Prevalence of Gastrointestinal Symptoms, Severity of Dysphagia, and Their Correlation with Severity of Amyotrophic Lateral Sclerosis in a Mexican Cohort.

OBJECTIVES: Our study aimed to identify the prevalence and severity of gastrointestinal (GI) symptoms and dysphagia in patients with amyotrophic lateral sclerosis (ALS) and to assess whether a correlation exists between these symptoms and the severity of ALS progression. METHODS: The presence and severity of GI symptoms and dysphagia were identified by means of the Gastrointestinal Symptom Rating Scale (GSRS) and the Functional Outcome Swallowing Scale (FOSS). The Revised ALS Functional Rating Scale (ALSFRS-R) was utilized to determine the severity of ALS. Analysis of data was performed with Spearman correlations in semi-qualitative variables of clinical scales. ALSFRS-R scores were divided into 2 categories: those with mild to moderate ALS (≥40-30 points) and patients with moderate to advanced ALS (29-≤20 points). RESULTS: We studied 43 patients with definite ALS. The most frequent GI symptoms were constipation (60.5%), rectal tenesmus (57.5%), hard stools (55.0%), and borborygmus (42.5%). The moderate to advanced ALS stage was correlated with constipation (r = 0.334; p = 0.028), acid regurgitation (r = 0.384; p = 0.013), eructation (r = 0.334; p = 0.032), rectal tenesmus (r = 0.498; p = 0.001), and functional dysphagia (r = 0.656; p = <0.001). CONCLUSIONS: Early detection of these GI symptoms can guide timely therapeutic decisions to avoid weight loss, a predictor for worse prognosis. This study highlights the relevance of the detection of these symptoms in ALS patients who score ≤29 points in the ALSFRS-R scale to establish an appropriate treatment, prevent systemic complications, provide more comfort, and improve quality of life.

Functional Connectivity and Frequency Power Alterations during P300 Task as a Result of Amyotrophic Lateral Sclerosis.

Amyotrophic Lateral Sclerosis (ALS) is one of the most aggressive neurodegenerative diseases and is now recognized as a multisystem network disorder with impaired connectivity. Further research for the understanding of the nature of its cognitive affections is necessary to monitor and detect the disease, so this work provides insight into the neural alterations occurring in ALS patients during a cognitive task (P300 oddball paradigm) by measuring connectivity and the power and latency of the frequency-specific EEG activity of 12 ALS patients and 16 healthy subjects recorded during the use of a P300-based BCI to command a robotic arm. For ALS patients, in comparison to Controls, the results (p < 0.05) were: an increment in latency of the peak ERP in the Delta range (OZ) and Alpha range (PO7), and a decreased power in the Beta band among most electrodes; connectivity alterations among all bands, especially in the Alpha band between PO7 and the channels above the motor cortex. The evolution observed over months of an advanced-state patient backs up these findings. These results were used to compute connectivity- and power-based features to discriminate between ALS and Control groups using Support Vector Machine (SVM). Cross-validation achieved a 100% in specificity and 75% in sensitivity, with an overall 89% success.

Altered CSF cytokine network in amyotrophic lateral sclerosis patients: A pathway-based statistical analysis.

INTRODUCTION: Increased cytokine expression is a prominent finding in amyotrophic lateral sclerosis (ALS). Due to their interdependence and pleiotropism, interpretation of CSF concentrations of a single cytokine is challenging. We describe a cytokine analysis in ALS patients using a pathway-based statistical method to identify changes in the whole cytokine network. METHODS: We analyzed 19 cytokines in CSF of ALS patients and controls. An equality of concentration matrices was conducted that allowed us to evaluate disturbances in the relationship of cytokines between controls and ALS patients with less and more than 12months of disease length. MANOVA assessed differences in cytokines and interdependence was compared by partial correlations among specific pairs of cytokines. RESULTS: Seventy-seven ALS patients and 13 control subjects were included. Significant differences were identified in the cytokine pathway between controls and ALS patients with less (p<0.0001) and more than 12months of disease length (p<0.0001), and between ALS patients with less than 12months and those with more than 12months (p=0.0058). In ALS patients with a shorter disease length, IL4 and IL6 were negatively correlated (-0.3571), whereas in ALS>12months, a positive correlation was detected (0.4080). CONCLUSIONS: The pathway-based statistical method revealed remarkable variations in the whole cytokine pathway in ALS patients and controls. Cytokine-network changes and the positive correlation between IL4 and IL6 were related to disease length; these variations might explain the deleterious immunological effects on motor neurons. A further analysis using this method is needed to confirm the exact interaction among cytokines in ALS.

