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Abstract Details

Beyond the Upper Motor Neuron: Extra-motor Involvement in Primary Lateral Sclerosis
Neuromuscular and Clinical Neurophysiology (EMG)
S46 - Neuromuscular and Clinical Neurophysiology (EMG): Motor Neuron/Charcot Marie Tooth (3:42 PM-3:54 PM)
002

Primary lateral sclerosis (PLS) is an idiopathic, progressive motor neuron disease. In contrast with ALS, the defining clinical and neuropathological features of PLS reflect the selective degeneration of upper motor neurons without evident lower motor neuron involvement. However, as upper motor neuron degeneration is not specific to PLS, recognition of distinctive neuroanatomical patterns of disease involvement would have important implications for diagnostic and prognostic biomarker studies.

The objective of this study is to characterise the extra-motor profile of PLS in contrast to ALS and healthy controls.


A prospective, quantitative neuroimaging study was conducted with 49 PLS patients, 100 ALS patients and 100 healthy controls with comprehensive genetic and clinical profiling. Standardised neurological assessments were performed to explore disease-specific characteristics. Surface-based and voxel-based morphometry and tract-based spatial statistics were used to systematically characterise extra-motor grey and white matter degeneration in PLS compared to controls, controlling for differences in disease duration and demographic factors.

The extra-motor profile of PLS included marked insular, inferior frontal and left pars opercularis pathology. Bilateral corticospinal tract alterations were common to both PLS and ALS and did not differ significantly.  In contrast with ALS , extensive bilateral cerebellar grey and white matter degeneration was identified in PLS which was not accounted for by the longer symptom duration in the PLS group in comparison with ALS. Somatosensory cortex and the corpus callosum genu degeneration were detected only in the ALS group despite prolonged disease duration in PLS. Preferential medial motor cortex degeneration was identified in PLS, consistent the observed lower limb predominant clinical involvement reflected in functional-rating scores and examination metrics. Clinical measures showed somatotopic correlations with imaging metrics in PLS.

PLS and ALS have distinguishing imaging signatures. Given the scarcity of post-mortem studies in PLS, imaging provides invaluable pathological insights.

Authors/Disclosures
Eoin Finegan, MBBS
PRESENTER
Dr. Finegan has nothing to disclose.
No disclosure on file
Ibrahim Laleka No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
Colette G. Donaghy, MD (Royal Victoria Hospital Belfast) Colette G. Donaghy, MC MRCP has nothing to disclose.
No disclosure on file
Siobhan Hutchinson, MD (St. James'S Hospital) Dr. Hutchinson has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Hutchinson has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Teva. Dr. Hutchinson has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Teva.
Orla Hardiman, MD, FRCPI, FAAN (Trinity Biomedical Sciences Institute) Dr. Hardiman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Wave Pharmaceuticals. Dr. Hardiman has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cytokinetics . Dr. Hardiman has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Hardiman 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 Taylor and Francis. The institution of Dr. Hardiman has received research support from Science Foundation Ireland. The institution of Dr. Hardiman has received research support from HRB.
Peter Bede, MD, PhD (Academic Unit of Neurology) Dr. Bede has nothing to disclose.