Search

The article focuses on an 18-year-old male presenting with acute altered mental status to an emergency room, detailing his medical history and initial symptoms. Topics include severe microcytic anemia prompting further investigation, empirical treatment for potential infections, and additional symptoms discovered during hospital admission.

Background: Patients presenting to the emergency department with acute vertigo pose a diagnostic challenge. While 'benign' peripheral vestibulopathy is the most common cause, the possibility of a posterior circulation stroke is paradoxically the most feared and missed diagnosis in the emergency department. Objectives: This review will attempt to cover the significant advances in the ability to diagnose acute vertigo that have occurred in the last two decades. The review discusses the role of neurological examinations, imaging and specific oculomotor examinations. The review then discusses the relative attributes of the Head Impulse-Nystagmus-Test of Skew plus hearing ('HINTS+') examination, the timing, triggers and targeted bedside eye examinations ('TiTrATE'), the associated symptoms, timing and triggers, examination signs and testing ('ATTEST') algorithm, and the spontaneous nystagmus, direction, head impulse testing and standing ('STANDING') algorithm. The most recent technological advancements in video-oculography guided care are discussed, as well as other potential advances for clinicians to look out for. [ABSTRACT FROM AUTHOR]

Background: Iatrogenic malnutrition is a significant burden to patients, clinicians, and health care systems. Compared with well-nourished patients, underfed patients (those who receive less than 80% of their daily energy requirement) have more adverse outcomes related to nutritional status. Volume-based protocols allow for catch-up titrations, are consistently superior to rate-based protocols, and can be implemented in most settings. Local Problem: This project was conducted in an 8-bed neuroscience intensive care unit in which up to 41% of patients who required enteral feeding were underfed. Methods: This quality improvement clinical practice change project used a before-and-after design to evaluate (1) the effect of implementing a volume-based feeding protocol on the delivery of enteral feeds and (2) the effect of a nutrition-based project on staff members' attitudes regarding nutrition in critical care. The effectiveness of a volume-based feeding titration protocol was compared with that of a rate-based feeding protocol for achieving delivery of at least 80% of prescribed nutrition per 24-hour period. Staff members' attitudes were assessed using a survey before and after the project. Results: During 241 enteral feeding days (n = 40 patients), the percentage of delivered enteral feeding volume and the percentage of days patients received at least 80% of the prescribed volume increased after volume-based feeding was implemented. After project implementation, 74 staff members reported increased emphasis on nutrition delivery in their practice and a higher level of agreement that nutrition is a priority when caring for critically ill patients. Conclusions: Using a volume-based feeding protocol with supplemental staff education resulted in improved delivery of prescribed enteral feeding. [ABSTRACT FROM AUTHOR]

This article presents a case study of a former professional football player who developed chronic traumatic encephalopathy (CTE) later in life. The study provides detailed information about the patient's symptoms, cognitive decline, and functional impairments, as well as findings from neuroimaging and neuropathological examinations. The article emphasizes the need for further research to better understand CTE and its associated pathologies. It also suggests that FDG-PET imaging may be a useful diagnostic tool for early-stage CTE and highlights the potential link between traumatic brain injury and other brain pathologies. [Extracted from the article]

It is not known why some patients develop persistent pain after nerve trauma while others do not. Among multiple risk factors for the development of persistent posttrauma and postsurgical pain, a neuropathic mechanism due to iatrogenic nerve lesion has been proposed as the major cause of these conditions. Because there is some evidence that the human leukocyte antigen (HLA) system plays a role in persistent postsurgical pain, this study aimed to identify the genetic risk factors, specifically among HLA loci, associated with chronic neuropathic pain after traumatic nerve injuries and surgery in the upper extremities. Blood samples were taken to investigate the contribution of HLA alleles (ie, HLA-A, HLA-B, HLA-DRB1, HLA-DQB1, and HLA-DPB1) in a group of patients with persistent neuropathic pain (n = 70) and a group of patients with neuropathy without pain (n = 61). All subjects had intraoperatively verified nerve damage in the upper extremity. They underwent bedside clinical neurological examination to identify the neuropathic pain component according to the present grading system of neuropathic pain. Statistical analyses on the allele and haplotype were conducted using the BIGDAWG package. We found that the HLA haplotype A*02:01-B*15:01-C*03:04-DRB1*04: 01-DQB1*03:02 was associated with an increased risk of developing persistent neuropathic pain in the upper extremity (OR = 9.31 [95% CI 1.28-406.45], P < 0.05). No significant associations were found on an allele level when correcting for multiple testing. Further studies are needed to investigate whether this association is on a haplotypic level or if certain alleles may be causing the association. [ABSTRACT FROM AUTHOR]

Background: Percutaneous transpedicular screw fixation was recently introduced as a minimally invasive approach for the treatment of thoracolumbar spine fracture. This approach is superior to the traditional open surgical approaches in decreasing intraoperative blood loss, infection rates, and paraspinal muscle damage with less hospital stay. Objective: This study aims to report our surgical experience, the feasibility and safety of percutaneous transpedicular screw fixation of thoracolumbar spine fractures. Method: we retrospectively reviewed the charts of all adult patients with thoracolumbar spine fractures who underwent percutaneous transpedicular screw fixation during the period between January 2020 to January 2023 at Ain Shams University Hospitals and Prince Mohamed Ibn Abdelaziz Hospital. Collected data included: demographics, pre- and postoperative neurological assessment, radiological data including type of fracture and cobb's angle pre- and postoperatively, operative time and amount of intraoperative blood loss. Results: Forty-three percutaneous pedicle screw fixation procedures were done for 42 patients. Thirty patients were males. Dorsal spine fractures accounted for 18 cases. Preoperative American spinal injury Association score was E in 37 cases. Mean operative time was 133 min (± 46.5), and mean intraoperative blood loss was 107.1 ml (± 84). The mean follow-up period was 8 months, and the average hospital stay was 7.7 days. Radiological complete bony fusion was achieved in 77% of fractures, and 93% had improved kyphotic angle with a mean correction degree of 7.9 (± 9). Conclusion: Percutaneous transpedicular screw fixation is a safe and effective treatment option for the management of thoracolumbar spine fractures with or without spine decompression that provides satisfactory functional outcomes. [ABSTRACT FROM AUTHOR]

Hypoxic-ischemic encephalopathy (HIE) is a major cause of newborn brain damage stemming from a lack of oxygenated blood flow in the neonatal period. Twenty-five to fifty percent of asphyxiated infants who develop HIE die in the neonatal period, and about sixty percent of survivors develop long-term neurological disabilities. From the first minutes to months after the injury, a cascade of events occurs, leading to blood-brain barrier (BBB) opening, neuronal death and inflammation. To date, the only approach proposed in some cases is therapeutic hypothermia (TH). Unfortunately, TH is only partially protective and is not applicable to all neonates. This review synthesizes current knowledge on the basic molecular mechanisms of brain damage in hypoxia-ischemia (HI) and on the different therapeutic strategies in HI that have been used and explores a major limitation of unsuccessful therapeutic approaches. [ABSTRACT FROM AUTHOR]

Background: While depression is increasing worldwide, some patients are diagnosed as having Major Depressive Disorder (MDD), but others are diagnosed with minor depression, however, the potential neuro mechanism is unknown. Methods: Sixty-two patients with minor depression, 44 adolescents with MDD and 54 healthy adolescents participated in this study. Functional near-infrared spectroscopy (fNIRS), both HAMD and HAMA data were collected from all of the participants. Results: The result indicates the pervasively decreased activation of BA, 11, 21, 45 and 46 were observed in the MDD group and reduced activation of BA 45 was observed in the minor depression group. However, cortical activation was not observed between the minor depression or MDD groups. Cortical activation was also not correlated with the depressive/anxious score in the minor and MDD groups separately. Conclusions: Cortical activation was pervasively decreased in the MDD group and slightly reduced in the minor depression group, which may be a potential neural mechanism. As reduced cortical activation in minor depression, interventions in the early stages of minor depression may help slow or even modify the development of the illness. [ABSTRACT FROM AUTHOR]

