Corpus Callosum: Broad plate of dense myelinated fibers that reciprocally interconnect regions of the cortex in all lobes with corresponding regions of the opposite hemisphere. The corpus callosum is located deep in the longitudinal fissure.Agenesis of Corpus Callosum: Birth defect that results in a partial or complete absence of the CORPUS CALLOSUM. It may be isolated or a part of a syndrome (e.g., AICARDI'S SYNDROME; ACROCALLOSAL SYNDROME; ANDERMANN SYNDROME; and HOLOPROSENCEPHALY). Clinical manifestations include neuromotor skill impairment and INTELLECTUAL DISABILITY of variable severity.Acrocallosal Syndrome: Autosomal recessive syndrome characterized by hypogenesis or agenesis of CORPUS CALLOSUM. Clinical features include MENTAL RETARDATION; CRANIOFACIAL ABNORMALITIES; digital malformations, and growth retardation.Corpus Luteum: The yellow body derived from the ruptured OVARIAN FOLLICLE after OVULATION. The process of corpus luteum formation, LUTEINIZATION, is regulated by LUTEINIZING HORMONE.Anisotropy: A physical property showing different values in relation to the direction in or along which the measurement is made. The physical property may be with regard to thermal or electric conductivity or light refraction. In crystallography, it describes crystals whose index of refraction varies with the direction of the incident light. It is also called acolotropy and colotropy. The opposite of anisotropy is isotropy wherein the same values characterize the object when measured along axes in all directions.Nerve Fibers, Myelinated: A class of nerve fibers as defined by their structure, specifically the nerve sheath arrangement. The AXONS of the myelinated nerve fibers are completely encased in a MYELIN SHEATH. They are fibers of relatively large and varied diameters. Their NEURAL CONDUCTION rates are faster than those of the unmyelinated nerve fibers (NERVE FIBERS, UNMYELINATED). Myelinated nerve fibers are present in somatic and autonomic nerves.Diffusion Tensor Imaging: The use of diffusion ANISOTROPY data from diffusion magnetic resonance imaging results to construct images based on the direction of the faster diffusing molecules.Magnetic Resonance Imaging: Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques.Cuprizone: Copper chelator that inhibits monoamine oxidase and causes liver and brain damage.Diffusion Magnetic Resonance Imaging: A diagnostic technique that incorporates the measurement of molecular diffusion (such as water or metabolites) for tissue assessment by MRI. The degree of molecular movement can be measured by changes of apparent diffusion coefficient (ADC) with time, as reflected by tissue microstructure. Diffusion MRI has been used to study BRAIN ISCHEMIA and tumor response to treatment.Anodontia: Congenital absence of the teeth; it may involve all (total anodontia) or only some of the teeth (partial anodontia, hypodontia), and both the deciduous and the permanent dentition, or only teeth of the permanent dentition. (Dorland, 27th ed)Abnormalities, MultipleNervous System Malformations: Structural abnormalities of the central or peripheral nervous system resulting primarily from defects of embryogenesis.Brain: The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.Internal Capsule: WHITE MATTER pathway, flanked by nuclear masses, consisting of both afferent and efferent fibers projecting between the WHITE MATTER and the BRAINSTEM. It consists of three distinct parts: an anterior limb, posterior limb, and genu.Diffuse Axonal Injury: A relatively common sequela of blunt head injury, characterized by a global disruption of axons throughout the brain. Associated clinical features may include NEUROBEHAVIORAL MANIFESTATIONS; PERSISTENT VEGETATIVE STATE; DEMENTIA; and other disorders.Image Processing, Computer-Assisted: A technique of inputting two-dimensional images into a computer and then enhancing or analyzing the imagery into a form that is more useful to the human observer.Functional Laterality: Behavioral manifestations of cerebral dominance in which there is preferential use and superior functioning of either the left or the right side, as in the preferred use of the right hand or right foot.Septum Pellucidum: A triangular double membrane separating the anterior horns of the LATERAL VENTRICLES of the brain. It is situated in the median plane and bounded by the CORPUS CALLOSUM and the body and columns of the FORNIX (BRAIN).Fornix, Brain: Heavily myelinated fiber bundle of the TELENCEPHALON projecting from the hippocampal formation to the HYPOTHALAMUS. Some authorities consider the fornix part of the LIMBIC SYSTEM. The fimbria starts as a flattened band of axons arising from the subiculum and HIPPOCAMPUS, which then thickens to form the fornix.PAX9 Transcription Factor: A paired box transcription factor that is involved in ODONTOGENESIS.Demyelinating Diseases: Diseases characterized by loss or dysfunction of myelin in the central or peripheral nervous system.Brain Diseases: Pathologic conditions affecting the BRAIN, which is composed of the intracranial components of the CENTRAL NERVOUS SYSTEM. This includes (but is not limited to) the CEREBRAL CORTEX; intracranial white matter; BASAL GANGLIA; THALAMUS; HYPOTHALAMUS; BRAIN STEM; and CEREBELLUM.Neural Pathways: Neural tracts connecting one part of the nervous system with another.Myelin Sheath: The lipid-rich sheath surrounding AXONS in both the CENTRAL NERVOUS SYSTEMS and PERIPHERAL NERVOUS SYSTEM. The myelin sheath is an electrical insulator and allows faster and more energetically efficient conduction of impulses. The sheath is formed by the cell membranes of glial cells (SCHWANN CELLS in the peripheral and OLIGODENDROGLIA in the central nervous system). Deterioration of the sheath in DEMYELINATING DISEASES is a serious clinical problem.Aicardi Syndrome: A rare genetic disorder characterized by partial or complete absence of the CORPUS CALLOSUM, resulting in infantile spasms, MENTAL RETARDATION, and lesions of the RETINA or OPTIC NERVE.Tooth Abnormalities: Congenital absence of or defects in structures of the teeth.Hydrocephalus: Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, INTRACRANIAL HYPERTENSION; HEADACHE; lethargy; URINARY INCONTINENCE; and ATAXIA.Cerebral Cortex: The thin layer of GRAY MATTER on the surface of the CEREBRAL HEMISPHERES that develops from the TELENCEPHALON and folds into gyri and sulchi. It reaches its highest development in humans and is responsible for intellectual faculties and higher mental functions.Oligodendroglia: A class of large neuroglial (macroglial) cells in the central nervous system. Oligodendroglia may be called interfascicular, perivascular, or perineuronal (not the same as SATELLITE CELLS, PERINEURONAL of GANGLIA) according to their location. They form the insulating MYELIN SHEATH of axons in the central nervous system.Malformations of Cortical Development: Abnormalities in the development of the CEREBRAL CORTEX. These include malformations arising from abnormal neuronal and glial CELL PROLIFERATION or APOPTOSIS (Group I); abnormal neuronal migration (Group II); and abnormal establishment of cortical organization (Group III). Many INBORN METABOLIC BRAIN DISORDERS affecting CNS formation are often associated with cortical malformations. They are common causes of EPILEPSY and developmental delay.Brain Mapping: Imaging techniques used to colocalize sites of brain functions or physiological activity with brain structures.Syndrome: A characteristic symptom complex.Atrophy: Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes.Congenital Abnormalities: Malformations of organs or body parts during development in utero.Intellectual Disability: Subnormal intellectual functioning which originates during the developmental period. This has multiple potential etiologies, including genetic defects and perinatal insults. Intelligence quotient (IQ) scores are commonly used to determine whether an individual has an intellectual disability. IQ scores between 70 and 79 are in the borderline range. Scores below 67 are in the disabled range. (from Joynt, Clinical Neurology, 1992, Ch55, p28)Leukoencephalopathies: Any of various diseases affecting the white matter of the central nervous system.Axons: Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body.Cerebral Ventricles: Four CSF-filled (see CEREBROSPINAL FLUID) cavities within the cerebral hemispheres (LATERAL VENTRICLES), in the midline (THIRD VENTRICLE) and within the PONS and MEDULLA OBLONGATA (FOURTH VENTRICLE).Hypertelorism: Abnormal increase in the interorbital distance due to overdevelopment of the lesser wings of the sphenoid.Pregnancy: The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.Urogenital Abnormalities: Congenital structural abnormalities of the UROGENITAL SYSTEM in either the male or the female.Microcephaly: A congenital abnormality in which the CEREBRUM is underdeveloped, the fontanels close prematurely, and, as a result, the head is small. (Desk Reference for Neuroscience, 2nd ed.)Brain Injuries: Acute and chronic (see also BRAIN INJURIES, CHRONIC) injuries to the brain, including the cerebral hemispheres, CEREBELLUM, and BRAIN STEM. Clinical manifestations depend on the nature of injury. Diffuse trauma to the brain is frequently associated with DIFFUSE AXONAL INJURY or COMA, POST-TRAUMATIC. Localized injuries may be associated with NEUROBEHAVIORAL MANIFESTATIONS; HEMIPARESIS, or other focal neurologic deficits.Dominance, Cerebral: Dominance of one cerebral hemisphere over the other in cerebral functions.Corpora Allata: Paired or fused ganglion-like bodies in the head of insects. The bodies secrete hormones important in the regulation of metamorphosis and the development of some adult tissues.Dandy-Walker Syndrome: A congenital abnormality of the central nervous system marked by failure of the midline