Term
| The etiology of Parkinson’s stems from mitochondrial dysfunction, oxidative stress, and proteosome dysfunction which leads to a build up of alpha-synuclein which builds up in structures that are the signature neuropath sign, called what? |
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Definition
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Term
| Name the components of the basal ganglia. |
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Definition
| Caudate nucleus, putamen, globus pallidus internal/external, substantia nigra pars compacta/reticulate, the subthalmic nucleus |
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Term
| The two main inputs to the striatum are the cerebral cortex and the nigrostriatal pathway, what are the neurotransmitters used by each including the spiny neurons of the striatum? |
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Definition
| Cortex pyramidal neurons - glutamate, Nigrostriatal - Dopamine, spiny neurons of striatum 95% use GABA (other 5% of interneurons use GABA and Ach) |
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Term
| The basal ganglia input and output loops are topographically organized, which results in what type of existing circuits? |
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Definition
| Parallel circuits co-existing in the same space |
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Term
| What is the difference between the two parts of the substantia nigra, the pars compacta and pars reticulate? |
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Definition
| PC - dopamine and is an input to the striatum, PR - GABA and is an output from the striatum to the superior colliculus |
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Term
| Outputs of the basal ganglia originate in only two regions, globus pallidus internal/external and the substantia nigra reticulate, where do their signals go and what neurotransmitters are used? |
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Definition
| GPi/e goes to VL thalamus motor nuclei and reticular formation to frontal cortex, SNr goes those plus the sup colliculus; GPi/Snr use GABA with substance P, GPe use GABA with enkephalin |
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Term
| Dopamine is used to facilitate movement in both the direct and indirect pathway through opposite effects, describe how this is done and what receptors are used. |
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Definition
| D1 - stims direct pathway, D2 - inhibits the breaks of indirect pathway; both occur at the input from the SNpc to the striatum/GP level |
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Term
| Parkinson’s disease is in 1-2% of the pop, 1.5/1 men to women, with onset before 50 and it results in tremors and postural instability, what type of neuron loss is the cause of this problem? |
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Definition
| Nigrostriatal dopaminergic neurons (mostly SNpc), which leads to decreased direct pathway stim and increased indirect stim |
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Term
| What are the possible treatments for Parkinson’s? |
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Definition
| Medical - L-DOPA therapy, Surgical - Lesions of the GPi, and high frequency stim of the GPi and Subthalamic nucleus |
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Term
| Trace the direct pathway of the basal ganglia starting with inputs. |
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Definition
| Cerebral cortex/SNpc to Caudate/Putamen to GBi to VL thalamus to frontal motor cortex |
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Term
| Trace the indirect pathway starting with the inputs. |
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Definition
| Cerebral cortex/SNpc to Caudate/Putamen to GPe to Subthalamic nucleus to GPi to VL thalamus to frontal cortex |
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Term
| Huntinton’s disease is a hyperkinetic, auto-dom disorder that has a triad of symptoms (motor, cognitive, psychiatric) but what is the type of neuron that is most affected by this disease? |
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Definition
| Medium sized GABAergic neurons in the striatum that lead to GPe (the indirect pathway), causing an imbalance in striatal outputs. |
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Term
| T or F: Huntington’s only affects adults in there 4th/5th decade and has a 15-20 year progression. |
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Definition
| False, there is also a juvenile onset with seizures and dystonia that typically has a paternal inheritance and CAG repeat with faster progression |
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Term
| Name the protein that aggregates in cells that is the result of >39 CAG repeats in the IT15 gene of patients with Huntingtons. |
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Definition
| Huntingtin, there is controversy as to whether it is toxic or its malformation causes a loss in protective function |
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Term
| Name the categories of drugs that are used to treat the triad symptoms of Huntington’s Disease. |
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Definition
| Motor - dopamine receptor blockers and depleters; behavioral symptoms - SSRI, benzo, neuroleptics |
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Term
| Coenzyme Q-10, Minocycline, Lithium, Creatine, and Urisodiol are all drugs used to treat what aspect of Huntington’s? |
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Definition
| They attempt to be neuroprotective in order delay onset of symptoms |
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Term
| Lesions of the subthalamic nucleus and L-DOPA therapy can cause symptoms like what disease? |
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Definition
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Term
| List all the components of dopamine synthesis and metabolism. |
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Definition
| Tyrosine to L-DOPA to Dopamine to DOPAC (via MAO-B) to HVA (via COMT). |
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Term
| What is the difference between the two dopamine receptor subtypes since they are all G-protein coupled? |
