The patient most likely suffered from which of the following?
Anonymous Quiz
50%
A. Amyotrophic lateral sclerosis
18%
B. Poliomyelitis
8%
C.Rabies
11%
D.Huntington disease
5%
E. Friedreich ataxia
7%
F. Vitamin 812 deficiency
Educational objective: Amyotrophic lateral sclerosis (ALS) causes both upper and lower motor neuron lesions. Loss of neurons of the anterior horns of the spinal cord (LMN lesion) causes muscle weakness and atrophy. Demyelination of the lateral corticospinal tract (UMN lesion) leads to spasticity and hyperretlexia.
A 16-year-old girl is brought to the emergency department after she became unresponsive. Temperature is 39.7 C (103.5 F), blood pressure is 70/40 mm Hg, and pulse is 130/min and thready. There is a diffuse petechial and ecchymotic skin rash. Laboratory testing reveals leukocytosis with left shift and evidence of disseminated intravascular coagulation and multiorgan failure. Despite aggressive interventions, the patient dies several hours after admission. Autopsy reveals hemorrhagic necrosis of many internal organs, including the bilateral adrenal glands.
Which of the following microbial components is directly responsible for the severity of disease in this patient?
Anonymous Quiz
22%
A. Capsular polysaccharide
15%
B. lmmunoglobulin proteas
30%
C. Lipo-oligosaccharide
10%
D.Lipoteichoic acid
22%
E. Superantigen exotoxin
Educational objective: Neisseria meningitidis can cause sepsis and circulatory collapse in previously healthy young individuals. Lipooligosaccharide, a virulence factor in the pathogens outer membrane, is the major underlying cause of disease severity.
An 8-month-old girl is brought to the office for evaluation of irritability and regression of motor skills. Her birth was unremarkable and she appeared to develop normally, but she can no longer sit or roll over. Her parents have also noticed that she startles easily with loud noises. Head circumference measurement is consistent with macrocephaly. Bilateral funduscopic evaluation shows a bright red fovea centralis that is surrounded by a contrasting white macula. Peripheral vision is decreased. Abdominal examination is normal.
Accumulation of which of the following metabolites is most likely present in this patient's tissues?
Anonymous Quiz
16%
A. Galactocerebroside
7%
B. Globotriaosylceramide
13%
C. Glucocerebroside
10%
D.Glycogen
34%
E. GM2 ganglioside
6%
F. Heparan sulfate
13%
G .Sphingomyelin
Educational objective: Tay-Sachs disease is an autosomal recessive disorder caused by [3-hexosaminidase A deficiency, which results in GM2 ganglioside accumulation. Key clinical features include progressive neurodegeneration and a cherry-red macular spot. In contrast to patients with Niemann-Pick disease, those with Tay Sachs disease have no hepatosplenomegaly.
A 67-year-old woman with a known history of lung cancer comes to the office due to hoarseness and
difficulty swallowing. She has no disturbances in vision or hearing. On examination, there is loss of the gag reflex on the left side; when the patient is prompted to say "ah," the uvula deviates to the right side.
a right lower lobe lung mass and several osteolytic rib lesions. MRI of the head also demonstrates multiple Her left shoulder is drooped and strength is reduced during left shoulder shrug testing. Chest x-ray shows
lesions consistent with metastasis.
difficulty swallowing. She has no disturbances in vision or hearing. On examination, there is loss of the gag reflex on the left side; when the patient is prompted to say "ah," the uvula deviates to the right side.
a right lower lobe lung mass and several osteolytic rib lesions. MRI of the head also demonstrates multiple Her left shoulder is drooped and strength is reduced during left shoulder shrug testing. Chest x-ray shows
lesions consistent with metastasis.
A lesion involving which of the following anatomical structures is most likely responsible for this patient's symptoms?
Anonymous Quiz
16%
A. Cerebellopontine angle
13%
B. Foramen magnum
10%
C.Foramen ovale
7%
D.Foramen rotundum
23%
E. Hypoglossal canal
31%
F. Jugular foramen
Educational objective: Lesions of the jugular foramen can result in jugular foramen (Vernet) syndrome, which is characterized by the dysfunction of cranial nerves IX, X, and XI. Symptoms include dysphagia, hoarseness, loss of gag reflex on the ipsilateral side, and deviation of the uvula toward the normal side.
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