Persistence of primitive reflexes associated with asymmetries in fixation and ocular motility values.

This cross-sectional study examined eye movement performance in patients aged 4 to 16 years. Measurements of eye movements were obtained before and after performing therapy for inhibition of four primitive reflexes, asymmetric tonic neck reflex, symmetric tonic neck reflex, labyrinthine tonic reflex and Moro reflex. Subsequently the scores of the four primitive reflexes were compared with the results of five variables: fixation maintenance, % mean saccade size, motility excursions, fixations during excursions and mean duration of fixations. The comparisons showed a significant reduction in evidence of fixation maintenance as well as mean saccade size due to the inhibition of the four primitive reflexes. There was also a significant increase in ocular motility while fixations per saccade and average duration of fixations also decreased significantly. Visual balance between values of both eyes improved in all tests. A device called VisagraphTM III, which measures eye movements, was used for data collection. These results suggest that the oculomotor improvements reflect the involvement of other maturational processes such as the emergence and inhibition of primitive reflexes, the whole reorganization being key to future reading and attentional processes.

Iron: Life's primeval transition metal.

Modern life requires many different metal ions, which enable diverse biochemical functions. It is commonly assumed that metal ions' environmental availabilities controlled the evolution of early life. We argue that evolution can only explore the chemistry that life encounters, and fortuitous chemical interactions between metal ions and biological compounds can only be selected for if they first occur sufficiently frequently. We calculated maximal transition metal ion concentrations in the ancient ocean, determining that the amounts of biologically important transition metal ions were orders of magnitude lower than ferrous iron. Under such conditions, primitive bioligands would predominantly interact with Fe(II). While interactions with other metals in certain environments may have provided evolutionary opportunities, the biochemical capacities of Fe(II), Fe-S clusters, or the plentiful magnesium and calcium could have satisfied all functions needed by early life. Primitive organisms could have used Fe(II) exclusively for their transition metal ion requirements.

A rare cause of hemoptysis: Primitive pulmonary synovial sarcoma.

Synovialosarcoma is a mesenchymal tumor with soft tissue predilection, metastasizing to various organs, including the lung. Primary pulmonary involvement is rare and requires histological examination for confirmation. In early stages, the treatment is surgical followed by radiotherapy to avoid recurrence, while there's no consensus in chemotherapy. We present a case of a 54-year-old patient with primary pulmonary synovialosarcoma. The patient underwent successful treatment, achieving tumor control and complete excision. This case discusses diagnostic approaches, prognosis, treatment modalities for primary pulmonary synovialosarcoma, emphasizing the significance of early intervention for favourable outcomes.

Molecular insights into hybrid tumors: Exploring the heterogeneity in plexiform ameloblastoma.

For many years, odontogenic tumors have been known to present both clinical and histopathological challenges due to their origins in the epithelial, ectomesenchymal, and/or mesenchymal components of tooth-forming tissues. Gaining a comprehensive understanding of both common and rare odontogenic tumors is crucial for their effective study and clinical management. One particularly puzzling tumor is the "plexiform ameloblastoma," a variant of the solid multicystic ameloblastoma. This term describes a distinct pattern of epithelial proliferation within the cystic cavity. Numerous studies have emphasized the variability of the stromal component, further highlighting the enigmatic nature of ameloblastoma. The presence of unique and rare features, such as primitive, mature desmoplastic, hemangiomatous, or ghost cells within the stroma of plexiform ameloblastoma, underscores the differentiation potential of the neoplastic odontogenic epithelium and offers significant insights into the tissue reactions associated with this condition. This case review discusses four instances of plexiform ameloblastoma, illustrating various atypical stromal changes and their influence on patient prognosis. It also provides important criteria for analyzing stromal alterations related to this complex odontogenic tumor.

Primitive proatlantal intersegmental artery associated with moyamoya disease: A case report.

Primitive proatlantal intersegmental artery (PPIA) is rare and can be divided into types I and II. PPIAs can be associated with some anatomical variations and vascular diseases. However, no case of PPIA combined with moyamoya disease (MMD) has been reported. Here, we reported such a case. A 54-year-old man experienced headache for 1 month. The results of the neurological examinations were unremarkable. Magnetic resonance angiography and digital subtraction angiography revealed a right type I PPIA with MMD. The PPIA serves as an important collateral path for MMD patients. Because the patient only experienced headache, he was discharged and underwent follow-up observation. This case indicates that, rarely, PPIA can be associated with MMD and serve as a collateral vessel for MMD patients.

