve sparing technique (LPNST) and radiotherapy (RT) on the lateral pectoral nerve (LPN) in patients applied with modified radical mastectomy (MRM). METHODS: The study included 66 patients who underwent MRM surgery. The patients were separated into 2 groups as those applied with LPNST and those who underwent standard surgery (Control group). Within these 2 groups, patients were again separated as those who received or did not receive RT. The EMG evaluations were made by a neurology specialist blinded to the patient groups. RESULTS: The mean age of the patients was 53.3 ± 10.6 years. Standard surgery was applied to 33 (50%) patients and LPNST to 33 (50%) patients, RT was applied to 32 (48.5%) patients and not to 34 (51.5%) patients. In the EMG evaluation, latency was 2.1 ms (1.4-3.2) in the LPNST and 3.7 ms (1.9-12.4) in the control (p <0.001) and amplitude values were 9650 mV (3120-36900) in the LPNST and 4780 mV (510-12.4) in the control (p <0.001). The latency values in the Control receiving and not receiving RT were 4.0 ms (1.9-12.4) and 2.6 ms (1.9-6.2) respectively (p <0.05). The latency values of the patients receiving and not receiving RT in the LPNST were 2.2 ms (1.8-3.2) and 2.0 ms (1.4-2.4) respectively (p <0.05). In the Control and LPNST Group, no significant difference was determined between receiving and not receiving RT groups in respect of amplitude values (p >0.05). CONCLUSION: The results of this study demonstrated that electromyographically the latency and amplitude values were better protected in the LPNST group. It was also seen that RT increased the formation of nerve damage in both groups.

Guillain-Barré syndrome in children: subtypes and outcome.

OBJECTIVE: This study reviews the clinical features, subtypes, and outcomes of childhood Guillain-Barré syndrome (GBS). METHODS: Fifty-four children who attended a tertiary care training and research hospital in Turkey were enrolled in the study. RESULTS: The mean age was 6.5 ± 4.2 years and 32 patients (59.5%) were male. The most common subtype of GBS was acute inflammatory demyelinating polyneuropathy (AIDP), which was seen in 27 patients (50%). Having antecedent history, especially upper respiratory tract infection was significantly more common in AIDP (P = 0.028). Sensorial symptoms were significantly more frequent in axonal type GBS (P = 0.001). When we compare the demyelinating and axonal forms, all of the groups had favorable outcome. CONCLUSION: The diagnosis of pediatric GBS can be delayed because of its variable presentation. Early admission to hospital and early treatment are important for decreasing the need for respiratory support and improving the outcome.

Beneficial effect of faradic stimulation treatment on the rehabilitation of hysterical paraplegia.

Hysterical paralysis, a type of conversion disorder, presents with the loss of motor or sensory function. Although this disorder is nonorganic, it resembles the symptoms of a structural disease of the nervous system. It is generally associated with a traumatic or social event. The patients often require excessive testing and comprehensive assessment in exposing this psychogenic ailment. We reported the most dramatic type of conversion disorder, hysterical paralysis, in which full recovery was obtained with early recognition and rehabilitation approach including faradic stimulation.

Implementation of a respiratory rehabilitation protocol: weaning from the ventilator and tracheostomy in difficult-to-wean patients with spinal cord injury.

