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Disability and Rehabilitation Jun 2024To understand the expectations and demand for a movement-tracking videogame (Bootle Blast) for home-based, upper limb (UL) rehabilitation among Costa Rican children with...
PURPOSE
To understand the expectations and demand for a movement-tracking videogame (Bootle Blast) for home-based, upper limb (UL) rehabilitation among Costa Rican children with cerebral palsy (CP).
METHODS
Data were collected via telephone screening () and child-parent dyads Zoom interviews (). Descriptive statistics and data transformation were used to report on success criteria (i.e., recruitment rate, having an appropriate screen and space to play, setting a weekly play time goal (PTG) ≥45 min, identifying one UL therapy goal). The DEPICT model for collaborative qualitative analysis was used in the thematic analysis of interview data.
RESULTS
Fifteen dyads participated (1.6 ± 1 recruited/month). All had a flat-screen TV in a suitable location to play, were able to set a UL therapy goal, and established PTGs ranging from 45-120 min per week. Identified themes were: 1) Socio-cultural factors heighten demand, 2) Feelings of hope prevail for the intervention, and 3) Collaborative goal setting supports realistic expectations for Bootle Blast.
CONCLUSIONS
Dyads had positive and realistic expectations about implementing the proposed videogaming intervention. This study provides insights on tailoring a family-centered, therapy gaming intervention to improve access to motor rehabilitation for children with CP in rural/remote settings and low-middle income countries.
PubMed: 38946018
DOI: 10.1080/09638288.2024.2362952 -
Brain and Behavior Jul 2024Semantic fluency is the ability to name items from a given category within a limited time, which relies on semantic knowledge, working memory, and executive function....
INTRODUCTION
Semantic fluency is the ability to name items from a given category within a limited time, which relies on semantic knowledge, working memory, and executive function. Similar to patients with Parkinson's disease (PD), patients with progressive supranuclear palsy (PSP) scored lower than healthy adults in the well-established semantic fluency test. However, it is unclear how unique are the produced words. This study examined the relationship between semantic fluency and words' uniqueness in patients with PSP.
METHODS
Twenty-seven patients with PSP Richardson's syndrome (PSP-RS), 37 patients with PD, and 41 healthy controls (HC) performed a standard semantic fluency test (animals), and their verbal responses were audio-recorded. We used the uniqueness to reflect the ability to produce both original and effective work, that is, creativity.
RESULTS
The PSP-RS group produced fewer correct words and fewer unique words than the PD and HC groups. Moreover, the correlation between fluency and uniqueness was positive in the HC and PD groups but negative in the PSP-RS group. Importantly, the actual levodopa dose was positively correlated with the fluency but negatively correlated with the uniqueness in PSP-RS. The PSP-RS patients who took a greater dose of levodopa tended to produce more correct words but fewer unique words.
CONCLUSIONS
These results suggested that levodopa may modulate semantic fluency and uniqueness in the early stages of PSP-RS.
Topics: Humans; Supranuclear Palsy, Progressive; Male; Female; Aged; Semantics; Levodopa; Parkinson Disease; Middle Aged; Neuropsychological Tests; Antiparkinson Agents
PubMed: 38945805
DOI: 10.1002/brb3.3606 -
The Lancet. Neurology Jun 2024
PubMed: 38945143
DOI: 10.1016/S1474-4422(24)00260-6 -
Pediatric Neurology Jun 2024Trihexyphenidyl and clonazepam are commonly used to treat dystonia in children with cerebral palsy (CP). However, there is a notable gap in the literature when it comes...
Efficacy of Oral Trihexyphenidyl Plus Clonazepam Versus Trihexyphenidyl for the Treatment of Dystonia in Children With Dystonic Cerebral Palsy: An Open-Label Randomized Controlled Trial.
BACKGROUND
Trihexyphenidyl and clonazepam are commonly used to treat dystonia in children with cerebral palsy (CP). However, there is a notable gap in the literature when it comes to studies that combine these first-line agents for the management of dystonia.