structures of the cerebellum to develop, dilation of the fourth ventricle, and upward displacement of the transverse sinuses, tentorium, and torcula. Clinical features include occipital bossing, progressive head enlargement, bulging of anterior fontanelle, papilledema, ataxia, gait disturbances, nystagmus, and intellectual compromise. (From Menkes, Textbook of Child Neurology, 5th ed, pp294-5)Marchiafava-Bignami Disease: A neurodegenerative condition that is characterized by demyelination or necrosis of the CORPUS CALLOSUM. Symptoms include DEPRESSION; PARANOIA; DEMENTIA; SEIZURES; and ATAXIA which can progress to COMA and death in a few months. Marchiafava-Bignami syndrome is seen often in alcoholics but has been found in non-alcoholics as well.Ultrasonography, Prenatal: The visualization of tissues during pregnancy through recording of the echoes of ultrasonic waves directed into the body. The procedure may be applied with reference to the mother or the fetus and with reference to organs or the detection of maternal or fetal disease.Prenatal Diagnosis: Determination of the nature of a pathological condition or disease in the postimplantation EMBRYO; FETUS; or pregnant female before birth.Meningomyelocele: Congenital, or rarely acquired, herniation of meningeal and spinal cord tissue through a bony defect in the vertebral column. The majority of these defects occur in the lumbosacral region. Clinical features include PARAPLEGIA, loss of sensation in the lower body, and incontinence. This condition may be associated with the ARNOLD-CHIARI MALFORMATION and HYDROCEPHALUS. (From Joynt, Clinical Neurology, 1992, Ch55, pp35-6)Lissencephaly: A "smooth brain" malformation of the CEREBRAL CORTEX resulting from abnormal location of developing neurons during corticogenesis. It is characterized by an absence of normal convoluted indentations on the surface of the brain (agyria), or fewer and shallower indentations (pachygryia). There is a reduced number of cortical layers, typically 4 instead of 6, resulting in a thickened cortex, and reduced cerebral white matter that is a reversal of the normal ratio of cerebral white matter to cortex.Organ Size: The measurement of an organ in volume, mass, or heaviness.Neuroimaging: Non-invasive methods of visualizing the CENTRAL NERVOUS SYSTEM, especially the brain, by various imaging modalities.Infant, Newborn: An infant during the first month after birth.Fetal Diseases: Pathophysiological conditions of the FETUS in the UTERUS. Some fetal diseases may be treated with FETAL THERAPIES.Cerebrum: Derived from TELENCEPHALON, cerebrum is composed of a right and a left hemisphere. Each contains an outer cerebral cortex and a subcortical basal ganglia. The cerebrum includes all parts within the skull except the MEDULLA OBLONGATA, the PONS, and the CEREBELLUM. Cerebral functions include sensorimotor, emotional, and intellectual activities.Spastic Paraplegia, Hereditary: A group of inherited diseases that share similar phenotypes but are genetically diverse. Different genetic loci for autosomal recessive, autosomal dominant, and x-linked forms of hereditary spastic paraplegia have been identified. Clinically, patients present with slowly progressive distal limb weakness and lower extremity spasticity. Peripheral sensory neurons may be affected in the later stages of the disease. (J Neurol Neurosurg Psychiatry 1998 Jan;64(1):61-6; Curr Opin Neurol 1997 Aug;10(4):313-8)Craniofacial Abnormalities: Congenital structural deformities, malformations, or other abnormalities of the cranium and facial bones.Tooth Exfoliation: Physiologic loss of the primary dentition. (Zwemer, Boucher's Clinical Dental Terminology, 4th ed)MSX1 Transcription Factor: A homeodomain protein that interacts with TATA-BOX BINDING PROTEIN. It represses GENETIC TRANSCRIPTION of target GENES and plays a critical role in ODONTOGENESIS.Cebus: A genus of the family CEBIDAE, subfamily CEBINAE, consisting of four species which are divided into two groups, the tufted and untufted. C. apella has tufts of hair over the eyes and sides of the head. The remaining species are without tufts - C. capucinus, C. nigrivultatus, and C. albifrons. Cebus inhabits the forests of Central and South America.Lipoma: A benign tumor composed of fat cells (ADIPOCYTES). It can be surrounded by a thin layer of connective tissue (encapsulated), or diffuse without the capsule.Image Interpretation, Computer-Assisted: Methods developed to aid in the interpretation of ultrasound, radiographic images, etc., for diagnosis of disease.Gestational Age: The age of the conceptus, beginning from the time of FERTILIZATION. In clinical obstetrics, the gestational age is often estimated as the time from the last day of the last MENSTRUATION which is about 2 weeks before OVULATION and fertilization.Neuronal Migration Disorders: Disorders resulting from defects in migration of neuronal cells during neurogenesis. Developing nerve cells either fail to migrate or they migrate to incorrect positions resulting in formation of heterotopias, lissencephaly, or other malformations and dysfunctions of the nervous system.Hyperglycinemia, Nonketotic: An autosomal recessive metabolic disorder caused by deficiencies in the mitochondrial GLYCINE cleavage system.Neuroglia: The non-neuronal cells of the nervous system. They not only provide physical support, but also respond to injury, regulate the ionic and chemical composition of the extracellular milieu, participate in the BLOOD-BRAIN BARRIER and BLOOD-RETINAL BARRIER, form the myelin insulation of nervous pathways, guide neuronal migration during development, and exchange metabolites with neurons. Neuroglia have high-affinity transmitter uptake systems, voltage-dependent and transmitter-gated ion channels, and can release transmitters, but their role in signaling (as in many other functions) is unclear.Neuropsychological Tests: Tests designed to assess neurological function associated with certain behaviors. They are used in diagnosing brain dysfunction or damage and central nervous system disorders or injury.Penis: The external reproductive organ of males. It is composed of a mass of erectile tissue enclosed in three cylindrical fibrous compartments. Two of the three compartments, the corpus cavernosa, are placed side-by-side along the upper part of the organ. The third compartment below, the corpus spongiosum, houses the urethra.Whale, Killer: The species Orcinus orca, in the family Delphinidae, characterized by its black and white coloration, and huge triangular dorsal fin. It is the largest member of the DOLPHINS and derives its name from the fact that it is a fearsome predator.Imaging, Three-Dimensional: The process of generating three-dimensional images by electronic, photographic, or other methods. For example, three-dimensional images can be generated by assembling multiple tomographic images with the aid of a computer, while photographic 3-D images (HOLOGRAPHY) can be made by exposing film to the interference pattern created when two laser light sources shine on an object.Aging: The gradual irreversible changes in structure and function of an organism that occur as a result of the passage of time.Lateral Ventricles: Cavity in each of the CEREBRAL HEMISPHERES derived from the cavity of the embryonic NEURAL TUBE. They are separated from each other by the SEPTUM PELLUCIDUM, and each communicates with the THIRD VENTRICLE by the foramen of Monro, through which also the choroid plexuses (CHOROID PLEXUS) of the lateral ventricles become continuous with that of the third ventricle.Dichotic Listening Tests: Tests for central hearing disorders based on the competing message technique (binaural separation).Corpus Luteum Maintenance: Process of maintaining the functions of CORPORA LUTEA, specifically PROGESTERONE production which is regulated primarily by pituitary LUTEINIZING HORMONE in cycling females, and by PLACENTAL HORMONES in pregnant females. The ability to maintain luteal functions is important in PREGNANCY MAINTENANCE.Reference Values: The range or frequency distribution of a measurement in a population (of organisms, organs or things) that has not been selected for the presence of disease or abnormality.Analysis of Variance: A statistical technique that isolates and assesses the contributions of categorical independent variables to variation in the mean of a continuous dependent variable.Echoencephalography: Use of reflected ultrasound in the diagnosis of intracranial pathologic processes.Sex Characteristics: Those characteristics that distinguish one SEX from the other. The primary sex characteristics are the OVARIES and TESTES and their related hormones. Secondary sex characteristics are those which are masculine or feminine but not directly related to reproduction.Surgically-Created Structures: Organs or parts of organs surgically formed from nearby tissue to function as substitutes for diseased or surgically removed tissue.Apraxias: A group of cognitive disorders characterized by the inability to perform previously learned skills that cannot be attributed to deficits of motor or sensory function. The two major subtypes of this condition are ideomotor (see APRAXIA, IDEOMOTOR) and ideational apraxia, which refers to loss of the ability to mentally formulate the processes involved with performing an action. For example, dressing apraxia may result from an inability to mentally formulate the act of placing clothes on the body. Apraxias are generally associated with lesions of the dominant PARIETAL LOBE and supramarginal gyrus. (From Adams et al., Principles of Neurology, 6th ed, pp56-7)Luteolysis: Degradation of CORPUS LUTEUM. In the absence of pregnancy and diminishing trophic hormones, the corpus luteum undergoes luteolysis which is characterized by the involution and cessation of its endocrine function.