Background: Recently, diagnostic criteria including a standardized MRI criterion were presented to identify patients suffering from idiopathic intracranial hypertension (IIH) proposing that IIH might be defined by two out of three objective findings (papilledema, ≥ 25 cm cerebrospinal fluid opening pressure (CSF-OP) and ≥ 3/4 neuroimaging signs). Methods: To provide independent external validation, we retrospectively applied the proposed diagnostic criteria to our cohort of patients with clinical suspicion of IIH from the Vienna IIH database. Neuroimaging was reevaluated for IIH signs according to standardized definitions by a blinded expert neuroradiologist. We determined isolated diagnostic accuracy of the neuroimaging criterion (≥ 3/4 signs) as well as overall accuracy of the new proposed criteria. Results: We included patients with IIH (n = 102) and patients without IIH (no-IIH, n = 23). Baseline characteristics were balanced between IIH and no-IIH groups, but papilledema and CSF-OP were significantly higher in IIH. For the presence of ≥ 3/4 MRI signs, sensitivity was 39.2% and specificity was 91.3% with positive predictive value (PPV) of 95.2% and negative predictive value (NPV) 25.3%. Reclassifying our cohort according to the 2/3 IIH definition correctly identified 100% of patients without IIH, with definite IIH and suggested to have IIH without papilledema by Friedman criteria, respectively. Conclusion: The standardized neuroimaging criteria are easily applicable in clinical routine and provide moderate sensitivity and excellent specificity to identify patients with IIH. Defining IIH by 2/3 criteria significantly simplifies diagnosis without compromising accuracy. [ABSTRACT FROM AUTHOR]

Background: Fragile X-associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder caused by CGG repeat expansion of FMR1 gene. Both FXTAS and neuronal intranuclear inclusion disease (NIID) belong to polyglycine diseases and present similar clinical, radiological, and pathological features, making it difficult to distinguish these diseases. Reversible encephalitis-like attacks are often observed in NIID. It is unclear whether they are presented in FXTAS and can be used for differential diagnosis of NIID and FXTAS. Case presentation: A 63-year-old Chinese male with late-onset gait disturbance, cognitive decline, and reversible attacks of fever, consciousness impairment, dizziness, vomiting, and urinary incontinence underwent neurological assessment and examinations, including laboratory tests, electroencephalogram test, imaging, skin biopsy, and genetic test. Brain MRI showed T2 hyperintensities in middle cerebellar peduncle and cerebrum, in addition to cerebellar atrophy and DWI hyperintensities along the corticomedullary junction. Lesions in the brainstem were observed. Skin biopsy showed p62-positive intranuclear inclusions. The possibilities of hypoglycemia, lactic acidosis, epileptic seizures, and cerebrovascular attacks were excluded. Genetic analysis revealed CGG repeat expansion in FMR1 gene, and the number of repeats was 111. The patient was finally diagnosed as FXTAS. He received supportive treatment as well as symptomatic treatment during hospitalization. His encephalitic symptoms were completely relieved within one week. Conclusions: This is a detailed report of a case of FXTAS with reversible encephalitis-like episodes. This report provides new information for the possible and rare features of FXTAS, highlighting that encephalitis-like episodes are common in polyglycine diseases and unable to be used for differential diagnosis. [ABSTRACT FROM AUTHOR]

Aim: To detect if we can use the reduction in the optic disc vessel density as an indicator to the reduction in intracranial tension in patients with residual optic disc elevation after shunt surgery as fundus examination in those cases is not conclusive. Patients and method: 21 patients with papilledema due to idiopathic intracranial hypertension underwent shunt surgery. Full neurological and ophthalmological assessments were done. The optic disc vessel density was measured before and 3 months after surgery. Patients were then divided according to the resolution of papilledema into 2 groups: 1) Residual disc elevation group. 2) Completely resolved disc edema group. CSF pressure was measured via lumber puncture preoperative for all patients and 3 months post-operative only for patients with residual disc edema. A comparison between both groups was done. Results: There was a highly statistically significant difference between the two groups as regard the papilledema grade (the residual disc elevation group had a higher grade of papilledema) with P-value=0.000. As regard the difference in the preoperative optic disc vessel density between the two groups, there were statistically significant differences (optic disc vessel density was more in the residual disc elevation group). As regard the postoperative optic disc vessel density, there were non-significant differences between the two groups in whole image, inside disc and peripapillary vessel density (either in macro or microvasculature). Conclusion: The optic disc vessel density decreased with normal postoperative CSF opening pressure in cases with residual disc elevation postoperatively. Thus, in cases of residual optic disc swelling after shunt surgery, we can detect the reduction of intracranial pressure by the reduction in the optic disc vessel density which is a safe non-invasive technique. That may help in cases of residual disc elevation. [ABSTRACT FROM AUTHOR]

Cervical spinal cord injury (SCI) causes devastating loss of upper limb function and independence. Restoration of upper limb function can have a profound impact on independence and quality of life. In low-cervical SCI (level C5–C8), upper limb function can be restored via reinnervation strategies such as nerve transfer surgery. The translation of recovered upper limb motor function into functional independence in activities of daily living (ADLs), however, remains unknown in low cervical SCI (i.e., tetraplegia). The objective of this study was to evaluate the association of patterns in upper limb motor recovery with functional independence in ADLs. This will then inform prioritization of reinnervation strategies focused to maximize function in patients with tetraplegia. This retrospective study performed a secondary analysis of patients with low cervical (C5–C8) enrolled in the SCI Model Systems (SCIMS) database. Baseline neurological examinations and their association with functional independence in major ADLs—i.e., eating, bladder management, and transfers (bed/wheelchair/chair)—were evaluated. Motor functional recovery was defined as achieving motor strength, in modified research council (MRC) grade, of ≥ 3 /5 at one year from ≤ 2/5 at baseline. The association of motor function recovery with functional independence at one-year follow-up was compared in patients with recovered elbow flexion (C5), wrist extension (C6), elbow extension (C7), and finger flexion (C8). A multi-variable logistic regression analysis, adjusting for known factors influencing recovery after SCI, was performed to evaluate the impact of motor function at one year on a composite outcome of functional independence in major ADLs. Composite outcome was defined as functional independence measure score of 6 or higher (complete independence) in at least two domains among eating, bladder management, and transfers. Between 1992 and 2016, 1090 patients with low cervical SCI and complete neurological/functional measures were included. At baseline, 67% of patients had complete SCI and 33% had incomplete SCI. The majority of patients were dependent in eating, bladder management, and transfers. At one-year follow-up, the largest proportion of patients who recovered motor function in finger flexion (C8) and elbow extension (C7) gained independence in eating, bladder management, and transfers. In multi-variable analysis, patients who had recovered finger flexion (C8) or elbow extension (C7) had higher odds of gaining independence in a composite of major ADLs (odds ratio [OR] = 3.13 and OR = 2.87, respectively, p < 0.001). Age 60 years (OR = 0.44, p = 0.01), and complete SCI (OR = 0.43, p = 0.002) were associated with reduced odds of gaining independence in ADLs. After cervical SCI, finger flexion (C8) and elbow extension (C7) recovery translate into greater independence in eating, bladder management, and transfers. These results can be used to design individualized reinnervation plans to reanimate upper limb function and maximize independence in patients with low cervical SCI. [ABSTRACT FROM AUTHOR]

Background: Treatment options available for meningoencephalitis of unknown origin (MUO) in dogs are suboptimal, and currently, no single treatment protocol appears to be superior. Objectives: Compare neurological deterioration rates at 7 days between dogs with MUO treated with corticosteroids alone or combined with cytosine arabinoside (CA) continuous rate infusion (CRI) and compare clinical deterioration and survival at 30 and 100 days. Animals: Sixty‐nine dogs with magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) features or both compatible with MUO. Methods: Parallel, blinded, randomized controlled trial. Simple randomization into 2 treatment groups: 4 mg/kg/day prednisolone (or dexamethasone equivalent) for 2 days or 200 mg/m2 CA CRI over 8 hours plus 2 mg/kg/day prednisolone. Blinding of the treatment protocol was carried out using reversible redaction of clinical records, and treatment failure was defined as deterioration of neurological assessment or death. Using intention‐to‐treat analysis, proportions failing treatment at 7, 30, and 100 days were compared using Fisher's exact test. All‐cause mortality at 100 days was compared using Kaplan‐Meier survival curves. Results: Thirty‐five dogs were allocated to corticosteroid only, and 34 dogs were allocated to combined CA CRI and corticosteroid. Proportions failing treatment at 7, 30, and 100 days were 7/35 (20%), 9/35 (26%), and 15/35 (43%) in the corticosteroid‐only group and 8/34 (24%), 11/34 (32%), and 23/34 (68%) in the corticosteroid and CA CRI group. All‐cause mortality at 100 days was not significantly different between groups (P =.62). Clinically relevant treatment‐related adverse effects were not observed. Conclusions and Clinical Importance: We found no difference in outcome between corticosteroid monotherapy and combined cytarabine CRI and corticosteroid therapy at 7, 30, and 100 days after diagnosis in dogs with MUO. [ABSTRACT FROM AUTHOR]

