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309 Cards in this Set
- Front
- Back
What genetic mutations are associatd with Alzheimers disease?
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episolon four of ApoE4, Presnilin 1 and 2, and Amyloid Precursor Protein
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Which mutations in Alzhemiers are thought to be associated to early onset disease?
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Presnilin and APP mutations
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Which mutation in Alzehmeirs is thought to be associated to late onset disease?
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Epsilon four (APO E4)
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Which mutation in apolipoprotein E is thought to be protective from alzhemiers?
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APO E2
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Whats the gross pathology of Alzhiemers disease, what lobes are affected and spared?
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Atrophy of temporal lobe (Frontal and Parietal), dilateion of ventricles (2ndary), and spared occipital lobe
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What chromosome is Amyloid precursor protein, and whats the significance?
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21, Alzeimers and Down syndrome are linked (by age 40 All downs ppl have it)
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What are the two Most important histology fndings of Alzhemiers?
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Senile plaques and Neurofibrillary Tangles
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Which major histological finding in alzehmiers is within the nueron?
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Tangles
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Which major histological findings in alzehmiers are outside the nueron?
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Senile Plaques
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Whats the substance found in the plaques of alzehmiers?
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Beta Amyloid
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Other than plaques and tangles, what are the 3 other "soft" findings of alzehimers?
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Hirano bodies of Hippocampus, Granulovacuolar degeneration of hippocampus, and Amyloid angiopathy
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What substance makes up the Neurofibrilary tangles of Alzehimers?
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Hyperphosphorylated Tau protein that make paired helical filaments
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What is a pyramidal neuron?
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Many dendrites, one axon
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Where are Tangles of Alzheimers most easily seen and with what stain?
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In large pyramidal neurons and Silver Stain
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Which pathologic feature of Alzhemiers correlates more to the stage of dementia, Tangles or plaques?
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Tangles
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Which pathologic feature of Alzhemiers is thought to be the primary event?
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Beta Amyloid Plaques (thought to be neurotoxic)
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What are Hirano bodies in Alzhemeris?
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Intracellular eisonophillic accumulations
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What happens in the Basal Nucleus of Meynert in Alzhemiers?
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Decrease of neurons and hence ach to cortex
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Whats the main cliniclla feature of Alzhemiers?
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Slow progressive decline of intellecutal detioration during several years
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Whats the most frequent early sign of Alzhemiers?
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Loss of recent memory ( also smell -enterohinal cortex)
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What is the most common cause of dementia?
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Alzhemiers
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Whats the most common kind of Alzhemiers, early or late sporadic?
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Late sporadic
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How many percent of people by age 95 have Alzhemiers?
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50%- that’s why we focus on Presenile forms
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Whats the main metabolic pathway that’s responsible for Alzhemiers?
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GSK-3Beta phosphorylates Beta Amyloid, which in turn activates GSK-3B
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What is the main pathogenesis behind neuronal apoptosis and dysfunction in Alzhemiers?
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Phospho-Beta amyloid accumulation
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How is WNT involved in Alzhemiers?
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Normal inhibits GSK-B (if dysfunctional-Alzhemiers)
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How does APP form Beta amyloid?
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Beta-secretases followed by gamma secretases turn APP to beta-amyloid
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How does insulin resistance lead to Alzehimers?
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Increased insulin= less insulin degrading enzyme, which normally clears Beta Amyloid
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Whats the normal role of Tau protein?
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Maintain Microtubules
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What happens to tau in Alzhemirs, and how?
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Hyperphosphorylated by GSK
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What does hyperphosphorylated Tau do?
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Forms tangles which cause death.
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What is Prolyl isomerase, and how is it related to Alzhemiers?
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Strips phosphates off of Tau (can be messed up in Alzheimers)
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What is it called when (like in Alzhemiers) lobes are destroyed and ventricles dilate?
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Hydrocephalus ex vacuo
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Are NF tangles pathognmoic for Alzhemiers?
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No, other disorders like huntingtons and Neiman picck have them
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What is the consequence of Amyloid Angiopathy in Alzhemeirs?
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Weaked walls, increased hemorrhage ( no different than HTN)
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How is the diagnosis of Alzhemiers made and confirmed?
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Only confirmed at autopsy, Clinically- rule out all other causes of Dementia
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How can I diagnosise Alzhemiers in the hospital?
