6/14 UWorld Flashcards

1
Q

What causes autosplenectomy in sickle cell patients

A

Vaso-occlusion

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2
Q

Why might you see macrocytic anemia is sickle cell patients

A

Hemolytic anemia leads to increased erythrocyte turnover and increased folic acid requirement

So patients are prone to developing relative folic acid deficiency

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3
Q

Functional units of cerebellum and their corresponding deep nuclei

A
  • REMEMBER:
    • Lateral to medial = Don’t Eat Greasy Foods
      • Dentate
      • Emboliform
        • Emboliform + Globose = Interposed
      • Globose
      • Fastigial
  • Vestibulocerebellum
    • Vermis + flocculonodular (most medial)
    • = Fastigial deep nucleus
  • Spinocerebellum
    • Vermis + paravermis
    • = Interposed (Emboliform + Globose) deep nucleus
  • Cerebrocerebellum
    • Lateral hemisphere
    • = Dentate deep nucleus
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4
Q

What is the presentation and cause of hemiballismus

A

Sudden, wild flailing of half of body (1 arm +/- ipsilateral leg)

Seen in lesion of contralateral subthalamic nucleus

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5
Q

What are the layers that you go through during a spinal tap?

A

Skin - superficial fascia - supraspinous ligament - interspinous ligament - ligamentum flavum - epidural space - dura mater - subdural space - arachnoid membrane - subarachnoid space (this is where CSF is)

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6
Q

Define athetosis and it’s cause

A

Slow, writhing movements, especially seen in the fingers

Due to lesion of basal ganglia (Huntington’s)

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7
Q

Define chorea and its cause

A
  • Sudden, jerky, purposeless movements
  • Due to lesion of basal ganglia (e.g. Huntington’s)
  • Sydenham chorea seen in acute rheumatic fever
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8
Q

Define dystonia

A

Sustained, involuntary muscle contractions

Due to Writer’s cramp, blepharospasm, torticollis

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9
Q

Define akathisia and its cause

A
  • Dancing in place (voluntary) / Restlessness
    • THINK: Anthony saying “Ayy, Kayla” when she is drunk and dancing in place
  • Compulsion to move
  • Seen with neuroleptic use or in Parkinson’s
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10
Q

Define asterixis and its cause

A
  • Extension of wrists causes “flapping” motion
  • Associated with hepatic encephalopathy, Wilson disease, and other metabolic derangements
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11
Q

What part of the brain is affected in Huntington’s

A
  • Atrophy of caudate and putamen with hydrocephalus ex vacuo (ventricles appear dilated due to loss of surrounding brain tissue)
  • Symptoms:
    • Chorea, athetosis, aggression, depression, dementia
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12
Q

Identify the location of the following in the spinal cord:

Dorsal column

Spinothalamic tract

Corticospinal tract (lateral and anterior)

A
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13
Q

What part of the spinal cord is damaged in Werdnig-Hoffman disease

A

Anterior motor horn - LMN deficit with SYMMETRIC weakness

(vs. poliomyelitis which is LMN deficit with ASYMMETRIC weakness)

Floppy baby

THINK: Hoff = hop, so you know it is associated with polio (hopping on one leg)

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14
Q

What part of the spinal cord is affected/presentation of complete occlusion of the anterior spinal artery

A
  • Damage to everything except dorsal column
    • Spinothalamic damage = loss of bilateral pain and temperature below the lesion
    • Corticospinal damage = UMN below the lesion
    • Anterior horn damage = LMN deficit at the level of the lesion
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15
Q

What part of the spinal cord is damaged/presentation of ALS

A
  • Aka Lou Gehrig disease
  • Damage to anterior motor horn and lateral corticospinal tract
  • Presents with both UMN and LMN deficits
  • Lack of sensory impairment distinguishes from syringomyelia
  • Caused by defect in superoxide dismutase (O2- à H2O2)
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16
Q

Describe the findings in Brown-Sequard syndrome

(is each spinal pathway affected ipsilaterally or contralaterally)

A
  • Ipsilateral UMN signs below level of lesion (corticospinal tract damage)
  • Ipsilateral loss of tactile, vibration, proprioception sense below level of lesion (dorsal column)
  • Contralateral pain and temp loss 2-3 segments below level of lesion (spinothalamic tract)
    • 2-3 segments below because remember info travelled up 2-3 segments via Lissaur’s tract
  • Ipsilateral pain and temp loss at level of lesion
  • Ipsilateral LMN signs at level of lesion
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17
Q

Presentation of multiple sclerosis

A
  • Charcot triad of symptoms = SIN:
    • Scanning speech
    • Intention tremor, Incontinence, Internuclear ophthalmoplegia
    • Nystagmus
  • Hemiparesis, hemisensory symptoms
18
Q

Describe MRI and lumbar puncture of MS

A
  • MRI = gold standard
    • Periventricular plaques (areas of oligodendrocyte loss and reactive gliosis)
  • Lumbar puncture
    • Increased protein
    • Oligoclonal IgG bands
19
Q

Presentation of Friedrich ataxia

A
  • Degeneration of cerebellum and spinal cord
    • Ataxia, muscle weakness, loss of vibratory sense and proprioception, diabetes mellitus, hypertrophic cardiomyopathy
    • Presents in childood with kyphoscoliosis
  • THINK: Friedreich is fratastic (frataxin): he’s your favorite frat brother, always staggering and falling but has a sweet, big heart
20
Q

