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Neurology
UWORLD Round 2 2021 Part 1
| Question | Answer |
|---|---|
| What is BH4? | Cofactor used by Hydroxylase enzymes in the synthesis of Tyrosine, Dopamine, and Serotonin |
| Which amino acids and/or neurotransmitters are synthesized with BH4 involvement? | Tyrosine, Dopamine and Serotonin |
| What is a common condition due to BH4 deficiency? | Phenylketonuria (PKU) |
| What cofactor is often deficient in development of PKU? | BH4 |
| What is the result f Dihydropteridine reductase deficiency? | BH4 deficiency leading to PKU |
| What is the hallmark symptom or sign of PKU? | Intellectual disability |
| What causes intellectual disability in patients with PKU? | Serotonin deficiency and hyperphenylalaninemia |
| What is the most common treatment regimen of PKU? | Low phenylalanine diet and BH4 supplementation |
| Which condition is suspected if patient follows a diet low on Phenylalanine ? | Phenylketonuria (PKU) |
| What amino acid is accumulated in excess with PKU? | Phenylalanine |
| What is the trinucleotide repeat of Fragile X syndrome? | CGG |
| What condition shall be suspected if the patient has a CGG trinucleotide repeat? | Fragile X syndrome |
| What gene is mutated or affected in Fragile X syndrome? | Fragile X mental retardation 1 (FMR1) |
| Where is the FMR1 gene affected on the affected chromosome? | Long arm of the X chromosome |
| Which condition is seen with a mutated protein at the long arm of the X chromosome? | Fragile X syndrome |
| What is the result of mutated FMR1 gene in Fragile X syndrome? | Hypermethylation and inactivation of FMR1 gene |
| What enzymatic modification is seen to FMR1 in patient with Fragile X syndrome? | HyperMETHYLATION |
| Clinically a patient presents with severe mental retardation, Hx of frequent falls. Genetic results provide a mutated protein that is hypermethylated. The condition is associated with gene at the long arm of the X chromosome. Dx? | Fragile X syndrome |
| To which family, does the West Nile Virus, belong? | Flavivirus |
| Single-stranded Flavivirus transmitted by mosquitoes, most commonly in the summer. | West Nile Virus |
| How are most patients clinically presented if infected with West Nile Virus? | Asymptomatic or with a flu-like illness (West Nile fever) |
| What is a common, but not always accompanying clinical feature of West Nile fever? | Maculopapular or morbiliforme rash |
| What are clinical features of severe or advanced West Nile Virus infection? | Meningitis, Encephalitis, or asymmetric flaccid paralysis |
| Which viral infection is often seen, if severe, with asymmetric flaccid paralysis, and often with meningitis and encephalitis, especially in hot summer days? | West Nile Virus |
| What viral summer associated condition is associated with Parkinsonian features? | West Nile Virus |
| Is not rare for a West Nile viral infection patient to develop what kind of movement features? | Parkinsonian features |
| What is a common condition that presents as generalized, excruciating headache, and often referred as the "worst headache of my life"? | Subarachnoid Hemorrhage (SAH) |
| What is the MCC of Subarachnoid hemorrhages? | Rupture of Saccular (Berry) aneurysms or AV malformations |
| What condition is often associated with Berry aneurysms in the Circle of Willis, and often diagnosed until adulthood? | ADPKD |
| What is a common association of brain lesion in ADPKD? | Rupture of Berry aneurysm in the Circle of Willis |
| What is Huntington disease? | AD neurodegenerative disorder that manifest with chorea and behavioral abnormalities |
| What are the neuroimaging findings of Huntington disease? | Atrophy of the Caudate nucleus |
| What is the result of atrophy of the Caudate nucleus in Huntington disease? | Enlargement of the Frontal horns of the lateral ventricles |
| Which neurodegenerative disease is seen enlargement of frontal horns of the lateral ventricle? | Huntington disease |
| What common neurodegenerative condition is seen with affected lateral ventricle, specifically at the frontal horns? | Huntington disease |
| What cranial nerve is affected by Diabetic Mononeuropathy? | Cranial Nerve III |
| What causes Diabetic mononeuropathy? | Central isquemia, which affects the somatic nerve fibers but spaces peripheral parasympathetic fibers |
| Which nerve fibers are affected by Diabetic mononeuropathy? | Somatic nerve fibres |
| Which nerve fibers, Somatic or Peripheral Parasympathetic, are spared in Diabetic mononeuropathy? | Peripheral parasympathetic nerve fibers |