Background: Equine neuroaxonal dystrophy/degenerative myeloencephalopathy (eNAD/EDM) is a neurodegenerative disease that primarily affects young, genetically predisposed horses that are deficient in vitamin E. Equine NAD/EDM has not previously been documented in Gypsy Vanner horses (GVs). Objectives: To evaluate: (1) the clinical phenotype, blood vitamin E concentrations before and after supplementation and pedigree in a cohort of GV horses with a high prevalence of neurologic disease suspicious for eNAD/EDM and (2) to confirm eNAD/EDM in GVs through postmortem evaluation. Animals: Twenty‐six GVs from 1 farm in California and 2 cases from the Midwestern U.S. Methods: Prospective observational study on Californian horses; all 26 GVs underwent neurologic examination. Pre‐supplementation blood vitamin E concentration was assessed in 17‐ GVs. Twenty‐three were supplemented orally with 10 IU/kg of liquid RRR‐alpha‐tocopherol once daily for 28 days. Vitamin E concentration was measured in 23 GVs after supplementation, of which 15 (65%) had pre‐supplementation measurements. Two clinically affected GVs from California and the 2 Midwestern cases had necropsy confirmation of eNAD/EDM. Results: Pre‐supplementation blood vitamin E concentration was ≤2.0 μg/mL in 16/17 (94%) of GVs from California. Post‐supplementation concentration varied, with a median of 3.39 μg/mL (range, 1.23‐13.87 μg/mL), but only 12/23 (52%) were normal (≥3.0 μg/mL). Normalization of vitamin E was significantly associated with increasing age (P =.02). Euthanized horses (n = 4) had eNAD/EDM confirmed at necropsy. Conclusions and Clinical Importance: GVs could have a genetic predisposition to eNAD/EDM. Vitamin E supplementation should be considered and monitored in young GVs. [ABSTRACT FROM AUTHOR]

A type A aortic dissection (TAAD) is a dangerous condition requiring emergency surgery. Due to the similarity of the symptoms of cerebral malperfusion in TAAD and the signs of ischemic stroke, a differential diagnosis of these diseases is not always available. Patients with TAAD after cerebral malperfusion can have a neurological deficit. Thrombolysis is performed in this case. It can worsen the patient's condition and increase the risk of mortality and disability. The aim of the study is to evaluate the new approach to restoring cerebral perfusion during aortic dissection. This approach includes endovascular recanalization and carotid stenting. Methods: Two clinical cases of TAAD complicated by cerebral malperfusion are described. The first patient is 73 years old and was admitted as planned to perform transcatheter aortic valve implantation (TAVI) for grade III aortic stenosis. The patient underwent transcatheter aortic valve implantation (TAVI) on the second day after admission. The second patient is 60 years old and was hospitalized by an ambulance with strong hypertension and ischemia. The surgical correction of aortic dissection was postponed until the neurological status assessment in both patients. Results: The surgery to correct the aorta dissection was deemed inappropriate. The carotid arteries have been reanalyzed, and cerebral perfusion has been restored in a short time in both patients. Conclusion: Acute bilateral internal carotid occlusion is a potentially fatal TAAD outcome. Emergency endovascular recanalization and carotid stenting may be considered one of the few ways to restore cerebral perfusion. [ABSTRACT FROM AUTHOR]

Background The implantation of a spacer is a common practice after anterior diskectomy in cervical spine. Polyether ether ketone (PEEK) cages have replaced titanium implants due to their better radiologic visibility and appearance in postoperative magnetic resonance imaging (MRI) scans. However, PEEK showed apparently higher nonunion rates than titanium cages. The aim of the study was to evaluate the fusion behavior of plain PEEK cages in comparison to titanium-coated PEEK (TiPEEK) cages. Method We randomized 104 patients with single-level cervical radiculopathy or mild myelopathy. They were divided into two groups of 52 patients each, receiving either a PEEK cage or the titanium-coated variant of the same cage type. The 1- and 2-year follow-ups were completed by 43 patients in the PEEK group and by 50 patients in the TiPEEK group. Fusion was determined by plain X-ray and lateral functional X-ray. Results Two years after surgery, a complete fusion was observed in 37 patients of the PEEK group (86%). Six cases were considered as nonunions. In the TiPEEK group, we found 41 fusions (82%) and 9 nonunions at this time. The difference was not considered significant (p = 0.59). The clinical evaluation of the two groups showed no difference in the neurologic examination as well in the pain scores over the time period. Conclusions Despite some assumptions about an advantage of TiPEEK over PEEK cages for fusion in cervical spine surgery, this prospective randomized controlled study did not find an accelerated or improved fusion using TiPEEK for anterior cervical diskectomy. [ABSTRACT FROM AUTHOR]

Objective: To investigate the effects of an adjuvant allogenic umbilical cord mesenchymal stromal cell (UC‐MSC) patch applied during fetal surgery on motor and sphincter function in the ovine MMC model. Design: MMC defects were surgically created at 75 days of gestation and repaired 14 days later. Population: Ovine MMC model: fetal lambs. Methods: We compared lambs that received a UC‐MSC patch with a control group of lambs that received an acellular patch. Main Outcome Measures: Clinical neurological assessment was performed at 2 and 24 hours of life and included determination of the Sheep Locomotor Rating scale (SLR), which has been validated in the ovine MMC model. Electrophysical examinations, spine scans and histological analyses were also performed. Results: Of the 13 operated lambs, nine were born alive: five had of these had received a UC‐MSC patch and four an acellular patch. At 24 hours of life, lambs in the UC‐MSC group had a significantly higher score (14 versus 5, P = 0.04). Amyotrophy was significantly more common in the control group (75% versus 0%, P = 0.02). All the lambs in the control group and none of those in the UC‐MSC group were incontinent. No significant differences were observed between the UC‐MSC and control groups in terms of the presence of spontaneous EMG activity, nerve conduction or spinal evoked potentials. In the microscopic examination, lambs in the UC‐MSC group had less fibrosis between the spinal cord and the dermis (mean thickness, 453 versus 3921 μm, P = 0.03) and around the spinal cord (mean thickness, 47 versus 158 μm, P < 0.001). Examination of the spinal cord in the area of the MMC defect showed a higher large neuron density in the UC‐MSC group (14.5 versus 5.6 neurons/mm2, P < 0.001). No tumours were observed. Conclusions: Fetal repair of MMC using UC‐MSC patches improves motor and sphincter function as well as spinal preservation and reduction of fibrosis. Linked article: This article is commented on by Anna L. David, pp. 768 in this issue. To view this mini commentary visit https://doi.org/10.1111/1471‐0528.17730. [ABSTRACT FROM AUTHOR]