Acute toxic leukoencephalopathy associated with a non-prescription weight loss supplement: a report of two cases.

Weight loss dietary supplements are used with some frequency by an increasingly overweight population. Some products are not adequately regulated and may pose potential health risks. We report two new cases of acute toxic leukoencephalopathy (ATL) due to the use of a supplement marketed as a thermogenic weight loss aid. ATL is a heterogeneous clinic-radiological entity that has been associated with various compounds, such as chemotherapeutic drugs and immunomodulators. It is characterized by an often reversible periventricular and infratentorial demyelination. The commercialization of non-regulated weight loss products continues to be a health risk in our population.

Amyotrophic lateral sclerosis in pregnancy: clinical outcome during the post-partum period after stem cell transplantation into the frontal motor cortex.

BACKGROUND AIMS: Amyotrophic lateral sclerosis (ALS) is rare in pregnant patients. Stem cell therapy has been proposed as a potential therapeutic strategy for ALS. METHODS: We describe a young woman with sporadic ALS that started during the second trimester of pregnancy with a rapid progression after delivery and severe motor impairment. Several drugs and stem cell injection by lumbar puncture were performed without changes before the patient was referred to our institution. RESULTS: After bilateral autologous stem cell transplantation into the frontal motor cortices, we observed stabilization in ALS functional rating scale, significant delay of ALS progression and an extension in her life expectancy. CONCLUSIONS: Stem cell transplantation may alter the clinical course of ALS and improve quality of life in pre-menopausal women.

Partial Cranial Reconstruction Using Titanium Mesh after Craniectomy: An Antiadhesive and Protective Barrier with Improved Aesthetic Outcomes.

OBJECTIVE: Describe a new, safe, technique that uses titanium mesh to partially cover skull defects immediately after decompressive craniectomy (DC). METHODS: This study is a retrospective review of 8 patients who underwent DC and placement of a titanium mesh. The mesh partially covered the defect and was placed between the temporalis muscle and the dura graft. The muscle was sutured to the mesh. All patients underwent cranioplasty at a later time. The study recorded and analyzed demographic information, time between surgeries, extra-axial fluid collections, postoperative infections, need for reoperation, cortical hemorrhages, and functional and aesthetic outcomes. RESULTS: After craniectomy, all patients underwent cranioplasty within an average of 112.5 days (30-240 days). One patient reported temporalis muscle atrophy, which was the only complication observed. During the cranioplasties, no adhesions were found between temporalis muscle, titanium mesh, and underlying dura. None of the patients showed complications in the follow-up computerized tomography scans. All patients had favorable aesthetic and functional results. CONCLUSIONS: Placing a titanium mesh as an extra step during DC could have antiadhesive and protective properties, facilitating subsequent cranioplasty by preventing adhesions and providing a clear surgical plane between the temporalis muscle and intracranial tissues. This technique also helps preserve the temporalis muscle and enhances functional and aesthetic outcomes postcranioplasty. Therefore, it represents a safe alternative to other synthetic anti-adhesive materials. Further studies are necessary to draw definitive conclusions and elucidate long-term outcomes, however, the results obtained hold great promise for the safety and efficacy of this technique.

Backup Frontal Drainage System for Urgent Tension Pneumocephalus Management After Chronic Subdural Hematoma Surgery: A Retrospective Cohort Study.