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Oritentation, Attention, Verbal recall, language, and spatial skills (minimental)
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What are the treatment options for Alzhemiers?
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ACH esterase inhibitors, and Memantine
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What is memantine?
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MEMan(memory) -- hence it prevents death or neurons. What kills nurons? Glutamate- so it’s a glutamate antag
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What is Pick's Disease?
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The "Other" Form of Dementia - Frontotemporal dementia with parkinsonian aspects and aphasia. Also there are Pick bodies which are intracellular aggregated Tau protein
Its a tau-pathy |
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What is Pick's Disease characterized by?
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Frontotemporal atrophy, with sparing of other lobes (abrupt sparing)
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What is the histology of Pick's Disease?
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Pick bodies- tau protein intracellular accumulations that are round (also fibrilary)
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What are the other to Tauopathies other than Alzhemiers?
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Pick's disease, and Corticobasilar degeneration, Progressive supranuclear Palsy
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What is the only dementia that has a motor component?
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Corticobasilar Degeneration (cortico and basilar) (s. nigra affected but no lewy bodies), Lewy body dementia
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Whats the motor component of Corticobasilar degeneration?
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Akinetic Rigidity (like Parkinsons)
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What can Corticobasilar degeneration be mistake for?
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Parkinsons' because ther is also s. nigra loss of pigment (no lewy bodies though)
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Other than parinaud syndrome, what is another syndrome in which verticle gaze is affected?
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Progressive supranuclear Palsy
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Unlike Parinaud syndrome which affects sup colliculi, what does progressive supranuclear palsy affect?
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Basal ganglia strucutres, and cerebellum pons communications
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What symptoms other than loss of verticle saccades does Progressive supranuclear palsy have?
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Parkinsonian and dementia
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Whats the inheritance of Huntingtons?
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Autosomal Dominant 4 CAG repeats (anticipating)
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What kind of disease is Huntingtons primarily?
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Movement and basal ganglia, can progress to Dementia
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Normally head of caudate bulges out into ventricles. What happens in Huntintons?
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Atrophy of caudate, so it becomes concave
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Whats the main structure grossly affected in huntingtons?
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Cuadate and striatum
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What are the accumulations in Huntingtons?
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Nice try- there are no accumulations
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When is the onset of huntingtons?
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30-40
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What type of disesase are the cerebellar ataxias?
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Triplet repeats, cause ataxia, show anticipation and autosomal dominant
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What causes Cretuzfeldt-Jakob disease?
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Prions- PrpScrapie
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What are risk factor for developin Cretuzfeldt-Jakob disease?
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Unclean electrodes, corneal transplant, eating brains, (most are sporadic)
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How can familial CJD be inherited ?
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Auto-dominantly. If your father was a brain eater, you will be too.
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What is the pathology of CJD?
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spongiform encephalopathy- bubbles and holes
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What are the clinical features of CJD?
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Rapid progressing disease (months) with dementia and myoclonus
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What is a prion disease that lay people know about?
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Mad Cow disease Bovine spongiform encephalopathy
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What is the main pathology of Parkinsons?
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Alpha-synnuclien Lewy bodies and loss of pigment in S. nigra pars compact
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What is the main presentation of parkinsons diseases?
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TRAP Voluntary muscle movements are off (Tremor, Rigidity, Akineseia, Postural imbalance)
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What are non-idiopathic causes of parkinsons?
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MPTP, Wilson's disease, encephalitis, trauma (boxers's parkinsons)
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What motor disorder do Boxers often get?
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parkinsons
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What hair condition is associated to parkinsons?
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Subherric Deramtitis Dandruff - M. Furfur
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What drugs can treat parkinsons?
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Levodopa, Selegline- maob inhibitor
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What percentage of Parkinsonian people go to dementia?
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20-40 percent
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When does Parkinsons present?
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45-65
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What is parkinsons and dementia together often a symptom of?
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Lewy Body dementia
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What is the primary probkem in Dementia with lewy bodies?
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Dementia >Parkinsons
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Whats the second most common cause for dementia (non-infarct)?
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Lewy Body dementia (actually infarct is second most common)
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What gene is Friedrich's ataxia and what is inheritiance?