Callosal and cortical contribution to procedural learning. (1/203)
Acallosal and callosotomized subjects usually show impairments on tasks requiring bilateral interdependent motor control. However, few studies have assessed the ability of these subjects to learn a skill that requires the simultaneous contribution of each hemisphere in its acquisition. The present study examined whether acallosal and callosotomized subjects could learn a visuomotor skill that involved a motor control from either both or a single hemisphere. Eleven adult patients, six acallosal and five callosotomized, participated in this study. Seven of these patients had epileptic foci located in the frontal and/or temporal areas and one of the acallosal patients showed bilateral prefrontal atrophy following surgical removal of an orbitofrontal cyst. The performance of the experimental subjects was compared with that of 11 matched control subjects, on a modified version of a serial reaction time task developed by Nissen and Bullemer (Cogn Psychol 1987; 19: 1-32). This skill acquisition task involved bimanual or unimanual key-pressing responses to a sequence of 10 visual stimuli that was repeated 160 times. A declarative memory task was then performed to assess explicit knowledge of the sequence. None of the experimental subjects learned the task in the bimanual condition. Patients with frontal epileptic foci or orbitofrontal damage also failed to learn the task in the unimanual condition when they were using the hand contralateral to the damaged hemisphere. All other subjects, including the acallosal and callosotomized patients with temporal foci, learned the visuomotor skill as well as their controls in the unimanual condition. In spite of the absence of transfer and interhemispheric integration of procedural learning, some of the acallosal and callosotomized patients were able to learn the sequence explicitly. These findings indicate that the corpus callosum and the frontal cortical areas are important for procedural learning of a visuomotor skill. They also confirm the dissociation described by Squire (Science 1986; 232: 1612-9 and J Cogn Neurosci 1992; 4: 232-43) between the declarative and procedural memory systems and extend this dissociation to processes involving simultaneous bihemispheric co-operation. (+info)Abnormalities in neuronal process extension, hippocampal development, and the ventricular system of L1 knockout mice. (2/203)
In humans, mutations in the L1 cell adhesion molecule are associated with a neurological syndrome termed CRASH, which includes corpus callosum agenesis, mental retardation, adducted thumbs, spasticity, and hydrocephalus. A mouse model with a null mutation in the L1 gene (Cohen et al., 1997) was analyzed for brain abnormalities by Nissl and Golgi staining and immunocytochemistry. In the motor, somatosensory, and visual cortex, many pyramidal neurons in layer V exhibited undulating apical dendrites that did not reach layer I. The hippocampus of L1 mutant mice was smaller than normal, with fewer pyramidal and granule cells. The corpus callosum of L1-minus mice was reduced in size because of the failure of many callosal axons to cross the midline. Enlarged ventricles and septal abnormalities were also features of the mutant mouse brain. Immunoperoxidase staining showed that L1 was abundant in developing neurons at embryonic day 18 (E18) in wild-type cerebral cortex, hippocampus, and corpus callosum and then declined to low levels with maturation. In the E18 cortex, L1 colocalized with microtubule-associated protein 2, a marker of dendrites and somata. These new findings suggest new roles for L1 in the mechanism of cortical dendrite differentiation, as well as in guidance of callosal axons and regulation of hippocampal development. The phenotype of the L1 mutant mouse indicates that it is a potentially valuable model for the human CRASH syndrome. (+info)Reduction cranioplasty for macrocephaly. Two case reports. (3/203)
Multi-stage reduction cranioplasty was performed on two children with severe macrocephaly secondary to hydrocephalus. One patient underwent a four-stage operation, and the other underwent a two-stage operation. The postoperative course of both patients was uneventful. Reduction cranioplasty improved quality of life for both patients, and good cosmetic results were achieved. Reduction cranioplasty is effective for the treatment of macrocephaly, and multi-stage surgery can reduce the associated risks. (+info)Dissociation of the pathways mediating ipsilateral and contralateral motor-evoked potentials in human hand and arm muscles. (4/203)
1. Growing evidence points toward involvement of the human motor cortex in the control of the ipsilateral hand. We used focal transcranial magnetic stimulation (TMS) to examine the pathways of these ipsilateral motor effects. 2. Ipsilateral motor-evoked potentials (MEPs) were obtained in hand and arm muscles of all 10 healthy adult subjects tested. They occurred in the finger and wrist extensors and the biceps, but no response or inhibitory responses were observed in the opponens pollicis, finger and wrist flexors and the triceps. 3. The production of ipsilateral MEPs required contraction of the target muscle. The threshold TMS intensity for ipsilateral MEPs was on average 1.8 times higher, and the onset was 5.7 ms later (in the wrist extensor muscles) compared with size-matched contralateral MEPs. 4. The corticofugal pathways of ipsilateral and contralateral MEPs could be dissociated through differences in cortical map location and preferred stimulating current direction. 5. Both ipsi- and contralateral MEPs in the wrist extensors increased with lateral head rotation toward, and decreased with head rotation away from, the side of the TMS, suggesting a privileged input of the asymmetrical tonic neck reflex to the pathway of the ipsilateral MEP. 6. Large ipsilateral MEPs were obtained in a patient with complete agenesis of the corpus callosum. 7. The dissociation of the pathways for ipsilateral and contralateral MEPs indicates that corticofugal motor fibres other than the fast-conducting crossed corticomotoneuronal system can be activated by TMS. Our data suggest an ipsilateral oligosynaptic pathway, such as a corticoreticulospinal or a corticopropriospinal projection as the route for the ipsilateral MEP. Other pathways, such as branching of corticomotoneuronal axons, a transcallosal projection or a slow-conducting monosynaptic ipsilateral pathway are very unlikely or can be excluded. (+info)The homeodomain protein vax1 is required for axon guidance and major tract formation in the developing forebrain. (5/203)
The homeodomain protein Vax1 is expressed in a highly circumscribed set of cells at the ventral anterior midline of the embryonic CNS. These cells populate the choroid fissure of the optic disk, the body of the optic stalk and nerve, the optic chiasm and ventral diencephalon, and the anterior midline zones that abut developing commissural tracts. We have generated mutant mice that lack Vax1. In these mice (1) the optic disks fail to close, leading to coloboma and loss of the eye-nerve boundary; (2) optic nerve glia fail to associate with and appear to repulse ingrowing retinal axons, resulting in a fascicle of axons that are completely segregated from optic nerve astrocytes; (3) retinal axons fail to penetrate the brain in significant numbers and fail to form an optic chiasm; and (4) axons in multiple commissural tracts of the anterior CNS, including the corpus callosum and the hippocampal and anterior commissures, fail to cross the midline. These axon guidance defects do not result from the death of normally Vax1(+) midline cells but, instead, correlate with markedly diminished expression of attractive guidance cues in these cells. Vax1 therefore regulates the guidance properties of a set of anterior midline cells that orchestrate axon trajectories in the developing mammalian forebrain. (+info)Neuropathological abnormalities of the corpus callosum in schizophrenia: a diffusion tensor imaging study. (6/203)
OBJECTIVES: Diffusion tensor imaging (DTI), a technique capable of examining water diffusion in different tissues and the organisation of white matter tracts, was used to investigate the neuropathology of the corpus callosum in vivo in patients with schizophrenia. METHODS: Diffusion tensor imaging was performed in 20 schizophrenic patients and 25 healthy controls. Two complementary measures, mean diffusivity and fractional anisotropy, which are considered to be sensitive indices of axonal integrity, were obtained from regions of interest in the genu (anterior) and splenium (posterior) of the corpus callosum. RESULTS: Mean diffusivity was significantly increased and fractional anisotropy significantly reduced in the splenium but not the genu of the corpus callosum in the schizophrenic group compared with controls. There were no significant sex differences in the DTI measures for either the schizophrenic or control group. Clinical variables such as age, duration of illness, dose of antipsychotic medication, and schizophrenic symptoms did not predict the DTI changes in the schizophrenic patients. CONCLUSIONS: The presence of DTI changes in the splenium but not the genu of the corpus callosum suggests that there may be a focal disruption of commisural connectivity in schizophrenia. However, these findings do not exclude the possibility of abnormalities in other areas of the corpus callosum or other regions of white matter and further research using different methods of analysis may enable us to clarify this. Diffusion tensor imaging is a valuable tool in investigating the structure of white matter in schizophrenia. (+info)Parallel visuomotor processing in the split brain: cortico-subcortical interactions. (7/203)
We tested nine patients with callosal pathology in a simple reaction time task with and without redundant targets in the same or opposite visual hemifield. Four patients showed large facilitation (redundancy gain) in the presence of a redundant target, exceeding probability summation models (neural summation). Five patients showed redundancy gain not exceeding probability models. Violation of probability models was not associated with a specific type of callosal lesion. Neural summation, which probably occurs at collicular level, may be modulated by cortical activity. To test this hypothesis, we used functional MRI. During detection of redundant simultaneous targets, activations in the extrastriate cortex were observed in a patient with callosal agenesis and redundancy gain violating probability models, but not in a patient with callosal agenesis and redundancy gain not exceeding probability models. We conclude that cortical activity in the extrastriate cortex may be a modulating factor in the magnitude of the redundancy gain during parallel visuomotor transforms. (+info)Agenesis of corpus callosum - a rare case. (8/203)
A case of corpus callosum agenesis associated with a chromosomal structural defect is described. (+info)CSPG6 Corpus callosum, agenesis of, with mental retardation, ocular coloboma and micrognathia; 300472; IGBP1 Corpus callosum, ... GATM Agenesis of the corpus callosum with peripheral neuropathy; 218000; SLC12A6 Aicardi-Goutieres syndrome 1, dominant and ... MSX1 Tooth agenesis, selective, 3; 604625; PAX9 Tooth agenesis, selective, 6; 613097; LTBP3 Tooth agenesis, selective, X-linked ... UPK3A Renal agenesis; 191830; RET Renal carcinoma, chromophobe, somatic; 144700; FLCN Renal cell carcinoma; 144700; DIRC2 Renal ...