METHODS
This open-label, randomized controlled trial aimed to compare the efficacy of adding oral clonazepam to trihexyphenidyl (THP + CLZ) versus using trihexyphenidyl alone (THP) in reducing the severity of dystonia, as measured by the Barry-Albright Dystonia (BAD) score. The study was conducted over a 12-week therapy period in children with dystonic CP aged two to 14 years.
RESULTS
Each group enrolled 51 participants. The THP + CLZ group showed significantly better improvement in dystonia severity at 12 weeks compared with the THP group alone (-4.5 ± 2.9 vs -3.4 ± 1.7, P = 0.02). Furthermore, the THP + CLZ group exhibited superior improvement in the severity of choreoathetosis, upper limb function, pain perception by the child, and quality of life, with P values of 0.02, 0.009, 0.01, and 0.01, respectively. The number of participants experiencing treatment-emergent adverse events was comparable in both groups (P = 0.67). Importantly, none of the participants in any of the groups reported any serious adverse events.
CONCLUSION
A combination of oral THP + CLZ proves to be more efficacious than using THP alone for the treatment of dystonic CP in children aged two to 14 years in terms of reducing the severity of dystonia.
PubMed: 38945037
DOI: 10.1016/j.pediatrneurol.2024.06.004 -
Journal of Pediatric Urology Jun 2024
Response to letter to editor re "Comparison of the efficiency of transcutaneous electrical nerve stimulation and manual therapy in children with cerebral palsy with lower urinary system dysfunction- a randomized prospective trial".
PubMed: 38945787
DOI: 10.1016/j.jpurol.2024.05.030 -
Archives of Physical Medicine and... Jun 2024To retrospectively evaluate the comparative effect of two wheelchair seating systems, Custom-Contoured Wheelchair Seating (CCS) and Modular Wheelchair Seating (MWS), on...
Effect of wheelchair seating systems on scoliosis progression for children with neurological and neuromuscular disorders: a retrospective study of Custom-Contoured Wheelchair Seating and Modular Wheelchair Seating.
OBJECTIVES
To retrospectively evaluate the comparative effect of two wheelchair seating systems, Custom-Contoured Wheelchair Seating (CCS) and Modular Wheelchair Seating (MWS), on scoliosis progression in children with neuromuscular and neurological disorders and to determine any predictors for scoliosis progression.
DESIGN
Longitudinal, retrospective cohort study SETTING: National Health Service regional posture and mobility service PARTICIPANTS: Non-ambulant paediatric wheelchair users with neuromuscular and neurological disorders (N = 75; 36 male, 39 female; mean age at seating intervention, 10.50 ± 3.97 years) issued CCS and MWS by the South Wales Posture and Mobility Service from 2012 to 2022.
INTERVENTIONS
Two specialized wheelchair seating systems, CCS and MWS.
MAIN OUTCOME MEASURES
A generalized least squares (GLS) model was used to estimate the effect of seat type on Cobb angle over time.
RESULTS
Of the 75 participants enrolled, 51% had cerebral palsy. Fifty were issued CCS and 25 were issued MWS. Baseline Cobb angle was 32.9±18.9° for the MWS group and 48.0±31.0° for the CCS group. The GLS model demonstrated that time since seating intervention (χ = 122, p < .0001), seating type (χ = 52.5, p < .0001), and baseline scoliosis severity (χ = 41.6, p < .0001) were predictive of scoliosis progression. Condition was not a strong predictor (χ = 9.96, p = .0069), and sex (χ = 5.67, p = .13) and age at intervention (χ = 4.47, p = .35) were not predictive. Estimated contrasts of medical condition with seat type over time demonstrated smaller differences between MWS and CCS over time. Predicted scoliosis velocity was found to attenuate with use of CCS over time compared to MWS, although, scoliosis deteriorated regardless of intervention.
CONCLUSIONS
Our findings showed paediatric wheelchair users with neurological and neuromuscular disorders prescribed CCS showed greater mitigation of scoliosis progression over time compared to those issued MWS.
PubMed: 38944100
DOI: 10.1016/j.apmr.2024.06.007 -
International Journal of Surgery Case... May 2024Primary intracranial neuroendocrine tumors (NETs) are exceedingly rare, often posing diagnostic challenges, particularly in non-secreting variants. These tumors may...
