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Old 11-10-2012
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Arrow Billy Step 1 Questions # 79

A 32-year-old woman presents to the physician with worsening anesthesia, weakness of both upper extremities, and headaches. The patient has no prior significant medical history and cannot recall any recent episodes of trauma. Physical examination is notable for the absence of motor deficits in both upper extremities, but positive for the absence of pain and temperature sensation and reflexes in both upper extremities. Position and vibration sense are intact in the upper extremities and there are no lower extremity abnormalities. An MRI of the spinal column shows dilation within the cervical spinal cord. Which of the following diagnoses is consistent with this patient’s fi ndings?

(A) Arnold-Chiari I malformation
(B) Communicating hydrocephalus
(C) Congenital aqueductal stenosis
(D) Dandy-Walker syndrome
(E) Wallenberg’s (lateral medullary) syndrome
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Old 11-10-2012
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clinical pic. s/o SYRINGOMYELIA..Usually a/w Arnold chiari II :+ AQUEDUCTAL STENOSIS.. So thinking of C. congenital aqueductal stenosis: become evident in adulthood
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Old 11-10-2012
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Default AA?

Arnold chiari type 1 manifests in adulthood?
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Old 11-10-2012
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confused..I think A)ARNOLD CHIARI 1 ..ADULTHOOD presentation,syringomyelia?
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Old 11-10-2012
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I choose A
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Default c?

I would also have gone with option A, if it was Arnold Chiari II instead of I. As far as I remember, Arnold Chiari I is asymptomatic. I think
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Default my answer :)

(C) Congenital aqueductal stenosis - Arnold-Chiari t.2
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Old 11-11-2012
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Correct Answer A is correct

The history, physical exam, and MRI findings are consistent with the diagnosis of syringomyelia, which may be primary (Arnold-Chiari malformation) or acquired as a result of trauma, tumor, or infl ammation. Arnold-Chiari malformations involve downward herniation of the cerebellar tonsils into the foramen magnum and are often associated with syringomyelia. Type II Arnold-Chiari malformations are always associated with spina bifida, whereas type I malformations are not. Syringomyelia consists of an enlargement of the central canal of the spinal cord, most commonly occurring at C8-T1. Crossing fibers of the spinothalamic tract are damaged (loss of pain and temperature sensation) with preserved dorsal column function (intact position and vibration sense)

Answer B is incorrect. Communicating hydrocephalus occurs as a result of a blockage of cerebrospinal fluid outside the brain and is typically
associated with malfunctioning arachnoid villi. This diagnosis would show ventricular enlargement on MRI

Answer C is incorrect. Congenital aqueductal stenosis is a common cause of congenital hydrocephalus and would present very early in life.

Answer D is incorrect. Dandy-Walker syndrome is a congenital noncommunicating hydrocephalus typically associated with a cluster
of abnormal fi ndings that include abnormal formation of the cerebellar vermis. This condition leads to obstruction of the outlet foramina of Luschka and Magendie.

Answer E is incorrect. Lateral medullary syndrome, or Wallenberg’s syndrome, usually occurs as a result of occlusion of one of the posterior
inferior cerebellar arteries. It presents with loss of pain and temperature sensations over the contralateral side of the body and the ipsilateral face. In this case, the patient’s symptoms show left-right symmetry.
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