PURPOSE: Following repeated weaning failures in acute care services, spinal cord injury (SCI) patients who require prolonged mechanical ventilation and tracheostomy are discharged to their homes or skilled nursing facilities, with a portable mechanical ventilator (MV) and/or tracheostomy tube (TT) with excess risk of complications, high cost and low quality of life. We hypothesized that many difficult-to-wean patients with cervical SCI can be successfully managed in a rehabilitation clinic. The aim of our study was to develop a respiratory rehabilitation, MV weaning and TT decannulation protocol and to evaluate the effectiveness of this protocol in tetraplegic patients. METHODS: A multidisciplinary and multifaceted protocol, including respiratory assessment and management themes, was developed and performed based on the findings from other studies in the literature. Tetraplegic patients with the diagnosis of difficult-to-wean, who were admitted to the rehabilitation clinic after having been discharged from the intensive care unit to their home with home-type MV and/or TT, were included in this prospective observational study. RESULTS: The respiratory rehabilitation protocol was applied to 35 tetraplegic patients (10 home-type MV and tracheostomy-dependent, and 25 tracheostomized patients) with C1-C7 ASIA impairment scale grade A, B, and C injuries. Seven out of 10 patients successfully weaned from MV and 30 of 35 patients were decannulated. Four patients were referred for diaphragm pace stimulation and tracheal stenosis surgery. The mean durations of MV weaning and decannulation were 37 and 31 days, respectively. CONCLUSIONS: A multifaceted, multidisciplinary respiratory management program can change the process of care used for difficult-to-wean patients with SCI. Implications for rehabilitation Findings from this study indicate the significance of a multidimensional evaluation of any reversible factors for prolonged MV- and/or TT-dependent SCI patients. Thus, rehabilitation specialists should take this into consideration and should provide the appropriate amount of time to these patients. The proposed protocol of respiratory rehabilitation for MV- and/or TT-dependent SCI patients shows promising results in terms of changing the care used for these patients. Successful implementation of a respiratory rehabilitation and weaning protocol is dependent on careful planning and detailed communication between the rehabilitation specialist and intensivist during the respiratory rehabilitation process. Because many of the so-called difficult- or impossible-to-wean patients were successfully weaned from MV and TT in the PMR clinic, the need for such an outlet for countries without specialized centers is supported.

Comparison of Short-Term Clinical and Electrophysiological Outcomes of Local Steroid Injection and Surgical Decompression in the Treatment of Carpal Tunnel Syndrome.

AIM: To investigate the effectiveness of local steroid injection and surgical decompression in the treatment of patients with severe carpal tunnel syndrome (CTS) and also to compare short-term outcomes using clinical and electrophysiological criteria. MATERIAL AND METHODS: The patients diagnosed as severe CTS were divided into two groups. Group 1 received local steroid injection and Group 2 underwent surgical decompression. The Boston Questionnaire that consists of two sections as the Boston Symptom Severity Scale (BSS) and the Functional Status Scale (FSS) was completed by the patients. RESULTS: A total of 33 patients completed the study. Since two patients had bilateral severe CTS, a total of 35 hands were evaluated in the study. In Group 1, a significant difference was recorded between some pre- and post-treatment clinical parameters (BSS and FSS scores) and all electrophysiological parameters excluding motor conduction velocities. In Group 2, a statistically significant difference was found between pre- and post-treatment BSS scores and all electrophysiological parameters excluding motor conduction velocity and distal latency. However intergroup differences were not statistically significant as for all clinical and electrophysiological parameters (BSS, FSS, sensory amplitude, sensory conduction velocity, distal latency, motor amplitude, motor conduction velocity). CONCLUSION: In the treatment of severe CTS, steroid injection and surgical decompression achieved favourable improvements in clinical and electrophysiological parameters within a short-term without superiority of one treatment over other. Therefore, in patients in whom surgical decompression can not be applied, local steroid injection can be recommended as a less invasive and a promising treatment alternative.

Ultrasonographic Evaluation of the Radial Nerves in Patients with Unilateral Refractory Lateral Epicondylitis.