Traumatic brain injury (TBI) significantly contributes to death and disability worldwide. However, treatment options remain limited. Here, we focus on a specific pathology of TBI, diffuse axonal brain injury (DABI), which describes the process of the tearing of nerve fibers in the brain after blunt injury. Most protocols to study DABI do not incorporate a specific model for that type of pathology, limiting their ability to identify mechanisms and comorbidities of DABI. In this study, we developed a magnetic resonance imaging (MRI) protocol for DABI in a rat model using a 3-T clinical scanner. We compared the neuroimaging outcomes with histologic and neurologic assessments. In a sample size of 10 rats in the sham group and 10 rats in the DABI group, we established neurological severity scores before the intervention and at 48 h following DABI induction. After the neurological evaluation after DABI, all rats underwent MRI scans and were subsequently euthanized for histological evaluation. As expected, the neurological assessment showed a high sensitivity for DABI lesions indicated using the β-APP marker. Surprisingly, however, we found that the MRI method had greater sensitivity in assessing DABI lesions compared to histological methods. Out of the five MRI parameters with pathological changes in the DABI model, we found significant changes compared to sham rats in three parameters, and, as shown using comparative tests with other models, MRI was the most sensitive parameter, being even more sensitive than histology. We anticipate that this DABI protocol will have a significant impact on future TBI and DABI studies, advancing research on treatments specifically targeted towards improving patient quality of life and long-term outcomes. [ABSTRACT FROM AUTHOR]

This cross-sectional study evaluates the rate of detection of cutaneous phosphorylated α-synuclein by skin biopsy in individuals with and without synucleinopathy. Key Points: Question: Can skin biopsy detect phosphorylated α-synuclein in individuals with synucleinopathies such as Parkinson disease (PD), multiple system atrophy (MSA), dementia with Lewy bodies (DLB), and pure autonomic failure (PAF)? Finding: In this cross-sectional study of 428 participants, the proportions of individuals with cutaneous phosphorylated α-synuclein detected by skin biopsy were 92.7% with PD, 98.2% with MSA, 96.0% with DLB, 100% with PAF, and 3.3% with no history of synucleinopathy (controls). Meaning: A high proportion of individuals meeting clinical consensus criteria for PD, DLB, MSA, and PAF had a skin biopsy positive for phosphorylated α-synuclein in this study, although further research is needed to validate the findings in unselected clinical populations. Importance: Finding a reliable diagnostic biomarker for the disorders collectively known as synucleinopathies (Parkinson disease [PD], dementia with Lewy bodies [DLB], multiple system atrophy [MSA], and pure autonomic failure [PAF]) is an urgent unmet need. Immunohistochemical detection of cutaneous phosphorylated α-synuclein may be a sensitive and specific clinical test for the diagnosis of synucleinopathies. Objective: To evaluate the positivity rate of cutaneous α-synuclein deposition in patients with PD, DLB, MSA, and PAF. Design, Setting, and Participants: This blinded, 30-site, cross-sectional study of academic and community-based neurology practices conducted from February 2021 through March 2023 included patients aged 40 to 99 years with a clinical diagnosis of PD, DLB, MSA, or PAF based on clinical consensus criteria and confirmed by an expert review panel and control participants aged 40 to 99 years with no history of examination findings or symptoms suggestive of a synucleinopathy or neurodegenerative disease. All participants completed detailed neurologic examinations and disease-specific questionnaires and underwent skin biopsy for detection of phosphorylated α-synuclein. An expert review panel blinded to pathologic data determined the final participant diagnosis. Exposure: Skin biopsy for detection of phosphorylated α-synuclein. Main Outcomes: Rates of detection of cutaneous α-synuclein in patients with PD, MSA, DLB, and PAF and controls without synucleinopathy. Results: Of 428 enrolled participants, 343 were included in the primary analysis (mean [SD] age, 69.5 [9.1] years; 175 [51.0%] male); 223 met the consensus criteria for a synucleinopathy and 120 met criteria as controls after expert panel review. The proportions of individuals with cutaneous phosphorylated α-synuclein detected by skin biopsy were 92.7% (89 of 96) with PD, 98.2% (54 of 55) with MSA, 96.0% (48 of 50) with DLB, and 100% (22 of 22) with PAF; 3.3% (4 of 120) of controls had cutaneous phosphorylated α-synuclein detected. Conclusions and Relevance: In this cross-sectional study, a high proportion of individuals meeting clinical consensus criteria for PD, DLB, MSA, and PAF had phosphorylated α-synuclein detected by skin biopsy. Further research is needed in unselected clinical populations to externally validate the findings and fully characterize the potential role of skin biopsy detection of phosphorylated α-synuclein in clinical care. [ABSTRACT FROM AUTHOR]

A systematic review was conducted to determine the trends in devices and parameters used for brain photobiomodulation (PBM). The revised studies included clinical and cadaveric approaches, in which light stimuli were applied to the head and/or neck. PubMed, Scopus, Web of Science and Google Scholar databases were used for the systematic search. A total of 2133 records were screened, from which 97 were included in this review. The parameters that were extracted and analysed in each article were the device design, actuation area, actuation site, wavelength, mode of operation, power density, energy density, power output, energy per session and treatment time. To organize device information, 11 categories of devices were defined, according to their characteristics. The most used category of devices was laser handpieces, which relate to 21% of all devices, while 28% of the devices were not described. Studies for cognitive function and physiological characterisation are the most well defined ones and with more tangible results. There is a lack of consistency when reporting PBM studies, with several articles under defining the stimulation protocol, and a wide variety of parameters used for the same health conditions (e.g., Alzheimer's or Parkinson's disease) resulting in positive outcomes. Standardization for the report of these studies is warranted, as well as sham-controlled comparative studies to determine which parameters have the greatest effect on PBM treatments for different neurological conditions. [ABSTRACT FROM AUTHOR]

Background: Spinal epidural lipomatosis (SEL) is a rare contributor of low back pain (LBP) that can present with or without radicular symptoms. Case Presentation: A 51-year-old and 65-yearold male presented with chronic LBP to the Veterans Affairs chiropractic clinic for a trial of care. One had a moderate degree of lumbar spinal stenosis with known SEL and the other had severe. The patient with moderate grade stenosis responded favorably with weeks of transient benefit after visits and the patient with severe grade did not find benefit with care. Summary: SEL is a condition that conservative care providers should be aware of as a potential cause of central canal stenosis or neuroforaminal narrowing. Chiropractic management of SEL has been scarcely reflected in the published literature, but may be a viable option for transient symptom management. [ABSTRACT FROM AUTHOR]

The aim of this study is to compare the neurological status, developmental parameters and physical fitness of children who engage in regular physical activity in the preschool period versus those who do not. The study included 305 healthy children aged 4–6 years, consisting of 154 girls and 151 boys. In addition to evaluating the demographic characteristics of children; Touwen Neurological Examination, Denver Developmental Screening Test (DDST) II, and Preschool Physical Fitness (PREFIT) test battery were used to assess neurological status, developmental parameters, and physical fitness, respectively. A statistically significant difference was found between neurological status (p = 0.046), developmental parameters (p = 0.000) and physical fitness (Standing Long Jump (p = 0.008), handgrip (p = 0.006)) of preschool children who engaged in regular physical activity compared to those who did not. Preschool children who participated in regular physical activity had no neurological problems and exhibited better developmental parameters and physical fitness than their inactive peers. [ABSTRACT FROM AUTHOR]

Hereditary sensory autonomic neuropathy type VIII (HSAN-VIII) is a rare genetic disease that occurs due to mutations in the PRDM12 gene. Here, we describe a novel homozygous mutation c.826_840dupTGCAACCGCCGCTTC (p.Cys276_Phe280dup) on exon 5 in the PRDM12 gene identified by WES and confirmed using Sanger sequencing method. [ABSTRACT FROM AUTHOR]

The finger tapping test (FTT) is a tool to evaluate the motor performance of the hand and fingers and eye-hand coordination with applicability in people with multiple sclerosis (pwMS). The aim of this study was to evaluate the intra- and inter-rater reliability of the TappingPro® mobile app and the construct validity between this app and validated clinical scales for motor performance in healthy subjects and pwMS. 42 healthy subjects (mean age 25.05) and 13 pwMS (mean age 51.69, EDSS between 3 and 7.5E) participated. FTT was performed with the TappingPro® mobile app. All participants were examined twice, with a one-week interval between the two appointments. For the analysis of construct validity, the Jamar® hydraulic hand dynamometer, Box and Blocks Test (BBT), and Nine Hole Peg Test (NHPT) were used. The intra-rater reliability showed a good correlation (Intraclass Correlation Coefficient, ICC > 0.787) for both upper limbs and both times of FTT for healthy subjects, and an excellent correlation (ICC > 0.956) for upper limbs and both times of FTT for pwMS. The ICC for the inter-rater reliability was good (ICC = 0.869) for the non-dominant upper limb in the FTT 10 s of the healthy subjects, and excellent (ICC > 0.904) for all the other measurements of the healthy subjects and pwMS. However, the Bland–Altman plots showed disagreement between observers and measurements that should be considered in the interpretation of clinical evaluations. The correlation analysis for healthy subjects showed poor associations between all variables, except for the association between hand grip strength and the FTT 60 s in the non-dominant upper limb, which had a moderate coefficient. For pwMS, there were moderate to excellent associations between BBT and the NHPT and FTT for both upper limbs. The correlations between hand grip strength and FFT were poor. This mobile app could be a useful and low-cost assessment tool in pwMS, allowing a simple evaluation and follow-up that has excellent correlation with clinical scales validated in this pathology. [ABSTRACT FROM AUTHOR]

