Neurology International

11 pages, 3056 KiB

Open AccessCase Report

Adult Case of Pontocerebellar Hypoplasia without the Claustrum

by Koji Hayashi, Shiho Mitsuhashi, Ei Kawahara, Asuka Suzuki, Yuka Nakaya, Mamiko Sato and Yasutaka Kobayashi

Neurol. Int. 2024, 16(5), 1132-1142; https://doi.org/10.3390/neurolint16050085 - 7 Oct 2024

Abstract

We describe the case of a 63-year-old man with pontocerebellar hypoplasia without the claustrum (CL). The patient had a history of cerebral palsy, intelligent disability, cerebellar atrophy, and seizures since birth. At age 61, brain computed tomography (CT) revealed significant cerebellar and brainstem [...] Read more.

We describe the case of a 63-year-old man with pontocerebellar hypoplasia without the claustrum (CL). The patient had a history of cerebral palsy, intelligent disability, cerebellar atrophy, and seizures since birth. At age 61, brain computed tomography (CT) revealed significant cerebellar and brainstem atrophy. At age 63, he was admitted to our hospital for aspiration pneumonia. Although he was treated with medications, including antibiotics, he died one month after admission. The autopsy revealed a total brain weight of 815 g, with the small-sized frontal lobe, cerebellum, and pons. The cross-section of the fourth ventricle had a slit-like appearance, rather than the typical diamond shape. In addition, bilateral CLs were not observed. Apart from CL, no other missing brain tissue or cells could be identified. Microscopic examinations disclosed neurofibrillary tangles in the hippocampus but not in the cortex; however, neither senile plaques nor Lewy bodies were detected. No acquired lesions, including cerebral infarction, hemorrhage, or necrosis, were noted. We pathologically diagnosed the patient with pontocerebellar hypoplasia without CL. As there have been no prior reports of pontocerebellar hypoplasia lacking CL in adults, this case may represent a new subtype. Congenital CL deficiency is likely associated with abnormalities in brain development. CL may play a role in seizure activity, and the loss of bilateral CLs does not necessarily result in immediate death. Further studies are needed to clarify the functions of CL. Full article

(This article belongs to the Collection Advances in Neurodegenerative Diseases)

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10 pages, 1565 KiB

Open AccessArticle

Aging Processes of Working Memory in Different Modalities

by Ohad Levi and Eyal Heled

Neurol. Int. 2024, 16(5), 1122-1131; https://doi.org/10.3390/neurolint16050084 - 30 Sep 2024

Abstract

Background: Working memory (WM) involves temporarily storing and manipulating information. Research on the impact of aging on WM has shown inconsistent results regarding the decline in visual and verbal WM, with a lack of studies on tactile WM. This study aimed to [...] Read more.

Background: Working memory (WM) involves temporarily storing and manipulating information. Research on the impact of aging on WM has shown inconsistent results regarding the decline in visual and verbal WM, with a lack of studies on tactile WM. This study aimed to assess the effects of aging on WM across verbal, visuospatial, and tactile modalities using span tasks of forward (storage) and backward (manipulation) stages. Methods: A total of 130 participants, divided into four age groups of 20–29, 60–69, 70–79, and 80–89, completed the Digit, Visuospatial, and Tactual Spans. Performance was analyzed using a 3 (Task) × 4 (Group) × 2 (Stage) mixed design repeated measures ANOVA. Results: The analysis revealed significant main effects for modality (p < 0.001, η_p² = 0.15), age (p < 0.001, η_p² = 0.48), and stage (p < 0.001, η_p² = 0.30). Digit Span outperformed the other modalities, while Tactual Span showed the worst performance. Additionally, task performance declined with age, and the forward stage was superior to the backward stage. Interaction effects indicated that Digit Span was less affected by aging compared to the Visuospatial and Tactual Spans (p = 0.004, η_p² = 0.07). Post hoc analyses further revealed that the Digit Span consistently outperformed the other modalities in both stages, with more pronounced differences observed in the forward stage. Conclusions: Verbal WM is more resilient to aging compared to the other modalities while tactile WM declines with age in a manner similar to verbal and visuospatial WM, suggesting a modality-specific impact of aging on WM. Full article

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10 pages, 5135 KiB

Open AccessCase Report

Froin’s Syndrome: A Comprehensive Review of the Literature and the Addition of Two New Cases

by Lucas Jacobs, Bertil Delsaut, Marta Lamartine S. Monteiro, Audrey Cambier, Ibrahim Alcan, Evelyne Maillart and Maxime Taghavi

Neurol. Int. 2024, 16(5), 1112-1121; https://doi.org/10.3390/neurolint16050083 - 29 Sep 2024

Abstract

Background. Froin’s syndrome (FS) is a rare entity with uncertain prevalence and prognosis, defined by a pathognomonic triad: cerebrospinal fluid (CSF) xanthochromia, elevated protein levels in the CSF, and hypercoagulated CSF, usually obtained through lumbar puncturing below the level of a partial or [...] Read more.

