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Objective: Intraventricular baclofen has been reported as an alternative to intrathecal baclofen for managing refractory spasticity and dystonia in some circumstances. In this report, we described a frame-based stereotactic approach for precisely positioning of the infusion catheter into the third ventricle., Material and Methods: High resolution contrast-enhanced anatomical MR sequences was acquired prior to surgery for pre-planning. Catheter trajectory was planned to pass from the lateral ventricle to the third ventricle through the Foramen of Monro. The surgical procedure was adapted from the frame-based deep brain stimulation surgery. The Touch-Loc kit (SceneRay, China) was used to temporarily secure the catheter in place during the surgery. From July 2022 to December 2022, three patients suffering from intractable spasticity and/or secondary dystonia received IVB successfully using the described technique., Results: No severe adverse events, including death, intracranial hemorrhage, infection, catheter migration or fracture, were documented at the last follow-up (range: 12‒24 months). Transient side effects included mild nausea following the initiation of infusion or the increase in infusion rate. All three patients responded to the IVB., Conclusions: The described frame-based stereotactic technique for IVB catheter implant is feasible and could be quickly mastered by neurosurgeons in related fields. Larger prospective cohorts with longer follow-up periods are necessary to further evaluate the long-term safety and efficacy of this procedure., (© 2024. The Author(s).)

BACKGROUND Hyperthermia is strongly associated with a worse neurological outcome in traumatic brain injury (TBI) and can be exacerbated by shivering. However, effective treatments for uncontrolled shivering have yet to be established. We successfully treated a patient with severe TBI and repeated episodes of shivering using dantrolene sodium hydrate (dantrolene). CASE REPORT A 28-year-old healthy male sustained an acute subdural hematoma with a midline shift following a traffic accident. He underwent emergency evacuation of the hematoma and craniectomy and was admitted to the Intensive Care Unit. The patient experienced severe shivering on the first day of hospitalization. Neuroprotective drugs were administered, and targeted temperature management at normothermia was initiated, initially controlling the shivering. On day 9 of hospitalization, shivering recurred, with a high fever. Despite the reintroduction of general anesthesia and targeted temperature management, the shivering was intractable. We administered dantrolene, which successfully eliminated the shivering. After the intervention, the patient's body temperature and intracranial pressure were well managed. The patient was subsequently transferred to a rehabilitation hospital, with a favorable neurological outcome 70 days after the injury. CONCLUSIONS This case report demonstrates that dantrolene is an effective option for managing uncontrollable shivering in the context of intracranial pressure control after severe brain injury. This finding suggests the potential for the broader use of dantrolene in similar clinical scenarios and supports further investigation of its efficacy and mechanisms of action in TBI care.

Introduction: Patients managed in the Pediatric Palliative Care Integral Unit (PPCIU) have serious neurological conditions that involve significant damage at central nervous system level. The movement disorder is a very common clinical problem and for the patients where an adequate control of muscle tone is not achieved with usual techniques or drugs, intrathecal baclofen therapy (IBT) should be considered., Materials and Methods: Descriptive retrospective study based on the review of clinical records of patients who received IBT being followed by the PPCIU of Madrid Autonomous Region in the timeframe between September 2012 and February 2021., Results: IBT was implanted in 8 patients affected by infantile cerebral palsy (ICP) with a Gross Motor Function Scale (GMFCS) IV-V, 3 patients was a Pantothenate kinase deficit-associated neurodegeneration (PKAN), 2 had Acquired Brain Damage, and the remaining 3 had, respectively, 2 glutaric aciduria type I (GA-1), and poly-malformative syndrome. In all patients we observed a period of clinical stability after IBT, we call this period "honeymoon". Two patients died while in the honeymoon period, at 24.9 and 19.6 months from implantation of the pump; the median of duration of the honeymoon period in the remaining 14 was 14.4 months (IQ: 8.3-25.8)., Conclusions: IBT was not only used in patients with non-progressive diseases, but also in the group of patients with neurodegenerative or progressive diseases. In all of them, after implantation of the device, we have objectified a period of clinical stability and a better control of muscle tone disorders., (Copyright © 2022 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.)

Paroxysmal positional nystagmus frequently occurs in lesions involving the cerebellum, and has been ascribed to disinhibition and enhanced canal signals during positioning due to cerebellar dysfunction. This study aims to elucidate the mechanism of central positional nystagmus (CPN) by determining the effects of baclofen on the intensity of paroxysmal positional downbeat nystagmus due to central lesions. Fifteen patients with paroxysmal downbeat CPN were subjected to manual straight head-hanging before administration of baclofen, while taking baclofen 30 mg per day for at least one week, and two weeks after discontinuation of baclofen. The maximum slow phase velocity (SPV) and time constant (TC) of the induced paroxysmal downbeat CPN were analyzed. The positional vertigo was evaluated using an 11-point numerical rating scale (0 to 10) in 9 patients. After treatment with baclofen, the median of the maximum SPV of paroxysmal downbeat CPN decreased from 30.1°/s [interquartile range (IQR) = 19.6-39.0°/s] to 15.2°/s (IQR = 11.2-22.0°/s, Wilcoxon signed rank test, p < 0.001) with the median decrement ratio at 40.2% (IQR = 28.2-50.6%). After discontinuation of baclofen, the maximum SPV re-increased to 24.6°/s (IQR = 13.1-34.4°/s, Wilcoxon signed rank test, p = 0.001) with the median increment ratio at 23.5% (IQR = 5.2-87.9%). In contrast, the TCs of paroxysmal downbeat CPN remained unchanged at approximately 3.0 s throughout the evaluation. The positional vertigo also decreased with the medication (Wilcoxon signed rank test, p = 0.020), and remained unchanged even after discontinuation of medication (Wilcoxon signed rank test, p = 0.737). The results of this study support the prior presumption that paroxysmal CPN is caused by enhanced responses of the semicircular canals during positioning due to cerebellar disinhibition. Baclofen may be tried in symptomatic patients with paroxysmal CPN., (© 2024. The Author(s).)

Competing Interests: Disclosures The authors have no conflicts of interest to disclose.