"Familial agenesis of the corpus callosum with anterior horn cell disease: a syndrome of mental retardation, areflexia, and ... "AGENESIS OF THE CORPUS CALLOSUM WITH PERIPHERAL NEUROPATHY; ACCPN". www.omim.org. Retrieved 2017-01-19. RESERVED, INSERM US14 ... "Orphanet: Corpus callosum agenesis neuronopathy syndrome". www.orpha.net. Retrieved 2017-01-19. Dupré, Nicolas; Howard, Heidi C ... Andermann syndrome also known as agenesis of corpus callosum with neuronopathy (ACCPN), Charlevoix disease among others is a ...
This notably includes agenesis of the corpus callosum. Disturbances in the genesis of neural elements can result in cortical ...
... he has been studying the implications of agenesis of the corpus callosum (i.e., congenital absence of the corpus callosum, the ... Brown, W.S. and Paul L.K., (2000) Psychosocial deficits in agenesis of the corpus callosum with normal intelligence. Cognitive ... "Travis Research Institute - Agenesis of the Corpus Callosum". Travis Research Institute. 2011. Retrieved 21 January 2015. ... on cognitive and social disabilities of individuals with agenesis of the corpus callosum. He has authored or coauthored over 75 ...
A correlation between agenesis of the corpus callosum and intellectual disability in LFS, however, has not been suggested. ... A relatively common brain anomaly seen with LFS is agenesis of the corpus callosum, an error of embryonic development in which ... Some features found in LFS, like agenesis of the corpus callosum and cartilage-related craniofacial anomalies, are similar to ... corpus callosum agenesis and hypotonia. Notable features of FGS that have not been reported with LFS include excessive ...
Familial agenesis of corpus callosum with anterior horn cell disease. Trans Am Neurol Assoc 1972; 97: 242-244 Andermann E, ...
... agenesis of corpus callosum). It was found that autism is commonly diagnosed in children where the corpus callosum does not ... May 2013). "Autism traits in individuals with agenesis of the corpus callosum". Journal of Autism and Developmental Disorders. ... of children with agenesis of the corpus callosum). A further example of brain structures relating to ASD is that children with ... A further example of how brain structure can influence ASD is looking at cases where the corpus callosum does not fully develop ...
Agenesis of the corpus callosum (ACC) is used to determine the frequency of social and behavioral problems in children with a ... For younger children, ages two to five, Agenesis of the corpus callosum causes problems in sleep. Sleep is critical for ... "Social and behavioral problems of children with agenesis of the corpus callosum". Child Psychiatry and Human Development. 38 (4 ... prevalence rate of about 2-3%. ACC is described as a defect in the brain where the 200 million axons that make the corpus ...
The corpus callosum (CC) is the major route of communication between the two hemispheres. At maturity it is a large mass of ... callosal agenesis, or hemispherectomy; Brain Res Cogn Brain Res 25(2), 2005 Bamiou D et al.; Auditory interhemispheric transfer ... With children the underdeveloped Corpus Callosum (CC) is unable, in any case, to transfer auditory streams arriving (from the ... with the development of the Corpus Callosum (CC), peaking before the fourth decade. During middle age and older the auditory ...
A reduced corpus callosum is present in some cases(agenesis of the corpus callosum). Gomez-Lopez-Hernandez Syndrome is ... Choudhri AF, Patel RM, Wilroy RS, Pivnick EK, Whitehead MT (2015). "GTrigeminal nerve agenesis with absence of foramina rotunda ...
Defects in KCC3 are linked to agenesis of the corpus callosum with peripheral neuropathy. This disorder is characterised by ... dysmorphic features and complete or partial agenesis of the corpus callosum. Gillen CM, Brill S, Payne JA, Forbush B (July 1996 ... "The K-Cl cotransporter KCC3 is mutant in a severe peripheral neuropathy associated with agenesis of the corpus callosum". Nat. ...
partial or complete agenesis of the corpus callosum intellectual disability motor abnormalities visual defects such as, ... Corpus callosum is the band of white matter connecting the two cerebral hemispheres. The corpus callosum plays an extremely ... The partial or complete absence of white matter, also known as agenesis of the corpus callosum results in anatomic ... It is a nonspecific finding and is associated with multiple neurological syndromes, including agenesis of the corpus callosum, ...
2002). "The K-Cl cotransporter KCC3 is mutant in a severe peripheral neuropathy associated with agenesis of the corpus callosum ... Mutations in this gene are associated with agenesis of the corpus callosum with peripheral neuropathy. Solute carrier family ... GeneReview/NIH/UW entry on Hereditary Motor and Sensory Neuropathy with Agenesis of the Corpus Callosum Race JE, Makhlouf FN, ...
These include Agenesis of the corpus callosum (80-99% patients) Hypopigmentation of the eyes and hair (80-99% patients) ... 1999). "Albinism and agenesis of the corpus callosum with profound developmental delay: Vici syndrome, evidence for autosomal ... 1999) Albinism and agenesis of the corpus callosum with profound developmental delay: Vici syndrome, evidence for autosomal ... 1988). "Agenesis of the corpus callosum, combined immunodeficiency, bilateral cataract, and hypopigmentation in two brothers". ...
Besides having small optic nerves, persons with ONH can have agenesis of the corpus callosum, absence of the septum pellucidum ... Abnormalities evident via neuroradiography can include agenesis (absence) or hypoplasia of the corpus callosum, absence or ... Predictors of significantly delayed development include hypoplasia or agenesis of the corpus callosum and hypothyroidism. The ... Hypoplasia of the corpus callosum, often in conjunction with other major malformations, is significantly associated with poor ...
One such study supported colour perception in callosal agenesis (Those born without a corpus callosum; Barr & Corballis, 2002 ... The corpus callosum allows for communication between the two hemispheres and is found only in placental mammals (the eutherians ... The great majority of fibers connecting the two hemispheres travel through the corpus callosum, which is over 10 times larger ... Anterior commissure posterior commissure corpus callosum This article incorporates text in the public domain from the 20th ...
Associated with agenesis (loss) of the corpus callosum, intellectual disabilities are common among individuals with FG syndrome ... and partial or complete loss of the corpus callosum. About a third of reported cases of individuals with FG syndrome die in ...
"Homozygous silencing of T-box transcription factor EOMES leads to microcephaly with polymicrogyria and corpus callosum agenesis ... and lack the corpus callosum, another region of the brain involved in hemisphere connections. Eomesodermin/Tbr2 has also been ...
Partial or complete absence of the corpus callosum in the brain (agenesis of the corpus callosum); Eye abnormalities known as " ... syndrome characterized by the partial or complete absence of a key structure in the brain called the corpus callosum, the ... J Child Neurol 2008;23:531-5 Aicardi J, Lefebvre J, Lerique-Koechlin A. A new syndrome: spasm in flexion, callosal agenesis, ...
Agenesis of the corpus callosum, in which there is a complete or partial absence of the corpus callosum. It occurs when the ... The fibers that would otherwise form the corpus callosum become longitudinally oriented within each hemisphere and form ... corpus callosum, the band of white matter connecting the two hemispheres in the brain, fails to develop normally, typically ...
This includes disorders such as HSAS[disambiguation needed], MASA syndrome, agenesis of the corpus callosum and spastic ... Fransen E, Lemmon V, Van Camp G, Vits L, Coucke P, Willems PJ (1996). "CRASH syndrome: clinical spectrum of corpus callosum ... Mutations in the L1 protein are the cause of three neurological syndromes known by the acronym CRASH (corpus callosum ...
