Chronic Ischemic Changes Research Articles

B - 3 The Role of Artificial Intelligence: Utilization of the Meyers Battery for Diagnostically Differentiating a Complex Neuropsychological Case

Abstract Objectives As medical technology progresses, artificial intelligence (AI) is becoming a common method for providing diagnostic information to patients. In neuropsychology, the Meyers Neuropsychological Battery (MNB) provides impartial data through artificial intelligence. The following case illustrates how AI provided diagnostic clarity and aided in treatment planning. Method A 65-year-old female was referred by her neurosurgeon due to cognitive decline, delusions and hallucinations, and episodes of aggression toward her caretaker. A full, comprehensive neuropsychological examination was administered. Her medical history was remarkable for hypertension, hypercholesterolemia, thyroid cancer, two cerebrovascular accidents, traumatic brain injury, and multiple ischemic strokes. The patient’s most recent imaging was remarkable for chronic encephalomalacia involving the R basal ganglia, mild chronic small vessel ischemic changes, a ventricular shunt within the anterior horn of the R lateral ventricle, and post-coil embolization of a right supraclinoid artery. Results The patient was administered the MNB along with psychological measures. Results were consistent with diffuse and lateralized (RCH > LCH) neuropsychological impairment. Employing the MNB algorithms and AI, this patient’s data matched statistically with Multi-Infarct Dementia, Hydrocephalus, and Lewy Body Dementia. Conclusion These independent applications and AI enable neuropsychologists to respond beyond the “referral question” and provide an objective perspective unique to the patient’s neuropsychological profile. AI is not applied as the only diagnostic approach but rather provides the patient’s medical team with an alternative perspective regarding the entire neuropsychological picture. MNB’s AI not only aids in “matching” neurological conditions to the patient’s profile but also excludes proposed diagnoses in question and underscores a realistic treatment plan.

Spectrum of neurodegenerative disorders in neurology outpatient department: A cross-sectional study.

Neurodegenerative disorders (NDDs) are a growing global health concern with a rise in prevalence with the aging population, leading to increased healthcare utilization and costs. Understanding its prevalence is crucial for effective diagnostics and resource allocation, especially in developing nations with limited resources. This study aims to explore the frequency and types of NDDs, while also collecting demographic, clinical, and neuro-radiological data from patients with NDDs attending a tertiary care hospital in Nepal. This was a single-center based cross-sectional descriptive study conducted at a Neurology outpatient department in a tertiary level hospital in Nepal in which patients aged 18 and above diagnosed with NDDs (May 2023-July 2023) were included. Data were collected and analyzed in SPSS Inc. This study has been presented by the STROBES guidelines. The mean age of the 71 patients included in the study was 65.6 ± 13.3 years. Parkinsonian disorder (n = 41, 57.7%) was the most common NDD diagnosed. Patients belonging to the age group 60-79 years represented 62% of all outpatient visits. Tremors of the upper extremity and impairment of memory were the most commonly encountered first symptoms at onset. Predominant cognitive changes in our study were memory impairment and mood changes. Extrapyramidal features such as gait disturbance, resting tremor, rigidity, and bradykinesia were present. More than half of the patients had age-related cerebral atrophy on neuroimaging followed by chronic small vessel ischemic changes. Diagnosing NDDs poses challenges, and our study underscores Parkinsonian disorder, specifically Parkinson's disease, as the prevailing neurodegenerative condition in our population. Emphasizing its prevalence among the elderly, particularly with tremors as the primary presenting symptom, highlights the necessity for targeted interventions in this demographic.

Pathological Characteristics and Classification of Unstable Coronary Atherosclerotic Plaques.

Important forensic diagnostic indicators of sudden death in coronary atherosclerotic heart disease, such as acute or chronic myocardial ischemic changes, sometimes make it difficult to locate the ischemic site due to the short death process, the lack of tissue reaction time. In some cases, the deceased died of sudden death on the first-episode, resulting in difficulty for medical examiners to make an accurate diagnosis. However, clinical studies on coronary instability plaque revealed the key role of coronary spasm and thrombosis caused by their lesions in sudden coronary death process. This paper mainly summarizes the pathological characteristics of unstable coronary plaque based on clinical medical research, including plaque rupture, plaque erosion and calcified nodules, as well as the influencing factors leading to plaque instability, and briefly describes the research progress and technique of the atherosclerotic plaques, in order to improve the study on the mechanism of sudden coronary death and improve the accuracy of the forensic diagnosis of sudden coronary death by diagnosing different pathologic states of coronary atherosclerotic plaques.