Importance: Stricter opioid prescribing guidelines have increased prescriptions of skeletal muscle relaxants (SMRs) for chronic pain, but the efficacy of long-term use of SMRs for chronic pain is unknown., Objective: To systematically review the effectiveness or efficacy of long-term use of SMRs for chronic pain., Evidence Review: Two reviewers systematically searched Ovid MEDLINE, Embase (Ovid), Web of Science, CINAHL, and Cochrane through December 4, 2023. They included articles published in English, Spanish, or Italian. Only randomized clinical trials (RCTs) and cohort studies with comparator groups evaluating at least 1-month duration of SMRs for chronic pain were included. The reviewers dually reviewed data abstraction, risk-of-bias, and quality. They characterized studies by chronic pain syndrome: low back pain, fibromyalgia, headaches, painful cramps or spasticity, and other syndromes., Findings: A total of 30 RCTs with 1314 participants and 14 cohort studies with 1168 participants assessed SMRs for chronic pain. Studies were primarily short-term (4-6 weeks). Nine unique SMRs were represented by the studies identified. Eleven studies (25%) examined baclofen, 8 (18%) examined tizanidine, and 7 (16%) examined cyclobenzaprine. Evidence for effectiveness was strongest for SMRs used for trigeminal neuralgia, neck pain, and painful cramps; evidence suggested SMRs for fibromyalgia, low back pain, and other syndromes were not more beneficial than placebo. The most common adverse effects were sedation and dry mouth. RCTs had a low to moderate risk of bias, and the quality of cohort studies was fair to good., Conclusions and Relevance: In this systematic review of long-term use of SMRs for chronic pain, findings suggest that their long-term use may benefit patients with painful spasms or cramps and neck pain; their long-term use for low back pain, fibromyalgia, and headaches did not appear to be beneficial. Clinicians should be vigilant for adverse effects and consider deprescribing if pain-related goals are not met.

Background: Cerebral palsy (CP) is the most cause of motor dysfunction in children. Selective dorsal rhizotomy (SDR) plays a major role in long term spasticity control. However, limited data exists on the effect of SDR on postoperative spasticity treatment requirements and supraspinal effects, and the stimulation responses of dorsal nerve roots in those with CP., Methods: The current study included the outcome for 35 individuals undergoing SDR for motor functional outcome, spasticity, baclofen dose changes, botulinum toxin injection frequency, and spasticity related orthopedic procedures. We also report on the stimulation responses in 112 individuals who underwent SDR at our institution., Results: There was a significant difference in gross motor function measures (GMFM)-66 scores at last follow up that remained present when considering only ambulatory children but not with non-ambulatory children. Ashworth scores were significantly decreased for both upper and lower extremities after SDR at all follow up points. There was a significant decrease in Baclofen dose and botulinum toxin injections requirements after SDR, but no significant difference in the need for orthopedic intervention. A total of 5502 dorsal nerve roots were tested showing a decrease in stimulation intensity and increase in grade on the right side and for descending lumbosacral levels., Conclusions: SDR improves gross motor scores during short term follow up but has additional benefits in decreasing baclofen dosing and botulinum toxin injections requirements after surgery. They stimulation responses of sectioned dorsal nerve roots adds to the limited available data and our understanding of the pathological changes that occur in CP., (© 2024. The Author(s).)

Malignant hyperthermia (MH) is a fatal hyperthermia with a high mortality, which usually occurs during induction of general anesthesia. Dantrolene sodium is a wonder drug currently used for treating malignant hyperthermia. However, preparing, storing, and maintaining dantrolene sodium are crucially expensive, thus making it financially unsatisfactory and difficult for clinicians to acquire in time. Monitoring patients' condition closely and intervening promptly when early signs of malignant hyperthermia occur can effectively prevent the condition from worsening and win over time for the arrival of dantraline sodium. This article is to report a case in which we successfully rescued a child occurring malignant hyperthermia without using dantrolene sodium., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 by Mutaz B. Habal, MD.)