Objective: To evaluate the possible radial nerve entrapment of patients with unilateral refractory lateral epicondylitis (LE) by using ultrasound (US) and electroneuromyography. Design: Cross-sectional study. Setting: Three physical medicine and rehabilitation departments. Subjects: Consecutive 44 patients (15 M, 29 F) with unilateral refractory LE. Methods: All patients underwent detailed clinical, electrophysiological and ultrasonographic evaluations. Ultrasound imaging was used to evaluate thickness and presence of abnormal findings of the common extensor tendon (CET) and cross-sectional area (CSA) of the radial nerve (at spiral groove and before bifurcation) bilaterally. Unaffected sides of the patients were taken as controls. Results: When compared with the unaffected sides, CET thickness and radial nerve CSAs (at both levels) were higher, and abnormal US findings regarding LE (47.7% vs. 6.8%) were more common on the affected sides than nonaffected sides (all P < 0.001). Grip strength values were lower on the affected sides ( P < 0.001). Electrophysiological studies were all normal, and similar between the two sides (all P > 0.05). When subgroup analyses were performed after taking into account the hand dominance, affected and dominant sides were found to be the same in 31 and different in 13 patients. In subgroups, CETs and radial nerve CSAs at both levels were higher on the affected sides (all P < 0.01). Conclusions: Radial nerves and the CETs seem to be swollen on the affected sides, independent from the hand dominance of the patients with refractory LE. These results morphologically support the previous literature that attributes some of the chronic complaints of these patients actually to radial nerve entrapment.

AbobotulinumtoxinA for Equinus Foot Deformity in Cerebral Palsy: A Randomized Controlled Trial.

BACKGROUND: Although botulinum toxin is a well-established treatment of focal spasticity in cerebral palsy, most trials have been small, and few have simultaneously assessed measures of muscle tone and clinical benefit. METHODS: Global, randomized, controlled study to assess the efficacy and safety of abobotulinumtoxinA versus placebo in cerebral palsy children with dynamic equinus foot deformity. Patients were randomized (1:1:1) to abobotulinumtoxinA 10 U/kg/leg, 15 U/kg/leg, or placebo injections into the gastrocnemius-soleus complex (1 or both legs injected). In the primary hierarchical analysis, demonstration of benefit for each dose required superiority to placebo on the primary (change in Modified Ashworth Scale from baseline to week 4) and first key secondary (Physician's Global Assessment at week 4) end points. RESULTS: Two hundred and forty-one patients were randomized, and 226 completed the study; the intention to treat population included 235 patients (98%). At week 4, Modified Ashworth Scale scores significantly improved with abobotulinumtoxinA; mean (95% confidence interval) treatment differences versus placebo were -0.49 (-0.75 to -0.23; P = .0002) for 15 U/kg/leg and -0.38 (-0.64 to -0.13; P = .003) for 10 U/kg/leg. The Physician's Global Assessment treatment differences versus placebo of 0.77 (0.45 to 1.10) for 15 U/kg/leg and 0.82 (0.50 to 1.14) for 10 U/kg/leg were also significant (both Ps < .0001). The most common treatment-related adverse event was muscular weakness (10 U/Kg/leg = 2; placebo = 1). CONCLUSIONS: AbobotulinumtoxinA improves muscle tone in children with dynamic equinus resulting in an improved overall clinical impression and is well tolerated.

Outcomes of Botulinum Toxin Type A Injection Followed by Rehabilitation in Cases of Cerebral Palsy With Upper Extremity Involvement.

We evaluated the efficiency of botulinum toxin type A injection followed by a rehabilitation program including individual therapy, group therapy, and occupational therapy in cases of cerebral palsy with upper extremity involvement. A total of 29 injections were performed on 25 patients, and the patients were placed on rehabilitation program. At 3-month and 6-month assessments, there was a significant improvement in lateral grip strength, 9 Hole Peg test, Upper Limb Physician's Rating Scale and pediatric functional independence measure total scores. There were significant decreases in active range of motion in elbow extension, supination, and wrist extension, and Modified Ashworth Scale in elbow flexion, elbow pronation, and wrist flexion at 6-week, 3-month, and 6-month assessments. Combination of group therapy with traditional therapy methods after injection is effective in cases of cerebral palsy with upper extremity involvement.

Ulnar tunnel syndrome with ultrasonographic nerve imaging.

A 32-year-old man presented to our clinic complaining of numbness of the little finger and the ulnar aspect of the ring finger of his right hand. He complained about the weakness of grip strength and ulnar-sided pain. At the first glance, wasting of the first interossei muscle could be recognized. In his detailed examination, the medial half of the palmar aspect of the hand, including the hypothenar eminence, along with the palmar side of the fourth and fifth digits showed decreased sensation to light touch. Severe weakness of the abductor digiti minimi (ADM) was noted. No sensory loss was found in the dorsum of the hand, excluding the diagnosis of ulnar neuropathy at the elbow.

