Selective dorsal rhizotomy for the treatment of severe spastic cerebral palsy: efficacy and therapeutic durability in GMFCS grade IV and V children.

BACKGROUND: Selective dorsal rhizotomy (SDR) has been established as an effective surgical treatment for spastic diplegia. The applicability of SDR to the full spectrum of spastic cerebral palsy and the durability of its therapeutic effects remain under investigation. There are currently limited data in the literature regarding efficacy and outcomes following SDR in Gross Motor Function Classification System (GMFCS) IV and V patients. Intrathecal baclofen has traditionally been the surgical treatment of choice for these patients. When utilised primarily as a treatment for the relief of spasticity, it is proposed that SDR represents a rational and effective treatment option for this patient group. We report our outcomes of SDR performed on children with severe cerebral palsy (GMFCS grade IV and V). The commensurate improvement in upper as well as lower limb spasticity is highlighted. Apparent benefit to urological function following SDR in this patient group is also discussed. METHOD: A retrospective review of prospectively collected data for 54 paediatric patients with severe cerebral palsy (GMFCS IV-V) who received SDR plus specialised physiotherapy. Mean age was 10.2 years (range, 3.0-19.5). SDR guided by electrophysiological monitoring was performed by a single experienced neurosurgeon. All subjects received equivalent physiotherapy. The primary outcome measure was change to the degree of spasticity following SDR. Spasticity of upper and lower limb muscle groups were quantified and standardised using the Ashworth score. Measures were collected at baseline and at 2-, 8- and 14-month postoperative intervals. In addition, baseline and 6-month postoperative urological function was also evaluated as a secondary outcome measure. RESULTS: The mean lower limb Ashworth score at baseline was 3.2 (range, 0-4). Following SDR, significant reduction in lower limb spasticity scores was observed at 2 months and maintained at 8 and 14 months postoperatively (Wilcoxon rank, p < 0.001). The mean reduction at 2, 8 and 14 months was 3.0, 3.2 and 3.2 points respectively (range, 1-4), confirming a sustained improvement of spasticity over a 1-year period of follow-up. Significant reduction in upper limb spasticity scores following SDR was also observed (mean, 2.9; Wilcoxon rank, p < 0.001). Overall, the improvement to upper and lower limb tone following SDR-generally to post-treatment Ashworth scores of 0-was clinically and statistically significant in GMFCS IV and V patients. Urological assessment identified pre-existing bladder dysfunction in 70% and 90% of GMFCS IV and V patients respectively. Following SDR, improvement in urinary continence was observed in 71% of affected GMFCS IV and 42.8% of GMFCS V patients. No serious postoperative complications were identified. CONCLUSIONS: We conclude that SDR is safe and-in combination with physiotherapy-effectively reduces spasticity in GMFCS grade IV and V patients. Our series suggests that spastic quadriplegia is effectively managed with significant improvements in upper limb spasticity that are commensurate with those observed in lower limb muscle groups. These gains are furthermore sustained more than a year postoperatively. In light of these findings, we propose that SDR constitutes an effective treatment option for GMFCS IV and V patients and a rational alternative to intrathecal baclofen.

Hydrocephalus and trigeminal neuralgia: exploring the association and management options.

We report the successful surgical management of three patients with trigeminal neuralgia and hydrocephalus. MRI revealed no neurovascular contact at the trigeminal root entry zone. Trigeminal neuralgic symptoms were controlled following alleviation of hydrocephalus. We hypothesize that trigeminal nerve traction, secondary to hydrocephalus, as the cause for their trigeminal neuralgia.

Selective dorsal rhizotomy as an alternative to intrathecal baclofen pump replacement in GMFCS grades 4 and 5 children.