Tetanus is a life-threatening, vaccine-preventable disease caused by an endotoxin produced by Clostridium tetani . We report a case of severe tetanus in an adult male with a history of intravenous drug use. The patient presented with a 1-day history of inability to open his jaw and a right lower extremity necrotic wound. Initial management consisted of tetanus toxoid, human tetanus immunoglobulin, antimicrobials and intermittent lorazepam. Due to progressive symptoms, wound debridement and placement of an advanced airway in the operating room ensued. Episodes of tetany were associated with fever, autonomic instability, acute desaturations and preemptive ventilator triggering despite maximum doses of continuous propofol and midazolam. Neuromuscular blockade with cisatracurium was added, resulting in control of tetany. Despite initial control, NMB could not be weaned due to recurrent spasms. Intravenous dantrolene was therefore sought as an alternative antispasmodic. Following an initial load, patient was successfully liberated from cisatracurium. Dantrolene was therefore converted to enteral to facilitate gradual down-titration of intravenous sedatives with subsequent conversion to oral benzodiazepines. After a prolonged hospital course, the patient was able to be discharged home. Dantrolene was thus effectively utilized as an adjunctive antispasmodic agent to facilitate liberation from cisatracurium and continuous sedation., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Traumatic brain injury (TBI) triggers a bevy of changes including mitochondrial dysfunction, apoptosis, oxidative stress, neurobehavioural impairment, and neuroinflammation, among others. Dantrolene (DNT), a muscle relaxant which inhibits intracellular Ca 2+ signaling from the ER, has been repurposed as a potential neuroprotective agent in various neurological diseases. However, there have been limited studies on whether it can mitigate TBI-induced deficits and restore impaired mitochondrial dynamics. This study sought to evaluate whether Dantrolene can potentially provide neuroprotection in an in vivo model of TBI. Male wistar rats subjected to TBI were treated with DNT (10 mg/kg) 1 h and 12 h post surgery. Animals were assessed 24 h post-TBI to evaluate neurobehavioural deficits and cerebral edema. We evaluated the protein expressions of apoptotic, autophagic, and neuroinflammatory markers by immunoblotting, as well as Mitochondrial Membrane Potential (MMP) and Reactive Oxygen Species (ROS) via Flow Cytometry to ascertain the effects of DNT on TBI. We further analysed immunofluorescence staining with Glial Fibrillary Acidic Protein (GFAP) and immunohistochemistry with NF-κβ to investigate neuroinflammation. H&E staining was also performed post-TBI. Our findings revealed DNT administration inhibits mitochondria-mediated apoptotis and reduces heightened oxidative stress. DNT treatment was also found to reverse neurobehavioural impairments and offer neuroprotection by preserving neuronal architechture. We also demonstrated that DNT inhibits neuronal autophagy and alleviates neuroinflammation following TBI by modulating the NF-κβ/Akt signaling pathway. Thus, our results suggest a novel application of DNT in ameliorating the multitude of deficits induced by TBI, thereby conferring neuroprotection., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: Literature is limited on functional outcomes in children with cerebral palsy (CP) following surgical procedures and a subsequent inpatient rehabilitation unit (IRU) stay., Objective: To compare functional outcomes and length of stay (LOS) in children with CP following a surgical procedure and IRU stay based on the surgical procedure performed, pattern of involvement, etiology, and Gross Motor Function Classification System (GMFCS) level., Design: Retrospective cohort study., Setting: Tertiary care pediatrics., Participants: Pediatric patients with CP who underwent one of three surgical procedures followed by an IRU stay., Interventions: Selective dorsal rhizotomy (SDR), single-event multilevel orthopedic surgery (SEMLS), or intrathecal baclofen (ITB) pump implantation and subsequent IRU stay., Main Outcome Measures: IRU LOS, Functional Independence Measure for Children (WeeFIM) total score, sub-scores, and efficiency., Results: Children undergoing SDR had a longer LOS (p ≤ .015). Children with spastic diplegia, GMFCS level II, and prematurity-based CP had higher WeeFIM efficiency scores (p ≤ .046, ≤.021, and .034 respectively). Greater changes in WeeFIM™ scores were associated with spastic diplegia, SDR, GMFCS level II, longer LOS, and higher admission scores (p ≤ .045)., Conclusions: Although statistically and functionally significant improvements in children with CP following surgical interventions and an IRU stay were seen, those with higher WeeFIM change scores tended to have spastic diplegia, to have undergone SDR, GMFCS level II, longer LOS, and higher admission scores., (© 2023 American Academy of Physical Medicine and Rehabilitation.)

Background: Spasticity can significantly affect a patient's quality of life, caregiver satisfaction, and the financial burden on the healthcare system. Baclofen is one of only a few options for treating spasticity. The purpose of this study is to investigate the impact of intrathecal baclofen (ITB) therapy on severe40.23 spasticity and motor function in patients with cerebral palsy., Methods: We conducted a systematic review in PubMed, Scopus, Ovid, and the Cochrane Library in accordance with the PRISMA guidelines. We included studies based on eligibility criteria that included desired participants (cerebral palsy patients with spasticity), interventions (intrathecal baclofen), and outcomes (the Ashworth scales and the Gross Motor Function Measure [GMFM]). The within-group Cohen's d standardized mean differences (SMD) were analyzed using the random effect model., Results: We screened 768 papers and included 19 in the severity of spasticity section and 6 in the motor function section. The pre-intervention average spasticity score (SD) was 3.2 (0.78), and the post-intervention average score (SD) was 1.9 (0.72), showing a 40.25% reduction. The SMD for spasticity reduction was - 1.7000 (95% CI [-2.1546; -1.2454], p-value < 0.0001), involving 343 patients with a weighted average age of 15.78 years and a weighted average baclofen dose of 289 µg/day. The SMD for the MAS and Ashworth Scale subgroups were - 1.7845 (95% CI [-2.8704; -0.6986]) and - 1.4837 (95% CI [-1.8585; -1.1088]), respectively. We found no relationship between the participants' mean age, baclofen dose, measurement time, and the results. The pre-intervention average GMFM (SD) was 40.03 (26.01), and the post-intervention average score (SD) was 43.88 (26.18), showing a 9.62% increase. The SMD for motor function using GMFM was 0.1503 (95% CI [0.0784; 0.2223], p-value = 0.0030), involving 117 patients with a weighted average age of 13.63 and a weighted average baclofen dose of 203 µg/day. In 501 ITB implantations, 203 medical complications were reported, including six new-onset seizures (2.96% of medical complications), seven increased seizure frequency (3.45%), 33 infections (16.26%), eight meningitis (3.94%), and 16 cerebrospinal fluid leaks (7.88%). Delivery system complications, including 75 catheter and pump complications, were also reported., Conclusion: Despite the risk of complications, ITB has a significant impact on the reduction of spasticity. A small but statistically significant improvement in motor function was also noted in a group of patients., (© 2024. The Author(s).)

Dystonia represents a significant source of disability in children. Generalized dystonia, which involves multiple body regions, leads to impaired mobility and motor function, resulting in substantial challenges in daily activities. Surgical treatments are used when medical treatments fail. Intrathecal baclofen (ITB) or deep brain stimulations (DBS) are the most employed surgical therapies. When these options are not feasible or ineffective, some authors have explored the use of intraventricular baclofen (IVB). In this report, we present four cases of pediatric patients with generalized dystonia who underwent treatment with IVB, resulting in notable improvements. To further explore the potential of this treatment modality, we conducted a comprehensive literature review. The findings from our study provide a comprehensive overview that can guide palliative management in similar cases., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

