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Abstract Details

Grey Matter Atrophy and Its Clinical Correlates in Myotonic Dystrophy Type 1: A Voxel-Based Morphometry Study
Muscle Disease/Neuromuscular Junction
(-)
007
DM1 patients often show cognitive impairment during the course of disease, beside the motor symptoms. To date, a few MRI studies have been conducted on relatively small samples of DM1 patients showing brain structural abnormalities not always related to clinical measures.
We enrolled 47 DM1 patients [n=14 with childhood-juvenile DM1 (cjDM1); n=33 with classic DM1 (cDM1)] and 41 age and sex-matched healthy controls (HC). Patients underwent clinical and neuropsychological evaluations and brain structural MRI. Voxel based morphometry (VBM) was performed to assess GM volume differences between patients and controls and the associations between GM abnormalities and clinical measures.
DM1 patients presented moderate muscle impairment, sleepiness and depression as well as attentive-executive and visuo-spatial deficits. DM1 subgroups did not differ for clinical measures but cjDM1 patients were significantly younger than cDM1 group. DM1 patients showed a significant and diffuse pattern of atrophy including cortical areas, cerebellum and bilateral thalami compared to HC. CjDM1 patients showed greater atrophy in bilateral frontal and right temporo-occipital regions than cDM1 group. Regression analyses revealed positive correlations between MMSE and GM volumes of the right hippocampus and parahippocampal cortex, cingulate cortex, right cuneus and bilateral superior temporal gyrus. Similar correlations were found between sleepiness and GM loss in bilateral fronto-temporo-parietal regions, left precuneus, thalamus and posterior cingulate cortex. Motor performance was negatively correlated to GM volumes of the bilateral thalami, cerebellum, right middle frontal gyrus and putamen.
Our data demonstrated in vivo the clinical relevance of GM atrophy in a large sample of DM1 patients. These findings are in line with neuropathological studies showing cortico-subcortical neurodegeneration in this multisystemic disease.
Authors/Disclosures
Lidia Sarro, MD (Ospedale San Raffaele)
PRESENTER 		No disclosure on file
Federica Agosta (San Raffaele Scientific Institute) Federica Agosta has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Philips. Federica Agosta has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier INC.
Stojan Peric, MD, PhD Dr. Peric has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Argenx. Dr. Peric has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Dianthus. Dr. Peric has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Takeda. Dr. Peric has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for argenx.
Francesca Caso, MD (Universita' Vita Salute San Raffaele) Dr. Caso has nothing to disclose.
No disclosure on file
Vladimir S. Kostic, MD, PhD (Institute of Neurology CCS) Dr. Kostic has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Roche. An immediate family member of Dr. Kostic has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Alkaloid. The institution of Dr. Kostic has received research support from Ministry of 好色先生, Science and Technological Development of Serbia.
Allison Brashear, MD, MBA, FAAN (Univeristy of Buffalo) Dr. Brashear has received personal compensation for serving as an employee of McKnight Brain Res Found. Dr. Brashear has received personal compensation for serving as an employee of American Board of Psychiatry and Neurology. Dr. Brashear has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for ABPN. Dr. Brashear has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for McKnight Brain Research Foundation i. Dr. Brashear has received personal compensation in the range of $0-$499 for serving as an officer or member of the Board of Directors for Care Directions- start up . Dr. Brashear has stock in Caredirections . The institution of Dr. Brashear has received research support from NINDS. Dr. Brashear has received publishing royalties from a publication relating to health care. Dr. Brashear has received personal compensation in the range of $500-$4,999 for serving as a Special government employee and study section reviewer with NIH. Dr. Brashear has received personal compensation in the range of $0-$499 for serving as a Adminstrative board -travel reimbursement with AAMC.
Massimo Filippi, MD, FAAN (Ospedale San Raffaele, Neuroimaging Research Unit) Dr. Filippi has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion, Almirall, Biogen, Merck, Novartis, Roche, Sanofi. Dr. Filippi has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion, Biogen, Bristol-Myers Squibb, Merck, Novartis, Roche, Sanofi, Sanofi-Aventis, Sanofi-Genzyme, Takeda. Dr. Filippi has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Bayer, Biogen, Celgene, Chiesi Italia SpA, Eli Lilly, Genzyme, Janssen, Merck-Serono, Neopharmed Gentili, Novartis, Novo Nordisk, Roche, Sanofi, Takeda, and TEVA. Dr. Filippi has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer Nature. The institution of Dr. Filippi has received research support from Biogen Idec, Merck-Serono, Novartis, Roche, the Italian Ministry of Health, the Italian Ministry of University and Research, and Fondazione Italiana Sclerosi Multipla.