Journal of Central Nervous System Disease最新文献

Background: Epidural spinal cord stimulation (EES) is a promising intervention for motor rehabilitation after spinal cord injury (SCI), but the extent and trajectory of motor recovery remain unclear.

Objective: This phase II trial evaluates the acquisition of voluntary movements in paraplegic patients (ASIA A or B) following SCI, assessed by the Fugl-Meyer Lower Extremity (FMA-LE) score and electromyography (EMG).

Methods: This interim analysis includes five patients implanted with EES and followed for 12 months. The primary outcome was motor recovery, measured by FMA-LE and EMG. Secondary outcomes included balance (Berg Balance Scale), spasticity (Modified Ashworth Scale), pain, autonomic functions, mood, quality of life (WHO-QOL), and safety. Adverse events were monitored.

Results: The FMA-LE score improved from 36 ± 9 (SD) to 55 ± 2 at 3 months (P < 0.05), 59 ± 2 at 6 months (P < 0.05), and 64 ± 4 at 12 months (P < 0.05). EMG confirmed increased voluntary activation. Balance and spasticity improved, while pain and autonomic functions remained unchanged. Motor gains plateaued after 5 months, reaching 68% above baseline. No serious adverse events occurred, though minor complications included transient nociceptive pain and a self-resolving pressure ulcer.

Conclusions: These findings support the role of EES in facilitating early motor recovery in SCI patients, consistent with prior studies. However, the plateau effect suggests a limit to long-term gains. Future research should explore strategies to sustain improvements, including regenerative therapies or optimized neuromodulation protocols.Trial registration number: NCT06847295.

Background and purpose: Nationwide data on childhood stroke prevalence and incidence in Mainland China are unavailable now. We aim to investigate the prevalence and incidence of childhood arterial ischaemic stroke and haemorrhagic stroke in China nationwide.

Methods: In 2013, we implemented a nationally representative, door-to-door epidemiological survey on stroke in China through a complex, multistage, probability sampling design. A post-hoc subgroup analysis was conducted to examine both the crude point prevalence and annual incidence of childhood stroke. Due to the small case number, the Poisson distribution was used to estimate the 95% confidence intervals (CIs) for the crude rates.

Results: In this survey, only 4 strokes were identified. The crude point prevalence of childhood stroke was 4.82 (95% CI: 1.31-12.34) per 100 000 children in China; 4.51 (95% CI: 0.55-16.29) per 100 000 boys, and 5.17 (95% CI: 0.63-18.69) per 100 000 girls. Haemorrhagic stroke prevalence was 3.61 (95% CI: 0.75-10.56) vs 1.20 (95% CI: 0.03-6.71) per 100 000 children for arterial ischaemic stroke. The crude annual incidence of childhood stroke was 2.34 (95% CI: 0.28-8.45) per 100 000 children in China; 5.01 (95% CI: 0.60-18.10) per 100 000 girls. The crude annual incidences of both arterial ischaemic stroke and intracerebral haemorrhage were the same at 1.17 (95% CI: 0.03-6.52) per 100 000 children.

Conclusions: The point prevalence and annual incidence estimates of childhood stroke were 4.82 and 2.34 per 100 000 children in China, respectively. Chinese children may have more prevalent cases of haemorrhagic than arterial ischaemic strokes.

Gastrointestinal (GI) dysfunction is a common and often underappreciated aspect of Parkinson's disease (PD), with symptoms manifesting at multiple levels of the digestive tract, from swallowing difficulties to challenges with defecation. These non-motor symptoms can be more debilitating than the hallmark motor impairments of PD, profoundly affecting patients' quality of life. The burden of GI issues in PD extends beyond discomfort, contributing to malnutrition, weight loss, and impaired medication absorption, which can exacerbate both motor and non-motor symptoms. Despite their clinical significance, GI symptoms are frequently overlooked or mismanaged in routine practice. Inappropriate treatments, including certain medications and dietary recommendations, may inadvertently worsen the disease course. Therefore, a comprehensive understanding of GI dysfunction in PD is critical for clinicians, especially neurologists, to optimize patient care. This review provides an updated overview of the common GI manifestations in PD, including drooling, dyspepsia and dysphagia, gastroparesis, constipation, H. pylori infection, and small intestinal bacterial overgrowth. We discuss current diagnostic approaches, non-pharmacological and pharmacological treatment strategies. Recognizing and appropriately managing GI dysfunction in PD is essential for optimizing symptom control and improving patients' overall well-being.