This systematic review aims to evaluate the use of intrathecal baclofen (ITB) for hereditary spastic paraparesis (HSP) treatment. An extensive search in two electronical databases was performed. We identified articles published between 1990 and 2022 (PubMed, Scopus), and applied the following inclusion criteria: diagnosis of HSP at the time of the intervention, either familial or sporadic; report on the effect of ITB in patients with HSP; test trial via either bolus injections or continuous infusion tests; and ITB pump implantation. A data extraction sheet based on the Cochrane Consumers and Communication Review Group's data extraction template was created and adapted to collect relevant data. A qualitative analysis was performed to present the results in narrative summary fashion. A total of 6 studies met our inclusion criteria. 51 patients with HSP had a pre-implantation ITB trial. The time since the diagnosis until the pump implantation ranged from 5 to 30 years. The initial bolus ranged from 20 to 50 μg and the mean doses used at steady state ranged from 65 to 705 μg. An improvement in spasticity was observed on the modified Ashworth Scale in patients treated with ITB. Although all studies reported a subjective gait improvement, not all found an objective improvement in gait. The most common side effect reported was catheter-related problems. The findings of this review support the use of ITB as an effective and a viable option for the treatment of spasticity in HSP refractory to conservative therapies., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To analyze the effectiveness and tolerability of various regimens for the use of tolperisone hydrochloride (TH) in the treatment of patients with acute nonspecific lower back pain (ALBP)., Material and Methods: 90 patients with ALBP (54.3 [48.7; 61.3] years) were divided into 2 groups: group 1 ( n =45) received basic therapy - NSAIDs, B vitamins and TH sustained release (450 mg/day per os ), group 2 ( n =45) used basic treatment and TH 2 ml intramuscularly 2 times a day for 5 days with a transition to a TH sustained release 450 mg/day per os . The total duration of the study was 30 days. We analyzed the visual analogue pain scale (VAS-B), the results of the modified modified Schober test (MMST), the medication adherence scale (Drug Attitude Inventory - DAI-10), the Epworth daytime sleepiness scale (ESS), functional activity associated with low back pain (Roland-Maurice questionnaire, RM), duration of TH intake (days), frequency and characteristics of adverse events (AE). At the screening visit, all patients were additionally divided depending on the intensity of pain according to VAS-B («mild», «moderate», «severe»)., Results: There were no significant differences on the VAS-B scale between groups 1 and 2 in patients with mild pain; there was significantly more complete pain control in group 2 with moderate pain after 14 and 30 days, ( p =0.0013, 14 days; p =0.0021, 30 days); among patients with severe pain, the best result after 30 days was observed in group 2 ( p =0.0016). In all patients the range of motion in the lumbosacral spine (MSST) significantly increased and functional activity (RM) improved compared to the initial state: more significant in group 2 with moderate pain after 14 and 30 days and with severe pain after 30 days. There was a high adherence to therapy (DAI-10), more pronounced in the first 14 days in patients of group 1. Excessive daytime sleepiness (ESS) was not observed. For moderate pain, patients in group 2 used TH for a significantly shorter period of time (16.4±1.6 days) compared to patients in group 1 (19.6±2.2 days, p =0.0022). AE were mild and did not serve as a reason to refuse therapy., Conclusion: A protocol for prescribing TH was proposed, based on stratification of patients according to VAS-B.The use of TH for ALBP is effective and safe. The proposed protocol for stratifying patients according to VAS-B level allows identifying the patients with step-by-step scheme of prescribing the drug for a period of 14 to 30 days.

Purpose: The use of intrathecal medications for the management of spasticity and various pain syndromes in the adult population has been previously reported. However, no evidence-based guidelines currently exist in the pediatric population. This case series presents patients (n = 8) with pediatric-onset disability who underwent placement of intrathecal baclofen pumps initially for management of severe spasticity. Despite titration of dose and use of oral analgesia, their uncontrolled pain persisted. Each patient was transitioned to a combination of baclofen and analgesic intrathecal therapy. The outcome in pain improvement and quality of life, as reported by patients and/or caregivers, were retrospectively reviewed., Methods: Retrospective review of the background and decision-making process regarding transition to combination intrathecal therapy identified patient selection characteristics. Each patient and/or their caregivers completed a survey regarding improvements in pain, spasticity, function, and quality of life following initiation of combination intrathecal medications., Results: Survey results revealed improvements in functional and pain assessments after initiation of combination baclofen and analgesic intrathecal medication. Patients and caregivers reported decreases in pain and oral spasticity medications., Conclusion: Use of pumps with antispasmodic and analgesic medication for combination intrathecal medication delivery should be considered in the management of patients with childhood-onset disabilities who have both severe spasticity and pain.

Eperisone Hydrochloride was launched in Japan in 1983 and has been used to improve muscle tone and treat spastic paralysis (Originator: Eisai Co., Ltd.). However, its biochemical mechanism of action is unknown. SB Drug Discovery was used to evaluate purinergic P2X (P2X) receptor antagonism using fluorescence. In this study, we discovered that its target protein is the P2X7 receptor. Also, P2X receptor subtype selectivity was high. This finding demonstrates the (Eperisone-P2X7-pain linkage), the validity of P2X7 as a drug target, and the possibility of drug repositioning of Eperisone Hydrochloride.

Background: Cerebral Palsy (CP) is a group of permanent, but not unchanging, disorders of movement and/or posture and motor function, which are due to a non-progressive interference, lesion, or abnormality of the developing/immature brain. One clinical presentation is muscle spasticity, which leads to a significant impact on the individual's functionality and quality of life. Spasticity treatment is multidisciplinary and includes pharmacological and physical intervention; intrathecal baclofen shows a positive effect in severe spasticity and suboptimal response to oral drugs, while local injection of Botulinum toxin type A (BTXA) improves muscle tone, motion and pain., Objective: The aim of this study was to evaluate the efficacy of the combined intrathecal baclofen infusion (ITB) - botulinum toxin treatment in the management of spasticity in CP., Methods: 8 patients with spastic tetraparesis were enrolled. All patients were treated with intrathecal Baclofen; in lower limbs, no spastic symptoms appeared, while marked spasticity was noted in upper limbs. We injected the right and left Biceps Brachial (BB) and Flexor Digitorum Superficialis (FDS) muscles with botulinum toxin type A. All patients underwent Myometric measurement, Ashworth Scale, Numerical Rating Scale, and Visual Analogic Scale evaluation before infiltration (T0), 30 days after injection (T1), 60 days after injection (T2), and 90 days after treatment (T3)., Results: All data demonstrated an improvement in spasticity, pain, quality of life, and self-care during the study, with p < 0.05. No side effects appeared., Conclusion: This study demonstrated the efficacy and safety of intrathecal baclofen infusion and botulinum toxin combined treatment in the management of spasticity, pain, quality of life, and selfcare in CP patients., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Abstract: We present the case of a posttraumatic C6 AIS A tetraplegic patient with spasticity treated with an intrathecal baclofen pump, who noticed a transient increase in his spasticity each time he used a digital tablet (Ipad) protected by a magnetic shell placed on his abdomen. Telemetry confirmed transient motor shutdown responsible for withdrawal symptoms each time the tablet was used. Symptoms resolved after the removal of the protective shell. Effects of magnetic fields like magnetic resonance imaging are known to stall the pump rotor, which recover at the end of magnetic resonance imaging. Other sources of magnetic fields like laptops or new smartphones with magnet charging technology may also interfere with implanted devices. We therefore recommend patients to avoid close contact of magnetic devices with the intrathecal baclofen pump. More robust studies are warranted to assess the effect of the new magnetic technologies on the function of intrathecal pumps., Competing Interests: Financial disclosure statements have been obtained, and no conflicts of interest have been reported by the authors or by any individuals in control of the content of this article., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