Postviral Vagal Neuropathy: What Is the Role of Laryngeal Electromyography in Improving Diagnostic Accuracy?

OBJECTIVES: To investigate the convenience of laryngeal electromyography (EMG) findings in patients with chronic cough thought to be postviral vagal neuropathy (PVVN) with the clinical symptoms. STUDY DESIGN: Prospective cohort study. METHODS: We applied PVVN questionnaire and chronic cough quality of life (QoL) questionnaire, which is for determining the effect of chronic cough on the QoL, to 20 chronic cough applicants who has no explanatory cause in differential diagnosis. We also carried out videolaryngostroboscopy (VLS) and laryngeal needle EMG in these patients. RESULTS: The mean duration of persisting cough was 1.875 months (SD ±0.825). The overall mean symptom score of chronic cough questionnaire was 58.80 (SD ±9.89). There was a significant positive correlation between total EMG score and chronic cough score (Spearman r, 0.489, P < 0.05). The correlation between VLS findings and either chronic cough scores or EMG scores did not reach statistical significance. CONCLUSIONS: Cranial nerves might be affected by inflammatory processes as occur in the PVVN, which must be considered in the etiology of chronic cough. We showed that the laryngeal EMG can be used as an appropriate diagnostic tool for these patients.

Functional reconstruction of the lower lip with Fujimori flap and long-term follow-up with clinical and electrophysiologic evaluations.

PURPOSE: The Fujimori gate flap is an innervated flap raised from the nasolabial area for reconstruction of the lower lip. No electromyographic or clinical long-term studies have analyzed the long-term outcomes of lower lip reconstruction performed using this method. The aim of the present study was to assess the outcomes of lower lip reconstruction with the Fujimori gate flap using clinical and electrophysiologic examinations. PATIENTS AND METHODS: Ten patients who had been treated with this procedure were evaluated clinically and electrophysiologically. The follow-up period was 1 year for all patients, and the patients underwent regular assessments. RESULTS: All flaps survived completely, and no wound healing problems were encountered. Four patients underwent revision in the late postoperative period. The electrophysiologic studies revealed the presence of reinnervation in all 10 patients. CONCLUSIONS: In the present study, we found that the Fujimori gate flap is a versatile flap for ideal reconstruction of lower lip defects. Our electrophysiologic assessments showed that the transferred muscle had undergone reinnervation and that the donor area innervation had been preserved. The findings from the serial clinical and electrophysiologic assessments indicated satisfactory results.

Importance of cutaneous silent period in fibromyalgia and its relationship with disease characteristics, psychological disorders and quality of life of patients.

INTRODUCTION: Cutaneous silent period (CSP) is an inhibitory spinal protective reflex and its afferents consist of A-delta nerve fibers. We aimed to evaluate patients with fibromyalgia (FM) and healthy controls to determine any differences between the groups in terms of CSP duration and latency, and if present, to determine whether there is any relationship with disease characteristics, psychological disorders and quality of life. MATERIALS AND METHODS: Thirty-two patients with FM and 32 healthy volunteers were included in the study. The patient and control groups were compared in terms of CSP latency and duration in both upper and lower extremities. Disease characteristics, psychological disorders and quality of life of patients were assessed using the Fibromyalgia Impact Questionnaire (FIQ) and Short Form-36 (SF-36). Patients with CSP measurements equal to or lower than those of the control group were compared with those with higher values than controls in terms of disease characteristics, psychological status and quality of life. RESULTS: Significantly prolonged CSP latencies in both upper and lower extremities were determined in patients compared to controls. We found that prolongation of CSP latency in the lower extremity is associated with disease severity and functional disability. CONCLUSIONS: CSP latencies in both upper and lower extremities in patients with FM are longer than in healthy volunteers. Moreover, prolongation of CSP latency in the lower extremity is associated with disease severity and physical functional disability.