Background. Froin’s syndrome (FS) is a rare entity with uncertain prevalence and prognosis, defined by a pathognomonic triad: cerebrospinal fluid (CSF) xanthochromia, elevated protein levels in the CSF, and hypercoagulated CSF, usually obtained through lumbar puncturing below the level of a partial or complete spinal block. Methods. We conducted a comprehensive review of the literature on FS from its first description in 1903 to December 2023, utilizing PubMed and Google Scholar, and included two new cases from our clinical practice. Results. We describe two patients who suffered from Froin’s syndrome secondary to spinal abscesses. According to our review, FS is caused by neoplasia in 33% of cases, non-malignant mechanical causes in 27%, infections in 27%, non-infectious inflammatory processes in 6%, and vascular in 6%. The most prevalent symptoms are paraplegia/paraparesis (64%), back pain (38%), altered mental state and/or confusion (23%), sciatica (17%), headaches (17%), leg sensory defects (17%), and urinary retention (14%), and are thought to be linked with the underlying causes rather than the CSF characteristics. FS holds a poor prognosis: only 22% recuperate fully after treatment, 22% die due to the cause leading to FS, and 14% retain sequelae. Conclusions. Xanthochromia and proteinorachia >500 mg/dL are not specific to any single pathological condition, but indicate defective CSF recirculation and spinal block, causing diffusive and/or inflammatory processes resulting in the hyperproteinosis and coagulation of the CSF. We reviewed the pathophysiology, etiologies, symptoms, outcomes, and workups of Froin’s syndrome according to the existing medical literature. Full article

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18 pages, 3275 KiB

Open AccessArticle

Neuroprotective Properties of Rutin Hydrate against Scopolamine-Induced Deficits in BDNF/TrkB/ERK/CREB/Bcl2 Pathways

by Inturu Sreelatha, Ga-Young Choi, In-Seo Lee, Omkaram Inturu, Hyun-Sook Lee, Yea-Na Park, Cheol-Won Lee, Inkyou Yang, Sungho Maeng and Ji-Ho Park

Neurol. Int. 2024, 16(5), 1094-1111; https://doi.org/10.3390/neurolint16050082 - 27 Sep 2024

Abstract

Background/Objectives: Alzheimer’s disease (AD) is an age-related degenerative brain disorder characterized by a progressive decline in cognitive function and memory. This study aimed to evaluate whether rutin hydrate (RH) has neuroprotective effects in an AD-like learning and memory impairment rat model induced [...] Read more.

Background/Objectives: Alzheimer’s disease (AD) is an age-related degenerative brain disorder characterized by a progressive decline in cognitive function and memory. This study aimed to evaluate whether rutin hydrate (RH) has neuroprotective effects in an AD-like learning and memory impairment rat model induced by scopolamine (SCO). Methods: The rats were administered with RH (100 mg/kg) and SCO (1.5 mg/kg) and underwent behavioral tests, including the Morris water maze test, Y-maze test, and passive avoidance test, to evaluate their learning and memory abilities. Additionally, long-term potentiation (LTP) was induced to observe changes in the field excitatory postsynaptic potential (fEPSP) activity. Results: RH treatment attenuated the SCO-induced shortening of step-through latency in the passive avoidance (PA) test, increased the percentage of alternation in the Y-maze, and increased the time spent in the target zone in the Morris water maze (MWM). Moreover, RH increased the total activity of fEPSP following theta burst stimulation and attenuated the SCO-induced blockade of fEPSP. RH also ameliorated the SCO-induced decrease in the expression levels of the BDNF, TrkB, ERK, CREB, and Bcl-2 proteins and the increase in the Bax protein level in the rat hippocampus. This demonstrates that RH has beneficial neuroprotective effects in the brain, improving learning, memory, and synaptic plasticity in rats. Conclusions: Our results highlight the molecular and cellular mechanisms through which RH exerts its neuroprotective effects in the prevention and treatment of learning and memory deficit disorders. RH could potentially be used as a therapeutic strategy for the restoration of learning and memory function and the prevention of the progression of AD. Full article

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8 pages, 501 KiB

Open AccessArticle

Validity and Reliability of the Japanese Version of the Frontal Assessment Battery in Patients with Stroke

by Katsuya Sakai, Yuichiro Hosoi, Yusuke Harada, Kenji Morikawa and Yuichi Kato

Neurol. Int. 2024, 16(5), 1086-1093; https://doi.org/10.3390/neurolint16050081 - 25 Sep 2024

Abstract

Background: The Frontal Assessment Battery (FAB), which is used to assess executive function, has been translated into several languages and shown to be valid and reliable. However, the validity and reliability of the Japanese version in patients with stroke are unknown. This study [...] Read more.

