Intrathecal baclofen (ITB) is an effective treatment for hypertonia in children involving the implantation of a pump and catheter system. The highest concentration of ITB is at the catheter tip. The catheter tip location is most commonly within the lumbar or thoracic spine. The cervical tip location has traditionally been avoided because of concerns of hypoventilation and pneumonia; however, these complications in cervical compared with thoracic or lumbar placement have not been reliably proven. Some studies have suggested that cervical ITB location better treats upper-extremity hypertonia. There are limited data describing the safety and efficacy of cervical ITB on hypertonia. The authors present a single-institution retrospective case series highlighting the safety and efficacy of using cervical ITB location for the treatment of hypertonia.
Retrospective data analysis was performed for children who underwent continuous dosing cervical ITB between April 2022 and October 2023. Nonmodifiable risk factors, clinical variables, operative characteristics, and adverse outcomes were collected.
This study included 25 patients (8 female). The mean age at implantation was 12.4 years, and the mean operative duration was 90 minutes. The mean Barry-Albright Dystonia Scale score decreased by 9.5 points (p = 0.01). The mean aggregated modified Ashworth scale score in the upper extremities decreased by 2.14 points (p = 0.04), and that in the lower extremities decreased by 4.98 points (p < 0.01). One patient each (4%) had infection and baclofen toxicity. Two patients (8%) had respiratory depression requiring continuous positive airway pressure. There was no incidence of pneumonia or wound dehiscence.
The cervical catheter tip location for ITB is safe, is effective to control tone, and should be considered for the treatment of hypertonia. Larger studies with longer follow-up are necessary to further determine upper-limit dosing safety along with long-term functional benefits in these patients.

On demand and localized treatment for excessive muscle tone after spinal cord injury (SCI) is currently not available. Here, we examine the reduction in leg hypertonus in a person with mid-thoracic, motor complete SCI using a commercial transcutaneous electrical stimulator (TES) applied at 50 or 150 Hz to the lower back and the possible mechanisms producing this bilateral reduction in leg tone. Hypertonus of knee extensors without and during TES, with both cathode (T11-L2) and anode (L3-L5) placed over the spinal column (midline, MID) or 10 cm to the left of midline (lateral, LAT) to only active underlying skin and muscle afferents, was simultaneously measured in both legs with the pendulum test. Spinal reflexes mediated by proprioceptive (H-reflex) and cutaneomuscular reflex (CMR) afferents were examined in the right leg opposite to the applied LAT TES. Hypertonus disappeared in both legs but only during thoracolumbar TES, and even during LAT TES. The marked reduction in tone was reflected in the greater distance both lower legs first dropped to after being released from a fully extended position, increasing by 172.8% and 94.2% during MID and LAT TES, respectively, compared with without TES. Both MID and LAT (left) TES increased H-reflexes but decreased the first burst, and lengthened the onset of subsequent bursts, in the cutaneomuscular reflex of the right leg. Thoracolumbar TES is a promising method to decrease leg hypertonus in chronic, motor complete SCI without activating spinal cord structures and may work by facilitating proprioceptive inputs that activate excitatory interneurons with bilateral projections that in turn recruit recurrent inhibitory neurons.NEW & NOTEWORTHY We present proof of concept that surface stimulation of the lower back can reduce severe leg hypertonus in a participant with motor complete, thoracic spinal cord injury (SCI) but only during the applied stimulation. We propose that activation of skin and muscle afferents from thoracolumbar transcutaneous electrical stimulation (TES) may recruit excitatory spinal interneurons with bilateral projections that in turn recruit recurrent inhibitory networks to provide on demand suppression of ongoing involuntary motoneuron activity.

