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Pachygyria
Pachygyria ( gyri) is a congenital malformation of the cerebral hemisphere. It results in unusually thick convolutions of the cerebral cortex. Typically, children have developmental delay and seizures, the onset and severity depending on the severity of the cortical malformation. Infantile spasms are common in affected children, as is intractable epilepsy. Presentation The term 'pachygyria' does not directly relate to a specific malformation but rather is used to generally describe physical characteristics of the brain in association with several neuronal migration disorders; most commonly disorders relating to varied degrees of lissencephaly. Lissencephaly is present in 1 of 85,470 births and the life span of those affected is short as only a few survive past the age of 20. Pachygyria is a condition identified by a type of cortical genetic malformation. Clinicians will subjectively determine the malformation based on the degree of malposition and the extent of thickened abno ...
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Central Nervous System Disease
Central nervous system diseases or central nervous system disorders are a group of neurological disorders that affect the structure or function of the brain or spinal cord, which collectively form the central nervous system (CNS). These disorders may be caused by such things as infection, injury, blood clots, age related degeneration, cancer, autoimmune disfunction, and birth defects. The symptoms vary widely, as do the treatments. Central nervous system tumors are the most common forms of pediatric cancer. Brain tumors are the most frequent and have the highest mortality. Some disorders, such as substance addiction, autism, and ADHD may be regarded as CNS disorders, though the classifications are not without dispute. Signs and symptoms Every disease has different signs and symptoms. Some of them are persistent headache; pain in the face, back, arms, or legs; an inability to concentrate; loss of feeling; memory loss; loss of muscle strength; tremors; seizures; increased reflex ...
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Lissencephaly
Lissencephaly (, meaning 'smooth brain') is a set of rare brain disorders whereby the whole or parts of the surface of the brain are smooth. It is caused by defective neuronal migration during the 12th to 24th weeks of gestation, resulting in a lack of development of brain folds (gyri) and grooves (sulci). It is a form of cephalic disorder. Terms such as ''agyria'' (no gyri) and '' pachygyria'' (broad gyri) are used to describe the appearance of the surface of the brain. Children with lissencephaly generally have significant developmental delays, but these vary greatly from child to child depending on the degree of brain malformation and seizure control. Life expectancy can be shortened, generally due to respiratory problems. Signs and symptoms Affected children display severe psychomotor impairment, failure to thrive, seizures, and muscle spasticity or hypotonia. Other symptoms of the disorder may include unusual facial appearance, difficulty swallowing, and anomalies of ...
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Aplasia
Aplasia (; from Greek ''a'', "not", "no" + ''plasis'', "formation") is a birth defect where an organ or tissue is wholly or largely absent. It is caused by a defect in a developmental process. Aplastic anemia is the failure of the body to produce blood cells. It may occur at any time, and has multiple causes. __TOC__ Types Pure red cell aplasia Pure red cell aplasia (PRCA) is caused by the selective destruction or inhibition of erythroid progenitor or precursor cells. It is characterized by anemia and reticulocytopenia and can be chronic or acute.  Diamond–Blackfan anemia is a type of PRCA that occurs at birth. PRCA can be acquired as a primary disorder or as a result of another disorder. Immunosuppressive drugs, particularly corticosteroids, will usually result in a temporary or permanent remission. The final outcome is primarily determined by the underlying disorder. Aplasia cutis congenita Aplasia cutis congenita is a condition in which some or large portions of ...
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Muscle Tone
In physiology, medicine, and anatomy, muscle tone (residual muscle tension or tonus) is the continuous and passive partial contraction of the muscles, or the muscle's resistance to passive stretch during resting state.O’Sullivan, S. B. (2007). Examination of motor function: Motor control and motor learning. In S. B. O’Sullivan, & T. J. Schmitz (Eds), Physical rehabilitation (5th ed.) (pp. 233-234). Philadelphia, Pennsylvania: F. A. Davis Company. It helps to maintain posture and declines during REM sleep. Muscle tone is regulated by the activity of the motor neurons and can be affected by various factors, including age, disease, and nerve damage. Purpose If a sudden pull or stretch occurs, the body responds by automatically increasing the muscle's tension, a reflex which helps guard against danger as well as helping maintain balance. Such near-continuous innervation can be thought of as a "default" or "steady state" condition for muscles. Both the extensor and flexo ...