Inadvertent Discovery of Gerstmann's Syndrome During a Skull MRI and Its Anatomical Basis: A Case Report

A rare neurological disorder in which an individual is unable to perform tasks related to language, spatial recognition,mathematics, and cognition is known as Gerstmann's syndrome(GS). Gerstmann hypothesised that the four symptoms thatmakeup Gerstmann's syndrome are caused by a shared cognitive problem (Grundstörung). Gerstmann asserted that it is a bodyschema disorder that only affects the hand and fingers. Since then, the existence of a Grundstörung has been disputed. The studyproposed a shared psychoneurological element, but it should be connected to changes in mental pictures rather than the bodilyschema. The significant facts appear to be the common simultaneous impairment of writing, computation, and bodily awarenessafter left parietal lesion, beyond the diagnostic label of "pure" and "non-pure" Gerstamann's syndrome instances. Some functionaldomains can be linked to patients' symptoms despite being heterogeneous. A 65-year-old male was brought to the hospital witha history of injury to the head and complaints of confusion, speech difficulty, inability to perform simple calculations, severeheadache, and drowsiness. The patient was sent to the radiology department of Sree Balaji Medical College and Hospital,Chennai, for a Magnetic Resonance Imaging of Skull (MRI). The MRI study revealed chronic ischemic changes in periventricularand subcortical white matter in the parietal lobes. Certain ischemic changes were also observed in the frontal lobe, suggestingage-related changes and Gerstmann's syndrome. By cutting off various portions of the inferior parietal cortex, a lesion affectingsubcortical and subangular bundles of fibers affects many circuits with distinct roles. Therefore, the deficits seen in the patientare best explained by this anatomical explanation.

Evaluation of Diagnostic Value of Computed Tomography in Headache Patients in Benghazi

Headache is the most common complaint faced by physicians. Referring these cases for a computed tomography (CT) scan requires awareness of red flags in the history and examination by physicians. An assessment of the diagnostic utility of CT among headache patients will help determine the most prevalent causes of headache and identify those who get benefit from it. is to find out the proportion of cranial abnormalities in patients with headache without neurologic abnormalities with the use of a CT scan. Also, to illustrate the most common causes of headache in these patients. This study was carried out among 217 patients with isolated headache who underwent a plain, non-contrast enhanced CT of the brain and para-nasal sinus (PNS).The median age was 34 years. The most prevalent age group was between 20 and 39 years old. The most common cases were females. The female to male ratio was 1.5:1. The most frequently occurring cases in 2012 came from the ear, nose, and throat (ENT) department. The paranasal sinuses (PNS) scan was used by 58.53%, and the brain scan was used by 41.47%. The normal scan was 55.3% and the positive scan was 44.7%, which was further categorized into minor incidental findings (17.97%) and significant abnormalities (26.73%).Abnormal findings represent 44.7% of cases. The most common major abnormality was sinusitis. An equal proportion (3.45%) of major abnormalities included sino-nasal polyposis, chronic small-vessel ischemic changes, a suspicious brain tumor, and a suspicious nasopharyngeal mass.

Anterior Cervical Discectomy and Fixation Fusion for Hirayama Disease in a Young Male

Hirayama disease (HD) is a rare, monomelic amyotrophy described by Hirayama in 1959. The condition is caused by chronic ischemic changes to the anterior horn cells of the cervical spine, caused by posterior dural sac laxity. It is a nonprogressive and self-limiting disease and has been noted to be a source of significant disability for affected young individuals. Early surgical intervention in HD limits the progression and thus decreases the degree of disability.

Superficial and Deep Capillary Plexus Nonperfusion in Nonaccidental Injury on OCTA.

A 5-year-old Caucasian male with a history nonaccidental injury (NAI) at age 1 presented with new onset no light perception in the left eye due to a closed funnel retinal detachment. The right eye showed optic nerve pallor, peripheral vascular attenuation, and leakage. Optical coherence tomography angiography (OCTA) revealed significant parafoveal attenuation of the superficial vascular plexus, intermediate capillary plexus, and the deep capillary plexus. This correlated with inner and middle retinal layer thinning temporal to the fovea and preservation of the ellipsoid zone. The peripapillary vascular plexus was preserved. Laser photocoagulation was performed to the nonperfused peripheral retina and intravitreal bevacizumab was injected. OCTA may be used in patients with NAI to characterize macular ischemia changes. Attenuation of the superficial, intermediate, and deep capillary plexuses in our patient may represent chronic ischemic retinal changes that arise from traumatic injury to the vitreoretinal interface and inner retina in NAI.