BACKGROUND AND IMPORTANCE: Intrathecal baclofen (ITB) pumps are an effective management tool for spasticity and dystonia. In the pediatric population, ITB complications including infection are common, and strategies to minimize their impact are important. Current standard of care for pump infection involves a wean of the ITB therapy followed by explantation of the hardware, which can be a three to 6-month process. CLINICAL PRESENTATION: We present a 20-year-old female who developed an pump pocket infection after revision. The pump was removed from the body but left connected. The pocket was debrided, thoroughly washed, and packed with multiple microbicidal agents. Nine days later, a new pump and proximal catheter was re-implanted without complications or interruption of medication administered intrathecally. One year later, the patient is doing well without any infectious problems. CONCLUSION: To our knowledge, this is the first report of an externalized pump and expedited reimplantation without a break in ITB therapy. Further studies are required in order to best characterize this technique as a potential alternative for infected baclofen pump pockets., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Intrathecal baclofen (ITB) therapy is an effective method of treating spasticity in persons with spasticity due to spinal cord injury (SCI), but complications are not rare and can include spinal fluid leaks, infection, and catheter/pump malfunction., Case Presentation: This study presents information related to an adult male patient with traumatic SCI and a history of two prior ITB pump pocket infections that required removal due to pump infection. The patient then developed skin erosion over the third pump, and the fluid around the pump grew methicillin-sensitive Staphylococcus aureus, diphtheroids, and Candida parapsilosis. The patient was initially treated with antibiotics and anti-fungal medication without removal of the ITB pump. The ITB pump was eventually removed 27 months later, and the fourth pump was implanted 10 months later., Discussion: ITB pumps can be an effective treatment modality for spasticity in people with SCI; however, complications, including infection, can occur and require pump removal. This case illustrates a case of possible Candida colonization of the ITB pump, which was eventually removed., (© 2023. The Author(s).)

Background: The activities related to intrathecal baclofen (ITB) therapy could not be interrupted at the outbreak of COVID-19 pandemic due to possible life-threatening related complications such as withdrawal and over dosage syndromes. In this study we reported the different adopted strategies to manage patients with an ITB implanted infusion pump during the pandemic period and studied the impact of these strategies on experiences reported from patients and their caregivers, assessed through a specific survey., Methods: Thirty-five patients (mean age: 43.71±12.33 years) were included. Their clinical and medical data were recorded and observed from March 2020 to March 2021 and different strategies implemented in order to limit patients and providers risk of exposure to COVID-19. The impact of these strategies was assessed trough a survey that was performed after the first two months of pandemic (coinciding with the period of general lockdown) and after one year from the pandemic onset., Results: We observed a statistically significant improvement of the following items: difficulties in reaching medical clinic (P=0.0072), continuation of physical therapy (P=0.0021) and feelings of anxiety in medical conditions (P=0.0006). Considering the difficulties in communications with the medical staff we obtained optimal scores both at the beginning of pandemic and after one year from the pandemic onset without significant difference., Conclusions: Our survey showed that the adopted strategies provided a feeling of confidence and safety among ITB patients and their caregivers during the COVID-19 pandemic. We think that a clear communication is always of paramount importance to manage these patients.