Background: The Frontal Assessment Battery (FAB), which is used to assess executive function, has been translated into several languages and shown to be valid and reliable. However, the validity and reliability of the Japanese version in patients with stroke are unknown. This study aimed to investigate the validity and reliability of the Japanese version of the FAB in patients with stroke. Methods: The Japanese version of the FAB for dementia was modified and evaluated in 52 patients with stroke. FAB measurements were obtained twice over a 10-day period. Convergent validity was assessed using the Stroop Color Word Test (SCWT) and the Trail Making Test (TMT) part B. Internal consistency was measured using Cronbach’s alpha (Cα). Test-retest evaluations were performed using intraclass correlation coefficient [ICC (2.1)] measurements, and limits of agreement (LOA) were calculated using the total FAB score. Results: The mean total FAB score was 13.4 ± 2.8 points, the ICC (2.1) was 0.856, and Cα was 0.92. The total FAB score was correlated with SCWT scores for parts I through IV (r = 0.70 to 0.77) and the TMT score for part B (ρ = −0.53). The LOA were −1.7 to 2.9 points. Conclusions: The Japanese version of the FAB had higher validity and reliability in patients with stroke. Full article

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20 pages, 3035 KiB

Open AccessArticle

Contrasting Effects of an Atherogenic Diet and High-Protein/Unsaturated Fatty Acids Diet on the Accelerated Aging Mouse Model SAMP8 Phenotype

by Jesús Llanquinao, Claudia Jara, Daniela Cortés-Díaz, Bredford Kerr and Cheril Tapia-Rojas

Neurol. Int. 2024, 16(5), 1066-1085; https://doi.org/10.3390/neurolint16050080 - 23 Sep 2024

Abstract

Background/Objectives: Aging has been extensively studied, with a growing interest in memory impairment by a neurobiological approach. Mitochondrial dysfunction is a hallmark of aging, contributing to the aging phenotype; therefore, mitochondrial interventions seem fundamental. The diet is a physiological approximation for modifying mitochondria, [...] Read more.

Background/Objectives: Aging has been extensively studied, with a growing interest in memory impairment by a neurobiological approach. Mitochondrial dysfunction is a hallmark of aging, contributing to the aging phenotype; therefore, mitochondrial interventions seem fundamental. The diet is a physiological approximation for modifying mitochondria, which could impact the age-related phenotype. Methods: We studied two diets with low-carbohydrate and high-fat compositions, differing in the amount of protein and the fat type disposable—the atherogenic diet Cocoa (high protein/high saturated fat/high cholesterol) and the South Beach diet (very high-protein/high-unsaturated fat)—on oxidative stress, mitochondrial state, and hippocampus-dependent memory in 3-month-old Senescence-Accelerated Mouse Model (SAMP8) seed over 3 months to determine their pro- or anti-aging effects. Results: Despite its bad reputation, the Cocoa diet reduces the reactive oxygen species (ROS) content without impacting the energy state and hippocampus-dependent spatial acuity. In contrast to the beneficial impact proposed for the South Beach diet, it induced a pro-aging phenotype, increasing oxidative damage and the levels of NR2B subunit of the NMDA, impairing energy and spatial acuity. Surprisingly, despite the negative changes observed with both diets, this led to subtle memory impairment, suggesting the activation of compensatory mechanisms preventing more severe cognitive decline. Conclusions: Our results demonstrated that diets usually considered good could be detrimental to the onset of aging. Also, probably due to the brain plasticity of non-aged animals, they compensate for the damage, preventing a more aggravated phenotype. Nevertheless, these silent changes could predispose or increase the risk of suffering pathologies at advanced age. Full article

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27 pages, 452 KiB

Open AccessReview

A Systematic Review of Sporadic Creutzfeldt-Jakob Disease: Pathogenesis, Diagnosis, and Therapeutic Attempts

by Maria Carolina Jurcau, Anamaria Jurcau, Razvan Gabriel Diaconu, Vlad Octavian Hogea and Vharoon Sharma Nunkoo

Neurol. Int. 2024, 16(5), 1039-1065; https://doi.org/10.3390/neurolint16050079 - 20 Sep 2024

Abstract

Creutzfeldt-Jakob disease is a rare neurodegenerative and invariably fatal disease with a fulminant course once the first clinical symptoms emerge. Its incidence appears to be rising, although the increasing figures may be related to the improved diagnostic tools. Due to the highly variable [...] Read more.

Creutzfeldt-Jakob disease is a rare neurodegenerative and invariably fatal disease with a fulminant course once the first clinical symptoms emerge. Its incidence appears to be rising, although the increasing figures may be related to the improved diagnostic tools. Due to the highly variable clinical picture at onset, many specialty physicians should be aware of this disease and refer the patient to a neurologist for complete evaluation. The diagnostic criteria have been changed based on the considerable progress made in research on the pathogenesis and on the identification of reliable biomarkers. Moreover, accumulated knowledge on pathogenesis led to the identification of a series of possible therapeutic targets, although, given the low incidence and very rapid course, the evaluation of safety and efficacy of these therapeutic strategies is challenging. Full article

13 pages, 6389 KiB

Open AccessArticle

Outcome Prediction by Diffusion Tensor Imaging (DTI) in Patients with Traumatic Injuries of the Median Nerve

by Théa Voser, Manuel Martin, Issiaka Muriset, Michaela Winkler, Jean-Baptiste Ledoux, Yasser Alemán-Gómez and Sébastien Durand

Neurol. Int. 2024, 16(5), 1026-1038; https://doi.org/10.3390/neurolint16050078 - 19 Sep 2024

Abstract

Background/Objectives: The accurate quantification of peripheral nerve axonal regeneration after injury is critically important. Current strategies are limited to detecting early reinnervation. DTI is an MRI modality permitting the assessment of fractional anisotropy, which increases with axonal regeneration. The aim of this pilot [...] Read more.