Neonatal brain injury (NBI) is a critical condition for preterm neonates with potential long-term adverse neurodevelopmental outcomes. This prospective longitudinal case–control study aimed at investigating the levels and prognostic value of serum neuron-specific enolase (NSE) during the first 3 days of life in preterm neonates (<34 weeks) that later developed brain injury in the form of either periventricular leukomalacia (PVL) or intraventricular hemorrhage (IVH) during their hospitalization. Participants were recruited from one neonatal intensive care unit, and on the basis of birth weight and gestational age, we matched each case (n = 29) with a neonate who had a normal head ultrasound scan (n = 29). We report that serum NSE levels during the first three days of life do not differ significantly between control and preterm neonates with NBI. Nevertheless, subgroup analysis revealed that neonates with IVH had significantly higher concentrations of serum NSE in comparison to controls and neonates with PVL on the third day of life (p = 0.014 and p = 0.033, respectively). The same pattern on the levels of NSE on the third day of life was also observed between (a) neonates with IVH and all other neonates (PVL and control; p = 0.003), (b) neonates with II–IV degree IVH and all other neonates (p = 0.003), and (c) between control and the five (n = 5) neonates that died from the case group (p = 0.023). We conclude that NSE could be an effective and useful biomarker on the third day of life for the identification of preterm neonates at high risk of developing severe forms of IVH. [ABSTRACT FROM AUTHOR]

Objective: Unusual clinical course. Background: Primary central nervous system diffuse large B-cell lymphoma (DLBCL) is an extremely aggressive brain disease that rarely affects immunocompetent non-elderly patients, particularly with hemorrhagic presentation. Brain magnetic resonance imaging (MRI) plays an important role in the diagnosis of this entity, which typically demonstrates restricted diffusion and a T2 hypointense appearance, suggesting hypercellularity. Case Report: A 44-year-old man came to the emergency department with a persistent and treatment-resistant bilateral frontal headache that had been bothering him for the past 3 weeks. Upon conducting a neurological assessment, the patient displayed temporal disorientation and incoherent speech, but without any observable motor deficits. A non-contrast enhanced brain computed tomography scan was carried out, revealing a hyperattenuating, space-occupying lesion and hemorrhage in the left hemisphere of the brain. Subsequently, brain MRI demonstrated hypointense signal on T2-weighted images, restricted diffusion, and homogeneous lesional contrast enhancement, suggesting a very cellular expansive lesion with hemorrhage. To establish a definitive diagnosis, a brain biopsy was undertaken, confirming the presence of DLBCL of the primary central nervous system (germinal center phenotype). Conclusions: Hemorrhagic presentation of primary central nervous system DLBCL occurs very rarely, particularly in non-elderly immunocompetent patients. Brain MRI plays an important role in the diagnosis of this entity, which allows differentiation from high-grade glial or other lesions that present more frequently with hemorrhage. Therefore, it is crucial to suspect lymphoma before surgical intervention for appropriate patient management. [ABSTRACT FROM AUTHOR]

Objective: To test efficacy of a parent-delivered multidomain early intervention (Learning through Everyday Activities with Parents [LEAP-CP]) for infants with cerebral palsy (CP) compared with equal-dose of health advice (HA), on (1) infant development; and (2) caregiver mental health. It was hypothesized that infants receiving LEAP-CP would have better motor function, and caregivers better mental health. Methods: This was a multisite single-blind randomized control trial of infants aged 12 to 40 weeks corrected age (CA) at risk for CP (General Movements or Hammersmith Infant Neurologic Examination). Both LEAP-CP and HA groups received 15 fortnightly home-visits by a peer trainer. LEAP-CP is a multidomain active goal-directed intervention. HA is based on Key Family Practices, World Health Organization. Primary outcomes: (1) infants at 18 months CA: Pediatric Evaluation of Disability Inventory-Computer Adaptive Test (PEDI-CAT mobility); and (2) caregiver: Depression Anxiety and Stress Scale. Results: Of eligible infants, 153 of 165 (92.7%) were recruited (86 males, mean age 7.1±2.7 months CA, Gross Motor Function Classification System at 18 m CA: I = 12, II = 25, III = 9, IV = 18, V = 32). Final data were available for 118 (77.1%). Primary (PEDI-CAT mobility mean difference = 0.8 (95% CI -1.9 to 3.6) P = .54) and secondary outcomes were similar between-groups. Modified-Intention-To-Treat analysis on n = 96 infants with confirmed CP showed Gross Motor Function Classification System I and IIs allocated to LEAP-CP had significantly better scores on PEDI-CAT mobility domain (mean difference 4.0 (95% CI = 1.4 to 6.5), P = .003) compared with HA. Conclusions: Although there was no overall effect of LEAP-CP compared with dose-matched HA, LEAP-CP lead to superior improvements in motor skills in ambulant children with CP, consistent with what is known about targeted goal-directed training. [ABSTRACT FROM AUTHOR]

Purpose: The management of depressed skull fractures in infants can be either conservative or surgical. This study aimed to examine the outcomes of management with a negative-pressure vacuum device on depressed skull fractures in newborns. Methods: Twenty-eight patients (aged 1–6 days) with simple depressed skull fractures underwent skull elevation using negative-pressure vacuum devices. A protocol for nonsurgical management was adopted for infants with such fractures between 2010 and 2023. All patients were initially evaluated with neurological examination and complementary assessments—hematological and coagulation studies, transfontanel transcranial ultrasound, skull radiography, and computed tomography scanning with three-dimensional reconstruction—according to availability and clinical needs. Gentle (negative) extraction pressure was applied with one of several devices (according to institutional availability) for a maximum duration of 60 s; this was performed as soon as possible after diagnosis, preferably within 72 h. Follow-up data, available in the clinical records, were reported. Results: All patients exhibited satisfactory elevation of the depressed bone without associated injuries, except one patient who presented with an associated cephalohematoma which prevented optimal device coupling to generate sufficient vacuum pressure for correction. Neither neurological deficits nor development of epilepsy was noted; normal neurological assessment and oral alimentation tolerance were confirmed within 24 h post procedure. Conclusions: According to our data, ping-pong skull fracture elevation using the vacuum method is a safe and satisfactory treatment in the neonatal period. Early treatment allows for quick resolution, and in our opinion is the strategy of choice for depressed skull fractures in newborns. [ABSTRACT FROM AUTHOR]

The article focuses on hereditary hyperekplexia (HPX), a rare disorder characterized by exaggerated startle response and generalized muscle stiffness, often presenting in infancy. It discusses the standard treatment with clonazepam and explores the novel use of trihexyphenidyl to alleviate persistent generalized stiffness episodes in patients unresponsive to clonazepam.

