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Reply from the authors

  • Albert J. Schuette, Emory University School of Medicine, 1365 Clifton Rd NE Atlanta GA 30312ajschue@gmail.com
  • Jason Taub (Atlanta, GA; jstaub@emory.edu), Jeffery J. Olson (Atlanta, GA; jolson@emory.edu)
Submitted December 21, 2010

We thank Schott et al. for their interest and appreciate their providing an algorithm to approach this difficult-to-manage patient population. [3]

We reviewed the subset of our patients managed from 2004 to 2008 (n=28), which parallels the population studied by Schott et al. We found some interesting similarities. We would like to mention that all patients included in the study had rapidly progressive dementia. To further clarify, 63% of the patients presented initially with this symptom but ultimately all developed this symptom.

Between 2004 and 2008, we biopsied 28 of 51 patients. [1] Thirteen of the 28 biopsies were diagnostic (46.4%) and the most common diagnosis was CJD (46.1%), which was similar to Schott et al.'s finding of 47%. From 2004 to 2008, the NMDA and voltage gated potassium channel were not routinely part of the paraneoplastic panels sent out from our institution and therefore not ordered. Since 2008, these tests have become commercially available and are now routinely ordered on our patients. We agree that these tests would be an important addition to the workup.

The reason for the lack of primary neurodegenerative disorders on biopsy is likely due to a case selection difference and may be a result of time to biopsy from beginning of workup or the difference in a specific dementia tertiary care center versus a general tertiary care center. Treatment change was noted in 4 of the 14 diagnostic biopsies in the Schott et al. series (28%). This is similar to the 4 of 18 diagnostic biopsies in our series (22.2%) and illustrates our primary point that these biopsies rarely alter patient care.

We again commend Schott et al. on their algorithm for approaching these patients. We agree that biopsies play a role in treatment of these patients, but that role is shrinking with improved imaging, serologic and other studies. Additionally, we feel that treatment is difficult because both studies show that only 20-30% of patients actually receive any benefit even when a diagnosis is made.

Disclosures: See original article for full disclosure list.

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Neurology | Print ISSN:0028-3878
Online ISSN:1526-632X

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