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Definition
| D1/5 increases Adenylate cyclase, D2/3/4 decreases it |
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Term
| There are six routes to treat the symptoms of Parkinson’s with drugs, what are they? |
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Definition
| Increase dopamine synthesis, activate post-syn receptors, inhib dop metab, alter the balance with Ach, dopamine releasers, L-DOPA peripheral metab inhib |
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Term
| Why is L-DOPA used with carbidopa to treat Parkinson’s? |
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Definition
| L-DOPA increases synthesis capacity of dopamine since it can cross the BBB while carbidopa reduces periphery metabolism so more is available for the brain; the combo drug is Sinemet |
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Term
| MTPT is a comtaminant in the drug process that causes what disease in opiod users? |
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Definition
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Term
| Why does reserpine, antipsychotics, MPTP dopamine neurotoxin cause Parkinsonism? |
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Definition
| Because they act to reduce DA activity in the striatum via depletion of catcholamines, DA receptor blockers, and DA neurotoxicity respectively |
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Term
| Because triplet repeat disorders are dynamic mutations they have a variable nature and show what 5 molecular mechanisms? |
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Definition
| Incomplete penetrance, variable expressivity, parent of origin effects, premutation alleles (asymptomatic with a tendency to expand in next gen), and anticipation (tendency of earlier onset in each generation) |
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Term
| For the following triplet disorder list the mode of inheritance, clinical phenotype, molecular mechanism, and molecular testing for the disease: Fragile X syndrome. |
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Definition
| X-linked recessive/semi-dom, mental retardation and dysmorphic features, CGG repeat with pre/full mutation alleles needing hypermethylation, Southern for full mut and PCR for permutations and Methylation Southern blot |
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Term
| For the following triplet disorder list the mode of inheritance, clinical phenotype, molecular mechanism, and molecular testing for the disease: Myotonic dystrophy. |
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Definition
| Auto dom, myotonia and muscular dystrophy/cataracts/hypogonads, CTG repeat, southern blot |
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Term
| For the following triplet disorder list the mode of inheritance, clinical phenotype, molecular mechanism, and molecular testing for the disease: freidrich’s ataxia |
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Definition
| auto rec, gait and upper extremity ataxia with absent reflexes and cardiomyopathy/intellectual decline, GAA repeat, PCR or Southern |
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Term
| For the following triplet disorder list the mode of inheritance, clinical phenotype, molecular mechanism, and molecular testing for the disease: Huntington’s. |
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Definition
| Auto dom, middle age choreoathetosis with dementia, CAG repeat, PCR to size repeat length. |
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Term
| Define this type of tremor - asymmetric or symmetric rest tremor at 3-6 hz. |
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Definition
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Term
| Define this type of tremor - kinetic tremor that is coarse with large amplitude. |
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Definition
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Term
| Define this type of tremor - postural and/or kinetic at 8-12hz |
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Definition
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Term
| Define this type of tremor - postural at 8-12hz due to metab, toxin, stress, etc. |
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Definition
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Term
| Chorea is a brief, non-sustained movement define the three types that are seen. |
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Definition
| Primary from inherited disorders, secondary from acquired infections/lesions/metabolic probs, and physiologic which is seen in infancy |
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Term
| What is tardive dyskinesia? |
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Definition
| Choretic movements at the mouth resulting from the use of dopamine blockers or anti-emetics |
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Term
| Myoclonus is a rapid involuntary movement, either positive muscle contraction or negative muscle tone, that can be general, focal or segmental - but damage to what structures can cause it? |
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Definition
| Cortex, brainstem, peripheral nerves |
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Term
| Dystonia is the co-contraction of agonist and antagonist muscles, what is the treatment for generalized and focal problems. |
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Definition
| General - anti Ach, baclofen, benzos, levadopa, or deep brain stim of GPi; Focal - botox injections |
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Term
| The thalamic nuclei has 3 relay divisions (anterior/medial/lateral), an intralaminar nucleus, and a reticular nucleus - what are the functions of each system? Name the principal nuclei and there function. |
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Definition
| Anterior and Medial relay is the limbic system, lateral relay is the motor/sensory/auditory/visual (ant. to post.), intralaminar is consciousness and basal ganglia, and reticular regulates the other nuclei |
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Term
| What is the function of the medial and lateral geniculate nuclei in the thalamus? |
