Central Nervous System II
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Created by:
bustagrill on April 22, 2012
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43 terms
Terms | Definitions |
|---|---|
 Leukodystrophies | errors in metabolism storage diseases- break down subtances you can't get rid of degeneration of white matter |
| Metachromatic Leukodystrophy** | Most Common- usually in infancy, notice when they miss milestones Inactive aryl sulfate ARSA or PSAP lead to accumulation of sulfatid-rich lipids |
| Krabbe Disease | (Leukodystrophy) Globoid cell, accumulation of fatty acids Rare Lack of galactocerebroside- B-galactosidase |
| Adrenoleukodystrophy | metabolic error in lipid metabolism, build up of long chain fatty acids |
| 3 types of Adrenoleukodystrophy | Child-cerebral Andrenomyeoneuropath ADDISONS Disease- salt balance distrupted |
| Alexander's disease | (Leukodystrophy) Disease of astrocytes (GFAP) lakc of myelin formation in white matter Rosenthal fibers Megalencephaly |
| What is the most common Immunologic disease of the CNS and leading neurological disease in Young Adults | MS |
| MS | Autoimmune disease, demyelinating, AND Neurodegentive disease Occurs in 2nd to 4th decades of life |
| What histocompatibility antigens are found in affect peeps with MS | Myelin basic protein IgG in CSF |
| Diagnosis criteria for MS | more than one attack IgG in CSF brain lesions |
| 3 types of MS | Relapsing and remitting** Secondary Progressive- from RR to CONSTANT Primary Progressive- steady slow worsening |
| How is visual pathway affected with MS | Optic Neuritis Optic nerve is first site of predilection Field loss from loss of NFL in retina |
| What is the hallmark of MS | Plaque- due to loss of myelin and scarring Acquire astrocytes and becomes dense with gliosis |
| What percent of tumors are primary intracranial tumors | 50% |
| They highest percentage of primary intracranial tumors are a form of | Glioma |
| The other 50% of tumors are | due to metastasize from other parts of the body |
| 2 tumor cellular derivatives of the Nueral Tube (neuroectoderm) | Predominantly Glial Tumors (gliomas) Medulloblastoma** |
| 3 types of Pred. Gliomas | Astrocytomas (80%) Oligodendroglimas (10%) Ependymal- of 4th ventrical (10%) |
| 3 Cell bioliges of Gliomas- all of which are p53 mutations | Low Grade Astrocytoma- can be removed, loss of alleles from chromosome 17 Anaplastic Astrocytoma- loss of alleles from chrom 17 & 19 Glioblastoma- non-removable, most oncogenic deficit. Lss of alleles from 17, 19, & 10 |
| What is the most common intracranial neuroblastic tumor | Medulloblastoma |
| Medulloblastoma | arises in cerebelum highest freq in first decade spreads in CSF |
| 2 tumor cellular derivative of the Neural Crest (mesenchymal) | Meningiomas-20% Schwannomas- VIII acoustic neuromas |
| Ectopic issues | tissue left in places hwere it shouldn't be during embryonic develop. (cysta, lipomas) |
| Colloid Cysts | Vestigial embryonic structures in the 3rd ventricle grows slowly over decades |
| Common sources of metastases from cancer at other sites | lung and breast cancer, and melanomas |
| Lysosomal Storage Diseases (6) | Tay-Sach's Neimann-Pick Gaucher Metachromic leukodystrophy Fabry's Krabb's |
| Tay-Sachs | deficiency of enzyme hexoaminidase A or B, accumulation of ganglioside cherry spot in retina |
| Neimann-Pick disease | storage of sphingomyelin results from deficiency of sphincomyelinase |
| All the Lysosomal storage and MPS syndromes Autosomal recessive except | Hunter's - x-linked |
| 5 Mucopolysaccharides (MPS synromes) | Hurler's Hunter's Sanfilippo's Morquio's VII |
| Hurlers Syndrome | deficiency of a-L-iduronidase, leads to accumulation of mucopolysaccharides in lysosomes Gargoylism |
| 2 Thiamine (Vit B1) deficiencies | Associated with alcholism Wernicke's Syndrome Korsakoff Syndrome |
| Wernicke's | disturbed eye movements, gait, mental function |
| Korsakoff | lesions in hypothal and mammillary bodies, periaqueducta regions of midbrain and tegmentum of pons Amnesia, poor temp regulation REVERSIBLE |
| Vit. B12 deficiency | Megaloblastic anemia Uncoordinated movement, spinal cord disease |
| Nicotinic Acid Deficiency- 3 D's | Dermatitis Diarrhea Delirium |
| 3 Neurodegenerative Disease of CNS | Parkinson's Alzheimer's Huntington's |
| Parkinsons' | Extrapyramidal motor system affected Loss of dopamine Lewy bodies- dementia Shy Drager syndrome Tremor 30% |
| Most common TX of parkinsons | L-DOPA, death may occur by body wasting |
| Alzheimer's | neurofibrillary tangle in cortical neurons senile amyoid plaque Hirano Bodies and possible prions |
| Atrophy of Alzheimer's is located where | bilateral and symmectrical, frontal cortex and hippocampus |
| Pick's disease | course like azheimer's but selective atrophy of anterior and frontal lobes no plaques and tangles= Pick bodies in neurons |
| Huntington's Disease (Chorea) | movement disorder affects basal ganglia loss of neurons in corpus striatum leads to loss of motor control |
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