BACKGROUND: Conventionally, selective dorsal rhizotomy (SDR) has been reserved for ambulant children and implantation of intrathecal baclofen (ITB) pump for non-ambulant children with cerebral palsy. Rather than replacing the ITB pump in selected Gross Motor Function Classification System (GMFCS) grades 4 and 5 children, we elected to undertake SDR instead. We discuss the rationale and outcomes. OBJECTIVES: To assess if children with severe spasticity treated with long-term ITB pump would benefit from SDR as alternative procedure to replacement of ITB pump. METHOD: This study is a prospective review of ten children with severe spasticity. Indications for ITB pump replacement in 3/10 children were previous ITB pump infection and the remaining seven were nearing depletion of drug delivery system. Pre- and post-SDR mean modified Ashworth scores, assessment of urological function and survey of parent/carer satisfaction were undertaken. RESULT: Mean Ashworth score reductions post-SDR in the lower limbs and upper limbs were 2.4 and 1.70, respectively. An improvement in urological function was also noticed in 27% of patients. Overall, 90% of parents/carers felt that functional outcome with SDR was improved compared with that of ITB. CONCLUSION: SDR in comparison to ITB in this subgroup is cheaper, less intrusive by avoiding refills/replacement and found to be more effective than ITB in reducing spasticity and providing ease for nursing care. We therefore suggest that consideration should be given to SDR as an alternative in patients previously implanted with ITB systems complicated by infection or nearing end of battery life.

Combined selective dorsal rhizotomy and scoliosis correction procedure in patients with cerebral palsy.

UNLABELLED: Intrathecal baclofen (ITB) therapy for spasticity has been suggested to accelerate the development of scoliosis. We present the case of a 17-year-old female patient with cerebral palsy who had ITB therapy from the age of 11 years. During this period, she developed a severe scoliosis measuring 86° from T11 to L4, with pain due to costo-pelvic impingement. Her baclofen pump had reached its end of life and required replacement if ITB therapy was to continue. This coincided with plans for scoliosis corrective surgery. METHODS: We performed scoliosis correction along with removal of baclofen pump and selective dorsal rhizotomy (SDR), as a single combined procedure. SDR was performed instead of ITB pump replacement for management of spasticity. RESULTS: Following surgery, scoliosis improved to 24°. At 6 month follow-up, there was significant improvement in spasticity and quality of life. CONCLUSIONS: This report illustrates the feasibility of a combined procedure to correct scoliosis and manage spasticity with SDR. We present the case details, our management and review of the published literature regarding the factors influencing treatment of scoliosis and spasticity.

Spinal cord stimulation during the antepartum and intrapartum periods: a case report showing no deleterious effect of conventional paraesthesia producing stimulation.

We report on a 25-year-old woman who used her spinal cord stimulator (SCS), previously inserted for chronic regional pain syndrome (CPRS), to assist with both chronic and pregnancy-related pain management. We describe the therapeutic effect of the stimulator and briefly highlight the issues surrounding the use of SCS during pregnancy.

Development and research directions in ship recycling: A systematic literature review with bibliometric analysis.

Ship recycling has gained significant importance in recent years due to the growing awareness of environmental concerns and the need for sustainable practices within the maritime industry. As vessels reach the end of their operational life, proper recycling methods are crucial to mitigate environmental impacts and promote resource conservation. With an increasing number of ships being decommissioned annually, there has been a growing interest and emphasis on developing efficient and eco-friendly ship recycling practices worldwide. This article presents a systematic literature review and bibliometric analysis of 228 studies on ship recycling indexed in Scopus. Employing the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) methodology for its robustness in comprehensive literature analysis, this review uncovers key insights into prominent countries, authors, journals, collaborations, topics, and historical trends in ship recycling research, thereby extending the scope of previous reviews. Notably, major contributions from Turkey, India, Bangladesh, the USA, and China focus on environmental impact studies, reflecting urgent global sustainability concerns. The review discusses commonly adopted methodologies such as Life Cycle Assessment and Elemental Analysis, shedding light on their application in this field. Through thematic analysis across 8 categories, future research pathways are identified, highlighting crucial areas such as continuous environmental monitoring, innovative renewable energy extraction from end-of-life vessels, and the need for human factors in ship recycling. This comprehensive synthesis of existing knowledge and identification of emergent research needs and opportunities serve as a foundational resource for impactful future research and informed policymaking, particularly in aligning with global environmental and sustainability goals. Researchers, policymakers and other stakeholders in maritime safety and environmental sustainability may find the knowledge gained from this systematic literature review insightful.

