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Medulloblastomas
Medulloblastoma is a common type of primary brain cancer in children. It originates in the part of the brain that is towards the back and the bottom, on the floor of the skull, in the cerebellum, or posterior fossa. The brain is divided into two main parts, the larger cerebrum on top and the smaller cerebellum below towards the back. They are separated by a membrane called the tentorium. Tumors that originate in the cerebellum or the surrounding region below the tentorium are, therefore, called infratentorial. Historically medulloblastomas have been classified as a primitive neuroectodermal tumor (PNET), but it is now known that medulloblastoma is distinct from supratentorial PNETs and they are no longer considered similar entities. Medulloblastomas are invasive, rapidly growing tumors that, unlike most brain tumors, spread through the cerebrospinal fluid and frequently metastasize to different locations along the surface of the brain and spinal cord. Metastasis all the way d ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Primitive Neuroectodermal Tumor
Primitive neuroectodermal tumor is a malignant (cancerous) neural crest tumor. It is a rare tumor, usually occurring in children and young adults under 25 years of age. The overall 5 year survival rate is about 53%. It gets its name because the majority of the cells in the tumor are derived from neuroectoderm, but have not developed and differentiated in the way a normal neuron would, and so the cells appear "primitive".PNET belongs to the Ewing family of tumors. Genetics Using gene transfer of SV40 large T-antigen in neuronal precursor cells of rats, a brain tumor model was established. The PNETs were histologically indistinguishable from the human counterparts and have been used to identify new genes involved in human brain tumor carcinogenesis. The model was used to confirm p53 as one of the genes involved in human medulloblastomas, but since only about 10% of the human tumors showed mutations in that gene, the model can be used to identify the other binding partners of SV40 L ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Palisade (pathology)
In histopathology, a palisade is a single layer of relatively long cells, arranged loosely perpendicular to a surface and parallel to each other. A rosette is a palisade in a halo or spoke-and-wheel arrangement, surrounding a central core or hub. A pseudorosette is a perivascular radial arrangement of neoplastic cells around a small blood vessel. Rosette A ''rosette'' is a cell formation in a halo or spoke-and-wheel arrangement, surrounding a central core or hub. The central hub may consist of an empty-appearing lumen or a space filled with cytoplasmic processes. The cytoplasm of each of the cells in the rosette is often wedge-shaped with the apex directed toward the central core: the nuclei of the cells participating in the rosette are peripherally positioned and form a ring or halo around the hub. Pathogenesis Rosettes may be considered primary or secondary manifestations of tumor architecture. Primary rosettes form as a characteristic growth pattern of a given tumor type wh ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
PTCH1
Protein patched homolog 1 is a protein that is the member of the patched family and in humans is encoded by the ''PTCH1'' gene. Function PTCH1 is a member of the patched gene family and is the receptor for sonic hedgehog, a secreted molecule implicated in the formation of embryonic structures and in tumorigenesis. This gene functions as a tumor suppressor. The PTCH1 gene product, is a transmembrane protein that suppresses the release of another protein called smoothened, and when sonic hedgehog binds PTCH1, smoothened is released and signals cell proliferation. Clinical significance Mutations of this gene have been associated with nevoid basal cell carcinoma syndrome (AKA Gorlin's Syndrome), esophageal squamous cell carcinoma, trichoepitheliomas, transitional cell carcinomas of the bladder, as well as holoprosencephaly. Alternative splicing results in multiple transcript variants encoding different isoforms. Additional splice variants have been described, but their full leng ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Posterior Cranial Fossa
