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Abstract Details

Brain White Matter MRI Differentiates Kennedy’s Disease from Other Motor Neuron Disease Clinical Phenotypes
Neuromuscular and Clinical Neurophysiology (EMG)
P6 - Poster Session 6 (11:30 AM-1:00 PM)
15-019

KD is a rare X-linked neurodegenerative disorder mainly affecting the lower motor neurons. Differential diagnosis from amyotrophic lateral sclerosis (ALS) and, particularly, lower motor neuron-predominant disease (LMND) might be challenging. The extent of central nervous system involvement relative to other MND phenotypes still needs to be clarified.

To investigate cortical and white matter (WM) alterations in a sizeable sample of patients with Kennedy’s disease (KD).

Subjects were prospectively enrolled between October 2009 and April 2016. Twenty-five KD patients were compared with 24 healthy subjects, 25 patients with amyotrophic lateral sclerosis (ALS), and 35 with lower motor neuron-predominant disease (LMND). LMND patients were clinically differentiated into 24 fast and 11 slow progressors. All subjects underwent T1-weighted and diffusion tensor (DT) MRI. Whole-brain cortical thickness, WM tract-based spatial statistics, and tractography of the corticospinal tract (CST) were performed. CST measures were compared by using analysis of variance.

No significant difference in terms of cortical thickness was found between groups. ALS patients showed widespread decreased fractional anisotropy and increased mean (MD) and radial diffusivity (radD) in the CST, corpus callosum and fronto-temporal extra-motor tracts, compared with healthy controls and other patient groups. CST tractography showed significant alterations of DT MRI metrics in ALS and LMND-fast patients whereas KD and LMND-slow patients were comparable with healthy controls. LMND-fast patients also showed a nearly-significant (p=0.06) increase in MD values of the right CST and radD values of the left CST relative to KD.

Our study demonstrated the absence of WM abnormalities in patients with KD and LMND-slow, in contrast with diffuse WM damage in ALS and focal CST degeneration in LMND-fast. These results support the use of DT MRI measures as powerful tools to differentiate fast- and slow-progressing MND syndromes, including KD.

Authors/Disclosures
Edoardo G. Spinelli, MD
PRESENTER
Dr. Spinelli has nothing to disclose.
Giorgia Querin, MD (Universita Degli Studi Di Padova) No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
Gianni Soraru No disclosure on file
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.
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.