Chronic aryl hydrocarbon receptor activity impairs muscle mitochondrial function with tobacco smoking.

BACKGROUND: Accumulating evidence has demonstrated that chronic tobacco smoking directly contributes to skeletal muscle dysfunction independent of its pathological impact to the cardiorespiratory systems. The mechanisms underlying tobacco smoke toxicity in skeletal muscle are not fully resolved. In this study, the role of the aryl hydrocarbon receptor (AHR), a transcription factor known to be activated with tobacco smoke, was investigated. METHODS: AHR related gene (mRNA) expression was quantified in skeletal muscle from adult controls and patients with chronic obstructive pulmonary disease (COPD), as well as mice with and without cigarette smoke exposure. Utilizing both skeletal muscle-specific AHR knockout mice exposed to chronic repeated (5 days per week for 16 weeks) cigarette smoke and skeletal muscle-specific expression of a constitutively active mutant AHR in healthy mice, a battery of assessments interrogating muscle size, contractile function, mitochondrial energetics, and RNA sequencing were employed. RESULTS: Skeletal muscle from COPD patients (N = 79, age = 67.0 ± 8.4 years) had higher levels of AHR (P = 0.0451) and CYP1B1 (P < 0.0001) compared to healthy adult controls (N = 16, age = 66.5 ± 6.5 years). Mice exposed to cigarette smoke displayed higher expression of Ahr (P = 0.008), Cyp1b1 (P < 0.0001), and Cyp1a1 (P < 0.0001) in skeletal muscle compared to air controls. Cigarette smoke exposure was found to impair skeletal muscle mitochondrial oxidative phosphorylation by ~50% in littermate controls (Treatment effect, P < 0.001), which was attenuated by deletion of the AHR in muscle in male (P = 0.001), but not female, mice (P = 0.37), indicating there are sex-dependent pathological effects of smoking-induced AHR activation in skeletal muscle. Viral mediated expression of a constitutively active mutant AHR in the muscle of healthy mice recapitulated the effects of cigarette smoking by decreasing muscle mitochondrial oxidative phosphorylation by ~40% (P = 0.003). CONCLUSIONS: These findings provide evidence linking chronic AHR activation secondary to cigarette smoke exposure to skeletal muscle bioenergetic deficits in male, but not female, mice. AHR activation is a likely contributor to the decline in muscle oxidative capacity observed in smokers and AHR antagonism may provide a therapeutic avenue aimed to improve muscle function in COPD.

Recurrent Hyponatremia in the Setting of Autoimmune Disease with Sicca Syndrome: A Case Report.

Sjogren's syndrome is an autoimmune disease associated with xerostomia and xerophthalmia. The association of Sjogren's with hyponatremia has rarely been reported and has been attributed to syndrome of inappropriate antidiuretic hormone secretion. Here, we report a case of polydipsia secondary to xerostomia as a cause of chronic hyponatremia in the setting of Sjogren's syndrome. Analysis of the patient's medical record, including medication reconciliation and dietary habits, revealed several underlying causes of her recurrent hyponatremia. A thorough review of the patient's clinical history and good bedside examination may reduce prolonged hospitalizations and improve the quality of life of a hyponatremic population of patients who are predominantly elderly.

Asiatic acid attenuates infarct volume, mitochondrial dysfunction, and matrix metalloproteinase-9 induction after focal cerebral ischemia.