The posterior cranial fossa is part of the cranial cavity, located between the foramen magnum and tentorium cerebelli. It contains the brainstem and cerebellum. This is the most inferior of the fossae. It houses the cerebellum, medulla and pons. Anteriorly it extends to the apex of the petrous temporal. Posteriorly it is enclosed by the occipital bone. Laterally portions of the squamous temporal and mastoid part of the temporal bone form its walls. Features Foramen magnum The most conspicuous, large opening in the floor of the fossa. It transmits the medulla, the ascending portions of the spinal accessory nerve (XI), and the vertebral arteries. Internal acoustic meatus Lies in the anterior wall of the posterior cranial fossa. It transmits the facial (VII) and vestibulocochlear (VIII) cranial nerves into a canal in the petrous temporal bone. Jugular foramen Lies between the inferior edge of the petrous temporal bone and the adjacent occipital bone and transmits the internal ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Truncal Ataxia
Truncal ataxia (or trunk ataxia) is a wide-based "drunken sailor" gait characterised by uncertain starts and stops, lateral deviations and unequal steps. It is an instability of the trunk and often seen during sitting. It is most visible when shifting position or walking heel-to-toe. As a result of this gait impairment, falling is a concern in patients with ataxia. Truncal ataxia affects the muscles closer to the body such as the trunk, shoulder girdle and hip girdle. It is involved in gait stability. Truncal ataxia is different from appendicular ataxia. Appendicular ataxia affects the movements of the arms and legs. It is caused by lesions of the cerebellar hemispheres. Causes Truncal ataxia is caused by midline damage to the cerebellar vermis. There are at least 34 conditions that cause truncal ataxia. Common * Alcohol intoxication * Cerebral infarction * Cerebral hemorrhage * Cerebellar ataxia * Multiple sclerosis * Friedreich's ataxia * Drugs such as Benzodiazepines, Lit ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
CTNNB1
Catenin beta-1, also known as beta-catenin (β-catenin), is a protein that in humans is encoded by the ''CTNNB1'' gene. Beta-catenin is a dual function protein, involved in regulation and coordination of cell–cell adhesion and gene transcription. In humans, the CTNNB1 protein is encoded by the ''CTNNB1'' gene. In ''Drosophila'', the homologous protein is called ''armadillo''. β-catenin is a subunit of the cadherin protein complex and acts as an intracellular signal transducer in the Wnt signaling pathway. It is a member of the catenin protein family and homologous to γ-catenin, also known as plakoglobin. Beta-catenin is widely expressed in many tissues. In cardiac muscle, beta-catenin localizes to adherens junctions in intercalated disc structures, which are critical for electrical and mechanical coupling between adjacent cardiomyocytes. Mutations and overexpression of β-catenin are associated with many cancers, including hepatocellular carcinoma, colorectal carcinoma, lun ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Turcot Syndrome
Mismatch repair cancer syndrome (MMRCS) is a cancer syndrome associated with biallelic DNA mismatch repair mutations. It is also known as Turcot syndrome (after Jacques Turcot, who described the condition in 1959) and by several other names. In MMRCS, neoplasia typically occurs in both the gut and the central nervous system (CNS). In the large intestine, multiple colonic polyps develop; in the CNS, brain tumors. Genetics Under the name constitutional mismatch repair-deficiency, (CMMR-D), it has been mapped to MLH1, MSH2, MSH6 or PMS2. Monoallelic mutations of these genes are observed in the condition known as Lynch syndrome or hereditary nonpolyposis colorectal cancer, while biallelic mutations are observed in CMMR-D. People expressing the HNPCC (which itself is considered autosomal dominant) trait are considered carriers of CMMR-D, thus CMMR-D is classified as autosomal recessive. The term "childhood cancer syndrome" has also been proposed. Café-au-lait macules have been obser ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Gorlin Syndrome
Gorlin may refer to: People *Dan Gorlin, computer game programmer, designer and founder of Dan Gorlin Productions *Eitan Gorlin, filmmaker, author and actor *Mikhail Gorlin, Russian emigre poet *Richard Gorlin, American cardiologist, co-developed the Gorlin equation * Robert J. Gorlin, a professor and researcher at the University of Minnesota In medicine *Gorlin sign, the ability to touch the tip of the nose with the tongue and touch the elbow with the tongue *Gorlin syndrome Gorlin may refer to: People *Dan Gorlin, computer game programmer, designer and founder of Dan Gorlin Productions *Eitan Gorlin, filmmaker, author and actor *Mikhail Gorlin, Russian emigre poet *Richard Gorlin, American cardiologist, co-developed ..., also known as basal cell nevus syndrome *The Gorlin equation, a method to calculate the effective area of a heart valve during cardiac catheterization {{disambig ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Sonic Hedgehog (protein)
