6/14 UWorld

Question 1

What causes autosplenectomy in sickle cell patients

Answer 1

Vaso-occlusion

Question 2

Why might you see macrocytic anemia is sickle cell patients

Answer 2

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

So patients are prone to developing relative folic acid deficiency

Question 3

Functional units of cerebellum and their corresponding deep nuclei

Answer 3

• 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

Question 4

What is the presentation and cause of hemiballismus

Answer 4

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

Seen in lesion of contralateral subthalamic nucleus

Question 5

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

Answer 5

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

Question 6

Define athetosis and it’s cause

Answer 6

Slow, writhing movements, especially seen in the fingers

Due to lesion of basal ganglia (Huntington’s)

Question 7

Define chorea and its cause

Answer 7

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

Question 8

Define dystonia

Answer 8

Sustained, involuntary muscle contractions

Due to Writer’s cramp, blepharospasm, torticollis

Question 9

Define akathisia and its cause

Answer 9

• 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

Question 10

Define asterixis and its cause

Answer 10

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

Question 11

What part of the brain is affected in Huntington’s

Answer 11

• 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

Question 12

Identify the location of the following in the spinal cord:

Dorsal column

Spinothalamic tract

Corticospinal tract (lateral and anterior)

Answer 12

Question 13

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

Answer 13

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)

Question 14

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

Answer 14

• 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

Question 15

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

Answer 15

• 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)

Question 16

Describe the findings in Brown-Sequard syndrome

(is each spinal pathway affected ipsilaterally or contralaterally)

Answer 16

• 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

Question 17

Presentation of multiple sclerosis

Answer 17

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

Question 18

Describe MRI and lumbar puncture of MS

Answer 18

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

Question 19

Presentation of Friedrich ataxia

Answer 19

• 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

Question 20

What are Osler nodes and what disease are they associated with

Answer 20

Painful red nodules on finger and toe pads

Associated with bacterial endocarditis

Question 21

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

Answer 21

Janeway lesions

Question 22

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

Answer 22

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

Question 23

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

Answer 23

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

Question 24

Cardiac abnormalities associated with:

• DiGeorge
• Turner syndrome
• Down syndrome

Answer 24

• 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

Question 25

Cardiac disorder associated with Marfan's

Answer 25

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

Question 26

Cardiac abnormality associated with Tuberous sclerosis

Answer 26

* Valvular obstruction due to cardiac rhabdomyomas

Question 27

Cardiac abnormality associated with infact of a diabetic mother

Answer 27

* Transposition of great vessels

Question 29

Describe heart sounds of ASD

Answer 29

* Loud S1; wide, fixed split S2 * Increased blood in R heart delays closure of pulmonary valve

Question 30

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

Answer 30

Indomethacin closes PDA PGE keeps PDA open

Question 31

Location and presentation of infantile aortic coarctation

Answer 31

* 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

Question 32

Location and presentation of adult aortic coarctation

Answer 32

* 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

Question 33

Complications of aortic coarctation

Answer 33

* Heart failure, increased risk of cerebral hemorrhage, aortic rupture, endocarditis

Question 34

Describe the defect and required shunting in tricuspid atresia

Answer 34

* 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

Question 35

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

Answer 35

* 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

Question 36

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

Answer 36

* 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

Question 37

Equation for cardiac outpu

Answer 37

* CO = Stroke volume (SV) x Heart rate (HR)

Question 38

Equation for mean arterial pressure (MAP)

Answer 38

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

Question 39

How is CO maintained in early and sustained exercise?

Answer 39

* Early exercise: * CO maintained due to increased HR and increased SV * Sustained exercise: * CO maintained due to increased HR * SV plateaus

Question 40

Describe presentation of Sturge Weber syndrome

Answer 40

* Port-wine stain of the face in CN V1/V2 distribution * Seizures/epilepsy * Intellectual disability * Glaucoma

Question 41

Defect and presentation of multiple myeloma

Answer 41

* 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

Question 42

What disease is assoicated with thyroidization of the kidney

Answer 42

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