2008 Jun;66(2B):418-9. PMID 18641886 "Subtotal corpus callosum agenesis with recurrent hyperhidrosis-hypothermia (Shapiro ... and agenesis of the corpus callosum with onset typically on adulthood. The disease affects about 50 people worldwide. The ...
Additionally, she was born with Ebstein malformation, a congenital heart defect, agenesis of the corpus callosum, and hip ...
... with agenesis of the corpus callosum". Clinical Dysmorphology. 3 (4): 292-6. doi:10.1097/00019605-199410000-00003. PMID 7894733 ...
... or agenesis of the corpus callosum. Diagnosis is principally by MRI. Frequently, arachnoid cysts are incidental findings on MRI ...
A recent study of individuals with agenesis of the corpus callosum suggests that the corpus callosum plays a vital role in ... Corpus callosum[edit]. Main article: Corpus callosum. The corpus callosum is the largest commissural tract in the human brain. ... Age-related decline in the commissural fiber tracts that make up the corpus callosum indicate the corpus callosum is involved ... The corpus callosum allows for communication between the two hemispheres and is found only in placental mammals (the eutherians ...
What is Corpus callosum agenesis? Meaning of Corpus callosum agenesis as a legal term. What does Corpus callosum agenesis mean ... Definition of Corpus callosum agenesis in the Legal Dictionary - by Free online English dictionary and encyclopedia. ... Corpus callosum agenesis legal definition of Corpus callosum agenesis https://legal-dictionary.thefreedictionary.com/Corpus+ ... corpus. (redirected from Corpus callosum agenesis). Also found in: Dictionary, Thesaurus, Medical, Financial, Encyclopedia. ...
Other anomalies associated with Dandy-Walker Malformation include agenesis of the Corpus Callosum, Aqueduct of Sylvius stenosis ...
TF - Are you sure thats a corpus luteum? * Readout - Is that a paraovarian cyst? ... TF - Renal Agenesis and UPJ Obstruction * Skeletal , * TF - How NOT to miss a subtle vertebral segmentation anomaly ...
"Isolated corpus callosum agenesis: a ten-year followup after prenatal diagnosis (how are the children without corpus callosum ... P. H. Tang, A. I. Bartha, M. E. Norton, A. J. Barkovich, E. H. Sherr, and O. A. Glenn, "Agenesis of the corpus callosum: an MR ... Complete Corpus Callosum Agenesis: Can It Be Mild?. Matteo Chiappedi, Anna Fresca, and Ilaria Maria Carlotta Baschenis ... Corpus callosum agenesis (CCA) is among the most common brain malformations observed in humans [1]. Its incidence varies as a ...
Supporting those with Agenesis of the Corpus Callosum and Aicardi Syndrome. Corpal supports those affected by Agenesis of the ... Im Isla is going to to 6 2minute activities for Jacqui Latham because partial Agenesis of the corpus callosum ... Corpal - Supporting those with Agenesis of the Corpus Callosum and Aicardi Syndrome Registered charity number 1086019 ... Corpal - Supporting those with Agenesis of the Corpus Callosum and Aicardi Syndrome. ...
Partial agenesis of the corpus callosum. *Abnormal corpus callosum (size, anatomy) As diagnosed by an expert ultrasound fetal ... Isolated corpus callosum agenesis: a ten-year follow-up after prenatal diagnosis (how are the children without corpus callosum ... Infants With Agenesis of the Corpus Callosum (Dacci). The safety and scientific validity of this study is the responsibility of ... Agenesis of the Corpus Callosum Other: Psychometric scales for infants at 3 years of age Not Applicable ...
Synonyms: corpus callosum, agenesis of, with mental retardation, ocular coloboma and micrognathia; Graham-Cox syndrome; mental ... corpus callosum agenesis-intellectual disability-coloboma-micrognathia syndrome (DOID:0060816) Alliance: disease page Synonyms: ... Definition: A syndromic X-linked intellectual disability characterized by agenesis of the corpus callosum, intellectual ... corpus callosum, agenesis of, with mental retardation, ocular coloboma and micrognathia; Graham-Cox syndrome; mental ...
Synonyms: Andermann syndrome; Charlevoix disease; corpus callosum agenesis-neuronopathy syndrome ... agenesis of the corpus callosum with peripheral neuropathy (DOID:0090003) Alliance: disease page Synonyms: Andermann syndrome; ... Charlevoix disease; corpus callosum agenesis-neuronopathy syndrome Alt IDs: OMIM:218000, ICD10CM:G60.0, ORDO:1496 Definition: A ... variable degree of agenesis of the corpus callosum, amyotrophy, hypotonia, and cognitive impairment that has_material_basis_in ...
... complete agenesis of the corpus callosum; pAgCC=partial agenesis of the corpus callosum; bars represent standard error. ... Individuals with agenesis of the corpus callosum (AgCC) offer unique insights regarding the cognitive skills that depend ... Processing speed delays contribute to executive function deficits in individuals with agenesis of the corpus callosum.. Marco ... Therefore, while individuals with agenesis of the corpus callosum show real deficits on tasks of executive function, this ...
Hereditary motor and sensory neuropathy with agenesis of the corpus callosum (HMSN/ACC or ACCPN), also known as Andermann ... Retrieved from "https://www.SNPedia.com/index.php?title=Agenesis_of_the_Corpus_Callosum_with_Peripheral_Neuropathy&oldid=936227 ... and amyotrophy and variable degrees of dysgenesis of the corpus callosum.NIH ...
NINDS Agenesis of the Corpus Callosum Information Page www.ninds.nih.gov/health_and _medical/disorders/agenesis_doc.htm ... Handbook of Genetic Counseling/Hypoplasia - Agenesis of the Corpus Callosum. From Wikibooks, open books for an open world ... underdevelopment (hypoplasia) complete absence (agenesis) of the CC. Corpus collosum[edit]. *The area of the brain (large fiber ... Agenesis of the Corpus Collosum. Mental Retardation and Congenital Malformations of the Central Nervous System. Warkany, J, ...
It has been suggested that developmental defects of the corpus callosum may be more frequently encountered in patients with ... Dysgenesis of the corpus callosum has been recognized as a marker for aberrant development of the central nervous system. ... Dysgenesis of the corpus callosum has been recognized as a marker for aberrant development of the central nervous system. It ... Corpus Callosum / abnormalities*, pathology. Female. Humans. Magnetic Resonance Imaging. Male. Models, Biological. ...
... agenesis of the corpus callosum, hydrocephalus and choanal atresia.... ... All had an associated agenesis of the corpus callosum.. Key words. Median cleft face syndrome Frontonasal dysplasia ... François J, Eggermont E, Evens L, Logghe N, De Bock F (1973) Agenesis of the corpus callosum in the median facial cleft ... Median cleft face syndrome in association with hydrocephalus, agenesis of the corpus callosum, holoprosencephaly and choanal ...
Agenesis Corpus Callosum. Agenesis Corpus Callosum. Description. Agenesis of the corpus callosum (ACC) is a rare congenital ... Click here for the latest Australian research papers on Agenesis Corpus Callosum. ... abnormality in which there is a partial or complete absence of the corpus callosum. This is the area of the brain which ...
Prevalence and incidence statistics for Micrencephaly corpus callosum agenesis covering estimated populations and diagnosis ... About prevalence and incidence of Micrencephaly corpus callosum agenesis. *Micrencephaly corpus callosum agenesis: Introduction ... Treatments for Micrencephaly corpus callosum agenesis. *Doctors and Medical Specialists for Micrencephaly corpus callosum ... They list Micrencephaly corpus callosum agenesis as a "rare disease". More information about Micrencephaly corpus callosum ...
Note the absence of the corpus callosum, with the ventricles taking on the appearance of a moose head, with the third ventricle ...
The corpus callosum is absent.. Axial images: The bodies of lateral venticles have a parallel orientation. Fibers of white ... These are considered as the fibers that meant to make corpus callosum. The posterior parts of lateral ventricles are enlarged; ...
Medical article NINDS Agenesis of the Corpus Callosum Information Page NINDS including all symptom, diagnosis, misdiagnosis, ... Conditions: Agenesis of the Corpus Callosum What is Agenesis of the Corpus Callosum?. Agenesis of the corpus callosum (ACC) is ... of the corpus callosum. The corpus callosum is the area of the brain which connects the two cerebral hemispheres. In most ... Article title: NINDS Agenesis of the Corpus Callosum Information Page: NINDS ...
... answered by our Genetic and Rare Diseases Information Specialists for White matter hypoplasia-corpus callosum agenesis- ... The National Organization of Disorders of the Corpus Callosum provides a list of FAQs about disorders of the corpus callosum. ... White matter hypoplasia-corpus callosum agenesis-intellectual disability syndrome Title Other Names:. Curatolo Cilio Pessagno ... Corpus callosum agenesis is one of the more frequent congenital. malformations. It can be either asymptomatic or associated ...
