WBR0542

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Author [[PageAuthor::Rim Halaby, M.D. [1], Alison Leibowitz [2] (Reviewed by Alison Leibowitz)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathology
Sub Category SubCategory::Neurology
Prompt [[Prompt::A 42-year-old man, with a medical history of coronary artery disease, hypertension, hyperlipidemia, and type 2 diabetes mellitus, is brought to the emergency department with acute flaccid paralysis. The patient recently had bloody diarrhea and cerebrospinal fluid (CSF) analysis demonstrates high levels of proteins and a normal cell count. Which of the following pathologies corresponds to a similar immunological reaction?]]
Answer A AnswerA::Autoimmune hemolytic anemia (AIHA)
Answer A Explanation AnswerAExp::AIHA is a type II hypersensitivity.
Answer B AnswerB::Systemic lupus erythematosus (SLE)
Answer B Explanation AnswerBExp::SLE is type a II and III hypersensitivity.
Answer C AnswerC::Goodpasture's syndrome
Answer C Explanation AnswerCExp::Goodpasture's syndrome is a type II hyersensitivity.
Answer D AnswerD::Graft vs. host disease (GVHD)
Answer D Explanation AnswerDExp::GVHD is a type IV hypersensitivity, similar to Guillain-Barre syndrome.
Answer E AnswerE::Post-streptococcal glomerulonephritis (PSGN)
Answer E Explanation AnswerEExp::PSGN is a type III hypersensitivity.
Right Answer RightAnswer::D
Explanation [[Explanation::Guillain-Barre syndrome (GBS), an auto-immune disease, is characterized by a type IV hypersensitivity caused by antibodies against Schwann cells in the peripheral nervous system. GBS frequently manifests with a symmetric paralysis that usually starts in the distal lower extremities and gradually becomes more proximal. Upon physical examination, patients with GBS often have areflexia in the lower extremities. CSF demonstrates albuminocytologic dissociation, defined as a high level of CSF proteins and normal cell counts. Although GBS is generally regarded as a benign condition with a positive prognosis, 20% of affected patients remain severely disabled with a death rate reaching 5%.

GBS frequently develops following diarrhea or upper respiratory tract infection. The most common organism associated with GBS is Campylobacter jejuni in 30% of the cases, followed by cytomegalovirus (CMV) in 10%. Less common infectious agents associated with GBS are Epstein-Barr virus (EBV), varicella-zoster virus (VZV), and Mycoplasma pneumoniae.

GVHD is a type IV hypersensitivity reaction, similar to GBS.
Educational Objective: GBS frequently develops following diarrhea or upper respiratory tract infection. The most common organism associated with GBS is Campylobacter jejuni. GBS is characterized by flaccid paralysis with areflexia upon physical examination, and albuminocytologic dissociation upon CSF analysis.
References: Yuki N, Hartung HP. Guillain-Barre syndrome. N Eng J Med. 2012; 366:2294-304]]

Approved Approved::Yes
Keyword WBRKeyword::guillain-barre syndrome, WBRKeyword::campylobacter jejuni, WBRKeyword::albuminocytologic dissociation, WBRKeyword::CSF, WBRKeyword::cerebrospinal fluid, WBRKeyword::flaccid paralysis, WBRKeyword::areflexia, WBRKeyword::hypersensitivity, WBRKeyword::infection, WBRKeyword::microbiology
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