Our objective was to determine which pendulum test parameters are useful for detecting hypertonia in the knee muscles and assessing the group and individual responses to intrathecal baclofen (ITB) bolus injection among prospective pump recipients. We included 15 neurological patients with lower limb hypertonia (mainly spinal cord injury, n  = 7) and collected data the day before (baseline), and 2.5 and 5.0 h after the 50-µg ITB bolus injection. For comparison, data were collected in 15 healthy controls. The average over six test repetitions was obtained for the number of oscillations, swing time (SwingT), amplitudes of the first flexion and extension, maximum angular velocities of the first flexion (F1V) and extension (E1V), relaxation index, and damping coefficient (DampC). Across the patient group, all pendulum parameters indicated a significant decrease in hypertonia from baseline to postinjection (analysis of variance P  ≤ 0.004), except DampC. On the basis of the cutoffs from the receiver operating characteristic curve, all parameters were good or excellent discriminators of hypertonia in patients from normotonia in controls (area under the curve ≥0.85), with the highest sensitivity for SwingT and E1V (≥93%). Furthermore, all parameters except F1V revealed a significant shift from preinjection hypertonia to postinjection normotonia among patients (McNamar test P  ≤ 0.002, DampC excluded due to missing data), with the greatest responsiveness for E1V and relaxation index (≥73%). The results confirm the overall usefulness of pendulum test parameters in this patient population and indicate that some parameters are better at detecting hypertonia (SwingT, E1V) whereas others (E1V, relaxation index) are more responsive to the ITB injection., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Elevated tone (hypertonia) is a common problem in children with physical disabilities. Medications intended to reduce tone often have limited efficacy, with use further limited by a significant side effect profile. Consequently, there has been growing interest in the application of Neurosurgical Interventions for the Management of Posture and Tone (NIMPTs). Three main procedures are now commonly used: selective dorsal rhizotomy (SDR), intrathecal baclofen (ITB) and deep brain stimulation (DBS). This review compares these interventions, along with discussion on the potential role of lesioning surgery. These interventions variably target spasticity and dystonia, acting at different points in the distributed motor network. SDR, an intervention for reducing spasticity, is most widely used in carefully selected ambulant children with cerebral palsy. ITB is more commonly used for children with more severe disability, typically non-ambulant, and can improve both dystonia and spasticity. DBS is an intervention which may improve dystonia. In children with certain forms of genetic dystonia DBS may dramatically improve dystonia. For other causes of dystonia, and in particular dystonia due to acquired brain injury, improvements following surgery are more modest and variable. These three interventions vary in terms of their side-effect profile and reversibility. There are currently populations of children for who it is unclear which intervention should be considered (SDR vs ITB, or ITB vs DBS). Concerns have been raised as to the equity of access to NIMPTs for children across the UK, and whether the number of surgeries performed each year meets the clinical need., Competing Interests: Competing interests: DEL has received unrestricted educational grants from Medtronic to support attendance at scientific conferences., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Glutaric aciduria type 1 (GA1) is an autosomal recessive disease frequently leading to dystonia. Deep brain stimulation (DBS), intrathecal baclofen (ITB), and intraventricular baclofen (IVB) are the current interventional treatment options for refractory dystonia. We performed a scoping review, individual patient data (IPD) analysis, and clinical trials review to summarize the existing literature on these interventions in this population, characterize outcomes, and suggest directions for future investigation. PubMed, Embase, and Scopus were searched following PRISMA guidelines. IPD were extracted from studies providing IPD for GA1 patients. ClinicalTrials.gov was reviewed. Of 139 articles, 7 studies with 10 patients were included. In study-level data, 2/4 (50.0%) DBS studies found no improvement in dystonia and 3/3 (100%) on baclofen found decreased dystonia and enteral medication regimen. In the IPD analysis, four studies with 5 patients (2 IVB, 2 DBS, 1 ITB) were included. The average percent reduction in dystonia was 29.9% ± 32.5% (median:18%, IQR:18%-29.2%). Function improved in 4 (80.0%) patients. All patients with reported changes in enteral dystonia-related medication regimen (3/3, 100%) reported reduction in medication usage. No patients (0%) had perioperative complications. Mean follow-up length was 14.8 ± 12.2 months. No interventional clinical trials were found. ITB, IVB, and DBS represent present neuromodulatory approaches for the treatment of GA1. ITB and IVB reduce dystonia, while DBS has a heterogeneous effect. ITB and IVB improved function and reduced enteral medication regimens. These findings must be viewed with caution considering limited data and a serious risk of bias. Further large-scale studies are necessary to determine indications for ITB, IVB, and DBS and elucidate treatment algorithms., (© 2023 SSIEM.)

Objective: Patients requiring intrathecal baclofen (ITB) therapy are at high risk for surgical site infections (SSIs) given their poor functional status. After years of a nominal infection rate, there was an inexplicable increase in ITB pump infections at the authors' institution and multiple investigations offered no solution. Use of intraoperative topical antibiotics is well-documented in the orthopedic literature and was considered for ITB pump insertion. In this study, the authors investigated whether intraoperative vancomycin and tobramycin powder at the ITB pump site could reduce SSIs., Methods: Operative and infection data were collected and analyzed retrospectively to determine the efficacy of this change. Patients were stratified into three cohorts (1998-2009, 2010-2012, and 2013-2021) to better understand the trends before and after implementation of intraoperative topical antibiotics. Each cohort had similar demographics., Results: One hundred fifty-four patients underwent 272 ITB pump procedures between 1998 and 2021 (131 in 1998-2009, 49 in 2010-2012, and 92 in 2013-2021) for cerebral palsy (69.5%), spastic quadriparesis due to traumatic brain injury (7.1%), anoxic brain injury (6.5%), and other causes (16.9%). Infection rates were reduced from a high of 32% in 2010-2011 to 3.8% over the last 2.5 years (p = 0.0094). There were no adverse effects from the use of topical antibiotics., Conclusions: In the setting of an intractable rise in ITB pump infections, the addition of intraoperative topical antibiotics significantly reduced postoperative infections in a high-risk population. One could appreciate a significant drop each year in the rate of infections after the institution of intraoperative topical antibiotics. The reduction in SSIs significantly improved the long-term outcomes for these patients.

Symptomatic treatment of Multiple Sclerosis (MS) is crucial, since it helps to lessen the limitations that affect patients' daily lives. Tremors are a significant and frequent symptom in MS patients. However, there is still a lack of evidence supporting a specific therapeutic approach for MS patients' tremors. A 41-year-old man with a history of MS is presented in this study. He exhibited stiffness and tremors at the follow-up clinic, which affected his daily activities. For his spasticity, he received intrathecal baclofen (ITB). The patient's symptoms responded well to this treatment, as both stiffness and tremors decreased. In an MS patient, ITB therapy thus unexpectedly reduced and improved tremor symptoms., (Copyright: © Neurosciences.)

Objective: Intrathecal baclofen (ITB) is an effective treatment for lower limb spasticity. In ambulatory patients with spasticity, there is a justifiable concern that ITB treatment may compromise ambulatory function by reducing the heightened muscle tone, thereby unmasking underlying muscle weakness. ITB is hence offered with reservation in ambulant patients. In this article, we review the literature surrounding the effect of ITB therapy on ambulatory function in patients with concurrent spasticity and discuss the key findings., Data Sources: A literature search of ProQuest Medline and EBSCO CINAHL databases was performed., Review Method: Inclusion criteria included (a) studies reporting the effect of ITB in adult ambulatory patients; (b) studies with an intervention of screening test trial via either bolus injections or continuous infusion tests; and (c) studies with an intervention of ITB pump implantation. Seventeen eligible studies were identified and two authors independently assessed the study quality using the risk of bias in nonrandomised studies of interventions tool (ROBINS-I)., Results: Seventeen studies were included, with a total of 534 participants. Most of the patients remain ambulatory after ITB treatment, accompanied by improvements in gait speed and reduction in spasticity., Conclusion: ITB therapy when administered in carefully selected ambulatory patients with spasticity is not associated with loss of ambulatory function.