Simple Summary: Neurofibromatosis Type 1 is an inherited tumour predisposition syndrome with a varied clinical phenotype. Long-term monitoring through imaging is inconsistent and varies in high- and low-income countries. Implementation of a clinical practice guideline through a multidisciplinary clinic is instrumental to the care of adult Neurofibromatosis Type 1 patients. This systematic review aims to evaluate the association between a country's socioeconomic status and diagnostic modalities and strategies used for adult Neurofibromatosis Type 1 patients. Our results show multiple imaging modalities are used in high-income countries; however, there is limited use in low-income countries. The two most common diagnostic modalities used in developed countries are WB MRI and FDG PET/CT. Background: Neurofibromatosis Type 1 is an autosomal dominant tumour-predisposition condition commonly diagnosed in childhood and fully penetrant by adulthood. Long-term monitoring through imaging is inconsistent and varies between high- and low-income countries. Implementation of a clinical practice guideline through a multidisciplinary clinic is instrumental to the care of adult Neurofibromatosis Type 1 patients. We aim to systematically review international diagnostic modalities and strategies to evaluate any association between a country's socioeconomic status and diagnostic modalities or strategies used for Neurofibromatosis Type 1 patients. Methods: We searched PubMed, Embase, Web of Science, and Cochrane. Relevant clinical information on the surveillance of adult Neurofibromatosis Type 1 patients worldwide was reviewed, extracted, and synthesised. Results: We identified 51 papers reporting on 7724 individuals. Multiple imaging modalities are actively employed in high-income and upper-middle-income countries for surveying adult Neurofibromatosis Type 1 patients. We did not find any relevant papers from low- and middle-income countries. Conclusions: This systematic review suggests that there is robust data on diagnostic modalities for adult Neurofibromatosis Type 1 patients in high-income countries, but not for low- and middle-income countries. There is a lack of data on consolidated diagnostic strategies from both high- and low-income countries. Efforts should be made to publish data on usual clinical practice in low- and middle-income countries to develop clinical practice guidelines describing best medical practice to fit a local context. [ABSTRACT FROM AUTHOR]

Background: In some cases of patients with out-of-hospital cardiac arrest (OHCA) who underwent extracorporeal cardiopulmonary resuscitation (ECPR), negative pupillary light reflex (PLR) and mydriasis upon hospital arrival serve as common early indicator of poor prognosis. However, in certain patients with poor prognoses inferred by pupil findings upon hospital arrival, pupillary findings improve before and after the establishment of ECPR. The association between these changes in pupillary findings and prognosis remains unclear. This study aimed to clarify the association of pupillary examinations before and after the establishment of ECPR in patients with OHCA showing poor pupillary findings upon hospital arrival with their outcomes. To this end, we analysed retrospective multicentre registry data involving 36 institutions in Japan, including all adult patients with OHCA who underwent ECPR between January 2013 and December 2018. We selected patients with poor prognosis inferred by pupillary examinations, negative pupillary light reflex (PLR) and pupil mydriasis, upon hospital arrival. The primary outcome was favourable neurological outcome, defined as Cerebral Performance Category 1 or 2 at hospital discharge. Multivariable logistic regression analysis was performed to evaluate the association between favourable neurological outcome and pupillary examination after establishing ECPR. Results: Out of the 2,157 patients enrolled in the SAVE-J II study, 723 were analysed. Among the patients analysed, 74 (10.2%) demonstrated favourable neurological outcome at hospital discharge. Multivariable analysis revealed that a positive PLR at ICU admission (odds ration [OR] = 11.3, 95% confidence intervals [CI] = 5.17–24.7) was significantly associated with favourable neurological outcome. However, normal pupil diameter at ICU admission (OR = 1.10, 95%CI = 0.52–2.32) was not significantly associated with favourable neurological outcome. Conclusion: Among the patients with OHCA who underwent ECPR and showed poor pupillary examination findings upon hospital arrival, 10.2% had favourable neurological outcome at hospital discharge. A positive PLR after the establishment of ECPR was significantly associated with favourable neurological outcome. [ABSTRACT FROM AUTHOR]

Objective: Rare coexistence of disease or pathology. Background: Hemiballismus is the most severe form of chorea and is a hyperkinetic disorder characterized by involuntary, high-amplitude movements of the ipsilateral arm and leg, due to lesions of the contralateral side of the central nervous system. Ischemic or hemorrhagic strokes and nonketotic hyperglycemia are predominant etiologies of hemiballismus. Case reports highlighting hemiballismus associated with temporal and parietal lobe infarcts have been published, although research of frontal lobe involvement is limited. Case Report: A 78-year-old woman presented to the Emergency Department with sudden-onset left-sided hemiballismus. On examination, she was alert, oriented to self and time, and able to follow commands. Her neurologic examination was notable for left-sided hemiballismus, described by the provider as periodic, uncontrolled, and involving a "flinging" motion of the left upper and lower extremities, sparing the face. She was treated with benzodiazepines in the Emergency Department and administered intravenous levetiracetam. Computed tomography of the head without contrast revealed an old left basal ganglia lacunar infarct. The patient was then admitted to the inpatient service, where magnetic resonance imaging of the brain revealed an acute punctate left superior frontal gyrus cortical infarct. Outpatient electroencephalogram revealed right anterior hemisphere dysfunction. Conclusions: We describe a patient with left-sided sudden onset hemiballismus with an acute infarct of the ipsilateral superior frontal gyrus. This case highlights that brain lesions separate from the basal ganglia can induce hemiballismus, particularly within the frontal lobe, which warrants further research into precentral sulcus functioning and its role in modulating motor activity. [ABSTRACT FROM AUTHOR]

The article discusses a case study involving a 12-year-old boy with a novel SCN1A mutation presenting as hyperkinetic movement disorder, including multifocal dystonia and parakinesia, without seizures. It mentions that this unique presentation underscores the importance of genetic evaluation and individualized management approaches for patients with SCN1A mutations, leading to improved clinical outcomes.

Introduction: Non-traumatic visual impairment is rare in the pediatric population, but early diagnosis and treatment of the cause is crucial to prevent long-term consequences affecting children's neurocognitive development. The authors aim to determine the most common causes of non-traumatic visual impairment in pediatric patients according to age groups by magnetic resonance imaging (MRI). Methods: Images of patients who underwent contrast-enhanced cranial and orbital MRI for new-onset visual impairment between June 2019 and June 2022 were retrospectively reviewed. MRI findings were categorized as tumors, idiopathic intracranial hypertension, demyelinating disorders, infections, isolated optic neuritis, and others. The patients were grouped according to age as preschoolers, schoolchildren, and adolescents. Demographic features of patients and MRI findings were collected and compared among age groups. Results: One hundred seventeen of the 238 patients had pathologic MRI findings. The most common pathologies were tumors (26.4%), idiopathic intracranial hypertension (24.7%), demyelinating disorders (18.8%), infections (11.1%), and isolated optic neuritis (7.6%). Tumors (69.2%) in preschool children, idiopathic intracranial hypertension (36.3%) in schoolchildren, and demyelinating disorders (32.7%) in adolescents were the most common cause of vision impairment by age group. Conclusion: Children with acute vision impairment could have severe pathologies. Tumors in preschool children, idiopathic intracranial hypertension in schoolchildren, and demyelinating disorders in adolescents were the most common causes of new-onset vision impairment detected with MRI. Because of the difficulty of performing optimal ophthalmologic and neurologic examinations, especially in young children, cranial and orbital MRI should be considered to detect life-threatening pathologies. [ABSTRACT FROM AUTHOR]

Huntington's disease (HD) usually manifests in adulthood and is characterised by progressive neurodegeneration in the brain that causes worsening involuntary movements, mental health and cognition over many years. Depression, anxiety and apathy are common. HD is autosomal dominant and affects about 1 in 8,000 people in the UK. There are currently no disease-modifying treatments and so patient care centres on multidisciplinary therapy support and medical treatments to relieve distressing symptoms. Progression of HD is usually slow, and so acute deteriorations often indicate another problem, such as intercurrent infections, constipation, urinary retention, gastro-oesophageal reflux disease or poor dentition. In this review we outline common presentations in HD patients, both acute and chronic, consider therapeutic options and discuss specific considerations in advanced HD. [ABSTRACT FROM AUTHOR]