Primitive neuroectodermal tumor of the lumbosacral nerve plexus: A case report.

We report an uncommon case of primitive neuroectodermal tumor/ Ewing's sarcoma of the lumbar and sacral nerve plexus in a 17years old boy who presented with an intense pain in the lower back radiating to legs. Magnetic resonance imaging showed a soft tissue mass with thickening of lumbar and sacral spinal nerve roots (L5-S3 level), along with widening of the corresponding foramina. There was also posterior scalloping of L5/S1 vertebrae and invasion of the sacral bone. A Partial resection has been performed, and the ensuing histopathology confirmed the diagnosis of PNET/Ewing's sarcoma. MRI in conjunction with histopathology are the key to narrow down the differential diagnoses list. PNET of lumbosacral area remain scarce, and only few cases have been reported nowadays. Given to the aggressivity of these tumors, the prognosis is poor despite appropriate treatment.

Concurrent delayed recurrence of peripheral primitive neuroectodermal tumors in orbital and sellar/suprasellar regions in an older adult.

BACKGROUND: Peripheral primitive neuroectodermal tumors (pPNETs) are rare and aggressive small round cell tumors, tending to occur in the thoracic and paravertebral soft tissues in children and young adults. This report describes an exceptionally rare case of concurrent delayed recurrence of pPNET in the orbital and sellar/suprasellar regions in an older adult, with a discussion supported by a literature review. CASE PRESENTATION: We report an 82-year-old woman with a history of orbital pPNETs resection at age 62, followed by gamma knife radiosurgery for local recurrence at age 66. She presented left eye pain, left eye protrusion, decreased vision in the right eye, and right homonymous hemianopia. MRI revealed extensive lesions in the left orbital cavity and sellar/suprasellar region, contiguous through the optic canal. The recurrent tumor was treated through a two-stage resection via transcranial and transsphenoidal approaches, which resulted in symptom improvement and a pathologic diagnosis of pPNETs. CONCLUSION: This case highlights a highly rare instance of late-onset orbital pPNETs recurrence in an elderly patient, with evidence suggesting tumor progression into the sellar/suprasellar regions through the optic canal.

Primary Ewing's sarcoma of the intestine: case report and literature review.

Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumor is a highly aggressive malignant tumor that typically presents in bone and soft tissue. Primary ES of the intestine is relatively rare, which poses a challenge in distinguishing it from other primary tumors of the small intestine through imaging. This article details a case study of ES originating in the intestine. Computed tomography (CT) imaging suggested a small intestinal stromal tumor, and so the patient underwent resection of the small bowel and omental tumor. Pathology results confirmed the diagnosis of ES of the small intestine. Following surgery, the patient underwent six cycles of chemotherapy, and a follow-up positron emission tomography-CT revealed widespread dissemination of the disease with intraperitoneal metastasis, ultimately resulting in the death of the patient.

Ewing sarcoma presenting in the lung: a case report.

BACKGROUND: Ewing sarcoma is a malignant round-cell tumor that primarily affects bones in children. It can also arise in extraosseous tissues, such as the lung, kidneys, and liver. The presentation symptoms of Ewing sarcoma may include cough, dyspnea, and chest pain. CASE PRESENTATION: This report details the history of a 15-year-old Syrian boy with a previous diagnosis of Hodgkin lymphoma who presented with chronic shoulder pain. Imaging studies revealed an 80 mm mass in the apex of the left lung, which was confirmed through histopathological examination to be Ewing sarcoma following a computed-tomography-guided biopsy. The patient received multiple cycles of chemotherapy and subsequently underwent surgical resection of the remaining mass. CONCLUSIONS: This case highlights the rare occurrence of Ewing sarcoma in the lung and the unusual clinical presentation of shoulder pain without other accompanying symptoms.

Cerebral palsy in children: A clinical practice review.