A case of agenesis of the corpus callosum accompanied by a ruptured azygos anterior cerebral artery aneurysm and lipoma--a case ... A case of agenesis of the corpus callosum accompanied with a ruptured azygos anterior cerebral artery aneurysm and a lipoma a ... Agenesis of the corpus callosum associated with an azygos anterior cerebral artery, a lipoma and porencephalic cyst. South ... Interhemispheric lipoma associated with agenesis of corpus callosum in an infant: case report. Medical Ultrasonography 12(3): ...
... and corpus callosum agenesis. This mutation affects an invariant residue in the homeodomain of VAX1 and was absent from 96 ... VAX1 Mutation Associated with Microphthalmia, Corpus Callosum Agenesis, and Orofacial Clefting: The First Description of a VAX1 ... Home , VAX1 Mutation Associated with Microphthalmia, Corpus Callosum Agenesis, and Orofacial Clefting: The First Description of ... and corpus callosum agenesis. This mutation affects an invariant residue in the homeodomain of VAX1 and was absent from 96 ...
Corpus callosum agenesis from asymptomatic to mild or severe neurodevelopmental disorders. However, few cases have been ... The corpus callosum is the main pathway connect the interhemispheric communication. ... agenesis of the corpus callosum; widened lateral ventricle. agenesis of corpus callosum; Morphological changes of lateral ... agenesis of the corpus callosum; widened lateral ventricle. agenesis of corpus callosum; Morphological changes of lateral ...
AgenesisDysplasiaCorpus callosumBrain developmentNeuroradiology. Background. The corpus callosum (CC) is the primary neuronal ... Partial agenesis is the absence of some but not all components of the corpus callosum. Hypoplastic corpus callosum is a thin ... Sagittal MRI images displaying classes and subclasses of corpus callosum abnormalities. Classes of corpus callosum ... displayed complete agenesis of the corpus callosum with other abnormalities, while only 18 (9 %) displayed complete agenesis ...
Corpus Callosum Agenesis. Introduction. Agenesis of corpus callosum (ACC) [OMIM 217990] is one of the most common congenital ... has been described in agenesis of corpus callosum. Partial agenesis of corpus callosum has correlations with sigmoid bundles ... Depending on the part of corpus callosum affected, agenesis of the corpus callosum may classify into one of two varieties:. * ... As a rule of thumb, primary agenesis of corpus callosum (total agenesis) can be excluded by the presence of the rostrum. One ...
Isolated agenesis of the corpus callosum is not innocuous, and patients with this defect require a strict neurocognitive follow ... Patients with isolated agenesis of the corpus callosum may present with severe intellectual disability, although a proportion ... An 8-year-old Hispanic boy with total agenesis of the corpus callosum attended for medical follow-up. The defect was identified ... Here, we report an illustrative case of a school-age child with isolated agenesis of the corpus callosum and normal ...
HumanLeftBodyDevelopment of the corpus callosumMalformationPartialAbnormalitiesMental retardationCallosal agenesisInclude agenesis of the cAnomaliesAbnormalityCerebralDisordersHypoplasticInterhemisphericAbsentFetalTotal agenesisCorticalAbnormal corpus callosumUltrasoundDiagnosisDefectsKnown as agenesisSyndromesHemispheresAnteriorGenuAutosomal recessivePrevalenceCyst associatedLipomaAttention-deficit-IntraventricularCompleteCollosum
- Corpus might be used to mean a human body, or a body or group of laws. (thefreedictionary.com)
- In the law of estates, the corpus of an estate is the amount of property left when an individual dies. (thefreedictionary.com)
- Corpus juris means a body of law or a body of the law. (thefreedictionary.com)
- corpus juris civilis, the body of the Civil law. (thefreedictionary.com)
- The literature reviewed here suggested that patients with agenesis of the corpus callosum require a strict neurocognitive follow-up during postnatal life, as they may present neuropsychological deficits during adolescence, when development of the corpus callosum is completed and there is maximum reliance on this structure. (biomedcentral.com)
- Richards, L. J., Plachez, C. and Ren, T. (2004) Mechanisms regulating the development of the corpus callosum and its agenesis in mouse and human. (edu.au)
- The development of the corpus callosum depends on a large number of different cellular and molecular mechanisms. (edu.au)
- Data in humans and in mice suggest the possibility that different mechanisms may regulate the development of the corpus callosum across its rostrocaudal and dorsoventral axes. (edu.au)
- Structural abnormalities (cysts) that interfere with normal development of the corpus callosum. (ourmed.org)
- Problems during development of the corpus callosum can range from underdevelopment, where there are fewer than normal numbers of fibers connecting the two sides of the brain (partial agenesis) to the complete absence all connections (complete agenesis). (ohiofetalmedicine.org)
- We propose ZNF148 as a gene involved in a newly described ID syndrome with a recurrent phenotype and postulate that the ZNF148 is a hitherto unrecognized but crucial transcription factor in the development of the corpus callosum. (nih.gov)
- (britannica.com)
- In rare cases, one of these features may be missing (especially lack of development of the corpus callosum). (floridahealthfinder.gov)
- Corpus callosum agenesis is a relatively common brain malformation. (hindawi.com)
- Agenesis of the corpus callosum is the most frequent brain malformation. (clinicaltrials.gov)
- Corpus callosum malformation and dysfunction are increasingly recognized causes of cognitive and behavioral disability. (nih.gov)
- Agenesis of the corpus callosum (ACC) is a developmental brain malformation associated with a wide spectrum of structural brain abnormalities and genetic loci. (biomedcentral.com)
- Thus, physicians require a better knowledge of the cognitive features of agenesis of the corpus callosum to improve their approach to this cerebral malformation. (biomedcentral.com)
- Agenesis of the corpus callosum (AgCC) is a rare brain malformation that can occur isolated or associated with other anatomical defects as part of a complex congenital syndrome [ 1 ]. (biomedcentral.com)
- Raybaud C. The corpus callosum, the other great forebrain commissures, and the septum pellucidum: anatomy, development, and malformation. (sickkids.ca)
- Dysgenesis is a broad term for any malformation of the corpus callosum that is not a complete agenesis. (ourmed.org)
- Loss of Nf2 causes malformation of midline guideposts and Slit2 upregulation, resulting in callosal agenesis. (biologists.org)
- Agenesis of the corpus callosum (ACC) is a congenital malformation that can occur in isolation or in association with other neurological conditions. (peertechz.com)
- Developmental absence (agenesis) of the corpus callosum (AgCC) is a congenital brain malformation resulting from disruption of corpus callosum formation, a structure that is crucial for the transfer and integration of information, including attention processes, across the brain. (maastrichtuniversity.nl)
- Dr. Elliott Sherr and his collaborators at University of California, San Francisco (UCSF) are studying the genetic causes of disorders of cognition and epilepsy, in particular disorders of brain development that affect the corpus callosum, such as Aicardi syndrome, as well as two additional brain malformations, polymicrogyria and Dandy-Walker malformation. (clinicaltrials.gov)
- To participate in the study, you will be asked to provide a copy of the magnetic resonance imaging (MRI) documenting Agenesis Corpus Callosum (ACC), Polymicrogyria (PMG), or Dandy-Walker malformation (DWM), clinical information, and blood samples from the affected individual and from the parents. (clinicaltrials.gov)
- Progress in neuroimaging has revealed that the central nervous system malformation in AS is not limited to agenesis of the corpus callosum but consists of a complex of abnormalities characterized by severe neuronal migration defects with periventricular and subcortical heterotopias, cortical polymicrogyria, and a tendency toward the development of cystic formations in the choroid plexuses and in other parts of the brain (3-5). (slideshare.net)
- All 19 (100%) expressed variable degrees of hypoplasia, complete or partial agenesis (ACC). (biomedsearch.com)
- Agenesis of the corpus callosum (ACC) is a rare congenital abnormality in which there is a partial or complete absence of the corpus callosum. (brainfoundation.org.au)
- Partial agenesis is the absence of some but not all components of the corpus callosum. (biomedcentral.com)
- Agenesis of corpus callosum (ACC) [OMIM is one of the most common congenital cerebral malformations which is morphologically the complete or partial absence of corpus callosum, and not defined by functional or behavioral abnormalities (as in autism). (statpearls.com)
- Partial agenesis (hypogenesis or dysgenesis), in which only the splenium and/or rostrum are absent. (statpearls.com)
- Partial agenesis of corpus callosum has correlations with 'sigmoid bundles,' which asymmetrically connect the frontal lobe with the contralateral occipitoparietal cortex. (statpearls.com)
- The defect is "complete" when total absence of the corpus callosum (CC) occurs or "partial" when only certain regions of the structure are formed. (biomedcentral.com)
- In the case reported by Baker and Graves the agenesis was partial. (jamanetwork.com)
- Birth defect that results in a partial or complete absence of the CORPUS CALLOSUM. (sickkids.ca)