Brain MRI Screening and Abnormal Findings in Sickle Cell Patients, a Single Center Experience

Background Neuropsychiatric effects of Sickle Cell Disease (SCD) are not very well understood. Sickle cells cause vaso-occlusion and ischemic manifestations that can affect various organ systems. The cumulative effect of insults to the brain can be detrimental resulting in potential cognitive delays. Regular blood transfusions and hydroxyurea are established as preventative and therapeutic measures to decrease the risk of cerebrovascular accidents. Strong evidence for timing of screening for brain damage in SCD patients is not available (S C Thust et al). A conditional recommendation from the ASH guideline panel 2020 suggests at least a one-time screening MRI to detect silent cerebral infarcts in adults with SCD (DeBaun MR et al) Objectives 1. To determine rates of obtaining screening MRI for adult SCD patients enrolled in the Sickle Cell Clinic at Henry Ford Hospital 2. To investigate brain parenchymal changes manifested by Brain MRIs and Head CTs and study associated factors Methods: We performed a retrospective chart review on 105 patients enrolled in the sickle cell clinic at Henry Ford Hospital who have been followed from June 2013 - June 2022. Baseline characteristics, laboratory findings and presence of comorbidities along with provided therapies are compared among patients with abnormal MRI findings. Results Out of the 105 patients, 20 (19%) had a Brain MRI done at least once during their lifetime for various reasons. A total of 14/20 MRI reports picked up abnormal findings (70%). Conversely, 44 patients had a CT head done (42%), 17 of which described abnormal findings (39%). Of note, only 4 patients had a transcranial doppler ultrasound, all of which were normal. Dissecting those 14 brain MRI reports, 7 cases had ischemic changes/chronic infarcts, 2 cases had white matter changes and 6 cases had other findings including tonsillar ectopia, cerebral and cerebellar volume loss, aneurysms, and degenerative changes. The baseline characteristics as well as clinical parameters of those patients are described in tables 1 and 2. Looking at the 17 total abnormal head CT reports: 9 cases were described as chronic ischemic changes, 3 hemorrhage cases (one subarachnoid hemorrhage, one subdural and one intracranial hematoma), 2 cases had brain atrophy and 4 cases had other findings including encephalomalacia, punctate hyper-density and trabeculation or lacy appearance of the skull base. Discussion At least 25% of individuals with SCD will have a neurological complication over their lifetime (Lonergan G et al). Despite paucity of literature looking into prevalence of neuroradiological findings, they have been reported in 44-60% of patients with SCD (Silva et al). Looking at our center's experience approximately half of the screened patients had chronic ischemic changes reported, which intuitively corelates with silent infarcts known to happen in SCD patients. Patients with neurological deficits and developmental delays are more likely to be screened. Despite the lack of statistical power to extrapolate further, it seemed that patients with abnormal brain MRI reports were more likely to have Congestive Heart Failure and Deep Vein Thrombosis. They also possibly consumed more alcohol and illicit drugs. Those patients were slightly more likely to be on hydroxyurea and had more frequent blood transfusions, emergency department visits and hospitalizations. Worth mentioning, out of the 14 patients with positive MRI findings, 13 had head CT scans performed concomitantly. 6 of those CT scans reported abnormal findings (46%) and 7 were normal (54%). This infers that there is a high proportion of patients who have normally read head CT scans but have brain parenchymal changes if screened by an MRI. Conclusions Our center presently screened SCD patients by brain MRI in only 19% of cases. The results of this study help to encourage incorporating and reinforcing the implementation of ASH 2020 guidelines. It is likely that patients with more comorbidities have been screened earlier than others. Detection of neurological complications in SCD may potentially reduce ED visits and hospitalizations if picked up and managed early. Our results propose to re-study patient characteristics on a larger scale once more screening is performed and to compare it with our current data, aiming to have more informative conclusions on SCD patients with neuroradiological findings. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

Abstract 10115: Unique Imaging Morphology and Rare Location of Papillary Fibroelastoma Resulting in a Cardioembolic Stroke