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Definition
| Medial auditory relay, lateral visual relay to the cortex |
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Term
| What are the functions of each of the cerebral cortex lobes? |
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Definition
| Frontal - motor, temporal - auditory, limbic - emotions and long term memory, parietal - somatosensory, occipital - visual |
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Term
| What is the function of the premotor and supplementary motor cortex in the frontal lobe? |
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Definition
| Premotor - PREparing for movement, supplementary - programming complex movements and mental rehearsal |
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Term
| The prefrontal cortex, part of the frontal lobe, has two main areas - dorsolateral and orbital medial, what is the function of each? |
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Definition
| Dorsolateral is planning and problem solving and working memory, orbital medial is self-control |
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Term
| Damage to the dominant language hemisphere (usually left) causes either broca’s or wernicke’s aphasia, what is the difference between the two? |
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Definition
| Broca’s produces non-fluent, motor, or expressive aphasia; Wernicke’s produces fluent, sensory, or receptive aphasia. |
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Term
| When damage occurs to a person’s non-dominant hemisphere at the parietal association cortex it can cause a particular syndrome, what is it and is it the same for both sides? |
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Definition
| Contralateral neglect syndrome, No |
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Term
| Lesions to the parietal-occipital-temporal association cortex can produce either agnosia or apraxia, what are they? |
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Definition
| Agnosia is inability to recognize an object despite a functional sense, apraxia is when you can’t perform a motor skill on command |
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Term
| Describe which arteries supply the lateral and medial surfaces of the brain as well as thalamus and basal ganglia regions. |
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Definition
| Lateral - middle cerebral, medial - anterior and posterior, thalamus is posterior, basal ganglia is deep branch of middle called letniculostriate arteries |
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Term
| The primary cholinergic innervation to the cerebral cortex comes from which nucleus? |
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Definition
| The basal nucleus; degeneration of this cell and its Ach creating enxyme leads to Alzheimer’s |
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Term
| T or F: upper motor neuron signs are usually due to damage of the corticospinal tract alone. |
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Definition
| False, it is usually due to multiple descending pathways that include projections from the cortex, brainstem (red nuc, reticular form), or pathways |
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Term
| There are two types of cells in the cerebral cortex called pyramidal and non-pyramidal cells, what is the difference? |
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Definition
| Pyramidal are the primary projection or output neuron that go to subcortical areas, non-pyramidal neurons called granule or stellates or baskets are local GABA interneurons |
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Term
| In the laminar organization of the cerebral cortex there are six layers, ID them and where the receiving and projecting areas are. |
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Definition
| Molecular, ext. granule, ext. pyramidal, int. granule (receiving), int. pyramidal (projecting), multiform |
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Term
| The visual pathway of the cerebral cortex terminates in what visual cortex area? |
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Definition
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Term
| Since a stroke is a permanent injury to the brain via reduced blood flow or bleeding what are the 3 types of stroke? |
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Definition
| Infarction, intracerebral hemorrhage, subarachnoid hemorrhage |
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Term
| After a sudden disruption of cerebral blood supply what is the window of time for a infarct to form? |
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Definition
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Term
| After a stroke what is ischemic penumbra? |
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Definition
| The areas of salvageable tissue around the infarct |
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Term
| What is the most common stroke subtype? |
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Definition
| Large vessel ischemia stroke |
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Term
| The following clinical features define what type of stroke: progressive defecits common, onset while asleep common, preceeding brief TIA common, cortical signs frequent. |
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Definition
| Large artery ischemic stroke |
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Term
| The following clinical features define what type of stroke: maximal deficits at onset, onset while asleep uncommon, preceeding TIA uncommon, cortical signs frequent. |
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Definition
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Term
| The following clinical features define what type of stroke: progressive onset common, onset while asleep common, preceding TIA fairly common, cortical signs rare. |
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Definition
| Small vessel lacunr stroke; e.g. deep branch of middle cerebellar artery reaching basal ganglia |
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Term