UNASSIGNED: Intrathecal baclofen (ITB) therapy, a viable alternative for unsuitable candidates of conventional spasticity medications, is a preferred method of administration over the oral route. Owing to its enhanced bioavailability, ITB ensures a more effective delivery at the target site.
UNASSIGNED: There is a lack of conclusive evidence regarding the use of ITB treatment in managing ambulatory patients with spastic dystonia. Before ITB pump implantation, patients commonly undergo an ITB bolus injection trial to rule out potential adverse reactions and verify the therapeutic effects on hypertonic issues. In this report, we highlight a case of spastic dystonia, particularly focusing on an ambulatory patient who demonstrated significant improvement in both the modified Ashworth scale (MAS) score and gait pattern following the ITB injection trial.
UNASSIGNED: This case report outlines the medical history of a 67-year-old male diagnosed with left-side hemiplegia and spastic dystonia, resulting from his second episode of intracranial hemorrhage in the right thalamus. An ITB injection trial was initiated because the patient was not suitable for continued botulinum toxin injections and oral medications. This was due to the persistent occurrence of spastic dystonia in both the upper and lower extremities. The patient underwent a four-day ITB injection trial with progressively increasing doses, resulting in improved MAS scores and gait parameters, including cadence, step length, step time, stride length, and stride time were increased. Particularly, kinematic gait analysis demonstrates a substantial improvement of increased knee flexion in the swing phase in stiff knee gait pattern. These findings indicated a gradual reduction in spasticity-related symptoms, signifying the positive effect of the ITB injection trial. The patient eventually received an ITB pump implantation.
UNASSIGNED: In this post-stroke patient with spastic dystonia, ITB therapy has demonstrated effective and substantial management of spasticity, along with improvement in gait patterns.

A physical examination of a 9-month-old female infant presenting with vomiting and diarrhea revealed tenderness in the right upper abdomen and heightened abdominal muscle tone. Abdominal ultrasonography identified an irregular hypoechoic area within the right lobe of the liver. While a subsequent enhanced CT examination disclosed a well-defined lesion exhibiting internal focal calcification and delayed heterogeneous enhancement. Subsequently, she underwent surgical resection, and postoperative pathology revealed areas of epithelioid hemangioendothelioma and cavernous hemangioma. Immunohistochemistry demonstrated positive expression of CD34, CD31, FLI-1, and F-VIII. The pathologic diagnosis was confirmed as composite hemangioendothelioma (CHE).

To the best of the authors\' knowledge, no data are available about the use of isokinetic resistance training for managing ankle plantarflexor spastic hypertonia in people with multiple sclerosis (MS). The aim of this proof-of-concept study was to explore the feasibility and effects of concentric contractions on spasticity-related resistance to passive motion, strength, and mobility in people with MS and ankle plantarflexor spasticity.
In this pretest/posttest case series, 5 people with MS (mean age = 53.6 [SD = 8.8] years; median Expanded Disability Status Scale score = 5; Modified Ashworth Scale range = 1-4) received 6 weeks of isokinetic resistance training of the spastic plantarflexors. Before and after the intervention, the following outcomes were assessed: average peak torque during passive robotic mobilization, isometric strength, surface electromyography (sEMG) from the spastic muscles, time to complete the 10-m Walk Test, and the Timed \"Up & Go\" Test. The standardized effect size was used to test pretest and posttest effects at the individual level. Group-level analyses were also performed.
Following the training, the average peak torque recorded from the plantarflexors during passive motion at a velocity of 150 degrees per second was found to be decreased by at least 1 SD in all participants but 1, with a significant reduction at the group level of 23.8%. Conversely, no changes in sEMG activity were detected. Group-level analyses revealed that the maximal strength of the trained plantarflexors increased significantly (31.4%). Fast walking speed increased and time to complete the Timed \"Up & Go\" Test decreased in 4 participants, although not significantly at the group level.
Isokinetic resistance training proved safe and feasible in people who had MS and ankle plantarflexor spasticity. The observed reductions in resistance to passive motion from the spastic plantarflexors in the absence of sEMG changes might suggest a mechanical rather than a neural effect of the training.
Based on these preliminary findings, isokinetic resistance training does not exacerbate hypertonia in people with MS and ankle plantarflexor spasticity and could be safely used to manage muscle weakness in this population.