Background: Blebs are small bulges on the surface of intracranial aneurysms (IAs) that increase rupture risk. Among Chinese individuals, the prevalence, distribution, and clinical, geometric, and local hemodynamic characteristics of small- and medium-sized (<15 mm) blebbed IAs remain unclear.

Objectives: To investigate the prevalence, distribution, and associated clinical, geometric, and hemodynamic features of blebs in small- and medium-sized IAs among Chinese patients, and to identify predictors of aneurysm rupture and bleb formation.

Design: A retrospective observational study.

Methods: CTA or DSA data from 214 patients with ruptured (RIAs) and unruptured IAs (UIAs) (<15 mm), with or without blebs, were analyzed. Three-dimensional reconstruction, geometric measurement, and computational fluid dynamics (CFD) analysis were conducted using Mimics and ANSYS Fluent. Hemodynamic parameters were assessed across the neck, body, and dome, and logistic regression was used to identify predictors of rupture and bleb formation.

Results: Aneurysms from 214 patients (93 men, 121 women; mean age 59.90 ± 11.76 years) were analyzed. Blebs were found in 107 aneurysms (56.7% of RIAs, 39.1% of UIAs). They were more frequent in ACoA, PCoA, and bifurcation aneurysms (all P < 0.05) and were associated with rupture. RIAs had larger blebs than UIAs (2.73 ± 1.28 mm vs 2.06 ± 1.07 mm, P = 0.009). Blebbed IAs exhibited larger size, more irregular shape, higher AR, SR, BN, HWR, and lower NWSS, TAWSS, OSI (all P < 0.05). SR was the strongest rupture predictor (AUC = 0.718, SR > 1.3144). Aneurysms at ACoA (OR = 8.812,CI:2.455-31.634), PCoA (OR = 6.376,CI:2.094-19.414), and high SR (OR = 2.738,CI:0.98-7.651) were significant rupture risk factors. PCoA (OR = 2.261,CI:0.759-6.739) and SR (OR = 4.683,CI:1.937-11.324) independently predicted bleb formation.

Conclusion: Blebs are common in small- and medium-sized IAs, especially at the ACoA, PCoA, and bifurcations, and are associated with an increased risk of rupture. Larger blebs are more often seen in ruptured IAs. A high SR is a key predictor of both rupture and bleb formation. Further studies on bleb-related hemodynamics in the neck, body, and dome are warranted.

Background: Cognitive impairment is common in patients with multiple sclerosis (MS). Physical activity is clearly linked to cognitive performance, and several studies have shown the importance of regular cognition testing, but such testing is still not routinely performed in clinical practice.

Objective: This study aimed to investigate the association between cognition, disability, and physical performance in relapsing-remitting MS (RRMS) patients.

Methods: A total of 41 patients with RRMS with an Expanded Disability Status Scale (EDSS) level of 0-5.5 and 20 healthy controls completed the MS Functional Composite (MSFC) test and the Symbol Digit Modality Test (SDMT). Six-Minute Walk (6MW) was evaluated for all participants, and they used an accelerometer for seven days.

Results: A significant correlation was found between cognition and disability level measured by the MSFC (MSFC/SDMT, r = 0.668, P = .001) and between disability and 6MW (EDSS/6MW, r = -0.516, P = .001; MSFC/6MW, r = 0.348, P = .028) in the patients' group. Cognition results (SDMT) were statistically significantly weaker in patients with EDSS >2.5 vs EDSS ≤2.5 or control group. Total daily activity (MVPS) correlated with cognition as measured by the SDMT in the control group but not in the patients' group. In the EDSShigh group, better results on the 6MW test were associated with better cognition results as measured by the SDMT (r = 0.505, P = .039).

Conclusion: There was a clear association between disability, 6MW and cognition. Better results on the 6MW predicted better cognition and disability.Clinical trial registration number: NCT04115930.

Introduction: Calcium pyrophosphate deposition (CPPD) disease is characterized by calcium pyrophosphate crystals in hyaline and fibrocartilage. Chondrocalcinosis, a radiographic hallmark for CPPD, becomes more prevalent with age. Although CPPD mainly targets peripheral joints, spinal involvement, affecting intervertebral discs and spinal ligaments, is less common but significant, seen in 24.3% of hospitalized patients with CPPD disease. This report describes a rare case of spinal CPPD causing spinal canal stenosis in the lumbar region.