| What are some of the ocular symptoms associated with Cranial nerve III (3) palsy? | Ptosis, a "down-and-out" gaze, and normal light and accommodation reflexes |
| What nerve palsy is seen with normal Pupillary light and Accommodation reflexes? | Cranial nerve III palsy |
| How is Essential tremor presents? | As a tremor of the hands that is suppressed at rest, exacerbated by outstretched arms, and move produced during goal-directed movements |
| What is the most common inheritance mode of Essential tremor? | Autosomal dominant |
| What is Carpal Tunnel syndrome? | Peripheral neuropathy, characterized by pain/paresthesia i a a median nerve distribution, weakness on thumb abduction/opposition, thenar atrophy, and a (+) Tinel and/or Phalen test |
| Which nerve is affected in Carpal Tunnel syndrome? | Median nerve |
| What are some characteristics of Carpal Tunnel syndrome? | - Weakness on thumb, - Abduction/ Opposition - Thenar atrophy - (+) Tinel and/or Phalen test |
| At which point (structure) is the Median nerve compressed leading to Carpal Tunnel syndrome? | Transverse Carpal ligament |
| What condition is associated with Compression of a nerve at the Transverse Carpal ligament? | Carpal Tunnel syndrome |
| What is the most common cause of Uncal herniation? | Expanding ipsilateral mass lesion of the brain |
| What is the 1st sign of Uncal herniation? | Fixed, dilated pupil on the side of the lesion |
| What conditions often associated with early fixed, and dilated pupil on the side of the suspected lesion? | Uncal herniation |
| On which side of the brain, right or left, would a person with Left fixed mydriasis would have the uncal herniation expanding compression? | Left |
| What are the late signs of Uncal herniation? | 1. Contralateral or Ipsilateral hemiparesis 2. Contralateral homonymous hemianopia with macular sparing |
| What is the MCC of Deep Intraparenchymal hemorrhage? | Hypertensive vasculopathy of the small penetrating branches of the cerebral arteries |
| What Is ta common cause, specific pathology, that leads to deep intraparenchymal hemorrhage? | Charcot-Bouchard aneurysm rupture |
| What is a common example of hypertensive vasculopathy of the the small penetrating arteries leading to Intraparenchymal hemorrhage? | Charcot-Bouchard aneurysm rupture |
| Which type of aneurysm is causative fo SAH? | Saccular (Berry) aneurysm |
| Which type of aneurysm is known to be causative of Deep Intraparenchymal hemorrhage? | Charcot-Bouchard aneurysm |
| How is Ulnar neuropathy clinically characterized? | Numbness of the 4th and 5th digits, pain and weakness |
| What type of cell is injured in Ulnar neuropathy? | Schwann cell |
| What is the defining characteristic of Demyelination injury? | Causes slowed or blocked nerve conduction velocity |
| What does Axonal injury causes? | Reduction in signal strength |
| An axonal injury will cause slow nerve conduction velocity or a poor signal strength? | Poor signal strength |
| If nerve conduction studies shows a slow nerve conduction velocity, it is more likely to have a Dyemilynating or axon injury? | Demyelinating nerve injury |
| What are the 2 neurons signs of the SNS that regulate visceral function? | Cholinergic preganglionic neurons and Adrenergic postganglionic neurons |
| Which glands are an exemption to the 2-signal transmission of the SNS in regards to viceral functionality? | Eccrine sweat glands and Adrenal medulla are only innervated by Cholinergic neurons |
| Which nervous system, PNS or SNS, uses both Preganglionic and Postganglionic cholinergic neurons? | PNS |
| If a transmission signal has a Cholinergic preganglionic neuron and a adrenergic postganglionic neuron, is it SNS or PNS? | SNS |
| If a nerve signal has both pre- and postganglionic cholinergic neuron, is it more likely to be PNS or SNS? | PNS |
| What are some common Dopamine receptor blockers? | Metoclopramide and Prochlorperazine |
| What two common uses for Dopamine receptors blockers? | 1. Antiemetics 2 Pain relievers for severe migraine headache associated with nausea and vomiting |
| What medication can be given to a patient with severe migraines, other than a triptan? | Dopamine receptor blocker |
| What are common type of adverse effects due to Dopamine receptor blockers? | Excess cholinergic activity |
| What is commonly used to prevent the excess cholinergic activity seen with Dopamine receptor blockers? | Diphenhydramine |
| Why is Diphenhydramine and Metoclopramide often used together? | The Diphenhydramine prevents the excessive cholinergic activity caused by Metoclopramide |