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Subcortical Band Heterotopia
The cerebral cortex, also known as the cerebral mantle, is the outer layer of neural tissue of the cerebrum of the brain in humans and other mammals. It is the largest site of neural integration in the central nervous system, and plays a key role in attention, perception, awareness, thought, memory, language, and consciousness. The six-layered neocortex makes up approximately 90% of the cortex, with the allocortex making up the remainder. The cortex is divided into left and right parts by the longitudinal fissure, which separates the two cerebral hemispheres that are joined beneath the cortex by the corpus callosum and other commissural fibers. In most mammals, apart from small mammals that have small brains, the cerebral cortex is folded, providing a greater surface area in the confined volume of the cranium. Apart from minimising brain and cranial volume, cortical folding is crucial for the brain circuitry and its functional organisation. In mammals with small brains, ther ...
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Glial Cells
Glia, also called glial cells (gliocytes) or neuroglia, are non-neuronal cells in the central nervous system (the brain and the spinal cord) and in the peripheral nervous system that do not produce electrical impulses. The neuroglia make up more than one half the volume of neural tissue in the human body. They maintain homeostasis, form myelin, and provide support and protection for neurons. In the central nervous system, glial cells include oligodendrocytes (that produce myelin), astrocytes, ependymal cells and microglia, and in the peripheral nervous system they include Schwann cells (that produce myelin), and satellite cells. Function They have four main functions: * to surround neurons and hold them in place * to supply nutrients and oxygen to neurons * to insulate one neuron from another * to destroy pathogens and remove dead neurons. They also play a role in neurotransmission and synaptic connections, and in physiological processes such as breathing. While glia wer ...
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Gyrus
In neuroanatomy, a gyrus (: gyri) is a ridge on the cerebral cortex. It is generally surrounded by one or more sulci (depressions or furrows; : sulcus). Gyri and sulci create the folded appearance of the brain in humans and other mammals. Structure The gyri are part of a system of folds and ridges that create a larger surface area for the human brain and other mammalian brains. Because the brain is confined to the skull, brain size is limited. Ridges and depressions create folds allowing a larger cortical surface area, and greater cognitive function, to exist in the confines of a smaller cranium. Development The human brain undergoes gyrification during fetal and neonatal development. In embryonic development, all mammalian brains begin as smooth structures derived from the neural tube. A cerebral cortex without surface convolutions is lissencephalic, meaning 'smooth-brained'. As development continues, gyri and sulci begin to take shape on the fetal brain, with deepe ...
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Hypoplasia
Hypoplasia (; adjective form ''hypoplastic'') is underdevelopment or incomplete development of a tissue or organ.Definition: hypoplasia
Dictionary of Cell and Molecular Biology (11 March 2008) Although the term is not always used precisely, it properly refers to an inadequate or below-normal number of cells.Hypoplasia
Stedman's Medical Dictionary. lww.com
Hypoplasia is similar to aplasia, but less severe. It is technically ''not'' the opposite of
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Forebrain
In the anatomy of the brain of vertebrates, the forebrain or prosencephalon is the rostral (forward-most) portion of the brain. The forebrain controls body temperature, reproductive functions, eating, sleeping, and the display of emotions. Vesicles of the forebrain (prosencephalon), the midbrain (mesencephalon), and hindbrain (rhombencephalon) are the three primary brain vesicles during the early development of the nervous system. At the five-vesicle stage, the forebrain separates into the diencephalon (thalamus, hypothalamus, subthalamus, and epithalamus) and the telencephalon which develops into the cerebrum. The cerebrum consists of the cerebral cortex, underlying white matter, and the basal ganglia. In humans, by 5 weeks in utero it is visible as a single portion toward the front of the fetus. At 8 weeks in utero, the forebrain splits into the left and right cerebral hemispheres. When the embryonic forebrain fails to divide the brain into two lobes, it results i ...
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Glial Fibers
Glia, also called glial cells (gliocytes) or neuroglia, are non-neuronal cell (biology), cells in the central nervous system (the brain and the spinal cord) and in the peripheral nervous system that do not produce Action potential, electrical impulses. The neuroglia make up more than one half the volume of neural Tissue (biology), tissue in the human body. They maintain homeostasis, form myelin, and provide support and protection for neurons. In the central nervous system, glial cells include oligodendrocytes (that produce myelin), astrocytes, ependymal cells and microglia, and in the peripheral nervous system they include Schwann cells (that produce myelin), and Satellite glial cell, satellite cells. Function They have four main functions: * to surround neurons and hold them in place * to supply nutrients and oxygen to neurons * to Myelination, insulate one neuron from another * to destroy pathogens and remove dead neurons. They also play a role in neurotransmission and Synaps ...
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