Background/Objectives: The accurate quantification of peripheral nerve axonal regeneration after injury is critically important. Current strategies are limited to detecting early reinnervation. DTI is an MRI modality permitting the assessment of fractional anisotropy, which increases with axonal regeneration. The aim of this pilot study is to evaluate DTI as a potential predictive factor of clinical outcome after median nerve section and microsurgical repair. Methods: We included 10 patients with a complete section of the median nerve, who underwent microsurgical repair up to 7 days after injury. The follow-up period was 1 year, including the current strategy with clinical visits, the Rosén–Lundborg score and electroneuromyography. Additionally, DTI MRI of the injured wrist was planned 1, 3 and 12 months post-operatively and once for the contralateral wrist. Results: The interobserver reliability of DTI measures was almost perfect (ICC 0.802). We report an early statistically significant increase in the fractional anisotropy value after median nerve repair, especially in the region located distal to the suture. Meanwhile, Rosén–Lundborg score gradually increased between the third and sixth month, and continued to increase between the sixth and twelfth month. Conclusions: DTI outcomes three months post-operation could offer greater predictability compared to current strategies. This would enable faster decision-making regarding the need for a potential re-operation in cases of inadequate early reinnervation. Full article

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12 pages, 2391 KiB

Open AccessCase Report

Anterograde Intramedullary Nailing without Bone Grafting for Humeral Shaft Nonunion Associated with Early Exploration of Secondary Radial Nerve Palsy: A Case Report

by Dan Viorel Nistor, Răzvan Marian Melinte and Romana von Mengershausen

Neurol. Int. 2024, 16(5), 1014-1025; https://doi.org/10.3390/neurolint16050077 - 15 Sep 2024

Abstract

Background: Humeral shaft fractures are relatively common. Complications associated with this type of fracture and its treatment include nonunion and radial nerve palsy. Plate osteosynthesis with autologous bone grafting is considered the gold standard for treating nonunion. However, bone grafts might not always [...] Read more.

Background: Humeral shaft fractures are relatively common. Complications associated with this type of fracture and its treatment include nonunion and radial nerve palsy. Plate osteosynthesis with autologous bone grafting is considered the gold standard for treating nonunion. However, bone grafts might not always be necessary in cases of hypertrophic nonunion, and treatment should be tailored to the specific type and characteristics of the nonunion. The treatment of radial nerve palsy is debated, with some favoring expectant management based on the nerve’s ability to regenerate, and others preferring early surgical exploration to prevent possible lasting nerve damage. Methods: We present the case of a 46-year-old male patient with a six-year-old humeral shaft fracture resulting in hypertrophic nonunion. We treated the nonunion with anterograde intramedullary nailing without bone grafting. Postoperatively, the patient developed severe radial nerve palsy. After repeated electrophysiological studies, a decision was made to surgically explore the nerve 10 days after the nonunion surgery. The nerve was subsequently found to be intact and treated with neurolysis. Results: Bony union was shown at six months after nonunion surgery. Four months after the nonunion surgery, the patient started to show clinical signs of nerve recovery, and at 12 months he achieved nearly full clinical recovery of radial nerve function. Conclusions: Anterograde intramedullary nailing without autologous bone grafting may be considered an option for treating hypertrophic nonunion. The management of radial nerve palsy requires effective cooperation and communication between patient and physician. Further research is necessary to be able to better predict nerve recovery. Full article

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9 pages, 258 KiB

Open AccessBrief Report

Romanian Translation and Cultural Adaptation of the Seizure Severity Questionnaire

by Ionut-Horia Cioriceanu, Dan-Alexandru Constantin, Bianca Zamfirescu, Petru Cezar Podasca, Luigi Geo Marceanu and Liliana Rogozea

Neurol. Int. 2024, 16(5), 1005-1013; https://doi.org/10.3390/neurolint16050076 - 13 Sep 2024

Abstract

The aim of this study was to report the translation into Romanian of the Seizure Severity Questionnaire (SSQ), an instrument for the evaluation of the frequency and severity of epileptic seizures, and the results of applying it to a group of patients with [...] Read more.