Clinical Presentation: Afro-Caribbean female, age 59, with a comorbidity of hypertension presented with acute painless right-sided vision loss. Brain MRI revealed numerous scattered T2/FLAIR hyperintense foci throughout the periventricular, deep, and subcortical white matter, which were favored to represent chronic microvascular ischemic changes. CTA of the head and neck demonstrated mild atherosclerotic calcification of the cavernous segment of the left internal carotid artery without significant stenosis, mild noncalcified atherosclerotic plaque at the right carotid bifurcation, and mild irregularity and narrowing of the mid basilar artery. Cardiac Imaging: Transthoracic Echocardiography (TTE) demonstrated severe concentric left ventricular hypertrophy, left ventricle ejection fraction 60-65%, and a mobile, intracavitary mass measuring 1.60cm x 1.59cm. Repeat TTE estimated the mass to be 1.8cm x 1.2cm in the region of the posteromedial papillary muscle. Transesophageal Echocardiography (TEE) redemonstrated a mobile left ventricular mass measuring 2.0cm x 1.8cm along the inferior wall of the interventricular septum. Left heart catheterization demonstrated nonobstructive coronary artery disease with a 30% stenosis of the proximal Right Coronary and Distal Circumflex. Patient underwent surgical removal of the cardiac mass, biopsy-proven to be a Papillary Fibroelastoma (PFE). Discussion: PFEs account for 7.9% of all primary benign cardiac tumors and are the most common valvular tumor. More than 80% of these tumors are attached on the aortic or mitral valve, unlike in this patient where it was attached to the left ventricular trabeculae without involvement of the mitral valve or sub-valvular apparatus. The classic appearance of PFEs on TTE is pedunculated and often compared to a sea anemone but appeared homogenous in nature with central vacuolization in this case. This made characterizing the mass as a thrombus or cardiac tumor challenging.

Temporal macular thinning and vessel density correlation in children and young adults with sickle cell disease.

Optical coherence tomography angiography (OCTA) is a recent noninvasive imaging technology that has proved to provide a comprehensive evaluation of retinal vascular abnormalities in adult patients with sickle cell disease (SCD). However, the pediatric population remains less studied. The purpose of this study is to evaluate the correlation between temporal vessel density (VD) in OCTA and temporal macular thinning in optical coherence tomography (OCT) in children and young adults with SCD. We reviewed medical records, OCT and OCTA (Spectralis®) scans from a sample of 32 eyes from 16 patients (7 female + 9 male) under 25 years old. The OCT macular thickness data and OCTA image data were processed using Python 3.9 programming language, and statistical analysis was performed. Pearson Correlation Coefficient between macular thickness and VD for inner and outer temporal areas was 0.47 (p-value = 0.006) and 0.74 (p-value < 0.001), respectively. A t-test was also performed to prove that there is a statistically significant VD difference in patients with higher and lower macular thickness (p < 0.001). These results show that children with outer temporal macular thinning on OCT have lower outer temporal VD on OCTA suggesting that microvascular insults may lead to chronic ischemic changes in the inner retinal layers.

Embolic infarct topology differs between atrial fibrillation subtypes and embolic stroke of undetermined source

BackgroundThe lack of superiority of anticoagulation over antiplatelet therapy in embolic stroke of undetermined source (ESUS) may be in part due to the misclassification of radiographic ESUS patterns as cardioembolic. In this imaging analysis, we sought to differentiate clinical and radiographic patterns of ESUS patients from patterns in patients with a highly probable cardioembolic source. Materials & MethodsA prospective registry of consecutive adults with acute infarction on diffusion-weighted magnetic resonance imaging was queried. Patients with infarctions due to small vessel disease, large vessel disease, and other causes were excluded. Multivariable logistic regression was used to identify independent predictors of two potentially embolic patterns: (1) multifocal and (2) cortical lesions, comparing patients with ESUS against those with atrial fibrillation (AF). ResultsAmong 1243 screened patients, 343 (27.6%) experienced strokes due to ESUS or AF. Prior to the index stroke, patients with AF as compared to ESUS were older (median 75 vs. 65, p<0.01) and had more heart failure (25.9% vs. 8.4%, p<0.01). The odds of multifocal infarction were the same between patients with ESUS and both AF subtypes (p>0.05), however, cortical involvement was more associated with both AF versus ESUS (77.7% vs. 65.7%, P=0.02). A higher Fazekas grade of white matter disease was inversely associated with cortical infarction among included patients (aOR 0.77, 95% CI 0.62-0.96). ConclusionCortical infarctions were twice as common among patients with AF versus ESUS. Subcortical infarct topography was strongly associated with chronic microvascular ischemic changes and therefore may not represent embolic phenomena. Larger-scale investigations are warranted to discern whether large or multifocal subcortical infarcts ought to be excluded from the ESUS designation.