BACKGROUND AND PURPOSE: Asiatic acid (AA) has been shown to attenuate cerebral infarction in a mouse model of focal ischemia and shows promise as a neuroprotective stroke therapy. To facilitate translation of these findings to clinical studies, we determined pharmacokinetics, a dose-response relationship, the therapeutic time window, and efficacy using multiple stroke models. We also explored potential mechanisms of action. METHODS: Escalating doses of intravenous AA were administered and serum concentrations were measured at multiple time points for the pharmacokinetic studies. Subsequently, a dose-response relationship was determined followed by administration at different intervals after the onset of ischemia to establish a therapeutic time window for neuroprotection. Outcome measurements included both histological and behavioral. Mitochondrial function and matrix metalloproteinase activity in controls and treated rats were also determined. RESULTS: The pharmacokinetic studies showed that AA (75 mg/kg) has a half-life of 2.0 hours. AA significantly decreased infarct volume and improved neurological outcome even when administration was at time points up to 12 hours after the onset of ischemia. Infarct volume was also significantly decreased in female rats and spontaneously hypertensive rats. AA attenuated mitochondrial dysfunction and reduced matrix metalloproteinase-9 induction. CONCLUSIONS: Our study shows AA is effective against multiple models of focal ischemia, has a long therapeutic time window, and is also effective in females and hypertensive animals. AA may mediate neuroprotection by protecting mitochondria and inhibiting matrix metalloproteinase-9 induction and activation. Taken together these data suggest that AA is an excellent candidate for development as a stroke therapy.

'Pseudofailure' of spinal cord stimulation for neuropathic pain following a new severe noxious stimulus: learning points from a case series of failed spinal cord stimulation for complex regional pain syndrome and failed back surgery syndrome.

Failure of spinal cord stimulation (SCS) may be due to hardware problems, migration of electrodes and, in the long-term, plasticity in the spinal cord with habituation to the stimulation current. We describe a series of seven patients who experienced acute therapeutic loss of SCS effects following an acute nociceptive event unrelated to primary pathology. There were no hardware problems. We called this 'Pseudofailure', as the effective stimulation returned in all patients following a period off stimulation or reprogramming. This phenomenon has not been reported previously in the literature. Over a 4-year period, we managed seven patients with this feature: four had received SCS for complex regional pain syndrome and three for failed back surgery syndrome. In all seven cases, there was cessation of the pain relief afforded by SCS following an acute painful event: four patients had trauma, two patients had domestic electric shock and one patient suffered shingles (varicella zoster infection). We excluded hardware-related problems in all cases. In two patients, SCS effects could be regained by an initial attempt at reprogramming. In the remaining five cases reprogramming was unsuccessful, and stimulation was switched off for several months before recommencing, when we discovered a return of good therapeutic effect. We conclude that SCS may seem to fail following a separate strong nociceptive stimulus. Stimulation may be regained with reprogramming or following a period with stimulation switched off. We would, therefore, advise against removal of SCS hardware in the first instance.

Cervical implantation of intrathecal baclofen pump catheter in children with severe scoliosis.

OBJECT: Intrathecal baclofen (ITB) pump catheter placement is traditionally performed through entry into the spinal sac at the lumbar spine. A minority of children with cerebral palsy have severe concomitant neuromuscular scoliosis. In these children, whether surgically treated or not, access to the intradural space via the lumbar spine may prove technically challenging. The authors report on a series of children in whom, for various reasons, an ITB catheter was implanted using a posterior cervical spine approach. METHODS: The records of 20 children in whom a baclofen catheter had been placed were retrospectively reviewed to assess the demographic details, indications, and outcome of this procedure. RESULTS: This approach was successful in all but one of the children in whom the procedure was abandoned given the presence of significant extradural scar tissue. Of the 20 children, 7 had previously undergone lumbar ITB catheter implantation, although the catheter was subsequently, iatrogenically transected during scoliosis surgery. Nine children had had corrective scoliosis surgery, and the fusion mass obviated access to the lumbar spinal sac. Four children had untreated scoliosis and corrective surgery was being contemplated. Complications included infection requiring explantation (2 patients), catheter migration (1 patient), intolerance to ITB (1 patient), and failure of implantation (1 patient). All patients who tolerated the ITB experienced improvement in spasticity. No complications were associated with the spinal level of catheter insertion. CONCLUSIONS: Implantation of an ITB catheter via a cervical approach is safe and feasible and should be considered in children with severe corrected or uncorrected scoliosis, and thus avoiding the lumbar spinal sac.