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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.


A lesion involving which of the following anatomical structures is most likely responsible for this patient's symptoms?
Poll
  •   A. Cerebellopontine angle
  •   B. Foramen magnum
  •   C.Foramen ovale
  •   D.Foramen rotundum
  •   E. Hypoglossal canal
  •   F. Jugular foramen
2875 votes


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.


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.


Accumulation of which of the following metabolites is most likely present in this patient's tissues?
Poll
  •   A. Galactocerebroside
  •   B. Globotriaosylceramide
  •   C. Glucocerebroside
  •   D.Glycogen
  •   E. GM2 ganglioside
  •   F. Heparan sulfate
  •   G .Sphingomyelin
1904 votes


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.


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.


Which of the following microbial components is directly responsible for the severity of disease in this patient?
Poll
  •   A. Capsular polysaccharide
  •   B. lmmunoglobulin proteas
  •   C. Lipo-oligosaccharide
  •   D.Lipoteichoic acid
  •   E. Superantigen exotoxin
1924 votes


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.


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.


The patient most likely suffered from which of the following?
Poll
  •   A. Amyotrophic lateral sclerosis
  •   B. Poliomyelitis
  •   C.Rabies
  •   D.Huntington disease
  •   E. Friedreich ataxia
  •   F. Vitamin 812 deficiency
1597 votes


A Caucasian male who suffers from a severe neurological disease dies of an overwhelming respiratory infection. Autopsy shows an atrophic precentral gyrus and thin anterior roots of the spinal cord. Light microscopy reveals a severe loss of neurons in the anterior horn of the spinal cord and in the hypoglossal and ambiguous cranial nerve nuclei; corticospinal tracts stain only faintly, indicating demyelinization.


Educational objective: Osmotic demyelination syndrome results from overly rapid correction of chronic hyponatremia. Clinical features include quadriplegia, pseudobulbar palsy, and reduced level of consciousness. MRI demonstrates focal demyelination of the pons.


Which of the following most likely predisposed this patient to his current condition?
Poll
  •   A.Atheroma formation in the vertebrobasilar arteries
  •   B. Hematogenous dissemination of microbial agents
  •   C.Nutritional deficiency of water-soluble vitamin
  •   D.Overly rapid correction of electrolyte abnormalities
  •   E. Paraneoplastic production of anti-neuronal antibodies
1213 votes


A 54-year-old hospitalized man develops new-onset slurring of speech and extremity weakness. Medical history is significant for alcohol abuse. Physical examination reveals confusion, dysarthria, dysphagia, and quadriparesis. MRI of the brain shows symmetric areas of demyelination in the pons.


Educational objective: Tuberculous (TB) meningitis is characterized by formation of a thick, gelatinous exudate in the base of the brain; cerebral vasculitis; and hydrocephalus. It frequently presents with subacute, slowly progressive nausea, vomiting, fever, cranial nerve deficits, and strokes.


Which of the following is the most likely diagnosis?
Poll
  •   A. Herpes simplex infection
  •   B. Neurocysticercosis
  •   C.Primary CNS lymphoma
  •   D.Toxoplasmosis
  •   E. Tuberculosis infection
1441 votes


A 55-year-old man with HIV comes to the emergency department due to 3 weeks of increasing headache, fever, and vomiting. Examination shows low blood pressure and neck stiffness. The patient's condition quickly deteriorates, and he dies in the hospital despite appropriate treatment. Autopsy of the brain shows a diffuse gelatinous exudate covering the base of the brain. Cut sections of the brain show marked ventriculomegaly and frontal lobe infarcts but no intraparenchymal mass lesions.


Educational objective: Subdural hematoma occurs due to the rupture of cortical bridging veins. In young patients, it results from a fall or motor vehicle accident, and manifests with gradual onset of headache and confusion.
In elderly
patients it may occur after a minor trauma and present with a variety of neurologic symptoms. You should know how to recognize this on CT scan.

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