Giant Coronary Aneurysms After Pediatric Heart Transplant: Giant Problem

<h3>Introduction</h3> Cardiac allograft vasculopathy (CAV) is a leading cause of death and retransplant after heart transplantation (HTx). Coronary aneurysms are not typically seen in CAV. We present a case of atypical CAV with coronary aneurysms resulting in premature death. <h3>Case Report</h3> A 4-year-old male underwent HTx after failed single ventricle palliation. His first year after HTx was uneventful with no cellular rejection score greater than 1R and no antibody mediated rejection on multiple surveillance endomyocardial biopsies. Coronary angiography was normal at 3 months. At 1-year post-HTx, surveillance coronary angiography demonstrated a diffusely dilated appearance with multiple aneurysmal segments of the right and left coronary systems (Figure). No filling defects or areas of focal stenosis were appreciated. His pulmonary capillary wedge pressure was elevated at 15 mmHg, but stable from previous assessments. There was no known history of Kawasaki disease in the donor. The patient had no history of prolonged fevers or other symptoms of vasculitis. He was placed on enoxaparin and aspirin for anticoagulation. He remained asymptomatic with no regional wall abnormalities and normal systolic function by echocardiogram. He denied episodes of palpitations and had a Holter without any ectopy or arrhythmias. While awaiting further evaluation with additional advanced imaging, he had a sudden cardiac arrest and died 10 months after coronary aneurysms were found. His autopsy revealed segmental fusiform aneurysmal expansion with severe concentric intimal fibrosis and luminal narrowing of multiple segments of the right and left coronary systems consistent with severe CAV. Left ventricular histopathology exhibited chronic ischemic changes to the subendocardium. <h3>Summary</h3> Our patient with severe CAV presented with coronary aneurysm formation within 1 year of HTx. Coronary aneurysm formation may be an atypical and more severe form of CAV that should prompt close monitoring and early consideration for retransplant.

A possible case of Limbic-predominant age-related TDP-43 encephalopathy (LATE) in a Black Female with caregiver burden and limited resources: A Novel Diagnosis with presentation similar to Alzheimer's

<h3>Introduction</h3> Limbic-predominant age-related TDP-43 encephalopathy (LATE) is a proposed neurodegenerative disorder of older adults (typically >80 years old)⁽¹⁾. It presents primarily as an amnestic dementia and is thought to be due to a TDP-43 proteinopathy in limbic system structures, including the hippocampus. There could be coexisting hippocampal sclerosis pathology in some patients presenting with LATE. In community-based autopsy cohorts, ∼25% of brains had a sufficient burden of LATE-neuropathology changes (LATE- NC) to be associated with discernible cognitive impairment. (ADNC) ⁽³⁾. In a retrospective study described by Kapasi et Al, there exist a class I evidence that LATE-NC and Alzheimer's disease pathologic changes are associated with different trajectories of late-life cognitive decline. Persons with pure LATE-NC follow a slower trajectory compared to those with pure ADNC. LATE-neuropathology changes may be co-morbid with Alzheimer's disease neuropathological changes. Those with mixed LATE/ADNC have a steeper decline than individuals with either pathology alone ⁽⁴⁾. The following is a unique case presentation of a patient with possible LATE or a combination of LATE/AD NC as demonstrated by rapid amnestic dementia and changes in brain MRI. In addition, this case shows how the treatment is tailored based on family preference and available resources <h3>Methods</h3> A single patient case of interest was chosen and verbal consent was obtained from the patient. Our initial review was conducted with a literature search at Pubmed. 57 articles were retrieved which was narrowed down to 10 articles after irrelevant topics were excluded. The clinical presentation of the case was found to be correlated with the relevant reports in the research literature implying a possible rationale for LATE vs ADNC for the patient. <h3>Results</h3> Case presentation: This is a case of a 91-year-old female with no past psychiatric history, a medical history significant of HTN, and stable angina who presented to the memory clinic with a decline in short-term memory in the past 18 months that has progressively worsened over the past 6 -8 months. The patient would forget conversations and has been misplacing items at home. The patient would engage in dangerous behaviors to include forgetting the stove on. Also associated were neuropsychiatric symptoms of agitation, irritability, and insomnia. The patient became easily irritable and would attempt to hit others. These behaviors were all new to this patient and her family was very concerned. Taking care of her became increasingly challenging and they decided to consult professional help. The patient became increasingly dependent on all ADLs except feeding and toileting. She had been unable to perform all IADLS for the past two years. On MMSE, she scored 24/30 with most points lost in immediate recall. Further neuropsychiatric testing revealed deficiency in verbal fluency, clock drawing, delay recall, and word list learning. The MRI of the brain indicated a decrease in Hippocampal volume at 7 percentiles for her age as per NEUROQUANT evaluation; almost 2 standard deviations below the mean. Mild/moderate chronic white matter microvascular ischemic changes were also seen. No acute ischemia was noted. The caregiver burden was extremely high and memory medication was not a priority for the family. The family did not qualify for Medicaid and declined much-needed social services that could provide respite and promote social engagement. Paying out of pocket was a hindering factor. A low-dose second-generation antipsychotic was initiated with good remission of agitative symptoms. <h3>Conclusions</h3> The patient's advanced age, delayed onset of the cognitive impairment, precipitous memory decline, and the findings on brain MRI are suggestive of a possible diagnosis of LATE versus LATE ADNC. However, this diagnosis or other forms of dementia cannot be ruled out without a brain biopsy. This case indicates that further research on diagnosing and targeting patients with TDP-43 might greatly improve the disease burden. Furthermore, the differences in social-economic status can greatly add to disparities in dementia care. <h3>This research was funded by</h3> None