| The following clinical features define what type of stroke: smoothly progressive defecits over 10-20 min, onset while asleep uncommon, focal symptoms, symptoms of raised intercranial pressure (headache, nausea and vomiting). |
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Definition
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Term
| The following clinical features define what type of stroke: thunderclap headache, onset while asleep uncommon, symptoms of intercranial pressure, focal symptoms uncommon. |
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Definition
| Subarachnoid hemorrhage stroke |
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Term
| What 3 diagnostic studies are used for stroke and which one is the gold standard for intracranial aneurysms? |
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Definition
| CT (90% sensitive), if CT neg then LP, Angiography is gold standard |
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Term
| T or F: there is no grade of aneurismal subarachnoid hemorrhage that qualifies for surgery. |
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Definition
| False, grades 1,2,3 are candidates |
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Term
| What is the most important determinant of outcome after subarachnoid hemorrhage? |
|
Definition
| Neurological condition on arrival at hospital |
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Term
| If a patient has an infarct showing contralateral leg weakness and sensory loss with bladder incontinence what artery is affected? |
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Definition
| Anterior cerebral artery syndrome |
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Term
| If a patient has an infarct and presents with contralateral face and arm weakness and sensory loss with Broca’s aphasia or contra neglect (depending on hemisphere) what artery is affected? |
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Definition
| Middle cerebral superior division |
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Term
| If a patient has an infarct and presents with contra hemianopia, Wernicke aphasia or contra neglect (depends on hemisphere) then which artery is affected? |
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Definition
| Middle cerebral inferior division |
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Term
| If a patient present with contra hemianopia, alexia without agraphia, and visual agnosias then what artery is affected? |
|
Definition
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Term
| Which of the following could be the result of a lacunar infarct - pure motor hemiparesis, pure sensory stroke, ataxic hemiparesis, dysarthria, sensorimotor? |
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Definition
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Term
| If a patient has an infarct and presents with ataxia, vertigo, diplopia, dysphagia, bilateral weakness and sensory loss, what artery was affected? |
|
Definition
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Term
| What is the difference between diffusion and perfusion weighted imaging? |
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Definition
| Diffusion measures the change in water movement within minutes of ischemia causing decreased diffusion coefficient, whereas perfusion measures contrast flow into ischemic areas to visualize hemodynamic compromise; the difference between the two ID’s the penumbra area |
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Term
| tPA allows stroke patients greater recovery and less disability if given in what window of time? |
|
Definition
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|
Term
| What are the warning signs of stroke? |
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Definition
| Sudden headache, change in vision, difficulty speaking, weakness, or dizziness. |
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Term
| T or False: Post TIA has a lower level of stroke recurrence than post-minor stroke. |
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Definition
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Term
| T or F: Mortality decreases with each successive stroke. |
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Definition
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|
Term
| A carotid endarterectomy should be done is stenosis is at what percent in symptomatic and asymptomatic patients? |
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Definition
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Term
| If a patient is less than 65 with no risk factors, which med should be used to prevent stroke - aspirin or warfarin? |
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Definition
| Aspirin. Warfarin is used if the patient is older or has risk factors |
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Term
| The most common risk factor for stroke is …? |
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Definition
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Term
| What are the 3 main findings of early stroke on non-contrast CT? |
|
Definition
| Loss of sulcus visibility, loss of grey-white matter differentiation due to edema, bright MCA sign (only in 10-15% of strokes) |
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Term
| What color is an infarct in an DWI-MRI and in an ADC map? |
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Definition
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|
Term
| What is the main utility of MRI in stroke? |
|
Definition
| To select patients for aggressive thrombolysis based on the penumbra model, ie. Those who have a PWI-DWI mismatch |
|
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Term
|
Definition
| It is a modality that eliminates fluid like CSF to visualize microvascular ischemic changes in place with edema |
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Term
| What is the sensitivity of non-contrast CT in the first 3 hours and 6 hours in diagnosing cerebral infarct? |
|
Definition
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|
Term
| What is the benefit of DWI-MRI versus FLAIR or T2 MRI? |
|
Definition
| FLAIR or T2 can visualize edema, DWI can help differentiate between cytotoxic edema (due to infarcts) and vasogenic edeme (due to tumor or infection) |
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