| What are two commonly used first generation H1-blockers? | Diphenhydramine and Chlorpheniramine |
| Which type of drugs are highly contraindicated in a patient already taking diphenhydramine? | Benzodiazepines |
| Why are benzodiazepines not co-administered with a 1sst generation H1- receptor blocker? | The H1-blockage generates significant sedation, and the benzodiazepines can further increased CNS depression |
| Which adrenergic receptor stimulation causes inhibition of uterine contractions? | B-2 adrenergic receptor stimulation |
| What is an important effect of B-1 stimulation? | Inhibition of uterine contractions |
| What is a significant ocular effect of alpha-1 receptor stimulation? | Contraction of the Pupillary dilator muscle resuinti in mydriasis (pupillary dilation) |
| Does stimulation of the alpha-1 receptor causes miosis or mydriasis? | Mydriasis |
| What would be the effect of alpha-1 receptor blockers on the pupil? | Miosis |
| What part of the basal ganglia is damaged that causes UMN lesions? | Internal capsule |
| What type of clinical features or deficits are expected in Internal capsule stroke? | UMN lesions that cause contralateral weakness with clasp-knife spastic rigidity, hyperreflexia, and (+) Babinski sign |
| Are the deficits caused by Internal capsule stroke UMN or LMN lesions? | UMN lesion |
| Are the UMN lesions associated with Internal Capsule stroke contralateral or ipsilateral? | Contralateral |
| Which parts the of nervous system are affected in Internal capsule stroke? | Pyramidal motor system by damage to the Corticospinal tracts |
| How does the Corticospinal tract run along the nervous system? | From the Precentral gyrus (Primary motor cortex) through the internal capsule to the brainstem and Spinal cord |
| What would be a common list of symptoms due to Right Internal Carotid artery emboli/stenosis? | Transient attacks resulting Left leg weakness and vison loss in the right eye |
| Vision loss due to internal carotid emboli is ipsilateral or contralateral to the side of lesion? | Ipsilateral |
| If a patient has an obstruction in the Left Internal Carotid artery, the patient will develop right or left leg weakness? | Right leg weakness |
| Which important arteries branch off directly from the Aortic arch? | Left Common Carotid and Left Subclavian arteries |
| Which arterial vessels branch of the Brachiocephalic artery? | Right Subclavian and Right Common Carotid arteries |
| Does the right ro left Common Carotid and Subclavian arteries are divisions of the Brachiocephalic artery? | Right Subclavian and Right Common Carotid arteries |
| What are common symptoms involving Trigeminal nerve infarct of the anterior portion of the medial pons? | Dysarthria and Contralateral hemiparesis/lower facial palsy |
| What causes the symptoms in anterior med-pons trigeminal nerve infarct ? | Disruption of the Ipsilateral Corticospinal and Corticobulbar tracts |
| Which tracts are affected in Trigeminal nerve infarct involving the anterior portion of the mid-pons? | Ipsilateral Corticospinal and Corticobulbar tracts |
| Which facial palsy is due to trigeminal nerve damage, lower or upper facial palsy? | Lower facial palsy |
| Where in the brainstem does the Trigeminal nerve arises from? | Level of the middle cerebral peduncle at the lateral aspect tohe mid-pons |
| Self-limited syndrome characterized by sudden onset of prominent anterograde amnesia accompanied by repetitive questioning. Dx? | Transient Global aniea |
| What part of the brain anatomy is involved in Transient Global amnesia? | Hippocampus |
| What is the hippocampus responsible for? | Formation of new memories |
| If a patient is unable to form new memories, it is very likely to have which part of the brain affected or damaged? | Hippocampus |
| What are features of Transient Global amnesia? | Severe anterograde amnesia and repetitive questioning |
| What part of the Spinal cord is damaged by Anterior Spinal artery occlusion? | Anterior 2/3 of the spinal cord |
| What artery is block or disrupted in if the anterior 3/2 of the Spinal Cord are damaged? | Anterior Spinal Artery |
| What are the clinical symptoms of Anterior Spinal artery syndrome? | 1. Bilateral distal loss of pain, temperature, and crude touch 2. Paralysis |
| What tract damage causes the bilateral loss of temperature, pain a, crude touch in ASA syndrome? | Spinothalamic tract damage |
| What tract is damaged in ASA syndrome that leads to paralysis? | Lateral corticospinal tracts |
| What are the sensations or function transmission of the Dorsal columns? | Vibration, Proprioception, and Light touch |