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GAA repeats Auto recessive of Frataxin
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Most common cause of death in Friedrich's ataxia?
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Hypertrophic cardiomyopathy
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What is the frataxin gene involved in, and what do mutated frataxin cells do?
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Mitochondria (they thend to apoptosize)
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What neuronal structures are most affected by fredreich's ataxia?
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Spinal cord (lateral corticospinal), Spinocerebellar (ataxia), and DCML and DRGs
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How is spinothalamic tract in Fredreich's ataxia?
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normal
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What does MRI of Freidrichs ataxia patients show?
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Spinal atrophy
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What metabolic disoder is often seen with Freidreich's ataxia?
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Diabetes Type I
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What foot pathologies are seen with Friedrich's ataxia?
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Pes Cavus and Hammertoes
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What is amyotrophic Lateral sclerosis?
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UMN and LMNS are destroyed
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What is ALS caused by?
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Defective Superoxide Dismutase 1 and Betel nut ingestion
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What is often the first LMN sign in Lou Gehrigs disease?
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Loss of intrinsic hand muscles
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What parts of the spinal cord are affected in ALS?
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Lateral tracts and anterior horns
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Whats the prognsosi of ALS?
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Death in 3-5 yrs due to respiratory failure
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Whats the treatment for ALS?
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Riluzole
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Whats the pathology of ALS?
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Bunina Bodies in residual motor neurons (contain cystatin C)
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What are the upper motor neuron signs of ALS?
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Babinksi and spasticity
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What are the lower motor nueron signs of ALS?
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Loss of intrinsic hand muscles
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How does Riluzole work in ALS/
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Glutamate antag (like memantine)
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How is ALS diagnosed?
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Nerve conduction studies
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What do people die from in ALS?
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Respiratory failure
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If you have a motor neuron disease, and there are sensory changes what can be ruled out?
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ALS, Werndig Hoffman, Polio
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True or False. ALS leads to bladder and bowel incontinence
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False no
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What is spinal muscluar atrophy?
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Lower motor nueron failure in infants (werndig-hoffman)
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How is Werndig Hoffman inherited?
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Auto-recessive
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What does Werndig Hoffman present as?
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Floppy baby, tongue fasiculations, respiratory failure and early death
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What is Wilson's Disease?
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Abnormal metabolism of Copper due to defective excretion in bile
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What are the signs of Wilsons's disease?
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Keyesr-Flesher ring, low ceruloplasmin, high copper in urine
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What are the non-brain problems of wilson's disease?
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Liver cirrhosis, HCC, Corneal deposits,
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How is Wilson's disease treated?
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Penicillamine Copper chelator
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What are the neural problems in Wilson's?
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Parkinsonian, Demntia, and Chorea
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What area of CNS is affected by Wilsons mostly?
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Basal Ganglia (globus pallidus, and putamen = lenticular nucelsu)
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What is the gross pathology of Wilsons disease in the brain?
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Cystic cavitation of putamen and basal ganglia in general
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Whats the pneumonic for Wilson disease?
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ABCD A-asterixis B- Basal Ganglia C- Cirrhosis and Carcinoma D-Dementia (keyers-fleisher)
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What is wernicke-korsakoff syndrome?
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Wernike encephalopathy progresses to Korsakoff psychosis
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What causes wernicke's encephalopathy?
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Alcoholic Thiamine Dieficiency
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What are the triad of symptoms in Wernicke's ?
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Ataxia, Opthalmoplegia, and Confusion
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Are the symptoms of Wernicke's encephalopathy reversible?
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Yes Treat with B1- Thiamine
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What gross changes are present in Wernicke's encephalopathy?
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Hemorrhage into mamillary bodies, and walls of ventricles- reactive gliosis too
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What structure of the brain must be remembered with Wernicke's ?
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MAMILLARY BODIES
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What is a demylinating disease which involves destruction of myelin in CNS?
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Multiple Sclerosis
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What is a demylinating non-viral disease which involves destruction of oligodendrocytes in CNS?
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Multiple Sclerosis
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What is a demylinating viral disease which kills oligodendrocytes?
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JC virus -progressive multifocal leukoencephalopathy
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What is HLA-Dr2 related to?
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Lupus, Multiple sclerosis, Good Pastures, and Hay Fever
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What type of Hypersensivity is MS?
