Week 1
Idiopathic Intracranial Hypertension can be due to a toxic level of which vitamin?
Vitamin A!
Bonus (+100): what's one food that is known for this?
Give and example that displays each of the following:
1. Rationalization
2. Intellectualization
3. Sublimation
nice
Will injury to the thalamus disrupt motor, sensory, or both?
Sensory only
Which type of collagen is defective in Classical EDS?
Type V
How many teeth does an adult human have?
32
For which enzymes is B1 (thiamine) a cofactor?
Be APT!
Branched-chain ketoacid dehydrogenase
Alpha-ketoglutarate dehydrogenase
Pyruvate dehydrogenase
Transketolase
A 5 year-old girl who had previously meeting all her developmental milestones has suddenly become much more dependent on her mother. You also note diffuse red marks over her hands from constant rubbing. What's your leading diagnosis?
Rett syndrome
A stroke to which artery would present as contralateral lower limb deficit, both sensory and motor?
ACA
In which childhood brain tumor would you expect to see Rosenthal Fibers on histo?
Pilocytic astrocytoma
What are the 3 muscles of mastication, and what innervates them?
Masseter, temporalis, pterygoid
V3
To which receptor in the CNS does Ketamine bind?
NMDA
Describe mood reactivity. What pathology is it indicative of?
The ability to have an elevated mood in response to positive events in the context of depression.
Indicates atypical depression.
Describe (or draw out) a lesion that could create Right Superior Homonymous Hemianopia
lesion of left temporal optic radiation (Meyer's loop)
(150) which two metabolic diseases feature a cherry red macula?
(150) how can you differentiate the two on physical exam?
Tay-Sachs and Niemann Pick
Niemann Pick will have hepatosplenomegaly, TS won't
Describe (or draw on the board) a step-by-step of the PLR
CNII -->PTN-->E-W nucleus-->parasympathetic fibers with CNIII-->ciliary ganglion-->ciliary muscle
What are the signs of MCAD deficiency (on labs), and why are the signs not usually seen immediately after birth?
BONUS +100: How would you differentiate this from a carnitine deficiency?
hypoketotic hypoglycemia
Dicarboxylic FAs
Signs not seen immediately because pt can still break down glucose just fine. Will only experience trouble when in a starvation state.
Both MCAD and carnitine deficiencies will present with hypoketotic hypoglycemia. However, MCAD will have the dicarboxylic FAs as well as high acylcarnitine levels.
(200) differentiate between NMS and SS
(200) which medication is used to rescue from each?
NMS: lead rigidity, areflexia
rescue with dantrolene, bromocriptidine
SS: Myoclonus, hyperreflexia
Rescue with ciproheptidine
Can you explain why a man who suffers a traumatic fracture of his temporal bone is out cold, then up again, then out cold again?
slices middle meningeal artery-->epidural hemorrhage
Out from trauma, then up, then down again due to ICP on his medullary drive center
What is the significance of a dilated pupil in cerebral aneurisms? What's the pathophysiology?
Indicates whether the aneurysm has ruptured or not!
If it hasn't ruptured, the aneurysm is simply pressing on the CNIII (which is surrounded by a sheath of parasympathetic efferents to the ciliary muscle). The 'pressing' will inhibit both the CNIII motor function and the parasympathetic efferent function, leaving the pupil dilated.
If the aneurysm ruptures, it causes an ischemia, but preferentially of the CNIII fibers (the parasympathetic fibers on the outside have their own vasa vasorum and will be fed). Therefore, the pupil will not be dilated.
A 20-month-old girl is brought to the emergency department for difficulty breathing. The patient has had rhinorrhea and nasal congestion for the past 2 days. She developed a cough and noisy breathing this evening. Medical history is otherwise negative. She is growing well, meeting milestones, and up-to-date on immunizations. On examination, the patient has suprasternal and intercostal retractions, inspiratory stridor, and a barking cough. Inflammation and edema of which of the following sites is most likely causing this patient's stridor?