The aim of this study was to report the translation into Romanian of the Seizure Severity Questionnaire (SSQ), an instrument for the evaluation of the frequency and severity of epileptic seizures, and the results of applying it to a group of patients with epilepsy evaluated at a hospital in Romania. Methods: Four translators were involved in obtaining conceptual analogies and the cultural importance of the translated notions. The final version was obtained for the Romanian population, with the same appearance as the original instrument. Sixty-seven patients with epilepsy completed the SSQ and the Patient-Weighted Quality of Life in Epilepsy Inventory—QOLIE-31-P. Results: Females had a lower mean SSQ total score (TS) and perceived seizures less seriously than men. Patients with epilepsy with aura had a higher mean SSQ TS, with a more severe seizure perception, compared to those without aura. According to the frequency of seizures, patients with epilepsy with rare seizures had the lowest mean SSQ total score (TS) compared to those with frequent seizures. Patients who were on monotherapy had a less severe perception of epileptic seizures compared to those who were treated with two or more antiepileptic drugs. All QOLIE-31-P domains and TS correlated statistically significantly with the SSQ TS. Conclusions: This study explored SSQ translation, evaluated preliminary results, and showed the correlation between seizure frequency and severity, clinical factors, and quality of life. This tool could be useful for measuring seizure severity in Romanian patients with epilepsy and conducting comparative studies. Full article

(This article belongs to the Special Issue Selected Papers from "Brainstorming Research Assembly for Young Neuroscientists (BraYn))

13 pages, 1724 KiB

Open AccessArticle

Cerebrovascular Reactivity Assessed by Breath-Hold Functional MRI in Patients with Neurological Post-COVID-19 Syndrome—A Pilot Study

by Leonie Zerweck, Uwe Klose, Annerose Mengel, Tobias Hoheisel, Melinda Eikemeier, Vivien Richter, Natalie Sophie Joos, Ulrike Ernemann, Benjamin Bender and Till-Karsten Hauser

Neurol. Int. 2024, 16(5), 992-1004; https://doi.org/10.3390/neurolint16050075 - 9 Sep 2024

Abstract

Endothelial dysfunction represents a potential pathomechanism of neurological post-COVID-19 syndrome (PCS). A recent study demonstrated reduced cerebrovascular reactivity (CVR) in patients with PCS. The aim of this pilot study was to prospectively assess CVR in patients with PCS using breath-hold functional MRI (bh-fMRI). [...] Read more.

Endothelial dysfunction represents a potential pathomechanism of neurological post-COVID-19 syndrome (PCS). A recent study demonstrated reduced cerebrovascular reactivity (CVR) in patients with PCS. The aim of this pilot study was to prospectively assess CVR in patients with PCS using breath-hold functional MRI (bh-fMRI). Fourteen patients with neurological PCS and leading symptoms of fatigue/memory issues/concentration disorder (PCS_fmc), 11 patients with PCS and leading symptoms of myopathy/neuropathy (PCS_mn), and 17 healthy controls underwent bh-fMRI. Signal change and time to peak (TTP) were assessed globally and in seven regions of interest and compared between the subgroups using one-way ANCOVA adjusting for age, time since infection, Fazekas score, and sex. No significant differences were observed. In PCS patients, the global CVR exhibited a slight, non-significant tendency to be lower compared to healthy controls (PCS_fmc: 0.78 ± 0.11%, PCS_mn: 0.84 ± 0.10% and 0.87 ± 0.07%). There was a non-significant trend towards lower global TTP values in the PCS subgroups than in the control group (PCS_fmc: 26.41 ± 1.39 s, PCS_mn: 26.32 ± 1.36 s versus 29.52 ± 0.93 s). Endothelial dysfunction does not seem to be the sole pathomechanism of neurological symptoms in PCS. Further studies in larger cohorts are required. Full article

(This article belongs to the Special Issue COVID-19, Neuroinflammation and Therapeutics, 2nd Edition)

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16 pages, 4936 KiB

Open AccessArticle

Fluorescein Angiography for Monitoring Neural Blood Flow in Chronic Nerve Compression Neuropathy: Experimental Animal Models and Preliminary Clinical Observations

by Kosuke Saito, Mitsuhiro Okada, Takuya Yokoi, Shunpei Hama and Hiroaki Nakamura

Neurol. Int. 2024, 16(5), 976-991; https://doi.org/10.3390/neurolint16050074 - 5 Sep 2024

Abstract

Pathologies associated with neural blood disturbance have been reported in patients with chronic nerve compression (CNC) neuropathy. Fluorescein angiography (FAG) and laser Doppler flowmetry (LDF) are effective for real-time peripheral nerve blood flow assessment. However, their reliability in severe neuropathy models in large [...] Read more.

Pathologies associated with neural blood disturbance have been reported in patients with chronic nerve compression (CNC) neuropathy. Fluorescein angiography (FAG) and laser Doppler flowmetry (LDF) are effective for real-time peripheral nerve blood flow assessment. However, their reliability in severe neuropathy models in large animals or clinical conditions remains unclear. Initially, we aim to apply FAG to two different CNC animal models and evaluate their characteristics in comparison with those of LDF. In FAG, we quantified the peak luminance at the compression site following fluorescein injection. Then, we positioned the LDF probe at the center of the compression site and recorded the blood flow. Subsequently, we analyzed whether the FAG characteristics obtained in this animal experiment were consistent with those of clinical studies in patients with severe carpal tunnel syndrome (CTS). In the CNC rat model, FAG and LDF effectively monitored reduced neural blood flow over time. We observed significant blood flow reduction using both techniques in a newly developed severe CNC rabbit model. Notably, FAG correlated strongly with the compound muscle action potential (CMAP) amplitude in electrodiagnostic findings, unlike LDF. As a next step, we performed FAG after open carpal tunnel release in clinical cases of CTS. FAG correlated significantly with preoperative CMAP amplitude. This indicates FAG’s importance for assessing nerve blood flow during surgery, potentially improving diagnostic accuracy and surgical outcomes. Full article