OBJECTIVE: To describe a simple variation of burr hole craniostomy for the management of chronic subdural hematoma (CSDH) that uses a frontal drainage system to facilitate timely decompression in the event of tension pneumocephalus and spares the need for additional surgery. METHODS: We conducted a retrospective analysis of 20 patients with CSDH who underwent burr hole craniostomy and 20 patients who underwent the same procedure alongside the placement of a 5 Fr neonatal feeding tube as a backup drainage for the anterior craniostomy. Depending on the situation, the secondary drain stayed for a maximum of 72 hours to be opened and used in emergency settings for drainage, aspiration, or as a 1-way valve with a water seal. RESULTS: The outcomes of 20 patients who underwent this procedure and 20 controls are described. One patient from each group presented tension pneumocephalus. One was promptly resolved by opening the backup drain under a water seal to evacuate pneumocephalus and the other patient had to undergo a reopening of the craniostomy. CONCLUSIONS: The described variation of burr hole craniostomy represents a low-cost and easy-to-implement technique that can be used for emergency decompression of tension pneumocephalus. It also has the potential to reduce reoperation rates and CSDH recurrence. Prospective controlled research is needed to validate this approach further.

A simple but effective solution for proximal mark absence on some microcatheters in intracranial aneurysm embolization: Technical note.

Background: Endovascular coil embolization is increasingly being used for the treatment of intracranial aneurysms and other pathologies such as arteriovenous (AV) malformations and AV fistulas. Appropriate embolization technique requires a microcatheter with two radiopaque marks, one proximal and one distal. We present an alternative coils deployment technique for intracranial aneurysms, using a microcatheter without a proximal radiopaque mark. Methods: We describe the technique for embolization that was used in a 36-year-old female patient, in which we used a microcatheter without a proximal radiopaque mark for coil embolization of an intracranial aneurysm. Results: We used a Headway Duo flow directed microcatheter for a coiling embolization of an intracranial aneurysm, solving the absence of the proximal radiopaque mark by cannulating the microcatheter with a Traxcess 0.014 microguidewire, and placing an external mark on the screen in the proximal portion of the microguidewire 30 mm radiopaque tip to indirectly mark the proximal mark of the microcatheter. Conclusion: There is scarce evidence supporting the use of microcatheters with no proximal radiopaque mark for coil embolization. This report attempts to disclose how an easy and simple technique can be used as a rescue method to solve the proximal radiopaque mark absence during endovascular coil release procedures. To the best of our knowledge, this technique has not been previously described; therefore, its use is not widespread among neurointerventionists.

Capsular warning syndrome and its clinical awareness and therapeutic approach: two case reports and a systematic review of the literature.

Introduction: Capsular warning syndrome (CWS) is characterized by recurrent stereotyped episodes of unilateral transient motor and/or sensory symptoms affecting the face and upper and lower limbs, without cortical signs in 24 h and with a high risk of developing stroke. Among the possible underlying mechanisms, small perforating artery disease is the most common. The aim was to assess the most common risk factors, the therapeutic alternatives, and the different outcomes in patients with CWS, along with the presentation of two cases treated in our Emergency Department. Methods: Stroke Code, launched at our institution in January 2017, was triggered 400 times, and by December 2022, 312 patients were admitted as having an acute ischemic stroke. Among them, two of them fulfilled the criteria of CWS. A systematic search was carried out in PubMed, Scopus, and Web of Science databases to seek demography and therapeutic approaches in CWS. Results: Of 312 cases, two with acute ischemic stroke exhibited CWS. The first patient had six events of right hemiparesis with recovery in 10-30 min; after MRI and digital subtraction angiography (DSA), he received apixaban and clopidogrel; however, a day after admission, he developed ischemic infarction with partial recovery. The second patient presented five transient events of right hemiparesis. After MRI and DSA with an intra-arterial infusion of nimodipine, oral aspirin, and ticagrelor, he presented another event-developing stroke and was discharged with partial recovery. A systematic review found 190 cases of CWS in 39 articles from 1993 to 2022. Most were male subjects (66.4%), and hypertension (60%), smoking (36%), diabetes (18%), and dyslipidemia (55%) were the most common risk factors. Over 50% of the cases were secondary to small perforating artery disease. The most commonly used treatments were dual antiplatelet therapy (DAT), recombinant tissue plasminogen activator, and anticoagulant therapy (ACT), where the combination of DAT plus ACT was linked to the most positive functional outcomes (82.6%). Conclusion: Our cases fit with the description of patients with partial recovery and risk factors (hypertension, diabetes, and smoking) in male patients. There is a lack of evidence regarding the best treatment option; dual antiplatelet therapy and anticoagulation therapy are strong contenders for a favorable result.