- The types of agenesis of the corpus callosum are: complete agenesis of the corpus callosum (ACC), partial ACC, hypoplasia of the corpus callosum, and dysgenesis of the corpus callosum. (ourmed.org)
- In partial ACC, the corpus callosum began to develop, but does not complete development. (ourmed.org)
- Since the corpus callosum develops from front to back, the part of the corpus callosum that is present in partial ACC usually will be toward the front of the brain, with the back portion missing. (ourmed.org)
- Partial ACC includes the entire range of partial absence, from absence of only a small portion of callosal fibers to absence of most of the corpus callosum. (ourmed.org)
- Thus, partial ACC and hypoplasia of the corpus callosum would be forms of dysgenesis, as would any other form of inadequate callosal development. (ourmed.org)
- Disc1 tr transgenic mice display enlarged lateral ventricles, reduced cerebral cortex, partial agenesis of the corpus callosum, and thinning of layers II/III with reduced neural proliferation at midneurogenesis. (jneurosci.org)
- The brain computed tomography scan and the magnetic resonance showed the lipoma and the partial agenesis of the corpus callosum. (ijmedicalimaging.org)
- Defects in callosal neuron specification and production, midline patterning, guidepost formation or guidance cue expression and reception might result in complete or partial agenesis of the corpus callosum. (biologists.org)
- Complete or partial (usually of the posterior part) absence of the corpus callosum in a mid-sagittal view of the brain. (fetalmedicine.com)
- The MRI revealed partial agenesis of the corpus callosum , bilateral hypoplastic hippocampi but bilaterally intact amygdala. (symptoma.com)
- Partial agenesis of the corpus callosum , hippocampal atrophy, and stable intellectual disability associated with Roifman syndrome. (symptoma.com)
- Partial agenesis of the corpus callosum and central diffuse cortical cerebral atrophy was documented on computed tomography. (symptoma.com)
- Imaging of his brain revealed partial agenesis of the corpus callosum (ACC). (symptoma.com)
- An uncommon association of infantile spasms, septo-optic dysplasia, partial agenesis of the corpus callosum and an interhemispheric cyst is described in a child. (symptoma.com)
- Chorioretinal lacunae Criteria that are highly suggestive of Aicardi syndrome Partial or complete callosal agenesis Cortical dysplasia Gross asymmetry of the hemispheres Periventricular or subcortical heterotopias Cysts of the choroid plexus or around the third ventricle is highly suggestive of AS DIAGNOSIS: DIAGNOSIS: AICARDI SYNDROME ASSOCIATED WITH MULTIPLE CORTICAL DYSPLASIAS THAT INCLUDE HEMIMEGALENCEPHALY, LISSENCEPHALY, HETEROTOPIAS, AND SEPTO-OPTIC DYSPLASIA. (slideshare.net)
- A congenital abnormality in which there is a partial or complete absence of the corpus callosum. (nodcc.org)
- A genetic disorder characterized by the partial or complete agenesis of the corpus callosum, infantile spasms (a characteristic form of childhood seizures), mental retardation, and an ocular (eye) abnormality called chorioretinal lacunae in which there are lacunae (holes) in the retina of the eye. (nodcc.org)
- The objectives of the present study were to determine the prevalence of developmental defects of the corpus callosum in patients attending a genetics-metabolic disorders clinic, to describe the spectrum of abnormalities in brain development in patients with confirmed inborn errors of metabolism and abnormalities of the corpus callosum as ascertained by neuroimaging and/or postmortem studies. (biomedsearch.com)
- The routine prenatal ultrasound examination showed abnormal morphology of corpus callosum in fetus 1, dysplasia of the corpus callosum in fetus 3, the corpus callosum absence in fetus 2 and fetus 4, and fetus 4 also had cardiac abnormalities. (researchsquare.com)
- The genetic mechanism of corpus callosum agenesis is variably complex, and its clinical phenotype may occur either alone or in association with other abnormalities. (researchsquare.com)
- To characterize the diverse callosal morphologies and malformations of brain development associated with ACC, we report on the neuroimaging findings of 201 individuals diagnosed with corpus callosal abnormalities. (biomedcentral.com)
- Forty-one (20 %) displayed complete agenesis of the corpus callosum with other abnormalities, while only 18 (9 %) displayed complete agenesis without associated brain abnormalities. (biomedcentral.com)
- In contrast, hippocampal abnormalities, colpocephaly, and Probst bundles were significantly more frequent in complete agenesis compared to hypoplasia or dysplasia group. (biomedcentral.com)
- Collectively, our results underscore the broad diversity of morphological variants of the corpus callosum and associated brain abnormalities in individuals with ACC. (biomedcentral.com)
- Agenesis of the corpus callosum can present as an isolated condition or associated with other cerebral abnormalities. (statpearls.com)
- Al-Hashim AH, Blaser S, Raybaud C, MacGregor D. Corpus callosum abnormalities: neuroradiological and clinical correlations. (sickkids.ca)
- Many people with agenesis of the corpus callosum do not have any symptoms or the symptoms may range from subtle or mild to severe, depending on whether and which associated brain abnormalities are present. (cdc.gov)
- The life expectancy for someone with corpus callosum agenesis depends on the presence of other abnormalities. (cdc.gov)
- DISCUSSION DISCUSSION: The Aicardi syndrome (AS) is classically defined as a triad of abnormalities that includes agenesis of the corpus callosum, infantile spasms, and chorioretinal lacunae (1,2). (slideshare.net)
- IMSEAR at SEARO: Association of corpus callosum agenesis with mental retardation and attention deficit hyperactivity disorder. (who.int)
- We aim to report a case of corpus callosal agenesis (CCA) associated with mental retardation and hyperactivity. (who.int)
- Presentation of case: We report case of a 7 year old boy having CCA, abnormal facial morphological features, mental retardation and attention deficit hyperactivity disorder (ADHD), devoid of any chromosomal rearrangements or somatic malformations known to be associated with corpus callosal changes. (who.int)
- of corpus callosum , polysyndactyly and mental retardation. (symptoma.com)
- However, the disorder is typically characterized by underdevelopment (hypoplasia) or absence (agenesis) of the corpus callosum and moderate to severe mental retardation. (nodcc.org)
- The image illustrates the dramatic lack of inter hemispheric connections in callosal agenesis. (freerepublic.com)
- Additional common features include agenesis of the corpus callosum, sensorineural hearing loss, intellectual disability, and congenital diaphragmatic hernia and/or omphalocele. (nih.gov)
- A syndromic X-linked intellectual disability characterized by agenesis of the corpus callosum, intellectual disability, ocular coloboma, micrognathia, sensorineural hearing loss, skeletal anomalies, and short stature that has_material_basis_in mutation in the IGBP1 gene on chromosome Xq13.1. (jax.org)
- Agenesis of the corpus callosum and cerebral anomalies in inborn errors of metabolism. (biomedsearch.com)
- 73 Agenesis of the corpus callosum, with facial anomalies and cerebellar ataxia: An autosomal recessive intellectual disability syndrome characterized by congenital microcephaly, low anterior hairline, bitemporal narrowing, low-set protruding ears, strabismus and tented thick eyebrows with sparse hair in their medial segment. (malacards.org)
- Corpus Callosum, Agenesis of, with Facial Anomalies and Cerebellar Ataxia, also known as birk-flusser syndrome , is related to agenesis of the corpus callosum with peripheral neuropathy and aicardi syndrome . (malacards.org)
- An important gene associated with Corpus Callosum, Agenesis of, with Facial Anomalies and Cerebellar Ataxia is FRMD4A (FERM Domain Containing 4A). (malacards.org)
- Accuracy of prenatal ultrasound in the diagnosis of corpus callosum anomalies. (nature.com)
- Corpus callosum and limbic system: neuroanatomic MR evaluation of developmental anomalies. (ijmedicalimaging.org)
- However, few cases have been previously reported in the literature on prenatal diagnosis between corpus callosum agenesis and chromosomes abnormality. (researchsquare.com)
- The study included prenatal diagnosis of 4 fetuses in whom prenatal ultrasound showed corpus callosum abnormality, isolated or associated with other central nervous lesions. (researchsquare.com)
- This study revealed the microabnormalities of multiple chromosomes are related to the corpus callosum abnormality. (researchsquare.com)
- Agenesis of the corpus callosum (ACC) is a birth defect in which the structure that connects the two hemispheres of the brain (the corpus callosum) is partially or completely absent due to an abnormality of development during the fetal period. (ourmed.org)
- Abnormality of the corpus callosum (AbnCC) is etiologically a heterogeneous condition and the prognosis in prenatally diagnosed cases is difficult to predict. (nature.com)
- Copy number variations found in patients with a corpus callosum abnormality and intellectual disability. (nature.com)
- The corpus callosum is the area of the brain which connects the two cerebral hemispheres. (rightdiagnosis.com)
- Namely, she had an agenesis of the corpus callosum, an azygos anterior cerebral artery, an aneurysm and a lipoma. (eurekamag.com)
- There are some reports in which an agenesis of the corpus callosum is accompanied with a lipoma and an azygos anterior cerebral artery is accompanied with an aneurysm. (eurekamag.com)