Purpose: To assess attainment of individual treatment goals one year after intrathecal baclofen (ITB) pump implantation in individuals with dyskinetic cerebral palsy (CP)., Materials and Methods: A multi-center prospective cohort study was conducted including 34 non-walking individuals with severe dyskinetic CP, classified as Gross Motor Function Classification System (GMFCS) IV/V, aged 4-24 years, 12 months after pump implantation. The main outcome measure was Goal Attainment Scaling (GAS). Predictors of GAS results were analyzed. Complications were registered systematically., Results: Seventy-one percent of individuals with dyskinetic CP fully achieved one or more treatment goals. One or more treatment goals were partially achieved in 97% of individuals. Two factors were found to be associated with attainment of goals: Dyskinesia Impairment Scale (DIS) score at baseline and the difference in pain score between baseline and follow-up. These two variables explain 30% of the variance in the outcome., Conclusions: Intrathecal baclofen is effective in achieving individual treatment goals in children and young adults with dyskinetic CP after nine to 12 months of ITB treatment. A positive outcome on treatment goals is, for a small part, associated with higher severity of dystonia at baseline and with improvement of pain during treatment., Clinical Trial Registration Number: Dutch Trial Register, number NTR3642.Implications for rehabilitationIntrathecal baclofen treatment is effective in attainment of personal treatment goals, one year after pump implantation in patients with dyskinetic cerebral palsy.A positive outcome on treatment goals is, for a small part, related to higher severity of dystonia at the start and on improvement of pain during treatment.

Objective: To investigate patients' expectations, met/unmet expectations and satisfaction with intrathecal baclofen treatment in relation to effect on spasticity, pain intensity, sleep quality, occupational performance, well-being and self-efficacy., Design: A prospective longitudinal study with follow-up at 1 year., Patients: Consecutive patients, age ≥ 18 years with a disabling spasticity of cerebral or spinal origin selected for intrathecal baclofen treatment at 2 university hospitals in Sweden were included. From August 2016 to June 2019, 35 patients began intrathecal baclofen treatment; 29 patients were included and completed the study., Methods: Baseline and 1-year follow-up included assessment of spasticity by physiotherapists, a semi-structured interview regarding occupational performance using the Canadian Occupational Performance Measure and a questionnaire., Results: Overall satisfaction with treatment and satisfaction with occupational performance were reported as moderate. Important variables that explained satisfaction with occupational performance were improvements in performance, expectations and performance before treatment. Patients had higher expectations compared with the 1-year outcomes regarding occupational performance, spasticity, pain intensity and sleep quality, although improvements were reported., Conclusion: A thorough discussion of goal setting with intrathecal baclofen treatment before implantation is necessary to give patients individual and realistic expectations.

Purpose: The goal of this retrospective chart review study was to explore factors that contributed to consideration of or actual pump explantation in pediatric patients with intrathecal baclofen (ITB) pumps., Methods: Medical records of 30 patients with ITB pumps were reviewed. Quantitative data, including demographic, clinical, psychosocial, and service utilization variables were culled from the records. Qualitative data were collected from clinic visit notes, pump-related follow-up phone calls, and any pump-related emergency room visits., Results: Of those reviewed, six underwent ITB pump explantation, and two considered explantation. Factors contributing to pump explantation or consideration of explantation included the following: postoperative infection, pump malfunction, non-adherence, anxiety/behavioral factors impacting the patient's tolerance of the pump, distance to the medical provider, frequency of required pump refill appointments, lack or perceived lack of intrathecal baclofen effect, and difficulty transitioning to adult care providers., Conclusion: Due to the complex care regimen associated with ITB pumps and various psychosocial and logistical factors that impact treatment success, a standardized multidisciplinary pre-implantation education, screening, and assessment process should be developed. Such a process would ensure that patients/families receive appropriate education, including proactively identifying treatment barriers and potential complications, possibly minimizing dissatisfaction with treatment and the need for explantation.

Baclofen is often considered a first-line treatment option for spasticity management in children. However, adverse effects, administration, and dosing can be barriers to effectiveness. In my practice, other medications for spasticity management are often used prior to initiating baclofen. In this article, baclofen use for spasticity management in children is briefly reviewed along with discussion of approaches using other medications as first-line treatment options. I will present a rationale for medication selection for spasticity management and discuss the approaches I take in medication selection that incorporate spasticity severity, patient goals, and medication side effect profiles.

We describe a unique case of intrathecal baclofen overdose mimicking brainstem death, during bilateral anterior cingulate cortex deep brain stimulation (DBS) for pain. A 37-year-old man with chronic regional pain syndrome requiring an intrathecal baclofen pump underwent DBS under general anaesthesia and experienced an intraoperative generalised tonic-clonic seizure on dural opening. Once the operation was completed, the patient was noted to have fixed, dilated pupils bilaterally and was transferred for an emergency computed tomography scan of the head, which did not reveal any acute intracranial pathology. The patient was transferred to the intensive care unit for management of concurrent hypotension, bradycardia and supportive management of his low Glasgow Coma Scale (GCS) score. A trial of atropine to counter the bradycardia was unsuccessful. Intrathecal baclofen toxicity was suspected as a diagnosis of exclusion, necessitating urgent aspiration of the baclofen pump. The patient's GCS score improved after pump aspiration and he was discharged home several days later. It was noted that the intrathecal baclofen pump had been refilled several days previously and the patient had reported intermittent episodes of somnolence. In perioperative patients with intrathecal baclofen pumps in situ, baclofen toxicity should always be considered as a differential in perioperative complications, even if it is considered a rare event.