Primary Neuroendocrine Carcinoma of the Cerebellopontine Angle: A Case Report and Literature Review.

Primary intracranial neuroendocrine tumors are extremely rare malignancies with very few cases reported in the world literature. We describe a primary neuroendocrine carcinoma arising from the right cerebellopontine angle, the second case that has been described in this location. The possible origin in this place and treatment are described. A 29-year-old male patient, diagnosed with schwannoma of the right cerebellopontine angle, and treated with radiosurgery at another institution, came to our hospital six months later, The patient presented with a history of rapid progression of numbness on the right side of the face, diplopia, dizziness, vomiting, and facial palsy. On examination, the right cranial nerves V, VI, VII, VIII, and IX were affected. The MRI showed tumor growth occupying the right cerebellopontine angle, with compression of the brain stem and cerebellum. A right retromastoid craniectomy removed the tumor partially and the histopathological examination revealed a high-grade neuroendocrine carcinoma. We describe a primary neuroendocrine tumor of the brain that, despite its rarity, must be considered in the differential diagnosis. There are currently no guidelines for the management of these tumors. According to previously reported cases, surgery is the first line of treatment, followed by radiotherapy or chemotherapy. We consider that such a rare case is needed to be reported for a better understanding of the disease and its neurobiology.

Minimally Invasive versus Open Surgery for Spinal Metastasis: A Systematic Review and Meta-Analysis.

Bones are the third most common location for solid tumor metastasis affecting up to 10% of patients with solid tumors. When the spine is involved, thoracic and lumbar vertebrae are frequently affected. Access to spinal lesions can be through minimally invasive surgery (MIS) or traditional open surgery (OS). This study aims to determine which method provides an advantage. Following the PRISMA (Preferred Inventory for Systematic Reviews and Meta-Analysis) guidelines, a systematic review was conducted to identify studies that compare MIS with OS in patients with spinal metastatic disease. Data were analyzed using Review Manager ver. 5.3 (RevMan; Cochrane, London, UK). Ten studies were included. Operative time was similar among groups at -35.23 minutes (95% confidence interval [CI], -73.36 to 2.91 minutes; p=0.07). Intraoperative bleeding was lower in MIS at -562.59 mL (95% CI, -776.97 to -348.20 mL; p<0.00001). OS procedures had higher odds of requiring blood transfusions at 0.26 (95% CI, 0.15 to 0.45; p<0.00001). Both approaches instrumented similar numbers of levels at -0.05 levels (95% CI, -0.75 to 0.66 levels; p=0.89). We observed a decreased need for postoperative bed rest at -1.60 days (95% CI, -2.46 to -0.74 days; p=0.0003), a shorter length of stay at -3.08 days (95% CI, -4.50 to -1.66 days; p=0.001), and decreased odds of complications at 0.60 (95% CI, 0.37 to 0.96; p=0.03) in the MIS group. Both approaches revealed similar reintervention rates at 0.65 (95% CI, 0.15 to 2.84; p=0.57), effective rates of reducing metastasis-related pain at -0.74 (95% CI, -2.41 to 0.94; p=0.39), and comparable scores of the Tokuhashi scale at -0.52 (95% CI, -2.08 to 1.05; p=0.41), Frankel scale at 1.00 (95% CI, 0.60 to 1.68; p=1.0), and American Spinal Injury Association Scale at 0.53 (95% CI, 0.21 to 1.37; p=0.19). MIS appears to provide advantages over OS. Larger and prospective studies should fully detail the role of MIS as a treatment for spine metastasis.

Knowledge of ischemic stroke risk factors and warning signs after a health education program by medical students.