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10 pages, 5382 KiB

Open AccessArticle

The Clinical Manifestations, Risk Factors, Etiologies, and Outcomes of Adult Patients with Infectious Meningitis and Encephalitis: Single Center Experience

by Seraj Makkawi, Shatha Alqurashi, Wejdan Hubayni, Saleha Almahdawi, Sadeem Bahkali, Abeer Alharbi, Osama Khojah, Aisha Halawani and Israa Malli

Neurol. Int. 2024, 16(5), 966-975; https://doi.org/10.3390/neurolint16050073 - 5 Sep 2024

Abstract

(1) Background: Central nervous system (CNS) infections, including meningitis and encephalitis, are serious conditions which are associated with high morbidity and mortality. This study aims to identify the clinical manifestations, etiologies, and outcomes of meningitis and encephalitis in adult patients in Saudi Arabia, [...] Read more.

(1) Background: Central nervous system (CNS) infections, including meningitis and encephalitis, are serious conditions which are associated with high morbidity and mortality. This study aims to identify the clinical manifestations, etiologies, and outcomes of meningitis and encephalitis in adult patients in Saudi Arabia, addressing the current gap in understanding these conditions within this population. (2) Methods: This is a single-center retrospective study which included all adult patients diagnosed with meningitis and encephalitis from March 2016 to May 2022. (3) Results: This study found that most cases of meningitis and encephalitis occurred due to unknown pathogens. Pretreatment with antibiotics prior to lumbar puncture (LP) was found in 71.2% of patients with meningitis. Altered mental status and seizures were common presenting symptoms among patients with encephalitis while altered mental status and fever were common among patients with meningitis. (4) Conclusions: Adherence to guidelines in treating meningitis and encephalitis and performing LPs in a timely manner are important. Establishing national biobanks with biological samples from patients suspected of having meningitis or encephalitis will significantly enhance our understanding of these conditions in Saudi Arabia. Full article

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8 pages, 1528 KiB

Open AccessCase Report

Transient Ipsilateral Hemineglect Following Brain Laser Ablation in Patient with Focal Cortical Dysplasia

by Georgios Ntolkeras, Fatemeh Mohammadpour Touserkani, Michelle Y. Chiu, Sanjay P. Prabhu, Scellig Stone and Alexander Rotenberg

Neurol. Int. 2024, 16(5), 958-965; https://doi.org/10.3390/neurolint16050072 - 3 Sep 2024

Abstract

Sensory integration is the province of the parietal lobe. The non-dominant hemisphere is responsible for both body sides, while the dominant hemisphere is responsible for the contralateral hemi-body. Furthermore, the posterior cingulate cortex (PCC) participates in a network involved in spatial orientation, attention, [...] Read more.

Sensory integration is the province of the parietal lobe. The non-dominant hemisphere is responsible for both body sides, while the dominant hemisphere is responsible for the contralateral hemi-body. Furthermore, the posterior cingulate cortex (PCC) participates in a network involved in spatial orientation, attention, and spatial and episodic memory. Laser interstitial thermotherapy (LiTT) is a minimally invasive surgery for focal drug-resistant epilepsy (DRE) that can target deeper brain regions, and thus, region-specific symptoms can emerge. Here, we present an 18-year-old right-handed male with focal DRE who experienced seizures characterized by sensations of déjà vu, staring spells, and language disruption. A comprehensive evaluation localized the seizure focus and revealed a probable focal cortical dysplasia (FCD) in the left posterior cingulate gyrus. The patient underwent uneventful LiTT of the identified lesion. Post-operatively, he developed transient ipsilateral spatial neglect and contralateral sensory loss, as well as acalculia. His sensory symptoms gradually improved after the surgery, and he remained seizure-free after the intervention for at least 10 months (until the time of this writing). This rare case of ipsilateral spatial and visual hemineglect post-LiTT in epilepsy underscores the importance of recognizing atypical neurosurgical outcomes and considering individual variations in brain anatomy and function. Understanding the dynamics of cortical connectivity and handedness, particularly in pediatric epilepsy, may be crucial in anticipating and managing neurocognitive effects following epilepsy surgery. Full article

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13 pages, 2074 KiB

Open AccessArticle

Quantifying the Transition from Unconscious to Conscious Detection of Temporal Patterns in Vigilance Tasks: A Unique Adaptation of Mackworth’s Clock Test

by Guaraci Lima de Morais, Tatiana Okubo Rocha Pinho, Leonardo Crespim and Osmar Pinto Neto

Neurol. Int. 2024, 16(5), 945-957; https://doi.org/10.3390/neurolint16050071 - 31 Aug 2024

Abstract

This study investigates the cognitive mechanisms underlying vigilance and pattern recognition using a novel adaptation of Mackworth’s Clock Test. We aimed to quantify the time it takes for temporal patterns detected unconsciously through implicit learning to surface in the conscious mind within a [...] Read more.

