WBR0006

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Author PageAuthor::Anonymous (Edited by Will Gibson, Alison Leibowitz, and Yazan Daaboul)
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Microbiology
Sub Category SubCategory::Neurology
Prompt [[Prompt::A 32-year-old man is brought to the emergency department by his wife for progressive bilateral weakness, loss of sensation and distal paresthesia in his lower extremities over the past 5 days. The patient can no longer walk and must be carried onto the exam bed. He denies any recent history of trauma. The only illness he can recall is an episode of nausea, vomiting, and bloody diarrhea 3 weeks ago, from which he recovered spontaneously without medications. On exam, motor strength is 1/5 in both lower extremities with loss of deep tendon reflexes. What is the most likely organism responsible for this patient's condition?]]
Answer A AnswerA::''Streptococcus pyogenes''
Answer A Explanation [[AnswerAExp::Guillain-Barré syndrome is not commonly caused by Streptococcus pyogenes. S. pyogenes infection is associated with scarlet fever, rheumatic fever, pharyngitis, as well as many other clinical manifestations.]]
Answer B AnswerB::''Clostridium'' species
Answer B Explanation [[AnswerBExp::Clostridium species include:
  • Clostridium botulinum: Associated with laccid paralysis due to toxin
  • Clostridium difficile: Associated with pseudomembranous colitis.
  • Clostridium tetani. Associated with spastic paralysis. Toxin inhibits the release of inhibitory neurotransmitters.

None of these species are most commonly associated with the development of Guillain-Barré syndrome.]]

Answer C AnswerC::''Staphlococcus aureus''
Answer C Explanation [[AnswerCExp::Guillain-Barré syndrome is not commonly cause by Staphylococcus aureus. S. aureus is a gram positive cocci that is a component of normal skin flora. However, it commonly causes rapid onset food poisoning, cellulitis and pneumonia. Rarer conditions caused by S. aureus include toxic shock syndrome,and scalded skin syndrome.]]
Answer D AnswerD::''Campylobacter'' species
Answer D Explanation [[AnswerDExp::Campylobacter jejuni is one of the most common infections associated with Guillain-Barré syndrome.]]
Answer E AnswerE::''Neisseria meningitidis''
Answer E Explanation [[AnswerEExp::Neisseria meningitidis is not associated with the development of Guillain-Barré syndrome. Neisseria meningitidis is an encapsulated, gram-negative diplococcus that ferments maltose. Neisseria meningitidis typically causes meningitis in adults.]]
Right Answer RightAnswer::D
Explanation [[Explanation::This patient has symptoms and signs consistent withGuillain-Barré syndrome (GBS). GBS is an autoimmune peripheral neuropathy characterized by an acute areflexic paralysis that usually occurs several weeks after a self-limited upper respiratory tract or abdominal infection. It is nowadays considered the most common cause of acute flaccid paralysis. The pathogenesis involves segmental demyelination and secondary axonal degeneration due to infiltration of T-cells and macrophages, complement activation, and antibody formation against Schwann cells by the process of molecular mimicry. It is a neurological emergency, in which the patient must be monitored in an intensive care setting because of its high likelihood of progression to diaphragmatic paralaysis and respiratory failure. Mechanical ventilation is eventually required in up to 30% of patients. Although generally regarded to have a good prognosis, approximately 20% of patients suffer permanent disability, and approximately 5% die despite appropriate therapy. GBS is thought to be caused by a Type IV hypersensitivity reaction in which schwann cells are targeted for destruction. Most patients recover spontaneously from the disease. Miller Fisher syndrome is an important variant of GBS; it is characterized by the presence of ophthalmoplegia, ataxia, and areflexia. Diagnosis is often suspected by the presence of symptoms and signs, and is confirmed by nerve conduction studies. In suspected cases of GBS, a lumbar puncture is frequently performed to rule out infectious etiologies of neuropathies. Classically, the lumbar puncture among GBS patients reveals albuminocytologic dissociation that is found in approximately half of patients within the first week of illness. Immunotherapy by plasma exchange or IVIg. The use of steroids is not effective.

Campylobacter species is one of the most common organisms involved in the development of GBS; it is associated with 30% of GBS cases. Campylobacter jejuni is a comma-shaped gram-negative oxidase-positive rod that is considered a major cause of infectious bloody diarrhea. It is transmitted by the fecal-oral route. Spoiled poultry, meat, and unpasteurized milk are common sources of Campylobacter jejuni gastrointestinal infection. Campylobacter jejuni is known for its ability to grow at 42oC. Other less common infectious agents associated with GBS include CMV, EBV, VZV, and Mycoplasma pneumoniae
Educational Objective: Campylobacter jejuni is the most common cause of Guillain-Barré syndrome
References: Yuki N, Hartung H. Guillain-Barre syndrome. N Engl J Med. 2012;366:2294-304 First Aid 2014: page 484 First Aid 2013: page 444]]

Approved Approved::Yes
Keyword WBRKeyword::Neurology, WBRKeyword::Neuropathy, WBRKeyword::Paralysis, WBRKeyword::Hypersensitivity, WBRKeyword::Autoimmune, WBRKeyword::Schwann, WBRKeyword::Guillain, WBRKeyword::Barre, WBRKeyword::Syndrome, WBRKeyword::Campylobacter, WBRKeyword::jejuni, WBRKeyword::gastroenteritis, WBRKeyword::bloody, WBRKeyword::diarrhea
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