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87 Cards in this Set
- Front
- Back
Two theories of dysraphic disorders?
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failure of neural tube closure due to primary failure of neuroectoderm or mesoderm OR repoening or rupture of previously closed tube
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What kinds of prescribed drugs can cause neural tube defects?
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Valproic acid
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Two genetic disorders associated with neural tube defects?
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Patau's and Edwards
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Often asymptomatic; may be associated with foot abnormalities and gait. What is the lesion?
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Spina bifida occulta- non-closed vertebral arches with overlying skin, most often lumbosacral and lest severe NT defect
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What is a meningocele?
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dura and leptomeninges hernate through vertebral defect but skin is still covering it.
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Myelomeningocele?
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meninges and spinal cord herniate through defect. If no skin covering, is myeloschisis which is what usually occurs
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SC defect with no elecated AFP?
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spina bifida occulta
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Anencephaly more common in ?
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female fetuses
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What is area cerebrovasculosa?
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the mass of malformed neural tissue present in the cranial vault seen leftover in anencephaly
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absent calvaria, baby has rachishisis. What else does the baby have and what is noted during pregnancy?
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anenchephaly: elevated AFP and polyhydramnios
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What is encephalocele?
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When a mass of cerebral tissue protrudes through a cranial defect, usaully occipital, confined to one hemisphere, and covered
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What is a nasal glioma?
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When encephalocele is misnamed because of its projection into ethmoid sinus, orbit, or nasal cavity.
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Disorders of migration of forebrain include?
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sulcation, cleavage, or genesis
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What is sulcation induced by?
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presence of normally migrated neuronal population
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Smooth brain with many small bumps. Etiology?
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this is polymicrogyria, caused by intrauterine ischemia/infection which interferes with normal gyral development.
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Miller Dieker syndrome?
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Triad: seizures, mental retardation, lissencephaly (Lisa is a miller dieker)
LIS1 gene on chromosome 17 involvement |
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Arrest of neuroblast migration at end of 4th fetal month causes?
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lissencephaly or pachygyria
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nodular gray matter heterotopias in ventricular wall, what genetic defect?
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LIS1 on Ch 17, this is found in agyria
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mnemonic for agyria?
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lisa was Ch17 when she discovered miller-DIECKEr
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Cyclopia and hypotelorism, what malformation is suspected?
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Arhinencephaly
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Disturbance that results in arhinencephaly?
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formation of cranial end of neural tube with lack of formation of olfactory buds and cleavage during 4-6 week!
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alobar holoprosencephay?
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no hemispheric fissure
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lobar holoprosencephaly?
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hemispheres joined at frontal pole
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semilobar holoprosencephaly?
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shallow interhemispheric fissure
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Sx of corpus callosum agenesis?
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no symptoms except perceptual and language tests
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X-linked, lethal in males, bat-wing lateral ventricles?
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Aicardi syndrome: agenesiss of crpus callosum, chorioretinal defects, and seizures
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Most common malformation of posterior fossa?
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Arnold Chiari Type 1, usually asymptomatic chronic tonsillar herniation
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Z-shaped sagittal brainstem/spinal cord section. What clinical disorder exists?
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true arnold chiari type 2, with lumbar myelominingocele, syringomyelia, and hydrocephalus
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Dandy-walker malformation?
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DWalker is an ACE:
Agenesis of vermis, Cystic dilation of fourth ventricle Enlargement of posterior fossa, hydrocephalus also frequently present. |
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Static motor defecit due to prenatal and perinatal insults?
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Cerebral palsy, due to ischemia/hemorrhage; classified according to motor impairment
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Chalky yellow plaques around lateral ventricles? due to ischemia?
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periventricular leukomalacia,WHITE MATTER hypoxia/ischemia
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What is multicystic encephalopathy?
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due to 3rd trimester ischemic insult, it is where white matter and deep cortical layers are replaced by sponge-like glial lined cysts
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Perinatal cerebral hemorrhage, originates where?
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this would be a subependymal germinal matrix hemorrhage that originates between caudate and thalamus where the grminal layer is
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Why is germinal matrix most likely to bleed?
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it has a rich capillary bed not supported well by capillary tissue.
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Grading of sybependymal hemorrhaged based upon?
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confinement of bleed and degree of ventricular dilatation
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Factors affecting degree of damage caused by ischemia?
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collateral cirulcation, duration , and severity and rapidity of reduced flow
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Most vulnerable region in transient global ischemia?
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HIPPOcampus, Cerebral neocortex (lamina 3,4,5), and verebellum (purkinje cells)
HCC |
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Most vulnerable cells during transient ischemia?
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Neurons, then their helpers, oligodendrocytes, then astrocytes
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Acute phase of permanent global ischemia results in?
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coma
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Diffuse cortical injury indicated by?
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FLAT EEG
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brainstem damage indicated by?
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absent reflexes, respiratory drive, and cerebrla perfusion
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What does respirator brain look like?