This study investigates the cognitive mechanisms underlying vigilance and pattern recognition using a novel adaptation of Mackworth’s Clock Test. We aimed to quantify the time it takes for temporal patterns detected unconsciously through implicit learning to surface in the conscious mind within a dynamic vigilance task environment. Forty-eight participants detected random and non-disclosed rhythmic anomalous clock hand movements in this setting. Our results indicate significant variability in detection accuracy, reaction times, and the ability to recognize the hidden pattern among participants. Notably, 23% of all participants and 56% of those who consciously reported the pattern exhibited statistically lower reaction times indicative of knowledge of the pattern 40 s before conscious identification. These findings provide valuable insights into the transition from unconscious to conscious detection, highlighting the complexity of sustained attention and pattern recognition. The study’s implications extend to designing training programs and tasks for high-stakes professions requiring prolonged vigilance. Future research should further explore the cognitive and neural correlates of these processes and the impact of task complexity on performance. Full article

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12 pages, 770 KiB

Open AccessReview

Perspectives on Stem Cell Therapy in Diabetic Neuropathic Pain

by Tadeu Lima Montagnoli, Aimeé Diogenes Santos, Susumu Zapata Sudo, Fernanda Gubert, Juliana Ferreira Vasques, Rosalia Mendez-Otero, Mauro Paes Leme de Sá and Gisele Zapata-Sudo

Neurol. Int. 2024, 16(5), 933-944; https://doi.org/10.3390/neurolint16050070 - 30 Aug 2024

Abstract

Diabetes mellitus-related morbidity and mortality are primarily caused by long-term complications such as retinopathy, nephropathy, cardiomyopathy, and neuropathy. Diabetic neuropathy (DN) involves the progressive degeneration of axons and nerve fibers due to chronic exposure to hyperglycemia. This metabolic disturbance leads to excessive activation [...] Read more.

Diabetes mellitus-related morbidity and mortality are primarily caused by long-term complications such as retinopathy, nephropathy, cardiomyopathy, and neuropathy. Diabetic neuropathy (DN) involves the progressive degeneration of axons and nerve fibers due to chronic exposure to hyperglycemia. This metabolic disturbance leads to excessive activation of the glycolytic pathway, inducing oxidative stress and mitochondrial dysfunction, ultimately resulting in nerve damage. There is no specific treatment for painful DN, and new approaches should aim not only to relieve pain but also to prevent oxidative stress and reduce inflammation. Given that existing therapies for painful DN are not effective for diabetic patients, mesenchymal stromal cells (MSCs)-based therapy shows promise for providing immunomodulatory and paracrine regulatory functions. MSCs from various sources can improve neuronal dysfunction associated with DN. Transplantation of MSCs has led to a reduction in hyperalgesia and allodynia, along with the recovery of nerve function in diabetic rats. While the pathogenesis of diabetic neuropathic pain is complex, clinical trials have demonstrated the importance of MSCs in modulating the immune response in diabetic patients. MSCs reduce the levels of inflammatory factors and increase anti-inflammatory cytokines, thereby interfering with the progression of DM. Further investigation is necessary to ensure the safety and efficacy of MSCs in preventing or treating neuropathic pain in diabetic patients. Full article

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15 pages, 2726 KiB

Open AccessArticle

Understanding the Changes in Brain Activation When Viewing Products with Differences in Attractiveness

by Emily L. L. Sin, Clive H. Y. Wong, Bolton K. H. Chau, Matthias Rauterberg, Kin Wai Michael Siu and Yi-Teng Shih

Neurol. Int. 2024, 16(5), 918-932; https://doi.org/10.3390/neurolint16050069 - 28 Aug 2024

Abstract

Product design and attractiveness are pivotal factors that determine people’s positive reactions when viewing a product and may eventually affect their purchasing choices. Comprehending how people assess product design is crucial. Various studies have explored the link between product attractiveness and consumer behavior, [...] Read more.

Product design and attractiveness are pivotal factors that determine people’s positive reactions when viewing a product and may eventually affect their purchasing choices. Comprehending how people assess product design is crucial. Various studies have explored the link between product attractiveness and consumer behavior, but these were predominantly behavioral studies that offered limited insight into the neural processes underlying perceptions of product attractiveness. Gaining a deeper understanding of these neural mechanisms is valuable, as it enables the formulation of more objective design guidelines based on brain activity, enhancing product appeal and, ultimately, spurring consumer purchases. In our study, we sought to (1) elucidate the neural network engaged when individuals evaluate highly attractive product images, (2) delineate the neural network activated during the evaluation of less attractive product images, and (3) contrast the differences in neural networks between evaluations of highly and less attractive images. We utilized fMRI to investigate the neural activation patterns elicited by viewing product images of varying attractiveness levels. The results indicated distinct neural activations in response to the two types of attractive images. Highly attractive product images elicited activity in the anterior cingulate cortex (ACC) and the occipital pole, whereas less attractive product images stimulated the insula and the inferior frontal gyrus (IFG). The findings of this project provide some of the first insights of its kind and valuable insights for future product design, suggesting that incorporating more positive and rewarding elements could enhance product appeal. This research elucidates the neural correlates of people’s responses to product attractiveness, revealing that highly attractive designs activate reward-related brain regions, while less attractive designs engage areas associated with emotional processing. These findings offer a neuroscientific basis for further studies on developing design strategies that align with consumers’ innate preferences, potentially transforming product design and marketing practices. By leveraging this knowledge, designers can craft products that not only meet functional needs but also resonate more deeply on an esthetic level, thereby enhancing consumer engagement and purchase likelihood. Full article