Pupil sparing Cranial Nerve III Palsy and Hemiparesis, Weber Syndrome: A Case Report and literature review

Midbrain stroke especially Weber syndrome is a rare case seldom reported in the literature. It involves oculomotor nerve palsy and contralateral hemiparesis. A 46-year-old female presented with sudden onset of blurring of vision along with right-sided hemiparesis, right upper motor neuron type cranial nerve (CN) VII palsy, left-sided CN III palsy, and left-sided ptosis. Magnetic resonance imaging (MRI) revealed T2 flair/hyperintensity in the left side of the midbrain, bilateral gangliocapsular regions, and centrum semi vale indicating acute infarct and chronic ischemic changes respectively as well as a high signal area on the T2/FLAIR sequence, indicating sinusitis. Ocular manifestations of a midbrain stroke are highlighted in this case study, particularly Weber syndrome, which also entails contralateral hemiparesis. A better prognosis can be achieved with early diagnosis and treatment. In our case report, a CT scan was found to be normal and the diagnosis of Weber syndrome was made only after MRI confirming its sensitivity to detect intracranial lesions.

Mesenchymal stem cells derived from epicardial adipose tissue reverse cardiac remodeling in a rabbit model of myocardial infarction.

Myocardial infarction (MI) is one of the most important causes of death. MI-related tissue loss and cardiac remodeling may result in heart failure. Intramyocardial injection of mesenchymal stem cells derived from adipose tissues, in acute MI animal models, has shown promising regenerative capabilities. This study aimed to investigate the myocardial regenerative capacity of epicardial adipose tissue-derived mesenchymal stem cells (ADSCs) in a rabbit model of MI. A rabbit model of MI was performed in three groups: a sham-operated group, a control group, and a treatment group. MI was induced by coronary artery ligation via thoracotomy in the first operation. Four weeks after the first operation, intramyocardial injections of phosphate-buffered saline (PBS; control group) or ADSCs (10×106 in 100 μL; treatment group) were performed in the peri-infarct zone. Four weeks after the second operation, rabbits were sacrificed for further analysis. A significant increase in ejection fraction (p<0.0001) was detected in the treatment group, along with a significant increase in vascular density (p<0.001) and a significant decrease in infarct size (p<0.05) compared to the control group. Epicardial adipose tissue is a rich source of mesenchymal stem cells, which can differentiate into cardiomyocytes, as well as having neoangiogenic properties. Due to its potential to ameliorate chronic ischemic changes in the heart, it may be preferable in cardiac regenerative cell therapies.

