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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor={{Rim}}
|QuestionAuthor={{Rim}}, {{AJL}} {{Alison}}
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Pathology
|MainCategory=Pathology
Line 20: Line 20:
|MainCategory=Pathology
|MainCategory=Pathology
|SubCategory=Neurology
|SubCategory=Neurology
|Prompt=A 42 year old patient with a past medical history significant for coronary artery disease, hypertension, hyperlipidemia, and type 2 diabetes mellitus, is brought to the emergency department with acute flaccid paralysis.  The family explains that the patient has had a recent bloody diarrhea.  Cerebrospinal fluid (CSF) analysis shows high levels of proteins and normal cell count.  Which of the following pathologies corresponds to a similar immunological reaction as that observed in the patient?
|Prompt=A 42-year-old male, with a medical history of coronary artery disease, hypertension, hyperlipidemia, and type 2 diabetes mellitus, is brought to the emergency room 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?
|Explanation=Guillain-Barre syndrome (GBS) is an auto-immune disease characterized by a type IV hypersensitivity caused by antibodies against Schwann cells in the peripheral nervous system.  The most common presentation of GBS is a symmetric paralysis that usually starts in the distal lower extremities and gradually becomes more proximal.  On physical examination, patients with GBS generally have areflexia in the lower extremities.  CSF shows 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 good prognosis, recent research has shown that GBS prognosis is not as good as first believed.  20% of affected patients remain severely disabled with a death rate reaching 5%.
|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 is believed to be a pathology that subsequently develops following a recent diarrhea or upper respiratory tract infection.  The most common organism associated with GBS is Campylobacter jejune in 30% of the cases, followed by cytomegalovirus (CMV) in 10%. Other less common infectious agents associated with GBS are: Epstein-Barr virus (EBV), varicella-zoster virus (VZV), and mycoplasma pneumoniae.  
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]]''.  


Among the list of possible options, only GVHD is a type IV hypersensitivity reaction, similar to GBS.
GVHD is a type IV hypersensitivity reaction, similar to GBS.


Educational Objective: Guillain-Barre syndrome is a type IV hypersensitivity that usually follows a diarrhea or upper respiratory tract infection following C. jejuni infection. It is characterized by flaccid paralysis with areflexia on physical examination and albuminocytologic dissociation on CSF analysis.
|EducationalObjectives= 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.


Reference: Yuki N, Hartung HP. Guillain-Barre syndrome. N Eng J Med. 2012; 366:2294-304
|References= Yuki N, Hartung HP. Guillain-Barre syndrome. N Eng J Med. 2012; 366:2294-304


|AnswerA=Autoimmune hemolytic anemia (AIHA)
|AnswerA=Autoimmune hemolytic anemia (AIHA)
|AnswerAExp=AIHA is a type II hypersensitivity.
|AnswerAExp=AIHA is a type II hypersensitivity.
|AnswerB=Systemic lupus erythematosus (SLE)
|AnswerB=Systemic lupus erythematosus (SLE)
|AnswerBExp=SLE is a type III hypersensitivity.
|AnswerBExp=SLE is type a II and III hypersensitivity.
|AnswerC=Goodpasture's syndrome
|AnswerC=Goodpasture's syndrome
|AnswerCExp=Goodpasture's syndrome is a type II hyersensitivity.
|AnswerCExp=Goodpasture's syndrome is a type II hyersensitivity.
|AnswerD=Graft vs. host disease (GVHD)
|AnswerD=Graft vs. host disease (GVHD)
|AnswerDExp=GVHD is a type IV hypersensitivity, similar to Guillain-Barre syndrome.
|AnswerDExp=GVHD is a type IV hypersensitivity, similar to Guillain-Barre syndrome.
|AnswerE=Post-streptococcal glomerulonephritis (PSGN)
|AnswerE=Post-streptococcal glomerulonephritis (PSGN)
|AnswerEExp=PSGN is a type III hypersensitivity.
|AnswerEExp=PSGN is a type III hypersensitivity.
|RightAnswer=D
|RightAnswer=D
|WBRKeyword=guillain, barre, syndrome, guillain barre syndrome, guillain-barre syndrome, c. jejuni, campylobacter, jejuni, campylobacter jejuni, albuminocytologic, dissociation, albuminocytologic dissociation, CSF, cerebrospinal fluid, flaccid, paralysis, flaccid paralysis, areflexia
|WBRKeyword= guillain-barre syndrome, campylobacter jejuni, albuminocytologic dissociation, CSF, cerebrospinal fluid, flaccid paralysis, areflexia, hypersensitivity, infection, microbiology
|Approved=No
|Approved=Yes
}}
}}

Revision as of 18:27, 31 July 2014

 
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 male, with a medical history of coronary artery disease, hypertension, hyperlipidemia, and type 2 diabetes mellitus, is brought to the emergency room 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
Linked Question Linked::
Order in Linked Questions LinkedOrder::