BACKGROUND AND PURPOSE: A delay in recognizing early warning signs (WS) and risk factors (RF) of ischemic stroke causes a delay in treatment. We evaluated knowledge of RF and WS and the impact of an educational program by medical students. METHODS: We first surveyed individuals to determine knowledge of WS and RF. Then, after a 6-month education program, knowledge was reassessed. The questionnaire included sociodemographic and comorbidity data. A χ(2) and Mann-Whitney U test, as well as a multivariate logistic regression analysis to determine variables associated with knowledge, were used. RESULTS: We performed 329 baseline and 355 posteducation surveys. Initially, 57.1% mentioned at least 1 RF; this later increased to 65.9%. Mentions of obesity, dyslipidemias, hypertension, and diabetes mellitus increased significantly. With regard to WS, 37.6% mentioned at least 1, which increased to 48.1% who mentioned weakness in 1 limb, in half the body, severe headache, and altered vision. Educational level (OR, 2.53; 95% CI, 1.42-4.53; P=0.001), employment (OR, 1.72; 95% CI, 1.08-2.74; P=0.021), a family history of brain infarction (OR, 2.35; 95% CI, 1.35-4.11; P=0.02), obesity (OR, 1.63; 95% CI, 1.026-2.6; P=0.038), and having received information in the last 6 months (OR, 2.7; 95% CI, 1.51-4.83; P=0.001) were associated with a better understanding of RF and WS. CONCLUSIONS: The educational program was cost-effective and had a positive impact on knowledge of RF and WS of ischemic stroke. More education programs are required to improve knowledge of ischemic stroke.

Survival and clinical features in Hispanic amyotrophic lateral sclerosis patients.

The demography, survival, and motor phenotypes of amyotrophic lateral sclerosis (ALS) patients have been rarely described in Hispanic countries. The clinical characteristics and survival of a series of Mexican ALS patients are described. Mexican patients with definite ALS were included in a five-year retrospective longitudinal study. Their demographic and clinical features, cumulative survival rates, and independent predictive factors for survival were analysed. Sixty-one definite ALS patients were included. The median follow-up period was 35 months (range 12-108 months). Males were predominant (1.8: 1), the mean age at onset was 47.5 ± 10.5 years, and the median interval from onset to diagnosis was 12 months. Spinal onset occurred in 66% of patients. Upper motor neuron phenotype was predominant in 53% of patients. The overall mean survival from onset was 68.6 months, and from diagnosis was 57.8 months. Longer survival was determined in patients aged ≤ 40 years (54.7 months) compared with other age groups (p = 0.006). In conclusion, the clinical heterogeneity, male predominance, and survival rates in our sample are consistent with those of other studies. Patients in this series had a younger age at onset and a clear trend toward longer survival compared with those of other population studies.

Aneurysms of the Lenticulostriate Artery: A Systematic Review.

BACKGROUND: Lenticulostriate artery aneurysms (LSAs) are rare vascular aberrations. Despite the potentially catastrophic sequelae of aneurysmal rupture, the optimal management strategy for LSA aneurysms has not been determined. The aim of the present review is to provide an overview of the clinical presentation and treatment strategies for LSA aneurysms. METHODS: Using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, a literature search was conducted in the PubMed, Cochrane, EBSCOhost, Scopus, Web of Science, and ProQuest search engines to identify reported studies of LSA aneurysms until July 1, 2020. A descriptive analysis was performed. RESULTS: A total of 71 studies with 112 cases of LSA aneurysms were included. Patient age ranged from 2 months to 83 years (median, 44.5 years). Male and female patients were affected similarly (49% and 51%, respectively). The most common presentation was aneurysmal rupture (78%), and headache was the most frequently reported symptom (36%). Overall, 48% of the patients had undergone underwent surgical treatment, 30% conservative management, 21% endovascular treatment, and 1% radiosurgery. Four patients died, all of whom had presented with aneurysmal rupture. CONCLUSION: We have summarized the reported cases of LSA aneurysms, with their clinical presentation, management, and outcomes, for physicians who may be confronted with this diagnosis. Future studies that use available classification systems and include as much detail as possible should be encouraged to fully elucidate the optimal management strategy for these patients.