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swollen with flattened gyri often herniated with slitlike ventricles
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Factors affecting degree of damage caused by ischemia?
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collateral cirulcation, duration , and severity and rapidity of reduced flow
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Most common site of thrombosis?
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carotid bifurcation, MCA origin, ends of basilar artery
MCA Basilar, Carotid |
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Most common vessel affectd by emboli?
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MCA, which is bad because it is not part of the circle of willis
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Most vulnerable region in transient global ischemia?
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HIPPOcampus, Cerebral neocortex (lamina 3,4,5), and verebellum (purkinje cells)
HCC |
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hemorrhagic infarct most likely due to?
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embolism because fibrinolytic events after the occlusion = hemorrhage
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Most vulnerable cells during transient ischemia?
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Neurons, then their helpers, oligodendrocytes, then astrocytes
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Factors affecting degree of damage caused by ischemia?
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collateral cirulcation, duration , and severity and rapidity of reduced flow
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Acute phase of permanent global ischemia results in?
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coma
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Most vulnerable cells during transient ischemia?
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Neurons, then their helpers, oligodendrocytes, then astrocytes
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Acute phase of permanent global ischemia results in?
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coma
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What does respirator brain look like?
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swollen with flattened gyri often herniated with slitlike ventricles
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Diffuse cortical injury indicated by?
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FLAT EEG
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brainstem damage indicated by?
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absent reflexes, respiratory drive, and cerebrla perfusion
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What are two microvasculopathy's associated with demtnia?
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Binswanger's disease and CADASIL
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What does respirator brain look like?
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swollen with flattened gyri often herniated with slitlike ventricles
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What is CADASIL?
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most common hereditary stroke disorder: mutation of NOTCH 3 on chromosome 19.
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Most common site of thrombosis?
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carotid bifurcation, MCA origin, ends of basilar artery
MCA Basilar, Carotid |
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Most common vessel affectd by emboli?
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MCA, which is bad because it is not part of the circle of willis
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What causes binswanger's?
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subcortical infarcts caused by hypertension and resulting dementia due to white matter distruction.
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hemorrhagic infarct most likely due to?
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embolism because fibrinolytic events after the occlusion = hemorrhage
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PAthogenesis of notch 3 mutation?
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causes intracytoplasmic accumulation in smooth muscle cells in blood vessels which cause infarcts and dementia in CADASIL
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Incomplete infarct causes what cells to die?
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neurons, they are first before oligos and astros
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most common area of cerebral venous thrombosis?
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superior sagittal and lateral sinuses
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most common cause of primary intraparenchymal hemorrhage?
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hypertension
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aneurysms associated with HTN?
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charcot bouchard (they are microaneurysms, not saccular like berry)
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Why does htn cause hemorrhage?
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hyaline arteriolosclerosis weakens vessels and cause aneuryms that can go BOOM
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Most common vascular malformation?
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AV malformation, more common in men 10-30 yrs
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most common AV malformation where?
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MCA (seems like all path goes here)
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Difference between AV malformation an cavernous angioma?
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AV has intervening brain tissue wherase canvernous has no tissue and usually from prior hemorrhage
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What can cause a lobarhemorrhage?
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amyloid angiopathies that weaken small/medium arterioles
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Subarachnoid hemorrhages due to?
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rupture of congenital berry aneurysms (80%)
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Most berry aneurysms located where?
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between ACA and anterior communication branch of circle of willis
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Where are fusiform aneurysms usually found and alongside what disease process?
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Basilar artery, usually HTN artherosclerosis
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lacurnar infarcts due to?
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hyaline arteriosclerosis due to HTN and diabetes.
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RElationship between lacunar infarcts and Binswanger?
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If enough white cortical matter is damaged by lacunar infarcts, one can see dementia! It is the dementia due to these infarcts that is termed Binswanger
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Optic neuritis in a young female. What is elevated in CSF?
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MS, she has protein with IgG in CSF
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Multifocal, hyperintsne plaques seen in MRI with bladder dysfunction and CN signs. What HLA type and hypersensitivity?
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HLA-DR2, Type 4 which is cellular mediated
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PAthogenesis of MS?
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cd4_ t cells react against myelin and secrete cytokines that activate macrophages to nom nom nom
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An acute version of MS?
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monophasic demyelinating disease activated by viral illness or immunization
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Demylination common in alcoholics that undergo too rapid correction of hyponatremia?
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Central Pontine Myelinosis
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Cause of PML?
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JC virus infection of oligodendrocytes, casuing rapdily progressing demyelination in HIV pts.
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Inclusions in PML
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intraNUCLEAR in oligodendrocytes.
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Central pontine myelinolysis area affected?
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upper 2/3 of pons
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What is central pontine myelinolysis?
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demylination of pons nerve cells due to too rapid correction of profound hyponatremia
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What are the leukodystrophies?
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inherited defects of metabolism that affect myelin synthesis or turnover. NO neuronal storage defects. white matter only.
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