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41 Cards in this Set
- Front
- Back
What is the clinical presentation of Huntington's?
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Chorea, difficulty with breathing and swallowing, progressive dementia
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What would a person with Huntington's CT and MRI look like?
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Enlarged Lateral ventricles
cell death in basal ganglia (gross degeneration of the caudate nucleus) |
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What is the gene defect that causes Huntington's?
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36+ repeats of CAG on Chromosome 4 in the region of the HTT gene.
Celine Always Goes Huntintin (the protein) 4 |
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How is Huntington's inherited?
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Autosomal dominant. SUCKS! Person always has 50% chance of getting it
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Why did the Hungtington's patient get a lung infection or have lung problems?
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Due to moderate aspiration during swallowing, something probably got in his lung
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What does a serum ceruloplasmin/copper study check for?
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Wilson's diease
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What is the main difference between Wilson and Huntington's disease?
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Wilson's disease usually is accompanied by liver problems
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What functions are associated with the caudate nucleus?
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Inhibition of nigrostriatal pathway ( destruction causes chorea) via GABAeric neurons of the nigrostriatal pathway which is the feedback system for movement
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What types of neurotransmitter is involved with the nigrostriatal pathway?
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DOPE comes from Nigga
GABA goes from striatum to Substania nigra |
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What mood changes are involved with a Huntington's patient?
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Depression
Perhaps even grandiosity and egoism |
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What does increased CAG repeat correspond with?
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Age of onset in Huntington's patient (90 CAG's = 20 yrs of age onset)
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What would chronic alcoholism cause in the brain?
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Anterior Lobe Syndrome (of cerebellum); loss of lower limb coordination
alcohol is unlikely to cause only ipsilateral effects |
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What structures would a right anterior cerebellar lesion affect?
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It could affect all brainstem levels; in our patient:
CN 5, 7, 8, 9 near pontomedullary junction Medial Lemniscus |
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What would cause unilateral anterior lob syndrome and cafe au lait spots?
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Neurofibromas especially Type I
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What gene defect causes neurofibromas and how is it inherited?
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NF1 gene which is AD inherited
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What does a patient with right hemiparesis, speech difficulty, and right homonymous hemianopia have?
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MCA infarct that affected FPOT left lateral cortex
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What would ACA occlusion cause?
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leg weakness and sensory loss with face spared
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What would PCA occlusion cause?
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CN 5-11 defects, horner's syndrome, vision loss
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What does a MRI of a occluded MCA patient look like?
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abnormal tissue/lack of gyri, occlusion of MCA
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How did the neruocystercercosis patient relapse into non-communicating hydrocephalus?
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larvae formed a cyst by his shunt, thereby blocking it
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What caused unconsciousness in the hydrocephalus patient?
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Pressure on cardiorespiratory centers in the medulla.
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Why should a lumbar puncture not be taken in someone with increased intracranial pressure?
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could cause brain stem herniation
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What is SSEP? What does it tell you?
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Somatoseonsory evoked potential - helps pinpoint location of lesion
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What disease:
High IgG, nystagmus, weakness/tingling? |
Multiple sclerosis
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What is Eaton Lambert?
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Autoantibodies that attack Ca channels on the presynaptic axon at the NMJ so ACh cannot be released.
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Why would administration of anesthesia to an Eaton-Lambert patient cause paralyzed respiration?
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The anesthesia exacerbated her condition and had an anticonvulsant and inhibitory affect of musculature.
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What is paraneoplasmic syndrome?
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When antibodies in cancer start attacking non-cancer host cells :(
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What is the treatment for Eaton Lambert's and how does it work?
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3,4-diaminopyridine; blocks K channels to increase AP potential duration and thus greater ACh release
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What does an EMG help assess?
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NMJ function and differentiation of pre and post synaptic diseases
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What is the action of botulism?
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Cleaves vesicle docking proteins in the presynaptic neuron of the NMJ so ACh is not released.
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Where did the patient with occlusion of MCA have ictal EEG waves? What kind of seizure did he have?
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Seizure onset was in anterior left temporal lobe.
Aura indicates limbic system functioning and so does his loss of conscience which means this was a complex partial seizure |
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What about seizures does SPECT show?
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SPECT shows perfusion and thus areas of high metabolic activity to indicate site of origin and spread of seizure
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What other disorder did the kid with petit mal have to rule out?
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ADHD
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How do anti epileptic drugs work?
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GABA agonists, Na or CA channel blockers to hyperpolarize cells.
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What might febrile convulsions indicate?
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both the possibility of kindling and the genetic predisposition to be more likely to have seizures.
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Which MRI weight is dark?
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T1
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What scanning method mesaures glucose untilization?
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PETS love sugar
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What detects cerebral blood flow and intense tissue metabolism
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SPECT, much like in our seizure case
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What is the best scanning method for spatial resolution?
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MRI>CT>PET>SPECT
Mr. CT Pets snakes |
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What is the best for temporal resolution
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EEG
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What are CT scans good for detecting
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STV - strokes, tumors, ventricles, bone
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