INTRODUCTION AND IMPORTANCE
Primary intracranial neuroendocrine tumors (NETs) are exceedingly rare, often posing diagnostic challenges, particularly in non-secreting variants. These tumors may initially present with nonspecific symptoms, leading to delayed diagnosis and potential neurological complications.
CASE REPORT
We present the case of a 33-year-old male admitted with a one-year history of progressively worsening headache accompanied by acute left ptosis and diplopia. Initial examination revealed left eye ptosis and hypotropia, indicative of third and fourth cranial nerve paralysis. Cavoscopy revealed a mild anterior wall bulge of the sphenoid with normal mucosa. MRI imaging unveiled an expansive clival process extending towards the sellar region and left cavernous sinus, completely occupying the sphenoid sinus and exerting mass effect on the pituitary stalk. Hormonal assays were within reference ranges, ruling out a hormonally-active tumor. Endoscopic endonasal surgery for biopsy revealed a low-grade neuroendocrine tumor positive for cytokeratin AE1/AE2, chromogranin A, synaptophysin, and beta-catenin, with a Ki-67-labeling index <2 %. Somatostatin receptor scintigraphy confirmed intense hyper fixation of the tracer in the sphenoidal tumor, supporting its neuroendocrine origin. The patient declined surgical intervention after informed consent, opting for C1 somatostatin analogs prior to radiotherapy. One-year follow-up demonstrated symptom stability with no tumor progression.
CLINICAL DISCUSSION
To this day, no consensus among reports concerning the optimal management of these cases. Imaging assessment is crucial to validate the primary nature of the tumor and to exclude any distant localization. Various therapeutic modalities, such as surgery, radiotherapy, and somatostatin analogs, should be considered based on the specific characteristics and extent of the tumor.
CONCLUSION
Our case is a clear reminder that neuroendocrine tumors should be considered as a differential diagnosis for skull base neoplasms.
PubMed: 38943936
DOI: 10.1016/j.ijscr.2024.109750 -
Journal of Clinical Neuroscience :... Jun 2024Aneurysms of the posterior cerebral circulation constitute a burdensome condition with high mortality and morbidity. In the modern era, there has been a trend toward...
When microsurgery becomes the only lifesaving resource: An institutional experience treating patients from low-income backgrounds affected by posterior circulation brain aneurysms.
BACKGROUND AND OBJECTIVE
Aneurysms of the posterior cerebral circulation constitute a burdensome condition with high mortality and morbidity. In the modern era, there has been a trend toward favoring an endovascular approach over microsurgery for aneurysm cases. Nevertheless, this transition has yet to be mirrored in low-to-middle-income countries where endovascular therapy may not be widely available. Herein, we aim to illustrate our experience treating these challenging conditions in patients from low-income backgrounds and discuss the relevance of the clinical setting in the treatment decision.
METHODS
The authors conducted a retrospective review of the health records of patients who received microsurgical treatment for aneurysms in the posterior circulation, including the basilar artery (BA), posterior cerebral artery (PCA), and posterior inferior cerebellar artery (PICA) in an institution providing treatment to people of low-income backgrounds. Epidemiological data, microsurgical technique, and neurological function were retrieved and analyzed.
RESULTS
Surgical clipping was employed for 12 patients (75% female) harboring 15 aneurysms (87% in the posterior circulation and 62% ruptured at presentation). Aneurysms were predominately located in the BA (69%), followed by the PCA (15%) and PICA (15%). Among neurological complications, 25% of patients developed oculomotor nerve palsy. The mortality rate was 17% owing to complications such as cerebral vasospasm, infarction, and severe intracranial hypertension. At the 6-month follow-up, 90% of patients had a good clinical outcome (modified Rankin scale scores of 0-2).
CONCLUSION
The present case series illustrates the manifest role of microsurgical techniques for posterior circulation aneurysms, particularly in a scenario where endovascular techniques are not easily accessible. Importantly, this clinical setting pressure could exhort trainees to strive for microsurgical mastery and gain a competitive advantage.
