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Pathology neuro

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Answer
Tau pathology   Much more spatial course than amyloid. First seen in temporal lobe>frontal>parietal (not really seen in occipital lobe)  
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Neurofibrillary Tangles   NT is due to TAU and has a more typical spatial course than amyloid plaques. Tau causes death of neuron and is highly resistant to degradation  
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TDP-43 pathology   Normally found in nucleus; should not be found in cytoplasm-->in FTLD-TDP43 it is found outside of the nucleus  
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akathesia   (extrapyramidal)--condition marked by motor restlessness, ranging from anxiety to inability to lie or sit quietly or to sleep, a common extrapyramidal side effect of neuroleptic drugs.  
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Glioblastoma Multiforme   Typically presents with seizures or subtle deficits (memory). Typically leads to death because of mass effect and not metastasis. Most patients die within year of diagnosis  
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TDP-43   normally only in cytoplasm. In pathological states it is found in the cytoplasm. Diffuse inclusions =Skein-Like -->ALS  
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Parkinson's   Lewy Bodies (alpha-synuclein inclusions in substancia nigra, locus cereleus, hypothalamus, and dorsal nucleus of vagus) TDP-43 pathology  
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Lewy Bodies   Parkinson unless it is also found in the cortex: in that case it is DIFFUSE LEWY body dsx. Lewy bodies (in cortex) + neurofibrillary tangles --> alzheimers  
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Florid Inclusions   Inclusions seen in HIV positive patients with opportunistic CMV infections  
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EMG with spontaneous activity (fibrillation potential) and a “myopathic” pattern induced by voluntary muscle activity   Polymyositis (may be also inclusion body myositis?)  
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myopathy differential   (+) auto-antibodies- (EG ANA) 1)Dermatomyositis--if papular rash on face or joints on hand 2)Polymyositis-- 3)No Ab-- Inclusion body myositis  
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Todd's paralysis/paresis   focal weakness in a part of the body after a seizure  
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Fried Egg appearance   this pathological finding is common in oligodendrogliomas due to cytoplasmic swelling  
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Perivascular pseudorosettes may describe _____   pathological finding in ependymoma  
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Negri Bodies are   eosinophilic inclusions present in cerebellum of patients infected with rabies  
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what type of cancer would you be looking at if you saw "whorls," Psammoma bodies/Calcification?   meningioma  
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Chronic Inflammatory demyelinating polyneuropathy   chronic GBS. Associated with certain HLAs and not a antecedent viral illness. Onion bulb changes are commonly found  
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Which inflammatory myopathy does not respond to glucocorticoids?   Inclusion body myositis...this is the disease within this class that is more common in males and is unresponsive to glucocorticoids  
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Spinal muscular atrophy is due to mutations in what gene?   SMN-(survival motor neuron gene) This gene is needed in most cells, but leads to selective degeneration of lower motor neurons.  
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Which subtypes of SMA are consistent with normal life span   Type 2 and 3; type 1 usually causes leads to death by the age of 2.  
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ALS pathology   motor neurons dsx (SOD1) mutation is needed for onset --astrocytes and inflammatory cells secrete toxic substances (pathological) and are responsible for progression  
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Leigh's (subacute necrotizing encepalopathy)   a mitochondrial abnormality  
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Alzheimer's type II Astrocytes   (not Alzheimer's disease). A pathological finding associated with hepatic disease and Wilson's dsx.  
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Wilson's disease   an AR inherited mutation in copper-transporting ATPase. Onset is usually in the 2nd decade of life. You can find copper in their urine and treat this with chelating agents  
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Kernicterus   Due to high levels of circulating bilirubin. Typically in babies who lack a fully conjugating system. Will see yellowing of brain  
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What are some bedside signs of frontal lesions   The inability to suppress primitive reflexes (grasp, suck, and root reflex). Inability to have an antisaccade  
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Describe the behaviors you might see in ANOSOGNOSIA.   The patient who has a parietal lesion(s) will not recognize their contralateral limb  
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Rolandic (Central mid-temporal spikes)are present   in benign focal epilepsy of childhood  
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Ganglioglioma   Is most common in children and is associated with seizures. On imaging you see cystic enhancing mural nodules in temporal lobe. Usually recur due to glial component  
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LOH of 1q and 19q vs no LOH   patients with a deletion are much more likely to survive 10-15 and benefit from surgery. Tumors with loss of heterozygosity are responsive to treatment to chemo  
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Medulloblastoma   is a tumor of the CNS mainly seen in children. they present with ataxia and increased ICP. They are malignant, rapidly growing and undifferentiated (in cerebellum)  
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The treatment of medulloblastoma   is chemotherapy. Though this is a highly malignant tumor it is very sensitive to radiation. Side: high levels of radiation will cause permanent deficits in the kids.  
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Schwannomas and meningiomas   are both typically cured with surgery. Meningiomas become malignant and invade (will show psamomma bodies and whorls).  
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