WBR0296

Author	[[PageAuthor::Ogheneochuko Ajari, MB.BS, MS [1]]]
Exam Type	ExamType::USMLE Step 1
Main Category	MainCategory::Embryology
Sub Category	SubCategory::Neurology
Prompt	[[Prompt::A 32-year old male presents to the physician’s office with complaints of progressive numbness of the arms, weakness and hiccups. Past medical history is unremarkable. Physical examination reveals bilateral loss of pain and temperature sensation in the upper extremities, with preservation of touch sensation. What is the most common associated finding of this condition?]]
Answer A	AnswerA::Ependymoma
Answer A Explanation	[[AnswerAExp::Ependymoma is a cause of syringomyelia but not the most common cause or associated finding. Ependymomas are also seen with Neurofibromatosis type II]]
Answer B	AnswerB::Chiari II malformation
Answer B Explanation	AnswerBExp::See explanation
Answer C	AnswerC::Thoracolumbar myelomeningocele
Answer C Explanation	AnswerCExp::Thoracolumbar myelomeningocele is associated with Chiari II malformation and not syringomyelia
Answer D	AnswerD::Holoprosencephaly
Answer D Explanation	[[AnswerDExp::Holoprosencephaly is associated with Patau syndrome, severe fetal alcohol syndrome and cleft lip/palate]]
Answer E	AnswerE::Duodenal atresia
Answer E Explanation	AnswerEExp::Duodenal atresia is associated with trisomy 21 and not syringomyelia
Right Answer	RightAnswer::B
Explanation	[[Explanation::This is a case of Syringomyelia and the most commonly associated finding is Chiari II malformation or Arnold-Chiari malformation. 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. Educational Objective: Chiari II malformation is the most common associated finding in syringomyelia References: First AID for the USMLE Step 1 Pg 127]]
Approved	Approved::Yes
Keyword	WBRKeyword::Malformations, WBRKeyword::Loss of temperature sensation, WBRKeyword::Loss of pain sensation, WBRKeyword::Genetics, WBRKeyword::Syringomyelia, WBRKeyword::Brain malformation, WBRKeyword::Congential malformation
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