What are Osler nodes and what disease are they associated with

A

Painful red nodules on finger and toe pads

Associated with bacterial endocarditis

21
Q

What are the painless macules on palms and soles associated with bacterial endocarditis

A

Janeway lesions

22
Q

Derivatives of the aortic arches (1, 2, 3, 4, 6)

A
  • 1st:
    • Will degenerate
    • Part of maxillary artery
  • 2nd:
    • Will degenerate
    • Stapedial artery and hyoid artery
  • 3rd:
    • Common carotid artery and proximal part of internal carotids
  • 4th:
    • On left – aortic arch
    • On right – proximal part of R subclavian
  • 6th:
    • Proximal part of pulmonary arteries
    • On left – ductus arteriosus
23
Q

Describe the transition from fetal circulation to adult circulation (e.g. closure of shunts)

A

When the infant takes a breath, this decreases intrathoracic pressure, thus decreasing resistance in pulmonary vasculature

Decreased resistance leads to more blood entering the pulmonary artery (less leaving through ductus arteriosus), and thus more blood entering the LA

Increased LA pressure causes closure of the foramen ovale

Highly oxygenated blood in the aorta causes closure of ductus arteriosus

24
Q

Cardiac abnormalities associated with:

  • DiGeorge
  • Turner syndrome
  • Down syndrome
A
  • Turner syndrome:
    • Coarctation of aorta
    • Bicuspid aortic valve
  • Down syndrome
    • Endocardial cushion defect (ASD and VSD)
      • Complete atrioventricular canal defect
  • DiGeorge syndrome
    • Tetralogy of Fallot
    • Truncus arteriosus
25
Q

Cardiac disorder associated with Marfan’s

A

Aortic insufficiency due to abnormal aortic valve (e.g. aortic dissection and aneurysm)

26
Q

Cardiac abnormality associated with Tuberous sclerosis

A
  • Valvular obstruction due to cardiac rhabdomyomas
27
Q

Cardiac abnormality associated with infact of a diabetic mother

A
  • Transposition of great vessels
28
Q
A
29
Q

Describe heart sounds of ASD

A
  • Loud S1; wide, fixed split S2
    • Increased blood in R heart delays closure of pulmonary valve
30
Q

What drug do you use to keep the PDA open and to close it?

A

Indomethacin closes PDA

PGE keeps PDA open

31
Q

Location and presentation of infantile aortic coarctation

A
  • Coarctation is proximal to ductus arteriosus
    • Associated with PDA
  • Presentation:
    • Lower extremity cyanosis in infants – blood going to LE is deoxygenated (from RV to pulmonary artery to PDA)
  • Associated with Turner syndrome
32
Q

Location and presentation of adult aortic coarctation

A
  • Coarctation is distal to ductus arteriosus
  • Presentation:
    • HTN in upper extremities and hypotension with weak pulses in lower extremities
    • “Notching” of ribs on X-ray
      • Collateral arteries form and engorged intercostal erode ribs
    • Figure 3 sign on XR
33
Q

Complications of aortic coarctation

A
  • Heart failure, increased risk of cerebral hemorrhage, aortic rupture, endocarditis
34
Q

Describe the defect and required shunting in tricuspid atresia

A
  • Absence of tricuspid valve and hypoplastic RV
  • Requires 2 shunts:
    • ASD to get blood from RA to LA
    • VSD to get blood from LV to RV so it can go to the lungs
35
Q

Describe the defect in Total anomalous pulmonary venous return (TAPVR)

A
  • Pulmonary veins drain into R heart circulation (e.g. SVC, coronary sinus, RA)
  • Associated with ASD and sometimes PDA to allow for R-to-L shunting
36
Q

What are all the R-to-L shunt defects?

A
  • Early cyanosis – deoxygenated blood going to systemic circulation
  • Usually require urgent surgical treatment and/or maintenance of PDA
  • The 5 T’s
    • Truncus arteriosus (1 vessel)
    • Transposition (2 switched vessels)
    • Tricuspid atresia (3 = tri)
    • Tetralogy of Fallot (4 = tetra)
    • TAPVR (5 letters in the name)
    • Also Ebstein anomaly
37
Q

Equation for cardiac outpu

A
  • CO = Stroke volume (SV) x Heart rate (HR)
38
Q

Equation for mean arterial pressure (MAP)

A
  • MAP = CO (Q) x Total peripheral resistance (TPR)
  • MAP = (2/3 diastolic pressure) + (1/3 systolic pressure)
39
Q

How is CO maintained in early and sustained exercise?

A
  • Early exercise:
    • CO maintained due to increased HR and increased SV
  • Sustained exercise:
    • CO maintained due to increased HR
    • SV plateaus
40
Q

Describe presentation of Sturge Weber syndrome

A
  • Port-wine stain of the face in CN V1/V2 distribution
  • Seizures/epilepsy
  • Intellectual disability
  • Glaucoma
41
Q

Defect and presentation of multiple myeloma

A
  • Malignant proliferation of monoclonal plasma cell within the marrow
    • Recall: Plasma cells = mature B-cells that produce immunoglobulin
  • Classic presentation:
    • Anemia – plasma cells packed in bone marrow inhibit production of other cells
    • Renal insufficiency – excessive antibodies plug up kidney and form casts
    • Back pain – plasma cells stimulate osteoclasts
    • Hypercalcemia – plasma cells stimulate osteoclasts
42
Q

What disease is assoicated with thyroidization of the kidney

A

Chronic pyelonephritis

Eosinophilic casts dilate the tubules, causing the tubules to have a colloid/thyroid-like appearance