PubMed: 38943907
DOI: 10.1016/j.jocn.2024.06.014 -
Gait & Posture Jun 2024Many children with cerebral palsy (CP) show impairments in trunk posture control, one crucial factor contributing to impairments in gait and arm manipulation.
BACKGROUND
Many children with cerebral palsy (CP) show impairments in trunk posture control, one crucial factor contributing to impairments in gait and arm manipulation.
RESEARCH QUESTION
The goal of this study was to test the feasibility of the cable-driven hippotherapy system on improving trunk posture control and walking function in children with CP.
METHOD
Ten children were recruited in this study with average age 6.4 ± 3.0 years old, and were randomly assigned to the robotic group and the conventional balance training group. A custom designed cable-driven robotic hippotherapy system was used to deliver controlled pelvis perturbations while children were sitting astride. Participants from both groups underwent intensive robotic hippotherapy training or conventional balance training, depending on their group assignment, for 6 weeks (3 time/week). Outcome measures were assessed pre and post 6 weeks of robotic or conventional balance training, and 8 weeks after the end of training (follow-up test). The primary outcome measure was trunk control, which was measured using the Trunk Control Measurement Scale (TCMS). In addition, the Gross Motor Function Measure (GMFM-66), self-selected overground walking speed, and 6-minute walking distance were also assessed.
RESULTS
The change in TCMS score from baseline to the post intervention was significantly greater for the robotic group than that for the conventional group (i.e., 6 ± 3 vs. -1 ± 5, p = 0.048, Mann-Whitney test). GMFM scores, self-selected overground walking speed, and 6-minute walking distance showed no significant improvement after robotic hippotherapy and after conventional balance training (p > 0.05).
SIGNIFICANCE
Results from this study indicated that it was feasible to improve trunk posture control in children with CP using a cable-driven robotic hippotherapy system. Knowledge obtained from this study may provide an insight for the development of new perturbation-based intervention approaches for improving trunk posture control in children with CP.
PubMed: 38943825
DOI: 10.1016/j.gaitpost.2024.06.020 -
Pediatric Research Jun 2024Preterm white matter injury (PWMI) is the most common cause of brain injury in premature neonates. PWMI involves a differentiation arrest of oligodendrocytes, the...
BACKGROUND
Preterm white matter injury (PWMI) is the most common cause of brain injury in premature neonates. PWMI involves a differentiation arrest of oligodendrocytes, the myelinating cells of the central nervous system. Clemastine was previously shown to induce oligodendrocyte differentiation and myelination in mouse models of PWMI at a dose of 10 mg/kg/day. The minimum effective dose (MED) of clemastine is unknown. Identification of the MED is essential for maximizing safety and efficacy in neonatal clinical trials. We hypothesized that the MED in neonatal mice is lower than 10 mg/kg/day.
METHODS
Mouse pups were exposed to normoxia or hypoxia (10% FiO2) from postnatal day 3 (P3) through P10. Vehicle or clemastine at one of four doses (0.5, 2, 7.5 or 10 mg/kg/day) was given to hypoxia-exposed pups. Myelination was assessed at age P14 and 10 weeks to determine the MED. Clemastine pharmacokinetics were evaluated at steady-state on day 8 of treatment.
RESULTS
Clemastine rescued hypoxia-induced hypomyelination with a MED of 7.5 mg/kg/day. Pharmacokinetic analysis of the MED revealed C 44.0 ng/mL, t 4.6 h, and AUC 280.1 ng*hr/mL.
CONCLUSIONS
Based on these results, myelination-promoting exposures should be achievable with oral doses of clemastine in neonates with PWMI.
IMPACT
Preterm white matter injury (PWMI) is the most common cause of brain injury and cerebral palsy in premature neonates. Clemastine, an FDA-approved antihistamine, was recently identified to strongly promote myelination in a mouse model of PWMI and is a possible treatment. The minimum effective dose in neonatal rodents is unknown and is critical for guiding dose selection and balancing efficacy with toxicity in future clinical trials. We identified the minimum effective dose of clemastine and the associated pharmacokinetics in a murine chronic hypoxia model of PWMI, paving the way for a future clinical trial in human neonates.
PubMed: 38942888
DOI: 10.1038/s41390-024-03326-w