Cerebral palsy is a disorder characterized by abnormal tone, posture, and movement. In clinical practice, it is often useful to approach cerebral palsy based on the predominant motor system findings - spastic hemiplegia, spastic diplegia, spastic quadriplegia, extrapyramidal or dyskinetic, and ataxic. The prevalence of cerebral palsy is between 1.5 and 3 per 1,000 live births with higher percentage of cases in low to middle income countries and geographic regions. Pre-term birth and low birthweight are recognized as the most frequent risk factors for cerebral palsy; other risk factors include hypoxic-ischemic encephalopathy, maternal infections, and multiple gestation. In most cases of cerebral palsy, the initial injury to the brain occurs during early fetal brain development. Intracerebral hemorrhage and periventricular leukomalacia are the main pathologic findings found in preterm infants who develop spastic cerebral palsy. The diagnosis of cerebral palsy is primarily based on clinical findings. Early recognition of infants at risk for cerebral palsy as well as those with cerebral palsy is possible based on a combination of clinical history, use of standardized neuromotor assessment and findings on magnetic resonance imaging; however, in clinical practice, cerebral palsy is more reliably diagnosed by 2 years of age. Magnetic resonance imaging scan is indicated to delineate the extent of brain lesions and to identify congenital brain malformations. Genetic testing and tests for inborn errors of metabolism are indicated to identify specific disorders, especially treatable disorders. Because cerebral palsy is associated with multiple associated and secondary medical conditions, its management requires a sustained and consistent collaboration among multiple disciplines and specialties. With appropriate support, most children with cerebral palsy grow up to be adults with good functional abilities.

Hypoxia-sonic hedgehog axis as a driver of primitive hematopoiesis development and evolution in cavefish.

The teleost Astyanax mexicanus consists of surface dwelling (surface fish) and cave dwelling (cavefish) forms. Cavefish have evolved in subterranean habitats characterized by reduced oxygen levels (hypoxia) and exhibit a subset of phenotypic traits controlled by increased Sonic hedgehog (Shh) signaling along the embryonic midline. The enhancement of primitive hematopoietic domains, which are formed bilaterally in the anterior and posterior lateral plate mesoderm, are responsible for the development of more larval erythrocytes in cavefish relative to surface fish. In this study, we determine the role of hypoxia and Shh signaling in the development and evolution of primitive hematopoiesis in cavefish. We show that hypoxia treatment during embryogenesis increases primitive hematopoiesis and erythrocyte development in surface fish. We also demonstrate that upregulation of Shh midline signaling by the Smoothened agonist SAG increases primitive hematopoiesis and erythrocyte development in surface fish, whereas Shh downregulation via treatment with the Smoothened inhibitor cyclopamine decreases these traits in cavefish. Together these results suggest that hematopoietic enhancement is regulated by hypoxia and Shh signaling. Lastly, we demonstrate that hypoxia enhances expression of Shh signaling along the midline of surface fish embryos. We conclude that hypoxia-mediated Shh plasticity may be a driving force for the adaptive evolution of primitive hematopoiesis and erythrocyte development in cavefish.

Persistence and activity levels of primitive reflexes in young high-level football players.

Introduction: Primitive reflexes (PR) induce involuntary automatic movements in response to specific stimuli. This study aimed to determine the prevalence of active PR in young high-level football players. Methodology: Sixty-nine national-level football players from a French academy were tested (17.0 ± 1.4 years; 69.6 ± 8.0 kg; 178.9 ± 6.9 cm) to evaluate the persistence of PR, following the methodology of the Institute for Neuro-Physiological Psychology (INPP) and the classification by a global score (GS). Based on the sum of seven tests, each was rated between 0 = null and 4 = max. The GS is classified into five categories from no activity to maximal (0-1 = no activity, 2-7 = low, 8-13 = medium, 14-21 = high, and 22-28 = maximal). Result: Around two-thirds (68.1%) of players presented active PR at different activity levels. Among them, a small proportion (7.2%) had medium GS, while 60.9% had a low GS. The GS was not dependent on field position or the age of the players (p > 0.05). However, playing football in an age category higher than their own was associated with significantly more active primitive reflexes (PR) compared to being in their age category (p < 0.01). The results showed that 72.7% of "upgraded" football players had low GS and 18.2% had medium GS, compared to 55.3% and 2.1% in the non-upgraded group. Discussion: The findings of the current study demonstrate that PR could still be active in a healthy population of high-level football players. Practicing a single sport for years and upgrading players could create a negative environment that can ultimately lead to the activation of otherwise integrated PR.

Ewing sarcoma among children 5 years of age or younger: Is it a different disease?

