Abstract Details

Title Grey-Matter Atrophy in Primary Craniocervical Dystonia

Topic Movement Disorders

Presentation(s) P02 - (-)

Poster/Presentation
Number 085

Objective

Background BACKGROUND: PCCD is the most common adult-onset dystonia. It manifests clinically as blepharospasm, oromandibular, lingual, laryngeal and cervical dystonia either isolated or combined. Prior voxel-based morphometry (VBM) studies described GM changes in patients whit PCCD, however without a clear pattern.

Design/Methods DESIGN/METHODS: We analyzed 30 patients with PCCD (59.47 ± 12.06 years) compared with 105 healthy controls (43.44 ± 16.62 years). All patients had normal TOR1A gene exon 5 sequencing. Disease duration was 10 ± 6.91 years, duration of toxin botulinum therapy 5.47 ± 5.14 years and Marsden-Fahn Scale score was 5.44 ± 2.68. T1-weighted images were performed on a 3 Tesla scanner. We used SPM8/Dartel software implemented in Matlab R2011b for VBM analysis. Homogeneity test using covariance lead to exclusion of 5 patients and 1 control. For the statistical analysis, we performed two-sample test (family-wise error correction with p<0.05) and extent threshold of 20 voxels.

Results RESULTS: In PCCD, VBM demonstrated significant atrophy (clusters ? 100 voxels) of GM at the left hemisphere: temporal pole of the superior temporal gyrus, middle temporal gyrus; middle occipital gyrus; inferior frontal gyrus (orbital part and triangular part), superior medial frontal gyrus and dorsolateral prefrontal cortex. In the right hemisphere the clusters were manly in the frontal lobe, however too small to be reported. There was no significant GM excess or significant correlation between clinical and imaging findings.

Conclusions CONCLUSIONS: We were able to demonstrate significant brain atrophy in multiple cortical areas in patients with PCCD. Interestingly, there was a clear lateralization towards the left in our sample and a predominant frontal lobe involvement. Our results are somewhat different than previously reported, however our sample is larger, with well defined and much larger control group.

Authors/Disclosures
PRESENTER	No disclosure on file
Luiza G. Piovesana, MD (Unicamp/ Neurology)	No disclosure on file
Shyam Prabhakaran, MD (University of Chicago)	Dr. Prabhakaran has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for University of Cincinnati. Dr. Prabhakaran has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for University of Cincinnati. The institution of Dr. Prabhakaran has received research support from NIH . The institution of Dr. Prabhakaran has received research support from AHRQ. Dr. Prabhakaran has received publishing royalties from a publication relating to health care.
Maria Cristina J. Santos, MD (R. Dr. Antonio Loureiro Borges)	No disclosure on file
Rachel P. Guimaraes, PT (Unicamp)	Ms. Guimaraes has nothing to disclose.
Paula C. Azevedo, MD	Dr. Azevedo has nothing to disclose.
	No disclosure on file
	No disclosure on file
	No disclosure on file
Iscia Lopes-Cendes, MD, PhD (University of Campinas - UNICAMP)	No disclosure on file
Fernando Cendes, MD, PhD, FAAN (Departamento de Neurologia; FCM; UNICAMP)	Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB Pharma. Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for UCB Biopharma. Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for United Medical – Brazil. Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Zodiac Pharma . Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Eurofarma – Brazil . Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Epilepsia. Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Frontiers in Neurology - Epilepsy. The institution of Dr. Cendes has received research support from São Paulo Research Foundation - FAPESP. The institution of Dr. Cendes has received research support from Conselho Nacional de Desenvolvimento Científico e Tecnológico - Brazil . The institution of Dr. Cendes has received research support from NIH.
Anelyssa Cysne Frota D' Abreu, MD, PhD, MPH, FAAN (Corewell Health Outpatient Neurology)	The institution of Dr. Cysne Frota D' Abreu has received research support from U.S. NIH Institute on Aging. The institution of Dr. Cysne Frota D' Abreu has received research support from Biogen. The institution of Dr. Cysne Frota D' Abreu has received research support from American College Of Radiology. The institution of Dr. Cysne Frota D' Abreu has received research support from COGNITION THERAPEUTICS, INC.. The institution of Dr. Cysne Frota D' Abreu has received research support from Eli Lilly. The institution of Dr. Cysne Frota D' Abreu has received research support from Jansen. Dr. Cysne Frota D' Abreu has received personal compensation in the range of $10,000-$49,999 for serving as a Lecturer with Pri_med.

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