Case description: A 79-year-old woman with a 3-year history of low back pain presented with severe left-sided pain and mobility impairment. Initial examination showed lumbar tenderness and normal muscle strength. Computed tomography (CT) and magnetic resonance imaging scans revealed a calcified extradural mass occupying the anterior portion of the lumbar spinal canal, most likely associated with the posterior longitudinal ligament. The patient underwent L3-L5 hemilaminectomies and dorsal spondylodesis, removing a whitish intraspinal mass. Histopathology confirmed CPPD. Post-surgery, the patient experienced initial pain relief but required emergency surgery due to complications. Over the next year, her mobility and pain improved significantly.

Discussion: Spinal CPPD manifests with varied clinical presentations, complicating diagnosis. Imaging reveals calcifications ranging from deposits to mass-like lesions causing compression. CT provides detailed visualization of characteristic calcifications, aiding in diagnosis, while histopathology remains the gold standard. Multidisciplinary collaboration is vital for accurate diagnosis and optimal management.

Background: Action Observation Therapy (AOT) and Neuromuscular Electrical Stimulation (NMES) are widely adopted techniques for upper-limb rehabilitation in post-stroke patients. Although AOT and NMES are individually effective, studies investigating a potential synergistic effect on enhancing rehabilitative outcomes are lacking. Objectives: This study aims at comparing the effect of AOT and NMES applied together (AOT-NMES) on muscle synergies with respect to either AOT alone or a Motor Neutral Observation treatment alone (MNO, involving neither AOT nor NMES) on motor function recovery of upper limb. Design: Randomized Controlled Trial (RCT) with n = 60 post-stroke patients with resulting upper limb disability, randomly allocated (1:1:1 ratio) in three interventional arms: AOT-NMES (n = 20), AOT (n = 20) and MNO (n = 20). Methods and Analyses: All rehabilitation treatments will consist of n°15 60 min-long rehabilitative sessions. Primary outcome measure will be upper limb motor function, assessed using the Fugl-Meyer Assessment scale for upper limb (FM-UL), collected at the baseline (T0), post-intervention (T1) and at follow-up (T2, 6-months after T1). Other outcome measures will be collected through a multidimensional evaluation including assessing stroke-associated quality of life, neurophysiological data, biomechanical and MRI measures. The innovative protocol will also be evaluated for usability and safety. Discussion: We expect to determine the efficacy, usability and safety of the AOT-NMES rehabilitation approach for the recovery of upper limb motor function in post-stroke patients. The obtained results will also help reveal the neural underpinnings of motor recovery, as assessed by neurophysiological data, biomechanical and MRI measures.

We present a case of paraneoplastic tumefactive demyelination in a 55-year-old female with an underlying anaplastic thyroid carcinoma (ATC), alongside a review of the literature on all cases of tumefactive demyelination associated with non-CNS neoplasia. In the presented case the patient developed a right-sided subacute sensorimotor hemiparesis. The initial cerebral MRI revealed a bilateral frontoparietal tumefactive mass lesion with marked gadolinium uptake and mass effect. Cerebrospinal fluid revealed CSF-specific oligoclonal bands type III, with negative cell count, protein and pathogen testing. Brain biopsy indicated demyelination and T-cell infiltrates and foamy macrophages. A body CT revealed an anaplastic thyroid carcinoma. Despite steroids, plasma exchange, rituximab, and cancer treatment, the patient died due to clinical fluctuation and cancer progression. In addition to our case 9 cases of tumefactive demyelinating have been reported in patients with newly diagnosed extracranial neoplasia, most commonly seminoma germ cell tumour (7/10). 8/10 (80%) of patients were male, with mean age at diagnosis was 52.9 years 95% C.I. [43.8, 62.0]. 5/10 patients presented with sensorimotor hemiparesis and/or confusion/neurocognitive deficits. 4/10 with visual deficits and 2/10 with aphasia. In all cases neoplasia was diagnosed simultaneously or after neurological manifestations. All cases presented initially as solitary lesions. A malignancy specific-treatment as well as steroid treatment in different regiments were applied. In addition in 2/10 plasmapheresis was implemented and 1/10 patients received intravenous immunoglobulins. In the majority of cases including the presented case partial neurological improvement was documented whereas malignancy usually progressed. To our knowledge, this is the first report of paraneoplastic tumefactive demyelination associated with an ATC highlighting the importance of a thorough workup in these patients. This is the first reported case of paraneoplastic tumefactive demyelination associated with ATC, underscoring the necessity of a comprehensive diagnostic approach in similar patients.