INTRODUCTION: Children ≤5 years of age with Ewing's sarcoma (ES) possibly have a distinct disease biology, data on which are scarce. We evaluated clinical features, outcomes, and prognostic factors of ES among children with age ≤5 years. METHODS: Children with ES registered between 2003 and 2019 were included. Baseline clinical and treatment details were retrieved from medical records. Prognostic factors were identified using multivariable Cox regression. Clinical features and outcomes of children ≤5 years were compared with those greater than 5 years by chi-square and log-rank tests. Propensity score-matched (PSM) analysis was done to evaluate the impact of age on survival in the metastatic and localized subgroups. RESULTS: Out of the 859 patients, 86 (10%) were ≤5 years of age (median age 4 years, 60 males [69.8%]). The most common location was the extremities (37.2%), followed by thorax (27.9%) and head and neck (H&N) (22.1%); baseline metastases were seen in 25 patients (29.8%). The median event-free-survival (EFS) and overall survival (OS) were 25.6 and 68.7 months, respectively. Metastatic disease predicted inferior OS (hazard ratio [HR] = 2.54, p = .018) and EFS (HR = 2.47, p = .007], symptom duration ≤3 months predicted an inferior OS (HR = 2.17, p = .048). Compared to age greater than 5 years, younger children had more H&N and less pelvic primaries (p < .001) and lesser baseline metastases (p = .037). PSM analysis did not reveal any significant impact of age on OS in the metastatic (HR = 1.59, p = .29) or localized cohort (HR = 1.77, p = .09). CONCLUSIONS: Children with ES ≤5 years of age have a distinct favorable clinical presentation. However, age is not an independent prognostic factor for survival outcomes when adjusted for confounders.

Unraveling the Neural Landscape of Mental Disorders using Double Functional Independent Primitives (dFIPs).

Mental illnesses extract a high personal and societal cost, and thus explorations of the links between mental illness and functional connectivity in the brain are critical. Investigating major mental illnesses, believed to arise from disruptions in sophisticated neural connections, allows us to comprehend how these neural network disruptions may be linked to altered cognition, emotional regulation, and social interactions. Although neuroimaging has opened new avenues to explore neural alterations linked to mental illnesses, the field still requires precise and sensitive methodologies to inspect these neural substrates of various psychological disorders. In this study, we employ a hierarchical methodology to derive double functionally independent primitives (dFIPs) from resting state functional magnetic resonance neuroimaging data (rs-fMRI). These dFIPs encapsulate canonical overlapping patterns of functional network connectivity (FNC) within the brain. Our investigation focuses on the examination of how combinations of these dFIPs relate to different mental disorder diagnoses. The central aim is to unravel the complex patterns of FNC that correspond to the diverse manifestations of mental illnesses. To achieve this objective, we used a large brain imaging dataset from multiple sites, comprising 5805 total individuals diagnosed with schizophrenia (SCZ), autism spectrum disorder (ASD), bipolar disorder (BPD), major depressive disorder (MDD), and controls. The key revelations of our study unveil distinct patterns associated with each mental disorder through the combination of dFIPs. Notably, certain individual dFIPs exhibit disorder-specific characteristics, while others demonstrate commonalities across disorders. This approach offers a novel, data-driven synthesis of intricate neuroimaging data, thereby illuminating the functional changes intertwined with various mental illnesses. Our results show distinct signatures associated with psychiatric disorders, revealing unique connectivity patterns such as heightened cerebellar connectivity in SCZ and sensory domain hyperconnectivity in ASD, both contrasted with reduced cerebellar-subcortical connectivity. Utilizing the dFIP concept, we pinpoint specific functional connections that differentiate healthy controls from individuals with mental illness, underscoring its utility in identifying neurobiological markers. In summary, our findings delineate how dFIPs serve as unique fingerprints for different mental disorders.

[Fatal outcome of complete resection of undifferentiated pleomorphic cardiac sarcoma : About a case]. / Évolution fatale d'une résection complète d'un sarcome cardiaque pléomorphe indifférencié : à propos d'un cas.

INTRODUCTION: Undifferentiated pleomorphic sarcoma (UPS) is a very rare malignant primary cardiac tumor with a poor prognosis. OBSERVATION: A 34-year-old woman complained of dyspnea evolving in a febrile context with altered general condition: Transthoracic echocardiography (TTE) revealed a left intra-atrial mass measuring 51/26 mm in diameter. A thoracic computed tomography (CT) revealed a mass occupying the left atrium measuring 42/28/34 mm, associated with two pulmonary metastases. The cardiac mass was resected and the postoperative suites were fatal. Histological diagnosis was UPS. CONCLUSION: We present an atypical case of invasive UPS, which was surgically resected and scheduled for radio-chemotherapy with a fatal outcome.