Background: The intrathecal baclofen (ITB) pump is an effective treatment for patients with spasticity unresponsive to oral medication., Case Report: A 31-year-old woman with spinal cord injury sequelae was admitted to the emergency department due to sudden headache and excessive confusion. The patient was on ITB for lower limb spasticity. On presentation, her vital signs revealed blood pressure of 171/106 mm Hg, heart rate of 141 beats/min, and temperature of 39.0°C. Antibiotics were promptly administered intravenously for suspicion of bacterial meningitis. Based on magnetic resonance imaging and cerebrospinal fluid findings, as well as clinical signs such as marked lower limb spasticity, the final diagnosis was determined to be baclofen withdrawal syndrome complicated by reversible posterior leukoencephalopathy syndrome (RPLS). Improved consciousness was quickly achieved on blood pressure control. Resolution of spasticity was achieved after intravenous midazolam and intrathecal baclofen via lumbar puncture. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Baclofen withdrawal syndrome is the leading differential diagnosis for impaired consciousness and fever among patients using ITB pumps. Moreover, baclofen withdrawal syndrome mimics a wide variety of conditions, including sepsis. This unique case was complicated by intrathecal baclofen withdrawal syndrome coexisting with RPLS, thus presenting more similarly to bacterial meningitis than the usual baclofen withdrawal, due to marked headache, altered consciousness, and generalized convulsive symptoms. In addition to general withdrawal management, commencement of antihypertensive therapy for RPLS may have an immediate effect on the improvement of impaired consciousness., (Copyright © 2022. Published by Elsevier Inc.)

Spinal cord injury (SCI) affects an estimated three million persons worldwide, with ∼180,000 new cases reported each year leading to severe motor and sensory functional impairments that affect personal and social behaviors. To date, no effective treatment has been made available to promote neurological recovery after SCI. Deficits in motor function is the most visible consequence of SCI; however, other secondary complications produce a significant impact on the welfare of patients with SCI. Spasticity is a neurological impairment that affects the control of muscle tone as a consequence of an insult, trauma, or injury to the central nervous system, such as SCI. The management of spasticity can be achieved through the combination of both nonpharmacological and pharmacological approaches. Baclofen is the most effective drug for spasticity treatment, and it can be administered both orally and intrathecally, depending on spasticity location and severity. Interestingly, recent data are revealing that baclofen can also play a role in neuroprotection after SCI. This new function of baclofen in the SCI scope is promising for the prospect of developing new pharmacological strategies to promote functional recovery in patients with SCI.

Objective: To develop an algorithm for the selection of adults with disabling spasticity for treatment with intrathecal baclofen (ITB) and/or botulinum toxin type A (BoNT A)., Methods: A European Advisory Board of 4 neurologists and 4 rehabilitation specialists performed a literature review on ITB and BoNT A treatment for disabling spasticity. An online survey was sent to 125 physicians and 13 non-physician spasticity experts. Information on their current clinical practice and level of agreement on proposed selection criteria was used to inform algorithm design. Consensus was considered reached when ≥75% of respondents agreed or were neutral., Results: A total of 79 experts from 17 countries completed the on-line survey (57%). Agreement was reached that patients with multi-segmental or generalized disabling spasticity refractory to oral drugs are the best candidates for ITB (96.1% consensus), while those with focal/segmental disabling spasticity are ideal candidates for BoNT A (98.7% consensus). In addition the following are good candidates for ITB (% consensus): bilateral disabling spasticity affecting lower limbs only (97.4%), bilateral (100%) or unilateral (90.9%) disabling spasticity affecting lower limbs and trunk, and unilateral or bilateral disabling spasticity affecting upper and lower extremities (96.1%)., Conclusion: This algorithm will support the management of adult patients with disabling spasticity by aiding patient selection for ITB and/or BoNT A treatments.

Purpose: The aim of the study was to evaluate the use of a novel technique for baclofen delivery using an intrathecal catheter inserted through a lumbar laminotomy with the tip placed at the cisterna magna (supraspinal baclofen (SSB)) for the treatment of severe generalized secondary dystonia., Methods: A cohort study of six individuals (4M/2F, mean±SD = 15±4.86 years) with generalized dystonia unresponsive to oral medications were treated with SSB and followed clinically for 9 years. Intrathecal catheter tips were positioned under fluoroscopic guidance just above the level of the foramen magnum, at the cisterna magna., Results: Five of the 6 patients experienced sustained benefit with SSB; the group mean modified Fahn-Marsden scale scores decreased from 95 to 55 (t = 3.29, p = 0.02). One patient suffered complex pump pocket infection, and therefore underwent pump explantation., Conclusion: Supraspinal baclofen may be an effective method for infusing baclofen into the ventricular system of the brain for treatment of secondary dystonia which is unresponsive to oral therapy.

Purpose: The purpose of this study is to describe the efficacy and adverse events of neuraxial anesthesia for post-operative pain control in non-ambulatory children with cerebral palsy with pre-existing intrathecal baclofen (ITB) pumps undergoing hip reconstructive or palliative surgery., Methods: Twelve children (mean age 11.25 years) were included in the study with the following neuraxial anesthesia methods: indwelling epidural catheter (8 patients), neuraxial opioids administered through the side port of the ITB pump (3 patients), and single injection spinal anesthetic (1 patient). Observational pain scores and opioid requirements were quantified for all patients., Results: There were no ITB pump or surgical complications at a mean follow-up of 2.2 years. The average length of stay was 6 days. Patients had good post-operative pain control with a mean observational pain score of 0.7 and mean morphine equivalent use of 0.26mg/kg/day. Four patients required anti-emetics to control nausea and three patients had urinary retention requiring repeat catheterization, but all medical complications resolved prior to discharge., Conclusion: Neuraxial anesthesia can effectively control post-operative pain in children with a pre-existing ITB pump. Utilizing the side port of the ITB pump for administration of neuraxial opioids is an option when epidural or spinal anesthesia is not possible.

Tolperisone is a nonopioid, centrally acting muscle relaxant in clinical development in the USA for the treatment of symptoms associated with acute, painful muscles spasms of the back. CLN-301, RESUME-1, is a 14-day double-blind, randomized, placebo-controlled, parallel-group Phase III study of the efficacy and safety of tolperisone administered orally three-times daily in 1000 male and female subjects at approximately 70 clinical sites in the USA experiencing back pain due to or associated with muscle spasm of acute onset. Tolperisone is a promising therapeutic for managing acute, painful muscle spasms of the back as it appears to lack the off-target CNS effects often seen with conventional skeletal muscle relaxants. Clinical Trials registration number: NCT04671082 .