| Which tract carries information of Crude touch? | Spinothalamic tract |
| Which spinal cord tract carries Light touch sensation? | Dorsal columns |
| Are the Dorsal columns spared or affected by ASA syndrome? | Spared |
| What is Kinesin? | Microtubule-associated ATP-powered motor protein that facilitates the anterograde transport of neurotransmitter-containing secretory vesicles down the axons to synaptic terminals |
| Kinesin is involved in anterograde or retrograde transport? | Anterograde |
| What is the MCC of meningitis in AIDS patients? | Cryptococcus neoformans infection |
| What is the main CSF finding in Cryptococcus neoformans infection? | Polysaccharide capsule |
| What is the finding in India Ink stain of C. neoformans sample? | Round or oval budding yeast |
| Which stain is used to stain for C. neoformans? | India Ink stain |
| Common budding yeast causative of meningitis in AIDS patients | Cryptococcus neoformans |
| What neurotransmitter is inhibited by Botulinum toxin? | Prevents release of ACh from presynaptic nerve terminal |
| Which neurotoxin is known to prevent the release of ACh from presynaptic nerve terminals? | Botulinum toxin |
| What Is impaired by the the prevention of ACh release due to Botulinum toxin? | Peripheral muscarinic and Nicotinic neurotransmission, causing both autonomic symptoms and skeletal muscle weakness |
| What are tome Autonomic symptoms seen with Botulism? | Fixed pupillary dilation and dry mouth |
| What are some skeletal muscle weakness symptoms seen with Botulism? | Diplopia, dysphagia, and depressed respiration |
| Does repetitive motor nerve stimulation in a patient with Boltu.iusm increase or decrease the muscle response? | Decrease |
| What areas or structures are referred as the Deep Brain structures? | Basal ganglia and pons |
| What are the Subcortical White matter areas or the brain? | Internal capsule and Corona radiata |
| What are Lacunar infarcts? | Small, ischemic infarcts(<15mm) involving the deep brain structures and Subcortical white matter |
| What is the MCC of Lacunar infarcts? | Hypertension |
| How does HTN lead to Lacunar infarcts? | Hardening and thickening of the vessel wall, leading to Thrombotic vessel occlusion |
| What are the Neuropathologic features of Alzheimer disease? | Neurodegeneration, amyloid plaques, and neurofibrillary tangles |
| What are the amyloid plaques in Alzheimer disease? | Extracellular accumulation of B-amyloid |
| how are Neurofibrillary tangles form in Alzheimer disease? | As TAU becomes hyperphosphorylated and dissociates from the microtubules to form paired helical filaments |
| What is Tau? | Protein associated with neuronal microtubules |
| What neurodegenerative condition is associated with Tau protein? | Alzheimer disease |
| What is a common development of a older child with Hx of persistent absence seizure? | Tonic-Clonic or myoclonic seizures |
| What medication is solely used to treat absence seizures? | Ethosuximide |
| What is a proper treatment for both, Absence and Tonic-clonic seizures? | Valproic acid |
| What is Valproic acid? | Broad - spectrum antiepileptic, that treats both absence and tonic-clonic (myoclonic) seizures |
| What are some common CYP450 inducers? | Phenobarbital, Carbamazepine, and Rifampin |
| Rifampin is a CYP450 inducer or inhibitor? | Inducer |
| Common SNRI: | Duloxetine |
| What is the MOA of SNRIs? | 1. Decrease the reuptake of Serotonin and NE 2. Inhibition of pain signals |
| What are the 2 neurotransmitters that are not reuptaken with the use of SNR? | Serotonin and NE |
| What is a secondary function of SNRIs? | Block pain signals |
| What is a non-emotional condition often treated with SNRI, such as Duloxetine? | Neuropathic pain |
| How does Duloxetine aid in treating neuropathich pain? | Increase NE in Central synapses, which modulates the descending pain pathway, leading to a decrease central perception of pain |
| What does the onset of action is dependent of gas anesthetics? | Solubility in the blood |
| What is the value used to describe a drugs' solubility in blood? | Blood/gas partition coefficient |
| What does a decrease in Blood/gas partition coefficient represent? | Quick saturation in blood, which lead to a fast rise in partial pressure |
| What does a slower rise in partial pressure of a drug represent in terms of solubility? | Increased Blood/gas coefficient which indicates high solubility in blood |
| Does large or small blood:gas coefficient in blood represent high or low solubility? | High solubility |
Created by:
rakomi
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