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Type 2 and Type 4 (Abs are also invovled)
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What do T Cells recognize as an antigen in MS?
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Mylein basic Protein (CNS)
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Does MS affect peripheral myelin?
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no
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Whats the prominent finding on Pathology for MS?
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Demylinating Plaques (usually periventricular)
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The clinical course of MS is a. relapsing and remitting b. Progressive and quick
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Relapsing and remitting
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What is the most common cause of optic nueritis?
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Multiple Sclerosis
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What physical exam sign is pathgnomic for Multiple Sclerosis in a young woman?
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Bilateral INO
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What are the main categories of problems in MS?
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Motor (Weakness and UMN), Eyes (MLF and nueritis of optic nerve), Sensory, Cerebellar, Autnomics (MESCA-line)
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In what disease does flexion of the neck produce electrical sensations down the spine?
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MS
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What is Charcot's Triad for MS?
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Scanning speech, Incontince, intention tremor, Intranuclearopthalmoplegia, Nueritis =SIN
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What are the lab findings in MS?
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Oligoclonal bands, increased IGG, increased white cells
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What is the gold standard for diagnosis of MS?
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MRI
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What is the mainstay of treatment for MS?
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Interferon B
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What does rapid correction of hyponatremia cause?
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Central Pontine Myelinolysis
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What is Central Pontine myelinolysis?
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Loss of myelin in central pons = duh
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What is the course of PML?
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Rapid progressive and Fatal
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What kind of nerves are affected in guillan barre?
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Peripheral nerves mostly
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What illness is a demylinating disease of the peripheral nerves?
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Guiallan barre syndrome
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What is thought to be the cause of Guillan Barre?
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molecular mimicry. Post -campylobacter infection, or allergic
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How is the weakness of Guillan Barre characterized?
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Ascending weakness and paralyiss- starting low and going up. "We can catch u will u fall= put em on a respirator"
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Whats the most important lab finding in guillan barre?
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Albumino-cytologic dissociation. Greatly increased protein, no increase in cells
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Paralysis of ____ muscles is found in half of the cases of Guiallan Barre?
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Facial muscles!
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True or False. Autonomic function is spared in Guillan Barre syndrome?
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False- Autonomics are affected (hypo or hypertension , and cardiac abnormalities) Peripheral nerves carry autonomics you fool
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True or False. Guillan Barre has a very grave prognosis.
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False. Just put them on respiratory support and plasmapharessis
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What are all the demylinating diseases?
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Central: MS, PML, ADEM, and Peripheral: Guiallan Barre and Inherited
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What is Acute disseminated Encephalomyelitis also known as?
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Post-infecitous encepholomyeltis
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What specific areas are demylinated in postinfecitous encephalomyelitis?
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Peri-venular
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What does Acute disseminated Encephalomyelitis often follow?
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Measles, Chickenpox or certain vaccinations
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What is one of the dangers of getting Chickenpox at a later age?
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increased ADEM
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What are the three main inherited problems with myelin and unique feature with all?
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Metachromatic leukodystrophy, adrenoleukodystrophy, and Krabbe's disease optic atrophy
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Which two hereditary myelin problems are in the same biochemical pathway?
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Metachromatic leukodystrophy and Krabbe's disease
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What enzyme is deficient in Metachromatic LeukoDystrophy?
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Aryl Sulfatase A
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What enzyme is deficient in Krabbes Disease?
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Galactocerbrosidase
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Which of the three inherited myelin disease is X-linked?
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Adrenoleukodystrophy
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What is the main problem in metachromatic luekodystrophy?
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Central and Peripheral demylination
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How does infantile Metachromatic leukodystrophy tend to present?
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Infantile form- regression of developmental milestones and retardation
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How does Adult and juvenile metachromatic leukodystrophy present?
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Ataxia, Optic atrophy, behavioral distrubances, and dementia
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Whats the prognosis for infantile and juvenile MLDystrohpy?
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Death by age 10
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What are the Sx for Adult Metachromatic leukodystrophy?
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Optic atrophy, Dementia, Ataxia,
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What is Krabbe's disease?
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Loss of Beta galactocerebrosidase
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What does Krabbe's disease present as?
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baby losing milestones, optic atrophy, peripheral neuopathy- early death
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What is the characteristic histologic feature of Krabbes Disease?