Structural and Perfusional Study of Successfully Repaired Diabetic Tractional Retinal Detachment Involving the Macula

Purpose: Severe visual sequelae can be frequently observed in patients with diabetic tractional retinal detachment (TRD) involving the macula. We analyzed the postoperative structural spectral-domain (SD)-OCT and optical coherent tomography angiography (OCT angiography) findings in four selected eyes that were enrolled after successful anatomical diabetic macular detachment repair. These diabetic macular tractional detachments were evolved within 16 weeks. We also correlated the postoperative mean best-corrected visual acuity with the postoperative SD-OCT and OCT angiographic findings. <i>Methods</i>: This case series included 4 selected eyes of 4 patients with a mean diabetes evolution period of 18.4±4.8 years SD. Vitrectomy techniques were successfully used for TRD involving the macula. Postoperative SD-OCT, and final postoperative functional evaluations, including BCVA and OCT angiographic evaluation were performed at the final postoperative follow-up visit. <i>Results</i>: The mean difference between the preoperative (0.87±0.15) and final postoperative (0.35±0.21) BCVA was significant (p<0.05). The mean duration of vision loss before surgery was 11.6±2.3 weeks. The mean time for postoperative macular detachment resolution was 3.6±1.7 weeks SD. A mean follow-up evaluation of 13.1±2.7 SD months. Final postoperative imaging tests demonstrated multiple abnormalities of the foveal avascular zone and different areas of non-perfused macula on OCT angiography. The presence of disorganization of the retinal inner layers (DRIL) and chronic ischemic macular edema changes in 3 eyes (75.0%) as well as the OCT angiography abnormal findings in 3 (75.0%) were analized and correlated. <i>Conclusion</i>: In successfully reattached macula, postoperative microcirculatory abnormalities consistent with superficial and deep plexuses deficiencies and vessel densities alterations were detected as well as persistent DRIL, EZ and ELM line abnormalities.

Hirayama Disease can be Caused by Loss of Attachment of the Cervical Posterior Dura to the Pedicle due to Immunological Abnormalities of the Dura and Posterior Ligaments: A New Hypothesis

Hirayama disease is a rare and self-limiting cervical myelopathy related to neck flexion in adolescents, predominantly male, originally described by Hirayama in 1959 and until today reported worldwide. It characteristically presents with asymmetric distal upper extremity muscular weakness and atrophy without objective sensory disturbance or lower extremity involvement, which spontaneously halts within 3-5 years after onset with some sequelae. Neuroradiological and autopsy/neuropathological studies have shown that the condition is caused by chronic ischemic changes to the anterior horn cells of the lower cervical cord due to abnormal forward-shifting of the lower cervical posterior dura on neck flexion. Although the true pathomechanism of the dural changes is still unknown, various hypothetical theories have been formulated: "tight dural canal in flexion", "flexion myelopathy", "engorgement of the posterior venous plexus", and "disproportional growth between the vertebral column and the contents of the spinal canal during puberty" (Hirayama). However, rarity of the condition and asymmetric cord involvement cannot be explained with these theories alone. Combination of a "posterior epidural ligament [sparse at C6-C7] factor" proposed by Shinomiya , "loss of dorsal dural attachment from the pedicle even in neutral position in the MRI" pointed out by some neuroradilogists, and "pathological abnormalities of elastic and collagen fibers in the operatively-resected dura" reported by several spinal surgeons suggest that abnormal changes of the dura and posterior ligaments, as well as the existence of "myodural bridge" at C1-C2 described by some neuroanatomists, can cause the dural forward-shifting of the lower cervical cord. In addition to these mechanical factors, immunological abnormalities such as hyperIgEemia and upreguration of some cytokines and chemokines in serum/CSF of patients with Hirayama disease have been reported. Furthermore, immune cells comprise -17% of all cells in the dura in rats. In conclusion, although further immunological studies of the patient's cervical dura and surrounding structures are required, I propose, in addition to Hirayama's theory, a new hypothesis about the pathomechanism of Hirayama disease, "loss of dorsal dural attachment from the pedicle due to immunological abnormalities of the dura and posterior ligaments", which can resolve the remaining problems of the condition. (Received May 25, 2020; Accepted July 2, 2020; Published December 1, 2020).

In Reply: Early Diffusion Magnetic Resonance Imaging Changes in Normal-Appearing Brain in Pediatric Moyamoya Disease.