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13 pages, 5853 KiB

Open AccessArticle

HSPB4/CRYAA Protect Photoreceptors during Retinal Detachment in Part through FAIM2 Regulation

by Cagri G. Besirli, Madhu Nath, Jingyu Yao, Mercy Pawar, Angela M. Myers, David Zacks and Patrice E. Fort

Neurol. Int. 2024, 16(5), 905-917; https://doi.org/10.3390/neurolint16050068 - 26 Aug 2024

Abstract

Our previous study discussed crystallin family induction in an experimental rat model of retinal detachment. Therefore, we attempted to evaluate the role of α-crystallin in photoreceptor survival in an experimental model of retinal detachment, as well as its association with the intrinsically neuroprotective [...] Read more.

Our previous study discussed crystallin family induction in an experimental rat model of retinal detachment. Therefore, we attempted to evaluate the role of α-crystallin in photoreceptor survival in an experimental model of retinal detachment, as well as its association with the intrinsically neuroprotective protein Fas-apoptotic inhibitory molecule 2 (FAIM2). Separation of retina and RPE was induced in rat and mouse eyes by subretinal injection of hyaluronic acid. Retinas were subsequently analyzed for the presence αA-crystallin (HSPB4) and αB-crystallin (HSPB5) proteins using immunohistochemistry and immunoblotting. Photoreceptor death was analyzed using terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick end labeling (TUNEL) staining and cell counts. The 661W cells subjected to FasL were used as a cell model of photoreceptor degeneration to assess the mechanisms of the protective effect of αA-crystallin and its dependence on its phosphorylation on T148. We further evaluated the interaction between FAIM2 and αA-crystallin using a co-immunoprecipitation assay. Our results showed that α-crystallin protein levels were rapidly induced in response to retinal detachment, with αA-crystallin playing a particularly important role in protecting photoreceptors during retinal detachment. Our data also show that the photoreceptor intrinsically neuroprotective protein FAIM2 is induced and interacts with α-crystallins following retinal detachment. Mechanistically, our work also demonstrated that the phosphorylation of αA-crystallin is important for the interaction of αA-crystallin with FAIM2 and their neuroprotective effect. Thus, αA-crystallin is involved in the regulation of photoreceptor survival during retinal detachment, playing a key role in the stabilization of FAIM2, serving as an important modulator of photoreceptor cell survival under chronic stress conditions. Full article

(This article belongs to the Collection Advances in Neurodegenerative Diseases)

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14 pages, 1533 KiB

Open AccessArticle

Quality of Life Assessment in Romanian Patients with Spinal Muscular Atrophy Undergoing Nusinersen Treatment

by Bogdana Cavaloiu, Iulia-Elena Simina, Lazar Chisavu, Crisanda Vilciu, Iuliana-Anamaria Trăilă and Maria Puiu

Neurol. Int. 2024, 16(5), 891-904; https://doi.org/10.3390/neurolint16050067 - 26 Aug 2024

Abstract

Spinal muscular atrophy (SMA), identified over a century ago, is characterized by severe muscle wasting and early mortality. Despite its rarity, the high carrier frequency of the responsible genetic mutations and the variability in its manifestations make it a significant research focus. This [...] Read more.

Spinal muscular atrophy (SMA), identified over a century ago, is characterized by severe muscle wasting and early mortality. Despite its rarity, the high carrier frequency of the responsible genetic mutations and the variability in its manifestations make it a significant research focus. This prospective cross-sectional descriptive study evaluated health-related quality of life (HRQoL) across eight health domains in 43 Romanian SMA patients treated with nusinersen, using the SF-36 questionnaire to analyze influencing factors. The survey was conducted online with informed consent, and the data were analyzed using MedCalc software, employing both parametric and non-parametric statistical tests for accurate interpretation. The results revealed significant variations in HRQoL. Most patients were non-ambulatory (74.4%), reflecting SMA’s impact on mobility. Urban residents reported better outcomes, particularly in physical functioning (p = 0.014), which may be attributed to improved access to healthcare services. Younger participants (under 14), represented by proxy responses, noted better general health (p = 0.0072) and emotional well-being (p = 0.0217) compared to older participants. These findings suggest that younger patients or their proxies perceive a better health status, highlighting the need for age-specific approaches in SMA management and the potential optimistic bias associated with proxy reporting on perceived health outcomes. Full article

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