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Globoid cells- macrophages fat with lysosomal galactocerebroside
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What is Adrenoluekodystrophy characterized by?
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Addisons disease plus demylination
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What is the defect in adrenoluekodystrophy?
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Vlong chain Fatty acids accumulate
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Whats worse Krabbes or metachromatic leukodystrophy?
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Krabbes - earlier death
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What diseases are characterized by hereditary motor and sensory neurophathies wih defective productive of proteins invovled with peripheral nerve structure, function, or myelin
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Charcot-Marie-Tooth Disease
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Whats the classic sign of Charcot marie tooth disease?
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Inverted bottle appearance of legs and hands (muscle atrophy due to deinnervation
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How is Charcot-Marie-Tooth disease inherited?
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Autosomal dominantly
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What is the inheritance of Acute intermittant porphyria?
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Autosomal dominant
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What is the deficiency in Acute intermittant porphyria?
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Porphobilinogen Deaminase
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What two substrates increase in AIP?
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Porphobilinogen and Aminolevilunic acid
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What is the classic sign for Acute intermittant porphyria?
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Window-sill test. Leave urine out, it will change color(first is colorless) port wine color
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What is the effect of decreasing heme on AIP symptoms?
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Heme feedbacks on ALA synthase - so more porphobilinogen is made- WORSE symptoms
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What are the symptoms of Acute intermittant porphyria/
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Dementia/Psychosis/Neuropathy, and abouts of severe pain
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What does the belly full of scars sign point to?
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Acute Intermittant porphyroa- don’t mistake for surgical scars
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What drug is contraindicated in porphyria?
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Barbiturates
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What is a high frequency neuronal firing that is synchronnized?
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seizure
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What is a disorder of afebrile seizures?
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Epilepsy
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True of False. Epileptic Seizures can be febrile.
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False- no Infectious seizures are febrile
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What is a partial seizure?
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Characterized by one part of the brain seizing- medial temporal lobe usually
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What part of the brain do partial seizures usually begin in?
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medial temporal lobe
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True or False. All seizures come without any warning.
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False- some seizures have an aura beforehand
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What is the difference between complex and simple partial seizures?
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Complex- lose conciousness
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What are generalized seizures?
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Diffuse through brain
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True or False? Partial seizures can progress to generalized seizures?
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Yes
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What does clonic mean?
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Movement
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What does tonic mean?
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Stiffening
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What seizure is quick, repeteive jerks?
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Myoclonic
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What seizure is a drop seizure- drop to floor?
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Atonic
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What type of seizure is a grand mal seizure?
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Tonic-clonic
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What is vertigo?
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Illusion of movement or spinning
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What are the two types of vertigo?
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Peripheral and central
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Which type of vertigo is more common?
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Peripheral
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Which type of vertigo is characterized by a delayed horizontal nystagmus on positional testing?
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Peripheral
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What vertigo is characterized by immediate nystagmus that may swtich directions?
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Central
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What are three causes for peripheral nystagmus?
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Menetrier's disease, debris, vestibular nerve infection
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What does central vertigo indicate?
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Brainstem or cerebellar lesion
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True or False. A headache is pain due to irritation of brain paraenchyma.
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False. Dura, cranial nerves, or extracranial structures are affected
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True or false. A migraine headache is unilateral.
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Yes
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True or false. A migraine headache is not associated with photophobia, nausea, or phonophobia.
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False. Migraines are associated with photobia, nause, and phonophobia
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True or false. Migraines are associated with ipsilateral lacrimation, rhinorhea, and and periorbital pain.
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False. These are cluster headaches, which also have horner's in addition
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Cluster headaches are associated with long or brief headaches? Are they unilateral or bilateral?
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Unilateral brief headaches that are repeptetive
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True or false. Cluster headaches are commonly in females.
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False. They are in males
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What is the only type of bilateral headache?
|
Tension headache.
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How long do bilateral headaches last?
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Greater than 30 minutes- tension headache
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True or False. Tension headaches are not associated with photophobia or phonophobia.
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True they are not. Only migraines are
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What does irritation of cranila nerve 5, and the release of Vasoactive peptide, and CGRP lead to?
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Migraines
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Which type of headaches have an aura before they start, migraines or tension headaches?
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Migraines
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True or False. Tension headaches are treated with propanolol.