Sonic hedgehog protein (SHH) is encoded for by the ''SHH'' gene. The protein is named after the character ''Sonic the Hedgehog''. This signaling molecule is key in regulating embryonic morphogenesis in all animals. SHH controls organogenesis and the organization of the central nervous system, limbs, digits and many other parts of the body. Sonic hedgehog is a morphogen that patterns the developing embryo using a concentration gradient characterized by the French flag model. This model has a non-uniform distribution of SHH molecules which governs different cell fates according to concentration. Mutations in this gene can cause holoprosencephaly, a failure of splitting in the cerebral hemispheres, as demonstrated in an experiment using SHH knock-out mice in which the forebrain midline failed to develop and instead only a single fused telencephalic vesicle resulted. Sonic hedgehog still plays a role in differentiation, proliferation, and maintenance of adult tissues. Abnormal act ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Brainstem
The brainstem (or brain stem) is the posterior stalk-like part of the brain that connects the cerebrum with the spinal cord. In the human brain the brainstem is composed of the midbrain, the pons, and the medulla oblongata. The midbrain is continuous with the thalamus of the diencephalon through the tentorial notch, and sometimes the diencephalon is included in the brainstem. The brainstem is very small, making up around only 2.6 percent of the brain's total weight. It has the critical roles of regulating cardiac, and respiratory function, helping to control heart rate and breathing rate. It also provides the main motor and sensory nerve supply to the face and neck via the cranial nerves. Ten pairs of cranial nerves come from the brainstem. Other roles include the regulation of the central nervous system and the body's sleep cycle. It is also of prime importance in the conveyance of motor and sensory pathways from the rest of the brain to the body, and from the body back to t ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Decerebrate
Decerebration is the elimination of cerebral brain function in an animal by removing the cerebrum, cutting across the brain stem, or severing certain arteries in the brain stem. As a result, the animal loses certain reflexes that are integrated in different parts of the brain. Furthermore, the reflexes which are functional will be hyperreactive (and therefore very accentuated) due to the removal of inhibiting higher- brain centers (e.g. the facilitatory area of the reticular formation will not receive regulating input from cerebellum, basal ganglia and the cortex). Methods Decerebration describes the ligation along the neural axis in distinct parts of the brain in experimental animals. Generally: * lower decerebration (the cut is made above the upper border of the pons) * middle decerebration (cut is made through the red nucleus) * upper decerebration (cut is made so the cortical area is removed) Lower decerebration results in a "bulbospinal" animal: reflexes which are integ ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Pathologic Nystagmus
Nystagmus is a condition of involuntary (or voluntary, in some cases) eye movement. Infants can be born with it but more commonly acquire it in infancy or later in life. In many cases it may result in reduced or limited vision. Due to the involuntary movement of the eye, it has been called "dancing eyes". In normal eyesight, while the head rotates about an axis, distant visual images are sustained by rotating eyes in the opposite direction of the respective axis. The semicircular canals in the vestibule of the ear sense angular acceleration, and send signals to the nuclei for eye movement in the brain. From here, a signal is relayed to the extraocular muscles to allow one's gaze to fix on an object as the head moves. Nystagmus occurs when the semicircular canals are stimulated (e.g., by means of the caloric test, or by disease) while the head is stationary. The direction of ocular movement is related to the semicircular canal that is being stimulated. There are two key forms of ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
