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|SubCategory=Neurology
|SubCategory=Neurology
|Prompt=A 30-year-old female presents to the ER with complaints of a worsening headache, leg weakness, difficulty with swallowing, and progressive numbness of both hands. Physical examination reveals a bilateral loss of pain and temperature sensation in the upper extremities, with preserved touch sensation. An MRI reveals [[hydrocephalus]]. Which of the following is the most likely cause of this patient’s symptoms?
|Prompt=A 30-year-old female presents to the ER with complaints of a worsening headache, leg weakness, difficulty with swallowing, and progressive numbness of both hands. Physical examination reveals a bilateral loss of pain and temperature sensation in the upper extremities, with preserved touch sensation. An MRI reveals [[hydrocephalus]]. Which of the following is the most likely cause of this patient’s symptoms?
|Explanation=The patient in this scenario likely has communicating [[syringomyelia]], where there is a path from the 4th ventricle to the central canal. CSF flows from the 4th ventricle to the central canal and a syrinx develops very rapidly.  The lower part of the cerebellum protrudes from its normal location in the back of the head in the cervical or neck portion of the spinal canal. A syrinx may develop in the cervical region of the spinal cord, interrupting the decussating spinothalamic fibers that mediate pain and temperature sensibility, resulting in the loss of these sensations, while light touch, vibration and position senses are preserved. The syrinx may extend into the medulla, producing a [[syringobulbia]] that could cause [[dysphagia]].
|Explanation=The patient in this scenario likely has communicating [[syringomyelia]], manifesting in the path from the 4th ventricle to the central canal. A rapidly developing syrinx blocks the flow of [[cerebrospinal fluid]] (CSF) from the 4th ventricle to the central canal and the lower part of the cerebellum protrudes from its normal location in the back of the head into the cervical or neck portion of the spinal canal. A syrinx may develop in the cervical region of the spinal cord, interrupting the decussating spinothalamic fibers that mediate pain and temperature sensibility, resulting in the loss of these sensations, while the gentle touch, vibration, and position senses are preserved. The syrinx may extend into the medulla, producing a [[syringobulbia]] that could result in [[dysphagia]].


Educational Objective
|EducationalObjectives= [[Syringomyelia]] can manifest in the bilateral loss of pain and temperature sensations and the preservation of touch sensation.


Syringomyelia is associated with bilateral loss of pain and temperature sensations with preservation of touch sensation
|References= First AID for the USMLE Step 1 2011 Pg 127
 
Reference: First AID for the USMLE Step 1 2011 Pg 127


|AnswerA=[[Ependymoma]]
|AnswerA=[[Ependymoma]]
 
|AnswerAExp= [[Ependymoma]], an ependymal cell tumor, is most commonly found in the 4th ventricle. It can cause hydrocephalus but will not typically manifest with the bilateral loss of pain and temperature sensation and the preservation of touch sensation.
 
 
 
 
|AnswerAExp=Incorrect. Ependymoma is an ependymal cell tumor most commonly found in the 4th ventricle. It can cause hydrocephalus but would not present typically with bilateral loss of pain and temperature sensation and preservation of touch sensation
 
 
|AnswerB=[[Diabetic neuropathy]]
|AnswerB=[[Diabetic neuropathy]]
|AnswerBExp=Incorrect. Diabetic neuropathy is associated with reduced ability to feel temperature or pain sensations and not a complete loss of pain and temperature sensation
|AnswerBExp= [[Diabetic neuropathy]] is associated with a reduced ability to feel temperature or pain sensations, but not a complete loss of pain and temperature sensations.
|AnswerC=[[Meningioma]]
|AnswerC=[[Meningioma]]
|AnswerCExp=Incorrect. Meningioma is associated with progressive spastic weakness in legs, increased intracranial pressure and focal seizures
|AnswerCExp= [[Meningioma]] is associated with a progressive spastic weakness in legs, increased intracranial pressure, and focal seizures.
 
 
|AnswerD=[[Spinal muscular atrophy]]
|AnswerD=[[Spinal muscular atrophy]]
|AnswerDExp=Incorrect. Spinal muscular atrophy is associated with muscle weakness, poor muscle tone, limpness or a tendency to flop (the "floppy baby" syndrome)
|AnswerDExp= [[Spinal muscular atrophy]] is associated with muscle weakness, poor muscle tone, limpness, or a tendency to flop (the "floppy baby" syndrome).
|AnswerE=[[Syringomyelia]]
|AnswerE=[[Syringomyelia]]
|AnswerEExp=Correct. See explanation
|AnswerEExp= See explanation
|RightAnswer=E
|RightAnswer=E
|WBRKeyword=Hydrocephalus; Loss of temperature sensation; Loss of pain sensation
|WBRKeyword=Hydrocephalus; Loss of temperature sensation; Loss of pain sensation
|Approved=Yes
|Approved=Yes
}}
}}

Revision as of 14:58, 9 July 2014

 
Author [[PageAuthor::Ogheneochuko Ajari, MB.BS, MS [1] (Reviewed by Alison Leibowitz)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Embryology
Sub Category SubCategory::Neurology
Prompt [[Prompt::A 30-year-old female presents to the ER with complaints of a worsening headache, leg weakness, difficulty with swallowing, and progressive numbness of both hands. Physical examination reveals a bilateral loss of pain and temperature sensation in the upper extremities, with preserved touch sensation. An MRI reveals hydrocephalus. Which of the following is the most likely cause of this patient’s symptoms?]]
Answer A [[AnswerA::Ependymoma]]
Answer A Explanation [[AnswerAExp::Ependymoma, an ependymal cell tumor, is most commonly found in the 4th ventricle. It can cause hydrocephalus but will not typically manifest with the bilateral loss of pain and temperature sensation and the preservation of touch sensation.]]
Answer B [[AnswerB::Diabetic neuropathy]]
Answer B Explanation [[AnswerBExp::Diabetic neuropathy is associated with a reduced ability to feel temperature or pain sensations, but not a complete loss of pain and temperature sensations.]]
Answer C [[AnswerC::Meningioma]]
Answer C Explanation [[AnswerCExp::Meningioma is associated with a progressive spastic weakness in legs, increased intracranial pressure, and focal seizures.]]
Answer D [[AnswerD::Spinal muscular atrophy]]
Answer D Explanation [[AnswerDExp::Spinal muscular atrophy is associated with muscle weakness, poor muscle tone, limpness, or a tendency to flop (the "floppy baby" syndrome).]]
Answer E [[AnswerE::Syringomyelia]]
Answer E Explanation AnswerEExp::See explanation
Right Answer RightAnswer::E
Explanation [[Explanation::The patient in this scenario likely has communicating syringomyelia, manifesting in the path from the 4th ventricle to the central canal. A rapidly developing syrinx blocks the flow of cerebrospinal fluid (CSF) from the 4th ventricle to the central canal and the lower part of the cerebellum protrudes from its normal location in the back of the head into the cervical or neck portion of the spinal canal. A syrinx may develop in the cervical region of the spinal cord, interrupting the decussating spinothalamic fibers that mediate pain and temperature sensibility, resulting in the loss of these sensations, while the gentle touch, vibration, and position senses are preserved. The syrinx may extend into the medulla, producing a syringobulbia that could result in dysphagia.

Educational Objective: Syringomyelia can manifest in the bilateral loss of pain and temperature sensations and the preservation of touch sensation.
References: First AID for the USMLE Step 1 2011 Pg 127]]

Approved Approved::Yes
Keyword WBRKeyword::Hydrocephalus; Loss of temperature sensation; Loss of pain sensation
Linked Question Linked::
Order in Linked Questions LinkedOrder::