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False. Migraines are treated with propanolol
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What can you treate migraines with chronically and acutely?
|
Propanolol chornic, and Sumatriptan acutely
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What are migraines caused by?
|
Irritation of Cranial nerve 5, and involve release of substance P, Calcitonin gene related Peptide, and vasoactive peptide
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What are cluster headaches treated with?
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Sumatriptan or 100 percent oxygen
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What is the contraindication to sumatriptan?
|
CAD or Prinzmetal angina (these drugs cause vasoconstriction)
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Whats the mechanism of action for Sumatriptan?
|
5-HT (1B-1D) agonist- Serotonin agonist causes vasoconstriction, inhibition of trigeminal activation and vasoactive peptide release.
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What disease is characterized by a port-wine stain in the opthalamic distribution of a child?
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Sturge-Weber syndrome
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What is sturge weber syndrome with its port wine stain like to cause in the child/
|
Seizures due to underlying calcifications due to AV malformations
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What is a nevus flammeus associated with?
|
Port wine stain on person = sturge weber
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What is a phakomatosis?
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Neurocutaneous syndrome
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True or False. Of the Phakomatosis, Sturge Weber is the most common.
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False, it is the least common neurocutanous syndrome- Neurofibromatosis is the most common
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How is Sturge Weber inherited?
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Its not..Its sporadic
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How is Neurofibramotosis inherited?
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Autosomal dominantly
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Since nuerofibramatosis is Auto-dom, do all chidlren of affected people get it?
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No..imcomplete penetrance
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How is Tuberous Sclerosis inherited?
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Autosomal Dominantly
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Why chromosome is NF1 located on?
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17 - 17 letters in Neurofibromatosis
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What disease is characterized by lisch nodules, café-au-lait spots, axillary freckling, and pedunculated masses
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NF1 - vonrecklinghausens disease
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True or False. Neurofibromas can occur on palms and soles in NF1?
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False, they spare the palms and soles
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What kind of proteins are merlin and neurofibromin?
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Tumor suppresor genes
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What tumors are associated to NF1?
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Wilms tumor and Pheochromocytoma and Optic Gliomas
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What are lisch nodules?
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Found in NF1, hamartomas of the iris
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What are optic gliomas often associated with?
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NF1 - vonrecklinghausens disease
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What type of glial cell do optic gliomas rise from?
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Astrocytoma
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What are the symptoms of optic glioma?
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vision loss, involuntary eye movements
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What are shagreen spots, and hypopigmented ash leaf spots associated with?
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Tuberous sclerosis
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What are sebaceuous adenomas?
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angiofibromas on face (fibrous tissue papules)
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Which Phakomatosis has mitral regurigiation as a symptom/
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Tuberous sclerosis
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Which Phakomatosis has renal angiomyolipomas as a symptom/
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Tuberous sclerosis
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Which Phakomatosis has pheochromocytomas as a symptom?
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NF1 - vonrecklinghausens disease
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Which Phakomatosis has Subependymal giant cell astrocytomas?
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Tuberous sclerosis
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Which Phakomatosis is not inherited autosomal dominantly?
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Sturge Weber Syndrome
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What is almost 100 percent predictive of tuberous sclerosis/
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Rhabdomyoma of heart
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What do seizure, mental retardation, an increased likelhood of sebacsous adenomas point to/
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Tuberous sclerosis
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What is the consequences of a subfalcine hernation?
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Compression of ACA
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What herniates in a subfalcine herniation?
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Cingulate gyrus to the other side
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What kind of herniation can occur at the tentorium cerebelli?
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Medial temporal lobe - an Uncal Herniation
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What are the 3 classic signs of an uncal herniation?
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Ipsilateral Down and out fixed pupil with ptosis, Contralateral homonymous hemianopia, and ipsilateral hemiparalysis
Also, you can have duret hemorrhages, that cause caudal displacement of the midbrain |
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What three structures are most commonly affected in an uncal herniation?
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1. Oculomotor and parasympathetics go 2. PCA goes 3. Contralateral cerebral peduncle 4. Compression of midbrain causes duret hemorrhages
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When can a herniation lead to coma and death?
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When the brainstem is compressed
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What can an uncal herniation cause hemorrhage in?