BACKGROUND Moyamoya disease often leads to ischemic strokes visible on diffusion-weighted imaging (DWI) and T2-weighted magnetic resonance imaging (MRI) with subsequent cognitive impairment. In adults with moyamoya, apparent diffusion coefficient (ADC) is correlated with regions of steal phenomenon and executive dysfunction prior to white matter changes. OBJECTIVE To investigate quantitative global diffusion changes in pediatric moyamoya patients prior to explicit structural ischemic damage. METHODS We retrospectively reviewed children (<20 yr old) with moyamoya disease and syndrome who underwent bypass surgery at our institution. We identified 29 children with normal structural preoperative MRI and without findings of cortical infarction or chronic white matter ischemic changes. DWI datasets were used to calculate ADC maps for each subject as well as for 60 age-matched healthy controls. Using an atlas-based approach, the cerebral white matter, cerebral cortex, thalamus, caudate, putamen, pallidum, hippocampus, amygdala, nucleus accumbens, and brainstem were segmented in each DWI dataset and used to calculate regional volumes and ADC values. RESULTS Multivariate analysis of covariance using the regional ADC and volume values as dependent variables and age and gender as covariates revealed a significant difference between the groups (P < .001). Post hoc analysis demonstrated significantly elevated ADC values for children with moyamoya in the cerebral cortex, white matter, caudate, putamen, and nucleus accumbens. No significant volume differences were found. CONCLUSION Prior to having bypass surgery, and in the absence of imaging evidence of ischemic stroke, children with moyamoya exhibit cerebral diffusion changes. These findings could reflect microstructural changes stemming from exhaustion of cerebrovascular reserve.

Cerebrovascular pathology and misdiagnosis of multiple system atrophy: An autopsy study

BackgroundMultiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by a combination of dysautonomia, parkinsonism, and cerebellar ataxia. Other disorders can mimic MSA, but it is unknown whether cerebrovascular pathology, so-called “vascular parkinsonism,” can mimic MSA. This study aimed to determine the clinicopathological features and red flags for vascular parkinsonism masquerading as MSA. MethodsUsing a brain bank database, we screened 270 patients with an antemortem diagnosis of MSA, who did not have pathologic evidence of MSA, but rather cerebrovascular pathology, including leukoencephalopathy, lacunar infarcts, and microinfarcts. Histologic sections from the neocortex, basal ganglia, thalamus, brainstem, and cerebellum were reviewed. Medical records were reviewed to characterize the clinical features. The probability of a clinical diagnosis of MSA was assigned retrospectively, guided by current consensus criteria. ResultsFour patients had cerebrovascular pathology without neurodegenerative processes. Chronic ischemic changes in periventricular white matter, subcortical leukoencephalopathy, lacunar infarcts, or microinfarcts were detected in basal ganglia of all patients. Cerebrovascular pathology that might contribute to autonomic failure was not identified. Clinically, two patients were diagnosed with possible MSA-parkinsonism, one with probable MSA-parkinsonism, and one with possible MSA-cerebellar type; however, they also had one or more non-supporting features of MSA (e.g., onset >75-years of age, dementia), vascular risk factors, and other etiologies (e.g., autonomic neuropathy) that could cause autonomic failure. ConclusionsWhen combined with cerebrovascular risk factors and comorbidities, cerebrovascular pathology may masquerade as MSA. The important lesson from this study is that the diagnosis of MSA requires exclusion of other causes, including cerebrovascular disease.

Early Diffusion Magnetic Resonance Imaging Changes in Normal-Appearing Brain in Pediatric Moyamoya Disease.

Moyamoya disease often leads to ischemic strokes visible on diffusion-weighted imaging (DWI) and T2-weighted magnetic resonance imaging (MRI) with subsequent cognitive impairment. In adults with moyamoya, apparent diffusion coefficient (ADC) is correlated with regions of steal phenomenon and executive dysfunction prior to white matter changes. To investigate quantitative global diffusion changes in pediatric moyamoya patients prior to explicit structural ischemic damage. We retrospectively reviewed children (<20 yr old) with moyamoya disease and syndrome who underwent bypass surgery at our institution. We identified 29 children with normal structural preoperative MRI and without findings of cortical infarction or chronic white matter ischemic changes. DWI datasets were used to calculate ADC maps for each subject as well as for 60 age-matched healthy controls. Using an atlas-based approach, the cerebral white matter, cerebral cortex, thalamus, caudate, putamen, pallidum, hippocampus, amygdala, nucleus accumbens, and brainstem were segmented in each DWI dataset and used to calculate regional volumes and ADC values. Multivariate analysis of covariance using the regional ADC and volume values as dependent variables and age and gender as covariates revealed a significant difference between the groups (P<.001). Post hoc analysis demonstrated significantly elevated ADC values for children with moyamoya in the cerebral cortex, white matter, caudate, putamen, and nucleus accumbens. No significant volume differences were found. Prior to having bypass surgery, and in the absence of imaging evidence of ischemic stroke, children with moyamoya exhibit cerebral diffusion changes. These findings could reflect microstructural changes stemming from exhaustion of cerebrovascular reserve.