Objective: To evaluate magnetic resonance neurography (MRN) for the longitudinal assessment of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods: Prospective examination of twelve CIDP patients by neurological assessment, MRN, and nerve conduction studies in 2016 and 6 years later in 2022. Imaging parameters were compared with matched healthy controls and correlated with clinical and electrophysiological markers. The MRN protocol included T2‐weighted imaging, diffusion tensor imaging (DTI), T2 relaxometry, and magnetization transfer imaging (MTI). Results: Nerve cross‐sectional area (CSA) was increased in CIDP patients compared to controls (plexus: p = 0.003; sciatic nerve: p < 0.001). Over 6 years, nerve CSA decreased in CIDP patients, most pronounced at the lumbosacral plexus (p = 0.015). Longitudinally, changes in CSA correlated with changes in the inflammatory neuropathy cause and treatment validated overall disability sum score (INCAT/ODSS) (p = 0.006). High initial nerve CSA was inversely correlated with changes in the INCAT/ODSS over 6 years (p < 0.05). The DTI parameter fractional anisotropy (FA) showed robust correlations with electrodiagnostic testing both cross‐sectionally and longitudinally (p < 0.05). MTI as a newly added imaging technique revealed a significantly reduced magnetization transfer ratio (MTR) in CIDP patients (p < 0.01), suggesting underlying changes in macromolecular tissue composition, and correlated significantly with electrophysiological parameters of demyelination (p < 0.05). Interpretation: This study provides evidence that changes in nerve CSA and FA reflect the clinical and electrophysiological course of CIDP patients. Initial nerve hypertrophy might predict a rather benign course or better therapy response. [ABSTRACT FROM AUTHOR]

Orientation is a fundamental cognitive faculty and the bedrock of the neurologic examination. Orientation is defined as the alignment between an individual's internal representation and the external world in the spatial, temporal, and social domains. While spatial disorientation is a recognized hallmark of Alzheimer's disease (AD), little is known about disorientation beyond space in AD. This study aimed to explore disorientation in spatial, temporal, and social domains along the AD continuum. Fifty‐one participants along the AD continuum performed an ecological orientation task in the spatial, temporal, and social domains while undergoing functional MRI. Disorientation in AD followed a three‐way association between orientation domain, brain region, and disease stage. Specifically, patients with early amnestic mild cognitive impairment exhibited spatio‐temporal disorientation and reduced brain activity in temporoparietal regions, while patients with AD dementia showed additional social disorientation and reduced brain activity in frontoparietal regions. Furthermore, patterns of hypoactivation overlapped different subnetworks of the default mode network, patterns of fluorodeoxyglucose hypometabolism, and cortical atrophy characteristic of AD. Our results suggest that AD may encompass a disorder of orientation, characterized by a biphasic process manifesting as early spatio‐temporal and late social disorientation. As such, disorientation may offer a unique window into the clinicopathological progression of AD. Significance statement: Despite extensive research into Alzheimer's disease (AD), its core cognitive deficit remains a matter of debate. In this study, we investigated whether orientation, defined as the ability to align internal representations with the external world in spatial, temporal, and social domains, constitutes a core cognitive deficit in AD. To do so, we used PET‐fMRI imaging to collect behavioral, functional, and metabolic data from 51 participants along the AD continuum. Our findings suggest that AD may constitute a disorder of orientation, characterized by an early spatio‐temporal disorientation and followed by late social disorientation, manifesting in task‐evoked and neurodegenerative changes. We propose that a profile of disorientation across multiple domains offers a unique window into the progression of AD and as such could greatly benefit disease diagnosis, monitoring, and evaluation of treatment response. [ABSTRACT FROM AUTHOR]

Why you should read this article: • To enhance your knowledge of the elements of neurological assessments in children • To improve your understanding of when to perform neurological observations and when to escalate them • To contribute towards revalidation as part of your 35 hours of CPD (UK readers) • To contribute towards your professional development and local registration renewal requirements (non-UK readers). Neurological observations are a vital part of the assessment of infants, children and young people with a suspected or confirmed acquired brain injury (ABI). They are designed to identify neurological deterioration and inform the management of ABIs. Children’s nurses may perform many of these neurological observations, including by using tools such as the AVPU scale, the Glasgow Coma Scale, pupillary response and limb assessment. This is the second of two articles that aim to encourage accuracy and consistency when performing neurological observations in infants, children and young people with a suspected or confirmed ABI to optimise their care. The article explains how to undertake various elements of a neurological assessment, how often to perform neurological observations, when to escalate concerns and why it is important to use family-centred care. [ABSTRACT FROM AUTHOR]

Wernicke's encephalopathy (WE) is a serious neurological disorder that is underdiagnosed. Despite limited clinical guidelines, the standard use of intravenous (IV) thiamine is underutilized and remains an area of research deserving much attention. We conducted a systematic review using Medline, Embase, and CENTRAL databases to identify and summarize the literature on IV thiamine treatment in WE. Human studies with WE patients who received ≥100 mg of thiamine IV met inclusion criteria. Randomized controlled trials, cross-sectional studies, and case reports were included. A total of 27 studies were included: 20 case reports, five retrospective studies, one prospective study and one randomized control trial. Of the case reports, 11 (55%) cases were female, and the average age of all cases was 45 years (SD = 15). The other seven studies included 688 patients; the average age was 52 years (SD = 9), and 266 (38.7%) were female. Among the case reports, neurological and clinical findings were used to diagnose WE in 16 (80%) cases. MRI was utilized to diagnose 15 (75%) cases. 500 mg IV thiamine TID was reported in 12 case reports (60%). 18 (90%) of case reports had partial or complete resolution of symptoms following IV thiamine. IV thiamine can alleviate neurological symptoms, cognitive dysfunction, and brain imaging lesions associated with WE. We found key limitations in the evidence for IV thiamine and diagnostic standards for WE. Future targeted research should establish clear diagnostic and treatment guidelines for WE to prevent this serious condition from being underdiagnosed or undertreated. [ABSTRACT FROM AUTHOR]

Rosai-Dorfman disease (RDD) is a benign histiocytosis with unknown etiology. It generally occurs in cervical lymph nodes. Isolated central nervous system (CNS) RDD is very rare in the literature. We reported a case of no systemic involvement Rosai-Dorfmann which is rarely seen and shows CNS involvement by mimicking meningioma. A 32-year-old man presented with diplopia and a headache he has been experiencing for the past two years. His neurological examination showed left facial paresthesia, consistent with trigeminal nerve trace. Tendon reflexes were increased at the right side and the right plantar reflex was extensor. Brain magnetic resonance imaging demonstrated irregularly shaped, tumorlike lesions in the bilateral cerebellopontin area that were compressing pons. Rosai-Dorfman disease can be differentiated from IgG4 related disease (IgG4-RD) by its characteristic features such as plasma cell density and emperipolesis seen in its histopathology. Rosai-Dorfman disease can be confused with other diseases radiologically and histopathologically, especially the IgG4-RD, so be careful about differential diagnosis. [ABSTRACT FROM AUTHOR]

This article describes a case study of a 67-year-old man who presented with speech and gait disturbances following an episode of gastroenteritis. The patient had cerebellar dysarthria and limb incoordination, and an MRI revealed bilateral lesions in the white matter of the brain. Further testing confirmed a diagnosis of progressive multifocal leukoencephalopathy (PML) caused by reactivation of the John Cunningham virus (JCV) in the context of HIV infection. The article discusses the typical features of PML on MRI and highlights the importance of early recognition and treatment. [Extracted from the article]

Background: Qualitative sensory testing (QualST) is a simple, standardised, chairside method for evaluating somatosensory function; however, testing focuses on detection of cold, touch and pain with no recognition of perceptions of pleasantness and unpleasantness. Objectives: The study aimed to utilise the stimuli in QualST, with the addition of a soft brush, to investigate stimulus‐evoked perceptions of pleasantness and unpleasantness on the facial skin and if any side‐to‐side differences. Additional aims were to determine the inter‐ and intra‐rater reliability using the modified QualST protocol and in the side‐to‐side differences. Methods: Thirty healthy adult female participants underwent three sessions of sensitivity testing as per the modified QualST protocol. Stimuli were applied bilaterally to the facial skin, and participants provided separate yes/no responses for presence of stimulus‐evoked pleasantness, unpleasantness and/or differences between sides. Results: The stimuli were able to evoke sensations of pleasantness and unpleasantness with little differences in responses between the Q‐tip and goat hair brush for the perceptions. Side‐to‐side differences in evoked perceptions were observed and greatest, when evaluating for pinprick‐evoked unpleasantness (range between sessions = 18–19 participants). Acceptable percentage (≥90%) and excellent Cohen's Kappa (≥0.762) inter‐ and intra‐rater agreements were identified for one or more positive responses for each stimulus modality and the targeted perception. Conclusion: The modified QualST protocol provides a simple, reproducible method for the investigation of perceptions of pleasantness and unpleasantness, with readily accessible instrumentation to dental professionals and allowing for a more holistic approach in somatosensory testing. [ABSTRACT FROM AUTHOR]