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Paramedian artery rupture in pons/midbrain
Duret's hemorrhages |
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What can herniate through the foramen magnum?
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Cerebellar tonsils - Arnold Chiari Type II
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What causes the Contralateral hemianopia in uncal herniation?
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Infarct of PCA to visual cortex
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What causes the ipsilateral hemiparalysis in uncal herniation (what is it called)?
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Compression of the contralateral cerebral peduncle (kernohan's notch)
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What causes duret hemorrhages in uncal herniation?
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Caudal displacement of brainstem (midbrain compression)
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How does the chiari malformation (cereberllar tonsil herniation) cause Cardiorespiratory arrest?
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Compressing the brainstem
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What is the Chiari malformation?
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Herniation of cerebellar tonsils through the foramen magnum
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What do you want to produce in someone with head trauma to prevent vasogenic edeam?
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Respiratory alkalosis, H leaves, K goes in , and smooth muscles relax
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What is papilledema caused by?
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Increased intercranial pressure (any cause- even benign)
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What does papilledema look like on fundoscopy?
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Loss of margins, and swelling of disk
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Who is psuedotumor cerebri most often seen in?
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Fat Fertile Females
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What two drugs commonly taken by females can result in psuedotumor cerebri?
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Isotretinoin(acne) and Tamoxifen
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What is the pathogenesis of psuedotumor cerebri?
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Less absorption at arachnoid granulations
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What are the Clinical findings in Psuedotumor cerebri?
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Headache, blurry vision, double vision
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Are there any focal neurologic signs in psuedotumor cerebri?
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No, these are only in cerebral edema
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What two conditions can cause Parinaud syndrome?
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Lesion of sup colliculi: Cerebral aquaduct stenosis, and pinealoma (verticle gaze problem)
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Whats the mechanism of communicating hydrocephalus?
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Doesn’t abosrb in the arachnoid granulations- post-meningitic scarring( crypto neoformans)
or increased production (choroid plexus papilloma) |
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What is the mechanism of Non-communicating hydrocephalus?
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Obstruction of CSF through ventricular system
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What are some common causes of obstructing hydrocephalus?
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Kids=stricture of aquaduct, Dandy Walker or Arnold Chiari, Tumors either ependymomas or medulloblastomas
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What does stricture of the aquaduct cause in vision terms?
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Parinaud syndrome
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What does normal pressure hydrocephalus cause?
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stretching of fibers (wacky, wet, wobbly) sacral motor and limic fibers
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What is the wide based gait, urinary incontinence, and dementia triad indicate?
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normal pressure hydrocephalus
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What marker goes up when the neural tube doesn't close?
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Alpha feto protein (maternal)
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Whats the difference between meningomyolocele and menigocele?
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Meninogmyelocele actually has spinal cord protruding, meninogocele only has subarachnoid space (meninges)
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What is Arnold Chiari malformation and what is it associated to?
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Herniation of the cerebellar tonsils, and its related to meningomyelocele and Syringomyelia
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What can both Arnold Chiari and Dandy Walker lead to?
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Non-communicating hydrocephalus
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Do adults with obstructing hydrocephalus get enlarged head circumference?
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no sutures have already fused
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What is the Dandy Walker Malformation ?
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Cerebellar atrophy and cystic dilation of the 4the ventricle
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Why does Dandy Walker occcur?
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Failure of opening of luscka and magendie
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What is anencephaly cause in the amniotic sac?
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polyhydraminos because of the lack of swallowing center in the brain
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Why does anencephaly cause polyhydraminos?
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No Swallowing center in brain
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What is holoprosencephaly?
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Failure of splitting of cerebral hemispheres.- leads to cyclops
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What is syringomyelia?
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Enlargement of the central canal of spinal cord
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What condition is syringomyelia often seen in?
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Arnold Chiari Type II
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Whats the classic location and symptoms for syringomyelia?
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C8 to T1, loss of spinothalamic, preservation of position
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What is a late symptom of syringomelia?
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Loss of Intrinsic hand muscles, Horners, etc…
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If you have loss of motor to intrinsic hand muscles, what two conditions do you suspect, and what differentiates them?
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Suspect Syringomyelia and ALS. ALS doesn’t have sensory deficit
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How does diabetes mellitus lead to peripheral neuropathy?
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Schwann cell damage via osmotic damage
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