Neonatal Schwartz-Jampel syndrome type II is a rare and severe form of genetic disorder. Different from the classical appearance in infancy, neonatal presentation involves respiratory and feeding difficulties, along with characteristic pursed appearance of the mouth, myotonia, skeletal dysplasia and severe fatal hyperthermia. The clinical spectrum of this syndrome is so wide that it easily baffles with more common differentials. In this case report, a neonate born to third-degree consanguineous marriage with previous two abortions presented with respiratory difficulty, severe hyperthermia and feeding difficulty, which were daunting challenges to manage due to being refractory to standard line of management. Severe myotonia and gross dysmorphism were challenging dots to connect. Targeted exome sequencing was a ray of hope, which revealed homozygous mutation in the leukaemia inhibitory factor receptor gene on chromosome 5p13, confirming the genetic diagnosis for a fairly common spectrum of symptoms. The neonate later developed pneumoperitoneum and succumbed to underlying severe neonatal illness.

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BACKGROUND: Pain management is an essential part of good obstetrical care. The rapid onset of pain relief after combined spinal-epidural (CSE) analgesia may cause a transient imbalance in maternal catecholamine level, leading to uterine hyperactivity and fetal heart rate (FHR) abnormalities. How to manage the uterine basal tone and FHR abnormalities after labor analgesia still remains controversial.
UNASSIGNED: A 33-year-old nulliparous woman at 40 weeks\' gestation underwent induction of labor after premature rupture of membranes. CSE analgesia was provided when the patient described her pain as the top on a scale of 10 during induction of labor with oxytocin infusion.
UNASSIGNED: Uterine hypertonus and fetal bradycardia were diagnosed within 10 minutes after CSE analgesia.
METHODS: Oxytocin infusion and CSE analgesia were immediately suspended, and measures of staying in left lateral decubitus position and giving supplemental oxygen were attempted to resuscitating the baby. Because of suspicious fetal distress, the baby was rapidly delivered by emergency cesarean section.
RESULTS: The Apgar score of the baby was 8 and 10 at 1 and 5 minutes after birth. Subsequent follow-up confirmed that both mother and baby were in good condition.
CONCLUSIONS: The loss of the tocolytic effect of epinephrine after CSE analgesia and continuous oxytocin infusion may work together to form a totally synergistic function, finally leading to inevitable uterine hypertonus and fetal bradycardia. Both the obstetrical provider and anesthesiologist should carefully monitor all patients in the first 15 minutes after CES analgesia induction. Oxytocin administration in this critical period deserves attention. Additionally, intraprofessional collaboration is also necessary to ensure high quality and safe delivery for all childbearing women.

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Myofascial pelvic pain is a chronic and debilitating condition, sometimes associated with pelvic floor disorders (PFD) such as urinary incontinence, defecatory dysfunction or pelvic organ prolapse. Our aim was to identify risk factors in women with PFD and hypertonic pelvic floor, compared to controls without hypertonicity.
Case control study (2009-2017) of patients with PFD and a diagnosis of hypertonic pelvic floor. Cases were matched with patients who presented with the same PFD but without pelvic floor hypertonicity. Postoperative patients with hypertonic pelvic floor were matched with patients who underwent surgery for the same PFD but did not develop pain. Risk factors were compared between groups.
Ninety-five cases were matched; 71% had urogynecologic surgery as a possible trigger for myofascial pain. Most were post-menopausal. Overall, case patients were younger than controls (mean 54 vs 59, P = 0.002). Multivariate logistic regression identified risk factors of younger age (OR 1.45, 95%CI 1.04-2.07), history of depression (OR 3, 95%CI 1.03-9.09), musculoskeletal spine injury (OR 4.32, 95%CI 1.01-21.26) and transobturator midurethral sling (OR 8.36, 95%CI 2.68-31.32). Retropubic midurethral sling was protective against pelvic floor hypertonicity (OR 0.37, 95%CI 0.15-0.86). A clinical prediction model including depression, endometriosis, irritable bowel, spine injury and type of midurethral sling was developed to estimate the probability for myofascial pain after urogynecologic surgery.
Specific risk factors predispose women with PFD to chronic pelvic floor hypertonicity. Knowledge of these can help with patient counselling and choice of midurethral sling prior to PFD surgery.