Joint synergy and muscle activity in the motion of the ankle-foot complex.

The movement of the ankle-foot complex joints is coupled as a result of various physiological and physical constraints. This study introduces a novel approach to the analysis of joint synergies and their physiological basis by focusing on joint rotational directions and the types of muscle contractions. We developed a biomimetic model of the ankle-foot complex with seven degrees of freedom, considering the skeletal configuration and physiological axis directions. Motion capture experiments were conducted with eight participants performing dorsiflexion and plantarflexion in open-chain states, as well as various walking tasks in closed-chain states, across different ground inclinations (±10, ±5, 0 deg) and walking speeds (3 and 4 km h-1). Hierarchical cluster analysis identified joint synergy clusters and motion primitives, revealing that in open-chain movements, plantarflexion of the ankle, tarsometatarsal and metatarsophalangeal joints exhibited synergy with the inversion of the remaining joints in the complex; meanwhile, dorsiflexion was aligned with eversion. During closed-chain movements, the synergies grouping was exchanged in the subtalar, talonavicular and metatarsophalangeal joints. Further analysis showed that in open-chain movements, synergy patterns influenced by multi-joint muscles crossing oblique joint axes contribute to foot motion. In closed-chain movements, these changes in synergistic patterns enhance the propulsion of the center of mass towards the contralateral leg and improve foot arch compliance, facilitating human motion. Our work enhances the understanding of the physiological mechanisms underlying synergistic motion within the ankle-foot complex.

Urinary cytologic features of primitive neuroectodermal tumor primarily involving the kidney: A case report.

Renal primitive neuroectodermal tumor(rPNET) is an extremely rare and distinct entity that can be mistaken for a variety of other round cell tumors. Findings from fine needle aspiration cytology have been reported, but there have been no reports of PNET cytomorphological features in urine cytology specimen. Here, we report a case of PNET characterized by urine cytology. A 24-year-old girl presented with hematuria for half a month. Computed tomography showed a large tumor involving the upper pole of the right kidney. Urinary cytology exhibited highly cellular clusters composed of small, round, atypical cells with little cytoplasm and a high nuclear/cytoplasmic ratio. Following a right nephrectomy, the biopsy confirmed the diagnosis of rPNET. Additionally, immunohistochemistry (IHC) staining of the decolorized liquid-based cytology slide was positive for CD99, further confirming the diagnosis through urine cytology. Our observations suggest that urinary cytology in combination with cytological morphology and immunocytochemistry may be a potentially useful method for the detection of PNET.

Selective Transarterial Embolization for a Ruptured Persistent Trigeminal Artery Variant Aneurysm.

We report a male patient with a ruptured persistent primitive trigeminal artery variant aneurysm that resulted in a fistula with the cavernous sinus. He presented with left conjunctival hyperemia and exophthalmos. Cerebral angiography revealed a left direct carotid-cavernous fistula; however, a balloon occlusion test determined that the source was actually a ruptured aneurysm located on the trunk of a persistent primitive trigeminal artery. Endovascular trapping of the persistent primitive trigeminal artery was performed, which resulted in fistula occlusion and symptom resolution.

A Case of Cerebral Infarction due to Internal Carotid Artery Occlusion Accompanied by Persistent Primitive Trigeminal Artery.

An 85-year-old woman with a history of diabetes mellitus became aware of motor weakness of the left lower extremity. Magnetic resonance imaging showed multiple small cerebral infarctions in the right corona radiata. Angiography revealed persistent primitive trigeminal artery (PPTA) originating from the right internal carotid artery (ICA) and connecting to basilar artery, and the right ICA occluded distal to PPTA. Collateral blood circulation had developed, and sufficient collateral blood flow was expected. From these findings, the right ICA was considered to show stenosis due to atherosclerotic changes before occlusion. Conservative treatment was conducted with the transoral administration of rivaroxaban. It is important to correctly diagnose the anatomy and existence of an anomalous vessel in a stroke patient, not only when endovascular treatment is planned, but also for conservative medical treatment. Rapid and accurate radiological examinations facilitate safe and effective treatment.