Balo's Concentric Sclerosis with monophasic course: A report of 2 cases.

INTRODUCTION: Balo's Concentric Sclerosis (BCS) is a rare demyelinating disease sometimes considered a variant of multiple sclerosis. It is characterized by an acute or subacute neurological symptoms with characteristic MRI "onion-like" white matter lesions. BCS has a wide range of presentations but is mostly self-limiting. Steroids are indicated in patients with aggressive disease. CASE PRESENTATION: We report 2 cases of BCS with monophasic course of stroke-like symptoms and single periventricular concentric lamella with onion-like appearance on MRI without inflammatory reaction in the CSF. They were treated with corticosteroids achieving clinical improvement and without neurological deficit or relapse over the following years. CLINICAL DISCUSSION: A number of cases of BCS are described in the literature that show marked recovery with early diagnosis and treatment with steroids. CONCLUSION: BCS appears to have a good prognosis when treated early in its diagnosis with steroids.

Evaluation of a P300-Based Brain-Machine Interface for a Robotic Hand-Orthosis Control.

This work presents the design, implementation, and evaluation of a P300-based brain-machine interface (BMI) developed to control a robotic hand-orthosis. The purpose of this system is to assist patients with amyotrophic lateral sclerosis (ALS) who cannot open and close their hands by themselves. The user of this interface can select one of six targets, which represent the flexion-extension of one finger independently or the movement of the five fingers simultaneously. We tested offline and online our BMI on eighteen healthy subjects (HS) and eight ALS patients. In the offline test, we used the calibration data of each participant recorded in the experimental sessions to estimate the accuracy of the BMI to classify correctly single epochs as target or non-target trials. On average, the system accuracy was 78.7% for target epochs and 85.7% for non-target trials. Additionally, we observed significant P300 responses in the calibration recordings of all the participants, including the ALS patients. For the BMI online test, each subject performed from 6 to 36 attempts of target selections using the interface. In this case, around 46% of the participants obtained 100% of accuracy, and the average online accuracy was 89.83%. The maximum information transfer rate (ITR) observed in the experiments was 52.83 bit/min, whereas that the average ITR was 18.13 bit/min. The contributions of this work are the following. First, we report the development and evaluation of a mind-controlled robotic hand-orthosis for patients with ALS. To our knowledge, this BMI is one of the first P300-based assistive robotic devices with multiple targets evaluated on people with ALS. Second, we provide a database with calibration data and online EEG recordings obtained in the evaluation of our BMI. This data is useful to develop and compare other BMI systems and test the processing pipelines of similar applications.

Coiling as a Rescue Strategy for Flow Diverter Prolapse into a Giant Intracranial Aneurysm.

BACKGROUND: Up to 3.2% of the adult population has an unruptured intracranial aneurysm (IA). Flow diversion is a relatively new treatment technique that is especially useful for large and morphologically unfavorable IAs. METHODS: A previously healthy woman aged 32 years presented with a 6-month history of ptosis and ophthalmoplegia of the left eye. A magnetic resonance imaging scan revealed a giant left internal carotid artery aneurysm. She was admitted for treatment using flow diversion. After delivery of the flow diverter (FD), prolapse of the proximal end of the stent into the aneurysmal sac was observed. FD stabilization was achieved by deploying multiple coils through a previously placed microcatheter to push the prolapsed end away from the aneurysmal lumen. RESULTS: The patient had a favorable outcome, with reduction of preoperative mass effect symptoms and complete obliteration of the aneurysm persisting at the 3-month follow-up. CONCLUSIONS: Giant IAs remain one of the most daunting clinical problems to treat. FD displacement is a rare (0.5%-0.75%) and possibly fatal complication. Currently, no clinical guidelines exist for its management. Adjunctive coiling is a possible rescue strategy for stabilizing an FD that foreshortened and prolapsed into the aneurysmal sac. Further studies are needed to identify the best approach to this complication.