1. Pharynx
2. Esophagus
3. Trachea
4. Epiglottis
3. Larynx
Croup causes Tracheal stenosis (steeple sign on X-ray)
A 5-month old boy is brought into he clinic due to poor feeding. His mother says he can't hold his head upright and his suckling is weak. He is SGA and head circumference is in a low percentile. All four limbs are hypotonic, and you notice cardiomegaly on CXR. A muscle biopsy shows fibers with lysosomes full of stuff that stains positive for PAS. Which enzyme is most likely deficient in this kid?
1. Glucocerebrocidase
2. Galactokinase
3. Alpha acid-glucokinase
4. Glycogen phosphorylase
4. Pyruvate Kinase
3. Alpha acid-glucokinase (AKA alpha 1,4 - glucosidase). The cardiomegaly is indicative of Pompe Disease.
A 25-year-old graduate student comes to the office for evaluation of anxiety. She describes episodes of sudden-onset palpitations, shortness of breath, and diaphoresis during which she wants to "run away before anyone notices." The patient arrives early for her classes to get a seat close to the door and has left during lectures due to feeling "out of control." There are no specific triggers for these events, but she is worried about having another episode next week during her final examinations. She does not drink alcohol or use illicit drugs. Physical examination, ECG, and laboratory evaluation are normal. The patient is diagnosed with panic disorder. After discussion of treatment options, she decides to pursue cognitive- behavioral therapy and start a short-term anxiolytic medication that also has muscle- relaxant and anticonvulsant properties. Which of the following best describes the mechanism of action of this medication?
A. Antagonism at GABA A benzodiazepine receptor sites
B. Increased duration of chloride channel opening
C. Increased frequency of chloride channel opening
D. Inhibition of serotonin and norepinephrine reuptake
E. Inhibition of serotonin reuptake
c. Benzos for acute management of panic disorder
Barbs not recommended bc of risk for respiratory depression
A 68-year-old man with a history of Parkinson disease is hospitalized for pneumonia and sepsis. The patient is treated with intravenous (IV) fluids and antibiotics. He is also started on an infusion of IV dopamine for hemodynamic support. Although the manifestations of Parkinson disease are attributed to dopaminergic neuron degeneration in the substantia nigra, IV dopamine does not improve this patient's Parkinson symptoms. Which of the following cell structures accounts for this lack of responsiveness?
A. Desmosomes
B. Fenestrae
C. Gap junctions
D. Hemidesmosomes
E. Intermediate junctions
F. Tight junctions
F. BBB is held together by tight junctions
a 61 year-old man is brought into the ED after being found on the sidewalk. He smells strongly of alcohol and vomit. Resuscitation is attempted, but it soon becomes apparent that he is gone, likely due to aspiration of vomit. On autopsy, the brain is dissected and it is found that the mammillary bodies are necroses, as well as many foci around the ventricles. The patient's brain findings are most likely associated with a decrease in which of the following?
1. Erythrocyte transketolase activity
2. Erythrocyte G6PD activity
3. Serum methylmalonic acid level
4. Serum lactate level
5. Serum NADH level
1. Thiamine deficiency from Wernicke-Korsakoff syndrome
A 3-year-old boy is brought to the clinic due to left ear pain over the past 3 days. Last week, the patient developed a runny nose and cough, which resolved 2 days prior to the development of ear pain. He has no chronic medical conditions. Temperature is 38.4°C (101.1°F). There is purulent drainage from the left ear. Otoscopic examination of the left ear reveals a normal-appearing external canal and an erythematous tympanic membrane with purulent fluid pooling within the distal external canal. Which of the following organisms is the most likely cause of this patient's diagnosis?
Answer choices:
A. Klebsiella pneumoniae
B. Moraxella catarrhalis
C. Proteus mirabilis
D. Pseudomonas aeruginosa
E. Staphylococcus aureus
F. Streptococcus pneumoniae
Strep pneumonia! The most common culprit of OM.
Pseudomonas is big for OE :(