This article provides an overview of vestibular disease in horses, explaining the anatomy of the vestibular system and discussing the clinical signs and common causes of the disease. It emphasizes the importance of diagnostic tests in reaching a diagnosis. The article titled "Ethics Statement" by Sarah F. Colmer and Amy L. Johnson discusses the absence of an ethics statement in a clinical commentary and explains the purpose and importance of an ethics statement in research articles. [Extracted from the article]

Summary: A descriptive case series of the presentation, diagnosis, treatment and outcome of eight horses presenting to a UK equine veterinary hospital with suspected idiopathic peripheral vestibular syndrome over a 7‐year period (2014–2021). Idiopathic vestibular syndrome in the horse causes unilateral clinical signs including; ataxia, nystagmus, strabismus and a head tilt. Diagnosis is aided by neurological examination, endoscopy of the upper airway and cross‐sectional imaging (i.e. magnetic resonance imaging and computed tomography). This case series indicates that horses with this condition can make a full recovery with return to ridden exercise. Treatment with anti‐inflammatory medication did not appear to influence outcome. [ABSTRACT FROM AUTHOR]

Acute generalised muscle weakness in children is a paediatric emergency with a broad differential diagnosis. A careful history and neurologic examination guides timely investigation and management. We review some of the more common causes of acute generalised muscle weakness in children, highlighting key history and examination findings, along with an approach to lesion localisation to guide differential diagnosis and further investigation. [ABSTRACT FROM AUTHOR]

Aim To investigate the correlation of body mass index (BMI) with severity of intervertebral disc degeneration. Methods The study enrolled patients who had undergone surgical intervention for a herniated disc at the Department of Neurosurgery of the Cantonal Hospital Zenica. Patients underwent thorough preoperative evaluation, including medical history, neurological and physical assessments, and radiological analysis. The surgical intervention consisted of a posterior lumbar discectomy, and the excised disc material was preserved and subjected to histopathological analysis based on Histopathologic Degeneration Score (HDS). Patients were divided in two groups according to Body Mass Index (BMI): study group with BMI≥25 and control group with BMI<25. Results Among 69 patients with herniated IVD, 26 (37.7%) were with BMI≥25 (study group), and 43 (62.3%) were with BMI<25 (controls). The study group displayed substantial increase in height, 1.80±0.06 m compared to controls, 1.74±0.06 m (p=0.001). Weight and BMI were significantly higher in the study group of patients (weight: 91.60±10.22 vs. 67.37±9.20 kg, BMI: 28±2 vs. 22±2; p<0.001). Differences were confirmed in HDS values in the study group comparing to the control group (p<0.001). The study group exhibited significant differences in chondrocyte proliferation, tears and clefts, granular changes, and mucous degeneration (p<0.05), and positive correlations were found between BMI and these alterations found in the herniated discs (p<0.05). Therefore, HDS showed positive correlations with BMI (R=0.599; p<0.001) and weight (R=0.696; p<0.001). Conclusion The study's findings confirmed that BMI has a significant impact on intervertebral disc degeneration, emphasizing the importance of weight management in preventing disc degeneration. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this study was to evaluate the prevalence of idiopathic intracranial hypertension (IIH) in fibromyalgia (FMS) patients by utilizing ultrasound to measure the optic nerve sheath diameter (ONSD), a marker of elevated intracranial pressure and also to investigate the relationship with function, fatigue, quality of life (QOL), central sensitization (CS) and neuropathic pain. Methods: The study encompassed 80 female FMS patients and 75 healthy controls. Ultrasound was employed to measure the average ONSD in both groups. Conditions potentially elevating intracranial pressure were ruled out following neurological assessments. Pain (via visual analog scale, VAS), function (revised Fibromyalgia Impact Questionnaire, r‐FIQ), QOL (Short Form‐36, SF‐36), fatigue (fatigue severity scale, FACIT), CS (Central Sensitization Scale), and neuropathic pain (Douleur Neuropathique‐4) were evaluated. Results: The average ONSD was significantly higher in the patient group than the control group. Patients with ONSD >5.5 mm consistent with IIH were categorized as Group 1 (n = 54, 67.5%), while those with a diameter of 5.5 mm and below‐formed Group 2. VAS pain (p =.033) and FIQ‐R scores (p =.033) were significantly higher in Group 1 than Group 2. Headache was found more common in Group 1. Conclusion: This study unveils a substantial occurrence (67.5%) of IIH in FMS patients, suggesting shared pathophysiological mechanisms contributing to symptoms like fatigue, headache, and cognitive dysfunction. Additionally, these findings implicate heightened functional impairment, CS, headache, and fatigue in FMS patients with IIH. [ABSTRACT FROM AUTHOR]

Objective: The aim of the study is to determine what improvement on the American Spinal Injury Impairment Scale correlates with functional status after a traumatic spinal cord injury. Design: We performed an observational cohort study, analyzing prospective data from 168 patients with traumatic spinal cord injury admitted to a single level 1 trauma center. A multivariable analysis was performed to assess the relationship between functional status (from the Spinal Cord Independence Measure) at 1-year follow-up and American Spinal Injury Impairment Scale grade (baseline and 1-yr follow-up), while taking into account covariables describing the sociodemographic status, trauma severity, and level of neurological injury. Results: Individuals improving to at least American Spinal Injury Impairment Scale grade D had significantly higher Spinal Cord Independence Measure score compared with those not reaching American Spinal Injury Impairment Scale D (89.3 ± 15.2 vs. 52.1 ± 20.4) and were more likely to reach functional independence (68.5% vs. 3.6%), regardless of the baseline American Spinal Injury Impairment Scale grade. Higher final Spinal Cord Independence Measure was more likely with an initial American Spinal Injury Impairment Scale grade D (β = 1.504; 95% confidence interval = 0.46–2.55), and a final American Spinal Injury Impairment Scale grade D (β = 3.716; 95% CI = 2.77–4.66) or E (β = 4.422; 95% CI = 2.91–5.93). Conclusions: Our results suggest that reaching American Spinal Injury Impairment Scale grade D or better 1 yr after traumatic spinal cord injury is highly predictive of significant functional recovery, more so than the actual improvement in American Spinal Injury Impairment Scale grade from the injury to the 1-yr follow-up. [ABSTRACT FROM AUTHOR]

To assess interrater reliability and examiners' characteristics, especially specialty, associated with scoring of neurology objective structured clinical examination (OSCE). During a neurology mock OSCE, five randomly chosen students volunteers were filmed while performing 1 of the 5 stations. Video recordings were scored by physicians from the Lyon and Clermont-Ferrand university teaching hospitals to assess students performance using both a checklist scoring and a global rating scale. Interrater reliability between examiners were assessed using intraclass coefficient correlation. Multivariable linear regression models including video recording as random effect dependent variable were performed to detect factors associated with scoring. Thirty examiners including 15 (50%) neurologists participated. The intraclass correlation coefficient of checklist scores and global ratings between examiners were 0.71 (CI95% [0.45-0.95]) and 0.54 (CI95% [0.28-0.91]), respectively. In multivariable analyses, no factor was associated with checklist scores, while male gender of examiner was associated with lower global rating (β coefficient = –0.37; CI 95% [–0.62–0.11]). Our study demonstrated through a video-based scoring method that agreement among examiners was good using checklist scoring while moderate using global rating scale in neurology OSCE. Examiner's specialty did not affect scoring whereas gender was associated with global rating scale. [ABSTRACT FROM AUTHOR]

The article describes the case of a 14-year-old boy with benign recurrent aseptic meningitis complicated by pseudohypoparathyroidism. Topics discussed include results of his laboratory tests, findings on cerebrospinal fluid examination, and main manifestations of benign recurrent aseptic meningitis.