- But a case of agenesis of the corpus callosum with an azygos anterior cerebral artery was rarely reported. (eurekamag.com)
- The corpus callosum connects cerebral hemispheres and is the largest axon tract in the mammalian brain. (biologists.org)
- Corpus callosum , bundle of nerve fibres in the longitudinal fissure of the brain that enables corresponding regions of the left and right cerebral hemispheres to communicate. (britannica.com)
- The corpus callosum has played an important role in the elucidation of functions specific to each of the cerebral hemispheres. (britannica.com)
- Our lab has shown that de novo and inherited genetic events play an important role in causing disorders of cerebral development, specifically focusing on patients with agenesis of the corpus callosum (ACC). (ucsf.edu)
- Corpus callosum agenesis from asymptomatic to mild or severe neurodevelopmental disorders. (researchsquare.com)
- That just should not happen and I hope that more and more medical professionals will become better educated about Agenesis of the Corpus Callosum and other corpus callosum disorders and think before they attempt to speak the future of that little baby or child. (blogspot.com)
- The National Organization of Disorders of the Corpus Callosum provides a list of FAQs about disorders of the corpus callosum. (cdc.gov)
- Hypoplastic corpus callosum is a thin but structurally intact corpus callosum. (biomedcentral.com)
- The corpus callosum is markedly hypoplastic and deficient. (slideshare.net)
- The corpus callosum is the main pathway connect the interhemispheric communication. (researchsquare.com)
- The emerging view is that the corpus callosum consists of many parallel interhemispheric channels for communication and control, and that every transfer channel is context-dependent and modulated by attention. (mit.edu)
- The corpus callosum is absent. (radiopaedia.org)
- Corpus callosum agenesis is a birth defect in which the structure that connects the two sides of the brain (the corpus callosum ) is partially or completely absent. (cdc.gov)
- Fetal ultrasound can detect agenesis of the corpus callosum as early as the 16th week of gestation. (statpearls.com)
- When one of these factors interfere with development of the fetal brain (between the fifth and sixteenth week of pregnancy), agenesis of the corpus callosum may result. (ourmed.org)
- Achiron R, Achiron A. Development of the human fetal corpus callosum: a high-resolution, cross-sectional sonographic study. (nature.com)
- As a rule of thumb, primary agenesis of corpus callosum (total agenesis) can be excluded by the presence of the rostrum. (statpearls.com)
- An 8-year-old Hispanic boy with total agenesis of the corpus callosum attended for medical follow-up. (biomedcentral.com)
- At the age of 4, he was subjected to a control brain magnetic resonance imaging that showed total agenesis of the corpus callosum and colpocephaly. (biomedcentral.com)
- The axons and dendrites of the neurons in the corpus callosum synapse with cortical neurons on symmetrically related points of the hemispheres. (britannica.com)
- Children born to women treated for hypothyroidism during pregnancy show abnormal corpus callosum development. (sickkids.ca)
- Agenesis of the corpus callosum can be diagnosed in the fetus by ultrasound or Magnetic resonance imaging (MRI) of the head. (ourmed.org)
- The purpose of this study is to assess the neurological development at three years of age of children born after prenatal diagnosis of 'isolated' agenesis of the corpus callosum. (clinicaltrials.gov)
- The term 'incidence' of Micrencephaly corpus callosum agenesis refers to the annual diagnosis rate, or the number of new cases of Micrencephaly corpus callosum agenesis diagnosed each year. (rightdiagnosis.com)
- To provide a better understanding of the chromosomal variation and the corpus callosum agenesis in prenatal diagnosis, we present our study on prenatal diagnosis of four corpus callosum structural abnormal fetuses by CMA. (researchsquare.com)
- Isolated corpus callosum agenesis: a ten-year follow-up after prenatal diagnosis (how are the children without corpus callosum at 10 years of age? (nature.com)
- Sotiriadis A, Makrydimas G. Neurodevelopment after prenatal diagnosis of isolated agenesis of the corpus callosum: an integrative review. (nature.com)
- It has been suggested that developmental defects of the corpus callosum may be more frequently encountered in patients with inborn errors of metabolism. (biomedsearch.com)
- Credit: Lynn Paul/Caltech (Medical Xpress) Building on their prior work, a team of neuroscientists at Caltech now report that rare patients who are missing connections between the left and right sides of their brain a condition known as agenesis of. (freerepublic.com)
- Genetic factors: Agenesis of the corpus callosum often correlates with several aneuploidy and non-aneuploidy syndromes. (statpearls.com)
- The complex developmental processes required for formation of the corpus callosum may provide some insight into why such a large number of human congenital syndromes are associated with agenesis of this structure. (edu.au)
- Clinical, genetic and imaging findings identify new causes for corpus callosum development syndromes. (nature.com)
- Corpus callosum is the largest white matter structure containing 200 million axons, connecting the two hemispheres of the brain. (statpearls.com)
- The corpus callosum is indicated in red, fading as the fibers enter the hemispheres in order to suggest that they continue on. (freerepublic.com)
- Study of the corpus callosum, the bands of tissue uniting the brain's two hemispheres, is central to understanding neuroanatomy, neurophysiology, and behavior. (mit.edu)
- The neurons in the corpus callosum also are insulated by a myelin sheath, which facilitates the rapid conduction of electrical impulses between the hemispheres. (britannica.com)
- For example, studies of individuals being treated for epilepsy in whom the corpus callosum has been severed, allowing the two hemispheres to function largely independently, have revealed that the right hemisphere has more language competence than was thought. (britannica.com)
- Recently a reduction in the number of von Economo neurons, which are large spindle-shaped neurons localized to the anterior cingulate cortex and frontoinsular cortex, has been described in agenesis of corpus callosum. (statpearls.com)
- Anterior and posterior commissures in agenesis of the corpus callosum: Alternative pathways for attention processes? (maastrichtuniversity.nl)
- This study aimed to investigate previously proposed candidates for alternative inter-hemispheric pathways in AgCC by examining (1) white matter volume and microstructure of the anterior and posterior commissures in children with AgCC compared to typically developing controls (TDC), and (2) in children with AgCC, examine the associations of white matter volume and microstructure of the anterior and posterior commissures and any remaining corpus callosum with attention processes. (maastrichtuniversity.nl)
- 001). In children with AgCC, we found microstructural properties of the anterior commissure associated with attentional processes, specifically, mean FA of the anterior commissure was associated with better divided attention (p = .03), and the association between alerting attention and mean AD and RD was found to be moderated by age (p = .027, p = .008) and the degree of corpus callosum agenesis (p = .025, p = .016). (maastrichtuniversity.nl)
- 9 These cells are the site of origin of the anterior commissure and the corpus callosum, respectively. (appliedradiology.com)
- Sagittal T2WI (Figure 3) shows the CSF signal intensity mass associated with aplasia of the rostrum and portion of the genu of the corpus callosum (CC). The remainder of the CC is well formed. (appliedradiology.com)
- A neurodegenerative disease characterized by autosomal recessive inheritance with early onset of severe sensory-motor polyneuropathy, variable degree of agenesis of the corpus callosum, amyotrophy, hypotonia, and cognitive impairment that has_material_basis_in homozygous or compound heterozygous mutation in the SLC12A6 gene on chromosome 15q14. (jax.org)
- The term 'prevalence' of Micrencephaly corpus callosum agenesis usually refers to the estimated population of people who are managing Micrencephaly corpus callosum agenesis at any given time. (rightdiagnosis.com)
- Precontrast MR T1 images showing a huge right sided intraventricular /parenchymal cyst associated with agenesis of the septum pellucidum. (slideshare.net)
- The agenesis of the corpus callosum and lipoma is a very rare association. (ijmedicalimaging.org)
- Mehta NM, Hartnoll G. Congenital CMV with callosal lipoma and agenesis. (ijmedicalimaging.org)
- A reduced amount of tissue in the corpus callosum also has been associated with attention-deficit/hyperactivity disorder . (britannica.com)
- Complete Corpus Callosum Agenesis: Can It Be Mild? (hindawi.com)
- We present the case of a girl with a complete agenesis of the corpus callosum discovered at birth. (hindawi.com)
- Complete agenesis (CAG) is a callosal variant lacking all components of the corpus callosum. (biomedcentral.com)
- Archambault, 3 in 1911, described a case of complete agenesis. (jamanetwork.com)
- MRI images from a neurotypical control (left) and an adult with complete agenesis of the corpus callosum (right). (freerepublic.com)
- We conducted resting-state fMRI in eight adult humans with complete agenesis of the corpus callosum (AgCC) and normal intelligence, and compared their data to those from eight healthy matched controls. (jneurosci.org)
- Agenesis of the Corpus Collosum. (wikibooks.org)